Giant bile duct dilatation in newborn: A case report

Abstract

BACKGROUND

Giant congenital biliary dilation (CBD) is a rare condition observed in clinical practice. Infants born with this condition often experience a poor overall health status, and the disease progresses rapidly, leading to severe biliary obstruction, infections, pressure exerted by the enlarged CBD on abdominal organs, disturbances in the internal environment, and multiple organ dysfunction. The treatment of giant CBD using laparoscopy is challenging due to the high degree of variation in the shape of the bile duct and other organs, making it difficult to separate the bile duct wall from adjacent tissues or to control bleeding.

CASE SUMMARY

Herein, we present the details of an 11-d-old male newborn who was diagnosed with giant CBD. The patient was admitted to the neonatal surgery department of our hospital due to a history of common bile duct cyst that was detected more than 3 mo ago, and also because the patient had been experiencing yellowish skin for the past 9 d. The abnormal echo in the fetal abdomen was first noticed by the patient’s mother during a routine ultrasound examination at a local hospital, when the patient was at 24 wk + 6 d of pregnancy. This finding raised concerns about the possibility of congenital biliary dilatation (22 mm × 21 mm). Subsequent ultrasound examinations at different hospitals consistently confirmed the presence of a congenital biliary dilatation. No specific treatment was administered for biliary dilatation during this period. A computed tomography scan conducted during the hospitalization revealed a large cystic mass in the right upper quadrant and pelvis, measuring approximately 9.2 cm × 7.4 cm × 11.3 cm. Based on the scan, it was classified as a type I biliary dilatation.

CONCLUSION

The analysis reveals that prenatal imaging techniques, such as ultrasound and magnetic resonance imaging, play a crucial role in the early diagnosis, fetal prognosis, and treatment plan for giant CBD. Laparoscopic surgery for giant CBD presents certain challenges, including difficulties in separating the cyst wall, anastomosis, and hemostasis, as well as severe biliary system infection and ulceration. Consequently, there is a high likelihood of converting to laparotomy. The choice between surgical methods like hepaticojejunostomy (HJ) or hepaticoduodenostomy has not been standardized yet. However, we have achieved favorable outcomes using HJ. Preoperative management of inflammation, biliary drainage, liver function protection, and supportive treatment are particularly vital in improving children’s prognosis. After discharge, it is essential to conduct timely reexamination and close follow-up to identify potential complications.

Keywords: Acute cholangitis; Congenital biliary dilation; Laparoscopic surgery vs Open hepatic duct-jejunostomy; Case report

Core Tip: Clinically, giant congenital biliary dilation (CBD) is a rare condition compared to ordinary CBD. There are numerous research articles discussing surgical treatment options for common CBD. The choice of specific surgical method should be based on the individual circumstances of the hospital, the child, and the family members. In our case report, we present the experience of treating a very young child (11 d old) with a significantly dilated biliary tract. The overall condition of the child before the operation was critical. During laparoscopic biliary reconstruction, we encountered a thin and ulcerated cyst wall, making separation and bleeding control challenging. Therefore, we opted for an open laparotomy and performed a Roux-en-Y anastomosis between the common hepatic duct and jejunum. Taking into consideration the analysis of similar cases indexed in PubMed, we suggest that open surgery is more advantageous than laparoscopic treatment for children with giant CBD who are in critical condition and have severe biliary tract distortion.