Wake Forest University Baptist Medical Center

Department of Plastic and Reconstructive Surgery

Medical Center Blvd. | Winston-Salem, NC 27157-1075 | Phone 336-716-4171 | Fax 336-716-6642

The North Carolina Center For Cleft & Craniofacial Deformities

Each year in the United States, one in every 2,000 newborns is born with a craniofacial disorder. Such a diagnosis can bring many uncertainties for the patient and their families. At Wake Forest University Baptist Medical Center, our goal is to dispel the doubt and uncertainties associated with craniofacial disorders, and to offer a bright outlook to those we evaluate and treat.

Our craniofacial team has more than 25 years of experience. We have treated more than 2500 children with a wide array of craniofacial deformities. The team approach is vital for efficient treatment to minimize disruptions to both familial and school routines.

If you have any questions or concerns about your child's condition, we are happy to speak with you anytime. You can reach the Center for Cleft and Craniofacial Disorders by calling 336-716-4579. We feel the patient and their family are the most important part of our team.

Commonly Treated Craniofacial Conditions

Isolated Cleft Lip and Palate

Cleft lip is the fourth most frequent birth defect, and the most common congenital facial defect. One of every 700 newborns is affected by cleft lip and/or cleft palate. Cleft lip can also be associated with a cleft palate.

A cleft lip is a separation of the two sides of the lip. The separation often includes the bones of the upper jaw and/or upper gum. A cleft palate is an opening in the roof of the mouth in which the two sides of the palate did not fuse, or join together, as the unborn baby was developing. Cleft lip and cleft palate can occur on one side (unilateral cleft lip and/or palate), or on both sides (bilateral cleft lip and/or palate). Because the lip and the palate develop separately, it is possible for the child to have a cleft lip, a cleft palate, or both cleft lip and cleft palate.

Cleft lip and cleft palate are congenital defects, or birth defects, which occur very early in pregnancy. The majority of clefts appear to be due to a combination of genetics and environmental factors. The risks of recurrence of a cleft condition are dependent upon many factors, including the number of affected persons in the family, the closeness of affected relatives, the race and sex of all affected persons, and the severity of the clefts.

A child born with a cleft frequently requires several different types of services, e.g., surgery, dental/orthodontic care, and speech therapy, all of which need to be provided in a coordinated manner over a period of years. This coordinated care is provided by interdisciplinary cleft palate/craniofacial teams comprised of professionals from a variety of health care disciplines who work together on the child's total rehabilitation.

Cleft palate can be isolated or a part of several craniofacial syndromes. The occurrence is typically multifactorial and like the cleft lip, is surgically repaired within the first year of life.

To view progressive stages of an incomplete cleft lip click here.*
To view a complete cleft lip click here. *
To view before and after images of a unilateral cleft lip click here.*
*The wounds shown here are intended for medical purposes and are graphic in nature.

Syndromic Cleft Lip or Palate

Van der Woude Syndrome: This syndrome commonly exhibits characteristics that include lower lip pits, hearing loss, missing teeth, cleft lip with or without cleft palate, and a cleft uvula. Van der Woude syndrome is an autosomal dominant disorder, which means 50% of any future children of these parents, or the child, may have this syndrome. This syndrome is commonly treated by repairing the cleft lip and/or palate by one year of age, and excising the lip pits.

Velocardiofacial Syndrome: This syndrome commonly exhibits characteristics that include short stature, hearing loss, soft palate cleft, hypoplastic midface, long face, retruded mandible with a chin deficiency, hyperextensible hands and cardiac defects. Velocardiofacial syndrome is an autosomal dominant disorder. Treatment for this syndrome requires that each abnormality receive individualized treatment. Traditionally, the best speech outcome is obtained by repairing the cleft palate within the first year of life.

Pierre Robin Syndrome: This syndrome commonly exhibits characteristics that include airway problems secondary to a small lower jaw, and a tongue that frequently falls back blocking the airway and a soft palate cleft. Pierre Robin syndrome occurs in otherwise healthy children, but may occur in association with other craniofacial syndromes. This syndrome’s treatment is initially focused on airway protection and may require a lip adhesion, tracheostomy, or mandibular distraction. Once the airway is secured, the palate can be repaired by one year of age to maximize speech outcome.

