Pediatric Plastic Surgery

CLEFT LIP

INTRODUCTION

Cleft lip is a separation in one or both sides of the lip that is present at birth. Early in the development of the baby inside the mother, the left and right sides of the face and the roof of the mouth join together or “fuse.” If the two sides do not come together correctly, an opening in the lip may occur.

EPIDEMIOLOGY

Cleft lip is the second most common embryonic (congenital) deformity. Cleft lip occurs in approximately one in 750–1,000 live births. Cleft lip occurs more commonly in males, while cleft palate is more likely to occur in females. Cleft lip alone (without cleft palate) occurs in approximately 20% of cases across both genders. The majority of cases—80%—have both cleft lip and cleft palate. A unilateral cleft lip, commonly occurring on the left side, is more common than a bilateral cleft lip.

CAUSES

Most cases of cleft lip have no known cause. However, there is a strong genetic correlation. Single gene defects that are associated with cleft lip include: Van der Woude syndrome, Aarskog syndrome, Fryns syndrome, Waardenburg syndrome. Approximately 5% of cleft conditions are associated with a genetic syndrome. Most of these syndromes do not include mental retardation.

Facial cleft has been implicated with maternal exposure to environmental causes, such as rubella or medications that can harm the developing embryo. These medications include steroids, antiseizure drugs, vitamin A, and oral anti-acne medications (such as Acutane) taken during the first three months of pregnancy. Cleft lip is also associated with fetal alcohol syndrome and maternal diabetes. Similarly, moms who don’t get the right amount of prenatal nutrients (for example, not enough folic acid) may increase their baby’s risk of having a cleft.

Women who smoke cigarettes, use drugs, or drink alcohol during pregnancy also increase their baby’s risk for birth defects. Research has shown that moms who binge drink (drinking four or more drinks in a short period of time) during the first weeks of pregnancy have a higher risk of having a baby with a facial birth defect like a cleft lip or cleft palate.

Risk of cleft lip increases with paternal age, especially over 30 years at the time of conception.

When the affected child has unilateral cleft lip and palate, the risk for subsequent children increases to 4.2%. Advances in high resolution ultrasonography (prenatal ultrasound exam) have made it possible to detect facial abnormalities in the developing embryo (in utero).

TYPES OF CLEFT LIP

There are several types of cleft lip, ranging from a small groove on the border of the upper lip to a larger deformity that extends into the floor of the nostril and part of the maxilla (upper jawbone).

Unilateral cleft lip

Unilateral cleft lip results from failure of the maxillary prominence on the affected side to fuse with medial nasal prominences. The result is called a persistant labial groove. The cells of the lip become stretched and the tissues in the persistent groove break down, resulting in a lip that is divided into medial (middle) and lateral (side) portions. In some cases, a bridge of tissue (simart band) joins together the two incomplete lip portions.

Bilateral cleft lip

Bilateral cleft lip occurs in a fashion similar to the unilateral cleft. Patients with bilateral cleft lip may have varying degrees of deformity on each side of the defect. An anatomical structure (intermaxillary segment) projects to the front and hangs unattached. Defects associated with bilateral cleft lip are particularly problematic due to discontinuity of the muscle fibers of the orbicularis oris (primary muscle of the lip.) This deformity can result in closure of the mouth and pursing of the lip.

In addition to classification as unilateral or bilateral, cleft lips are further classified as complete or incomplete.

Complete cleft lip

A complete cleft involves the entire lip, and typically the alveolar arch.

Incomplete cleft lip

An incomplete cleft involves only part of the lip.

INDICATIONS FOR SURGERY

A cleft lip does not join together (fuse) properly during embryonic development. Surgical repair corrects the defect, preventing future problems with breathing, speaking, and eating, and improving the person’s physical appearance. CONSULTATION

Facial clefts have a wide range of clinical presentations, ranging from a simple microform cleft to the complete bilateral cleft involving the lip, palate, and nose. A comprehensive physical examination is performed immediately after birth, and the defect is usually evident by visual inspection and examination of the facial structures.

Care must be taken to diagnose other physical problems associated with a genetic syndrome. Weight, nutrition, growth, and development should be assessed and closely monitored.

Presurgical tests include a variety of procedures, such as hemoglobin studies. It is important for the patient’s parents and physician to discuss the operation prior to surgery.

TIMING OF THE SURGERY

The cleft lip usually is corrected early in a child’s life, between 3 to 6 months of age, but sometimes later.

LOCATION

The plastic and reconstructive surgeon performs the procedure in a state of the art surgical suite at IASO Children’s hospital which is equipped with a surgical department.PREPARATION OF THE SURGERY

When general anesthesia is needed, there are important rules for eating and drinking that must be followed in the hours before the surgery. One business day before your child’s surgery, you will receive a phone call from a nurse between the hours of 1 and 9 p.m. (Nurses do not make these calls on weekends or holidays.) Please have paper and a pen ready to write down these important instructions. If these instructions are not followed exactly, it is likely your child’s surgery will be cancelled.

The nurse will give you specific eating and drinking instructions for your child based on your child’s age. Following are the usual instructions given for eating and drinking. No matter what age your child is, you should follow the specific instructions given to you on the phone by the nurse.

For children older than 12 months:fter midnight the night before the surgery, do not give any solid food or non-clear liquids. That includes milk, formula, juices with pulp, and chewing gum or candy.

For infants under 12 months:

Up to 6 hours before the scheduled arrival time, formula-fed babies may be given formula.
Up to 4 hours before the scheduled arrival time, breastfed babies may nurse.

For all children:

Up to 2 hours before the scheduled arrival time, give only clear liquids. Clear liquids include water, Pedialyte, Kool-Aid and juices you can see through, such as apple or white grape juice.
In the 2 hours before the scheduled arrival time, give nothing to eat or drink.
You may bring along a “comfort” item — such as a favorite stuffed animal or “blankie” — for your child to hold during the surgery.
You should bring a long-sleeve T-shirt, slightly larger than your child’s usual size, to the hospital on the day of surgery. It will help make your child more comfortable on the day you take your child home from the hospital.
You may want to purchase a bottle of hydrogen peroxide, a tube of antibiotic ointment, such as bacitracin, and a box of Q-Tips to have on hand so you can take care of your child’s lip and nose when you get home from the hospital.

DAY OF THE SURGERY

Your child’s cleft lip repair will be done at state of the art surgical suites at IASO PAIDON HOSPITAL. When you have checked in at the Same Day Surgery Center, you and your child will be called to an examination room where your child’s health history will be taken and vital signs will be checked.

You will meet with one of the doctors on your child’s surgical team to go over the surgery. He or she will answer any last-minute questions you might have at this time. A member of the anesthesia staff also will meet with you and your child to review his or her medical information and decide which kind of sleep medication he or she should get. As the parent or legal guardian, you will be asked to sign a consent form before the anesthesia is given.

When it is time for your child to go the operating room, you will be asked to wait in the surgical family waiting area.

If your child is very scared or upset, the doctor may give a special medication to help him or her relax. This medication is flavored and takes effect in 10 to 15 minutes.
If relaxation medicine is needed, you may stay with your child as he or she becomes drowsy; you will be asked to wait in the surgical waiting area when your child is ready to move to the operating room.
Young children get their sleep medication through a “space mask” that will carry air mixed with medication. Your child may choose a favorite scent to flavor the air flowing through the mask. There are no shots or needles used while your child is still awake.
Once your child is asleep, an intravenous (in-tra-VEE-nuss) or IV line will be inserted into a vein in your child’s arm or leg so that medication can be given to keep him or her sleeping throughout the surgery. Your child will have no pain during the surgery and no memory of it afterward.

THE SURGERY

While the patient is under general anesthesia, the anatomical landmarks and incisions are carefully demarcated with methylene blue ink. An endotracheal tube prevents aspiration of blood. The surgical field is injected with a local anesthestic to provide further numbing and blood vessel constriction (to limit bleeding). Myringotomy (incisions in one or both eardrums) is performed, and myringotomy tubes are inserted to permit fluid drainage.

There are several operative techniques for cleft lip reconstruction. The Millard rotation advancement (R-A) technique is the most widely accepted form of repair. This method involves rotation of the entire philtral dimple (groove in the upper lip) and Cupid’s bow (double curve of the upper lip). The scar falls along the new philtral column (central section of the upper lip), and is adjusted as required since the procedure allows for flexibility.

Nasal deformity can be dealt with by a procedure known as the McComb nasal tip plasty, which elevates the depressed nasal dome and rim. Cartilage from the cleft side is freed from the opposite side, and is positioned and reshaped using nylon sutures.

Cupid’s bow is a critical part of the repair, making it very important to accurately determine the high point of Cupid’s bow on the lateral lip.

LEGEND: The edges of the cleft between the lip and nose are cut (A and B). The bottom of the nostril is formed with suture (C). The upper part of the lip tissue is closed (D), and the stitches are extended down to close the opening entirely (E).

IMMEDIATE RECOVERY

When your child is moved to the recovery room, you will be called so that you can be there as he or she wakes up.

Your child will need to stay in the recovery room to be watched until he or she is alert and vital signs are stable. The length of time your child will spend in the recovery room will vary because some children take longer than others to wake up after anesthesia.
Your child will still have the IV in. A nurse will remove it before your child leaves the hospital, when he or she drinking well.
Your child will have set of padded arm restraints called “no-no’s” placed on his or her arms to prevent them from bending at the elbow. These no-no’s will need to stay in place for about 2 weeks as the surgical scar heals.
Children coming out of anesthesia may react in different ways. Your child may cry, be fussy or confused, feel sick to his or her stomach, or vomit. These reactions are normal and will go away as the anesthesia wears off.
You may notice some swelling around your child’s mouth, lips and eyes, as well as some dried blood or oozing where the cleft lip was repaired. The swelling may look worse on the day after the surgery, but it will go down over the next weeks.
You may see sutures on the outside of the skin. Sometimes, only “dissolvable” sutures will be used, and these sutures do not need to be removed. As the skin heals, the parts of the sutures on the inside of the lip and mouth will dissolve on their own, and the parts you can see on the outside of the skin will dry up and fall off. If non-dissolvable sutures were used, they will be removed by the doctor at your child’s first follow-up visit.
Your child may have a nasal retainer in place to help reshape the nose during healing. The retainer, which acts as a splint inside the nose, may stay in place for up to 3 months.
Your child can be given pain medication every 4 to 6 hours, as needed, when he or she wakes up.
When your child is alert, he or she will be moved to a hospital room so the nursing staff can continue his or her care. If you need help, the nurse will show you how to feed your child and clean his or her scar so that you will become comfortable caring for your child at home.

RECOVERY AT HOME

After the surgery, and for the weeks afterward at home, your child will only be allowed to drink liquids or semi-liquids from a bottle or cup. No utensils or straws should be used until your child’s surgeon says it is OK.

Within the first 24 hours after the surgery, while your child is still in the hospital, he or she will be allowed to drink clear liquids from a bottle or cup.
Your child will stay in the hospital until he or she is drinking well and urinating normally.
When your child is drinking well, the IV will be removed by a nurse before you leave the hospital.
Some children will need to stay for more than 1 day if they are not drinking and urinating normally, or if their parents need extra time to learn how to care for them.
Your child may be given Tylenol^® with codeine prescription medication for pain relief when he or she leaves the hospital. Over-the-counter Motrin^® can be combined with the Tylenol with codeine if your child is still having pain, but most children only need over-the-counter Tylenol once they get home. DO NOT give your child any over-the-counter Tylenol while he or she is still on Tylenol with codeine.
The long-sleeve T-shirt you brought from home will be used on the day your child goes home. Place the T-shirt on your child, then put the no-no’s on. Roll the cuff of the T-shirt over the edge of the no-no’s and pin them to the T-shirt with a safety pin. Additional safety pins can be used to pin the no-no’s to the T-shirt at the shoulders as well. A nurse will show you what to do if you have any questions.
As soon as you get home, you should call to make an appointment for your child to be checked 1 week after surgery, and to have any sutures removed, if needed.

