This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (3)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by DeRusso, P. A.
Right arrow Articles by Schwarz, K. B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by DeRusso, P. A.
Right arrow Articles by Schwarz, K. B.
Related Collections
Right arrow Gastrointestinal Tract
PEDIATRICS Vol. 112 No. 1 July 2003, pp. 185-188

EXPERIENCE AND REASON

Fractures in Biliary Atresia Misinterpreted as Child Abuse

Patricia A. DeRusso, MD, Melissa R. Spevak, MD and Kathleen B. Schwarz, MD

Department of Pediatrics
Division of Pediatric Gastroenterology and Nutrition
Johns Hopkins School of Medicine
Baltimore, MD 21287
Division of Pediatric Imaging
Russell H. Morgan Department of Radiology and Radiological Science
Johns Hopkins Hospital
Johns Hopkins University
Baltimore, MD 21287
Department of Pediatrics
Division of Pediatric Gastroenterology and Nutrition
Johns Hopkins School of Medicine
Baltimore, MD 21287


   ABSTRACT
TOP
 ABSTRACT
CASE REPORTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Bone fractures in children without a history of injury are highly suspicious for child abuse. Biliary atresia is a disorder associated with metabolic bone disease, and there are numerous reports of osteopenia, rickets, and/or fractures in this population. We report 3 cases of children with biliary atresia who had bony fractures as well as osteopenia whose caretakers were investigated for child abuse. Pediatricians should be aware of an increased incidence of fractures and overall prevalence of bone disease in this population.

Key Words: biliary atresia • osteopenia • fractures • abuse

Abbreviations: ED, emergency department • CPS, Child Protective Services

Bone fractures in young children without a history of injury are considered highly suspicious for nonaccidental trauma. In children <1 year of age, long bone fractures especially involving the metaphyses are particularly concerning and are strongly associated with child abuse.1,2 When abuse is suspected, the radiographic skeletal survey is recommended for global imaging.3 The role of imaging, however, is to not only identify the extent of physical injury but also to assess all imaging findings that suggest alternative diagnoses.3 In addition to radiographic studies, history and clinical findings are also important.

There are childhood disorders in which metabolic bone disease occurs and can lead to bone fractures. Numerous studies have shown that bone disease is a well-known complication in children with biliary atresia. Radiographic findings in this population include osteopenia, rickets, and/or fractures.

We report 3 cases of infants with biliary atresia who came to the emergency department (ED) for apparent bone pain. When multiple fractures were seen on radiographic studies, the Child Protective Services (CPS) team in the ED investigated the caretakers for possible child abuse.


   CASE REPORTS
TOP
 ABSTRACT
 CASE REPORTS
DISCUSSION
 CONCLUSIONS
 REFERENCES
 
Case 1
A 4-month-old black girl with biliary atresia underwent a hepatic portoenterostomy and received a short course of steroids. She was brought to the ED 1 month later for irritability and left arm pain. Radiographic study of the left humerus revealed a fracture proximally. She underwent a skeletal survey which, in addition to the humeral fracture, revealed generalized osteopenia with fracture of bilateral scapulae and fracture of the right distal radius and ulna (Fig 1). The types and numbers of fractures were thought highly suspicious for nonaccidental trauma. The family was immediately evaluated by the CPS team in the ED. Subsequent to the CPS evaluation, blood analyses revealed hypocalcemia (ionized calcium: 0.7 mmol/L; total calcium: 5.7 mg/dL). The 25-OH vitamin D level was below normal despite the patient being supplemented with 10 times the recommended daily allowance for vitamin D. She was admitted to the hospital for intravenous calcium. The orthopedic team recommended splinting of left upper extremity. The patient was discharged from the hospital with 1,25 (OH)2D (Rocaltrol) and a calcium carbonate supplement in addition to her previous medications.


Figure 1
View larger version (63K):
[in this window]
[in a new window]
  		Fig 1. A, Fracture through the lateral cortex of the left humerus (arrow). B, Right scapular fracture (arrow). Note the osteopenia. A similar fracture was seen in the left scapula.

 
Case 2
A 14-month-old black girl with biliary atresia underwent a hepatic portoenterostomy. Approximately 9 months after surgery she was brought to the ED after falling downstairs and refusing to bear weight on her left leg. Radiographic studies revealed osteopenia and buckle fractures of the distal left tibia and fibula (Fig 2). A metaphyseal corner-type fracture in the medial femoral metaphysis was also seen. This type of fracture is frequently seen in nonaccidental trauma. The radiologist referred the patient back to the ED where the family was investigated by CPS for possible child abuse. No blood chemistries were performed at the time. The patient had been receiving >8 times the recommended daily allowance for vitamin D supplementation and previous blood analyses had revealed normal 25-OH vitamin D and total calcium levels.


Figure 2
View larger version (83K):
[in this window]
[in a new window]
  		Fig 2. Lateral view of the left leg showing osteopenia and buckle fractures of the distal tibia (arrow) and fibula (arrowhead).

