ORIGINAL ARTICLE Pharyngeal Flap and the Internal Carotid in Velocardiofacial Syndrome Sherard A. Tatum III, MD; JaKwei Chang, MD; Natalie Havkin, MS; Robert J. Shprintzen, PhD nternal carotid artery anomalies have been documented as a common clinical feature in velocardiofacial syndrome. There has been some controversy over the need for preopera- tive imaging procedures, such as magnetic resonance angiography, when planning pharyn- geal surgery for correcting velopharyngeal insufficiency. The purpose of this article is to Idescribe 20 patients with velocardiofacial syndrome who received comprehensive evaluation and underwent pharyngeal flap surgery within a 2-year period and to report the technique used for dissecting the flap and the surgical outcomes. Anomalies of the major neck vessels were present in all cases, but 5 of these 20 cases had particularly severe anomalies of the internal carotid arteries that placed the vessels directly deep within the donor site for the pharyngeal flap. Surgery was car- ried out successfully in all 20 cases using a modified approach after radiographic imaging was per- formed to locate the arteries. In the 5 cases with severe malpositioning of the internal carotid ar- teries, it was clear that the vessels could have been injured had their location not been identified and the surgical approach modified to avoid them. Arch Facial Plast Surg. 2002;4:73-80 Velocardiofacial syndrome (VCFS) is the standard angiography to demonstrate ec- most common multiple anomaly syn- topic and medial placement of the inter- drome associated with cleft palate, con- nal carotid arteries in 3 cases selected spe- stituting 8% of patients with cleft palate,1 cifically because of previous observations including overt, submucous, and occult from nasopharyngoscopy that showed submucous cleft palate. Although the fre- prominent arterial pulsations in the pos- quency of VCFS among individuals with terior pharyngeal wall during workup for cleft lip is not known, cleft lip does occur pharyngeal flap surgery. The abnormal as a finding in the syndrome at least oc- placement of the arteries was considered casionally.2 It has been reported that ap- a contraindication to pharyngeal flap sur- proximately 5% of all patients at large in- gery in these cases.3 In a more compre- terdisciplinary cleft palate–craniofacial hensive study using magnetic resonance centers have VCFS.1,2 Because velopha- angiography (MRA), Mitnick et al5 as- ryngeal insufficiency (VPI) is such a com- sessed 19 consecutive patients with VCFS mon disorder in the spectrum of anoma- referred for pharyngeal flap surgery. The lies in VCFS, it is likely that many of the MRA results were correlated to findings patients will present for surgical manage- from nasopharyngoscopic examinations ment of hypernasal speech. for observations of visible pulsations in the Anomalies of the internal carotid ar- pharyngeal walls. It was found that obser- teries in VCFS were initially reported in vations of pulsations did not predict me- 1987.3,4 MacKenzie-Stepner et al3 used dial deviation of the internal carotid ar- teries, and medially deviated arteries did From the Division of Facial Plastic and Reconstructive Surgery, Departments of not always result in visible pulsations. Otolaryngology and Pediatrics (Dr Tatum); the Division of Neuroradiology, 5 Department of Radiology (Dr Chang); the Communication Disorder Unit (Ms Havkin); Mitnick et al concluded that some type and the Center for the Diagnosis, Treatment, and Study of Velo-Cardio-Facial of vascular imaging procedure was nec- Syndrome, Department of Otolaryngology and Communication Science essary before undertaking pharyngeal flap (Dr Shprintzen), State University of New York Upstate Medical University, Syracuse. surgery because the placement of the (REPRINTED) ARCH FACIAL PLAST SURG/ VOL 4, APR-JUNE 2002 WWW.ARCHFACIAL.COM 73 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 SUBJECTS AND METHODS Velopharyngeal insufficiency was assessed from clini- cal speech and language evaluation, multiview videofluo- roscopy, and nasopharyngoscopy using the International SUBJECTS Working Group rating scale (Figure 1).8 The ratings of the components of velopharyngeal closure are listed in Table The study sample comprised 20 consecutively referred pa- 2 along with the degree of perceived hypernasality. tients with VCFS. All cases were confirmed by FISH (fluo- rescent in situ hybridization) to have a 22q11 deletion, and FLUOROSCOPIC AND ENDOSCOPIC ASSESSMENTS all patients were examined by the fourth author (R.J.S.) to confirm the clinical diagnosis. There were 11 male sub- Preoperatively, velar motion varied among the sample but jects and 9 female subjects, ranging in age from 4 to 17 years was rated above 0.5 for only 1 case (Table 2).8 A rating of (Table 1). This sample represents all cases of VCFS re- 0.5 indicates velar motion of half of the distance from the ferred for surgical management of VPI from the Center for rest position to the posterior pharyngeal wall. In 14 cases, the Diagnosis, Treatment, and Study