INSTRUCTIONS

A complete list of instructions for taking care of your child at home will be given to you before you leave the hospital. The main things to remember are:

If you notice any of the following changes in your child, call the surgeon right away:
- Fever higher than 101.4˚F
- Trouble breathing or skin color changes (pale, blue or gray)
- Bleeding or foul-smelling drainage from the scar or nose
- Signs of dehydration, including lack of energy, sunken eyes, dry mouth or not urinating enough/fewer wet diapers
- Any redness, swelling, or any “in-and-out movement” of the nasal retainer
Your child can drink any kind of liquid once he or she gets home. Your child may also eat any kind of food that can be watered down and poured from a cup or bottle, including yogurt, pudding, milkshakes, or anything that you can grind in a blender to be as smooth as baby food. Remember, though—no utensils or straws!
The no-no’s must stay on your child’s arms for at least 2 weeks. You should check them every 2 to 4 hours to make sure they are not too tight, and take them off briefly several times a day to allow your child to bend and move his or her arms. Change the long-sleeve T-shirt after you bathe your child.
Your child may be given an antibiotic for the first couple of weeks after surgery.
Using a clean jar with a lid, mix up a solution of 50 percent hydrogen peroxide and 50 percent water. Using a clean Q-Tip, gently clean the lip area as often as needed, at least 2 or 3 times each day.
If your child had a nasal retainer placed during surgery, you will need to clean it the same way you clean the lip area.
If your child has any non-dissolvable sutures, they will be taken out at the first follow-up visit, approximately 4 to 5 days after surgery. You should make sure that your child has had nothing to eat and is very hungry at the time of the appointment. Bring a bottle or cup of liquid with you to the appointment. The sutures will be taken out while you feed your child so that he or she be distracted and will not feel the sutures being removed.
After the sutures are taken out, continue to wash the lip and nose with the hydrogen peroxide/water mix and apply a light layer of antibiotic ointment for the next 2 days. You will be told when to begin to use a moisturizing lotion or scar cream to moisturize and massage the scar.

RISKS

There may be excessive scarring and contraction of the lips. Two types of scars, hypertrophic or keloid, may develop. Hypertrophic scars appear as raised and red areas that usually flatten, fade in color, and soften within a few months. Keloids form as a result of the accelerated growth of tissue in response to the surgery or trauma to the area. The keloid can cause itching and a burning sensation. Scratching must be avoided because it can lead to healing problems. Some patients require minimal revision surgery, but in most cases, the initial redness and contracture is part of the normal healing process.

Generally, cleft lip repair is well-tolerated in healthy infants. There are no major health problems associated with this reconstructive surgery. Depending on the results, it may be necessary to perform additional operations to achieve desired functional and cosmetic outcomes.

In summary possible cleft surgery risks include:

Bleeding (hematoma)
Infection
Poor healing of incisions
Irregular healing of scars including contracture (puckering or pulling together of tissues)
Residual irregularities and asymmetries
Anesthesia risks
Allergies to tape, suture materials and glues, blood products, topical preparations or injected agents
Damage to deeper structures—such as nerves, blood vessels, muscles and lungs—can occur and may be temporary or permanent
Possibility of revisional surgery

OTHER PROCEDURES

Common surgical procedures that may need to be performed at a later stage in life in children with cleft lip are

Cleft palate repair at 6-9 months (if cleft palate is present)
Alveolar cleft repair at 8-9 years of age (usually if cleft palate is present)
Rhinoplasty around 16 years of age
Scar revisions at any age
Orthognathic surgery after 16 years of age (usually if cleft palate is present)
Orthodontic surgery

HOW TO HELP YOUR CHILD

It can be hard when kids miss school for frequent doctor’s appointments, or when speech problems prevent them from communicating well with their peers. In spite of these and other social, psychological, and educational challenges, kids with clefts just want to be treated like everyone else.

Children may face teasing and bullying because of how a cleft lip and palate look. If your child is teased, talk about it and be a patient listener. Try to give your child tools to confront teasers. Find out what your child would like to say to them and practice it together.

Some kids struggle growing up with a cleft lip or cleft palate and might need help handling certain situations. The psychologists and social workers on the treatment team can help guide you through these difficult times, so turn to them if your child needs added support. The good news is that most kids with cleft lip or cleft palate grow up to be healthy, happy adults.

As your child nears the teen years, remember that your interactions may change. You could be his or her greatest confidante, or your child may not share his or her worries with you. Either way, stay open to communication and support your child with these tips:

Encourage your child to be confidentand not allow the cleft to define who he or she is.
Make your home a warm, supportive, and accepting place where individual worth and accomplishments are celebrated.
Encourage your child’s friendships and relationships. Make friends with people who are different from you and lead by example.
As for all kids, being involved in sports, music, and other activities will go a long way toward helping kids with clefts realize their self-worth.
Give your child the freedom to make decisions and take appropriate risks. Having chances to make decisions early on — like picking out which clothes to wear or foods to eat — gives kids the confidence and skills to make bigger decisions later. It will help them become more independent.
If your child — especially a young child — wants to talk to the class about cleft lip and palate, set up something with the teacher. Consider asking someone from the treatment team to attend. But remember that your child may need an extra boost of confidence to get up in front of the class, so give lots of encouragement.

RESULTS

Ideal surgical results for cleft lip include symmetrically shaped nostrils, and lips that appear as natural as possible and have a functional muscle. Many characteristics of the natural lip can be achieved; however, the outcome ultimately depends on a number of factors, including the skill of the surgeon, accurate presurgery markings, alignment of bones within the affected area, uncomplicated healing of the initial repair, and the effect of normal growth on the repaired lip. Additional surgical correction to reconstruct nasal symmetry is sometimes necessary.

Case 1

LEGEND: Correction of bilateral cleft lip at 3 months of age. Upper left photo before the surgery, Upper right photo markings for the incisions (Mulliken repair), Bottom photo right after surgery

Case 2

LEGEND: Correction of incomplete unilateral cleft lip at 4 months of age. Upper left photo before the surgery, Upper right photo markings for the incisions (Millard repair), Bottom photo at 3 years after surgery just before an otoplasty repair

CLEFT PALATE

INTRODUCTION

A cleft palate is a situation when a baby is born with an opening in the or roof of the mouth (palate). Cleft palate is commonly associated with cleft lip and is one of the most common birth defects.

Cleft palate is treatable. Most kids can have surgery to repair them within the first year or two of life.

CAUSES

Doctors don’t always know why a baby develops cleft lip or cleft palate, but many clefts are thought to be a combination of genetic (inherited) and environmental factors (like certain medications orvitamin deficiencies). Both mothers and fathers can pass on a gene or genes that cause cleft palate or cleft lip.

Taking certain medicines (such as some anti-seizure medications) during pregnancy can increase a woman’s chances of having a baby with a cleft lip or palate. Similarly, moms who don’t get the right amount of prenatal nutrients (for example, not enough folic acid) may increase their baby’s risk of having a cleft. A mother’s exposure to certain chemicals also may cause a cleft.

TYPES OF CLEFT PALATE

LEGEND: Most common types of cleft palate.

Unilateral cleft lip with alveolar cleft (partial cleft palate)
Bilateral cleft lip with alveolar cleft
Unilateral cleft lip with unilateral cleft palate (partial cleft palate)
Bilateral cleft lip with bilateral cleft palate
Cleft palate alone

TREATMENT

If your child has a cleft palate and/or cleft lip, your pediatrician will talk to you about a cleft lip and palate treatment team. This team of health care professionals will work together to meet your family’s needs and, ultimately, help your child transition to adult care.

Besides the pediatrician, your child’s treatment team may include:

Plastic surgeon
Ear, nose, and throat physician (otolaryngologist)
Oral surgeon
Orthodontist
Dentist
Speech-language pathologist
Audiologist
Nurse
Social worker
Psychologist or therapist
Geneticist

Your child might visit this care team a few times a year, depending on his or her needs. Who is seen during a visit depends on the care team’s structure and your child’s needs. The team coordinator will help organize the visits, and the team should communicate with your doctor about the treatment plan.

SURGERY

A cleft lip is usually surgically repaired in the hospital using general anesthesia when a child is 6 to 9 months old.

Plastic surgeons connect the muscles of the soft palate and rearrange the tissues to close the cleft. This surgery requires general anesthesia and a short hospital stay for recovery.

LEGEND: Basic explanation of the surgery. Incisions are made on each side of the two palate parts and the mucosal and muscular tissues are mobilized towards the middle in order to close the gap

The goal of surgery is to create a palate that works well for speech. Some kids, however, will continue to sound nasal after cleft palate repair, and some may develop a nasal voice later on.

More surgeries may be needed as children grow older and their facial structure changes — this can include surgeries like pharyngoplasty, which helps improve speech, or alveolar bone grafts, which help provide stability for permanent teeth. A bone graft closes gaps in the bone or gums near the front teeth and is usually done when kids are between 6 and 10 years old.

As children become teens, they will likely want to (and should) be more involved in their care. They may want to have their scars made less noticeable, improve the appearance of their nose and upper lip, or improve their bite with orthognathic surgery. These operations may improve speech and breathing, overbites/underbites, and appearance.

DENTAL / ORTHODONTIC TREATMENT

Maintaining healthy teeth and preventing cavities is very important for kids with cleft lip and palate, who can have more dental problems than other kids. They should see a dentist regularly and brush and floss every day.

Kids with cleft lip and palate may begin orthodontic treatment as early as 6 years of age. It may start with palatal expansion, a process that makes the width of the palate normal. Later, it may involve braces to position the teeth. Your orthodontist will discuss these issues with you.

Some kids with a cleft might be missing a permanent tooth, which can be replaced with a removable appliance or, in early adulthood, a dental implant.

SPEECH THERAPY

Some kids will need speech therapy after a repair is done. The speech-language pathologist will closely monitor your child’s progress and talk with you about whether therapy is needed.

RECOVERY AT HOME

Within the first 24 hours after the surgery, while your child is still in the hospital, he or she will be allowed to drink clear liquids from a bottle or cup.
Your child will stay in the hospital until he or she is drinking well and urinating normally.
When your child is drinking well, the IV will be removed by a nurse before you leave the hospital.
Some children will need to stay for more than 1 day if they are not drinking and urinating normally, or if their parents need extra time to learn how to care for them.
Your child may be given a painkiller with codeine prescription medication for pain relief when he or she leaves the hospital.
The long-sleeve T-shirt you brought from home will be used on the day your child goes home. Place the T-shirt on your child, then put the no-no’s on. Roll the cuff of the T-shirt over the edge of the no-no’s and pin them to the T-shirt with a safety pin. Additional safety pins can be used to pin the no-no’s to the T-shirt at the shoulders as well. A nurse will show you what to do if you have any questions.
As soon as you get home, you should call to make an appointment for your child to be checked 1 week after surgery, and to have any sutures removed, if needed.

INSTRUCTIONS

A complete list of instructions for taking care of your child at home will be given to you before you leave the hospital. The main things to remember are:

If you notice any of the following changes in your child, call the surgeon right away:
- Fever higher than 101.4˚F
- Trouble breathing or skin color changes (pale, blue or gray)
- Bleeding or foul-smelling drainage from the scar or nose
- Signs of dehydration, including lack of energy, sunken eyes, dry mouth or not urinating enough/fewer wet diapers
- Any redness, swelling, or any “in-and-out movement” of the nasal retainer
Your child can drink any kind of liquid once he or she gets home. Your child may also eat any kind of food that can be watered down and poured from a cup or bottle, including yogurt, pudding, milkshakes, or anything that you can grind in a blender to be as smooth as baby food. Remember, though—no utensils or straws!
The no-no’s must stay on your child’s arms for at least 2 weeks. You should check them every 2 to 4 hours to make sure they are not too tight, and take them off briefly several times a day to allow your child to bend and move his or her arms. Change the long-sleeve T-shirt after you bathe your child.
Your child may be given an antibiotic for the first couple of weeks after surgery.
Using a clean jar with a lid, mix up a solution of 50 percent hydrogen peroxide and 50 percent water. Using a clean Q-Tip, gently clean the lip area as often as needed, at least 2 or 3 times each day.
If your child had a nasal retainer placed during surgery, you will need to clean it the same way you clean the lip area.
If your child has any non-dissolvable sutures, they will be taken out at the first follow-up visit, approximately 4 to 5 days after surgery. You should make sure that your child has had nothing to eat and is very hungry at the time of the appointment. Bring a bottle or cup of liquid with you to the appointment. The sutures will be taken out while you feed your child so that he or she be distracted and will not feel the sutures being removed.
After the sutures are taken out, continue to wash the lip and nose with the hydrogen peroxide/water mix and apply a light layer of antibiotic ointment for the next 2 days. You will be told when to begin to use a moisturizing lotion or scar cream to moisturize and massage the scar.

RISKS

In cleft palate surgery, the goal is to close the palate so the child can eat and learn to speak properly. The soft palate is the mobile, functional part of the palate that works in coordination with the posterior pharynx to regulate speech sounds. It is frequently closed during infancy prior to the closure of the hard palate. Separation of the palate repair can occur secondary to feeding trauma, infection, or surgical tension on the repair. These occurrences will necessitate secondary surgery. Even in the presence of a completely successful anatomic closure of the palate, speech development may be abnormal and necessitate secondary surgery (in around 10% of patients).

