INTRODUCTION

Microsurgery is a procedure performed on parts of the body that require a microscope to be viewed and operated on. These include small blood vessels, nerves, and tubes. Microsurgery is typically performed on the areas of the ear, nose, and throat because these have small and delicate structures.

The procedure, which uses an operating room microscope or a high-powered loupe magnification, is usually not the first option for the treatment of reconstructive problems, but it can be used to fix complex reconstructive issues in place of other procedures such as primary closure, skin grafting, healing by secondary intention, or local and regional flap transfer. This way skilled Microsurgeons are able to use a patient’s own tissue to reconstruct parts of the body affected by cancer, known as reconstructive microsurgery.

After isolating tissue from one part of the body on an artery and vein, surgeons can completely detach the tissue and transfer it elsewhere in the patient’s body for reconstruction. Usually referred to as a “free flap,” this tissue may be composed of skin, fat, muscle or even bone, or a combination of these structures.

Free tissue transfer is accomplished with the aid of a microscope that allows magnification up to 50 times that of the naked eye. Using stitches finer than a single hair, surgeons are able to reconnect the tiny blood vessels from the free flap to recipient vessels in the area of the patient’s body affected by cancer. Because these blood vessels are very small, measuring 1-3 mm in diameter, the microscope enables surgeons to work with precision, and to avoid complications such as clotting or kinking.

The process of using a patient’s own tissue with healthy blood flow promotes healing, reduces scarring and creates a more natural-appearing reconstruction. The technique also minimizes damage to the area of the body (donor site) where tissue is removed, providing optimal surgical results, faster recovery and decreased rates of complications.

INDICATIONS

Plastic surgery also now heavily relies on microsurgery for the reconstruction of damaged skin, muscles, and tissues, and it is also very helpful in the reattachment or replantation of amputated body parts.

Thus, people who should undergo the procedure are those who need the following surgery or treatment:

1. Reattachment of an amputated body part
2. Complex reconstructive plastic surgery involving the jaws, ear, nose, and throat, muscle and skin
3. Reconstruction of any area of the body after removal of complex cancer
4. Reconstruction of any body part after trauma
5. Restoration of certain functions like paralysis of the face or any other paralysis of due to injury to nerves

MICROSURGGERY EQUIPMENT

The equipment used for a microsurgery helps magnify the operating field and allows precision in movement despite the high magnification. The magnification allows for operation of structures that are otherwise very hard to see because of their microscopic size.

The tools most vital to a microsurgery are the microscope, microsurgical instruments, and a microscopic mat.

LEGEND: Perfoming microsurgery with the help of a surgical microscope

The different microsurgical instruments used for microsurgery are specially designed. These instruments are made capable of maneuvering even microscopic structures within the body.

Specialized threads and needles are used for suturing and stitching. Each suture thread used for this procedure has a size that depends on the kind of procedure it is used for.

MICROSURGERY TRAINING

For a surgeon to perform microsurgery in a clinical setting, extensive training and practice are required. After a thorough introduction to the operating microscope and other microsurgical equipment, basic techniques are introduced using small animals as the experimental model.

Specifically, surgeons must be taught how to maintain correct posture and to maintain constant visual contact with the microscope during surgery, how to properly hold and use the instruments, how to minimize the amount of hand tremor, and how to perform basic techniques, such as suturing. After becoming proficient at these skills, more advanced techniques can be taught, including procedures regarding how to treat specific conditions.

Extensive and ongoing practice is necessary for a surgeon to maintain adequate proficiency at microsurgical techniques.

SURGICAL TECHNIQUES

Most microsurgical procedures utilize a set of basic techniques that must be mastered by the surgeon. These include blood vessel repair, vein grafting, and nerve repair and grafting.

• BLOOD VESSEL REPAIR.

Blood vessel, or vascular anastomosis, is the connection of two cut or separate blood vessels to form a continuous channel. Anastomoses may be end-to-end (between two cut ends of a blood vessel) or end-to-side (a connection of one cut end of a blood vessel to the wall of another vessel).

LEGEND: Left arrow shows an example of vein to vein microsurgical connection (anastomosis) and the right arrow shows an artery to artery microsurgical connection (anastomosis). These are photos taken from the camera of the microscope

• VEIN GRAFTING.

Vein grafting is an alternative procedure to end-to-end anastomosis and may be pursued if cut ends of a blood vessel cannot be attached without tension. Nonessential veins similar in diameter to the recipient blood vessel can be removed from the hand, arm, or foot and used as bridges to reconnect injured arteries or veins.

• NERVE REPAIR.

The process of connecting two cut ends of a nerve is called neurorrhaphy, or nerve anastomosis. Peripheral nerves are composed of bunches of nerve fibers called fascicles that are enclosed by a layer called the perineurium; the epineurium is the outer layer of the nerve that encases the fascicles. Nerve repair may involve suturing of the epineurium only, the perineurium only, or through both layers.

• NERVE GRAFTING.

If there is a large gap between the cut ends of a nerve, neurorrhaphy cannot be performed without creating tension in the nerve that can interfere with postsurgical function. A piece of nerve from another part of body may be used to create a nerve graft that is stitched into place using anastomosis techniques. A disadvantage to nerve grafting is that a loss of function or sensation is experienced from the donor nerve site. A common nerve used for grafting is the sural nerve, which innervates parts of the lower leg.

LEGEND: The arrow and the tip of the instrument show a nerve to nerve suturing (coaptation). Photograph taken with the microscope camera

COMMON MICROSURGICAL PROCEDURES

Microsurgery makes possible a number of reconstructive procedures that would be more difficult or impossible with conventional surgery. Some of the more frequently performed microsurgical procedures include:

• Replantation. This emergency surgery is performed to reattach an amputated body part such as a finger, arm, or foot. Replantation surgery requires a series of time- and energy-intensive steps to reattach all of the structures while the amputated part is still viable. The cut bone must be shortened slightly so that blood vessels and nerves can be reattached without tension. Anastomoses are created between cut arteries and veins and blood flow is reestablished to the amputated part. Tendons (if present) are then repaired, followed by nerves and soft tissues. Further procedures may be necessary to completely the reconstruction depending on the extent of the injury.
• Transplantation. In some cases an amputated part cannot be reattached, or tissue is deformed because of a congenital defect or an injury. Transplantation may then be an option. The great toe or second toe may be removed from a patient’s foot and transplanted to the hand to replace a missing finger. A segment of rib along with its blood supply can be used to reconstruct bones in the face and jaw.

• Free-tissue transfers. Also called free flaps, free-tissue transfers may be used to reconstruct damaged tissues that cannot be treated with skin grafts, closed by traditional methods such as suturing, or allowed to heal without intervention. This includes tissues that have constricted after a burn, injuries in which there is not sufficient skin to properly close the wound, or tissues that have been removed as a result of treatment for cancer. Examples of tissues that may be transferred with microsurgical techniques are skin, muscle, fat, bone, and intestine.

 

RECOVERY

After the surgery, patients are given intravenous fluids and placed on a liquid diet for about 12 to 24 hours and a regular diet soon thereafter. They are also advised to take a lot of caution and rest and provided round-the-clock assistance or care.

The surgery also requires patients to keep warm and to stay properly hydrated following the procedure. Most importantly, the area of the body, where the surgery was performed, must be kept in an elevated position so that the excess fluids would drain properly. Some pain may be expected after the surgery, but this is easily managed with pain medications.

As a result of the surgery, the skin in the part of the body operated on might feel warm and become flushed or reddish. The area must be closely observed and any changes in its color, temperature, capillary refill, and tissue turgor or fullness must be reported to the doctor.

Certain tests may be recommended to further evaluate the surgical site. These include:

1. Doppler ultrasound.
2. Intravenous fluorescein.
3. Pulse oximetry.
4. Arteriography.

Exposure to tobacco must be limited for at least six weeks following the surgery, as nicotine interferes with circulation. The patient must remain warm as body temperature also affects circulation. Bed rest may be prescribed for a period of days to weeks after surgery, depending on the procedure. Patients who have had a hand, finger, or multiple fingers replanted must keep the part elevated at heart level to help blood flow and decrease swelling.

Some form of rehabilitation is often recommended after microsurgery. This includes a program of individualized exercises used to restore function to a replanted or transplanted body part.

RISKS

All surgeries have risks particularly those that are complex and involve microscopic body parts. Microsurgery may cause the following complications and risks:

1. Flap congestion
2. Fat necrosis
3. Hematoma
4. Infection
5. Wound breakdown/complication
6. Systemic complications associated with anesthesia
7. Deep vein thrombosis and pulmonary embolism

DEFINITION

At its most basic level, a flap is a portion of tissue that can be dissected, elevated, and inset into a non-anatomic position as a consequence of its vascular supply and outflow. By this definition, flaps may be composed of any combination of skin, fascia, muscle, and bone.

Free tissue transfer is defined as the vascular dissection and detachment of an isolated and specific region of the body (eg, skin, fat, muscle, bone) and transfer to another region of the body, with anastomosis of the divided artery and vein to a separate artery and vein located at the site of the defect. The latter portion of this procedure ensures the perfusion and drainage, and ultimately the survival, of the flap. This ability to transplant living tissue from one region of the body to another has greatly facilitated the reconstruction of complex defects.

The numerous advantages of this technique include stable wound coverage, improved aesthetic and functional outcomes, minimal donor site morbidity, and the ability to utilize vascularized tissue from remote parts of the body that are outside the zone of injury (trauma, malignancy, infection, irradiation, etc). Since the introduction of free tissue transfer in the 1960s, the success rate has improved substantially, currently being 95-99% among experienced surgeons.

LEGEND: A free flap “carries” its own blood supply in the form of a pedicle that consists of an artery, a vein and many times a nerve

INDICATIONS

Free tissue transfer currently is used for the reconstruction of complex defects and disorders throughout the body.