of Velo-Cardio- velar motion was under 0.5. Poor or absent lateral pharyn- Facial Syndrome of the State University of New York Up- geal wall motion (a rating of 0.2 or lower) was found in all state Medical University, Syracuse, within a 2-year period cases except 1. In 2 cases, there was asymmetric lateral pha- (1998-1999). These 20 cases represented 25% of all cases ryngeal wall motion, with the right lateral pharyngeal wall of VCFS referred to the VCFS center within this 2-year pe- showing motion rated at 0.2 and the left lateral wall show- riod. The other cases were not referred for surgery for a ing no motion (0.0) in both cases. A rating of 0.2 indicates variety of reasons, including age (too young), no evidence motion less than half of the distance to the pharyngeal mid- of VPI (about 10% of the sample), refusal of additional sur- line. In all other cases, the lateral pharyngeal walls were gery because of previous failures elsewhere, or successful rated at 0.0 or 0.1 bilaterally. In such cases, very wide sub- treatment elsewhere prior to referral. obstructing pharyngeal flaps are recommended. All patients received a comprehensive evaluation that included MRA or computed tomography (CT) scanning, MAGNETIC RESONANCE ANGIOGRAPHY video nasopharyngoscopy, and multiview videofluoros- copy. Five patients had previously undergone other sur- Nineteen patients had an MRA prior to pharyngeal flap sur- gical procedures for VPI (Table 1). Four of the patients had gery. One patient required contrast-enhanced CT scan- previous failed sphincter pharyngoplasties, and 1 had a failed ning because of the presence of a pacemaker. In most cases, Furlow palate repair as a secondary procedure. In all 5 cases, MRA was performed within a week of surgery, but in a few hypernasality was not corrected by the surgery. cases MRA was done several months prior to admission. Of the total sample, 1 patient had an overt cleft of the The MRA protocol includes scanning of the entire head and secondary palate, 10 had obvious submucous clefts includ- neck and the upper chest to the aortic arch using 7-mm- ing bifid uvula, and 8 had occult submucous clefts (Table thick abutting slices. We also scan the spine because of the 1). One patient had no evidence of a cleft, and 2 patients frequency of tethered cord and other spinal anomalies in had asymmetric VPI related to pharyngeal hypotonia on the VCFS. The brain is also assessed from the magnetic reso- left (Table 2). The frequency of congenital heart anoma- nance imaging scans. The MRA is formatted in coronal, lies is also listed in Table 1. All patients had grossly nor- transverse, and sagittal views, and 3-dimensional recon- mal expressive language at the time of surgery. structions of the vessels are done as well. The common ca- rotid, internal carotid, external carotid, and vertebral ar- ASSESSMENT PROCEDURES teries are all isolated in relation to their position within the pharyngeal soft tissues. One patient had a pacemaker, which All patients were evaluated by the interdisciplinary team necessitated the substitution of contrast-enhanced 3-di- at the Center for the Diagnosis, Treatment, and Study of mensional CT angiography instead of MRA. Velo-Cardio-Facial Syndrome. Evaluation procedures in- cluded the following: ASSESSMENT OF TONSILS 1. speech and language evaluation including a group rat- Previous reports have shown that tonsillectomy prior to pha- ing of nasal resonance on a 5-point scale (hyponasal, ryngeal flap surgery is an important component in the avoid- normal, mild, moderate, and severe hypernasality) ance of obstructive sleep apnea following surgery.9,10 Ton- 2. genetic/dysmorphologic evaluation sils were assessed using both videofluoroscopy and 3. cytogenetic/molecular genetic evaluation including FISH nasopharyngoscopy (Figure 2 and Figure 3). It has been 4. flexible fiber optic nasopharyngoscopic evaluation our experience that tonsillar hypertrophy is not always well 5. multiview videofluoroscopic evaluation in at least fron- recognized on oral examination. In cases of VCFS, the phar- tal and lateral views ynx (including both the oropharynx and nasopharynx) is 6. facial plastic surgery typically deep secondary to platybasia11 and a short, defi- 7. immunologic evaluation cient palate.12 When tonsils are assessed perorally using the 8. audiologic evaluation familiar scale (0 to 4+), the rating is based on the medial pro- 9. magnetic resonance angiography of the neck vessels jection of the tonsils. When the pharynx is deep, as in VCFS, and magnetic resonance imaging of the brain and spine it may be that the path of least resistance for tonsillar growth or CT scanning is posterior, posteroinferior, or posterosuperior. Previous re- 10. otolaryngologic evaluation ports have documented that tonsils can grow behind the pal- 11. a variety of evaluations from other disciplines, as needed, ate and faucial pillars.13,14 When this posterior growth oc- including endocrinology, neurology, cardiology, ne- curs, the tonsils can be seen on endoscopic examination phrology, and hematology.