Use of Preoperative Cervical Vascular Imaging in Patients With Velocardiofacial Syndrome and Velopharyngeal Dysfunction in the United Kingdom

Abstract

Objective: In patients with velocardiofacial syndrome (VCFS), medial displacement of the internal carotid arteries (ICAs) may increase the risk of vascular injury during the surgical correction of velopharyngeal dysfunction (VPD). Some surgeons advocate the use of vascular imaging studies prior to surgery. Nevertheless, the role of preoperative imaging is still controversial. This study aimed to review the current practice of the UK cleft units and also examine our own practice at the Evelina London Children’s Hospital in relation to children with VCFS undergoing speech surgery over the previous 7 years. Design: A questionnaire was sent to all UK cleft surgeons to enquire about the management and use of preoperative vascular imaging in patients with VPD and VCFS. A retrospective study was also conducted of the unit’s 7-year series of patients with VPD and VCFS. Results: Thirty-four completed questionnaires were returned (response rate 100%). Most UK surgeons (73.5%) do not regularly order preoperative vascular imaging for patients with VCFS although some reportedly would consider it if a posterior pharyngeal wall pulsation was visible. In our unit, between 2013 and 2019, a total of 40 patients affected by VCFS have been assessed for VPD. A magnetic resonance angiography (MRA) was performed for 23 patients. Medial deviation of the ICAs was identified in 7 (30%) patients. Conclusions: The results of the national survey showed no consensus on routine use of preoperative vascular imaging. Our retrospective study showed a 30% prevalence of medialized ICAs in our patient cohort. In these patients, the MRA findings influenced the choice of speech surgery.

Preoperative Imaging in Patients with 22q11 Deletion Syndrome Undergoing Velopharyngeal Surgery

“…First, the presence of all ICA anomalies were collected, which encompassed medialization along with other ICA aberrancies, such as hypoplasia, tortuosity, kinking, and anterior deviation. 3,23,24,28,29,32 Vessels with a bifurcation at a more cranial or caudal level than expected or asymmetric vessels were not considered an anomaly. 3 Then, ICAs with medialization were specifically extracted, defined as deviating from a vertical course parallel with cervical vertebrae and instead aiming toward the pharyngeal midline 25% from normal course or more.…”

“…3,9,[18][19][20][21] In the usual position, the ICA is 1 to 1.5 cm away from the pharyngeal operative field; however, medialization can reduce this distance to millimeters. 22 The concern of ICA medialization in 22qDS velopharyngeal procedures was first described in 1987 by Mackenzie-Stepner et al 9,22,23 During routine preoperative nasopharyngoscopy to assess VPD, three children were noted to have posterior pharyngeal wall pulsations. The authors hypothesized that ICA displacement was causing the pulsations, which was then confirmed by imaging.…”

“…22 Since this article's publication, many proponents have recommended universal imaging of the ICAs for operative planning and to assess for medialization before velopharyngeal surgery in children with 22qDS. 9,[22][23][24] However, routine imaging for preoperative preparation remains controversial. 3,11,23 Routine imaging was contested in 1998 when Witt et al advised pharyngeal surgery for VPD would be safe without imaging; however, the article has been criticized due to deficient substantial scientific evidence errors including data based on anecdotes and surveys.…”

“…9,[22][23][24] However, routine imaging for preoperative preparation remains controversial. 3,11,23 Routine imaging was contested in 1998 when Witt et al advised pharyngeal surgery for VPD would be safe without imaging; however, the article has been criticized due to deficient substantial scientific evidence errors including data based on anecdotes and surveys. 1,21,25,26 Moreover, routine imaging is not benign, as there are concerns of radiation exposure, sedation, and costeffectiveness.…”

“…13,24,25,28,31,32 Although ICA medial displacement is a concern in velopharyngeal surgeries, there is no consensus on whether routine preoperative imaging should be obtained in all children with 22qDS before VPD surgery. 3,11,23 Therefore, we performed a systematic review and metaanalysis to evaluate the role of ICA imaging before VPD surgery in children with 22qDS.…”