Boy with cleft lip and palate before (inset) and after cleft lip and palate repair at MassGeneral Hospital for Children.
Cleft lip and palate defects vary in severity and can be unilateral or bilateral. A cleft lip is the separation or opening in the upper lip that can continue into the nose. A cleft palate is a gap in the hard and/or soft palate, which results in an open connection between the oral and nasal cavities.
Children with cleft lip or palate represent a complex patient population. The cleft and resultant secondary deformities dramatically impact patients’ form and function. Most patients have speech and hearing problems, chronic ear infections, and difficulty eating or swallowing. The deformity also affects breathing, and diminished upper-jaw growth produces a malocclusion (abnormal bite).
Interdisciplinary Care at the Craniofacial/Cleft Lip and Palate Clinic
The Craniofacial/Cleft Lip and Palate Clinic at MassGeneral Hospital for Children, which is credentialed through the American Cleft Palate-Craniofacial Association, provides a comprehensive, interdisciplinary approach to treatment of clefts and other craniofacial deformities. The clinic’s core team of specialists includes oral and maxillofacial surgery, plastic surgery, speech/language pathology, orthodontia, genetics, and a clinic coordinator. The team closely collaborates with otolaryngology, pediatric radiology, neurosurgery, psychiatry, and other specialists, drawing from Massachusetts General Hospital and MassGeneral Hospital for Children’s vast medical expertise.
Comprehensive Treatment of Cleft Lip and Palate
1 in 600Number of infants born in the United States affected by a cleft lip, cleft palate, or both. The defects occur during early fetal development if the upper jaw, mouth, and nose fail to fuse together properly.
The Craniofacial/Cleft Lip and Palate Clinic at MassGeneral Hospital for Children treats both children and adults. Treatment of infants born with a cleft lip or palate begins within months after birth, although parents may consult with the Craniofacial/Cleft Lip and Palate Clinic team prenatally as the abnormality is often detected in utero.
Surgery to close a cleft usually occurs during the first year of life. The goal of the operation is not only to close the separation but also to maximize function and provide an environment for normal growth. Cleft palate repair, in particular, can be especially challenging because the surgeon must close the defect, separating the mouth from the nose and creating enough length to facilitate normal feeding and speech development. Oftentimes, cleft patients require multiple operations over many years to improve function and appearance.
Patients also require follow-up treatment for secondary problems throughout childhood and adolescence. Each stage of life requires a different focus of care. For example, otolaryngology is often necessary in the first year or two because the patients are more prone to ear infections. Speech therapy is needed as children begin talking.
Cleft patients often require speech therapy, specialized dental care, orthodontia, additional corrective jaw surgery, bone grafting, and genetic counseling for family planning. During the teenage years, a significant number of cleft patients require upper-jaw surgery to correct their malocclusion. Adult patients are also monitored annually or biannually for any tissue changes or other problems that may occur as a result of cleft repair.
Exploring Pathways to Prevention
In addition to caring for patients at all stages in their treatment process, researchers at MassGeneral Hospital for Children are working to prevent the defect. The hospital is currently conducting grant-funded research to isolate the gene(s) involved with cleft lip and palate. Researchers hope that this work will lead to prevention therapies for clefts.
MassGeneral Hospital for Children Research Helps Advance Craniofacial Technique
Courtesy of William Gerald Austen Jr., MD
MassGeneral Hospital for Children’s Craniofacial/Cleft Lip and Palate Clinic is also advancing research to improve treatment of cleft lip and palate. Oral and maxillofacial surgeons are pioneers in the technique of distraction osteogenesis (DO), which allows surgeons to gradually lengthen bones by placing tension forces across an osteotomy using specialized devices.
Surgeons use this technique to lengthen the upper jaw, which is often required in patients with complete cleft lip and palate once skeletal maturity is reached. DO is also used to correct upper- and lower-jaw deformities in children with syndromic and nonsyndromic Robin sequence, obstructive sleep apnea, hemifacial microsomia, and Treacher Collins and Nager syndromes, and in patients with syndromic and nonsyndromic midface hypoplasia (underdevelopment of the upper jaw).
Using DO, surgeons can lengthen the jaw without using bone grafts or soft tissue flaps from donor sites elsewhere on the patient’s body, as in traditional surgery. This significantly reduces morbidity as well as improves outcomes. Traditional surgery carries a high risk for relapse due to the inability of the soft tissue to accept the rapid level of movement of the jaw from surgery. In traditional surgery, the jaw is cut and fully extended all at once using bone grafts. In contrast, DO involves moving the jaw gradually, which is better tolerated by surrounding tissues.
Functionality of Distraction
Side view of patient when the devices were removed showing increased projection of the jaw.
