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- Title
Perioperative Risk Factors in Patients With 22q11.2 Deletion Syndrome Requiring Surgery for Velopharyngeal Dysfunction.
- Authors
Stransky, Carrie; Basta, Marten; McDonald-McGinn, Donna M.; Solot, Cynthia B.; Drummond, Denis; Zackai, Elaine; LaRossa, Don; Kirschner, Richard; Jackson, Oksana
- Abstract
Objective: To determine the prevalence of cardiac, cervical spine, and carotid artery abnormalities in patients with 22q11.2 deletion syndrome (22q11.2DS) undergoing surgery for velopharyngeal dysfunction (VPD), associations between the presence of these abnormalities, and whether these abnormalities caused changes in surgical management or perioperative complications. Design: Retrospective review. Setting: Tertiary pediatric hospital. Patients: Seventy patients with 22q11.2DS with complete preoperative cervical vascular and spine imaging and cardiac evaluation between 1998 and 2011. Main Outcome Measures: Incidence of cardiac, cervical spine, and vascular abnormalities; related perioperative complications; and resulting changes in surgical, anesthetic, or perioperative management plan. Results: Cardiac abnormalities occurred in 45 patients (64.3%), and 8 patients required cardiac anesthesia. Thirty-eight patients (54.3%) had at least one vascular abnormality of the neck, and 14% had medial deviation of the internal carotid artery. Surgery was not performed in one patient, and the surgical plan was altered in three patients because of carotid anomalies. Cervical spine abnormalities were found in 24 patients (34.3%); 8 patients demonstrated radiographic evidence of cervical instability and were treated with spinal precautions during surgery. The presence of one anomaly was not predictive of any other finding, and there were no complications related to the heart, cervical spine, or carotid arteries. Conclusions: Anomalies of the heart, cervical spine, and cervical vasculature occur frequently in 22q11.2DS, vary drastically in severity, and are impossible to predict based on other features of the syndrome. Preoperative diagnosis of these comorbidities with routine imaging can minimize the risk of avoidable surgical complications.
- Subjects
BLOOD-vessel abnormalities; CERVICAL vertebrae abnormalities; ACADEMIC medical centers; CHROMOSOME abnormalities; CONGENITAL heart disease; FISHER exact test; MAGNETIC resonance imaging; MEDICAL protocols; RISK assessment; SURGICAL complications; VELOPHARYNGEAL insufficiency; RETROSPECTIVE studies; SEVERITY of illness index; DATA analysis software; MAGNETIC resonance angiography; MANN Whitney U Test
- Publication
Cleft Palate Craniofacial Journal, 2015, Vol 52, Issue 2, p183
- ISSN
1055-6656
- Publication type
Article
- DOI
10.1597/13-206