Craniofacial Clefts

There are more than 14 facial clefting patterns, which were first described by Dr. Paul Tessier in Paris, France. Their occurrence is usually sporadic. The treatment involves the correction of both soft tissue and underlying bony deficiencies. The soft tissue closure is completed early in infancy while the bony reconstruction is performed once the child is a little older.

Craniosynostosis

* To view the dynamic spring mediated cranioplasty procedure click here .
* To view before and after pictures of a child treated with spring mediated cranioplasty click here .
* To view the cranial vault reconstruction procedure click here.
*The wounds shown here are intended for medical purposes and are graphic in nature.

Description of Procedure:

Craniosynostosis is the premature closure of one or more cranial sutures that causes cranial asymmetry. This occurs in about one in 3,000 births as an isolated problem, and is considered a sporadic occurrence. If craniosynostosis is part of a syndrome, it is usually an autosomal dominant inheritance pattern. Craniosynostosis can be a developmental problem if not treated in a timely fashion. Treatment involves cranial reconstructive surgery within the first year of life.

Click here for plagiocephaly diagrams and equipment.

Positional Plagiocephaly

In 1992, the American Academy of Pediatrics released their recommendations for placing infants to sleep in the supine position to reduce the risk of SIDS. Since the recommendation was made, the incident rate of misshapen heads from compressive forces has increased dramatically. Currently one in every 70 infants is affected by some degree. By using a soft-shell, passive-molding helmet for between 3 to 6 months, the problem can be corrected. It is very important to diagnose this early, before the soft spot on the head closes, for the helmet therapy to be effective.

Craniofacial Syndromes

Apert Syndrome: This syndrome commonly exhibits characteristics including a high and full looking forehead, a flattened occiput, coronal craniosynostosis, shallow orbits, widely spaced eyes, visual problems, small nose and flattened midface. Syndactyly (webbed fingers) are also common. Apert’s Syndrome follows an autosomal dominant inheritance pattern. The treatment consists of staged craniofacial reconstruction, midface advancement and repair of the syndactyly.

Crouzon Syndrome: This syndrome commonly exhibits characteristics including bulging eyes secondary to shallow orbits, widely spaced eyes, a prominent forehead, visual disturbances, a small midface, variable hearing loss, and premature fusion of multiple skull sutures. Crouzon’s syndrome is an autosomal dominant disorder with variable expression. Treatment for the syndrome traditionally stages surgery, with the initial cranial reconstruction typically taking place at less than one year of age.

Goldenhar Syndrome: Characteristics of this syndrome include a small midface and mandible, a lateral mouth cleft, weak facial muscles, external ear abnormalities, variable hearing loss, abnormal function of the tongue and palatal muscles and hypoplastic vertebrae. This is the bilateral manifestation of hemifacial microsomia. A genetic cause has not been identified. It is believed that most cases occur because of an unknown event that disrupts the normal development of the ear and jaw in early embryonic life. Surgery is needed for advancement of the midface and mandible. Also, the mouth cleft and ears are reconstructed within the first few years of life.

Hallerman Streiff Syndrome: This is a rare syndrome that includes dental, ear, eye and soft tissue abnormalities. Hallerman Streiff syndrome is transmitted as an autosomal recessive trait. The primary initial focus in treatment should be detecting any cardiac and visual abnormalities. As the child grows, dental issues will need to be addressed, and specific treatment will depend on the components of the syndrome that the child exhibits.

Hemifacial Microsomia: The characteristics, cause and treatments are similar to those associated with Goldenhar Syndrome, except hemifacial microsomia involves only one side of the face.

Moebius Syndrome: This syndrome exhibits characteristics that include an expressionless or mask-like facial appearance with a bilateral loss of facial nerve function. There is no known genetic link for Moebius syndrome. It is most often a sporadic event. Treatment commonly includes the use of gold weights to assist with eye closure and nerve and muscle grafting as indicated.