MassGeneral Hospital for Children’s Craniofacial/Cleft Lip and Palate Clinic uses semiburied distraction devices, which are implanted along the mandible (lower jaw) or the maxilla (upper jaw). Only a metal rod activation arm protrudes from the tissue surface. To lengthen the jaw, the pin is turned once or twice each day, usually by a family member. One turn of the pin equals 0.5 mm of jaw movement. This lengthens the jaw incrementally as the body forms bone in the resultant gap.
The clinic’s Skeletal Biology Research Center, under the direction of Leonard B. Kaban, DMD, MD, is studying the biology of DO to better understand possible outcomes. They are also designing new approaches to DO, which may revolutionize jaw-lengthening techniques.
Design of State-Of-The-Art, Automated Distraction Device
With a second-phase grant from the National Institutes of Health, MassGeneral Hospital for Children’s Craniofacial/Cleft Lip and Palate Clinic is developing the first fully automated and buried miniature distraction device. The device will contain a micromotor and computer chip so that physicians can program the device to automatically lengthen the jaw. This will facilitate jaw lengthening without relying on the patient or a family member to turn the pin. In addition, the device is completely undetectable, with no pins protruding from the tissue.
Minimally Invasive Surgical Placement Techniques
The Clinic has already developed a number of new, endoscopic surgical techniques for placement of the miniature buried device. The techniques will allow surgeons to fully bury the distraction device with as little trauma to the jaw as possible.
Three-Dimensional Treatment Planning
Once the automated distraction device is implanted, it cannot be adjusted. That is why precision placement is critical to optimal outcomes. To ensure proper placement, MassGeneral Hospital for Children has developed an advanced three-dimensional treatment planning system. The system uses data from fine-cut computed tomograms (CTs) and produces lifelike spatial views of the facial structures. The comprehensive and realistic three-dimensional rendering helps guide surgical planning. It can also provide outcome prediction for cleft and other facial deformity cases. The clinic is already using three-dimensional treatment planning technology with semiburied devices.
Continuity of Care
The Craniofacial/Cleft Lip and Palate Clinic’s approach to planning and treatment of care from infancy to late adulthood not only potentially improves outcomes but also offers patients the comfort of true continuity of care. This continuity is unique in an industry in which cleft patients must often adapt to a new hospital environment when they graduate from a children’s program. At MassGeneral Hospital for Children’s Craniofacial/Cleft Lip and Palate Clinic, a patient’s entire treatment plan is often carried out by the same physicians who created it. In fact, the clinic also cares for entire families—adults who have had cleft deformities as well as their children who inherited the same defect. This continuity offers confidence to a uniquely affected population of patients whose treatment of this congenital deformity can last throughout their lifetime.
- Cleft lip and palate are among the most common genetic defects in the United States
- Cleft patients have complex needs that require lifelong care, treatment, and monitoring by an interdisciplinary team
- MassGeneral Hospital for Children is a pioneer in the technique of distraction osteogenesis (DO), which allows surgeons to gradually lengthen jawbones without the use of bone grafts in patients with clefts and other facial deformities
- With a second-phase grant from the National Institutes of Health, the Clinic’s Skeletal Biology Research Center is currently developing the first fully automated and buried miniature distraction device
Chief, Adult and Pediatric Plastic and Reconstructive Surgery
Massachusetts General Hospital and MassGeneral Hospital for Children
Chief, Oral and Maxillofacial Surgery, Massachusetts General Hospital
Walter C. Guralnick Professor and Chairman, Oral and Maxillofacial Surgery, Harvard School of Dental Medicine
Elizabeth Shannon, PhD, PNP
Clinic Coordinator, Craniofacial/Cleft Lip and Palate Clinic, MassGeneral Hospital for Children
MassGeneral Hospital for Children
MassGeneral Hospital for Children (MGHfC) is a full-service, family-centered, pediatric "hospital within a hospital." MGHfC provides the entire spectrum of pediatric care—from primary care to a broad, continually expanding range of specialty and subspecialty pediatric services. With more than 250 physicians representing more than 60 pediatric specialties and subspecialties, MGHfC has the expertise and experience—as well as the state-of-the-art facilities and technologies—to provide exceptional care for infants, children, and adolescents, including those who are critically ill and/or have rare disorders. MGHfC provides patient care services at its main campus in Boston, as well as at a number of community locations.
- To refer a patient, please call the MGHfC Access and New Appointment Center, Monday through Friday, 8 am to 5 pm, at 888-MGHfC11 (888-644-3211), or visit the MassGeneral Hospital for Children website
- For more on this article, visit the Cleft Lip and Palate Program at MassGeneral Hospital for Children