2026 FSA Podium and Poster Abstracts

Introduction: VACTERL association is a constellation of congenital anomalies involving at least three of the following systems: Vertebral defects, Anal atresia, Cardiac malformations, Tracheoesophageal fistula/esophageal atresia, Renal abnormalities, and Limb anomalies. Although anorectal malformation is common, colorectal perforation due to anorectal malformation is rare (1.7%) and can present with rapid clinical deterioration. Historically, neonatal bowel perforation carried a very high mortality rate of around 75% [1,2]. Although outcomes have improved in recent years, mortality can still approach 50% in infants who present late or who are premature, hypothermic, or septic [3]. Mortality is mainly due to sepsis or disseminated intravascular coagulopathy. We present a critically ill 3-day-old male with high anorectal malformation, severe abdominal distention, renal and cardiac anomalies, and previously unrecognized pneumoperitoneum discovered immediately prior to planned anoplasty. This case highlights diagnostic complexity, rapid clinical deterioration, and the multidisciplinary considerations required in managing neonates with VACTERL association.

Methods: A 3-day-old term male was born at another hospital with an imperforate anus. Initial abdominal xray demonstrated gaseous distention of bowel segments with minimal gas seen in pelvis, no free air. VACTERL evaluation demonstrated a large multicystic dysplastic left kidney, and duplicated right collecting system with hydroureteronephrosis on kidney ultrasound. Echocardiography revealed PFO, ASD, and PDA. Upon anesthesia preoperative evaluation, he exhibited hypotonia and oxygen saturation of 90% on room air. Comprehensive metabolic panel showed creatinine rising from 0.9 to 2.0 mg/dL within the last 24 hours, hyponatremia (124 mEq/L), and hypocalcemia (6.7 mg/dL), likely due to reduced renal perfusion from progressive abdominal distention. 

Immediately prior to the anoplasty, a confirmatory xray for feeding tube placement revealed a new pneumoperitoneum, decreased lung volumes, and leftward mediastinal shift. The procedure was converted to an emergent exploratory laparotomy.

Anesthesia induction was performed with preoxygenation and mask induction with sevoflurane followed by fentanyl and rocuronium. Direct laryngoscopy using a GlideScope Miller S1 blade (monitor not used) provided a grade 1 view, and a 3.0 microcuffed ETT was placed without difficulty. Despite adequate tidal volumes, SpO2 remained around 90% until surgical decompression. Upon abdominal incision, a large release of free air and succus occurred. A 3-cm perforation along the antimesenteric border of the rectum and distal sigmoid colon was identified. Colectomy with partial resection, colostomy creation, and mucous fistula were performed.

Intraoperative anesthetic management prioritized limiting peak airway pressures, maintaining low tidal volumes, and avoiding increases in pulmonary vascular resistance given the patient’s ASD, PDA, and PFO. Sodium bicarbonate and calcium improved perfusion and cardiac output. Fluids were restricted due to renal impairment, and TPN was continued throughout surgery. After decompression, oxygen saturation gradually improved. 

Results: The infant remained hemodynamically stable and had an uneventful postoperative recovery.

Conclusion: This case illustrates the complexity of caring for neonates with VACTERL association, particularly when rapidly progressive abdominal pathology develops. Severe distention contributed to respiratory compromise, impaired renal perfusion, and diagnostic challenges. Successful management required early recognition, rapid interdisciplinary coordination, anesthetic planning tailored to cardiopulmonary anomalies, and timely surgical intervention.