ABSTRACT
Introduction: Congenital choledochal cyst (CCC) is a rare cystic dilatation of intrahepatic or extrahepatic biliary ducts. We present a case of a type IVb choledochal cyst presenting as recurrent acute pancreatitis in a young healthy female with initial negative screenings. Case Description/Methods: An 18-year-old-female with a history of COVID-19 presented to the emergency department with one month of persistent abdominal pain, nausea, and vomiting. She was hospitalized once prior for similar symptoms and was diagnosed with acute pancreatitis. This admission, blood work showed elevated lipase, elevated liver enzymes, mild bilirubinemia with a normal lipid panel and urine was significant for infection. She received fluids, antiemetics and was started on prophylactic antibiotics for ascending cholangitis. A right upper quadrant ultrasound ruled out cholelithiasis or acute cholecystitis, but showed dilation of the common bile duct. MRCP confirmed dilation with bulbous termination in the periampullary region diagnosed as type IVb choledochal cyst. Discussion(s): CCCs are rare in Western countries with an incidence between 1 in 100,000 to 150,000. 80% of these cysts are diagnosed in patients under the age of 10. They are difficult to diagnose due to variable clinical presentations. A study of 214 CCC patients demonstrated the most common symptom was abdominal pain, followed by jaundice and fever. When cysts are found in adults, symptoms resemble atypical acute biliary tract disease. Surgical cyst removal may be needed for patients with significant risk factors such as older age and age of symptom onset, due to increased risk of malignant transformation. Longer periods of observation have been documented to be associated with an increased chance of developing late complications, such as anastomotic stricture, biliary calculi and recurrent cholangitis. Type IVb CCCs, as seen in this case, consist of multiple extrahepatic cysts and hepaticojejunostomy is the treatment. This patient's young age and recurrent acute pancreatitis combined with her lab and imaging findings strongly suggest the diagnosis of CCC. The anatomical location of the CCC impeded flow of pancreatic enzymes through the ampulla of vater, leading to recurrent pancreatitis in an otherwise healthy young female. CCC, although very rare, should be considered in the differential of acute pancreatitis when other causes such as gallstones and heavy alcohol consumption cannot be identified, as prompt diagnosis and surgical removal is imperative.
ABSTRACT
Primary sclerosing cholangitis (PSC) is a cholestatic disorder wherein liver transplant is the definitive treatment for advance stages. However, recurrence of PSC after liver transplant is of concern which can leads to graft failure and may require retransplant. There is limited data on outcomes of living donor liver transplant (LDLT) in PSC. Also, in LDLT as donors are related there is possibility of disease recurrence. So, we conducted this retrospective study to analyse the outcomes of LDLT in PSC at a tertiary liver transplant centre in north India. Methods: We conducted a retrospective analysis of 3213 transplant recipients who underwent LDLT from January 2006 to May 2021. Of these 26 (0.80%) patients has PSC as indication for liver transplantation (PSC=24, PSC/AIH overlaP=2). Data analysis was done to look for baseline demographics, clinical details, transplant outcomes, PSC recurrence and survival. Results: Mean age of study group was 42(±13.8) years and 19 (73.1%) were males. All patients had decompensated cirrhosis at time of transplant. Mean CTP score and MELD score were 9.5(±1.8) and 18.9(±7.1) respectively. 16 patients received modified right lobe graft, 7 extended right lobe graft and 5 patients received left lateral graft. Average graft weight and GRWR were 633.5(IQR 473.5-633.5) grams and 1.23(SD±0.42) respectively. Most common biliary anastomosis was hepaticojejunostomy, done in 19(73.1%) while duct to duct anastomosis was performed in 7(26.9%) patients. Median follow- up was 96(36-123) months. One patient had ulcerative colitis and none had cholangiocarcinoma. Two (7.7%) patients had bile leak during early post-transplant period. Three (11.1%) patients developed graft rejection and managed successfully with steroid pulses. Three patients died during early post-transplant period while 7 deaths occurred during long term follow-up including one death due to COVID-19. Five (19.2%) patients had recurrence of PSC of which 2 patients lost their grafts including one after retransplantation. The overall 1 year and 5-year survival rates were 88.5% and 75% respectively. Conclusion: LDLT can be performed in PSC with good long-term outcomes with a risk of PSC recurrence in about 1/5th patients.