2013, Number 2
Protrusion of sphenoethmoidal encephalocele through the cleft palate of a patient with median cleft face syndrome: a review of the literature and a case report
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ABSTRACTThe incidence of median cleft face syndrome is low worldwide, occurring in only 1 in 1,000,000 births. Though the clinical picture varies, the condition appears as a 0–14 Tessier cleft, with incomplete closure of skeletal and/or facial soft tissue along the midline. Herniation of intracranial content through these fissures is common due to agenesis of the anterior arch, making protrusion and descent toward the oral cavity possible. As the condition is uncommon, it has received little consideration, therefore we have decided to draw attention to it and review the rather limited amount of literature dealing with it that exists. We report the case of a 9-day-old male patient in whom a pulsatile brain herniation—initially mistaken for a premaxilla—, was detected through the patient’s cleft palate. Diagnosis and examination were carried out by the Maxillofacial Surgery and Neurosurgery service at Ciudad Juárez Specialist Children’s Hospital.
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