Laparoscopic cystojejunostomy for type I cystic biliary atresia in children.

PURPOSE: The use of laparoscopy in the treatment of biliary atresia (BA) is still debated. We report our strategy using laparoscopy in type I cystic BA.
MATERIALS AND METHODS: We reviewed the records of patients treated for BA from 2002-2013. When the diagnosis was suspected, an ultrasound was performed. If it showed a cyst > 5 mm in the hilum with no patent gallbladder, we performed an initial explorative laparoscopy. In the case of a patent biliary tree above the cyst, a laparoscopic cystojejunostomy was performed. In cases of absent communication (type III), conversion and portoenterostomy were performed. Pre and postoperative data and overall survival rate with the native liver were reviewed.
RESULTS: Forty-four children were treated for BA. Six presented with a cystic form diagnosed by US. Three children had type I BA; three had type III BA. No postoperative complications were noted. Median follow-up was 62.2 months (22.7-93.5). One patient died of a cardiac malformation. The five remaining patients are alive with their native liver. Of the 38 treated for noncystic BA, 16 were transplanted.
CONCLUSION: We confirmed the prognosis of cystic BA, which is less severe than noncystic BA. Our strategy using laparoscopy allowed for the confirmation and qualification of the type of BA. In type I, complete treatment by laparoscopy has been performed safely.

INTRODUCTION

Biliary atresia (BA) is a progressive obliterative sclerosing disease of the extra and intrahepatic biliary system, which occurs in approximately 1 in 8000 to 1 in 18,000 live births. Three types of clinical forms are usually described according the most proximal level of obstruction. Despite an early diagnosis and prompt surgical treatment, the disease progresses to an irreversible cirrhotic stage in many patients. Kasai portoenterostomy is the treatment of choice for BA since its introduction in 1959.

The clinical outcome for children who undergo hepatic portoenterostomy is variable; 45% of patients will have successful long-term survival. The remaining 55% will need early liver transplantation within the first year or will progress to cirrhosis with the need of transplantation.[1] Cystic BA is a prognostic subgroup that previously has been shown to have a better prognosis.[2]

Amidst many controversies, laparoscopic Kasai portoenterostomy for BA in the pediatric population has been performed by a limited number of teams with clinical outcomes and long-term results comparable to the standard technique.[34567] Chan et al.[8] found the 5-year native liver survival rate and the jaundice-free native liver survival rate at 5 years after a laparoscopic procedure to be 50%, which is comparable to other series of patients who underwent open Kasai portoenterostomy. No study has reported the use of laparoscopic cystojejunostomy in cystic BA. We assessed our experience with laparoscopic cystojejunostomy for type I cystic BA in children.

MATERIALS AND METHODS

We retrospectively identified the records of infants diagnosed with cystic BA between 2002 and 2013 in our hospital's clinical database. Patients’ gender and age at time of surgery were reviewed. Data from antenatal ultrasonography were also noted. The diagnosis was suspected based on the combination of neonatal jaundice and persistent acholic stools. Then abdominal ultrasonography was systematically performed. If the ultrasound showed a cyst >5 mm in the hilum with no patent gallbladder [Figure 1], our surgical strategy consisted of an initial laparoscopy with abdominal cavity examination that confirmed the cholestasis liver and an atretic gallbladder. The hepatic hilum was carefully dissected to identify the cyst at the porta hepatis [Figure 2]. A direct cyst puncture cholangiogram was then performed. Communication of the cyst with intrahepatic bile ducts with apparent bile drainage corroborated the diagnosis of ‘correctable’ BA and a type I cyst, according to the Japanese classification of BA.[9] A cystojejunostomy was then performed laparoscopically [Figures 3 and 4].[10]

Figure 1

Ultrasonography (A) in a female newborn showing a cyst at the porta hepatis with no patent gallbladder

Figure 2

Operative view: Dissected area of the portal plate by laparoscopy. The hepatic artery was dissected to expose the cyst (*)

Figure 3

Operative view: Cyst incision with bile drainage

Figure 4

Operative view: Cystojejunostomy was created using interrupted 5-0 polygloctin suture

Conversely, the cystic dilatation of the common bile duct with clear fluid and no opacification of the intrahepatic biliary tree were classified as type III. In this case, a conversion to laparotomy procedure and a hepatoportoenterostomy was performed. The technique of laparoscopic cystojejunostomy was similar to our choledochal cyst laparoscopic technique previously described.[11] The umbilical incision was extended to 1.5 cm to exteriorize the jejunum. After identifying the ligament of Treitz laparoscopically, the proximal jejunum, 40 cm distal to the ligament, was localized and exteriorized through umbilical trocar. Roux-en-Y jejunojejunostomy with one-layer interrupted sutures was carried out manually. The closed jejunum limb was lifted through a mesocolon incision on the right side. The cyst was partially resected using laparoscopic ultrasonic coagulating shears and a monopolar Hook to apparently normal common hepatic duct. The cystojejunostomy was created using interrupted 5-0 polygloctin sutures. The laparoscopic procedure was completed with a hepatic biopsy in each of case to assess the BA diagnosis. The operative time and blood loss were noted.

