Management of Choledocholithiasis in a 5-Month-Old Male Postprimary Repair of Esophageal Atresia with Tracheoesophageal Fistula.

Choledocholithiasis (CDL) in neonates and infants is <1 in 5000. There is no gold standard for the management of CDL in the pediatric population. In English literature, this is the first case report of a baby born with esophageal atresia and tracheoesophageal fistula (Type C) with cholangitis, who underwent percutaneous transhepatic biliary drainage and peroral extraction of a large calculus in the common bile duct after primary repair of esophagus. Copyright:

INTRODUCTION

The incidence of choledocholithiasis (CDL) in neonates and infants is <1 in 5000.[1] There is no gold standard for the management of CDL in the pediatric population. In pediatrics, endoscopic retrograde cholangiopancreatography (ERCP) is considered safe and effective, and in centers where it is done routinely, it is often employed as the first option.[2] Likewise, laparoscopic CBD exploration has also been proven to be safe and is an appropriate alternative, especially in locations where pediatric ERCP is not available. Percutaneous transhepatic removal of the bile duct stones is feasible when the endoscopic procedure is unavailable or fails.[3] In English literature, this is the first reported case of a baby with esophageal atresia and tracheoesophageal fistula (Type C) who underwent primary repair of the esophagus, who developed a large calculus in the CBD with cholangitis, and who underwent percutaneous transhepatic biliary drainage (PTBD) and peroral extraction of the stone.

CASE REPORT

A male baby on day 1 of life born term and normal weight with antenatal history of polyhydramnios was admitted with drooling of saliva and increased respiratory rate since birth. Baby developed respiratory distress at birth and was intubated and referred to our center. On attempting nasogastric tube insertion, the tube coiled in the upper pouch of esophagus. Two-dimensional echocardiography showed a patent foramen ovale and small patent ductus arteriosus. On day 2 of life, the baby underwent right posterolateral thoracotomy. Intraoperatively, there was a gap of 2 cm between upper and lower esophagus. The upper esophagus was blind while the lower esophagus had a fistula to the trachea (Type C). Ligation of the tracheoesophageal fistula with primary end-to-end esophageal anastomosis was done.

Postoperative course of the baby was stormy, but the baby recovered and was discharged on day 18 on full oral feeds. The barium swallow showed free flow of contrast into the stomach. This baby received cephalosporins in the neonatal period postsurgery. Total parenteral nutrition was not given to this baby. At 5 months of age (weight 4.76 kg), the baby developed obstructive jaundice with clay-colored stools and icterus. The baby also had cholangitis and hypotension and was nutritionally depleted. The liver function test showed total bilirubin – 2.5, direct bilirubin – 2.2, serum glutamic-oxaloacetic transaminase – 105, serum glutamic pyruvic transaminase – 111, albumin – 4.2, and gamma-glutamyl transferase – 1306. IgG Cytomegalovirus was positive. Ultrasound abdomen was suggestive of CDL. Magnetic resonance cholangiopancreatography revealed a dilated CBD measuring 6.1 mm in caliber with a calculus of 4.5 mm diameter in the distal end of the CBD [Figure 1]. There was a smooth narrowing seen along the distal most aspect of the CBD approximately 6 mm in length, suggesting a distal CBD stricture. Ursodeoxycholic acid was administered before the endoscopic procedure, but the CDL persisted. PTBD was done under ultrasonography and fluoroscopy guidance by puncturing segment III duct and internalization using a 6 Fr catheter. Cholangiogram showed a filling defect in the distal CBD suggestive of a calculus. The baby had severe dyselectrolytemia (Na – 119, K+ – 8.2) and was shifted to pediatric intensive care unit and given supportive care. After 4 days, the electrolytes improved and the child was taken up for intraductal stone removal. PTBD access was used to pass the wire catheter combination into the duodenum and negotiated to the fundus of the stomach. Wire catheter combination used to secure a sheath, perorally into the stomach. Through the sheath, a snare was passed to catch the wire in the stomach and snared out the calculus perorally. The ampulla was dilated using a 4-mm Fogarty balloon, an occlusive balloon was passed orally into the CBD, and the calculus was swept into the duodenum. Check cholangiogram revealed no filling defect in the CBD [Figure 2]. An 8.2 Fr PTBD external drainage catheter was secured and connected to an external bag. The drain was clamped after 3 days and then removed. The child tolerated the procedure well and was discharged in a stable condition. Fusiform choledochal cyst appearance was suspected initially; however, on follow-up, the ultrasound showed a normal common bile duct (CBD). At present, the child is 5 years old and well.

Figure 1

Magnetic resonance cholangiopancreatography image showing choledocholithiasis

Figure 2

Postextraction of common bile duct calculus cholangiogram – no calculus

DISCUSSION

We present here a unique case scenario where the baby was born with esophageal atresia and tracheoesophageal fistula (Type C) who underwent ligation of tracheoesophageal fistula and primary end-to-end anastomosis of the esophagus at day 2 of life and recovered uneventfully. The baby then developed obstructive jaundice due to CDL, where endoscopic stone extraction was technically challenging, in view of a repaired esophagus and a small baby. He underwent PTBD and peroral extraction of the stone. In 2008, Kalfa et al.[4] described a unique management technique using a combined percutaneous and endoscopic approach in a 10-week-old infant with CDL. The infant first underwent percutaneous transhepatic cholangiography with biliary drainage. After 3 days of saline washing, the stone remained impacted in the distal CBD. A guide wire was passed through the existing transhepatic tube and snared endoscopically. This allowed for transoral anterograde sphincteroplasty and balloon extraction of the stone.

Open or laparoscopic CBD exploration is the accepted method for removal of bile duct stones in children.[5] The success rate of laparoscopic CBD exploration is between 60% and 87.5%, with 0%–30% complication rates.[5] Stone extraction may be performed endoscopically with or without sphincterotomy; ERCP has 95%–100% success rate and 3.2%–7.7% complication rate. However, pediatric ERCP is not always available in all centers.[2] Özcan et al.[3] reported the management of 12 children with CBD stones causing biliary obstruction. Complete clearance of the bile duct was achieved with symptomatic improvement by percutaneous stone expulsion into the duodenum in all cases. They concluded that percutaneous stone expulsion into the duodenum is a safe and effective method for the treatment of children with biliary stone disease.

Successful management of the baby who had major thoracic surgery at birth followed by an endoscopic removal of CBD stone has been presented here to highlight the uniqueness of this case. The baby is now 5 years old and growing well.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.