 
Case 3
An 18-month-old black girl with biliary atresia underwent a hepatic portoenterostomy and was residing in a convalescent hospital for children. She was referred to the ED for leg pain resulting in nonweight bearing that was noted shortly after a phlebotomist had held her by the ankles to draw blood from her foot. Radiographic studies revealed fractures in the distal metaphyses of both tibias and fibulas that were already healing (Fig 3). Severe osteopenia with abnormal modeling consistent with long-standing chronic liver disease was also reported. Blood analyses were not available. The phlebotomist was evaluated by the CPS team for possible child abuse.


Figure 3
View larger version (45K):
[in this window]
[in a new window]
  		Fig 3. A, Anterior-posterior view of the distal left leg. B, Lateral view of the distal left leg. Both views show fracture of the tibia (arrow) and fibula. The tibial fracture shows early callus formation, a sign of healing. Osteopenia is present. Nearly identical findings were seen on the right.

 

   DISCUSSION
TOP
 ABSTRACT
 CASE REPORTS
 DISCUSSION
CONCLUSIONS
 REFERENCES
 
Although large-scale investigations indicating the prevalence of bone disease in biliary atresia do not exist, there are studies that collectively show bone disease is a well-known complication.4–8 In a recent study of 42 patients4 with biliary atresia, 45% had signs of osteoporosis according to dual-energy x-ray absorptiometry. In a review5 of 38 cases of biliary atresia, osseous abnormalities were found in 21% and fractures were apparent in 11%. In an investigation6 of bone studies in children with biliary atresia, 4 of 10 patients had evidence of rickets on radiographic studies. In another study, bone mineral density was low in 2 of 8 patients with biliary atresia after successful hepatic portoenterostomy procedure.7 Radiographic evidence of osteopenia began at about 12 months of age in one study of infants with biliary atresia.8

Biliary atresia is one of the most common causes of neonatal cholestasis and malabsorption of nutrients is a significant problem.9 The etiology of bone disease in biliary atresia, however, remains unclear. Vitamin D deficiency has been implicated as a factor in fracture formation. Vitamin D is a fat-soluble vitamin that requires adequate hepatic excretion of bile acids into the intestinal lumen for absorption. Children with biliary atresia who have progressive injury of the intrahepatic bile ducts with subsequent cholestasis are at risk for vitamin D deficiency resulting from reduced presence of bile acids in the duodenum. Although these children are routinely supplemented with vitamin D, it has been reported that 66% of infants with cholestasis still have biochemical evidence of vitamin D deficiency despite routine supplementation.10 Absorption of ingested vitamin D2 (ergocalciferol) is impaired in cholestatic liver disease.12 Furthermore, inhibition of vitamin D 25-hydroxylase activity is seen with bile duct ligation or elevated bile acids suggesting a likely problem with children with biliary atresia.13

Additional factors related to bone disease include decreased intestinal absorption of calcium and phosphate attributable to fat malabsorption leading to insoluble soap formation.9 Magnesium depletion and osteopenia have been reported11 in a small group of individuals with cholestatic liver disease. Hyperparathyroidism has been reported in hepatobiliary disease of infancy.14 Hyperbilirubinemia or other substances present in serum may impair osteoblast proliferative capacity and play a role in bone disease associated with cholestatic jaundice.15

Although children with chronic illnesses may be at a higher risk for maltreatment,16 our experience has been that the parents of children with biliary atresia provide excellent care of these children. They never miss numerous appointments, are compulsive about giving medications, and genuinely feel fortunate to have these children in their lives. Subjecting these families to child abuse investigation is usually not only unnecessary but seemingly cruel.

In these 3 cases, the CPS team decided there was no evidence to substantiate child abuse. We would recommend that in similar circumstances, the ED physician contact the primary attending before proceeding with an evaluation by CPS. The information could be evaluated by physicians who have had a longer relationship with the child and family. Then a decision could jointly be made regarding how to proceed.


   CONCLUSIONS
TOP
 ABSTRACT
 CASE REPORTS
 DISCUSSION
 CONCLUSIONS
REFERENCES
 
Bone disease is a well-known complication of biliary atresia. There are well-documented findings of osteopenia and rickets occurring in childhood cholestatic liver disease. Osteopenia evidenced by cortical thinning and trabecular bone loss, changes of rickets, and/or multiple fractures would suggest an etiology other than child abuse. Pediatricians should be aware of an increased incidence of fractures and overall prevalence of bone disease in this population.


