341 Spleen Preserving Distal Pancreatectomy in a Known Case of VHL Syndrome Post Bilateral Open Adrenalectomy

Abstract Introduction Von Hippel-Lindau (VHL) syndrome is an autosomal-dominant disorder characterized by the development of multiple benign and malignant tumors, including pheochromocytomas and pancreatic neuroendocrine tumors (PNETs). We present a challenging case of a distal pancreatectomy, where splenic conservation was planned. Case Report A 25-year-old female with VHL syndrome, previously treated with an open bilateral adrenalectomy for pheochromocytomas in 2013, was found on surveillance imaging to have multiple pancreatic lesions, including a dominant 2x1.9 cm mass in the pancreatic body. In early 2024, she underwent laparoscopic distal pancreatectomy with spleen preservation. A low lithotomy position was given. Intraoperatively, a 3x2x2 cm lesion was identified in the distal pancreas. Dense adhesions and distorted anatomy were noted due to prior surgery. The left renal artery and vein were adherent to the lower border of the lesion. The splenic artery and vein were found in close proximity, superior to the renal vessels. Meticulous dissection was required to preserve both sets of vessels. The pancreatic parenchyma was divided using a vascular endostapler. Histopathology revealed a well-differentiated PNET and benign pancreatic cysts, consistent with VHL syndrome. Postoperatively, she developed a delayed pancreatico-cutaneous fistula, presenting as surgical site discharge and localized pain by day 14. Imaging showed a small collection, which resolved with conservative management. Follow-up imaging confirmed complete resolution of the fistula. This case highlights the technical complexity and anomalies associated with VHL, emphasizing the need for careful perioperative planning, multidisciplinary care, and vigilant long-term follow-up.