A male infant (G1P1, 39 + 3 weeks; birth weight, 3,430 g) was admitted to our neonatal unit immediately after birth with intestinal obstruction. Two months ago, an ultrasound examination revealed intestinal dilatation. Half a month ago, MRI suggested fetal meconium peritonitis combined with incomplete intestinal obstruction, and ascites was significantly less than before. Both parents were 25 years old, and neither reported a family history of gastrointestinal or liver disease. There was no maternal drug abuse during the entire pregnancy. She caught a cold in the 5th month of pregnancy and recovered quickly without medication.
After admission, fasting and gastrointestinal decompression were applied. A light green fluid drainage was seen out of the gastric tube. The abdomen was slightly distended with no veins showing, and the bowel sounds were hyperactive. White blood count (WBC) was 20.65*109/L, C-reactive protein (CRP) was 0.62 mg/L; serum albumin (ALB) was 36.0 g/L, direct bilirubin (DBIL) was 5.2 mumol/L, indirect bilirubin (IBIL) was 33.1 mumol/L, alanine aminotransferase (ALT) was 17 U/L, aspartate aminotransferase (AST) was 183 U/L, gamma-glutamyltransferase (GGT) was 1,002 U/L, and total bile acids (TBA) were 8.5 mumol/L. No significant peritoneal fluid was seen on ultrasound of ascites. Liver and biliary ultrasound did not show any significant abnormalities. An upright abdominal X-ray showed signs of intestinal obstruction (Figure 1A). Emergency laparotomy revealed local intestinal adhesions and a few calcified spots. Intestinal atresia was observed at 60 cm from the ligament of Treitz. The proximal 15 cm of the hypertrophic bowel was dilated about 3-4 cm in diameter. The distal bowel was of a thin wall and 0.5 cm in diameter. The dilated 15 cm of proximal and the tiny 5 cm of distal bowel were removed, and the ileocecal part was retained. A Santullienterostomy was performed. The small bowel was measured to be 100 cm (45 cm proximal to the stoma and 55 cm distal to the stoma). Intraoperatively, no abnormal fetal stool color was found, and the gallbladder was not explored. Intraoperative diagnosis: meconium peritonitis, type IIIA intestinal atresia. An abdominal X-ray on the first postoperative day showed low bowel inflation (Figure 1B). Two days after the operation, abdominal X-ray showed a large bubble of the stomach, and hydro-aerial(HA) levels of dilated small bowel (Figure 1C). The intestine was gradually filling with air (Figure 1D). The original proximal small intestine was significantly dilated, and intestinal dynamics could not be quickly and completely restored. After surgery, the small intestine gradually inflated, and dilatation was still visible on X-rays within a short period of time. Pathology (Figures 2A,B): (ileum), the bowel is congested and edematous with localized stenosis, atresia, and calcification. A PICC tube was placed 2 days after the operation for central venous parenteral nutritional support. Jaundice was treated by phototherapy. Enteral feeding with 3 ml q3h of deeply hydrolyzed milk formula was started after 2 weeks and was gradually increased. Two weeks has passed to full feeding and gradual discontinuation of intravenous nutrition.
However, after the operation, the DBIL gradually increased, and the stool color gradually became paler from yellow-green. After the cessation of intravenous nutrition, the stool color was still white, even white clay color. Liver function index showed that the ALB was 39.1 g/L, DBIL was 149.7 mumol/L, IBIL was 104.2 mumol/L, ALT was 235 U/L, AST was 211 U/L, GGT was 610 U/L, and TBA was 135.7 mumol/L. TBA was detected by liquid chromatography-tandem mass spectrometry (LC-MS/MS) at 145.732 mumol/L, including 28.283 mumol/L for glycochenodeoxycholic acid (GCDCA) and <0.025 mumol/L for chenodeoxycholic acid (CDCA). Serum matrix metalloproteinase-7 (MMP-7) was 68.12 ng/ml. Ultrasound after 6 h of fasting showed 1.6 cm* 0.4 cm of fibrous mass echogenicity in the common hepatic duct area, internal diameter of the hepatic artery was 0.27 cm with 34.9 cm/s of a peak flow velocity, RI: 0.73, internal diameter of the portal vein trunk was 0.5 cm with 25.1 cm/s of a peak flow velocity; the size of the gallbladder was 2 cm* 0.5 cm. One h after feeding, no significant change in the size of the gallbladder was observed. The diagnosis of BA was confirmed during an intraoperative cholangiography procedure (Figure 3) at 46 days of age, and a KPE followed. Further surgical exploration revealed typically a solid, dense fibroinflammatory proximal remnant at the portahepatis, which was identified as type III biliary atresia. Intraoperatively, the enterostomy was closed, and the total length of the small intestine was measured at 130 cm. Because of the short total length of the small intestine, the jejunum was severed at 20 cm from the ligament of Treitz. The distal jejunum was anastomosed with the proximal jejunum at a distance of 30 cm from the severed end. The small intestine was dragged posteriorly over the colon to make a Roux-en-Y limb, and a hepatic portoenterostomy was performed. Pathology (Figures 2C-F): hepatocytes were swollen and bilious with ballooning and hepatic giant cell formation. Fibrosis in the confluent area (Ohkuma's Grade 2), inflammatory cell infiltration (Grade 2), small bile duct hyperplasia (Grade 2), bile emboli in the confluent area (Grade 1), and vascular hyperplasia (Grade 1) were seen. No bile lake formation was seen, and bile duct plate malformation was visible. The gallbladder was congested and edematous with fibrous hyperplasia, local stenosis and atresia, inflammatory cell infiltration, and scattered multinucleated foreign body giant cells. Branching bile ducts and nerve fibers with inflammatory cell infiltration were seen within the fibrous tissue. Feeding of deeply hydrolyzed formula was gradually started 3 days after surgery, and full enteral nutrition was established after 1 week. There were no intestinal motility problems and no episodic biliary tract infections during the hospitalization. The infant gained weight steadily and was discharged at the age of 2 months and 6 days.
The bilirubin levels reached normal 2 months after KPE. At present, the follow-up to 7 months of age revealed no cholangitis, gradual improvement of liver function index (Figure 4A), and body weight showed a catch-up trend (Figure 4B). Five months after KPE, GCDCA, and CDCA were 4.487 and 0.643 mumol/L, respectively. MMP-7 was 18.37 ng/ml. The fat-soluble vitamins were all normal. Vitamin A was 583.31 ng/ml, vitamin D was 49.76 ng/ml, vitamin E was 7.16 mug/ml, and vitamin K1 was 2.09 ng/ml. Trace elements were detected as follows: magnesium, 1.49 mmol/L; copper, 15.32 mumol/L; iron, 7.55 mmol/L; zinc, 44.89 mumol/L.