1. After removal of complex tumors in various parts of the body, i.e., after mastectomy, after sarcoma surgery, after removal of big skin cancers.
2. Reconstruction of defects after major trauma
3. Reconstruction of complex congenital defects

 

TYPES OF FREE FLAPS

Free flaps are mainly classified regarding to the tissue they consist of:

1. Free cutaneous flaps
2. Free perforator flaps
3. Free musculocutaneous flaps
4. Free muscle flaps
5. Free osseous flaps
6. Free osteocuteneous flaps

LEGEND: Different type of flaps from various places of the body

PREOPERATIVE CONSIDERATIONS

Preoperative preparation is an essential component of the successful free tissue transfer. Preoperative evaluation includes analysis of the recipient site, consideration of available donor sites, and the clinical status of the patient. Proper patient selection is of utmost importance when analyzing outcomes. The specific factors are reviewed below.

ANALYSIS OF RECIPIENT AND DONOR SITES

Factors related to the recipient site include the size, depth, and location of the defect; quality of the surrounding tissue; exposure of vital structures or hardware; zone of injury; presence of bacterial colonization or infection; previous irradiation;  and functional and aesthetic considerations.

Factors related to the donor site include appropriate tissue match; length of the vascular pedicle; caliber of recipient vessels; surface area, volume, and thickness of the flap; and donor site morbidities.

CLINICAL STATUS OF THE PATIENT

The clinical status of the patient depends on a variety of factors that also may impact the free flap. These include age, nutritional status, tobacco usage, and the presence of underlying comorbidities (eg, diabetes mellitus, cardiopulmonary disease, peripheral vascular disease).

Although advanced age and tobacco use are not contraindications to free-flap operations, poor nutritional status can impede wound healing and recovery.

Patients with poorly controlled diabetes mellitus and peripheral vascular disease require adequate glucose control and may need revascularization procedures prior to free tissue transfer. Surgical clearance by a medical physician is recommended for patients with multiple medical problems.

DONOR TISSUES

Specific donor tissues are variable, and donor sites are chosen based on recipient site requirements. Available tissues include muscle, musculocutaneous, fasciocutaneous, osteocutaneous, and bone flaps. In general, free muscle flaps are indicated for soft tissue coverage of bone and synthetic materials and to obliterate a large dead space.

• Innervated muscle flaps are useful for facial reanimation operations and for upper extremity reconstruction
• Musculocutaneous free flaps are useful for large defects requiring aesthetic contouring
• Fasciocutaneous flaps permit tendon gliding in the extremities and provide excellent contouring of the head and neck
• Osseous and osteocutaneous free flaps are useful for segmental bone defects involving the mandible and extremities
• Adipocutaneous or perforator flaps are especially useful to minimize donor site morbidity
• For the irradiated wound, free tissue transfer is recommended and has been demonstrated to be safe and well tolerated, with no increased rate of partial or total free flap loss

INTRAOPERATIVE CONSIDERATIONS

The operative portion of the free tissue transfer requires absolute attention to detail. Numerous factors must be considered to predictably obtain a successful outcome. These include use of appropriate medications and solutions, properly functioning equipment and instruments, anastomotic issues, and flap insetting.

POSTOPERATIVE MEDICATIONS

Using postoperative medications to inhibit clot formation at the anastomosis is controversial. Studies evaluating the efficacy of heparin, dextran, and aspirin have demonstrated that none of these medications is absolutely necessary for an uncomplicated anastomosis.

RISKS

1. Infection
2. Hematoma (bleeding)
3. Partial flap loss
4. Total flap loss
5. Donor side morbidity
6. Ugly scarring
7. Reoperation

RESULTS

Usually 95% of all transplanted free flap procedures are successful and the transplanted free flaps survive for ever. In some cases depending on various  reasons part of the flaps may not survive and then small ancillary procedures may be needed to complete the reconstruction.

CASE EXAMPLE

LEGEND: 34 year old male with a severe working accident (avulsion of the left lower arm), he almost had his arm amputated. Initial treatment consisted of placement of an external fixator to heal the bone fractures. He developed osteomyelitis (bone infection) and soft tissue infection with a chronic wound

LEGEND: The first stage surgery consisted of excision of the infected bones, placement of a new ILIZAROV fixator and coverage of the wound with a microsurgical  free muscle flap transfer from the back (latissimus dorsi flap). Left photo shows how the free flap is harvested from the back and the right photo shows how the flap covered the wound

LEGEND: The X-ray on the upper left photo shows the gap between the bones (radius and ulna) of the arm. During the second stage surgery, the gap between the bones was reconstructed with a microsurgical free fibula bone flap from the leg (upper right and lower left photos). The xray on the bottom right photo shows how the bone gap was bridged with the free fibula with screws and plates.

INTRODUCTION

A nerve injury can result in weakness or paralysis of a muscle or in numbness of an area of skin. In some people, it also causes pain. To understand nerve injury and recovery, it is important to understand the different types of nerve injury. The type of nerve injury will determine the type of treatment.

PERIPHERAL NERVE ANATOMY

The human nervous system consists of four parts: the brain, spinal cord, autonomic nervous system, and peripheral nerves. Peripheral nerves are cord-like structures containing bundles of nerve fibers that carry information from regions of the body to the spinal cord (and vice versa). Constructed in a fashion similar to telephone cables, peripheral nerves have a tough outer layer of connective tissue that surrounds discrete groups of miniscule fibers, called “axons,” each originating from its own nerve cell.

PERIPHERAL NERVE FUNCTION

The primary function of a peripheral nerve is to transmit signals from the spinal cord to the rest of the body, or to transmit sensory information from the rest of the body to the spinal cord. For example, to flex a muscle, the brain sends a signal to the spinal cord that is received by a motor neuron in the spinal cord. This neuron sends the signal down an axon, which may be up to 3 feet long. This axon terminates in a muscle, where the impulse to flex is received. Once the muscle receives this signal, it flexes. The whole process requires only a fraction of a second to occur.

 PERIPHERAL NERVE INJURY AND HEALING

The central nervous system includes the brain and spinal cord. Injuries in these locations have only a very limited capacity to heal, because nerve regeneration tends not to occur. In contrast, peripheral nerves have a striking capacity for regeneration. Even completely severed peripheral nerves, if repaired in a timely fashion, can regrow, allowing the patients to enjoy complete, or nearly complete recovery in many cases.

When a peripheral nerve is cut, the axon segments distal to the injury (furthest away from the spinal cord) die off in a process called “Wallerian degeneration.” When the nerve is repaired, the axons in the proximal segment (closest to the spinal cord) regrow into the distal, denervated segment. This growth occurs at a rate of about 1 mm per day.

Once the axons regrow back into the denervated muscles, the muscles will begin to function again. During the time it takes for the axons to regrow into the muscles, a process that can take many months, or even years, the muscles will be paralyzed and will atrophy.

LEGEND: Partially cut nerve as seen with the help of a magnifying microscope

This is an important point when dealing with peripheral nerve injuries. The healing process almost invariably requires an extensive amount of time to occur. It is important for patients not to lose hope during this time. It is vital that they participate in their exercises, keeping the affected muscles and joints flexible and ready to be used once again when the axons re-grow into them.

It is not unusual for patients to undergo a lengthy, complex, peripheral nerve reconstruction procedure, only to see no evidence of recovery for a year or more. This can be immensely frustrating for the patient. Unfortunately, there is nothing in medical science currently that can make these axons grow any faster.

CAUSES OF NERVE INJURY

Injury to the peripheral nerves can occur through a variety of trauma. Common causes of nerve injuries include:

1. Laceration / stab wounds / sharp injuries
2. Focal contusion (gunshot wounds)
3. Stretch/traction injury
4. Compression / blunt injuries
5. Drug injection injury
6. Electrical injury

NERVE INJURY CLASSIFICATION

Nerve injuries are classified as follows:

1. A first-degree injury, or neurapraxia, will recover within days after the injury, or it may take up to three months. The recovery will be complete with no lasting muscle or sensory problems.
2. A second-degree injury, or axonotmesis, also will recover completely; however, the recovery will take much longer than with a first-degree injury.
3. A third-degree injury also will recover slowly; in addition, only partial recovery will occur.
4. A fourth-degree injury occurs when there is dense scar tissue within the nerve, completely blocking any recovery. Surgery is required for recovery.
5. A fifth-degree injury involves complete separation of a nerve, such as a cut nerve. Surgery is required for recovery.
6. A sixth-degree injury is a combination of other types of nerve injury. Recovery and treatment will vary depending on which types of nerve injury are present.

CONSULTATION

• History

A detailed history provides information about the symptoms experienced by the patient, how long they have been present, and how they have progressed. Mechanism and timing of injury, muscle weakness, numbness, and pain are all important components of the history.

• Neurological Examination

The peripheral nerve examination tests motor and sensory function to help localize the region of injury. Muscle atrophy and changes in the skin and nails can be helpful as well.

During the neurological examination many different muscles are tested. In addition, your sensory systems and reflexes will be tested too.

• Electrodiagnostic Studies

The most important diagnostic study in the evaluation of peripheral nerve problems is electromyography (EMG) with nerve conduction studies (NCS). This study provides accurate, quantitative information on the function of nerves and muscles and helps to more precisely localize the region of injury, and to pick up more subtle signs of injury and/or recovery that are otherwise undetectable to the examiner.

• Imaging Studies

Any imaging (MRI, CT, X-rays, etc.) will be reviewed.

Imaging studies can be quite helpful in patients with peripheral nerve problems. For example, patients who have peripheral nerve tumors almost invariably require an MRI. Such detailed pictures facilitate the planning of surgery to treat peripheral nerve tumors.