In summary possible cleft surgery risks include:

Bleeding (hematoma)
Infection
Oronasal fistula (small communication between the nose and the mouth)
Poor healing of incisions
Irregular healing of scars including contracture (puckering or pulling together of tissues)
Residual irregularities and asymmetries
Anesthesia risks
Allergies to tape, suture materials and glues, blood products, topical preparations or injected agents
Damage to deeper structures—such as nerves, blood vessels, muscles and lungs—can occur and may be temporary or permanent
Possibility of revisional surgery

There are no alternatives for this surgery. Obvious deformity and impairments of speech, hearing, eating, and breathing occur as a direct result of the malformation. These issues cannot be corrected without surgery.

OTHER PROCEDURES

Common surgical procedures that may need to be performed at a later stage in life in children with cleft palate are

Alveolar cleft repair at 8-9 years of age (usually if cleft palate is present)
Rhinoplasty around 16 years of age
Scar revisions at any age
Orthognathic surgery after 16 years of age (usually if cleft palate is present)
Orthodontic surgery

HOW TO HELP YOUR CHILD

Encourage your child to be confidentand not allow the cleft to define who he or she is.
Make your home a warm, supportive, and accepting place where individual worth and accomplishments are celebrated.
Encourage your child’s friendships and relationships. Make friends with people who are different from you and lead by example.
As for all kids, being involved in sports, music, and other activities will go a long way toward helping kids with clefts realize their self-worth.
Give your child the freedom to make decisions and take appropriate risks. Having chances to make decisions early on — like picking out which clothes to wear or foods to eat — gives kids the confidence and skills to make bigger decisions later. It will help them become more independent.
If your child — especially a young child — wants to talk to the class about cleft lip and palate, set up something with the teacher. Consider asking someone from the treatment team to attend. But remember that your child may need an extra boost of confidence to get up in front of the class, so give lots of encouragement.

RESULTS

The outcome of your child’s initial cleft lip and/or cleft palate repair will make a vast difference in his or her quality of life, ability to breathe, eat and speak. However, secondary procedures may be needed for functional reasons or to reﬁne appearance. The scars of a cleft palate repair are generally located within the normal contours of the mouth, they are not visible.

LEGEND: Left: unilateral cleft palate and incomplete cleft lip of a 3 month old boy. Right: photo of the boy at 3 years of age after repair of cleft lip at 3 months and the cleft palate at 6 months

CONGENITAL FACIAL PARALYSIS / MOEBIUS SYNDROME

INTRODUCTION

Congenital facial paralysis is a rare form of facial palsy affecting children at birth. To make a diagnosis, extensive testing, including physical examinations, is necessary to exclude other conditions. When a child is born with congenital facial paralysis, it’s somewhat common for other disorders to be present, including microtia, cleft palate, deformed extremities, and other muscle paralysis.

Congenital facial paralysis is organized into several different classifications, including:

Unilateral or Bilateral
Traumatic or Developmental
Complete or Incomplete
Syndromic (Mobius Syndrome) or nonsyndromic

LEGEND: Example of right sided unilateral congenital facial nerve palsy

MOEBIUS SYNDROME

Moebius syndrome is a form of facial paralysis that can be present at birth, which involves the absence of the necessary 6thand 7th cranial nerves for optimal and normal facial muscle function, particularly in relation to the eyes. Children with Moebius syndrome often experience great difficulty controlling eye movements on the affected side and making facial expressions. Other symptoms of the condition include:

Inability to smile
Inability to frown
Inability to blink
Inability to move eyes laterally
Inability to make ‘sucking’ mechanism

To treat children dealing with Moebius Syndrome, Dr. Azizzadeh enlists the help of his team of experts ranging from neurologists, ophthalmologists, and physical therapists to provide the highest level of care possible.

LEGEND: Example of child with Mobius syndrome

CAUSES

A lack of proper nerve and/or muscle development causes some cases of congenital palsy. The reason for this is unknown. Other palsies may result from stretching of the muscles or nerves during the birthing process. The incidence of facial palsy is rare. The chance of producing a child with Moebius syndrome is very rare. The incidence of other forms of congenital facial palsy is approximately 2 in every 1000 births. Most congenital facial palsies have no apparent cause but can occasionally be associated with syndromes of the head and neck.

SPECIALTIES INVOLVED

Congenital facial paralysis has several treatment options, including rehabilitative therapy and surgery. A multidisciplinary approach to your facial paralysis may include specialist from:

Plastic surgeons
Otolaryngologists
Neurologists
Ophthalmologists
Speech pathologists

Indications

Traumatic congenital facial nerve paralysis usually resolves spontaneously and does not require surgery. A general guideline when considering surgery in a traumatic facial paralysis patient is to determine if clinical and electrophysiologic tests reveal (1) complete unilateral paralysis (H-B grade VI), (2) evidence of temporal bone trauma based upon CT scanning and physical examination, (3) complete loss of function of the facial nerve at age 3-5 days, and (4) absence of improvement by age 5 weeks.

Surgery in patients with developmental facial paralysis is usually delayed until later in life.

diagnostic examinations

Most common tests include:

CT scan of the temporal bone
MRI of the Head and Neck to check for aplasia or hypolasia of the facial nerve
Electrophysiologic studies (EMG/NCS)
Genetic testing (FISH test) in some syndromic cases

These tests to be performed in this young age require anesthesia

TREATMENT

General

Treatment protocol for patients with congenital facial palsy is rapidly changing. Historically, patients would not be treated until they reached adulthood. Due to advancements in microsurgical techniques over the past decade, we have begun to treat children at an earlier age to help them get a head start in blending in psychosocially.

One of the most important factors of treating a younger patient dealing with facial palsy is the development of a completely safe and customized treatment plan, as this will allow for the best results in regards to improved facial reanimation and facial symmetry possible.

For infants with newly diagnosed facial palsy, eye protection is the primary concern. Lubricants are usually sufficient to prevent injury to the eye. When lubrication is not adequate, then the eyelids are partially sewn shut. This procedure called tarsorrhapy does not block the child’s vision.

A watchful, conservative approach is usually best for a child with congenital facial palsy.

Timing

Since many children improve, treatment should not start before the age of five or six except in the case of the eye as mentioned above. Once the child has reached the age of five or six, there are several treatment options available.

Methods

Two methods of treatment are static slings and dynamic muscle transfers.

Static slings

Static slings involve procedures in which a patient’s own tissue is used to elevate the sagging portions of the face. These slings may be applied to the portion of the face that produces a smile, as well as the eyelid region. These static slings improve facial balance and eyelid closure.

Dynamic procedures

Dynamic procedures include muscle transfers and man-made devices to improve lid closure. Muscle transfers involve moving locally available muscles, such as those for chewing, to substitute for non-functioning or absent facial muscles. Once the transfer is made, the patient relies on the act of 5 biting to contract and bring on a smile or to cause the eyelid to close.

Very sophisticated methods of muscle transfers have been developed. The “gold standard” at the present is a two-staged procedure. Nerves are first transferred from “the good side of the face” to the paralyzed side of the face. After this, a muscle transfer is done to reproduce a smile effect. Using a microscope, this muscle is transferred and hooked up to the nerve grafts. If this is successful, nerve activity from the “good side of the face” travels instantly through the nerve grafts to the new muscle on the opposite side. This can then cause a motion. However, this motion is, at best, unrefined because a few nerves and one muscle are being asked to take the place of many muscles that work together during normal facial expression.

LEGEND: Τhe double team approach to the surgical treatment. One team prepares the face to receive the muscle transplant, the other team harvests the muscle transplant (gracilis muscle) from the leg

Treatment of Mobius syndrome and bilateral facial palsy

When there is not a side of the face with normal motion as in the case of Moebius syndrome or bilateral facial palsy, muscles can be transferred using a microscopic technique. They are then connected to the masseteric nerves, nerves that activate biting muscles if done in a sequence on both sides. Facial motion can be restored, but the patient must bite in order to activate the muscles.

Patients with Moebius or bilateral facial paralysis require gracilis muscle flaps that are attached and controlled by the masseteric nerve as early as age 5. Patients with unilateral facial paralysis can potentially be treated with a combination of the masseteric to facial nerve transfer in with a cross facial nerve grafts or gracilis free flaps as early as age 2.

Treatment for the eyelids

Procedures have also been developed to improve eyelid closure. One method involves placing gold weights in the upper eyelid to help it close when the lid is relaxed. The use of surgical springs can accomplish the same thing.

RECOVERY

Depending on the procedure used for the surgical treatment the child may have to stay in the hospital from one day to a week for a complete recovery

The postoperative care of the newborn after facial rehabilitation is similar to any other surgical procedure. The child (if age appropriate) and parents should be instructed on exercises to improve facial rehabilitation.

After facial reanimation, return of some function has been found to occur within 18 months. Long-term treatment involves evaluating for any donor site morbidity, including tongue atrophy in patients with facial nerve–hypoglossal nerve transposition, difficulty with mastication in patients with masseter or temporalis transfer, and examination of the donor sites for the nerve grafts.

Routine ophthalmologic examinations and physical therapy for facial expression exercises are included in the long-term treatment of patients.

As the child ages, biofeedback can be used to facilitate training of the muscles after cable grafts, facial nerve–hypoglossal nerve transposition, and muscle transfers.

In children with developmental facial nerve paralysis who often have other congenital abnormalities, attention to appropriate weight gain and developmental milestones is necessary.

RISKS

The complications of facial reanimation in the early postoperative period include

Infection
Hematoma
Facial paralysis on the unaffected side in the case of a cross-facial graft
Loss or failure of the muscle transfer

Long-term complications relate to the

Failure of the reanimation technique and
Lingual atrophy when a facial nerve-hypoglossal nerve transfer has been performed.

RESULTS

The success of the operations varies from patient to patient. The success is dictated by the severity of the facial weakness. Normal, facial motion depends on multiple facial muscles and nerves working together to produce a full spectrum of motion. Presently, procedures result in a replacement of a portion of this facial activity. Complete normalization of the facial motion is rarely possible. Due to the many microscopic techniques, there is a chance that nerve growth may not be complete or that circulation to the transferred muscle may fail. This can result in no marked improvement. This occurs in 5%-10% of patients

CONGENITAL HAND DEFECTS

INTRODUCTION

Congenital deformities of the hand-that is, deformities a child is born with-can interfere with proper hand growth and cause significant problems in the use of the hand.

CLASSIFICATION

The classifications for hand deformities can vary. The classifications listed below have been accepted by the American Society for Surgery of the Hand.

Problems in development of the parts. This occurs when parts of the body stop developing while the baby is in the womb, causing either a complete absence of a part of the body, such as the hand, or a missing structure, such as part of the arm bone. In the case of the complete missing part, surgery is not indicated. These children may be introduced to prosthetic devices early in their childhood.

Failure of parts of the hand to separate. With this type of deformity, the parts of the hand, either the bones and/or the tissues, fail to separate in the womb. The most common type of this classification is syndactyly. Syndactyly is the most common congenital hand deformity, in which two or more fingers are fused together. There is a familial tendency to develop this deformity. If the fingers are completely fused together, it is considered complete. There are two types of syndactyly, including the following:

Simple syndactyly. This involves fusion between only the tissues of the fingers.

LEGEND: Preoperative case of syndactyly at 1 year of age (left). Postoperative photo at 3 years of age (right)

Complex syndactyly. This involves fusion between the bones. Another example of failure of the hand to separate is seen in contractures of the hand. Contractures of the hand may also develop as a result of failure of the cells to differentiate in utero. A contracture is an abnormal pulling forward of the digits of the hand, usually caused by problems with the muscles or skin. One of the common types of this classification includes congenital triggering. Congenital triggering occurs when one of the digits is unable to extend. It is usually seen in the thumb. It may take some time in the child’s development before it is noted that the child can not extend the thumb. Some of these cases improve on their own. Surgery is usually not performed until the second year of life, but preferably before the age of 3.

Duplications of digits. Duplication of digits is also known as polydactyly. The little finger is the finger that is most often affected.

LEGEND: Case of thumb polydactyly

Undergrowth of digits. Underdeveloped fingers or thumbs are associated with many congenital hand deformities. Surgical treatment is not always required to correct these deformities. Underdeveloped fingers may include the following:
- The digit is small
- Muscles are missing
- Bones are underdeveloped or missing
- There is complete absence of a digit
Overgrowth of digits. Overgrowth of digits is also known as macrodactyly, which causes an abnormally large digit. In this situation, the hand and the forearm may also be involved. In this rare condition, all parts of the finger (or thumb) are affected; however, in most cases, only one digit is involved (usually the index finger). Surgical treatment of this condition is complex and the outcomes may be less than desirable. Sometimes, amputation of the enlarged digit is recommended.

