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Utilization of Mandibular Distraction in Persistent Buccopharyngeal Membrane
T.Kevin Cook MD, Christopher B. Gordon MD, Armando Uribe Rivera DDA
University of Cincinnati
2010-03-30
Presenter: Thomas Cook
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Author Category: Resident/Fellow
Presentation Category: Clinical
Abstract Category: Craniomaxillofacial
The persistent buccopharyngeal membrane is a rare malformation of the upper aerodigestive tract. A total of 23 cases have been reported in the literature, and treatment regimens vary, with a significant mortality. Our institution has a series of 5 infants who have undergone staged airway reconstruction with splitting of the membrane and more recently, mandibular distraction. We present our experience, a review of the pertinent embryology, and treatment options.
The mouth develops at the junction of the stomodeum and foregut. Initially, the buccopharyngeal membrane forms as the head rotates and envelopes a portion of the pericardial area to form the primitive mouth. This membrane separates the ectoderm of the stomodeum from the endoderm of the foregut. Typically, this structure should regress at 28 days of gestation. Failure to regress results in a persistent membrane that separates the mouth from the airway. The tongue is often hypoplastic at birth, and there is risk for aspiration. Frequently, the affected infants die soon after birth, due to dehydration and inability to protect their airway.
Initial management involves tracheostomy and gastrostomy, followed by staged membrane split, minimizing tendency for recurrent membrane scar formation. We have employed mandibular distraction as an adjunct to develop the airway and provide access for pharyngeal reconstruction in 3 patients. Surgical and radiographic findings are utilized to guide localization of the glottis, tongue, velum, and assist in management of these complex cases.