CT myelography is used to evaluate brachial plexus traumatic injuries. Patients who have suffered severe brachial plexus trauma may actually have one or more avulsion injuries, in which the nerve roots are actually pulled out from the spinal cord in the neck. A CT myelogram is really the only way to reliably document this type of injury. To perform a CT myelogram, contrast dye is injected along into the spinal canal. Then spinal X-rays and a spine CT are performed.

NONSURGICAL TREATMENT

1. Acupuncture
2. Massage therapy
3. Medication
4. Orthotics
5. Physical therapy and rehabilitation

• Weight loss management

 INDICATIONS FOR SURGERY

If you have sustained a peripheral nerve injury that resulted in a neurological deficit such as severe weakness or numbness, and it has not improved with three months of non-operative management, then an operation is likely indicated.

If you have a peripheral nerve entrapment syndrome causing a neurological deficit and/or pain, and your symptoms progress or fail to improve with a trial of non-operative management, then you might be a candidate for surgery.

PREPARING FOR THE SURGERY

You will need to have preoperative medical clearance. This is a process whereby your personal physician (generally an internist, cardiologist, or other primary care physician) checks some blood and urine tests, an electrocardiogram, and a chest x-ray to see whether you have any medical conditions that might cause problems during or after your operation. For example, your blood pressure should be well-controlled, as should your blood sugar. Some patients may require additional testing before medical clearance is granted. For example, those with a history of heart disease may require a echocardiogram or a stress test in addition to the standard electrocardiogram.

The evening before your surgery, the hospital will notify you of your time of surgery, and what time you should arrive at the hospital. Other general instructions will include having nothing to eat after midnight the evening before surgery. Notify your physician if you do not receive instructions by 8 or 9 PM the evening prior to your surgery.

PERIPHERAL NERVE SURGERY

If surgery is necessary, there are several types of surgery that may be recommended. 

• PRIMARY NERVE REPAIR (END TO END REPAIR)

For nerve regeneration, the regenerating nerve fibers need the guidance of the nerve for direction to the muscle or sensory unit. If the nerve has been cut, a nerve repair is used to sew the two ends of a nerve together. This usually is possible when the nerve has been cut sharply.

LEGEND: Example of suturing the two ends of the median nerve of the hand after the nerve was cut with after a knife injury to the wrist level

• NERVE GRAFTING

However, in cases with more extensive damage, it may not be possible to sew the two ends of the nerve directly together once the damaged nerve has been trimmed away. In these cases, a nerve graft is used. A small piece of donor nerve is used to bridge the gap between the two nerve ends, and the nerve will regenerate across this “bridge” to provide recovery. The donor nerve is taken from other areas of your body using small, noncritical sensory nerves. 

LEGEND: Example of reconstruction of damaged brachial plexus nerves with nerve grafts

• NERVE TRANSFER

In some cases in which sensory or muscle recovery is not anticipated for a very long time, a nerve transfer may be used. Nerve transfers use functioning nerves that are close to the target muscle or sensory area, and the nerves are transferred to the injured nerve. 

LEGEND: Example of reconstruction of damaged brachial plexus nerves with nerve transfer

• NEUROLYSIS

A neurolysis (or nerve decompression) refers to the removal of scar or compressive structures (including fascia or tendonous edges of muscles) from the nerve and may be undertaken if external impediments (or tight “tunnels”) are pinching the nerve, limiting the ability of the regenerating unit to pass through on its way to its target. 

REHABILITATION

Rehabilitation of nerve injuries should focus on avoiding complications and further disability, improving function of the muscle, and enhancing recovery after surgical repair.  Contractions can occur after injury to a nerve supplying a muscle.  Patients and caregivers need to be taught passive range of motion exercises to be done daily to prevent contractures. 

Use of the limb should be encouraged for daily activities whenever possible.  There are several benefits of using the limb early.  It will prevent atrophy of the muscles from disuse and will help prevent contractures.  The use of the limb may also participate in the improvement of the nerve function. 

Some rehabilitation specialists use electrical stimulation of the muscles in order to prevent atrophy.  The use of this practice is considered controversial.  It is difficult to say that no form of electrical stimulation will be helpful in regaining muscle function. 

Ultrasound is another physical modality that may enhance recovery after peripheral nerve injury. 

Splinting is an important part of rehabilitation care after a peripheral nerve injury. Splinting can prevent contractures and can substitute for loss of motor function. 

RECOVERY

The patient is immobilized in a bulky dressing for a few days postoperatively. The postoperative dressing (including the drain and pain pump, if used) is removed 2-3 days after the procedure.

The area of nerve repair then is immobilized for a longer time postoperatively (nerve graft, 10-14 days; nerve repair, 3 weeks; nerve transfer, 7-10 days), though the patient is instructed in range-of-motion exercises for the joints proximal and distal to the immobilized region. For example, a median nerve repair at the wrist would be immobilized with a wrist-resting splint, and the patient would continue with range of motion for the fingers, elbow, and shoulder.

After the surgical procedure, the patient is sent to the hand therapist, initially for the splint and then for exercises. Initially, the goals of postoperative therapy are to regain passive range of motion of the joints and soft tissues that have been immobilized.

RESULTS

Initially, the patient is monitored for postoperative wound healing. After immobilization and once full passive range of motion has been regained, the patient is monitored every few months to evaluate for evidence of reinnervation.

With nerve regeneration, a Tinel sign progresses distally along the nerve. With muscle reinnervation, a muscle contraction is visible; and with sensory reinnervation, the patient responds to light touch.

Depending on the level of injury, the patient may continue to progress for varying periods. A basic rule of thumb is that nerve injuries recover with a rate of 1mm/day. Distal injuries respond more quickly than proximal brachial plexus injuries, which respond for 2-3 years after surgery.

RISKS

Complications from surgery include infection, bleeding and delayed wound healing of the primary surgical site or the donor nerve site in cases of nerve graft. Since this type of surgery involves nerves, there is a risk that your nerve function in the region of your injury – with respect to sensory or motor function – will be lessened, or pain may develop.

CASE

LEGEND: 32 year old male sustained an industrial accident (left photo), cutting all the tendons and the median nerve of the right wrist (“spaghetti wrist injury”). Right photo shows primary repair (end to end) of the nerve

INTRODUCTION

Facial paralysis is a loss of facial movement due to nerve damage. Your facial muscles may appear to droop or become weak. It can happen on one or both sides of the face. Facial paralysis can come on suddenly (in the case of Bell’s palsy, for example) or happen gradually over a period of months (in the case of a head or neck tumor). Depending on the cause, the paralysis might last for a short or extended period of time.

LEGEND: The facial nerve has five major brunches that control the function of 17 sets of muscles in the face

CAUSES

1. Idiopathic (Bell’s palsy) – most common
2. Cerebrovascular disease (stroke).
3. Iatrogenic
   1. Local anaesthetic for dental treatment.
   2. Following removal of the parotid gland
   3. Following removal of an acoustic neuroma 
4. Infection
   1. Herpesvirus (type 1).
   2. Herpes zoster (Ramsay Hunt syndrome)
   3. HIV.
   4. Epstein-Barr virus.
   5. Cytomegalovirus.
   6. Otitis media or cholesteatoma.
5. Trauma:

1. Fractures of the skull base.
2. Forceps delivery. 
3. Haematoma after acupuncture. 

6. Neurological:
   1. Guillain-Barré syndrome.
   2. Mononeuropathy
7. Neoplastic:
   1. Parotid gland tumours.
8. Hypertension in pregnancy and eclampsia.

SYMPTOMS

1. Inability to raise an eyebrow
2. Inability to close an eyelid and protect the eyeball from drying out
3. Looseness or sagging of the lower eyelid, leading to tears dripping down the cheek
4. Unbalanced or asymmetric facial structures.
5. Inability to smile
6. Inability to raise, lower or pucker the lips, leading to dribbling when drinking and slurring of some words when speaking
7. Aching pain below the ear or in the mastoid area is also common and may suggest middle ear or herpetic cause.

PROGNOSIS

1. 85% improve spontaneously within three weeks of its onset.
2. 71% recover fully.
3. 16% have significant sequelae, 5% severe:

1. • Facial asymmetry
   • Gustatory lacrimation
   • Inadequate lid closure
   • Brow ptosis
   • Drooling
   • Hemifacial spasms

 

CONSULTATION

A detailed examination of facial appearance at rest and with movement is then recorded with photographs. Any associated functional deficits with eye closure and oral competence are evaluated and documented. After review of individual’s scope of deficits and concerns, reconstructive and rehabilitative options are proposed and discussed. In many cases of facial paralysis, depending on individual needs, multiple treatments may be necessary in order to achieve an optimal aesthetic and functional result. Specialty services, such as ophthalmology, neurology, and physical therapy are also available and consulted on as needed basis.

 DIAGNOSTIC TESTS

1. Serology – Lyme, herpes and zoster (paired samples 4-6 weeks apart). It may not influence management but may reveal aetiology.
2. Schirmer’s tear test (reveals a reduced flow of tears on the side of a palsy affecting the greater palatine nerve).
3. Stapedial reflex (an audiological test, absent if the stapedius muscle is affected).
4. Electrodiagnostic studies (generally a research tool) reveal no changes in involved facial muscles for the first three days but a steady decline of electrical activity often occurs over the next week and will identify the 15% with axonal degeneration.
5. CT scan or MRI scan of the head and neck in certain cases
6. Angiography of the arteries and veins of the neck especially if free functioning muscle transplant is elected for the therapy

TREATMENT

Except in the mildest cases, ideally this should be a multidisciplinary approach, encompassing:

• Plastic surgeons
• Neurologists
• Ophthalmologists
• ENT surgeons
• Physiotherapists and
• Psychologists

Treatment options for facial paralysis and associated movement disorders are numerous and vary based on individual deficits, needs, and preferences.

Acute Facial Paralysis (0 days – 21 days)

Identifiable causes of acute paralysis are treated expediently with appropriate medical therapy, following proper identification of the cause (diagram – acute paralysis with facial nerve anatomically intact).