Congenital constriction band syndrome. This occurs when a tissue band forms around a digit (finger) or limb (arm), causing problems that can affect blood flow and normal growth. Ring constrictions are congenital (present at birth). This condition may be associated with other problems, such as clubfoot, cleft lip, cleft palate, or other craniofacial anomalies. The cause of the ring constrictions is unknown. Some theories suggest that amniotic banding may lead to ring constrictions around a finger or limb.

With this condition, there are four degrees of severity, including the following:

Simple constrictions
Constrictions that cause deformities beyond the constriction
Constrictions that are associated with the fusion of parts of the finger
Constrictions so severe that part of the finger will need to be amputated from the process

Other generalized problems with the skeletal system. These are a rare and complex group of problems.

DIAGNOSIS

In addition to an initial consultation appointment, additional diagnostic imaging tests may be ordered. These include:

X-ray:Images used to evaluate bones for deformity.
CT Scan:For more detailed imaging.

LEGEND: X-ray of simple syndactyly

TREATMENT

Limb manipulation and stretching
Splinting and Occupational Therapy of the affected limbs
Tendon transfers
External appliances (to help realign misshapen digits or hands)
Physical therapy (to help increase the strength and function of the hand)
Correction of contractures
Skin grafts. These involve replacing or attaching skin to a part of the hand that is missing skin or has been removed during a procedure.
These may be used when surgery is not an option, or in addition to surgical correction.

LEGEND: Correction of syndactyly

LEGEND: Incisions for correction of syndactyly

CONGENITAL MELANOCYTIC NEVUS (CMN)

DEFINITION OF CMN

Nevus (plural, nevi) in the broad sense, means “birthmark”, and can be used to describe virtually any mole or birthmark.

If it is present at birth, it is called congenital. One in every 50 to 100 people is born with a small CMN.
If a birthmark is caused by a pigment-producing cell, it is called a pigmented or Melanocytic Nevus.
Congenital Melanocytic Nevi can range in size from the small, common ones the size of a pinhead, up to large, rare forms that cover much of the body surface. The largest ones are described medically as giant nevi, and happen approximately once in every 20,000 births; the larger they are, the rarer.

PRESENTATION

Every congenital melanocytic nevus is unique. People of all races and colors of skin can be born with one. CMN generally grow in proportion to the child. In other words, they will usually continue to cover the same proportional part of the body surface as at birth—for instance, a part of a limb, or extending between the shoulder blades and the upper thigh.

Site:

Congenital melanocytic nevi can be on any part of the skin, including palms, soles and scalp, and even inside the mouth.

Color:

Usually some sort of shade of brown to black, sometimes congenital melanocytic nevi can be reddish. It is often very mixed inside the nevus, with smaller patches of different colors on a solid background. CMN at birth are often deep black, or even dark purple/red as they have many blood vessels visible through a thin surface. They can change color over time, as the child grows. When they change, they usually become paler, but can also become darker for those that were initially light or speckled. Lightening appears to happen more in children with light or red hair and pale skin than in those with darker hair and skin tone.

Hairiness:

Congenital melanocytic nevi usually have hair growing out of them, although this is often not visible at birth and can remain unnoticeable throughout life. The hair color can be darker than the child’s head hair, or the same, or very occasionally lighter. If a CMN is on the scalp, there is usually more luxuriant hair growth over the lesion and the hair grows faster than on the rest of the scalp. Body CMN—be they small or large—can develop terminal hair, which is more visible and often dense. Occasionally, congenital melanocytic nevus are completely hairless, even ones that occur in the scalp, or can have patchy hair loss or hairs without pigment (grey or white hair).

Texture:

The texture of large congenital melanocytic nevi tends to be different from that of normal skin, sometimes being softer, looser and more wrinkled. The CMN can be nearly flat or can be very folded or lumpy.

Number:

The total number of visible nevi can continue to increase after birth, although this does not always happen. When it does, they often develop most during the first few years of life, when the rate of appearance of different ones then usually tapers off. However, they are always relatively small. Such “new” nevi, sometimes called “satellites”, are considered by doctors to also be tardive. That is, they may have already been there as scattered, unpigmented congenital melanocytic nevi at the time of birth. Appearance of these additional nevi is more likely to occur in children with very large and multiple CMN, but in themselves are not a reason for concern.

ETIOLOGY

Research into the molecular and embryological causes of congenital melanocytic nevus has made great strides in the past few years.

Both CMN and acquired melanocytic nevi are associated with somatic mutations in intracellular proteins.

Large/giant CMN are usually sporadic, but familial occurrence has been rarely reported.

Multifactorial inheritance means that many factors, including a combination of genes from both parents, in addition to unknown environmental factors, cause the condition.

AFFETCED POPULATIONS

People of all races and colors of skin can be affected with large/giant CMN. A slight female preponderance has been noted in large/ giant CMN (male/female ratio of 1 to 1.4), but is not observed in the smaller population of people affected by NCM.

DIAGNOSIS

Making the diagnosis of a CMN is most often done by examining clinical and dermoscopic features. The larger CMN can easily be diagnosed based solely on their size. For smaller CMN, their history of presence since birth, surface topography, presence of hair or globular dermoscopic pattern can assist in diagnosis. When biopsied, the histological features of CMN are similar to those found in common acquired nevi which arise later in life

CLASSIFICATION

Congenital melanocytic nevi may be divided into the following types:

Small-sized congenital melanocytic nevusis defined as having a diameter less than 2 cm.
Medium-sized congenital melanocytic nevusis defined as having a diameter more than 2 cm but less than 20 cm.
Giant congenital melanocytic nevus is defined by one or more large more than 20cm, darkly pigmented and sometimes hairy patches.

MEDICAL PROBLEMMS ASSOCIATED WITH CMN

Cutaneous Problems

Fragility
Dryness
Decrease in subcutaneous fat
Absence of sweat glands

Neurological Problems

Problems in the brain or spinal cord are the most common serious complication seen in children with congenital melanocytic nevus. Because the pigment cells found in the skin and (yes, normally in) the meninges develop from the same cells as the brain in the early embryo, people with CMN can also have differences in their brain or spinal cord. Approximately one in fifteen to twenty people with large and giant forms of CMN have excess pigment cells in the brain or spinal cord. This can lead to a complication known as neurocutaneous melanocytosis (NCM).

Should a Person with a CMN have Magnetic Resonance Imaging (MRI)?

Current recommendations are that any child born with two or more congenital melanocytic nevus in any location should have a routine MRI scan of the brain and spine, preferably by the age of six months, though there is no strict cut-off age.

We also recommend that children with congenital melanocytic nevi larger than 20 cm (8 inches) projected adult diameter be followed up regularly to watch their neurological development, even if they do not have more than one nevus. In addition, any child with any type of CMN who has neurological symptoms like those described above should have an MRI.

We recommend that children who develop any new neurological problems such as recurrent headaches, visual disturbances, seizures, or developmental changes should be seen urgently by specialist doctors, such as a pediatric neurologist and a neuroradiologist, in a multidisciplinary institution if at all possible.

Malignant Melanoma

Melanoma is a cancer of melanocytes, the skin cells which produce pigment and which make up congenital melanocytic nevus. It used to be thought that melanoma was very common in people with CMN, but we now know that it is rare, occurring in around 1-2% of all people with CMN over their lifetime. However, the risk is higher in people with very large and numerous CMN, perhaps as much as 10%, and there is a peak of risk during childhood. When considering these risks, you need to bear in mind that a person without a CMN still has a risk of about 40% of developing some kind of malignant cancer at some time in his or her lifetime.

Melanoma in people with congenital melanocytic nevus can occur anywhere, not just within the CMN, and not just in children. It can present as a lump in the CMN, in another area of skin, or in the lymph nodes (which are throughout the body). However, melanoma is rarely if ever found in a disseminated “satellite” nevus. In about half the cases, it occurs directly within the brain or spine, when it can present with persistent headaches with nausea/vomiting, or visual disturbances, balance problems or seizures, like other tumors.

Psychological Challenges

People with congenital melanocytic nevi sometimes attract looks, stares, whispering and finger-pointing.

Children who grow up with a prominent congenital melanocytic nevi or many congenital melanocytic nevi may well have problems adjusting to the disfigurement that they perceive, particularly during the teenage years. This, however, is a very individual thing, and varies depending on the child’s personality and on the support from family and friends. The problems may manifest as anxiety, depression, or lowered self-esteem.

Family members sometimes hope that a nevus will ‘fade’ and the other potential medical challenges will also disappear. Some large congenital melanocytic nevi can lighten over time, but the birthmark itself, and potential challenges, remain. This reality requires not only the person with the CMN, but also their loved ones, to take some time to adapt.

TREATMENT

Many people with large nevi have problems with itching or other discomfort. Lotions and creams can alleviate some of that discomfort. But it is important to note that there are currently no creams, lotions or other topical products one can rub on the skin that can remove a nevus.

Monitoring

Removing a congenital melanocytic nevus is a personal decision to be taken ideally after consultation with more than one informed, trusted physician. Many patients who have a large CMN, or their families if they are too young to decide for themselves, prefer to leave it unoperated for various valid reasons.

Surgery – CMN removal and Reconstruction

When small nevi are removed, the edges of the surrounding skin are simply pulled together and stitched, this is called primary closure. Very often the CMN is excised in stages, this is a procedure called serial excision. Removal of a large nevus involves the replacement of the affected skin. Skin can be transferred from another area of the body (grafting), or moved in the form of a flap. Often in giant CMN adjacent skin can be stretched, then used to cover the area where the nevus was removed (tissue expansion). There are many details about the surgery you should consider and it is best to consult a surgeon experienced in the treatment of giant nevi.

LEGEND: Excision and primary closure of a medium size nevus of the thigh

LEGEND: Excision of a small sized CMN (2.5 cm) and reconstruction with transferring adjacent tissue in the form of a local flap

LEGEND: Excision of a medium sized CMN (8cm) and reconstruction with transferring adjacent tissue in the form of a local flap

Lasers

Lasers are successful for many superficial skin imperfections, but cannot completely remove a congenital melanocytic nevus. Lasers can destroy the surface cells of some nevi and such treatments are sometimes suggested by physicians. Some people report successful results in depigmentation with laser treatment, but others report poor results. When a CMN is not removed through the depth of the skin, no matter the technique used, the remaining cells have a tendency to repigment the region partially or fully. Laser treatments in children require general anesthesia.

It is important to remember that a congenital melanocytic nevus cannot be removed without leaving a scar. Scarring may be minimized at the hands of a highly skilled, experienced surgeon, and may also be remodeled at other points during a lifetime, so it is recommended you seek out a surgeon you will trust over a long period.

LIVING WITH A CMN

Sun Protection

People with congenital melanocytic nevi can and should enjoy a normal life. Affected children particularly, but all people with CMN, should use good sun protection—the same as we would recommend for all children. In particular, it is important to avoid sunburn. As a general rule: sun avoidance, coupled with good clothing protection, is more effective than sunscreen. The best sunscreens contain a reflectant barrier such as titanium dioxide; select a high protection factor (SPF), ideally 25 or more, and with high UVA protection if indicated. Sunscreen needs to be refreshed every 2 hours or so, more often when swimming or sweating.

Sun Avoidance

People with congenital melanocytic nevi should stay out of the direct sun as much as possible during the hottest hours of the day and in the hottest months of the year.

The hottest hours of the day are 10am-4pm. In most of the Northern Hemisphere, the hottest months of the year are April to October. For the Southern Hemisphere, October to April are the months in which to take care to reduce sun exposure.

UV exposure is much higher beside water or snow, so special care should be taken when swimming outside or skiing.
The sun is more harmful at higher altitudes.
The sun remains almost as harmful when it is cloudy, so even if the day is cloudy, the CMN patient should wear appropriate clothing such as a sunhat during the hottest times of the year.
Shade provides less protection when near water or snow, or when the weather is cloudy, because the sunrays are reflected into the shade.
The increased risk of skin cancer in people with CMN probably applies to their whole body, not just to their CMN.

CONGENITAL BLEPHAROPTOSIS

INTRODUCTION

A drooping eyelid is called ptosis or blepharoptosis. In ptosis, the upper eyelid falls to a position that is lower than normal. In severe cases of ptosis, the drooping eyelid can cover part or all of the pupil and interfere with vision, resulting in amblyopia.

GENERAL INFORMATION

If a droopy eyelid is present at birth or within the first year of life, the condition is called congenital ptosis. In most cases of congenital ptosis, the problem is isolated and does not affect the vision.

Congenital ptosis occurs equally among the different races.
Congenital ptosis occurs equally between males and females.
Congenital ptosis is usually present at birth but may manifest within the first year of life.

ETIOLOGY

In most cases of congenital ptosis, the cause is idiopathic (NO REASON).

Congenital ptosis may occur through inheritance. Common familial occurrences suggest that genetic or chromosomal defects are likely.

