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 surgical management of speech production disorders in patients with velocardiofacial syndrome without preoperative cervical vascular imaging studies. Anomalous internal carotid arteries have been shown to be a frequent feature of velocardiofacial syndrome. These vessels pose a potential risk for hemorrhage during velopharyngeal narrowing procedures. Magnetic resonance angiography, and other forms of cervical vascular imaging studies such as computerized tomography, have been advocated as aids to surgery by defining the preoperative vascular anatomy. However, it remains unclear whether these studies alter either the conduct or outcome of operations on the velopharynx. In the first part of this study, we reviewed the charts and videonasendoscopic evaluations of 39 consecutive patients with confirmed or suspected velocardiofacial syndrome who underwent sphincter pharyngoplasty or pharyngeal flap from 1978 to 1996. The charts were reviewed to determine (1) the frequency of identification of abnormal pharyngeal pulsations; (2) whether such pulsations affected the conduct of the operative procedure; and (3) whether the presence of pulsations affected surgical morbidity and/or surgical outcome. None of the patients underwent any type of cervical vascular imaging study. In the second part of this study, we surveyed plastic surgeons with numerous years of experience participating on cleft-craniofacial teams, to ascertain practice patterns relating to the management of patients with velocardiofacial syndrome. The questions related specifically to the surgeons' behavior in relation to angiography and their awareness of any cases of surgical morbidity related to the cervical vascular system in patients with velocardiofacial syndrome. We were interested in discerning both how commonly this situation arises clinically and the distribution of the various types of operative procedures in common use. Of our 39 patients, 10 patients (26 percent) had detectable pulsations on preoperative nasendoscopy. Of these, five patients underwent sphincter pharyngoplasty and five underwent pharyngeal flap procedures. Preoperative instrumental and intraoperative clinical assessment of pulsatile vessels allowed velopharyngeal reconstruction in all patients without surgical morbidity. Results of the questionnaire indicated that most cleft surgeons do not routinely order cervical vascular imaging studies for all of their patients with velocardiofacial syndrome. About half of the respondents indicated that their operative approach was influenced by information obtained from angiographic studies. None of the surgeons queried were aware of any cases of surgical morbidity related to the cervical vascular system in patients with velocardiofacial syndrome. Nearly 50 percent of surgeons use pharyngeal flap procedures most frequently, whereas 22 percent of surgeons use sphincter pharyngoplasty most frequently. Results of this study support the safety of sphincter pharyngoplasty or pharyngeal flap procedures in patients with velocardiofacial syndrome without preparatory angiography. These procedures can be performed safely, even in patients 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 velocardiofacial syndrome population.