Isolated polycystic liver disease in a child.

INTRODUCTION: Polycystic liver disease (PCLD) is a genetic disorder characterized by the growth of >10 cysts in the liver. PCLD is associated with polycystic kidney disease (PKD) in 80-90%of cases (Kothadia et al., 2023 [1]). PCLD can occur in isolation though rarely in children. We present a case report of a child with symptomatic isolated PCLD. CASE PRESENTATION: A 23-month old female child presented with a 17-month history of gradual increase in abdominal mass. She had acute onset of postprandial vomiting and shortness of breath while lying flat. On examination, she was irritable with massive abdominal distension. Liver function test done showed markedly elevated liver enzymes with preservation of liver synthesis function. Computed tomography (CT) scan showed a large intra-abdominal cyst and normal kidneys bilaterally. During laparotomy, we found multiple exophytic cysts arising from segment IVa of the liver. Hepatic resection was done successfully and patient recovered uneventfully. Histology showed Von Meyenburg complexes characteristic of PCLD. CLINICAL DISCUSSION: The goal of management should be to counter symptomatology by intervening on developed cysts. The therapeutic options are individualized to address the symptoms and improve the patients' quality of life. Follow up of the patients is based on the presentation and intervention performed, during which period recurrence of cysts is assessed. Complete resection of the liver cysts is recommended to avoid the risk of cholangiocarcinoma. CONCLUSION: Close follow up by physical examination, laboratory tests and imaging modalities is necessary to detect any recurring masses and malignancy transformation of the cysts to enable timely intervention.