LEGEND: In a droopy eyelid the muscle that lifts the eyelid (levator) does not have normal muscle fibers (left eye) but it looks more like a thin scar (right eye)

Other potential causes of congenital blepharoptosis include:

Blepharophimosis syndrome
Third cranial nerve palsy
Horner syndrome
Duane syndrome
Periorbital Tumors: for instance neurofibromas.
Kearns-Sayre syndrome
Myotonic dystrophy
Blepharochalasis
Myasthenia gravis

HISTORY

All pediatric patients presenting with either unilateral droopy eyelid or bilateral droopy eyelids need a thorough examination that includes a medical history, a family history, a history of drug or allergic reactions, and a review of systems.

Family photographs can help determine onset or variability of the ptosis.
A history of fluctuating ptosis with strabismus may indicate myasthenia gravis.
A careful medical history regarding cancer should be obtained. Metastatic or primary orbital tumors can result in malpositioning of the eyelid.
A history of trauma with orbital wall fractures can result in pseudoptosis with enophthalmos.
A history of drug or allergic reactions may be helpful. Allergic reactions can result in eyelid edema and droopy eyelid.
A history of difference in the size of the pupil may be helpful in diagnosing Horner syndrome. Neuroblastoma, which is one of the most common childhood cancers, should be ruled out.
A history of dry eyes, intermittent epiphora, or chronic conjunctivitis can indicate a dry eye disorder or corneal surface disease.

CLINICAL EVALUATION – CLASSIFICATION

Blepharotopsis is classified as

Mild
Moderate
Severe
And pseudoptosis (not real ptosis)

POTENTIAL DIAGNOSTIC TESTS

MRI of the head and neck to rule out tumors
CT Scan of the head and neck (or even chest) to rule tumors
Single fiber electromyography EMG to rule out myasthenia gravis
ECG for possibility of mitochondrial disorders
BLOOD WORK
Muscle biopsy to rule out mitochondrial disorders

MANAGEMENT

Although not all patients with congenital ptosis need surgical intervention, patients need to be closely monitored for the possible development of amblyopia. Since amblyopia may not be reversed after age 7-10 years, appropriate and timely medical and surgical treatment of congenital ptosis is critical to preserve the child’s vision.
•An abnormal eyelid position can have negative psychosocial effects.
•Uncorrected acquired blepharoptosis results in decreased field of vision and frontal headaches.

General Treatment

Early consultation to avoid amblyopia
Must be able to rule out and document other possible causes of ptosis (eg, Horner syndrome, third cranial nerve palsy)

Medical therapy

Observation is only required in mild cases of congenital ptosis if no signs of amblyopia, strabismus, and abnormal head posture are present.

Depending on the severity of the congenital ptosis, patients should be monitored every 3-12 months for signs of amblyopia due to congenital ptosis. External photographs can be helpful in monitoring patients.
Head posture should be carefully examined. If the patient acquires a chin-up posture due to the worsening of ptosis, surgery may be indicated.
The patient should be checked for astigmatism due to the compression of the droopy eyelid.

Medical follow up

Patients who underwent surgery for congenital ptosis are initially monitored every 2-4 weeks for signs of exposure keratopathy, infection, granuloma formation, and overcorrection and undercorrection. External photographic documentation can be helpful in monitoring patients.
•Following the surgery, visual acuity, head posture, and refractive error should be carefully monitored. Any residual amblyopia should be treated aggressively.

Patients with congenital ptosis may have other conditions that need to be addressed. These conditions include amblyopia, strabismus, craniofacial abnormalities, and other neurologic findings. Appropriate consultation may be needed depending on the associated findings.

Surgery

The method of repair depends on treatment goals, the underlying diagnosis, and the degree of levator function. Surgical correction of congenital ptosis can be undertaken at any age depending on the severity of the disease. Earlier intervention may be required if significant amblyopia or ocular torticollis is present. Severe cases of ocular torticollis may delay mobility in infants and toddlers because of the balance problems from extreme chin-up head posture. If intervention is not urgent, surgery is often delayed until age 3-4 years. Waiting until this age allows for more accurate measurements preoperatively.
Surgery for ptosis in patients with a history of dry eyes, seventh cranial nerve palsy, or significant extraocular muscle abnormalities, such as severe Graves ophthalmopathy, double elevator palsy, or progressive external ophthalmoplegia, should be approached with great caution to avoid exposure keratopathy following the surgery.

Surgical techniques

Levator muscle resection (the muscle that elevates the upper eyelid)
Frontalis suspension procedure (suspension of the brow in a higher position with use of:
- Autogenous tissue like the fascia lata
- Preserved (tissue bank) fascia lata
- Nonabsorbable suture material
- Synthetic material such as: Silicone bands

Surgical outcome: Patients may not be able to close their eyelids during sleep from a few weeks to several months following surgery. Families must be warned of this outcome before the operation. The problem of open lids during sleep improves with time; however, aggressive lubrication is needed to avoid exposure keratopathy.

Fasanella-Servat procedure
- The upper lid is elevated by removing a block of tissue from the underside of the lid. This tissue includes the tarsus, conjunctiva, and Müller muscle. This procedure is not commonly performed for cases of congenital ptosis.
Müller muscle–conjunctival resection
- This surgery is chosen if the eyelid has had a good response to phenylephrine.
- The conjunctiva and the Müller muscle are marked off, clamped, and sutured. The tissues are resected. Then, the conjunctival layer is closed.
- This procedure is not commonly performed for cases of congenital ptosis, although its use has been well-documented and its utility has increased in recent literature.

LEGEND: Before and after surgical correction photos of a 4 year old child with one sided (left) congenital blepharoptosis

Surgical follow up

Close follow up is necessary in the first few weeks following surgery to make sure that exposure keratoconjunctivitis doesn’t develop and is controlled if it does develop. This also allows for evaluation of the wound itself and for signs of infection or inflammation of any foreign implanted material.

COMPLICATIONS

Granuloma: If suspension materials are not placed well beneath the skin, granuloma formation may occur. Granulomas should be treated conservatively because they tend to eventually resolve.
Lid asymmetry
Overcorrection with exposure keratopathy and dry eyes
Undercorrection: Suspension materials may dissolve or break. Suture material may tear through soft tissue. Undercorrected congenital ptosis repair may require repeat surgery.
Infection

PROGNOSIS

The repair of congenital ptosis can produce excellent functional and cosmetic results.
With careful observation and treatment, amblyopia can be treated successfully.
Of patients who require surgical intervention, 50% or more may require repeat surgery in 8-10 years following the initial surgery.

MICROTIA

DEFINITION OF MICROTIA

Microtia is a congenital deformity resulting in an abnormally small, malformed or absent external ear. One in 8000–10,000 children are born with microtia every year. The cause of microtia is still unknown. condition.

CAUSES OF MICROTIA

Microtia usually occurs as an isolated deformity although it can present itself as part of a spectrum of other deformities, either minor or major such as hemifacial microsomia, Goldenhar syndrome and Treacher-Collins syndrome. Most patients with the most severe form of microtia also lack an external auditory canal, also known as “atresia”.

Although causes of microtia and other ear deformities are difficult to define, some theories include a decreased blood supply to the developing ear in-utero. Others have theorized certain medication taken by the mother during pregnancy such as Accutane, Thalidomide and retinoic acid. Environmental factors have also been questioned.

MICROTIA AND HEARING AND SPEECH PROBLEMS

Most children with microtia do have hearing loss in the affected ear because often there is no canal to transmit sound waves to the inner ear. This is called aural atresia. Children with microtia and atresia affecting one ear will have problems locating the direction of sound and have trouble hearing a conversation in noisy environments, but the need for a hearing device is controversial. When your child begins school, you should make sure you child has preferential seating that will enable them to hear the teacher best. If your child has microtia with hearing loss on both sides, it is important to improve your child’s hearing with a bone-conducting hearing aid such as BAHA. It should be fitted before the age 6 months. The sounds that babies hear in the first year of life are very important for speech and language development.

MICROTIA AND OTHER CONDITIONS

tHE most common condition in which microtia is seen is Hemifacial Microsomia, in which half the face does not grow in proportion to the other. The degree of hemifacial microsomia varies from barely perceptible to very noticeable. Treacher Collins Syndrome involves both ears and also affects the eyes which appear to have a downward slant or “pulled down appearance.” The cheek bones are small and the eyelids and jaw are affected. Goldenhar Syndrome may involves one or both ears and is characterized by incomplete development of ear, nose, soft palate, lip, and jaw as well as potential benign tumors of the eve.

CLASSIFICATION OF MICROTIA

SURGICAL RECONSTRUCTION OF MICROTIA

There is a surgical procedure that involves making an ear canal and an eardrum called an atresia repair or canalplasty. A CT scan of the middle ear should be done at 2½ years old to determine if your child is a candidate for the surgery. Most microtia patients have normal inner ears, and therefore, approximately 60% of children with aural atresia can have significantly improved hearing from a canal and ear drum reconstruction. This surgery can be done when your child is at least 3 years of age or older.

SURGICAL OPTIONS FOR MICROTIA:

Silicone Removable Ear Prosthetic
Rib Cartilage Reconstruction
MEDPOR Ear Reconstruction

SILICON EAR PROSTHETIC

A prosthetic ear can be created to look very similar to a patient’s normal ear. The specialist who can make and color the prosthesis is called a prostodontist. There are two methods that are used to attach the ear prostheses. One uses a glue to adhere the ear to the skin. The second method uses titanium implants which are placed onto the bone around the ear. The prosthesis can then be “snapped” into place (called an osteo-integrated prosthesis.)

LEGEND: Silicon made external ear prosthesis attached with implanted magnets

ADVANTAGES

Very realistic looking ear
Simpler process with less risk than surgical reconstruction
Provides an option for patients who are not candidates for surgery
Avoids chest surgery
May be used for failed medpor or cartilage surgeries

DISADVANTAGES

Difficulty hiding the seam between the prosthesis and cheek normal skin
Fading of ear color and deterioration of the prosthesis requires replacement every 2–3 years
Children may lose the prosthesis during sport or rough play
Needs to be removed at night and replaced each morning
Still requires surgery (unless adhesive-type is used)
The child will never feel like the ear is part of his/her body, although this may not be the case in a poorly reconstructed ear or an ear affected by trauma.

RIB CARTILAGE RECONSTRUCTION

Using a child’s rib cartilage to carve an ear framework has been the standard of care for more than 50 years. Cartilage reconstruction is an invasive technique that requires between 2–3 surgeries. Ear reconstruction with rib cartilage must be delayed is old enough to have sufficient cartilage to make an adult-sized ear, typically between 7–10 years of age. First the cartilage from several ribs is removed through an incision on the child’s chest. The cartilage is carved and pieced together to create an ear framework. This framework is buried under the scalp and after the ear has healed, more surgeries are required to complete the reconstruction: elevating the ear away from the scalp, repositioning the earlobe and other adjustments.

Unilateral Microtia (One Side)

For example, if a very small 6 year old presents to the office, it is reasonable to wait until 7 or 8 years of age until there is sufficient rib growth. If on the other hand, a very large 5 year old presents to the office, the reconstructions sooner due to adequate rib size.

The ideal time to begin the reconstruction is the summer before the first grade. By the time the child starts the first grade, at least two of the surgeries can be performed and the child now has the resemblance of an ear. Children tend to be made fun of during these early school years when they are not yet reconstructed.

Bilateral Microtia (both sides affected)

Timing of Surgery in Bilateral Microtia

Although the microtia surgeries using the rib technique used to be started as early as 4 years of age in the past, the preference is now to start the microtia reconstruction at about 6 to 7 years of age (depending on the size of the child). During this age, the child usually has enough rib growth to provide substantial cartilage for the reconstruction. If the child is still very small in size at this age, then it is imperative to wait a little longer until there is sufficient rib cartilage.

Combining Surgical Stages in Bilateral Microtia

Patients with bilateral microtia undergo a combination of stages involving both ears. This combination staging decreases the amount of surgeries needed to complete both ear reconstructions.

In bilateral microtia, the first surgical stage involving the rib cartilage grafting is always performed at separate times. If both sides were used for cartilage grafting at the same time, this could lead to respiratory problems secondary to the lack of inspiratory effort or complications from lung involvement.

Following the first rib graft surgery on one side, the rib graft surgery on the other side may be performed about six weeks later. Then after approximately two to three months, both earlobes can be created during a single operation. Both ears can then be elevated at the same time approximately two to three months later. If tragus reconstruction is necessary, this operation can be performed afterward.

By combining the surgeries on each side, the amount of surgery and anesthesia is minimized.

It is very important to start the external ear reconstructions BEFORE the middle ear surgery. Once an attempt is made to open the canal prior to the external ear reconstruction, the elasticity of the “virgin” skin as well as the circulation is compromised.

