Gall Bladder Duplication with Choledochal Cyst: A Rare Entity
Department of Paediatric Surgery, Lilavati Hospital and Research Centre, Mumbai, Maharashtra, India
Address for correspondence: Dr. Rahul Deo Sharma, Department of Paediatric Surgery, Lilavati Hospital and Research Centre, Bandra West, Mumbai - 400 050, Maharashtra, India. E-mail: dr.rdeosharma@gmail.comAbstract
A 4-year-old male child presented with complaints of abdominal pain and vomiting along with yellowish discoloration of the eyes. Investigations were suggestive of acute pancreatitis and double gall bladder (GB) with dilated common bile duct (CBD) with intraluminal calculi and Type II choledochal cyst. He underwent surgical resection of double GB with dilated CBD with hepatico-docho-jejunostomy. On follow-up, the patient was asymptomatic. Our case highlights the importance of preoperative diagnosis to deal with increased operative difficulty and complications.
INTRODUCTION
Duplication of the gall bladder (GB) is an uncommon congenital abnormality with an incidence of about 1 in 3–4000 cases, first reported by Boyden in 1926.[1] Less than 5% of reported cases of GB duplication occur in the pediatric population,[2] and no case reports have detailed an anatomic variant of a double GB with a choledochal cyst in a pediatric patient.
Various classifications have been proposed for double GBs. The most common classification is based on whether there is a common cystic duct or two separate cystic ducts.[3] Congenital GB duplication can be detected on antenatal ultrasound; however, most cases get detected when cholecystitis is present. Duplication of the GB can be seen on radiological investigations such as ultrasound, computed tomography, or magnetic resonance imaging. Preoperative delineation of double GB is important. As this may be associated with the increased complexity of biliary anatomy, it may lead to higher rates of complications. Preoperative magnetic resonance cholangiopancreatography (MRCP) imaging can help to delineate the anatomy of the biliary system.
CASE REPORT
A 4 year and 3-month-old male child presented with complaints of nonradiating upper abdominal colicky pain, associated with fever and nonbilious vomiting for 24 h. There was yellowish discoloration of skin and eyes for the past 2 months. A history of similar episodes of abdominal pain was present 1 year ago, where an ultrasonography abdomen had detected the presence of a double GB without cholecystitis.
On admission, blood investigations were reported as follows: serum amylase – 544, serum lipase – 2263, partial thromboplastin time – 22.2 s, international normalized ratio – 1.14, liver function test – normal, hemoglobin – 11.3 g/dl, total leukocyte count – 8240, and platelet – 1.07 lakh, suggestive of acute pancreatitis, which was managed conservatively. MRCP showed duplication of GB with two separate cystic ducts and dilated common bile duct (CBD) with intraductal calculi. The long dilated common channel presents with 12-mm intraluminal calculus with a saccular outpouching of 12 mm × 11 mm from anteromedial aspect of the common channel, which was suggestive of Type II choledochal cyst [Figure 1]. The prominence of intrahepatic ducts was seen. Peripancreatic T2 hyperintense signal was suggestive of edema/fluid. Peripancreatic, pericolic, pericaval, and perihilar enlarged lymph nodes were also present. At endoscopic retrograde cholangiopancreatography, it was not possible to cannulate the pancreatic duct. On exploration, an intraoperative cholangiogram showed double GB with one cystic duct draining into the dilated common hepatic duct (CHD) [Figure 2]. Double GB excision with excision of dilated bile duct till the head of the pancreas was carried out [Figure 3]. On choledochoscopy, stones were visualized, which were flushed out. Hepatico-docho-jejunostomy along with Roux-en-Y end-to-side jejunojejunostomy was performed. A broad-based Meckel's diverticulum and a splenunculus were noted and left untouched. Postoperative period was uneventful.
DISCUSSION
Double GB and choledochal cysts are rare congenital biliary anomalies, with an incidence of approximately 1 in 4000 and 1 in 100,000 people.[4] Double GB is thought to develop from either a single primordia, which divides around the 2nd month of gestation, or from two separate primordium.[4]
Harlaftis's classification of double GBs categorizes anatomical variations based on embryological origin [Figure 4].
Double GBs can be found incidentally during surgery because accessory GBs are mostly overlooked in preoperative imaging analysis. Double GBs do not need to be removed prophylactic in the case of asymptomatic patients.
The accessory GB has to be differentiated from other biliary anomalies, mainly Type II choledochal cysts, because different anomalies require different procedures.
In 1977, Todani et al. described five categories of congenital choledochal cysts.[5] Types I and II occur more commonly, presenting as saccular dilation and diverticula of the CBD, respectively, while Type III choledochal cyst also known as choledochocele arises from dilatation of the duodenal part of CBD.
Double GBs and choledochal cysts are different but similar entities, preoperatively differentiation in between the two, will help the surgeon's operative planning and management. For double GB, only excision is enough but choledochal cyst needs anastomosis to CBD with either duodenum or jejunum. To differentiate between the two, MRCP plays an important role. MRCP can show the two GBs with two cystic ducts draining into CHD and it can also show the anomalous pancreaticobiliary channel. The presence of anomalous pancreaticobiliary junction with the origin of the cyst from CBD favors the diagnosis of choledochal cyst while two cystic ducts or two GBs draining into CBD through one common cystic duct favor the diagnosis of double GB. In our case, both double GB and anomalous pancreaticobiliary junction with cyst with CBD were present, which is been diagnosed as a rare case of double GB with choledochal cyst.
The histopathology report only confirms the final diagnosis. In our case, the histopathology report shows two GBs with lymphoplasmacytic infiltrates in both GBs and the two GBs had two separate lumens separated by a common wall and the choledochal cyst was arising from CBD with cyst wall showing fibrocollagenous tissue and lined by columnar epithelium which at other places, is denuded suggestive of Type III choledochal cyst.
Because choledochal cysts have a malignant potential, they should be surgically excised once the diagnosis is confirmed. In our case, we did double GB cholecystectomy and traced the dilated CBD up to pancreatic head and excised and hepatico-docho-jejunostomy was done. Although no standard guidelines exist for this problem, we recommend resection of double GB and choledochal cyst with a Roux-en-Y hepatico-docho-jejunostomy and end-to-side jejunojejunostomy.
CONCLUSION
We report a rare case of GB duplication with the common pancreaticobiliary channel with a Type III choledochal cyst, Meckel's diverticulum, and splenunculus.
Our case highlights the importance of preoperative diagnosis which helps to manage such cases to avoid complications.
MRCP is a useful tool to delineate the anatomy in complex biliary anomalies. Cholecystectomy with disconnection of the biliary system from the pancreatic system helps in the resolution of such anomalies.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that their name and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
There are no conflicts of interest.