Pre and postoperative complications, length of stay in the hospital, and the jaundice clearance were reviewed and overall survival rate with native liver were noted.

Continuous variables are presented as averages ± the standard deviation (SD) or as medians with the 25th and 75th percentiles. Qualitative variables are presented as total numbers and percentages.

RESULTS

A total of 44 infants with BA were treated during the study period at our centre. Six patients (13.6%) had a cyst assessed by preoperative ultrasound (five females and one male). Cyst at the hepatic hilum was identified by antenatal sonography in two cases. The median age at surgery for these six infants was 57 days (ranges 19-153 days). Children with a cyst detected during the antenatal period underwent surgery sooner (at 20 and 25 days). There were three infants with type I BA with an extrahepatic cyst containing apparent bile and three infants with type III BA with a noncommunicating cyst. For the three patients who underwent laparoscopic cystojejunostomy, the mean operative time 265 min (range 210-300 min). No blood transfusion was needed per or postoperatively.

Median hospitalization period was 16 days (range 10-21 days). There were no conversions to open procedure and no intraoperative or postoperative complications. One child had cholangitis in the early postoperative period but responded to antibiotics.

Of the three children with type III BA, two had extrahepatic anomalies consistent with syndromic BA. Mean operative time was 217.5 min (range 190-300 min). No postoperative complications occurred.

The median follow-up time was 62.2 months (range 22.7-93.5 months). One child with type I BA had concomitant cardiac malformation and died early after surgery. Three infants had persistent jaundice (one type I, two type III), and one child had portal hypertension with esophageal varices (type III). All five children did not required liver transplantation.

During the same study period, 38 infants (86.3%) with noncystic BA received a Kasai portoenterostomy with a median age at surgery of 86 days (range 20-109 days). Sixteen infants (50%) required liver transplantation at a median age of 20 months (range 10-60 months). Median time between the Kasai procedure and liver transplantation was 18 months (range 8-55 months). Four children died before liver transplantation.

DISCUSSION

Kasai hepatoportoenterostomy is the gold standard in the treatment of BA. In an era of minimally invasive techniques, surgeons continue to approach complex laparoscopic procedures with hesitancy. An argument against the use of laparoscopy is the difficulty in overcoming the learning curve of the technique given the rarity of its application in the treatment of BA.[7] Secondly, due to inherent limitations in the movement of laparoscopic instruments, poor portal exposure has been accused of being responsible for inferior results after laparoscopic Kasai. In an animal study,[12] it was postulated that prolonged pneumoperitoneum had detrimental effects on livers with BA and may explain why the outcome was less favourable. Nevertheless, Chan et al.'s[8] experience with laparoscopic Kasai reported results comparable to those from the largest published series investigating Kasai by laparotomy. Results were comparable in terms of early biliary drainage and native liver survival rate over 5 years. With regard to postoperative complications in their laparoscopic group, one patient had intestinal volvulus and required combined liver and intestine transplantation; in the laparotomy group, one patient had intestinal obstruction and required an additional laparotomy and adhesiolysis. As in choledochal cyst excision by laparotomy, there is a significant adhesive intestinal obstruction rate following Kasai by laparotomy. Attempts to reduce this morbidity may support the use of the laparoscopic approach.

Aside from its feasibility and safety, the potential benefits of a laparoscopic procedure for BA include the clinical impression that children treated with laparoscopically have better early outcomes than those who underwent Kasai by laparotomy.[13] The avoidance of large-muscle incisions, resulting in quicker recovery after surgery and shorter hospital stays have been often reported. In addition, limiting liver mobilization outside the abdominal cavity has been suggested to decrease intra-abdominal adhesion, which facilitates hepatectomy in the case of liver transplantation.[13]

Patients’ age at first surgery markedly affected the outcome of BA. Caponcelli et al.[2] suggested that the correlation between age and the degree of fibrosis was stronger in patients with cystic BA than in patients with noncystic BA.

Despite the retrospective design of this study and the small sample examined, we remain in favor of early laparoscopic exploration in the case of biliary tract cysts detected by sonography in the fetus. In the case of cystic BA with communication with proximal bile ducts and patent bile drainage at the direct cyst puncture, referred to as the ‘correctable’ variant of BA, we recommend laparoscopic cystojejunostomy. However, in ‘noncommunicating’ cysts, the decision to use laparoscopy is left to experienced surgeons. Nevertheless, a study has found a benefit of Kasai by laparotomy over Kasai by laparoscopy in terms of native liver survival rate.[14]

Our study assessed the use of laparoscopy in treating cystic BA and found it to be an effective treatment. Our results confirm previous findings on the feasibility of laparoscopic hepaticojejunostomy.

CONCLUSION

BA is a rare and serious pediatric condition that requires early and adequate surgical management. We confirmed the prognosis of cystic forms of type I BA with a 5-year overall native liver survival rate of 83%. Our strategy using laparoscopy in cases of cystic BA allowed for the confirmation and qualification of the type of BA. In cases of type I, complete surgical treatment by laparoscopy was performed. Given comparable native liver survival and much better early outcomes, laparoscopic cystojejunostomy should be performed whenever possible but only in specialized centers. Further investigations of the laparoscopic approach for other forms of BA are still needed.