Grand Rounds

Caroli's disease in infancy - A report of 2 cases


Nupur Sanklecha1, Aashna Doshi1, Ira Shah2
1Seth G. S. Medical College and KEM. Hospital, Mumbai, India, 2Pediatric Gastroenterology, V Care Polyclinic, Mumbai, India

Address for Correspondence: Dr Nupur Sanklecha, Seth G. S. Medical College and KEM. Hospital, Mumbai, India. Email: nupursanklecha@gmail.com


Keywords: Caroli's disease, infancy

Clinical Problem:
A 9-month-old boy born of non consanguineous marriage presented with progressive abdominal distension with prominent abdominal veins since 2 months of age. He was born at full term and had achieved milestones appropriately for age. On examination, he was malnourished (weight = 5 kg, less than 5th centile; height = 64 cm, less than 5th centile), had tachycardia (heart rate = 140/min) respiratory rate of 42/min and blood pressure of 92/60 mm of Hg. He had pallor, palmar erythema and splenohepatomegaly with dilated abdominal veins. Investigations showed hemoglobin of 8.1 gm%, WBC count of 25,400/cumm and platelet count of 4,05,000/cumm. Liver function tests are depicted in Table 1. USG abdomen showed multiple round hypoechoic areas in the liver with collaterals around liver capsule and splenomegaly. A CT abdomen showed polycystic kidneys with dilated cysts in liver (Figure 1). Serum creatinine and urine examination was normal. Thus the child was diagnosed as Caroli’s syndrome with portal hypertension and polycystic kidney disease.

Table 1. Liver function tests in the patient.
Lab parameters Patient values
Bilirubin (mg/dl) 1
Direct bilirubin (mg/dl) 0.5
SGOT (IU/L) 100
SGPT (IU/L) 46
Total protein (gm/dl) 6.5
Albumin (gm/dl) 3.5
Alkaline phosphatase (IU/dl) 2550
Prothrombin Time (sec) 32.4
Partial thromboplastin Time (sec) 46.0


Figure 1. CT abdomen showing cysts in liver and bilateral kidney suggestive of Caroli’s syndrome.
Caroli


How common is Caroli’s syndrome in infancy? How to manage this case?


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