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Choledochal Cyst in the Context of Sickle Cell Disease: A Case Report
Pankaj Gharde1, Pramita M Gharde2, Meenakshi Yeola Pate3
1Surgery, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education and Research, Wardha, IND.
Insights
Choledochal cysts, a congenital anomaly, can mimic sickle cell disease symptoms. This rare case highlights the importance of thorough evaluation for accurate diagnosis and treatment of choledochal cysts.
Area of Science:
- Hepatology and Gastroenterology
- Congenital Disorders
- Surgical Pathology
Background:
- Choledochal cysts are rare congenital biliary tract anomalies.
- Symptoms like abdominal pain and jaundice can overlap with other conditions, including sickle cell disease.
- Early detection and accurate diagnosis are crucial for effective management.
Observation:
- A 30-year-old male with a history of sickle cell anemia presented with recurrent abdominal pain, nausea, and vomiting.
- Ultrasonography revealed a dilated common bile duct and intrahepatic biliary radicals, consistent with a choledochal cyst.
- The overlapping symptoms presented a diagnostic challenge.
Findings:
- Magnetic resonance cholangiopancreatography (MRCP) and endoscopic retrograde cholangiopancreatography (ERCP) are key diagnostic tools.
- ERCP can be therapeutic for common bile duct calculus removal and stenting.
- Surgical management typically involves total cyst excision, often with Roux-en-Y hepatico-jejunostomy.
Implications:
- This case underscores the need for comprehensive evaluation in patients with overlapping symptoms of congenital anomalies and chronic diseases.
- Accurate diagnosis through advanced imaging like MRCP is vital.
- Roux-en-Y hepatico-jejunostomy with cyst excision is the preferred surgical approach for choledochal cysts.
Abstract:
Choledochal cyst is a congenital pathology with an uncommon anomaly associated with common complaints of an abdominal lump and hepatic dysfunction. It may be presented equally in any phase of life, be it childhood, adolescence, or adulthood, and is majorly detected by ultrasonography (USG) on the appearance of primary symptoms in the hepato-biliary system. It has a classical triad consisting of a lump in the upper quadrant on the right side of the abdomen, pain in the upper part of the abdomen, and obstructive jaundice. A few of the clinical features overlap with sickle cell disease. A 30-year-old male patient with sickle cell anemia was diagnosed eight years ago. The patient was diagnosed with a choledochal cyst with the clinical presentation of abdominal pain, nausea, and vomiting, which hampered his routine life. Due to symptomatic recurrence, the patient was subjected to USG (abdomen), which showed a dilated common bile duct (CBD) and dilated intrahepatic biliary radicals. This is a rare case presentation with both sickle cell disease and choledochal cyst, which are symptomatically similar. Based on history, risk factor analysis, and diagnostic findings, the patient was advised to have a Roux-en-Y hepatico-jejunostomy. Endoscopic retrograde cholangiopancreatography (ERCP) and magnetic resonance cholangiopancreatography (MRCP) are the investigations of choice, with the better being MRCP. ERCP is a therapeutic and diagnostic modality that helps in the removal of CBD calculus and the placement of a stent. There may be increased bilirubin, showing features of obstructive jaundice in alcoholic stools. In surgical management, which is of total excision of the cyst, there are vital structures in proximity. The patients with these complaints need to be evaluated thoroughly, and detailed clinical examination and proper radiological investigations will be performed. Roux-en-Y hepatico-jejunostomy with cyst excision in toto is the procedure of choice.
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