Pediatric segmental Caroli disease: A case report and review of the literature

• Published in: Journal of pediatric surgery case reports Vol. 95; p. 102665
• Main Authors: Koo, Donna C., Scalise, P. Nina, Bakker, Andrea, Vargas, Sara O., Shaikh, Raja, Cuenca, Alex G., Kim, Heung Bae, Lee, Eliza J.
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01.08.2023; Elsevier
• Subjects: Caroli disease; Case report; Pediatric; Segmental; Case report; Segmental; Caroli disease; Pediatric
• ISSN: 2213-5766; 2213-5766
• DOI: 10.1016/j.epsc.2023.102665

Caroli disease is a rare congenital disorder causing nonobstructive segmental dilatation of intrahepatic bile ducts. Caroli disease can affect the liver diffusely or be confined to a single segment or lobe, although localized Caroli disease is extremely uncommon. We present a rare case of an 8-year-old child with incidentally found Caroli disease limited to two hepatic segments who was treated with partial hepatectomy, as well as a review of the literature regarding the presentation, diagnosis, and management of Caroli disease in children and adults. Our patient is an 8-year-old male with an incidentally found multiloculated cystic liver mass referred to our institution for further management. Due to its heterogeneous nature and lack of evidence of direct communication with the biliary system, our differential diagnosis included hepatic teratoma, mesenchymal hamartoma, and undifferentiated embryonal sarcoma. A decision was made to resect the lesion both for definitive diagnosis and to mitigate the risk of malignant transformation of this lesion. At the time of surgery, the mass was observed to occupy portions of liver segments 6 and 7, necessitating a formal right hepatectomy and cholecystectomy. The patient tolerated the procedure well, was extubated in the operating room, and recovered in the ICU in stable condition without further complications and was seen in clinic for follow-up without further postoperative concerns. Though rare, Caroli disease can present as an asymptomatic focal heterogeneous mass in children. Pediatric patients with segmental Caroli disease can be treated with partial hepatectomy safely.