Pfeiffer Syndrome: This syndrome commonly exhibits characteristics including a flat face, premature cranial suture fusion, widely spaced eyes, a small nose, broad thumbs and great toes and syndactyly (webbed fingers). Pfeiffer syndrome has an autosomal dominant inheritance pattern. Treatment usually consists of cranial reconstruction, midface advancement, and repair of the finger skin bridges.

Saethre-Chotzen Syndrome: This syndrome is a primary disturbance in cranial development, which includes craniosynostosis, thickened skull, flattened forehead, shallow orbits, and widely spaced eyes. Other common characteristics include fingers and toes with webbed skin, the hand typically has a single upper palmar crease, the thumbs can be flattened and appear finger like, and the elbow can have only limited extension. Saethre-Chotzen syndrome has an autosomal dominant inheritance pattern. The treatment traditionally addresses the cranial and extremity deformities, depending on the amount of involvement.

Stickler Syndrome: This syndrome commonly exhibits characteristics including flattened facies with a depressed nose, prominent eyes, midface and mandibular hypoplasia, palatal clefts, a Pierre Robin sequence, deafness, dental abnormalities, hypotonia, hyperextensible joints, hip subluxation, and joint problems. Stickler syndrome has an autosomal dominant inheritance pattern. Typically, by age one the clefts are repaired. Midface and mandibular advancements are done later in childhood.

Treacher Collins Syndrome: The clinical characteristics for this syndrome includes a small midface, lower eyelid defects, and external ear abnormalities. Small mandibles may cause early airway problems. Treacher Collins syndrome has an inheritance pattern that is autosomal dominant. Treatment typically includes airway management, ear reconstruction, and midface and mandibular advancement.

Congenital Ear Deformities

Microtia: Ear abnormalities most often occur as a part of a syndrome, but do also occur in isolation. There is a range in the level of involvement from a simple skin tag to complete absence of the ear. Should a large portion of the ear require reconstruction, there is often two to three surgical stages performed using rib cartilage. The typical age for initiating surgery of this type is around 6 to 7 years of age.

Vascular Anomalies

Hemangioma: One in every 700 newborns is diagnosed with a hemangioma, with 10% to 12% of Caucasian children having some form of it by the age of one.
Child with hemangioma

Females are five times more likely to have a hemangioma than males, and low birth weight infants (weighing less than 2.2 lbs.) are 26 times more like to develop one. Hemangiomas are rarely full size at birth, and the actual cause is unknown. The clinical stages involve a rapid proliferation (growth), stabilization and the involution stage where they begin a slow regression. A majority of hemangiomas can simply be observed. Those requiring treatment early on are secondary to complications from the hemangioma itself such as bleeding, obstruction, and infection. Treatment may include excision, steroids, laser surgery, and interferon.

Vascular Malformations: The exact cause is unknown, but they are known to occur less frequently than hemangiomas. Vascular malformations are named for the type of blood vessel that predominates the growth. Examples of common malformations include capillary malformations (port-wine stains), venous malformations or lymphatic malformations. An accurate diagnosis is imperative, as it affects treatment. Treatment is not always required on vascular malformations, but should intervention be necessary, resection and reconstruction are common.

Soft Tissue Tumors

Nevus: A nevus, usually a brown patch of skin, is a lesion that may be present at birth. If the nevus were located in an area that is at risk for sun exposure, or is large in size, excision is recommended. If the nevus is too large for simple excision, tissue expansion to recruit additional tissue may be needed to allow for an excision to be possible.

Malignancy of the Head and Neck Region:

Head and neck tumors are rare in childhood, but usually necessitates a wide resection with reconstruction. Adjuvant therapy may also be needed for optimal results.

Pediatric Traumatic Deformities

Trauma is a frequent problem in the pediatric population. To best utilize the natural rapid healing seen in children, repairs need to be done in an expedient fashion. Early treatment helps decrease permanent scarring and minimizes secondary growth disturbances.

Contact Form

Name: *

Email: *

Phone:

Preferred Contact Method:
Phone E-mail
Comments:

If you can see the box below this sentence, don't fill it out! Your message will not be submitted if you do.

With the exception of the Photo Gallery page, images used on this site are for illustrative purposes only; and persons depicted in the images are models. Images do not indicate that the model has any association with advertised services, and does not endorse any products or services presented.