CASE HISTORY
A Five-year-old female kid presented with complaints of multiple
episodes of vomiting of fresh blood for two days and black stool for a
day. There was no history of fever, jaundice, abdominal pain, loss of
appetite, generalized body swelling or any previous episodes of similar
blood mixed vomiting. There was no family history of any liver disease
or any cystic kidney disease.
The patient was asymptomatic at the time of the presentation. However,
on physical examination, she was pale and had mild abdominal distension
with venous dilatations around the umbilicus. The liver was palpable
upto 30cm below the right costal margin. Also, the spleen was palpable
4cm below the left costal margin and was firm and non-tender.
Cardiovascular, neurological, and respiratory examinations were
unremarkable. Her Blood Pressure was 120/80mmHg which was above the 99th
percentile for her age and height according to the WHO chart. (99th
percentile for her age and Height of 102.5cm= 114/78mmHg)
On hematological investigation, the patient had hemoglobin of 7.3gm/dl,
RBC- 3.89millions/μL. Leukopenia and thrombocytopenia were evident with
WBC and Platelets count of 2500/μL and 88,000/μL respectively. On
peripheral Blood Smear, anisopoikilocytosis and hypochromia were seen
with no evidence of any parasites. The liver function test showed an
increase in the Alkaline Phosphatase level i.e 376U/L (Normal:
<306U/L) with other results within the normal range. Renal
function test results were within normal limits. With the background of
pancytopenia, bone marrow aspiration was done which showed normocellular
marrow with erythroid hyperplasia without any evidence of atypical
cells, hemoparasites, or metastatic deposits.
For evaluation of upper gastrointestinal bleeding with suspected portal
hypertension, upper gastrointestinal endoscopy was performed which
revealed grade II esophageal varices. Ultrasonography of the abdomen
showed multiple fusiform dilatation of intrahepatic biliary ducts within
the right lobe of the liver. Bilateral kidney appeared echogenic with
loss of cortico-medullary differentiation. Computed Tomography portogram
with urography revealed multiple cystic dilatations of intrahepatic bile
ducts predominantly in the right lobe of the liver, largest measuring
approximately 20*14 mm in segment VIII, splenomegaly with non-visualized
main portal vein and multiple collaterals in the hepatic hilum,
perisplenic, peripancreatic region with esophageal, and rectal varices
along with ‘central-dot’ sign in the liver. (Figure 1,2 and 3)
Similarly, bilateral renal cysts with nephromegaly were seen. (Figure 4)
All findings were suggestive of Caroli’s disease with bilateral
polycystic kidney disease and portal hypertension. After admission,
esophageal variceal banding was done and treated prophylactically with
propranolol, and ranitidine. She was started on amlodipine to control
her increased blood pressure. A liver biopsy was not performed. During
the hospital stay, the patient received one pint of packed RBC. She was
discharged after nine days with no symptoms. She was symptom-free and
doing well on follow-up after one month and was kept on a regular
follow-up for monitoring the varices, blood pressure, liver and renal
functions.