Neuroenteric cysts are uncommon congenital malformations that can requ
ire early surgical treatment. The authors report on an unusual treatme
nt of a very large neuroenteric cyst that involved most of the small b
owel 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 fullnes
s and mild respiratory distress. A malformed sternum and asymmetric up
per thoracic vertebra were seen on the initial x-rays. The possibility
of a midthoracic right paravertebral mass was raised. Abdominal ultra
sound findings were consistent with a large bowel duplication cyst. La
boratory results were all normal except the bilirubin level, which was
(59 mmol/L). During laparotomy, the second part of the duodenum was f
ound to enter a dilated cyst, and the terminal ileum arose from the cy
st. The total length of the intact small bowel was 20 cm including a c
ompetent ileocecal valve. The site of the biliary duct entering the cy
st was not clear. The surgical procedure involved partial resection of
the anterior wall of the cyst, creation of an enteric tube from the p
osterior cyst wall to communicate between the duodenum and the ileum,
and addition of another 25 cm of length to the small bowel. An enteroc
utaneous fistula was created with the proximal portion of the cyst bec
ause 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 exc
retion into the gut or the proximal pouch. Fistulography showed a blin
d 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 gas
tric mucosa in the pouch as well as in the created enteric tube. A per
cutaneous transhepatic cholangiogram (PTC) showed communication of the
common bile duct with the duodenum. The patient underwent surgery aga
in, and the abdominothoracic portion of the neuroenteric cyst was exci
sed. Omeprazol was administered, and enteral and parenteral nutrition
were begun using predigested formula. At 8 months of age, the parenter
al supplementation was discontinued. This unconventional surgical trea
tment of neuroenteric cyst helped prevent the severe complications of
prolonged home total parenteral nutrition. Resection of the gastrin-pr
oducing enteric tube will be performed in the future. Copyright (C) 19
95 by W.B. Saunders Company