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A 29-year-old man presented to the hospital with complaints of abdominal pain and vomiting. Abdominal computed tomography revealed intestinal malrotation, a whirl sign of the small intestine, and occlusion of the superior mesenteric artery and vein. Due to dilation of the small intestine and poor contrast enhancement of the intestinal wall, he was diagnosed with strangulating small bowel obstruction with intestinal necrosis. Emergency surgery was performed owing to the diagnosis of small bowel volvulus with intestinal malrotation, which necessitated massive small bowel resection. The remnant small intestine was approximately 100cm in length. The patient developed short bowel syndrome postoperatively but eventually resumed a normal diet.

Short bowel syndrome is a malabsorptive condition, and long-term survival depends upon the process of intestinal adaptation. During this process, patients with short bowel syndrome frequently encounter problems such as developmental disorders, deficiency of vitamins and trace elements, liver dysfunction, catheter sepsis, etc. The treatment of these complications and surgical management, including intestinal lengthening procedures and intestinal transplantation, are described.

Isolated small bowel transplantation allows patients with irreversible small bowel failure to be weaned from total parenteral nutrition (TPN). We reported the first two cases of anesthesia for living related donor small bowel transplantation in our hospital. They had required long-term TPN. This resulted in thrombosis of the major blood vessels which critically restricted vascular access available for TPN, leading to a life-threatening condition for the patients. In patients with known difficult vascular access, performing a magnetic resonance imaging before surgery to evaluate patency of central veins seems reasonable, as well as inserting a central catheter before the surgical procedure. Graft reperfusion may cause hemodynamic instability due to changes in potassium, acid-base status as the preservative solution is flushed out of the implant. Hemodynamic, respiratory parameters and urinary output were well preserved throughout the procedure. Besides, a transitory increase in potassium following graft revascularization and biochemical changes were small. Anesthetic management requires comprehensive preoperative assessment and sufficient management without interfering with intestinal function, optimizing splanchnic perfusion to ensure implant viability.

During the past nine years period, we managed 7 cases with short bowel syndrome resulting from massive intestinal resection in the neonatal period. Six children, aged 2 to 8 years are alive, and one died from cardiomyopathy caused by selenium deficiency at the age of one year. The carbohydrate-free milk was used for initial enteral nutrition, and the result was encouraging in 4 cases. In 3 cases in which residual small bowel varied between 6.5 to 18 cm, the enteral feeding was not incapable. These cases underwent adjunctive surgical therapy. Tailoring jejunoplasty was performed in the first case, and bowel lengthening in the second case. In both cases enteral nutrition was advanced postoperatively. The third patient who had rapid intestinal transit time underwent isoperistaltic colon interposition. Intestinal transit time was increased and enteral nutrition was advanced gradually.

Despite the recent advent of total parenteral nutrition (TPN), the long-term survival of the patients with short gut syndrome is not satisfactory with complications of catheter-related sepsis and cholestasis. The causes of short gut syndrome in pediatric surgery are multiple intestinal atresias, necrotizing enterocolitis (NEC), and midgut volvulus. For the multiple atresias, multiple anastomosis without resection has been successfully undertaken to avoid developing short gut syndrome for the last 17 years. For the massive involvement of the intestines due to NEC or volvulus, high jejunostomy and peritoneal drainage with TPN support have been tried for the last two patients and successfully weaned from the TPN within two months after closure of jejunostomy. However, in case 2 malabsorption has been persistent presumably due to a severe degree of mucosal damage occurring in the remaining intestines. Whether this eventually gets back to the normal or not is unknown. For the patients with short gut syndrome, small bowel reversal procedure was successfully done and now doing well 10 years after surgery. This paper reports details of these 3 cases.