In rare instances, surgical intervention may be necessary to control infection and/or swelling around the facial nerve.

In a setting of facial nerve transection, such as during trauma or resection of cancer invading the facial nerve, several reconstructive options are available. These assist in minimizing sequelae of paralysis, optimize immediate patient recovery, and promote the return of facial nerve function (diagram – acute paralysis with facial nerve injury / transection).

Intermediate Facial Paralysis (21 days – 2 years)

During this stage, facial nerve recovery is monitored with serial EMG (electromyography) exams, which provide useful prognostic data. In a setting of poorly recovering facial nerve, several procedures can be considered to restore facial appearance and rehabilitate function around the eye and mouth (diagram – intermediate facial paralysis).

In the early stages, at 6 weeks – 3 months, gold weight placement to aid upper eyelid closure and static sling suspension of the mid-face and lip can be performed with minimal associated downtime.

In the later stages, if the facial nerve continues to display poor recovery on EMG, consideration is given to nerve transfer procedures designed to maintain neurological input of facial muscles. A graft from a nearby nerve, most commonly hypoglossal (CN 12), can provide such input.

This ultimately allows for preservation of tone in the native facial musculature. An alternative for definitive reconstruction, with vascularized gracilis muscle transfer, is also considered at this stage.

Chronic Facial Paralysis (>2 years)

Management of chronic facial paralysis depends on numerous factors, including patient preferences and desires.

Reconstructive options range from static suspensions to reanimation via muscle transfers to the paralyzed side. Both have its merits and serve a useful purpose in aesthetic and functional rehabilitation of facial paralysis.

Static slings represent the simplest solution with the quickest recovery time. They effectively reposition displaced tissues of the face back to the midline and aid in functional aspects such as lip closure and prevention of food spillage. Native fascia, sutures, and various manufactured materials (Alloderm, Gore-Tex) can be utilized for this purpose. Static slings may relax and descend over time, thus potentially requiring additional tightening.

Muscle transfer allows one to regain symmetry and movement on the paralyzed side. These are more extended procedures with longer recovery times, when compared to static slings. Transfer of the temporalis muscle (one of the muscles of mastication, anatomically adjacent to tissues requiring suspension in facial paralysis) represents a simpler option, however, requires one to bite down in order to activate the smile. The procedure of choice to regain involuntary smile is a 2-stage transfer of the gracilis muscle (transferred from the inner thigh). In the first operation, a branch of the facial nerve on the healthy side is grafted and carried across to the paralyzed side.

Approximately 6-9 months later, after the nerve has grown across, a segment of the gracilis muscle is transferred to the face and connected to the grafted nerve. In approximately 6 months this muscle becomes functional, providing movement on the paralyzed side. The gracilis transfer affords a better precision with respect to the smile angle, when compared to temporalis transfer.

 SURGERY

 Depending on the underlying cause, a number of surgical options exist for preventing or correcting the ocular complications associated with FNP. The following is an overview of the various surgical options that can improve the morbidity associated with FNP. 

1. Gold Weight

A number of patients with FNP develop incomplete eyelid closure requiring surgical intervention to minimize the risk of corneal exposure and damage. Implantation of a gold weight onto the upper eyelid has been shown to be a highly effective option to protect the cornea in such cases.

The placement of a gold weight can be associated with postsurgical complications, including astigmatism, pseudoptosis, migration, bulging, and extrusion of the implant.

LEGEND: Superior eyelid crease incision (a) is performed allowing the suturing of a gold weight (b,c) onto the upper eyelid (d)

 

2. Tarsorrhaphy

Tarsorrhaphy is a reasonable option to consider when preventing and treating corneal exposure. A principle disadvantage of the procedure is the poor cosmetic appearance and compromised peripheral vision.  The advantages of a tarsorrhaphy include its multiple surgical technical variations allowing customization of the procedure to the individual patient, a simple surgical technique and relative ease of reversing the procedure.

3. Canthoplasty

A Canthoplasty refers to any or all of the several procedures that can be used to change the configuration (or position) of the lateral canthus, which means the corner of your eyes where your upper eyelids meet your bottom eye lids.

The procedure involves cutting the insertion of the lateral canthus form the bone (top figure), pulling the canthus towards the corner of the eye in order to tighten and suspend the droopy lower eyelid and fixing it with sutures to the bone (bottom figure). 

4. Suspension of the lower eyelid with tendon graft

During this procedure a 4-6 cm long tendon graft (which is obtained from the palmaris longus tendon a useless muscle from the wrist area) is placed in the lower eyelid and tightened to the inner canthus (the native tendon of the lower eyelid)(left figure) and to the bone of the eye, in the same way as in the cantoplasty surgery (right figure).

5. Primary neurorraphy and interposition cable nerve grafting

Facial nerve grafting is indicated in the situation of partial resection of cranial nerve VII due to trauma or tumor. Both the proximal and distal portions of the nerve must be accessible to the surgeon for the procedure to be done.

Typically, a tension-free closure under microscopic magnification is necessary to assure a high rate of success. The great auricular and sural nerves are the most common sources for the graft.

Interposition nerve grafting typically optimizes facial functioning more than other medical or rehabilitative methods.

LEGEND: Primary reconstruction of the facial nerve with a sural nerve graft (center black bracket). The patient had the parotid removed due to cancer and the facial nerve was sacrificed. The left arrow shows the connection of the nerve graft to the proximal part of the facial nerve and the right arrow shows the connection of the nerve graft to the distal part of the injured facial nerve

6. Hypoglossal–facial nerve transfer (babysitter procedure)

Hypoglossal–facial nerve transfer is typically used for patients with complete FNP that is less than 18 months in duration. 

The hypoglossal nerve is connected to the facial nerve via a nerve graft or direct anastamosis. Only a partial portion of the hypoglossal nerve is harvested, thereby limiting the risk of hemitongue atrophy. This technique allows reinnervation of the facial muscles in order to give tone to the face with limited volitional facial movement.

7. Static suspension

Static suspension or sling of the oral commissure can be successfully employed in individuals with partial or complete FNP. Permanent sutures, acellular dermis, Gore-Tex and tensor fascia lata are the most commonly utilized materials for suspending the ptotic oral commissure.

Static sling can significantly improve oral incompetence and restore facial symmetry at rest. However, this procedure does not provide dynamic reanimation. Static sling is best utilized in patients who are not good candidates for spontaneous or dynamic reanimation.

LEGEND: Fascia lata harvested from the thigh placed on the paralyzed face of a lady with  Bell’s palsy (left). The fascia lata is tunneled under the skin (right figure) and sutured to the nasolabial fold (smile line – top arrow) and pulled upwards to restore symmetry of the face and suspended to the temporal fascia (bottom arrow)

8. Temporalis muscle transfer

Temporalis muscle transfer has been a valuable surgical procedure for patients with complete FNP over the past two decades. The technique utilizes the temporalis muscle that is innervated by the trigeminal nerve to help suspend the oral commissure and provide movement of the face, mainly smile. This is an easy procedure reserved mainly for older patients or patients who cannot go through long surgeries.

LEGEND: The middle portion of the temporal muscle is harvested (left), passed under the zygomatic arch (middle) and sutured to the corner of the mouth (commissure – right figure) 

9. Two-stage cross-facial nerve graft and gracilis muscle transfer

Spontaneous dynamic facial reanimation refers to mimetic emotional smile restoration. Currently, the most commonly employed method of achieving this goal is by the use of a two-stage operation that utilizes a gracilis-free neuromuscular flap powered by the contralateral facial nerve. In the first stage of the operation, the sural nerve is harvested and coapted to the normal contralateral facial nerve.

Typically a branch that innervates the zygomaticus muscle is utilized for reinnervation of the transferred muscle. There is minimal risk of permanent cranial nerve VII injury to the normal side as there is significant arborization of cranial nerve VII in the buccal and zygomatic branches.

LEGEND: Harvesting of a sural nerve graft from the leg

This cross facial nerve graft needs 8-12 months for appropriate neurotization. 

LEGEND: During the cross-facial nerve graft procedure, a branch of the facial nerve on the nonparalyzed side of the face is isolated, transected and sutured to the harvested sural nerve (black arrow). This nerve branch has to specifically stimulate the zygomaticus muscle group only (smile muscle). The sural nerve graft (red line) is tunneled under the skin towards the affected side. The nerve graft will remain buried in this area for approximately 12 months in order to get neurotized for the subsequent gracilis neuromuscular free flap.

In the second stage of the operation, a gracilis-free flap is utilized as a muscle source. The cross-face nerve graft is connected to the motor nerve (obturator nerve) of the harvested gracilis muscle. Within 6–12 months, the muscle shows spontaneous movement with appropriate physical therapy. This approach works best in the younger population but it can also work for patients up to 60 years old.

LEGEND: In the second stage of the procedure, free tissue transfer is performed utilizing the gracilis muscle (left figure). The obturator nerve that supplies motor innervation to the muscle is coapted to the cross-facial nerve graft, and the artery and vein of the muscle are conncted to arteries and veins of the neck area.

 

RECOVERY

Typically a 24 to 48 hour hospital stay is required after a nerve grafting procedure and 3-4 days after a free muscle transfer. All the other procedures described are one day surgery cases

Ambulation is begun on the first post-operative day and a clear liquid diet is utilized for two to three days. In the early post-operative period patients are requested to stay on a soft diet and rinse their mouth after meals. 

Exercise, heavy lifting, pressure to the cheeks and teeth brushing are all to be avoided until clearance is obtained from the operating surgeon. 

 

RISKS

1. Hematoma
2. Infection
3. Asymmetry
4. Loss of muscle flaps
5. Failure of the procedure
6. Ugly scars
7. Non working nerve grafts

RESULTS

Return of facial tone and motion will usually develop between the 9th and 12th post-operative month. Electrical stimulation and a home physical therapy program are frequently initiated several months after surgery. Depending on patient care, specifics to each case, follow-up and recovery times will vary.