   FOOTNOTES
 
Received for publication Nov 15, 2002; Revised Feb 6, 2003; Reprints requests to (P.A.D.) Department of Pediatrics, Division of Pediatric Gastroenterology and Nutrition, Johns Hopkins Children’s Center, 600 N Wolfe St, Brady 320, Baltimore, MD 21287. E-mail: tderusso{at}jhmi.edu


   REFERENCES
TOP
 ABSTRACT
 CASE REPORTS
 DISCUSSION
 CONCLUSIONS
 REFERENCES
 

  1. Thomas SA, Rosenfield NS, Leventhal JM, Markowitz RI. Long-bone fractures in young children: distinguishing accidental injuries from child abuse. Pediatrics.1991; 88 :471 –476[Abstract/Free Full Text]
  2. Skellern CY, Wood DO, Murphy A, Crawford M. Non-accidental fractures in infants: risk of further abuse. J Paediatr Child Health.2000; 36 :590 –592[CrossRef][ISI][Medline]
  3. American Academy of Pediatrics, Section on Radiology. Diagnostic imaging of child abuse. Pediatric.2000; 105 :1345 –1348
  4. Chongsrisawat V, Ruttanamongkol P, Chaiwatanarat T, Chandrakamol B, Poovorawan Y. Bone density and 25-hydroxyvitamin D level in extrahepatic biliary atresia. Pediatr Surg Int.2001; 17 :604 –608[CrossRef][ISI][Medline]
  5. Katayama H, Suruga K, Kurashige T, Kimoto T. Bone changes in congenital biliary atresia. Radiologic observation of 8 cases. Am J Roentgenol Radium Ther Nucl Med.1975; 124 :107 –112[Medline]
  6. Ohshima K, Kubo Y, Samejima N. Bone mineral analysis and x-ray examination of the bone in patients with biliary atresia. Jpn J Surg.1990; 20 :537 –544[CrossRef][Medline]
  7. Toki A, Todani T, Watanabe Y, et al. Bone mineral analysis in patients with biliary atresia after successful Kasai procedure. Tohoku J Exp Med.1997; 181 :213 –216[CrossRef][ISI][Medline]
  8. Glasgow JF, Thomas PS. The osteodystrophy of prolonged obstructive liver disease in childhood. Acta Paediatr Scand.1976; 65 :57 –64[ISI][Medline]
  9. Feranchak AP, Ramirez RO, Sokol RJ. Medical and nutritional management of cholestasis. In: Suchy RJ, Sokol RJ, Balistreri WF, eds. Liver Disease in Children. 2nd ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2001:195–235
  10. Book LS. Fat soluble vitamins in cholestasis. In: Adcock EW III, Lester R, eds. Neonatal Cholestasis: Causes, Syndromes, Therapies. Report of the Eighty Seventh Ross Conference in Pediatric Research. Columbus, OH: Ross Laboratories; 1984:104–110
  11. Heubi JE, Higgins JV, Argao EA, Sierra RI, Specker BI. The role of magnesium in the pathogenesis of bone disease in childhood cholestatic liver disease: a preliminary report. J Pediatr Gastroenterol Nutr.1997; 25 :301 –306[CrossRef][ISI][Medline]
  12. Heubi JE, Hollis BW, Specker B, Tsang RC. Bone disease in chronic childhood cholestasis. I. Vitamin D absorption and metabolism. Hepatology.1989; 9 :258 –264[ISI][Medline]
  13. Bolt MJ, Sitrin MD, Favus MJ, Rosenberg IH. Hepatic vitamin D 25-hydroxylase: inhibition by bile duct ligation or bile salts. Hepatology.1981; 5 :436 –440
  14. Kobayashi A, Kawai S, Utsunomiya T, Ohbe Y. Hyperparathyroidism in hepatobiliary disease in infancy. Eur J Pediatr.1975; 121 :5 –13[CrossRef][ISI][Medline]
  15. Janes CH, Dickson ER, Okazaki R, Bonde S, McDonagh AF, Riggs BL. Role of hyperbilirubinemia in the impairment of osteoblast proliferation associated with cholestatic jaundice. J Clin Invest.1995; 95 :2581 –2586
  16. American Academy of Pediatrics, Committee on Child Abuse and Neglect and Committee on Children With Disabilities. Assessment of maltreatment of children with disabilities. Pediatrics.2001; 108 :508 –512[Abstract/Free Full Text]
PEDIATRICS (ISSN 1098-4275). ©2003 by the American Academy of Pediatrics




This Article
Right arrow Abstract Freely available
Right arrow Full Text (PDF)
Right arrow P3Rs: Submit a response
Right arrow Alert me when this article is cited
Right arrow Alert me when P3Rs are posted
Right arrow Alert me if a correction is posted
Services
Right arrow E-mail this article to a friend
Right arrow Similar articles in this journal
Right arrow Similar articles in ISI Web of Science
Right arrow Similar articles in PubMed
Right arrow Alert me to new issues of the journal
Right arrow Add to My File Cabinet
Right arrow Download to citation manager
Right arrow reprints & permissions
Citing Articles
Right arrow Citing Articles via CrossRef
Right arrow Citing Articles via ISI Web of Science (3)
Right arrow Citing Articles via Google Scholar
Google Scholar
Right arrow Articles by DeRusso, P. A.
Right arrow Articles by Schwarz, K. B.
Right arrow Search for Related Content
PubMed
Right arrow PubMed Citation
Right arrow Articles by DeRusso, P. A.
Right arrow Articles by Schwarz, K. B.
Related Collections
Right arrow Gastrointestinal Tract