Article Text
Abstract
Introduction/Background No gut syndrome or near total enterectomy (NTE) is defined as the removal of the entire jejunum and ileum, a rarely performed procedure indicated in very specific situations and associated with parenteral nutrition (PN) dependency, multiple complications and poor quality of life in general. To date, only few cases have been reported in literature, all in the adult setting.
Aim The aim of this report is to raise awareness of this condition in paediatric patients and the difficulties encountered in their management.
Subjects and Methods Two male patients (2 and 5 year old) are presented. One secondary to an ischaemic event of unknown cause and the second post a failed small bowel transplant, both suffering complications specific to this condition.
Results Case 1: First-born to non-consanguineous parents at 37 weeks of gestation with 22 q11 deletion. Born with large VSD, had several respiratory infections initially. At 2.5 months while in PICU with a respiratory deterioration, developed intestinal infarction from duodenum to sigmoid colon and was left with D1-D2 (D2 showed patches of ischemia). A duodenal tube was left in the duodenal stump and was started on PN. A month later, a contrast study showed 2 fistulas between the duodenum and the rectum that resolved spontaneously. Currently he continues to be total PN dependent having only some water orally (thicker consistency can block the tube), has a balloon G/J tube pushed into the stomach due to a narrowed duodenal stump to try and minimise vomiting, and has issues with leakage around the tube. His liver function tests (LFTs) have been abnormal intermittently and his liver US shows hepatomegaly and gallstones.
Case 2: Third child to non-consanguineous parents born at 35 weeks of gestation with complex gastroschisis, jejunal atresia and hypoplastic colon - remaining anatomy 15 cm from GJ junction. Initial treatment involved placement/removal of a silo followed by bowel expansion, formation of a jejunostomy and a mucous fistula closed a year later when he had a jejunocolonic anastomosis. He had several interventions but was unable to tolerate minimal enteral amounts with a high stoma output despite treatment with octreotide. He underwent an isolated bowel transplant at 4 years of age followed by severe rejection not responsive to ATG/Campath and complicated with PTLD. He had a graft enterectomy with remaining anatomy 5 cm of duodenum and leakage from the duodenal stump. Currently he has a mucus fistula, an abdominal drain and a gastrostomy. Type 1 Chiari malformation was incidentally found and he developed seizures. He has intestinal failure associated liver disease and renal stones. He continues to have frequent admissions due to his health needs and frequent complications.
Summary and Conclusion Advances in PN have made possible the long-term survival of patients with no gut syndrome in specialized centres but complications, patient and family quality of life, use of health resources, medical and surgical challenges and overall outcomes are important aspects to be considered and more paediatric data is necessary.