Unconventional treatment of neuroenteric cyst in a newborn

Neuroenteric cysts are uncommon congenital malformations that can require early surgical treatment. The authors report on an unusual treatment of a very large neuroenteric cyst that involved most of the small bowel and extended into the chest. A 1-day-old boy was admitted because of abdominal distension. The prenatal ultrasound results at 8 and 36 weeks had been normal. Examination showed right upper quadrant fullness and mild respiratory distress. A malformed sternum and asymmetric upper thoracic vertebra were seen on the initial x-rays. The possibility of a midthoracic right paravertebral mass was raised. Abdominal ultrasound findings were consistent with a large bowel duplication cyst. Laboratory results were all normal except the bilirubin level, which was (59 mmol/L). During laparotomy, the second part of the duodenum was found to enter a dilated cyst, and the terminal ileum arose from the cyst. The total length of the intact small bowel was 20 cm including a competent ileocecal valve. The site of the biliary duct entering the cyst was not clear. The surgical procedure involved partial resection of the anterior wall of the cyst, creation of an enteric tube from the posterior cyst wall to communicate between the duodenum and the ileum, and addition of another 25 cm of length to the small bowel. An enterocutaneous fistula was created with the proximal portion of the cyst because the site of the papilla of Vater was suspected to enter this part of the cyst. A postoperative HIDA scan showed good uptake with no excretion into the gut or the proximal pouch. Fistulography showed a blind loop herniating to the posterior midthorax, with no communication to the biliary tree. Magnetic resonance imaging showed no communication of the pouch with the spinal cord, and a technetium 99 scan showed gastric mucosa in the pouch as well as in the created enteric tube. A percutaneous transhepatic cholangiogram (PTC) showed communication of the common bile duct with the duodenum. The patient underwent surgery again, and the abdominothoracic portion of the neuroenteric cyst was excised. Omeprazol was administered, and enteral and parenteral nutrition were begun using predigested formula. At 8 months of age, the parenteral supplementation was discontinued. This unconventional surgical treatment of neuroenteric cyst helped prevent the severe complications of prolonged home total parenteral nutrition. Resection of the gastrin-producing enteric tube will be performed in the future.