CASE 1

LEGEND: 28 year old patient with postoncologic (excision of sarcoma) facial nerve paralysis. Left photo shows inability to close the eye (lagopthalmus). Center photo shows facial asymmetry at rest and right photo shows smiling assymetry 

LEGEND: Same patient following facial reanimation surgery. Left photo shows full closure of the eye after gold weight placement and canthoplasty. Center photo shows facial symmetry at rest and right photo shows strong independent right sided smile after two staged reconstruction with Cross Facial Nerve Grafting and Gracilis Muscle Free Flap transplantation

 

CASE 2    

LEGEND: 72 year old patient with right sided facial paralysis (Bell’s Palsy). Left photo shows inability to close the eye (lagopthalmus) and paralytivc ectropion of the lower eyelid plus major facial asymmetry with corner of the mouth sagging and drooling. Due to the age of the patient facial reanimation surgery consiste of gold weight placement at the upper eyelid and canthoplasty of the lower eyelid and suspension of the face with a static sling (in this case bovine pericardium). Right photo three months after surgery shows facial symmetry at rest and full closure of the eye.

 

CASE 3

LEGEND: 50 year old patient with left sided paralytic lagothalmus

LEGEND: Incision and placement of a gold weight to the upper eyelid

LEGEND: 6 months later the patient can fully close her eye

INTRODUCTION

The brachial plexus is the network of nerves that sends signals from your spine to your shoulder, arm and hand. It allows people to control movements and feel sensations in the arms and hands. A brachial plexus injury occurs when these nerves are stretched, compressed, or in the most serious cases, ripped apart or torn away from the spinal cord.

CAUSES OF INJURY

Damage to the upper nerves that make up the brachial plexus tends to occur when your shoulder is forced down while your neck stretches up and away from the injured shoulder. The lower nerves are more likely to be injured when your arm is forced above your head.

These injuries can occur in several ways, including:

• Contact sports.Many sportsl players experience burners or stingers, which can occur when the nerves in the brachial plexus get stretched beyond their limit during collisions with other players.
• Difficult births.Newborns can sustain brachial plexus injuries when there are problems during birth, such as a breech presentation or prolonged labor. If an infant’s shoulders get wedged within the birth canal, there is an increased risk of a brachial plexus palsy. Most often, the upper nerves are injured, a condition called Erb’s palsy. Total brachial plexus birth palsy occurs when both the upper and lower nerves are damaged.
• Several types of trauma — including motor vehicle accidents, motorcycle accidents, falls or bullet wounds — can result in brachial plexus injuries.
• Inflammation may cause damage to the brachial plexus. A rare condition known as Parsonage-Turner syndrome (brachial plexitis) causes brachial plexus inflammation with no trauma and results in paralysis of some muscles of the arm.
• Noncancerous (benign) or cancerous tumors can grow in the brachial plexus or put pressure on the brachial plexus or spread to the nerves, causing damage to the brachial plexus.
• Radiation treatment.Radiation treatment may cause damage to the brachial plexus. This is called radiation plexitis.

 

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:

1. Avulsion – this means the nerve has been pulled out from the spinal cord and has no chance to recover.
2. Rupture – this means the nerve has been stretched and at least partially torn, but not at the spinal cord.
3. Neurapraxia – this means the nerve has been gently stretched or compressed but is still attached (not torn) and has excellent prognosis for rapid recovery
4. Axonotemesis – this means the axons (equivalents of the copper filaments in an electric cable) have been severed. The prognosis is moderate. 
5. Neurotemesis – this means the entire nerve has been divided. The prognosis is very poor.
6. 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 BRACHIAL PLEXUS INJURY

Less severe injuries

Minor damage often occurs during contact sports, such as football or wrestling, when the brachial plexus nerves get stretched or compressed. These symptoms usually last only a few seconds or minutes, but in some people may linger for days or longer. These are called stingers or burners, and can produce the following symptoms:

1. A feeling like an electric shock or a burning sensation shooting down your arm
2. Numbness and weakness in your arm

More-severe injuries

More-severe symptoms result from injuries that seriously injure or even tear or rupture the nerves. The most serious brachial plexus injury (avulsion) occurs when the nerve root is torn from the spinal cord. Signs and symptoms of more-severe injuries can include:

1. Weakness or inability to use certain muscles in your hand, arm or shoulder
2. Complete lack of movement and feeling in your arm, including your shoulder and hand
3. Severe pain

 

DIAGNOSIS AND TESTS

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

1. History taking and clinical examination
2. Electrodiagnostic studies (EMG, NCV, SNAP, SSEP)
3. Imaging studies (CT, MRI)

4. CT/MYELOGRAPHY

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

TREATMENT

Treatment depends on several factors including the severity of the injury, the type of injury, the length of time since the injury and other existing conditions. Nerves that have only been stretched may recover without further treatment. Your doctor may recommend physical therapy to keep your joints and muscles working properly, maintain the range of motion, and prevent stiff joints. The healing process sometimes forms scar tissue that must be removed surgically to improve the nerve’s function. Surgical repair is often required for nerves that have significant surrounding scar tissue or that have been cut or torn.

Surgery to repair brachial plexus nerves should generally occur within six to seven months after the injury. If nerve surgery occurs more than six to seven months after the injury, the muscles may not recover their function.

LEGEND: The V-shaped incision that is used in the neck to access and repair the brachial plexus

Types of surgical repair

• Neurolysis

This type of surgery is usually performed when the brachial plexus is “squeezed” from excessive scarring, usually after radiotherapy for brachial plexitis. In this surgery the goal is to remove all the scars around the brachial plexus.

LEGEND: Brachial plexus after neurolysis

• Nerve grafting

Nerve grafts are used when a patient has a nerve injury resulting in complete loss of muscle function or sensation.

A nerve graft is a surgical technique in which a segment of unrelated nerve is used to replace or bridge an injured portion of nerve. The donor nerve serves as a “track” along which axons (appendages of neurons, which transmit impulses from the spinal cord to the muscle) can grow down to the target area.

Grafts are selected from nerves that are considered expendable, or much less important than the function being restored. Common examples of nerves that are used for grafts include the sural nerve in the leg, which provides sensation to the side of the foot, and the medial antebrachial cutaneous nerve, which provides sensation to the inner aspect of the arm.

After surgery, a patient will have some numbness in the region of the donor nerve that will become smaller over several years.

LEGEND: Example of how nerve grafts are used to reconstruct a damaged portion of a nerve. In the left figure the area between the arrows depicts the injured portion (neuroma) of the brachial plexus. The injured portion is removed and the gap is bridged with expandable sensory nerves (grafts) that are harvested from the leg (right figure arrow)

LEGEND: Photo of nerve grafts (arrow) used to reconstruct the damaged brachial plexus

• Nerve Transfer

 A nerve transfer is a surgical technique that may be used when a patient has a nerve injury resulting in complete loss of muscle function or sensation.

Nerve transfers involve taking nerves with less important roles — or branches of a nerve that perform redundant functions to other nerves — and “transferring” them to restore function in a more crucial nerve that has been severely damaged.

The surgeon will use functioning nerves that are close to the target muscle or sensory area, and these nerves are transferred or “plugged in” to the injured nerve that no longer functions. The nerve that has been “plugged in” now supplies that function.

For example, if a breathing nerve is used to restore elbow flexion, the patient may initially have to take a deep breath to move the arm. The brain then learns this trick and soon the patient is able to move the arm simply by thinking about moving the arm.

Motor nerves are used to re-innervate muscles and sensory nerves are utilized to restore sensation. This technique provides a nearby source of nerve for faster recovery.

LEGEND: Example of nerve transfer. The 3rd and 4th intercostals nerves (nerves between the ribs) are transferred to the nerve of the biceps muscle (muscle that bents the elbow)

·         Tendon Transfer

Surgeons perform tendon transfers by releasing one end of a tendon from bone or soft tissue and reconnecting it to another bone or tendon.

Tendon transfers are undertaken to restore muscle function and can be used for any kind of nerve injury. Instead of moving the nerve supply from one muscle to another muscle — as is done with a nerve transfer — the surgeon takes the end of a tendon, cuts it and moves it to perform a different function.

Tendon transfers are used to treat many conditions. They are indicated when muscle function is lost due to nerve injury, and the nerve can no longer send signals to the muscle and cannot be repaired. Some common injuries treated with tendon transfers are radial, ulnar and median nerve injury; foot drop due to peroneal nerve injury; and spine injury as well as hand paralysis due to spinal cord injury.

One benefit of moving tendons is that there is no time limitation. The window for successfully repairing nerves is about a year, but a tendon transfer can restore muscle function many years after the initial injury.

LEGEND: examples of tendon transfers. Expandable tendons (flexor tendons in this figure) from the one side of the lower arm are transferred to the other side of the arm to restore a missing function

• Free functional muscle transplant

Free functional muscle transplant involves taking an “extra” muscle and transplanting it somewhere else in the body to restore movement.

For example, the gracilis muscle from the inside of the thigh — which is responsible for some movement in the leg — may be transplanted to replace the biceps in the arm.

The transplanted muscle must be connected to an artery and vein in `its new location to keep it alive. It then must be connected to a healthy nerve so that it can be used to move the limb.

As with tendon transfers, there is no time limit for performing free functional muscle transplants — the technique can restore muscle function many years after the initial injury.

This technique is particularly useful for restoring some very basic hand function after a severe brachial plexus injury.

Following this procedure, the patient must remain in the hospital for several days to ensure that the vessels to the new muscle continue to flow effectively so that it will stay alive and function in an effective manner.

