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Craniofacial Center

Each year in the United States thousands of infants are born with cleft lip, cleft palate and other complex facial deformities. Trauma and facial tumors also result in significant facial disfigurement in children. Without the right treatment, babies with birth anomalies or young children whose faces are damaged by disease or accident are likely to grow up feeling rejected and isolated by the indignities they often suffer because of their appearance. Fortunately, many of these children can achieve a happy life. Treatment for disfiguration among children has been increasingly successful in recent decades and is still being advanced. NewYork-Presbyterian/Columbia holds a leadership position in helping impaired children and their families to achieve a better future though the expertise of its Craniofacial Center staff.

Many specialists are needed to provide the expert consultation and skillful care required to diminish the problems of craniofacial disfiguration. Timing of such care is critical to its successful outcome but, unfortunately, it is usually the child's parents or pediatrician who are left to struggle with coordinating steps in managing treatment. The NewYork-Presbyterian/Columbia Craniofacial Center solves this problem by providing comprehensive specialty consultation and care for children with deformities. Center specialists meet and work together regularly to review the complex treatment plans that make an active and productive life possible for the children whose future lies in their hands. Management plans are developed with active participation from patients' families to help them understand the options available for their child and to impart confidence in their choice of treatment.

Strengthened by its multidisciplinary resources, the Craniofacial Center is fully prepared to assist parents and caregivers in every way that will help assure positive change in the lives of children afflicted by facial problems. After a patient is seen at the Center, a detailed report is sent to the family that includes the recommendations made by each of the team members and the specific phone numbers to call to arrange the suggested follow-up appointments The multidisciplinary Craniofacial Center team is composed of audiologists, geneticists, neurosurgeons, ophthalmologists, oral and maxillofacial surgeons, orthodontists, otolaryngologists, pediatric dentists, pediatricians, plastic surgeons, social workers, and speech and language pathologists. The team offers unqualified excellence in treating the following wide selection of troubling facial conditions:

• Cleft lip and cleft palate
• Facial clefting syndromes including Treacher Collins syndrome
• Craniosynostosis syndromes including Crouzons and Aperts syndromes
• Craniofacial trauma
• Facial and jaw tumors
• Dentofacial deformities and malocclusion

Audiology

Hearing difficulties and middle ear dysfunction often occur in infants and children with craniofacial anomalies. The auditory system and pathways can be assessed at any age from neonatal/infancy though adulthood. Audiologic services available include otoacoustic emissions (OAEs), auditory brainstem response (ABR) testing, emmittance audiometry, behavioral audiologic evaluations including visual reinforcement audiometry and hearing aid evaluations/fittings. Testing and treatment is conducted by the Craniofacial Center's specially trained audiologists.

Genetics

The Division of Genetics provides prenatal, pediatric, and adult diagnostic testing of birth defects and/or genetic conditions. Genetic counseling is an integral component of the program, including a discussion of genetic risks for future offspring. The Craniofacial Center is staffed by a clinical geneticist, genetic counselors and supporting staff. Cytogenetic and DNA diagnostic testing are done at the Center, and laboratories in other areas of the country are often used as well.

Oral Healthcare Services

• Orthodontics and Dentofacial Orthopedics
• Pediatric Dentistry
• Maxillofacial Prosthodontics

Infants and children with craniofacial anomalies require specialized dental services. The dental care must be an integrated part of the overall rehabilitative process. Services for these patients include not only primary care, but also routine maintenance throughout life. The necessary procedures focus on monitoring craniofacial growth and development, correcting jaw relationships and dental arches for surgical procedures including lip and palate repair, bone grafting and orthognathic surgery. Prosthetic appliances to assist in feeding or speech in the cleft palate patient and to replace or restore oral and facial structures can be provided.

Pediatrics

Pediatricians supervise the children's nutrition, growth, development, and other general medical care issues not covered by other team members. They also help coordinate the care provided by our team with the patient's primary care physician.

Surgical Services

• Neurosurgery
• Ophthalmic and Oculoplastic Surgery
• Oral and Maxillofacial Surgery
• Otolaryngology/Head and Neck Surgery
• Plastic and Reconstructive Surgery

Timely surgical management of patients with craniofacial anomalies including cleft lip and palate, facial clefts, facial tumors, craniosynostoses and dentofacial deformities can minimize the impact of these conditions on cranial and facial growth, development, final function and appearance of the patient. Team management also provides care for conditions including congenital deformities of the eyelids, eye muscles and tear ducts, swallowing, hearing and speech difficulties due to cleft palate and velopharyngeal insufficiency, and jaw and occlusal discrepancies. Trained in centers around the world, the team's surgeons bring in-depth expertise and experience to the care of their patients.

