Hepatobiliary Involvement in von Recklinghausen's Disease

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Abstract
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