RECOVERY

After the surgery you will have to wear a sling for the first few weeks to protect your arm and aid in wound healing. Your doctor may prescribe medications to relieve pain. Physical therapy involving range of motion exercises for the elbow, shoulder and hand may be advised a few weeks later. You also may be instructed to perform strengthening exercises after your arm movements are regained.

It may take more than a year to achieve complete improvement after surgery. Various factors which influence recovery after brachial plexus surgery are: location and extent of the injury, age of the patient and time interval between the injury and surgery. Younger patients recover faster, whereas recovery is slower (and usually incomplete) in patients over 45 to 50 years of age.

Adults with injury to the C5 and/or C6 nerves generally experience near complete recovery. Patients with complete paralysis and injury to the C5, C6 and C7 nerves may experience difficulty in achieving complete recovery to the original extent.

For patients with complete paralysis (C5,6,7,8 and T1) usually cannot expect complete recovery. However some recently developed techniques which involve direct transfer of nerves from the opposite (normal) brachial plexus have shown some striking results.

RISKS

As with any surgery, there are risks involved. Associated risks of nerve reconstruction surgery may include:

• Bleeding
• Infection
• Some tingling and weakness in areas from which nerves have been borrowed to reconstruct the damage nerves.
• Failure to improve

1. CARPAL TUNNEL SYNDROME

INTRODUCTION

Carpal tunnel syndrome (CTS) is a condition caused by increased pressure on the median nerve at the wrist. There is a space in the wrist called the carpal tunnel, where the median nerve and multiple tendons pass from the forearm into the hand. 

Carpal tunnel syndrome happens when swelling in this tunnel pinches, or compresses, the nerve. The resulting pressure on the nerve within the tunnel causes a tingling sensation in the hand, often accompanied by numbness, aching, and impaired hand function. This is known as carpal tunnel syndrome.

The goal of the treatment is to restore the sensation and the strength of the hand as soon as possible with minimal impact on the patient’s quality of life.

LEGEND: Schematic drawing of the carpal tunnel and its contents. The median nerve is the yellowed colored structure.

CAUSES AND RISK FACTORS

There are several factors that can contribute to the swelling of the carpal tunnel and the compression of the median nerve that can lead to carpal tunnel syndrome:

1. Chronic illnesses such ashypothyroidism, rheumatoid arthritis and diabetes.
2. Repetitive hand and/or wrist movements, especially if the wrist is bent so that the hands are lower than the wrists.
3. Pregnancy

 

SYMPTOMS

 

Common signs and symptoms of carpal tunnel syndrome include:

1. Pain
2. Numbness
3. Tingling
4. Weakness with grip
5. Tendency to drop things
6. Poor sleep due waking up at night due to pain and tingling in the hand
7. In severe cases, thinning of the muscles in the palm

The numbness and tingling often happens in the thumb, index, middle, and ring fingers. This may vary between individual patients. 

LEGEND: In patients with Carpal Tunnel Syndrome(CTS) 3 ½ finger and half the palm have decreased sensation

 

DIAGNOSIS

A thorough physical examination and discussion of the patient’s medical history, including current and past conditions, prior injuries and symptoms, will help diagnose carpal tunnel syndrome.

Depending on the examination of the patient’s hands, additional diagnostic tests may be needed to confirm the presence and extent of carpal tunnel syndrome. These include:

1. Electrodiagnostic nerve studies (EMG)
2. Laboratory tests
3. X-rays

TREATMENT

Treatment will depend on the severity of symptoms, which may be relieved without surgery. 

1. Non surgical treatments

There are several non-surgical treatment options that may help relieve symptoms and reduce the chance of causing long-term damage to the nerve.  In some cases, splinting of the hand and anti-inflammatory medications will relieve the problem.

These include:

1. Avoiding activities that cause numbness and pain
2. Rest for longer periods of time between activities that cause numbness and pain
3. Anti-inflammatory medications such as ibuprofen
4. Wearing a wrist splint
5. Corticosteroid injections into the carpal tunnel

2. Surgery

When symptoms are severe or do not improve, surgery may be the next step. Pressure on the median nerve is decreased with surgical release of the ligament over the carpal tunnel. In the operation, the surgeon makes an incision from the middle of the palm to the wrist. He or she will then cut the tissue that’s pressing on the nerve, in order to release the pressure. A large dressing and splint are used after surgery to restrict motion and promote healing. The scar will gradually fade and become barely visible.

LEGEND: Incision for carpal tunnel

LEGEND: Drawing of carpal tunnel decompression

LEGEND: Decompressed carpal tunnel, opened ligament, exposed median nerve

RESULTS

The results of the surgery will depend in part on how long the condition has existed and how much damage has been done to the nerve. For that reason, it’s a good idea to see a doctor early if you think you may have carpal tunnel syndrome.

2. CUBITAL TUNNEL SYNDROME

INTRODUCTION

Cubital tunnel syndrome is a condition caused by increased pressure on or stretch of the ulnar nerve that passes behind the elbow (also causes the “funny bone” sensation). People commonly notice it when sleeping or when holding the phone—any activity where the elbow is in a bent position for long period of time.

ANATOMY

There is a bump of bone on the inner portion of the elbow (medial epicondyle) under which the ulnar nerve passes. At this site, the ulnar nerve lies directly next to the bone and is susceptible to pressure. When the pressure on the nerve becomes great enough to disturb the way the nerve works, then numbness, tingling, and pain may be felt in the elbow, forearm, hand, and/or fingers.

CAUSES

Pressure on the ulnar nerve at the elbow can develop in several ways:

1. Compressive force: arm leaned against a table on the inner part of the elbow, thicker connective tissue over the nerve, or muscle structure variations over the nerve at the elbow
2. Traction or stretching: holding the elbow in a bent position for a long time, or following trauma
3. Friction: an unstable ulnar nerve at the elbow clicks back and forth over the bony bump (medial epicondyle) with repetitive elbow flexion

 

SYMPTOMS

Cubital tunnel syndrome symptoms usually include pain, numbness, and/or tingling. The numbness or tingling most often occurs in the ring and little fingers. The symptoms are usually felt when there is pressure on the nerve, such as sitting with the elbow on an arm rest, or with repetitive elbow bending and straightening. Some patients may notice weakness while pinching, occasional clumsiness, and/or a tendency to drop things. In severe cases, sensation may be lost and the muscles in the hand may lose bulk and strength

DIAGNOSIS

Your physician will assess the pattern and distribution of your symptoms, and examine for muscle weakness, irritability of the nerve to tapping and/or bending of the elbow, and changes in sensation. A test called electromyography (EMG) and/or nerve conduction study (NCS) may be done to confirm the diagnosis of cubital tunnel syndrome and stage its severity. This test also checks for other possible nerve problems, such as a pinched nerve in the neck, which may cause similar symptoms. X-rays of the elbow with cubital tunnel view is required to assess the bones and joint

TREATMENT

Symptoms may sometimes be relieved without surgery, particularly if the EMG/NCS testing shows that the pressure on the nerve is minimal.

Changing the patterns of elbow use may significantly reduce the pressure on the nerve. Avoiding putting your elbow on hard surfaces may help, or wearing an elbow pad over the ulnar nerve may help. Keeping the elbow straight at night with a splint also may help. A session with a therapist to learn ways to avoid pressure on the nerve may be needed. When symptoms are severe or do not improve, surgery may be needed to relieve the pressure on the nerve.

Several operations are used, including simple opening of the roof of the tunnel (decompression), moving the nerve into a new location at the front of the elbow (transposition) and widening the tunnel by removing some of its bony floor (medial epicondylectomy). Your surgeon can advise on the technique most appropriate to your problem.

LEGEND: Exploration of ulnar nerve, decompression and neurolysis

LEGEND: Transposition of the decompressed ulnar nerve above the “funny bone” to minimize tension to the nerve

LEGEND: Coverage of the of the decompressed ulnar nerve above the “funny bone” with soft tissue for extra protection

REHABILITATION

Following surgery, the recovery will depend on the type of surgery that was performed. Kinesiotherapy may be necessary. The numbness and tingling may improve quickly or slowly, and it may take several months for the strength in the hand and wrist to improve. Cubital tunnel symptoms may not completely resolve after surgery, especially in severe cases.

3. THORACIC OUTLET SYNDROME

INTRODUCTION

Thoracic outlet syndrome is a condition whereby symptoms are produced from compression of nerves or blood vessels, or both, because of an inadequate passageway through an area (thoracic outlet) between the base of the neck and the armpit.

 CAUSES

An inadequate passageway for nerves and blood vessels as they pass through an area (thoracic outlet) between the base of the neck and the armpit causes thoracic outlet syndrome. This can be constant or intermittent. Thoracic outlet syndrome can be caused by:

1. weight lifting
2. obesity
3. tumors in the chest
4. extra ribs extending from the seventh cervical vertebra at the base of the neck
5. occupations that involve heavy usage of the upper extremities against resistance, including jack-hammer operators and dental hygienists
6. poor posture

 

SYMPTOMS

1. neck, shoulder, and arm pain
2. numbness in the fingers
3. impaired circulation and flushed sensations to the extremities (causing discoloration)
4. the involved upper extremity can feel weak
5. Often symptoms are reproduced or worsened when the arm is positioned above the shoulder or extended.
6. Patients can have a wide spectrum of symptoms from mild and intermittent to severe and constant. Pains can extend to the fingers and hands, causing weakness.

DIAGNOSTIC TESTS

The diagnosis of thoracic outlet syndrome is suggested by the symptoms and supported by findings of the doctor during the examination. Certain maneuvers of the arm and neck can produce symptoms and blood vessel “pinching,” causing a loss of pulse. This includes the Adson’s maneuver, whereby the examiner moves the shoulder joint into positions that can cause pinching of both the nerves and artery to the tested arm.

