Clinical Challenges

What is your diagnosis? - September 2020

A 15-month-old, previously thriving boy from western urban India was brought in with high-grade pyrexia of unknown origin for the last 45 days. He had received multiple courses of antibiotics and antimalarials elsewhere without any response. Appetite and general activity were preserved. Examination revealed mild pallor, significant nontender soft hepatomegaly (liver span of 14 cm) without splenomegaly or peripheral lymphadenopathy. Investigations showed a hemoglobin of 9 g/dL, microcytic hypochromic smear, total leukocyte count of 48,900/mm3, neutrophils at 16%, lymphocytes at 23%, eosinophils at 58%, absolute eosinophil count of 28,362/mm3, platelet count of 490,000/mm3, bilirubin of 0.8 mg/dL, aspartate aminotransferase of 203 IU/L, alanine aminotransferase of 179 IU/L, total protein of 9.3 g/dL, albumin of 3.6 g/dL, alkaline phosphatase of 203 IU/L, and gamma-glutamyl transpeptidase of 107 IU/L. Ultrasound examination and computed tomography scans of abdomen showed no focal lesions or abscesses. A bone marrow biopsy revealed an increase in eosinophils and its precursors. Echocardiography, retroviral serology, and multiple blood and urine cultures were unyielding. Liver biopsy was performed for diagnosis (Figures A-C).


 

Capillaria hepatica infection


The liver parenchyma shows spindle-shaped eosinophilic eggs surrounded by eosinophilic inflammatory infiltrates and epithelioid granuloma (original magnification ×200). Figure B shows spindle-shaped eosinophilic eggs with shells, radial striations, and visible polar body, containing granular eosinophilic debris (original magnification ×1000), consistent with Capillaria hepatica. Figure C reveals crescent-shaped, degenerated adult worms of C. hepatica showing longitudinal bacillary bands, vacuolated intestine, and convoluted gonads surrounded by intense eosinophilic inflammation in liver parenchyma (original magnification ×400). The outer cuticle is not appreciated because the worms are degenerated.
A review of history revealed that the child played with stray cats and had pica. He was given 10 mg/kg of oral albendazole for 16 weeks and 1 mg/kg of oral prednisolone for the first 2 weeks to prevent paradoxical inflammatory response. Thereafter, prednisolone was tapered and stopped. Pyrexia, liver size, AEC, and liver enzymes normalized at 24 hours, 72 hours, 4 months, and 5 months, respectively. At 12 months of follow-up, the child is asymptomatic.
Capillaria hepatica is a rare nematodal invasive parasitosis where humans are the dead-end host; the main lifecycle occurs between rodents and their predators. Adult worms live, mate, and lay noninfective unembryonated eggs in rodent livers. Embryogenesis occurs only after contact with the soil in two settings: 1) the rodent is eaten by the predator and the unembryonated eggs are released in the predator's feces or 2) carcass disintegration after natural death of the rodent. Humans incidentally ingest the infective embryonated eggs by soil to mouth transmission. They hatch in the human intestine and the larvae migrate through the portal vein into the liver where they mature into adult worms. In the liver, the cycle continues with the adult worms mating and laying eggs. This elicits intense inflammation with systemic symptoms. 1 In the index case, we hypothesize that the toddler with pica would have come in contact with soil in the vicinity of the stray cats. This soil would have initially contained the feline feces with unembryonated eggs that later underwent embryogenesis. The triad of fever, hepatomegaly, and eosinophilia is the hallmark and characteristic liver histology clinches the diagnosis. Duration of anthelminthic therapy should be guided by AEC response. 2,3

References
1. Wright K.A. Observation on the life cycle of Capillaria hepatica with a description of the adult. Can J Zool. 1961;39:167-82.
2. Berger T. Degrémont A. Gebbers J.O. et al. Hepatic capillariasis in a 1-year-old child. Eur J Pediatr. 1990;149:333-633.
3. Choe G. Lee H.S. Seo J.K. et al. Hepatic capillariasis: first case report in the Republic of Korea. Am J Trop Med Hyg. 1993;48:610-25.

Next Article: