The otolaryngologist and the patient with velocardiofacial syndrome

Velocardiofacial syndrome is the most common syndrome associated with clefting of the secondary palate. The endoscopically diagnosed occult submucous cleft palate is the most common palate anomaly associated with this syndrome. Patients with velocardiofacial syndrome present a special challenge to the otolaryngologist, who must be familiar with the diagnostic and therapeutic aspects of this syndrome. We report the findings in 21 patients with velocardiofacial syndrome. Only 11 (52%) had the typical manifestations, and others had only partial phenotype. Adenoidectomy must be avoided in these patients, since undiagnosed occult and overt submucous cleft palate are the most common cause of velopharyngeal insufficiency after adenoidectomy. Three patients had aberrant carotid arteries. This anomaly must be excluded by nasoscopy and computed tomographic scanning before pharyngeal flap surgery is considered in these patients. Criteria for exclusion of high-risk patients from adenoidectomy are presented and discussed.