P1.078: Successful Delayed Closure of a Mushroom-Shaped Giant Omphalocele: A Technical Case Report

Introduction: Giant omphaloceles are typically managed with delayed closure due to inadequate abdominal domain and associated cardiopulmonary comorbidities. Some giant omphaloceles are "mushroom-shaped," posing a unique morphologic challenge because the orifice is small in relation to the herniated organs. We describe a successful delayed closure, emphasizing key technical aspects.

Case Description: A 2-year-old male, born at 37 weeks (3360 g) with a prenatal diagnosis of giant omphalocele, was initially managed non-operatively with silver-impregnated hydrofiber dressing to promote epithelialization. Postnatal examination revealed a giant omphalocele with a narrow stalk, along with a moderate-sized atrial septal defect and stable hemodynamic status. Given the significant herniation, a decision was made to pursue delayed closure.
Two weeks prior to surgery, an examination under anesthesia (EUA) was performed. Intraoperative ultrasound confirmed the superficial course of the suprahepatic IVC, and manipulation during EUA confirmed that closure would be feasible. At the time of definitive surgery, an elliptical skin incision was made, revealing non-adherent bowel but a loosely adhered liver to the epithelialized sac, which was easily separated with electrocautery. A sufficiently large inferior fascial incision allowed caudal repositioning of the liver without hepatic vein kinking. The herniated organs were successfully reduced, and fascial closure was achieved without tension. The patient had an uneventful recovery with no wound complications at three months postoperatively.

Discussion: Delayed closure of giant omphaloceles is dictated by the balance between the herniated organ volume and abdominal domain, not solely by morphology. Key technical considerations for “mushroom-shaped” giant omphalocele include:
1. Preoperative EUA with intraoperative ultrasound to delineate vascular structures and confirm the feasibility of closure. Additionally, performing EUA on a separate day optimizes OR utilization by preventing unplanned scheduling gaps if closure is deemed unfeasible.
2. Adequate caudal extension of fascial incision to facilitate organ reduction without vascular compromise.
3. Careful dissection of hepatic adhesions to minimize intraoperative bleeding and optimize reduction.

Conclusion: This case highlights the importance of technical strategies in delayed closure of mushroom-shaped giant omphaloceles. By ensuring adequate fascial release and leveraging intraoperative imaging, successful reduction and closure can be achieved with minimal morbidity.