Because these children are dependent on bone conduction hearing aids, the goal of starting earlier is to at least finish one ear. The canal may be drilled soon after the ear is reconstructed with the eventual goal of obtaining adequate hearing without the use of hearing aids. In addition, there is not a normal ear to compare to. As a result, two relatively smaller ears will not be as noticeable as one asymmetric ear.

ADVANTAGES

Technique uses the body’s own tissue (cartilage) instead of a manufactured product
In expert hands, the patient may have an excellent, long-lasting result
Reconstructed ear can withstand the rigors of most sports
Very safe
The new cartilaginous ear grows with the patient
Sports are of little to no concern

DISADVANTAGES

This technique requires 2–3 surgeries

Technically difficult
Delayed ear reconstruction means children will begin schooling with noticeable microtia
Requires a small incision over the rib area
Cartilage removal is painful and requires hospitalization, IV pain medications, a permanent chest scar and potential chest wall deformity
Canal surgery cannot begin until after the ear is completely reconstructed, often delaying hearing restoration for years
Cartilage ears do not project from the side of the head, often necessitating a procedure to “pin back” the opposite ear for symmetry.

MEDPOR EAR RECONSTRUCTION

Reconstruction with MEDPOR is a surgical technique that uses a porous polyethylene framework along with the child’s own tissue to create an ear. Reconstruction with MEDPOR can usually begin at age 3. If only one ear is involved, the MEDPOR framework is customized to match the normal ear, but is created slightly larger so the ear will be adult-sized. The framework is then covered by the patient’s own tissue (called a flap) which is brought down as a thin “living membrane” from underneath the scalp. In most cases, only one surgery is required to reconstruct the ear. Atresia repair can be done before or after MEDPOR reconstruction. A combined one stage reconstruction of both microtia and canal surgery using MEDPOR is possible.

LEGEND: Before and after right Ear Reconstruction with Medpor prosthetic

ADVANTAGES

Reconstruction may be done at 3 years old without the need for future surgery
Children with hearing loss may undergo canal reconstructions before or at the same time as the ear reconstruction
Minimal pain associated with MEDPOR enables children to go home an hour after the procedure
Reconstruction may be completed in one, outpatient surgery in 80% of cases
The reconstructed ear is designed to match the normal ear in size and projection eliminating the need to “pin back” the normal ear
MEDPOR ears are able to withstand the rigors of most sports
Avoids a chest incision
Can be started at a younger age

DISADVANTAGES

The MEDPOR framework is a foreign material
Few people in the world have been trained in this surgery and an inexperienced microtia surgeon may have poor results
Since MEDPOR ear reconstruction began in 1991, the outcome of the implant over a lifetime is not known
Technically very challenging.
Using the temporalis fascia flap is an invasive procedure
Placing a skin graft on the front part of the ear does not have the same protective sensation like normal skin
In some cases, the scalp has to be divided above the ear to obtain a tissue flap in order to cover the implant
There may be hair loss at the site of the incisions that may be very noticeable if the child has short hair
The skin may look different because most of the implant is covered by skin from another part of the body (usually the opposite ear)
The medpor/supor will not grow with the patient
The risk of fracture and exposure of the medpor/supor implant from trauma will exist for life

OBSTERICAL BRACHIAL PLEXUS PALSY (OBPP)

ANATOMY OF BRACHIAL PLEXUS

The brachial plexus is a network of nerves in the shoulder that carries signals from the spinal cord to the arms and hands. It allows people to control movements and feel sensations in the arms and hands. When the brachial plexus nerve in the shoulder is injured during the birthing process, this condition is called obstetric brachial plexus palsy.

WHAT IS OBPP

Injury to the brachial plexus is fairly common during the birthing process, occurring in 1-2 births per 1,000. Larger babies in difficult deliveries are particularly prone to this injury. The brachial plexus can also be injured when nerves are stretched by a blow to the shoulder or when bones around them are broken.

Obstetric brachial plexus palsy occurs in less than 1% of live births. It is most common when there is difficulty delivering the baby’s shoulder. During delivery, the baby’s shoulder may become impacted on the mother’s pubic bone causing the brachial plexus nerves to stretch or tear (shoulder dystocia). The prognosis for recovery depends on the pattern, complexity, and severity of injury. Erb’s Palsy refers to an injury of the upper brachial plexus nerves leading to loss of motion around the shoulder and ability to flex the elbow. Klumpke’s palsy refers to an injury of the lower brachial plexus leading to loss of motion in the wrist and hand.

TYPES OF BRACHIAL PLEXUS INJURIES

Brachial plexus injuries are categorized according to the type of trauma experienced by the nerve. The following are the types of brachial plexus injuries:

Avulsion– this means the nerve has been pulled out from the spinal cord and has no chance to recover.
Rupture– this means the nerve has been stretched and at least partially torn, but not at the spinal cord.
Neurapraxia– this means the nerve has been gently stretched or compressed but is still attached (not torn) and has excellent prognosis for rapid recovery
Axonotemesis– this means the axons (equivalents of the copper filaments in an electric cable) have been severed. The prognosis is moderate.
Neurotemesis– this means the entire nerve has been divided. The prognosis is very poor.
Neuroma– this refers to a type of tumor that grows from a tangle of divided axons (nerve endings), which fail to regenerate. The prognosis will depend on what percentage of axons do regenerate.

SYMPTOMS OF OBPP

Children and babies with brachial plexus injuries will typically have a number of the following symptoms:

Unable to lift their arm above their head
Unable to bring objects to their mouth
Unable to move their fingers
Unable to feel things in their arm, hand or fingers
Tingling or pain in their arm, hand or fingers
Pain
Loss of sensation
Muscle weakness

LEGEND: Example of 4 month old baby with Erb’s Palsy – typical posture of the right arm (waiter’s tip position of the hand)

Some patients may experience avulsion pain (a burning, crushing type of pain) in the distribution of the injured nerves.

DIAGNOSIS AND TESTS

Due to the complex spectrum of brachial plexus injuries, a detailed and comprehensive understanding of the exact nature of injury in each patient is required for proper management.

Multiple modalities are utilized to diagnose a brachial plexus injury including:

History taking and clinical examination
Electrodiagnostic studies (EMG,NCV, SNAP, SSEP)
Imaging studies (CT, MRI)
CT/MYELOGRAPHY

LEGEND: MRI of patient with OBPP. The arrows show myelomeningoceles, a sign of root avulsion

Some of these evaluations may need to be repeated on a regular basis to track the progression of recovery of function. Used in combination, these modalities provide valuable insights into the elements of the brachial plexus that have been injured including information about the severity of the injury and prognosis.

APPROACH CONSIDERATIONS

Most infants with birth-related brachial plexus palsy (OBPP) demonstrate spontaneous improvement in upper-extremity function and do not require surgical management. Spontaneous complete recovery rates have been reported to be as high as 93% by age 4 months.Recovery rates and extent depend on the injury type and severity. Mild injuries with rapid recovery clearly do not require surgery. Similarly, injuries involving a flail upper limb and Horner syndrome require operative management.

Discerning the conditions between these two extremes that would benefit from operative management is more challenging. The timing for surgical intervention has been debated in the literature Historically, absence of elbow flexion by age 3-4 months has been used as a predictor of benefit from operative management.

It should be noted that no single algorithm is universally applicable, and management decisions must be achieved based on individual circumstances and performance over time.

Brachial plexus reconstructions are complex, lengthy procedures that require skill, appropriate equipment, and a well-prepared team. The patient’s overall health status must be included in the consideration for operative management. The patient must be sufficiently healthy for a prolonged surgical procedure. The brachial plexus is in the vicinity of critical structures such as the great vessels and thoracic cavity. Systemic conditions such as coagulopathies may be contraindications for surgery.

TREATMENT

Due to the broad spectrum of brachial plexus injuries, it is difficult to estimate the rate of spontaneous recovery. The potential for spontaneous recovery depends on the type and severity of injury. Therefore, prognosis must be assessed for each patient individually based on the type and severity of their injury, and the progression of any spontaneous recovery that may be occurring.

Early diagnosis and treatment can seriously improve long-term outcomes for these injuries. The seriousness of these injuries can vary widely. Many children will regain all or most function through occupational therapy. If no improvement is seen after three months, however, a pediatric neurologist and pediatric neurosurgeon should determine if the child can benefit from other interventions or surgery. 1 in 10 babies with brachial plexus injury will require some level of surgery. If the injury occurred during the birthing process, the best age for surgery is between four and nine months, as waiting more than a year can result in long-term damage.

A.Nonsurgical treatment options for brachial plexus injuries

Depending on the degree of severity, some nerve injuries are able to heal on their own. If this is not a viable option for a particular patient, a surgical option may be recommended by the physician. Physical rehabilitation therapy is always part of the recovery process for a brachial plexus injury.

Physical therapy is used to maintain passive range of motion (ROM) of the affected joints. Some believe that in patients with OBPP transcutaneous electrical nerve stimulation (TENS) is useful in waking up muscles that have been successfully reinnervated over a period of time.However, no scientific studies support this conclusion, and the authors do not routinely employ this modality.

Glenohumeral dysplasia (GHD) with shoulder instability (dislocation or subluxation) may be managed with chemodenervation of internal shoulder rotators, closed reduction, and shoulder spica casting.

B. Surgical treatment options for brachial plexus injuries

The degree of functional impairment and potential for recovery depend on the mechanism, type, complexity of the brachial plexus injury, and time from injury. The most important decision your surgeons will make is determining if and when surgical intervention should occur.

Once the need for operative intervention has been determined, many reconstructive options are available, depending on the intraoperative findings, including the type of neural lesion encountered, the resulting neural gap, and the amount of available neural donor tissues. Surgical treatment involves brachial plexus exploration, neuroma excision, and nerve grafting or nerve transfer.

Preparation for surgery

Given the complexity and length involved with brachial plexus reconstruction procedures, appropriate preoperative planning can improve efficiency and outcomes. Throughout the process, communication among all caregivers is essential to minimize complications.

Operative techniques

Neurolysis – Neurolysis alone for the surgical management of neuroma-in-continuity provides functional improvements at 12-month follow-up for patients with upper-plexus, but not total-plexus, lesions.

Neuroma excision – Neuroma excision has been shown to have superior long-term results compared with neurolysis alone in children with birth-related brachial plexus lesions. After neuroma excision and interpositional nerve grafting, patients with OBPP regain their preoperative functional levels by 3-6 months postoperatively,indicating no downgrade of function for patients selected as operative candidates via the previously outlined algorithm.

Interposition nerve grafting Because neuroma resection and grafting yields improved outcomes in OBPP patients requiring surgical management, sural nerve grafts can be harvested as the first stage of the procedure. Sural nerve harvest for graft material results in minimal donor-site morbidity but does create a permanent insensate patch at the lateral foot, which, though measurable, often goes unnoticed by patients.

RECONSTRUCTION WITH NERVE GRAFTS

Nerve transfers Nerve transfers allow reconstruction of a nerve from a different nerve source and are useful in cases of root avulsion, late presentation, or isolated deficits. Transfers also allow reconstruction of specific motor or sensory deficits with a single nerve coaptation and can sometimes be performed in closer proximity to the target function to allow more rapid target innervation.

RECONSTRUCTION WITH NERVE TRANSFER

Reconstruction may consist of a combination of nerve grafts and transfers, if necessary, depending on the size of the neural gaps and the amount of nerve graft available. Priority is given to reconstruction of hand function, followed by the elbow and shoulder. Neural coaptations are performed with fibrin glue with the use of the operating microscope.

POSTOPERATIVE CARE

Physiotherapy should be started as soon as possible for a newborn baby with a brachial plexus injury. You will be given a range of movement stretches by your physiotherapist and you will be shown how to incorporate these exercises into your baby’s daily routines, e.g. bath time, during dressing. These exercises aim to keep the muscles and joints flexible and ready to work if and when the nerves and muscle function improve.

It is important to take extra care against injuring the affected arm. Because the feeling is reduced, children may not feel hot objects (i.e. burning) or pain and therefore may not complain of an injury.

