Mirande, Mitchell H.

Abstract Background: Congenital peritoneal encapsulation (cocoon deformity) is a rare variant of intestinal malrotation. Objectives: To characterize the clinical presentation and outcomes in patients undergoing surgery for intestinal malrotation with cocoon deformity. Design: Retrospective review. Settings: A large tertiary care center, from January 2003 to December 2022. Patients and Methods: All patients who underwent surgery for intestinal malrotation with cocoon deformity were included. Sample Size: Nine patients were identified. Main Outcome Measures: Patient demographics and operative outcomes. Results: Most patients were female (88.9%), with a median age of 37 years (interquartile range [IQR], 19–53). Most were symptomatic upon diagnosis (88.9%), with abdominal pain (77.8%) being the most common symptom. One patient presented acutely, requiring immediate surgical intervention. Computed tomography was the most frequently used modality to establish a diagnosis of intestinal malrotation. A laparoscopic Ladd procedure was attempted in all patients, with three requiring conversion to open surgery. The median operative time was 119 min (IQR, 96.5–202), with a median estimated blood loss of 25 mL (IQR, 15–125). The median length of hospital stay was 2 days (IQR, 1.5–7). The most common postoperative complications were ileus ( n = 3, 33.3%) and 30-day readmission ( n = 3, 33.3%). Most patients experienced partial or complete resolution of symptoms ( n = 7, 77.8%) and no patients required reoperation. The median follow-up duration was 71.8 months (IQR, 21.3–138.7). Conclusions: The laparoscopic Ladd procedure with excision of the cocoon deformity can be performed safely and is associated with significant improvement in preoperative symptoms. Limitations: Cocoon deformity was not diagnosed preoperatively, and there is currently no gold standard imaging modality for its detection.