Background Bile duct stenosis with gallbladder distention may be caused by a variety of conditions that are common in adults but rare in children. Although acalculous gallbladder hydrops is one of complications of Kawasaki disease (KD) with frequency of 5-13%, its aetiology remains unclear.
Analyse a case report with unusual explanation of usual complication of KD. 2. Review published evidence on gall bladder pathology in paediatric vasculitis.
Methods Case description and literature review
Results An 8-years old Caucasian boy was admitted for a 7-day history of fever and joint pain with no clear aetiology. The physical exam revealed cracked lips, strawberry tongue, tonsillitis, lymphadenopathy, rash and conjunctivitis. Blood tests demonstrated non-specific inflammation with thrombocytosis, elevated aminotransferases (2-3xULN) and alkaline phosphatase (ALP), normal Gamma-glutamyltransferase (GGT) and bilirubin levels. Infectious workup was negative. Echocardiography and abdominal ultrasound revealed no pathologies. Diagnosis of KD was made and intravenous immunoglobulins (2g/kg) with high-dose aspirin were administered with rapid response. The boy was discharged with a low-dose aspirin therapy. 27 days later he was admitted again for an acute abdominal pain with raised transaminases (10-20xULN), ALP (5xULN) and GGT (20xULN) and a new increase of ESR, CRP and platelet count. Abdominal ultrasound revealed distention of the gallbladder and the main bile duct dilatation. As there was no improvement on symptomatic treatment, magnetic resonance cholangiopancreaticography (MRCP) was performed with demonstration of suprapapillar stenosis of the main bile duct. ERCP with papillotomy and introduction of the stent (7Fr) was performed with restentation (10Fr) in 2 weeks. Prophylactic antiobiotics were administered. Due to presumed vasculitic origin of the stenosis prednisone was added to the therapy (2mg/kg/day) with slow taper over following 2 months with rapid improvement.
Conclusions PubMed search (1995-2015) has identified total of 32 KD patients with gallbladder hydrops. In 28 cases ultrasound and in 1 case CT was the only diagnostic method mentioned with no further information on hydrops aetiology or potential presence of obstructive hepatopathy. In 3 children with biochemical signs of biliary obstruction bile duct stenosis requiring stenting was found. None of them underwent abdominal arteriography. In our patient normal ultrasound findings and absence of obstructive hepatopathy at disease onset suggest potential vasculitic aetiology. Although vascular imaging was not performed it can be hypothesized that the scarring of the bile duct wall affected in acute stage of KD caused stenosis which resulted in obstructive clinical and laboratory features. We believe that biliary tree imaging should be considered in all children with vasculitis in whom gallbladder distention and obstructive hepatopathy develops in order to detect and treat potential stenosis before irreversible liver changes develop.
Cherry R. et al. Common bile duct stenosis and pancreatitis in Kawasaki disease: two cases and a review of literature. Pediatr Infect Dis J. 2010 Jun;29(6):571-3.
Disclosure of Interest None declared
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