Pre-Operative Imaging in Patients with 22qDS Undergoing VPD Surgery

CLINICAL QUESTION

Should children with 22q11 deletion syndrome (22qDS) routinely undergo pre-operative vascular imaging before velopharyngeal dysfunction (VPD) surgery to identify medialized internal carotid arteries (ICAs) and reduce operative risk?

BOTTOM LINE

Nearly half of children with 22qDS showed ICA medialization on imaging, and one in five required a change in the surgical plan. Although most authors favored routine imaging, high study heterogeneity and limited outcome data mean that its universal use remains uncertain.

BACKGROUND: Children with 22qDS frequently develop velopharyngeal dysfunction requiring pharyngeal surgery. Reports of medially displaced ICAs, sometimes lying within 3 mm of the pharyngeal wall, have raised concern about catastrophic hemorrhage during surgery. Since the 1980s, clinicians have debated whether all 22qDS patients should have pre-operative CT or MR angiography, balancing safety against radiation, anesthesia, and cost.

STUDY DESIGN: A systematic review and meta-analysis following PRISMA guidelines identified 11 studies including 398 unique patients (mean age 7.6 years; range 1.3–18.5). Of these, 372 underwent pre-operative ICA imaging before planned VPD surgery. Meta-analyses pooled rates of ICA medialization, high-risk vascular position, surgical modification or abandonment, complications, and nasopharyngoscopic accuracy in predicting ICA location.

SETTING: Data were synthesized from multicenter reports spanning seven countries and various tertiary craniofacial and pediatric otolaryngology centers.

SYNOPSIS: The pooled prevalence of ICA medialization was 47.1% (95% CI 29.2–65.5). Among these, 46.3% were deemed high risk, defined as an ICA < 3 mm from the pharyngeal mucosa or coursing retropharyngeally. Operative plans were modified in 19.4% of cases and abandoned in 1.2%; notably, no ICA injuries occurred. The pooled post-operative complication rate was 3.6% (95% CI 1.7–6.2). Nasopharyngoscopy proved unreliable for detecting ICA medialization, showing a true-positive rate of 54.1% and a false-negative rate of 18.3%, confirming that visible pulsations do not reliably indicate vascular proximity. Across studies, nine (81.8%) recommended routine imaging, while two advocated selective imaging based on clinical features or institutional protocols. The authors emphasized significant heterogeneity (I² = 91.6%) and the retrospective, small-sample nature of most studies. Although imaging frequently influenced surgical planning, its effect on reducing complications remains unproven. Prospective studies comparing outcomes with and without imaging are needed to establish evidence-based guidelines for pre-operative vascular screening in 22qDS. 

CITATION: Duckett KA, et al. Preoperative imaging in patients with 22q11 deletion syndrome undergoing velopharyngeal surgery. Laryngoscope. 2024;134:2551-2561. doi: 10.1002/lary.31181.

COMMENT: This systematic review shows that imaging has often been used pre-operatively for children with 22q11.2 deletion syndrome undergoing VPI surgery, though it has rarely resulted in changes in the surgery technique or plan. This article demonstrates that it is likely clinically okay to proceed with pharyngeal flap surgery or other VPI surgery for this patient population without pre-operative imaging unless there are anatomical concerns visualized (e.g., pulsating carotid in the posterior pharyngeal wall). Ryan Belcher, MD, MPH