COMPLICATIONS

Complications of surgery for BRBPP include the following:

Infection
Hematoma
Seroma
Injury to nearby structures, including vascular structures and the thoracic cavity

Unique to this surgery is the possibility of further inhibiting function by injuring components of the brachial plexus that are normal or recovering. In this patient population, injury to the phrenic nerve can result in devastating pulmonary compromise that could require urgent diaphragmatic plication. In theory, an intercostal motor branch or a nerve to the rectus muscle can be transferred to the distal end of the phrenic nerve, just above the diaphragm, to provide some reinnervation of the diaphragm

LONG TERM MONITORING AND FUTURE THERAPIES

Initially, patients with OBPP are monitored for wound management. At 4 weeks following surgery, patients are referred for therapy to regain active and passive ROM of the extremity. Recovery of some motor function may be noted as early as 3 months following surgery. Baseline motor function is usually achieved by 6 months. Patients continue to improve for up to 4 years, however, following surgery. Botulinum toxin has been used for management of muscle imbalance and has shown sustained benefits for elbow movement imbalance.Secondary procedures such as muscle transfers, tendon transfers, osteotomies, or shoulder releases may also be necessary to maximize function

KEY POINTS TO REMEMBER

Obstetric brachial plexus injuries occur at birth.
About one in 10 children needs surgery to help improve function of the arm.
If upper limb splinting is required you will be referred to an occupational therapist.
It is important to continue the stretches and exercises at home for the best possible outcome.
About one in 10 children need surgery to help improve function of the arm.
If your baby does require surgery, they will have treatment with a physiotherapist afterwards to make sure they have the best possible outcome.

RESULTS

The recovery of the function after a microneurosurgical reconstruction starts being obvious at 9-12 months and it takes about two years in average to be completed. Once functional recovery is completed the results are permanent.

LEGEND: 4 month old male with upper plexus (Erb’s) palsy. The baby is unable to lift the arm and flex the elbow

LEGEND: The exploration of the brachial plexus showed ruptured nerve roots with an obvious neuroma. The surgery included removal of the neuroma (nerve scar) and reconstruction with nerve grafts.

LEGEND: At 5 years of age the boy is able to fully lift the arm and flex the elbow

OTOPLASTY

INTRODUCTION

Prominent ear deformity is relatively common; this defect— inherited as an autosomal dominant trait—affects approximately 5% of white children. As such, surgical correction is a common operation performed by plastic surgeons.

TREATMENT RATIONALE AND PSYCHOLOGICAL SEQUELAE

For children and young adolescents undergoing otoplasty, the motivation for surgery may be aesthetic dissatisfaction, psychosocial distress, or distress anticipated by caregivers. Children with aesthetic deformities such as prominent ears are often the subjects of peer ridicule, which may give rise to emotional instability for the affected child.

For most children, the motivation for surgery is predominantly psychological and social distress. From a psychosocial perspective, favorable outcomes are produced post-correction. Most children and their families reported increased self-confidence after corrective surgery, and more than half of children reported that teasing and bullying diminished postoperatively. In a study of psychological and social outcomes of prominent ear correction, well-being had improved in an overwhelming majority of children 12 months postoperatively. Psychological evaluation should be sought for any child showing evidence of marked social isolation or acute distress preoperatively.

ANATOMY

The ear achieves about 85% of its adult size by age 3 to 4 years. Ear width reaches its mature size in boys at 7 years and in girls at 6 years, while ear length matures in boys at 13 to 15 years and in girls at 12 to 14 years. The ear has several proportions or “canons” that are common in the normal ear—as originally described by Da Vinci in his descriptions of normal facial aesthetics. Some of these canons include:

The width of the ear is about half the height.
The ear’s height is generally equal to the distance between the lateral margin of the ipsilateral orbital rim and the root of the ear.
The ear inclines posteriorly about 20 degrees off the vertical axis.

PATIENT EVALUATION

During preoperative evaluation, the following focused assessment should be performed by the pediatrician to determine whether to refer the patient for correction of the prominent ear deformity:

Evaluation of the ear:

          –Superior third of the ear (antihelical fold development).
          –Middle third of the ear (conchal bowl deformity).
          –Lower third of the ear (lobule prominence).

Psychological sequelae as a result of the deformity.
Motivations for surgery (patient and family).

The optimal timing of surgical correction is usually when the ear is almost fully developed—by age 4 to 6 years, around the time of the child’s entry into first grade. Given the largely aesthetic nature of the operation, informed consent with a detailed discussion of complications and postoperative course is critical to the optimization of patient and family expectations and outcomes.

NONSURGICAL CORRECTION

Some authors have achieved acceptable results in the neonatal period with nonsurgical molding of the prominent ear deformity. This principle is based on circulating neonatal estrogens that render the ear cartilage relatively pliable. There is evidence that this intervention is successful within the first few days of life until 3 to 4 months post-birth. Early initiation of treatment continued to about 6 months of age can produce improvements in ear shape.

SURGICAL APPROACH AND POSTOPERATIVE CARE

Various treatments and approaches have been developed to correct prominent ear deformity. These include techniques that modify or reposition the auricular cartilage and soft tissues using a combination of suturing, scoring, and/or excision. The goals of the operation are to provide a natural-appearing auricle, without an “operated” look. Operative approaches for the correction of the prominent ear can be considered as techniques used to recreate the antihelical fold (suture techniques or scoring techniques), to reduce the conchal defect (cartilage excision), and to correct lobule positioning (suture fixation techniques or skin excision techniques). The details of these techniques are beyond the scope of this review; however references are provided for in-depth explanations.

LEGEND: Drawing of the surgery. An elliptical excision of the skin of the back of the ear is done (left), then sutures are placed in the cartilage in order to create the folds of the ear (middle) and then the incision is sutured leaving a straight line (right)

LEGEND: Preoperative photo of left prominent ear (left). Intraoperative photograph right after otoplasty (right)

LEGEND: Preoperative photographs of prominent ears of a 15 year old teenager

LEGEND: Postoperative photos of the same teenager 4 weeks after otoplasty

After surgery, the ears are maintained in position by a bulky dressing of vaseline gauze, regular gauze, and gauze rolls. The initial dressing is left in place for approximately 1 week, and is then removed in the clinic. Postoperatively, a conforming ear bandage is worn by the patient for 3 weeks around the clock and then for the following 3 weeks only at night. Postoperative oral antibiotics and analgesics are prescribed for 5 to 7 days. In addition, children are to refrain from sports or other activities that may traumatize the ears for 6 weeks postoperatively.

COMPLICATIONS

Hematoma, one of the most common immediate postoperative complications, presents with unilateral acute and severe pain. This complication must be managed with dressing removal and hematoma evacuation to prevent a hematoma and subsequent cartilaginous deformity.
Infection is another potentially devastating complication of otoplasty because it can lead to chondritis and residual ear deformity. If pain, tenderness, swelling, and drainage are encountered, treatment with intravenous antibiotics is imperative— with or without wound debridement. The commonly implicated organisms include Staphylococcus, Streptococcus, and occasionally Pseudomonas.
Chondritis is a surgical emergency since, without treatment, an ear cartilage deformity may result. As such, prompt debridement of infected cartilage is required.
Recurrence
Extrusion of sutures
Hypertrophic scars and keloids

VASCULAR ANOMALIES

DEFINITION

A vascular anomaly is a kind of birthmark caused by a disorder of the vascular development, although it is not always present at birth. A vascular anomaly is a localized defect in blood vessels that can affect each part of the vasculature (capillaries, arteries, veins, lymphatics or a combination of these). These defects are characterized by an increased number of vessels and vessels that are both enlarged and sinuous.

Some vascular anomalies are congenital and therefore present at birth, others appear within weeks to years after birth and others are acquired by trauma or during pregnancy. Inherited vascular anomalies are also described and often present with a number of lesions that increase with patients’ age.

Vascular anomalies can also be a part of a syndrome and, occasionally, they can be acquired by trauma. The estimated prevalence of vascular anomalies is 4.5%.

Vascular anomalies can occur throughout the whole body (skin, bone, liver, intestines, i.e.), but in 60% of patients vascular anomalies are localized in the head and neck region.

In general there are two main categories in the family of Vascular Anomalies

Vascular tumors (most common example is the Hemangioma)
Vascular Malformations (most common example is the Capillary Malformation or “port wine stain”)

CAUSES

The cause for hemangiomas and vascular malformations is usually sporadic (occurs by chance). However, they can also be inherited in a family as an autosomal dominant trait. Autosomal dominant means that one gene is necessary to express the condition, and the gene is passed from parent to child with a 50/50 risk for each pregnancy. Males and females are equally affected and there is great variability in expression of the gene. In other words, a parent may unknowingly have had a hemangioma because it faded, but the child is more severely affected. The family may not come to the attention of a geneticist until the birth of the child with a more severe condition. Other relatives with mild expression of the gene are often discovered at that time, confirming autosomal dominant inheritance.

Hemangiomas and vascular malformations are a manifestation of many different genetic syndromes that have a variety of inheritance patterns and chances for reoccurrence, depending on the specific syndrome present.

SYMPTOMS

If a hemangioma or vascular malformation is very large or affects the breathing system (airway or lungs) or another large organ system, it could be life-threatening. If a hemangioma has uncontrollable bleeding, this could also be life-threatening. Depending on where a hemangioma or vascular malformation is, it may cause physical problems, such as with moving a part of the body or difficulty seeing, hearing or swallowing.

MULTIDISCIPLINARY EVALUATION

Large and/or life-threatening lesions should be evaluated by a multidisciplinary team of specialists that includes plastic surgeons, dermatologists, ophthalmologists, radiologists, and other specialists, depending on what organs are involved.

PRESENTATION

Vascular anomalies that are situated deep below the skin, appear blue and are often called cavernous. Superficial vascular anomalies appear as red-coloured stains and are associated with vascular anomalies affecting the dermis. Historically, vascular anomalies have been labeled with descriptive terms, according to the food they resembled (port wine, strawberry, cherry, salmon patch). This imprecise terminology has caused diagnostic confusion, blocked communication and even caused incorrect treatment, as it does not differentiate between various vascular anomalies. However, in 1982, Mulliken introduced a classification that replaced these descriptive terms and gave direction to the management of various vascular anomalies. This classification, based on clinical features, natural history and cellular characteristics, divides vascular anomalies into two groups: vascular tumors and vascular malformations. Although the appearance of both vascular tumors and vascular malformations can resemble, there are important differences between both.

What is a hemangioma?

A hemangioma is a type of birthmark. It is the most common benign (noncancerous) tumor of the skin. Hemangiomas may be present at birth (faint red mark) or may appear in the first months after birth. A hemangioma is also known as a port wine stain, strawberry hemangioma, and salmon patch. About 60 percent of hemangiomas occur in the head or neck area. Hemangiomas occur at least three times more often in females than in males. Most will continue to grow for the first six to 12 months of life before beginning to shrink.

What is a vascular malformation?

A vascular malformation is another type of birthmark, or congenital (present at birth) growth, made up of arteries, veins, capillaries, or lymphatic vessels. There are several different types of malformations and they are named according to which type of blood vessel is predominantly affected. A vascular malformation is also known as lymphangioma, arteriovenous malformation, and vascular gigantism.

What is the difference between a hemangioma and a vascular malformation?

Most hemangiomas are not usually present at birth or are very faint red marks. Shortly after birth, however, they grow rapidly–often faster than the child’s growth. Over time, they become smaller (involute) and lighter in color. The process of involution may take several years.

Vascular malformations are present at birth and enlarge proportionately with the growth of the child. They do not involute spontaneously and may become more apparent as the child grows.

VASCULAR TUMORS

Vascular tumors, often referred to as hemangiomas, are the most common tumors in infants, occurring in 1-2%. Prevalence is even higher (10%) in premature infants of very low birth weight.

Vascular tumors are characterized by overgrowth of normal vessels, which show increased endothelial proliferation. It can be present at birth, but often appears within a couple of weeks after birth or during infancy.

There are different kinds of vascular tumors, but the 4 most common types are:

1. Infantile Hemangioma

Infantile hemangioma is the most common vascular tumor. It is a benign tumor, which occurs in 4-5% of Caucasian infants. It is 4 times more frequently in females. Most commonly presents in the head and neck region (60%), but also involves the trunk and extremities. One third of these lesions is present at birth as a telangiectatic stain or ecchymotic area. During the first four weeks of life, 70% to 90% appear. Lesions that are situated beneath the skin may not appear until 3 to 4 months of age, when the tumor is large enough. During the first 9 months, IH undergoes rapid growth, which is faster than the growth of the child. This is called the proliferating phase. After 9 months, the growth of the tumor will decrease and equal the growth of the child for about 3 months. After 12 months, the tumor will start to involute and might even disappear. Involution occurs in one-third of patient by the age of 3 years, in 50% by the age of 5 years and in 72% by the age of 7 years. Involution may result in residual telangiestasis, pallor, atrophy, textural changes and sometimes fibrofatty residuum. Since 90% of IH is small, localized and asymptomatic, treatment mainly consists of observation and awaiting until involution is complete. IH can be treated with corticosteroids, which accelerate involution: in 95% of patients, growth is stabilized and 75% of tumors decrease in size. Intralesional corticosteroids are most effective, but may require additional injections, as the effect is only temporarily. Systemic corticosteroids may cause a number of side-effects and are only used in problematic IH, which is too large to treat with intralesional injections. During the proliferating phase, the tumor is highly vascular. Patients who undergo operative treatment during this period, are at risk for blood loss. Moreover, surgery during this phase, often leads to an inferior aesthetic outcome. However, patients may require intervention during childhood, because 50% of IH leave residual fibrofatty tissue, redundant skin, or damaged structures after involution. Waiting until involution is completed, ensures that the least amount of fibro fatty residuum and excess skin is resected, giving the smallest possible scar.^[5] Another option for treatment in the pulsed-dye laser. After involution residual telangiectasias can be treated with laser therapy.

