Ryo Ataka1, Narito Takada2, Akane Sekiya3, Ryuichiro Doi1. 1. Department of Surgery, Otsu Red Cross Hospital, Shiga, Japan. 2. Department of Pediatric Surgery, Otsu Red Cross Hospital, Shiga, Japan. 3. Department of Neonatology, Otsu Red Cross Hospital, Shiga, Japan.
A 2-day-old girl, born to a healthy 34-year-old woman at the 36th week of gestation, was transferred to our hospital after she presented with bilious emesis. A gastrointestinal x-ray series with contrast revealed microcolon and ileal obstruction (Figure 1). Our differential diagnosis for the neonatal small bowel obstruction included ileal atresia, intestinal malrotation, meconium plug syndrome, and Hirschsprung disease.
Figure 1.
Gastrointestinal x-ray series images with contrast at admission. (A) Contrast enema showing microcolon. (B) Contrast injected through the gastric tube shows ileal obstruction (arrows).
Gastrointestinal x-ray series images with contrast at admission. (A) Contrast enema showing microcolon. (B) Contrast injected through the gastric tube shows ileal obstruction (arrows).She underwent exploratory laparoscopy, which revealed no anatomical anomaly, although the small intestine was filled with meconium. At 100 cm from the terminal ileum, the color of meconium, as seen through the intestinal wall, changed from white to green. The color change was even more distinct when the intestine was incised, creating a sharp contrast (Figure 2). Moreover, the proximal jejunum demonstrated a caliber change 35 cm distal to the ligament of Treitz, and its full-thickness biopsy revealed near total intestinal aganglionosis, highly suggestive of extensive type of Hirschsprung disease. Hence, we performed diverting jejunostomy 1 week after the previous operation, and she is currently gradually growing with parenteral and enteral nutrition support.
Figure 2.
(A) The intestine showing an obvious change in color of its contents (forceps). (B) Color transition of meconium indicating the moment when bile began to flow into the duodenum.
(A) The intestine showing an obvious change in color of its contents (forceps). (B) Color transition of meconium indicating the moment when bile began to flow into the duodenum.Hirschsprung disease is a congenital disorder characterized by complete absence of neuronal ganglion cells at the end of the large intestine, with continuous involvement of the proximal intestine. In less than 1% of patients, aganglionosis extends through nearly the entire intestine, and, although rare, such extensive aganglionosis can be a cause of neonatal small bowel obstruction.[1]In the present case, the impressive color transition of meconium was representative of the precise moment when bile had begun to flow into the duodenum during the embryonic period. A hepatic diverticulum appears from the primitive midgut in the fourth week of gestation. The biliary system starts forming in the fifth week.[2,3] The common bile duct elongates with its lumen plugged by epithelial cells. Recanalization of its lumen starts at the end of the fifth week.[2,4] By the 13th week, bile flows through the extrahepatic biliary tree into the duodenum.[2,5] It is presumed that the color transition in the present case was distinct because ileal aganglionosis and poor peristalsis resulted in inadequate mixing of meconium.
DISCLOSURES
Author contributions: R. Ataka wrote the manuscript. N. Takada and A. Sekiya revised the manuscript for intellectual content and approved the final manuscript. R. Doi is the article guarantor.Financial disclosure: None to report.Informed consent was obtained for this case report.
Figure 1.
Figure 2.