Imaging Before Velopharyngeal Dysfunction Surgery in Patients with 22qDS?

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BACKGROUND

Velocardiofacial syndrome (VCFS), or 22q deletion syndrome (22qDS), occurs in one in 2,000 to 7,000 births. It is the most common genetic syndrome causing cleft palate. Additionally, there is a high prevalence of velopharyngeal dysfunction or velopharyngeal insufficiency in children with 22qDS due to a combination of functional and structural anomalies. Without repair, velopharyngeal dysfunction can cause hypernasal speech, nasal regurgitation, poor speech intelligibility, and difficulty swallowing. Treating velopharyngeal dysfunction in this population has historically been regarded as challenging due to concerns of medialization of the internal carotid artery (ICA).

ICA medialization in 22qDS velopharyngeal procedures was first described in 1987 when, during routine pre-operative nasopharyngoscopy, three children were noted to have posterior pharyngeal wall pulsations. This prompted imaging with CT scans, which confirmed ICA displacement. This further led to the conclusion that irregular pulsations on nasopharyngoscopy warranted CT imaging and that velopharyngeal surgery was contraindicated if the ICAs were displaced into the area of the flap. Since this article’s publication in 1987, many have recommended universal imaging of the ICAs for operative planning and to assess for medialization before velopharyngeal surgery in children with 22qDS. Others, however, have pointed out that routine imaging is not benign, as it comes with associated risks, including sedation, contrast, radiation exposure, and cost, that may outweigh its benefits. Over time, studies have begun to support the position that velopharyngeal surgery can be conducted in patients with VCFS and medialized carotid arteries without increased risk. Therefore, the need for pre-operative imaging in VCFS patients is called into question.

BEST PRACTICE

Routine universal imaging before velopharyngeal dysfunction operations in patients with 22qDS is not recommended. Rather, the surgical technique, with close attention to the underlying anatomy, including palpation before making an incision, utilization of ultrasound if there are vascular concerns at the time of surgery, and careful dissection, should be the priority. While pre-operative imaging demonstrating medialized ICAs has been associated with modifications in surgical approach, it is notable that in most cases, the planned procedure was still completed. Altering pharyngeal flap location or size is an adjustment made routinely following a focused physical exam, not reliant on imaging findings. A limitation to consider is the lack of comparison of vascular complication rates with or without imaging. No perioperative complications from an ICA injury were found in our literature review, though we acknowledge this may be a result of underreporting of negative outcomes. Overall, universal imaging confers additional risk to pediatric patients, is costly, and has not demonstrated substantial benefit to patient care.