The Use of Magnetic Resonance Angiography prior to Pharyngeal Flap Surgery in Patients with Velocardiofacial Syndrome

Abstract

Twenty consecutive patients with velocardiofacial syndrome underwent magnetic resonance angiography (MRA) to determine if abnormalities of the neck arteries would contraindicate pharyngeal flap surgery. All 20 patients were found to have anomalies to the carotid arteries, vertebral arteries, medially placed internal carotids, low carotid bifurcations, and tortuous or kinked internal carotids. The internal carotids were found to be almost directly under the mucous membrane of the pharynx in two patients. In these two patients, the arteries were close to the pharyngeal midline at the base of the first cervical vertebra and might easily be severed during the raising of a pharyngeal flap. Hypoplastic vertebral arteries also were found. One patient had an extra neck vessel. The anomalies of the internal carotids did not have a strong correlation with endoscopically observed pulsations in the position affected the location of the internal carotids did not have a strong posterior pharyngeal wall. It also was found that head position affected the location of the internal carotid arteries when they were located close to the pharyngeal mucous membrane. The information provided in the MRA studies allowed assessment of the arterial anomalies in relation to the flap donor site so that the patients in the sample who underwent pharyngeal flap surgery using a short superiorly based flap had no major bleeding complications.

Velo‐cardio‐facial syndrome: 30 Years of study

“…It has also been found that there is a high frequency of palatal and pharyngeal asymmetry in VCFS that can also only be determined by endoscopic assessment [Chegar et al, 2006]. Among the anomalies seen in the pharynx are major vascular anomalies including abnormal placement of the internal carotid arteries [MacKenzie-Stepner et al, 1987;Mitnick et al, 1996;Tatum et al, 2002] and tortuous or kinked vertebral arteries [Mitnick et al, 1996]. These vascular anomalies have been cited as potentially dangerous risks when pharyngeal surgery is being planned [MacKenzie-Stepner et al, 1987;Mitnick et al, 1996;Tatum et al, 2002].…”

“…Among the anomalies seen in the pharynx are major vascular anomalies including abnormal placement of the internal carotid arteries [MacKenzie-Stepner et al, 1987;Mitnick et al, 1996;Tatum et al, 2002] and tortuous or kinked vertebral arteries [Mitnick et al, 1996]. These vascular anomalies have been cited as potentially dangerous risks when pharyngeal surgery is being planned [MacKenzie-Stepner et al, 1987;Mitnick et al, 1996;Tatum et al, 2002]. It has also been reported that the muscle tissue in the pharynx has abnormally small fiber size and abnormal distribution of fiber type [Zim et al, 2003].…”

“…Major anomalies of the chest vessels [Shprintzen et al, 1978;Young et al, 1980;Shprintzen, 2005b;VCFS Educational Foundation, 2007], neck vessels [MacKenzie-Stepner et al, 1987;Mitnick et al, 1996;Shprintzen, 2000b;Tatum et al, 2002], and brain vasculature [Shprintzen, 2000a] have all been reported in individuals with VCFS. It has been hypothesized that the large number of vascular anomalies in VCFS in major vessels may result in a number of developmental sequences [Shprintzen et al, 1997], a notion supported by reports of Robin sequence [Shprintzen, 1988;Shprintzen and Singer, 1992], DiGeorge sequence [Robin and Shprintzen, 2005;Shprintzen, 2005b], Potter sequence [Devriendt et al, 1997] and holoprosencephaly sequence [Wraith et al, 1985] …”

“…Secondary reconstructive surgery is almost always necessary. It has been demonstrated that pharyngeal flap surgery can be highly effective [Tatum et al, 2002], but an anomalous course of the internal carotid arteries presents a major risk factor [Mitnick et al, 1996;Tatum et al, 2002]. The use of preoperative magnetic resonance angiography to locate the internal carotids has been recommended in children with VCFS [Mitnick et al, 1996;Tatum et al, 2002].…”

Qualitative MRI findings in adults with 22q11 deletion syndrome and schizophrenia

“…Because of reported skull and vertebral abnormalities (Arvystas and Shprintzen 1984;Beemer et al 1986;Finkelstein et al 1993;Ming et al 1997;Ryan et al 1997), the skull base structures were assessed. In addition, cerebral and cervical arteries were also assessed using magnetic resonance angiography (MRA) because of a reported high prevalence of carotid and vertebral arterial anomalies in 22qDS (Goldberg et al 1993;MacKenzie-Stepner et al 1986;Mitnick et al 1996).…”