Pediatric craniofacial anomalies
Welcome to the UTHSCSA Center for Reconstructive Pediatrics.
Patient appointments & physician referrals: - 210-257-1883
Mission Statement: To provide the best possible care to children born with craniofacial anomalies, to provide this care with compassion and respect, and to advance knowledge through education and research.
Craniofacial anomalies may be simple, or may be complex, requiring a comprehensive team approach. At our centers, we have specialists from nursing, genetics, ophthalmology, audiology, speech, ENT, dentistry, orthodontics and plastic surgery, with a team coordinator to assure your child's every medical need is met. We have two convenient clinic locations, one downtown and one in the medical center area. There are weekly clinic visits and monthly cleft palate team appointments.
Cleft Lip and Palate:
Approximately 1 in every 700 children born in South Texas have cleft lip and/or palate. Therefore, cleft lip and palate constitutes the most common condition treated at this center. When a child is born with cleft lip and palate, a member of our team will usually visit the baby and the parents in the hospital and provide immediate counseling. More frequently though, cleft lip and palate are diagnosed by prenatal ultrasound and counseling is begun prior to the child's birth.
After birth, once feeding is established and other anomalies excluded, the baby is seen by a pediatric dentist who will fabricate an oral plate. This plate decreases the cleft gap and molds the nose -- it's known as the presurgical nasal alveolar molding (PNAM).
The lip is usually repaired at approximately 10 weeks of age and the palate at 8 months old. Revision of the lip and the nose is done again as early as 4 years old if needed. The alveolar bone graft is performed at 7-9 yrs old. Sometimes, further surgeries may be necessary to repair nasal air leaks in speech (VPI), to advance the inhibited facial growth (orthognathic surgery), and correct nasal deformities (rhinoplasty).
Your child will be seen and evaluated annually by the entire cleft team of specialists. We feel this annual checkup is extremely important to insure the best possible results for your child. The annual checkup will allow us to schedule the necessary surgery and dental, hearing, and speech rehabilitation.
Premature fusion of skull sutures at birth causes deformation of the skull shape. Depending on the suture involved, the skull compensates and grows into a variety of head shapes. When the sagittal suture fuses, elongation of the skull occurs and is termed scaphocephaly. Other common conditions include fusion of the uni-coronal suture leading to anterior plagiocephaly, metopic suture leading to trigonocephaly, bicoronal suture leading to brachycephaly, and lambdoidal suture producing posterior plagiocephaly. Occasionally, the premature fusion of the skull sutures is combined with other anomalies and genetically inherited. The ones most frequently seen are the Apert's, and Crouzon's syndromes. The surgical release of the fused suture is often performed early in conjunction with neurosurgery. Endoscopic strip craniectomy is also offered at our center.
Several deformities of the ear exist and treatments will vary. Microtia, or arrested formation of the ear is graded by severity.
Grade I is a diminutive ear. Grade II is where the top and middle of the ear is deformed, and grade III is where only the lobule of the ear is present. Often, the canal is closed in the third grade.
Reconstruction of the ear usually involves recreating the outer ear, then the canal may or may not be opened, a decision made by the neurootologist.
The ear can be reconstructed using either the rib cartilage, or a medpor prosthetic. In our center, the majority of the ears are reconstructed by using the medpor ear implant, covered by the temoproparietal fascia and skin grafts. We favor this type of reconstruction because chest incision is avoided, fewer stages of surgery are required, and the ear can be reconstructed as early as four years old, allowing the child to avoid teasing in school.
This abnormal proliferation of blood vessels is seen in premature babies and first born children. The lesion is seen as a red spot at birth, then begins to proliferate shortly after. The proliferation of the vascular mass continues for the first year, then starts to involute. Within about five years, mass reduces by 50%; almost 10 years are required for the to reduce by 90%. The majority of hemangioma will not require treatment.
However, if the mass proliferates extensively during the first year, affecting adjacent structures or obstructing the vision, then early treatment with steroid injections will usually halt the rapid growth process. The steroid injection may have to be repeated during the first year.
Rarely, surgical removal of the hemangioma is desirable in situations where the mass is in the visible areas of the body, such as the face. The surgery is done during the involutional phase (after 1 year old), before the child reaches school age.
Vascular malformation differs from the hemangioma in that vascular malformation is present at birth and grows with the child. It usually does not have the proliferative and involutional phase as seen in hemangioma. Examples of vascular malformation include capillary malformation (port wine stain), venous malformation , arterial-venous malformation, and lymphatic malformations. Treatment of vascular malformation depends on the type of lesion; treatments may include laser, interventional radiology, sclerotherapy, and surgical debulking.
Nevus or mole can be present at birth. Usually they are brown in color and have a hair component. As the child gets older, the nevus becomes larger, the color becomes darker and the skin becomes more pebbly. The majority of nevi are benign; only a very small percentage turn into skin cancer. We often work together with a pediatric dermotologist and, in conjunction with the parents, we will formulate a decision on the management of the nevus. We offer surgery to remove these nevi through serial excision, skin graft, or using tissue expanders.
The development of the facial bone, upper jaw (maxilla) and lower jaw (mandible), as well as their relationship to each other, is important in a normal-appearing face.
When the maxilla is retrusive or the mandible is excessive, it produces an underbite, known as a class III jaw relationship.
When the mandible is retrusive, the teeth may have an overbite, or known as a class II jaw relationship. Surgical correction is sometimes necessary to achieve a harmonious facial profile. The process begins with careful planning with an orthodontist. The braces are next placed to align the teeth. This is followed by surgery, and a final adjustment of teeth with braces. Jaw deformities are often associated with cleft lip and palate, hemifacial microsimia, Treacher-Collins syndrome, Apert's and Crouzon's syndrome.
Lengthening of the jaw or facial bones can now be accomplished by using distraction osteogenesis. First, the bone is cut, followed by placement of a distraction device on either side of the bone. The device is activated to slowly pull the bone and overlying sort tisssue apart.
This procedure is particularly useful in babies born with small jaw (Pierre Robin Sequence), which can cause airway difficulties. The distraction device is applied internally or externally to the jaw to lengthen the mandible. Often, tracheostomy is avoided, or, if the baby already has the tracheostomy, it can now be removed sooner.
Distraction is also useful in patients with hemifacial microsomia, in which the smaller side of the jaw can be lengthened. In the middle of the face, the distraction is used in to advance the maxilla (Lefort I) in cleft lip and palate patients who have severely retruded mid-face. In Apert's and Crouzon's patients, the distraction is used to advance the midface, orbital rims (Lefort III) , and forehead (Monoblock).