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Case Report
A Case of Congenital Hepatoblastoma Presented with Hepatic Osteodystrophy Complicated by Multiple Bone Fractures
Clin Pediatr Hematol Oncol 2019;26:110-4.
Published online October 31, 2019
© 2019 Korean Society of Pediatric Hematology-Oncology and Korean Society for Pediatric Neuro-Oncology

Yoon Heui So1,2, Dae Sung Kim1, Bo Ae Yoon1, Yoo-Duk Choi3, Hee Jo Baek1,2 and Hoon Kook1,2

1Department of Pediatrics, Chonnam National University Hwasun Hospital, Hwasun,
Departments of 2Pediatrics and 3Pathology, Chonnam National University Medical School, Gwangju, Korea
Correspondence to: Hee Jo Baek
Department of Pediatrics, Chonnam National University Medical School, Chonnam National University Hwasun Hospital, 160 Ilsim-ri, Hwasun 519-809, Korea
Tel: +82-61-379-7695
Fax: +82-61-379-7697
E-mail: swan93@naver.com
ORCID ID: orcid.org/0000-0003-3830-8134
Received September 17, 2019; Revised October 8, 2019; Accepted October 14, 2019.
This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/4.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
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.
Keywords: Congenital hepatoblastoma, Fracture, Hepatic osteodystrophy
References
  1. Grosfeld J, O'Neill J, Coran A, Fonkalsru E. Pediatric surgery. 6th ed. Philadelphia: Mosby, 2006;502.
  2. Ergin H, Yildirim B, Dagdeviren E, et al. A prenatally detected case of congenital hepatoblastoma. Pathol Oncol Res 2008;14:97-100.
    Pubmed CrossRef
  3. Hill SA, Kelly DA, John PR. Bone fractures in children undergoing orthotopic liver transplantation. Pediatr Radiol 1995;25 Suppl 1:S112-7.
    Pubmed
  4. Hansen AE, Ziegler MR, McQuarrie I. Disturbance of osseous and lipid metabolism in a child with primary carcinoma of the liver. J Pediatr 1940;17:9-30.
    CrossRef
  5. Roberts MH, Sullivan C. Influence of the liver on bone metabolism;report of two cases. J Am Med Assoc 1955;159:1002-7.
    Pubmed CrossRef
  6. Teng CT, Daeschner CW Jr, Singleton EB, et al. Liver diseases and osteoporosis in children. I. Clinical observations. J Pediatr 1961;59:684-702.
    Pubmed CrossRef
  7. Archer D, Babyn P, Gilday D, Greenberg MA. Potentially misleading bone scan findings in patients with hepatoblastoma. Clin Nucl Med 1993;18:1026-31.
    Pubmed CrossRef
  8. Towbin AJ, Braojos Braga FDC, Zhang B, Geller JI, Tiao GM, Podberesky DJ. Fractures in children with newly diagnosed hepatoblastoma. Pediatr Radiol 2018;48:581-5.
    Pubmed CrossRef
  9. Gatta A, Verardo A, Di Pascoli M, Giannini S, Bolognesi M. Hepatic osteodystrophy. Clin Cases Miner Bone Metab 2014;11:185-91.
    Pubmed KoreaMed CrossRef
  10. Barbu EC, Chițu-Tisu CE, Lazar M, et al. Hepatic osteodystrophy:a global (re)view of the problem. Acta Clin Croat 2017;56:512-25.
    Pubmed CrossRef
  11. Okada T, Honda S, Miyagi H, Minato M, Taketomi A. Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia. World J Hepatol 2012;4:284-7.
    Pubmed KoreaMed CrossRef
  12. Katsura S, Ogita K, Taguchi T, et al. Effect of liver transplantation on multiple bone fractures in an infant with endstage biliary atresia: a case report. Pediatr Surg Int 2005;21:47-9.
    Pubmed CrossRef
  13. Shneider BL, Magee JC, Bezerra JA, et al. Efficacy of fat-soluble vitamin supplementation in infants with biliary atresia. Pediatrics 2012;130:e607-14.
    Pubmed KoreaMed CrossRef
  14. Venkat VL, Shneider BL, Magee JC, et al. Total serum bilirubin predicts fat-soluble vitamin deficiency better than serum bile acids in infants with biliary atresia. J Pediatr Gastroenterol Nutr 2014;59:702-7.
    Pubmed KoreaMed CrossRef


October 2019, 26 (2)
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  • Hee Jo Baek