LEGEND: Typical infantile hemangioma

2. Congenital Hemangioma

Congenital hemangioma can be distinguished from infantile hemangioma because it is fully developed at birth. It forms during prenatal life and has reached its maximal size at birth. Congenital hemangioma can even be diagnosed in utero by prenatal ultrasound. Unlike IH, CH is more common in the extremities, has an equal sex distribution, and is solitary, with an average diameter of 5 cm. It commonly presents in the head and neck and in the lower extremities. Congenital hemangioma are divided into 2 subgroups:

Therapidly involuting congenital hemangioma, RICH, presents at birth as a solitary raised tumor with a central depression, scar, or ulceration surrounded by a rim of pallor. It is noted for its involution, which typically begins several weeks after birth and is completed no later than 14 months of age. After regression RICH may cause a residual deformity, such as atrophic skin and subcutaneous tissue. It mainly affects the limbs (52%), but also the head and neck region (42%) and the trunk (6%).

LEGEND: RICH hemangioma of the knee of a 18 month old baby

Thenon-involuting congenital hemangioma, NICH, presents as a solitary, well-circumscribed reddish-pink to purple plaque with central telangiectasia and hypopigmented rim. In contrast to RICH, NICH does not involute and rarely ulcerates. It persists into late childhood and can even mimic a vascular malformation by growing commensurately with the child. Although NICH can resemble RICH in its external appearance, it can be differentiated from RICH by a greater elevation and coarse telangiectases. It mainly affects the head and neck region (43%), but also the limbs (38%) and the trunk (19%).

LEGEND: NICH hemangioma of the knee of a 9 month old baby

Surgical resection for congenital hemangiomas is rarely needed, because RICH undergoes postnatal regression and NICH is benign and often asymptomatic. Resection may be indicated to improve the appearance of the affected area, as long as the surgical scar is less noticeable than the lesion. Other indications are problematic ulcers with persistent bleeding or chronic infection. Although most NICH lesions are non-problematic and do not cause significant deformity, the threshold for resection of NICH is lower, because it neither involutes, nor responds to pharmacotherapy. RICH tumors are observed until involution is completed. Involuted RICH may leave behind atrophic tissue, which can be reconstructed with autologous grafts.There are effective pharmacologic treatments, which include intralesional corticosteroid injection, systemic corticosteroid injection, interferon α-2a or α-2b and angiogenic inhibitors. Other therapeutic options are embolization and pulsed-dye laser, which improves residual telangiectasias in RICH and in NICH.

3. Kaposiform Hemangioendothelioma

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that is locally aggressive but without metastatic potential. It occurs particularly in the skin, deep soft tissue, retroperitoneum, mediastinum, and rarely in bone. Although lesions occur solitary, they often involve large areas of the body, such as the head/neck region (40%), trunk (30%), or extremity (30%). Usually, it is present at birth as a flat, reddish-purple, tense and edematous lesion. Although half of lesions are congenital, 58% of KHE develop during infancy, 32% between age 1 and 10 years (32%) and 10% after 11 years of age. Both sexes are affected equally in children. Lesions are often greater than 5 cm in diameter and can cause visible deformity and pain. During early childhood, KHE may enlarge and after 2 years of age, it may partially regress. Though, it usually persists longterm. In addition, 50% of patients suffer from coagulopathy due to thrombocytopenia (<25,000/mm3), presenting with petechiae and bleeding. This is called the Kasabach-Merritt Phenomenon, which is caused by trapping of platelets and other clotting factors within the tumor. KHE tumors are diffuse involving multiple tissue planes and important structures. Resection of KHE is thus often difficult. Treatment of kaposiform hemangioendothelioma is therefore medical. The primary drug is interferon alfa, which is successful in 50% of children. Drug therapy is often used in shrinking the tumor and treating the coagulopathy. However, many of these kaposiform hemangioendotheliomas do not completely regress and remain as a much smaller asymptomatic tumor. However, KHE still has a high mortality rate of 30%. Although complete surgical removal with a large margin has the best reported outcome, it is usually not done because of the risk of bleeding, extensiveness, and the anatomic site of the lesion. Operative management may be possible for small or localized lesions. Removal of larger areas also may be indicated for symptomatic patients or for patients who have failed farmacotherapy. Resection is not required for lesions that are not causing functional problems, because KHE is benign and because resection could cause deformity.

LEGEND: Kaposiform hemngioendothelioma of the face and neck of a 1 year old baby

4. Pyogenic granuloma

Pyogenic granuloma, also known as lobular capillary hemangioma, is a small benign vascular tumor that primarily involves the skin (88.2%) and mucous membranes. Pyogenic granuloma appears as a red spot that grows rapidly, turns into a papule and eventually becomes pedunculated, being attached to a narrow stalk. Although these lesions are small, they are often complicated by bleeding, crusting and ulceration. Pyogenic granulomas are rarely congenital. It commonly develops in infants: 42.1% develops within the first 5 years of life. Most of the lesions seem to be associated with trauma, an underlying cutaneous condition, hormonal alterations and medications. Of all pyogenic granulomas, 62% is distributed on the head or neck, occurring mainly on the cheek and in the oral cavity. Lesions on the face may cause visible deformity.

Numerous treament methods have been described for pyogenic granuloma. Lesions involving the reticular dermis, may be out of the reach of pulsed-dye laser, cautery or shave excision and therefore have a recurrence rate of 43.5%.^] Definitive management requires full-thickness skin excision. Other options are currettageor laser therapy. Furthermore, thorough currettage and cauterization are often used for small lesions and full-thickness excision for larger lesion.

LEGEND: Typical Pyogenic Granuloma

VASCULAR MALFORMATIONS

Vascular malformation is a collective term for different disorders of the vasculature (errors in vascular development). It can be a disorder of the capillaries, arteries,veins and lymphatic vessels or a disorder of a combination of these (lesions are named based on the primary vessel that is malformed). A vascular malformation consists of a cluster of deformed vessels, due to an error in vascular development. Congenital vascular malformations are always already present at birth, although they are not always visible. In contrast to vascular tumors, vascular malformations do not have a growth phase, nor an involution phase. Vascular malformations tend to grow proportionately with the child. Vascular malformations never regress, but persist throughout life. Vascular malformations can be divided into slow-flow, fast-flow and complex-combined types.

1. Slow-flow vascular malformations

Capillary malformation(also known as port-wine stains): Capillary malformations are flat, reddish lesions that typically affect the skin, mostly around the head and the neck, and which darken with age, in contrast to birthmarks such assalmon patch, Nevus simplex or vascular stain, which lighten or disappear within the first few years of life. Capillary malformations constitute 11% of the vascular malformations. Syndromes associated with capillary malformations are: Sturge-Weber syndrome and Klippel-Trenaunay syndrome. Capillary malformations can be treated with IPL-(Intensed-pulsed-light)-therapy or surgical reduction.

LEGEND: Typical Capillary Malformation of the right hand

Venous malformationis a bluish lesion compressible on palpation; the masses enlarge with physical activity or if in a dependent position. The bluish lesion is caused by dilated venous vessels. Venous malformations can be painful in the morning due to stasis and microthrombi within the veins. Venous malformations usually occur in the head and neck.^[12]Venous malformations are the most common vascular anomaly, making up 40% of all vascular malformations.

They can be treated with sclerotherapy and surgical reduction.

LEGEND: Extended Venous Malformation of the lower face involving

skin, muscles and the lower jaw

Lymphatic malformationis a benign growth of the lymphatic system.^[14] They result from a blockage or defect of the lymphatic vessels as they are forming. 28% of all vascular malformations are lymphatic malformations. Lymphatic malformations can be treated with sclerotherapy and surgical reduction.

LEGEND: Giant Lymphatic Malformation of the abdomen of a 4 year old girl

2. Fast flow vascular malformations

All fast-flow malformations are malformations involving arteries. They constitute about 14% of all vascular malformations.

Arterial malformation
Arteriovenous fistula(AVF): a lesion with a direct communication via fistulae between an artery and a vein.
Arteriovenous malformation: a lesion with a direct connection between an artery and a vein, without an intervening capillary bed, but with an interposed nidus of dysplastic vascular channels in between.

LEGEND: Arteriovenous Malformation of the chest of a 9 year old boy

3. Combined-complex vascular malformations

a combination of various vascular malformations. They are ‘complex’ because they involve a combination of two different types of vessels.

CVM: capillary venous malformation
CLM: capillary lymphatic malformation
LVM: lymphatic venous malformation
CLVM: capillary lymphatic venous malformation. CLVM is associated withKlippel-Trenaunay syndrome
AVM-LM: Arteriovenous malformation- lymphatic malformation
CM-AVM: capillary malformation- arteriovenous malformation

EVALUATION

Clinical examination by a multidisciplinary team (Plastic Surgeon, Interventional Radiologist, Pediatrician, ENT surgeon, Opthalmologist etc.)
Genetic Counseling and Testing in selected cases
Xrays
Ultrasounds
MRI

LEGEND: MRI depiction of a NICH hemangioma of the scalp in 1 year old baby

TREATMENT

Treatment for hemangiomas

Treatment for hemangiomas depends on their size, location, and severity. Treatment is usually not recommended for small, noninvasive hemangiomas, since they will become smaller (involute) on their own.

However, hemangiomas that cause bleeding problems, feeding or breathing difficulties, growth disturbances, or impairment of vision may require medical or surgical intervention.

Treatment may include the following:

Steroid medications
Embolization of the blood vessels (injection of material into the blood vessels to block the blood inflow)
Laser or surgical removal
An oral medication called Propranolol: this is classically a heart medication that has been safely used to treat symptomatic hemangiomas.

Treatment for vascular malformations

Treatment for vascular malformations depends on the type of the malformation. Each type of malformation is treated differently.

LASER

Laser therapy is usually effective for capillary malformations or port wine stains, which tend to be flat, violet or red patches on the face.

Emboliazation

Arterial or arteriovenous malformations are often treated by embolization prior to surgical excision (blood flow into malformation is blocked by injecting material near the lesion).

LEGEND: Photo of an embolization procedure in the operating room

Sclerotherapy

Venous malformations and lymphatic malformations are usually treated by direct injection of a sclerosing (clotting) medication which causes clotting of the channels.

LEGEND: Pre-sclerotherapy Venous malformation of the right nostril (left). Post-sclerotherapy Venous Malformation of the right nostril (right)

SURGERY

There are surgical options for some lesions

Excision
Plastic surgery reconstruction with various techniques

LEGEND: Preoperative photos of AVM of the Thigh of a 14 year old girl (top). Intraoperative photo of incision design (middle left). Excised AVM (middle right). Defect after the excision (lower left). Immediate result after reconstruction (lower right)

Pediatric Plastic Surgery

CLEFT LIP

CLEFT PALATE

OTHER RELATED PROBLEMS

· Feeding Problems

· Middle Ear Fluid Buildup and Hearing Loss

· Dental Problems

· Speech Problems

TREATMENT

DENTAL / ORTHODONTIC TREATMENT

CONGENITAL FACIAL PARALYSIS / MOEBIUS SYNDROME

CONGENITAL HAND DEFECTS

CONGENITAL MELANOCYTIC NEVUS (CMN)

CONGENITAL BLEPHAROPTOSIS

MICROTIA

Unilateral Microtia (One Side)

Bilateral Microtia (both sides affected)

Timing of Surgery in Bilateral Microtia

Combining Surgical Stages in Bilateral Microtia

OBSTERICAL BRACHIAL PLEXUS PALSY (OBPP)

DIAGNOSIS AND TESTS

A.Nonsurgical treatment options for brachial plexus injuries

B. Surgical treatment options for brachial plexus injuries

Preparation for surgery

Operative techniques

LONG TERM MONITORING AND FUTURE THERAPIES

OTOPLASTY

VASCULAR ANOMALIES

What is a hemangioma?

What is a vascular malformation?

What is the difference between a hemangioma and a vascular malformation?

1. Infantile Hemangioma

2. Congenital Hemangioma

3. Kaposiform Hemangioendothelioma

4. Pyogenic granuloma

1. Slow-flow vascular malformations

2. Fast flow vascular malformations

All fast-flow malformations are malformations involving arteries. They constitute about 14% of all vascular malformations.

3. Combined-complex vascular malformations

Treatment for hemangiomas

Treatment for vascular malformations