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Hepatobiliary Involvement in von Recklinghausen's Disease
- COL. GEORGE W. MEYER;
- WILLIAM J. GRIFFITHS, M.D., Ph.D.;
- JACK WELSH, M.D.;
- LEONARD COHEN, M.D.;
- LEWIS JOHNSON, M.D.; and
- MICHAEL J. WEAVER, M.D.
- MC, USAF
- USAF Medical Center, Wright-Patterson Air Force Base Ohio; University of Oklahoma Health Science Center, Oklahoma City, Oklahoma; University of California, San Francisco, California; and Brown University School of Medicine, Providence, Rhode Island
Excerpt
Von Recklinghausen's disease is a syndrome characterized by multiple cutaneous neurofibromas, cafe-aulait spots, and a broad spectrum of clinical, pathologic, and radiologic findings. Von Recklinghausen's disease is inherited as a variably expressed dominant gene, with 50% of the patients having affected relatives. As many as 25% of patients with this illness have neurofibromas or other related neurogenic tumors in the gastrointestinal tract (1). Patients with these tumors usually present with gastrointestinal hemorrhage or obstruction. Hepatobiliary complications are rare, and we know of only one reported case (2). We report three patients with von Recklinghausen's disease and prominent hepatobiliary involvement.
Patient
Acknowledgments
ACKNOWLEDGMENTS: The authors thank Ms. Jean Veasey and Miss Betty Anstine for their secretarial assistance.
Article and Author Information
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The opinions expressed herein are those of the authors and are not necessarily those of the United States Air Force or the Department of Defense.
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▸Requests for reprints should be addressed to Col. George W. Meyer, MC, USAF; Wilford Hall USAF Med Center/SGHM; Lackland Air Force Base, TX 78236.
