Frey Procedure for Chronic Pancreatitis with Situs Inversus Totalis

Abstract

Situs inversus totalis is a rare congenital anomaly that occurs at an incidence of 1 in 10,000 in the general population and is characterized by mirroring of organs across the sagittal plane relative to normal orientation.1 This condition is asymptomatic, but presents an important surgical consideration. Cardiovascular and hepatobiliary malformations compound the technical difficulty of the surgical procedure. Advanced diagnostic imaging techniques, such as CT angiography, are necessary preoperatively to identify malformations and aberrant structural orientations and to guide operative planning. Gastrointestinal malformations are rarely associated with situs inversus, which may contribute to chronic pancreatic inflammation. Chronic pancreatitis results in irreversible structural and functional changes to pancreatic tissue, often requiring surgical decompression or resection. Surgical decompression can be achieved by the Frey, Puestow, or Beger procedure. A 32-year-old woman presented for evaluation of chronic pancreatitis, complicated by exocrine insufficiency, gastroduodenal artery aneurysm, pancreatic pseudoaneurysm, pseudocysts, and splenic vein thrombosis with gastric varices. Initially, she had episodes of acute pancreatitis triggered by heavy alcohol consumption and smoking, which was then compounded by abdominal trauma, but now she presented with the episodes occurring spontaneously and refractory to medical therapy. Genetic testing was negative for SPINK1 and PRSS1 mutations. Because of the severity of the disease with portal vein narrowing and multiple collateral varices, the patient was not a surgical candidate for pancreatic resection or total pancreatectomy with islet autotransplantation. Given the debilitating pain and dilated pancreatic duct with fibrosis at the head of the pancreas, the Frey procedure was recommended. Situs inversus totalis was previously diagnosed incidentally on endoscopic evaluation for epigastric pain. Preoperative CT angiography was obtained to elucidate the vascular anatomy and assist with operative planning (Fig. 1). The patient was taken to the operating room and the abdomen was opened through a midline incision. Situs inversus totalis was grossly evident. The omentum was lifted and the lesser sac entered. The scar tissue was excised to expose the anterior surface of the pancreas. The distal-most portion was densely adhered to the stomach because of a prior cystgastrostomy procedure. The body of the pancreas was opened and the head excised using an excavation Bern procedure. Approximately 4 cm of the pancreas body was opened and several large calculi were removed from the pancreatic duct, which was dilated to approximately 7 mm. The gland was extremely firm and the duct had a beefy red appearance. The bowel was divided approximately 20 cm distal to the ligament of Treitz, and the distal end of the stapled bowel was brought up for a lateral pancreaticojejunostomy, which was performed in two layers using 3-0 silk and 3-0 synthetic polyfilament absorbable sutures. A jejunostomy was created with a side-to-side staple technique in antiperistaltic fashion, and the resultant defect on the proximal side was stapled closed and oversewn with a 3-0 silk suture. The mesenteric defect was closed with 3-0 silk and the omentum was placed over the entire anastomosis. Fascia and skin were closed in standard fashion. The patient tolerated the operation well. Although the procedure was uneventful, the anatomical inversion of organs and vessels provided an interesting technical challenge. The patient did well postoperatively. After an uneventful brief stay in the postanesthesia care unit, she was transferred to the inpatient ward. Her postoperative Address correspondence and reprint requests to Christopher L. Kalmar, M.D., M.B.A., Department of Surgery, Virginia Tech Carilion, 1906 Belleview Avenue SE, Roanoke, VA 24014. E-mail: [email protected].