477 Surgical Solutions and Problems in An Adult Patient with VACTERL Association

Abstract

Abstract Background We report an adult patient with features of VACTERL - oesophageal and anal atresia, with cardiac malformation. In infancy he underwent colonic interposition for oesophageal atresia using the transverse colon as a conduit. As a teenager he underwent formation of a Koch continent ileostomy after previous stoma formation for anal atresia. Eventual failure of the Koch pouch nipple valve necessitated a laparotomy for attempted refashioning. Unfortunately, this operation was beset by life-threatening complications and a protracted post-operative course. He presented to our hospital with a laparostomy, high output enterocutaneous fistulae (ECF) necessitating home total parenteral nutrition (TPN), severe cachexia and deconditioning. Method Conservative therapy was utilised to improve the nutritional and physiological status of the patient. Simultaneously, attempts were made to define the anatomy of his abdomen in relation to previous surgery. However, operation notes for the original procedures (oesophageal reconstruction and Koch pouch formation) were lost due to closure of the hospital which he attended at the time. A laparotomy was subsequently performed to manage the ECFs. Most of the small intestine was unsalvageable due to multiple fistulae and adhesions, leaving 35cm of small bowel terminating in an end ileostomy. A cholecystectomy was performed to mitigate gallstone formation. Conclusions Efficient communication is imperative in the management of complex patients. The loss of original operation notes made interpretation of subsequent imaging and planning of surgery troublesome. In contrast, efficient communication between teams involved in his peri-operative care, particularly the respiratory and intensive care teams facilitated a smooth post-operative course and successful discharge from hospital.