Further supportive testing can include:

1. electrical tests, such as electromyogram (EMG) and somatosensory evoked responses (although these may not be positive in all patients).
2. angiogram X-ray tests that demonstrate the pinched area of the blood vessel involved.
3. MRI , Chest X-ray

 

TREATMENT

1. Physical Therapy

Treatment of the thoracic outlet syndrome can usually be successful with conservative measures. Treatments include a variety of exercises that effectively stretch open the tissues of the thoracic outlet.

1. Medicines

A health-care professional might prescribe medications to help improve the symptoms, such as anti-inflammatory medications (ibuprofen, naproxen) and muscle relaxants.

1. Surgical Treatment

Some patients with severe, resistant symptoms can require surgery to open the thoracic outlet. These procedures include interruption of the scalene muscle (scalenotomy) and removal (resection) of the first rib in order to spare injury to the affected nerve and blood vessels from ongoing compression. Thoracic outlet syndrome that affects the vascular system (veins and arteries) is more likely to require surgery to resolve the symptoms.

AFTER THE SURGERY

Patients should avoid prolonged positions with their arms held out or overhead. For example, it is best to avoid sleeping with the arm extended up behind the head. It is also helpful to have rest periods at work to minimize fatigue. Weight reduction can be helpful for obese patients. Patients should avoid sleeping on their stomach with their arms above the head. They should also not repetitively lift heavy objects. 

WHO TREATS THE CONDITION

• Plastic Surgeons – Microosurgeons
• Thoracic Surgeons
• Neurologists
• Physical-medicine doctors.

INTRODUCTION

Tumors of almost any type can form along peripheral nerves. Most commonly, these are entirely benign tumors that do not metastasize to other parts of the body. Generally slow growing, these tumors can form within the substance of the nerve itself, and cause the nerve to gradually expand. Untreated nerve tumors begin to compress the adjacent nerve fibers, causing nerve dysfunction. Patients may experience pain, numbness, and/or weakness in the distribution of the affected peripheral nerve.

In almost all cases surgical excision to obtain a diagnosis and to cure the tumor is indicated. It is important to have an experienced peripheral nerve surgeon perform this operation, as most peripheral nerve tumors form within the nerve itself, and thus are surrounded and intertwined with functional nerve fibers that must at all costs be preserved during removal of the tumor.

TYPES OF NERVES TUMORS

There are three major categories of nerve tumors. They are:

1. Neurofibroma. Most commonly found within the genetic disorder of neurofibromatosis.
2. Schwannoma. These are nerve sheath tumors and can occur in isolation. Less commonly these tumors can occur in patients suffering from neurofibromatosis or schwannomatosis.
3. Malignant Peripheral Nerve Sheath Tumor. These very aggressive tumors are cancerous nerve sheath tumors and should be managed by a multi-disciplinary team. They can occur in association with neurofibromatosis Type 1.

 

SYMPTOMS

The symptoms caused by these tumors can vary. Some patients are asymptomatic, meaning they have no symptoms. Possible symptoms may include:

• Pain
• A mass, or thickening in the muscle fibers
• Numbness, burning, or “pins and needles”
• Weakness in the affected muscles
• Dizziness/loss of balance

 

DIAGNOSIS

Your doctor will examine you carefully and may order several imaging studies including:

• MRI Neurography
• CT
• EMG(electrodiagnostic study to examine electrical pathways in the nerves)

 

TREATMENT

Nonsurgical treatment options

For many patients non-operative care is the most appropriate treatment. The tumors are often slow growing and can remain asymptomatic for a long period of time. Following patients with serial examinations and imaging studies can provide a guide to if, and or when, a patient may require surgery. 

Surgical treatment options

There are several factors in deciding to surgically remove these tumors. Some, like schwannomas are slow-growing and can be watched using imaging studies over time. Others, like malignant nerve sheath tumors need to be removed immediately, as they are very aggressive.

LEGEND: Excision of Schwanomma from the brachial plexus

 

1. NEUROFIBROMA

 

These are benign, slow-growing nerve tumors, similar to schwannomas. They commonly occur in patients who suffer from neurofibromatosis, a genetic disorder resulting in multiple tumors throughout the body. They can occasionally occur in patients without this genetic abnormality.

Cutaneous neurofibromas grow along small branches of nerves under the skin of patients with neurofibromatosis. They may present as painful lumps under the skin. They are not associated with large nerves, and are easily removed.

LEGEND: Intraoperative, magnified view of a cutaneous neurofibroma with associated small cutaneous sensory nerve just prior to excision.

Larger neurofibromas tend to grow within large, important nerves. Unlike schwannomas, however, they are more intimately intertwined with the nerve fibers, making removal more difficult. Nevertheless, with meticulous surgical technique, these difficult tumors may be safely removed, causing few if any deficits.

LEGEND: Intraoperative view of a medium-sized median nerve neurofibroma.

LEGEND: Intraoperative view of the same patient seen in the previous photo, following removal of the tumor. The nerve is both anatomically and functionally intact. The patient awakened without any deficit.

When a neurofibroma involves a particularly long segment of nerve or nerves, it is called a plexiform neurofibroma. These are generally impossible to remove without removing the entire nerve, necessarily causing a major neurological deficit. Therefore, these variants are not usually subjected to surgery.

A small percentage of plexiform neurofibromas change from benign to malignant, and can spread to other parts of the body. These cancers are almost uniformly lethal without aggressive treatment. If a plexiform neurofibroma becomes exceptionally painful and/or begins to rapidly expand, this suggests it has become cancerous, and it must be definitively treated.

1. SCHWANNOMA

 

These are benign, slow growing nerve tumors that can occur anywhere in the peripheral nervous system. Generally patients discover a painless, firm lump. Though these tumors form within nerves and are surrounded by normal nerve fibers, they are easily separated from the nerve and removed by an experienced peripheral nerve surgeon.

These are the most common nerve tumors operated on by non-peripheral nerve surgeons. Tragically, these patients undergo a “lymph node biopsy,” only to awaken with permanent weakness, numbness, and pain, only to find to the horror of patient and surgeon that they indeed had a nerve tumor instead.

CASE 1

LEGEND: MRI demonstrating a schwannoma involving the right saphenous nerve (T) of a 32 year old male.

LEGEND: Intraoperative photograph showing the saphenous nerve schwannoma seen on MRI in Figure 1, surrounded by normal nerve fascicles. Careless resection of these tumors by those not trained in peripheral nerve surgery can damage these vital neural elements, resulting in severe neurological deficits and chronic pain.

LEGEND: Intraoperative photograph showing an intact saphenous nerve (N) following complete removal of the schwannoma.

LEGEND: Intraoperative photograph of the schwannoma following excision from the parent nerve.

CASE 2

LEGEND: Intraoperative photograph showing a posterior tibial nerve schwannoma.

LEGEND: Intraoperative photograph of the schwannoma following excision from the arent nerve.

1. NEUROFIBROSARCOMA

INTRODUCTION

Also called neurofibrosarcoma, nerve sheath tumors are malignant (cancerous) tumors that grow in the cells surrounding peripheral nerves. A common cause of nerve sheath tumors is neurofibromatosis type 1 (NF1).

These tumors include cancers that arise within peripheral nerves as well as plexiform neurofibromas that start out as benign tumors but then subsequently become cancerous. Nerve cancers are generally rapidly-growing and painful.

Neurofibrosarcoma accounts for 5 to 7 percent of all soft tissue sarcoma cases.

CAUSES

The exact cause of neurofibrosarcoma is not entirely understood, however, studies have indicated that genetics may play a role in the formation of all soft tissue sarcomas.

Limited studies have shown a possible link between soft tissue sarcomas and other types of cancer.

If you have Neurofibromatosis (which involves alterations in the NF1 gene), you may be at an increased risk for developing neurofibrosarcoma.  These tumors occur in up to 16 percent of people with neurofibromatosis. Neurofibromatosis, also known as Recklinghausen’s disease, is a genetic (inherited) condition in which benign fibrous tumors develop inside nerve tissue.

 SYMPTOMS

Since neurofibrosarcoma affects tissue that is elastic and easily moved around instead of hard and stationary, a tumor may exist for a long time before being discovered, growing large and pushing aside surrounding tissue.

While each patient may experience symptoms differently, and symptoms can vary greatly depending on size, location, and spread of tumor, some of the most common include:

• a painless swelling or lump, usually in the arms or legs
• pain or soreness
• limping or other difficulty using the arms, legs, feet or hands

LEGEND: Neurofibrosarcoma of the mandible

TREATMENT

It is important to operate on these lesions in a timely fashion before they have a chance to spread to other parts of the body. Once they metastasize, they are typically rapidly lethal.

Goals of surgery for malignant nerve tumors are to obtain a tissue diagnosis, but not to necessarily remove the tumor. Although this seems counterintuitive to many people, attempting to remove the malignant tumor in the same fashion as removing a benign nerve tumor is essentially impossible.

That is because cancer cells, invisible to the surgeons eyes, have already spread along the course of the affected nerve, even before the surgery is performed. Therefore just removing the tumor that the surgeon can see and feel will always leave some of the cancer behind.

These tumors should be removed to prevent local recurrence of malignancy and to halt growth and possible spreading of the cancer (metastasis) throughout the body.

Once these tumors are diagnosed by an open biopsy, a cure generally requires either limb amputation or at least removal of the entire adjacent nerve and all of the surrounding tissues, including muscle, blood vessels, and fat. Sometimes radiation and/or chemotherapy can be helpful, depending upon the exact type of tumor it turns out to be.

PROGNOSIS

The prognosis greatly depends on:

• the extent of the disease
• the size and location of the tumor
• a presence of absence of metastasis
• the tumor’s response to therapy
• the age and overall health

 

1. NEUROFIBROMATOSIS – VON RECKLINGHAUSENS DISEASE

INTRODUCTION

Neurofibromatosis is an incurable, genetic disorder of the nervous system. It mainly affects the development of nerve cell tissues. Tumors known as neurofibromas develop on the nerves, and these can lead to other problems. The tumors may be harmless, or they may compress the nerves and other tissues, leading to serious damage.

