Perforated mucocele of the appendix in the left upper quadrant: A challenging anatomy and an improvised surgical technique

•Pseudomyxoma peritonei (PMP) is a devastating consequence of perforated appendicular mucocele. It is considered a rare disease.•The presence of asymptomatic gut malrotation in adults can complicate the clinical picture of acute abdomen. ** (starting a new highlight point): Not considering possible anatomical variations initially during assessment could delay the diagnosis and managment.•The introduction of Cytoreductive Surgery/Heated Intraperitoneal Chemotherapy (CRS/HIPEC) improved the outcome of many patients with PMP.•Preserving part of the colon during challenging colorectal resection to allow a colo-rectal anastomosis offers a superior quality of life. Pseudomyxoma peritonei (PMP) is a feared complication of appendicular mucocele perforation. Although a rare disease, its major sequel mandates recognition and early intervention. Intestinal malrotation is mostly asymptomatic in adults. Its significance arises when it complicates another coinciding condition by confusing the presentation, leading to delay in diagnosis and treatment. PMP and incidental finding of gut malrotation in adults are two rare events, and the chance of both occurring in the same patient is very slim. This can complicate the clinical picture and lead to devastating outcomes. We present a case of PMP in a patient with gut malrotation, managed with cytoreductive surgery (CRS) and heated intraperitoneal chemotherapy (HIPEC). Management with CRS/HIPEC has been found to improve outcomes for patients with PMP. However, the extensive disease and abnormal anatomy of the patient in our report proposed unique intraoperative challenges. Preserving part of the colon was possible with an improvised surgical technique that we used which proved to be safe and effective. Early recognition and consideration of uncommon but serious surgical conditions are essential for improved patient outcomes. To our knowledge, this is the first report in the English literature that describes the use of CRS/HIPEC for PMP in a case of intestinal malrotation. This improvised surgical technique was found to be safe and can provide a surgical solution for preserving part of the colon in selected patients.