IS PHARYNGOPLASTY A RISK IN VELOCARDIOFACIAL SYNDROME - AN ASSESSMENTOF MEDIALLY DISPLACED CAROTID ARTERIES

The association of medially positioned internal carotid arteries and v elocardiofacial (Shprintzen) syndrome was first made in 1987. This is also the most common syndrome associated with facial clefting. The pot entially dangerous implications in children with this syndrome requiri ng pharyngoplasty for velopharyngeal incompetence and stigmatized hype rnasal speech involve potential damage to these vessels. This three-pa rt study was undertaken to analyze this anatomic variant. First, a gro up of 25 children with velocardiofacial syndrome, velopharyngeal incom petence, and obvious posterior pharyngeal pulsations seen on nasendosc opy was studied by CT angiography to determine the degree of this abno rmal vascular pattern. This technique, together with three-dimensional reconstructions, made it possible to determine the precise location o f these abnormally positioned vessels. Second, our routine superiorly based pharyngeal flap was measured by lateral cervical x-ray to show t he distal tip of the flap. The variance was minimal and demonstrated t he tip of most flaps to be at the disk between the C2 and C3 vertebrae . By correlating this information with the CT angiography, the risk of surgery can be determined on strict anatomic grounds, allowing custom ized flap design in some unilateral cases. In this series of children, routine superiorly based pharyngoplasty would be safe in 52 percent, while in 28 percent a pharyngeal flap would be safe if custom designed , and in the remaining 20 percent surgery should not be attempted beca use the risk of damage to the carotid arteries is too great. Third, in a double-blind study, velocardiofacial children with obvious pulsatio ns seen on nasendoscopy were grouped with other children with palatal dysfunction. When only endoral examination was performed by plastic su rgeons and plastic surgical residents, no vascular pulsations were eve r seen. This indicates another important role of nasendoscopy in the p reoperative assessment of children for palatopharyngoplasty.