LIMITED VALUE OF PREOPERATIVE CERVICAL VASCULAR IMAGING IN PATIENTS WITH VELOCARDIOFACIAL SYNDROME

The purpose of this two-part study was to evaluate the safety of surgi cal management of speech production disorders in patients with velocar diofacial syndrome without preoperative cervical vascular imaging stud ies. Anomalous internal carotid arteries have been shown to he a frequ ent feature of velocardiofacial syndrome. These vessels pose a potenti al risk for hemorrhage during velopharyngeal narrowing procedures. Mag netic resonance angiography, and other forms of cervical vascular imag ing studies such as computerized tomography, have been advocated as ai ds to surgery defining the preoperative vascular anatomy. However, it remains unclear whether these studies alter either the conduct or outc ome of operations on the velopharynx. In the first part of this study, we reviewed tile charts and videonasendoscopic evaluations of 39 cons ecutive patients with confirmed or suspected velocardiofacial syndrome who underwent sphincter pharyngoplasty or pharyngeal nap from 1978 to 1996. The charts were reviewed to determine (1) the frequency of iden tification of abnormal normal pharyngeal pulsations; (2) whether such pulsations affected tile conduct of tile operative procedure; and (3) whether die presence of pulsations affected surgical morbidity and/or surgical outcome. None of the patients underwent arty type of cervical vascular imaging study. In the second part of this study, we surveyed plastic surgeons with numerous years of experience participating on c left-craniofacial teams, to ascertain practice patterns relating to th e management of patients with velocardiofacial syndrome. Tile question s related specifically to die surgeons' behavior in relation to angiog raphy and their awareness of any cases of surgical morbidity related t o tile cervical vascular system in patients with velocardiofacial synd rome. We were interested in discerning both how commonly this situatio n arises clinically and the distribution of the various types of opera tive procedures in common use. Of our 39 patients, 10 patients (26 per cent) had detectable pulsations on preoperative nasendoscopy. Of these , five patients underwent sphincter pharyngoplasty five underwent phar yngeal flap procedures. Preoperative instrumental and intraoperative c linical assessment of pulsatile vessels allowed velopharyngeal reconst ruction in all patients without surgical morbidity. Results of tile qu estionnaire indicated that most cleft surgeons do nor routinely order cervical vascular imaging studies for all of their patients with veloc ardiofacial syndrome. About half of the respondents indicated that the ir operative approach was influenced by information obtained from angi ographic studies. None of tile surgeons queried were aware of any case s of surgical morbidity related to the cervical vascular system in pat ients with velocardiofacial syndrome. Nearly 50 percent of surgeons us e pharyngeal nap procedures most frequently, whereas 22 percent of sur geons use sphincter pharyngoplasty most frequently. Results of this st udy support the safety of sphincter pharyngoplasty or pharyngeal flap procedures in patients with velocardiofacial syndrome without preparat ory angiography. These procedures can be performed safely even in pati ents having aberrant velopharyngeal pulsations. Given the market cost of magnetic resonance angiography ($1600), one must question the cost- efficacy of magnetic resonance angiography for routine use in the velo cardiofacial syndrome population.