Digestive Diseases and Sciences

, Volume 58, Issue 10, pp 2784–2788 | Cite as

Multiple Hepatic Adenomas in a Child with Microvillus Inclusion Disease

  • Jennifer C. Burgis
  • C. Allan Pratt
  • John P. T. Higgins
  • John A. Kerner
Stanford Multidisciplinary Seminars

Case Presentation and Evolution

The patient, who was born full term to non-consanguineous parents following a pregnancy complicated only by polyhydramnios, was admitted with severe dehydration, weight loss and diarrhea on the sixth day of life. His electrolytes were significant for sodium 149 mmol/L, potassium 5 mmol/L, chloride 130 mmol/L, and bicarbonate 4.5 mmol/L. For the next 2 months, he suffered recurrent episodes of sepsis and suspected necrotizing enterocolitis. His stool output was secretory and large volume, ranging from 100 to 200 ml/kg/day, and did not change with cessation of enteral feeds. Workup included stool cultures and serum titers negative for enteric bacteria, viral and parasitic infections, normal inflammatory markers, normal immunoglobulins and complement, normal gastrin and urine catecholamines. An endoscopic biopsy at 3 months of life showed total atrophy of the intestinal villi. Periodic acid-Schiff staining revealed targetoid structures in the cytoplasm of...


Adenoma Total Parenteral Nutrition Glycogen Storage Disease Galactosemia Hepatocellular Adenoma 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Conflict of interest



  1. 1.
    Veteläinen R, Erdogan D, de Graaf W, et al. Liver adenomatosis: re-evaluation of aetiology and management. Liver Int. 2008;28:499–508.PubMedCrossRefGoogle Scholar
  2. 2.
    Ruemmele FM, Schmitz J, Goulet O. Microvillous inclusion disease (microvillous atrophy). Orphanet J Rare Dis. 2006;1:22.PubMedCrossRefGoogle Scholar
  3. 3.
    Bell SW, Kerner JA Jr, Sibley RK. Microvillous inclusion disease. The importance of electron microscopy for diagnosis. Am J Surg Pathol. 1991;15:1157–1164.PubMedCrossRefGoogle Scholar
  4. 4.
    Sherman PM, Mitchell DJ, Cutz E. Neonatal enteropathies: defining the causes of protracted diarrhea of infancy. J Pediatr Gastroenterol Nutr. 2004;38:16–26.PubMedCrossRefGoogle Scholar
  5. 5.
    Halac U, Lacaille F, Joly F, et al. Microvillous inclusion disease: how to improve the prognosis of a severe congenital enterocyte disorder. J Pediatr Gastroenterol Nutr. 2011;52:460–465.PubMedCrossRefGoogle Scholar
  6. 6.
    Lee PJ. Glycogen storage disease type I: pathophysiology of liver adenomas. Eur J Pediatr. 2002;161:S46–S49.PubMedGoogle Scholar
  7. 7.
    Resnick MB, Kozakewich HP, Perez-Atayde AR. Hepatic adenoma in the pediatric age group. Clinicopathological observations and assessment of cell proliferative activity. Am J Surg Pathol. 1995;19:1181–1190.PubMedCrossRefGoogle Scholar
  8. 8.
    Stoot JHMB, Coelen RJS, De Jong MC, Dejong CHC. Malignant transformation of hepatocellular adenomas into hepatocellular carcinomas: a systematic review including more than 1600 adenoma cases. HPB (Oxford). 2010;12:509–522.CrossRefGoogle Scholar
  9. 9.
    Flejou JF, Barge J, Menu Y, et al. Liver adenomatosis. An entity distinct from liver adenoma? Gastroenterology. 1985;89:1132–1138.PubMedGoogle Scholar
  10. 10.
    Chiche L, Dao T, Salamé E, et al. Liver adenomatosis: reappraisal, diagnosis, and surgical management: eight new cases and review of the literature. Ann Surg. 2000;231:74–81.PubMedCrossRefGoogle Scholar
  11. 11.
    Barthelmes L, Tait IS. Liver cell adenoma and liver cell adenomatosis. HPB (Oxford). 2005;7:186–196.CrossRefGoogle Scholar
  12. 12.
    Kawakatsu M, Vilgrain V, Belghiti J, Flejou JF, Nahum H. Association of multiple liver cell adenomas with spontaneous intrahepatic portohepatic shunt. Abdom Imaging. 1994;19:438–440.PubMedCrossRefGoogle Scholar
  13. 13.
    Wang DQ, Fiske LM, Carreras CT, Weinstein DA. Natural history of hepatocellular adenoma formation in glycogen storage disease type I. J Pediatr. 2011;159:442–446.PubMedCrossRefGoogle Scholar
  14. 14.
    Di Rocco M, Calevo MG, Taro’ M, Melis D, Allegri AEM, Parenti G. Hepatocellular adenoma and metabolic balance in patients with type Ia glycogen storage disease. Mol Genet Metab. 2008;93:398–402.PubMedCrossRefGoogle Scholar
  15. 15.
    Raman SP, Hruban RH, Fishman EK. Hepatic adenomatosis: spectrum of imaging findings. Abdominal Imaging [Internet]. 2012 May 20. Available from: Cited 21 June 2012.
  16. 16.
    Greaves WOC, Bhattacharya B. Hepatic adenomatosis. Arch Pathol Lab Med. 2008;132:1951–1955.PubMedGoogle Scholar
  17. 17.
    van Aalten SM, Terkivatan T, van der Linden E, Verheij J, de Man RA, Ijzermans JNM. Management of liver adenomatosis by radiofrequency ablation. Dig Surg. 2011;28:173–177.PubMedCrossRefGoogle Scholar
  18. 18.
    Wellen JR, Anderson CD, Doyle M, et al. The role of liver transplantation for hepatic adenomatosis in the pediatric population: case report and review of the literature. Pediatr Transpl. 2010;14:E16–E19.CrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Jennifer C. Burgis
    • 1
  • C. Allan Pratt
    • 1
  • John P. T. Higgins
    • 2
  • John A. Kerner
    • 1
  1. 1.Division of Gastroenterology, Hepatology and Nutrition, Department of PediatricsLucile Packard Children’s Hospital at Stanford, Stanford University Medical CenterPalo AltoUSA
  2. 2.Department of PathologyLucile Packard Children’s Hospital at Stanford, Stanford University Medical CenterPalo AltoUSA

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