CAUSES

Neurofibromatosis (Nf) is the most common genetic neurological disorder that is caused by a single gene. The mutation in the gene means that the nerve tissue is not properly controlled.

There are three types: Nf1, Nf2 and Schwannomatosis. They are not related.

Nf1 and Nf2 are caused by a genetic mutation. The mutation is often inherited, but between 30 and 50 % result from an altered gene, which most likely happened spontaneously in the egg or sperm just before fertilization. If a person has the altered gene, it can be passed on to future generations. If one parent carries a faulty gene, any offspring have a 50 % chance of inheriting it.

TYPES OF NEUROFIBROMATOSIS

The three types of neurofibromatosis are Nf1, Nf2, and schwannomatosis.

Type 1 neurofibromatosis (Nf1)

Also known as von Recklinghausen’s disease, von Recklinghausen NF, or peripheral neurofibromatosis, NF1 is the most common type of neurofibromatosis. It is transmitted on chromosome 17. It mostly results from a mutation, rather than a deletion, of the Nf1 gene. It is thought to affect 1 in every 3,000 people. Shortly after birth, birthmarks may appear in different parts of the body. During late childhood, lesions, or tumors, may appear on or under the skin, numbering from a few to thousands. Occasionally, the tumors become cancerous. Nf1 can be barely noticeable, it can be unsightly, or it can lead to potentially serious complications. Around 60 percent of cases are minor.

Type 2 neurofibromatosis (Nf2)

Bilateral neurofibromatosis, or Nf2, normally stems from a mutation, rather than a deletion, of the Nf2 gene. It is transmitted on chromosome 22. Tumors form in the nervous system, generally within the skull. These are known as intracranial tumors. Intraspinal tumors may develop in the spinal canal. Acoustic neuromas are common, forming on the vestibulocochlear nerve, or the eighth cranial nerve. The vestibulocochlear nerve is responsible for hearing, and it affects the sense of balance and body position, so a loss of hearing and balance may occur. Symptoms tend to arise during the late teens and early 20s. Tumors may become cancerous.

Schwannomatosis

Schwannomatosis is a rare form of neurofibromatosis, genetically distinct from Nf1 and Nf2. It affects fewer than 1 in 40,000 people. Schwannomas, or tumors in the tissue around a nerve, can develop anywhere in the body, except for the vestibulocochlear nerve, the nerve that goes to the ear. It does not involve the neurofibromas characteristic of Nf1 and Nf2. The tumors can cause severe pain, numbness, tingling, and weakness in the toes and fingers.

SYMPTOMS AND SIGNS OF NEUROFIBROMATOSIS

LEGEND: Café au lait spots are a common sign of Nf1.Neurofibromatosis can affect all neural crest cells, including Schwann cells, melanocytes, and endoneurial fibroblasts. It may affect the bones, causing severe pain.

The disorder can spread throughout the whole body, leading to tumors and unusual skin pigmentation. It can manifest as bumps under the skin, colored spots, bone problems, pressure on spinal nerve roots, and other neurological problems. Learning disabilities, behavioral problems, and vision or hearing loss may arise. The signs and symptoms of neurofibromatosis depend on the type.

• Symptoms of Neurofibromatosis 1

Some patients with Nf1 have only a skin condition and no other related medical problems. Signs and symptoms generally appear during early childhood. They are not usually harmful to health.

LEGEND: Skin growths may occur with both Nf1 and Nf2.

Birthmarks and freckles are common. Coffee-colored marks appear on the skin, usually at birth. If more than six marks appear by the age of 5 years, this may indicate Nf1. The number of spots can increase, they may get bigger, and they may darken with time. Freckles may appear in an unusual location, such as the groin, under the breasts, or in the armpits.

Neurofibromas are tumors, generally non-cancerous, that grow on the nerves of the skin, and sometimes on nerves deeper inside the body. They look like lumps under the skin. In time, more may develop, and they may get bigger. Neurofibromas may be soft, or firm and round.

LEGEND: Example of cutaneous neurofibroma

Lisch nodules are very small brown spots that may appear in the iris of the eye. People with Nf1 also have a higher chance of hypertension, or high blood pressure.

LEGEND: Example of patient with NF1 and multiple head and neck cutaneous neurofibromas

• Symptoms of Neurofibromatosis 2

Nf2 is a more serious condition in which tumors grow on nerves deep inside the body. An acoustic neuroma is a common kind of brain tumor that develops on the nerve that goes from the brain to the inner ear. Symptoms may include:

• Facial numbness, weakness and sometimes paralysis
• Gradual, or more rarely sudden, hearing loss
• Loss of balance, dizziness, and vertigo
• Tinnitus, or ringing in the affected ear

Sometimes, tumors develop on the skin, brain, and spinal cord, with potentially serious consequences. Some tumors develop rapidly, but most grow slowly, and the effect may not be noticeable for several years. Regular monitoring can enable tumors to be removed before complications arise. Spots of light brown pigmentation may occur, but they will less common and fewer in number than in patients with Nf1. Cataracts may occur. If a child develops a cataract, this may be a sign of Nf2. These are easily removed, and not generally problematic, if treated.

DIAGNOSIS

• Diagnosis of Nf1

Nf1 is normally diagnosed during childhood. A diagnosis is confirmed when a patient has at least two of the following:

1. Family history of Nf1
2. A glioma, or tumor, on the optic nerve, usually without symptoms
3. Lesions in the bone
4. At least six café-au-lait spots measuring more than 5 millimeters across in children or 15 millimeters in adolescents and adults
5. At least two Lisch nodules, or small brown spots in the iris
6. Freckling in the armpits, under the breast, or in the groin area
7. Two or more neurofibromas, or one “plexiform”

 

• Diagnosis of Nf2

Symptoms of Nf2 normally appear around puberty or in adulthood. The most common age of onset tends to be from 18 to 24 years.

A diagnosis of Nf2 is made when there is:

• Acoustic neuroma in one ear, plus two or more typical symptoms, such as cataracts, brain tumors, and a family history of the condition
• Acoustic neuroma in both ears
• Acoustic neuroma plus brain or spinal tumors, detected by an MRI or CT scan
• A faulty gene, identified through a blood test

TREATMENT

Neurofibromatosis can’t be cured, but treatments are available for your signs and symptoms. Generally, the sooner the patient is under the care of a doctor trained in treating neurofibromatosis, the better the outcome. Although there is no cure for neurofibromatosis, symptoms can be treated.

Many patients with Nf1 have only mild symptoms and can live a normal healthy life. However, they will need monitoring to prevent complications from developing. Neurofibromas are not generally painful, but if the location on the body means that they graze or catch on clothing, they can be removed. This reduces the risk of itching, infection, numbness, and general discomfort. However, they may grow back.

1. Monitoring

If a person has Nf1, the doctor is likely to recommend yearly age-appropriate checkups to

1. Assess the skin for new neurofibromas or changes in existing ones
2. Check for signs of high blood pressure
3. Evaluate the child’s growth and development — including height, weight and head circumference — according to growth charts available for children with NF1
4. Check for signs of early puberty
5. Evaluate the child for any skeletal changes and abnormalities
6. Assess the child’s learning development and progress in school. Obtain a complete eye examination. If tumors growing on the optic nerve affect the patient’s eyesight, they can be surgically removed.
7. Hypertension can be treated with medications and lifestyle changes. Regular checks are advised.

Contact your doctor promptly if you notice any changes in signs or symptoms between visits. It’s important to rule out the possibility of a cancerous tumor and to obtain appropriate treatment at an early stage.

2. Surgery and other procedures

Your doctor might recommend surgery or other procedures to treat severe symptoms or complications of neurofibromatosis.

1. Surgery to remove tumors. Symptoms can be relieved by removing all or part of tumors that are compressing nearby tissue or damaging organs. If you have NF2 and have experienced hearing loss, brainstem compression or tumor growth, your doctor might recommend surgery to remove acoustic neuromas that are causing your problems. Complete removal of schwannomas in people with schwannomatosis can ease pain substantially.
2. Stereotactic radiosurgery. This procedure delivers radiation precisely to your tumor and doesn’t require an incision. Stereotactic radiosurgery might be an option to remove acoustic neuromas if you have NF2. Stereotactic radiosurgery can help preserve your hearing.
3. Auditory brainstem implants and cochlear implants. These devices might help improve your hearing if you have NF2 and hearing loss.
4. Surgery to correct Scoliosis (curvature of the spine)

 

3. Cancer treatment

Malignant tumors and other cancers associated with neurofibromatosis are treated with standard cancer therapies, such as surgery, chemotherapy and radiation therapy. Early diagnosis and treatment are the most important factors resulting in good outcome.

4. Pain medications

Managing pain is an important part of treatment for schwannomatosis. Your doctor might recommend:

1. Gabapentin (Neurotin) or pregabalin (Lyrica) for nerve pain
2. Tricyclic antidepressants such as amitriptyline
3. Serotonin and norepinephrine reuptake inhibitors such as duloxetine (Cymbalta)
4. Epilepsy medications such as topiramate (Topamax) or carbamazepine (Carbatrol, Tegretol)

PROGNOSIS

NF progresses differently for each person. It is hard to predict the course of the disease. A person’s prognosis depends on the types and locations of tumors that he or she develops.

Neurofibromatosis type 1: Most people will have mild to moderate symptoms that worsen over time. Patients can live normal and productive lives. In some cases, however, NF1 can affect quality of life.

Neurofibromatosis type 2: These tumors generally grow slowly. Balance and hearing may become worse over time. Sometimes tumors grow next to vital structures, such as the brain. If they are not treated, this situation can be serious.

Schwannomatosis: Some patients have mild pain, but most have significant pain. Pain can be managed with treatment.