Social Services

The Craniofacial Center's social workers facilitate medical, dental and surgical care of children, and also help with entry into long-term social service programs.

Speech and Language Pathology

Children with craniofacial abnormalities may present with articulation disorders, resonance disorders, swallowing disorders and secondary language disorders. Testing can be undertaken within the first year. As with hearing impairments, early diagnosis and intervention are critical. The Center's speech and language pathologists are available to evaluate and treat the children.

The NewYork-Presbyterian/Columbia Craniofacial Center, located in Morgan Stanley Children's Hospital of NewYork-Presbyterian, has access to state-of-the-art medical technology and laboratory resources for the diagnosis and treatment of its young patients. The Craniofacial Center's association with Morgan Stanley Children's Hospital offers patients and their families the advantage of access to expertise in every specialty.

Accomplishments and Research

Members of the team are involved in numerous research activities, including developing new treatments for speech disorders, new techniques to stimulate and promote cranial bone formation, new treatments for facial pain and headaches, and an evaluation of the role of complementary medicine in the treatment of pain.

In addition to publishing many articles in professional journals and writing numerous book chapters, several of our members have authored books, including one on soothing pain in children and another for mothers on providing medical care.

Team members have also organized and participated in a number of non-profit surgical missions to other countries, including Colombia, Guatemala, and China, to treat children with cleft lips, cleft palates, and other surgical problems. On a typical mission, 50 to 100 surgeries are usually performed.

Contact

Craniofacial Pain Syndromes

The most common craniofacial pain syndrome is trigeminal neuralgia ("TMJ," jaw joint pain or tic douloureux). Trigeminal neuralgia is a facial pain syndrome that usually develops in individuals over 50 years old. Its incidence is 4/100,000, and it is the most common facial pain sydrome in this age group.

Other neuropathic syndromes affect the trigeminal nerve and warrant different treatments with varied rates of success. Rarely, neuralgias of other cranial nerves mimic trigeminal neuralgia. Finally, it is essential to distinguish atypical facial pains from these neuropathic syndromes to avoid unsuccessful therapies.

Causes

Causes are uncertain, but there is considerable evidence that vascular compression of a nerve root is the cause. This may be due to branches of the superior cerebellar artery, basilar artery, or local veins compressing the trigeminal nerve root. In younger patients, trigeminal neuralgia can be caused by tumors in the region of cranial nerve V or (more commonly) by multiple sclerosis. These entities must therefore be ruled out.

Diagnosis

Although the diagnosis remains based exclusively on history and symptomatology, modern diagnostic techniques, particularly high-resolution magnetic resonance imaging, provides valuable new insight into the pathophysiology of these cases with additional implications for therapeutic strategies. Imaging studies, CT or MRI, with and without contrast enhancement, are normally performed on every patient with trigeminal neuralgia. Often, elongated and tortuous vessels can be seen and tumors of the region need to be excluded as a cause for the syndrome.

Symptoms

The character of classical trigeminal neuralgia is "electric-like shocks" lasting a few seconds but of a debilitating, intense nature. They are usually on one side of the face, most often in the area of the upper cheek down to the level of the jaw, the maxillary (V2) and mandibular (V3) branches of the trigeminal nerve. The ophthalmic division (V1), transmitting sensation from the skin of the forehead and the eye, is less commonly involved. The trigeminal nerve is also known as cranial nerve V. It transmits sensation from the face, eyes and teeth and controls the muscles of mastication (chewing). Painful attacks may occur spontaneously but are more often associated with a specific stimulus in a "trigger area." Common trigger points are the eyebrow for ophthalmic division pain, the upper lip for maxillary division involvement, and the lower molar teeth for mandibular division pain. Sensory stimuli by touch, cold, wind, talking or chewing can precipate the attacks. Pain-free intervals last for minutes to weeks, but long-term spontaneous remission is rare. The attacks cease during sleep but often occur upon arising in the morning. This constellation of symptoms provides the diagnosis.

Treatment

Pharmacological

The primary treatment of trigeminal neuralgia is pharmacological. Most patients obtain relief, at least initially, within 30 minutes of administration of carbamazepine (Tegretol). This drug does have side effects—mostly bone marrow depression and liver damage—and patients should be monitored by their physician. Some individuals may also respond to phenytoin (Dilantin).

Surgical

After pharmacologic failure, surgical intervention is necessary. Surgical options include blocking the trigger point with local blocks, neurectomy—block of the nerve branches, percutaneous rhizotomy of the trigeminal ganglion (destruction of the site of sensory nerve cell bodies just inside the skull and dura), and microvascular decompression of the nerve root zone. Decisions on which treatment is best for a given patient must be based on the nature of the pain, the health of the patient, imaging findings and consultation with the neurosurgeon.