Clin Pediatr Hematol Oncol.  2019 Oct;26(2):110-114. 10.15264/cpho.2019.26.2.110.

A Case of Congenital Hepatoblastoma Presented with Hepatic Osteodystrophy Complicated by Multiple Bone Fractures

• So YH
• Kim DS
• Yoon BA
• Choi YD
• Baek HJ
• Kook H

• KMID: 2464260
• DOI: http://doi.org/10.15264/cpho.2019.26.2.110

Abstract

Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone fractures despite adequate supplementation of fat-soluble vitamins including vitamin D. He was born by Caesarean section because of a 7 cm-sized abdominal mass detected by prenatal ultrasonography. The pathologic diagnosis was hepatoblastoma, PRETEXT staging III or IV. Whole body bone scan at the time of diagnosis showed no abnormal uptake. Oral vitamin D3 of 2,000 IU/day was administered with other fat-soluble vitamins. Serum direct bilirubin level gradually increased up to 28.9 mg/dL at postnatal 6 days and was above 5 mg/dL until 110 days of age. Bony changes consistent with rickets became apparent in left proximal humerus since 48 days of age, and multiple bone fractures developed thereafter. With resolving cholestasis by chemotherapy, his bony lesions improved gradually after add-on treatment of bisphosphonate and parenteral administration of vitamin D with calcium. High level of suspicion and prevention of osteodystrophy is needed in patients with hepatoblastoma, especially when cholestasis persists.

Keyword

Congenital hepatoblastoma; Fracture; Hepatic osteodystrophy

MeSH Terms

Bilirubin
Calcium
Cesarean Section
Cholecalciferol
Cholestasis
Diagnosis
Drug Therapy
Female
Fractures, Bone*
Hepatoblastoma*
Humans
Humerus
Infant
Liver Diseases
Male
Pregnancy
Rickets
Ultrasonography, Prenatal
Vitamin D
Vitamins
Bilirubin
Calcium
Cholecalciferol
Vitamin D
Vitamins