Case Report
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World J Gastroenterol. Jun 28, 2014; 20(24): 7979-7983
Published online Jun 28, 2014. doi: 10.3748/wjg.v20.i24.7979
Small bowel volvulus as a complication of von Recklinghausen’s disease: A case report
Thomas Artur Werner, Feride Kröpil, Martin Olaf Schoppe, Patric Kröpil, Wolfram Trudo Knoefel, Andreas Krieg
Thomas Artur Werner, Feride Kröpil, Wolfram Trudo Knoefel, Andreas Krieg, Department of Surgery (A), Heinrich-Heine-University and University Hospital Duesseldorf, 40225 Duesseldorf, Germany
Martin Olaf Schoppe, Institute of Pathology, Heinrich-Heine-University and University Hospital Duesseldorf, 40225 Duesseldorf, Germany
Patric Kröpil, Institute of Diagnostic and Interventional Radiology, Heinrich-Heine-University and University Hospital Duesseldorf, 40225 Duesseldorf, Germany
Author contributions: Werner TA designed and wrote the report, analyzed and interpreted the data; Krieg A, Kröpil F and Knoefel WT were attending doctors of the patient; Krieg A performed the operation; Schoppe MO performed pathological examinations; Kröpil P analyzed the CT scan; Krieg A, Kröpil P and Knoefel WT critically revised the report and gave important intellectual input.
Correspondence to: Andreas Krieg, MD, Department of Surgery (A), Heinrich-Heine-University and University Hospital Duesseldorf, Moorenstrasse 5, 40225 Duesseldorf, Germany. andreas.krieg@med.uni-duesseldorf.de
Telephone: +49-211-8116399 Fax: +49-211-8119205
Received: December 17, 2013
Revised: February 9, 2014
Accepted: March 19, 2014
Published online: June 28, 2014
Abstract

We report the case of a 25-year-old male with Neurofibromatosis type I (NF-1), who presented at the time of admission with clinical findings of an acute abdomen caused by a mechanical obstruction. Computerized tomography showed a volvulus of the terminal ileum with mesenteric swirling as the cause of the patient’s symptoms. Consecutive exploratory laparotomy confirmed the diagnosis and 70 cm of the small intestine was resected due to an affection of the mesentery by multiple neurofibromas. The gastrointestinal tract is affected in approximately 10% of patients with NF-1, however the mesentery is almost always spared. Here we describe the unique case of a patient with a volvulus caused by mesenteric manifestation of von Recklinghausen’s disease, emphasizing the role of surgery in a team of multidisciplinary specialists to treat this multiorganic disease.

Keywords: Volvulus, Obstruction, Von Recklinghausen´s disease, Neurofibromatosis type I, Mesenteric neurofibromas

Core tip: Neurofibromatosis type I (NF-1) is one of the most common single gene disorders known for its phenotypical presentation with café au lait macules and neurofibromas. Although every organ system can be involved, the intra-abdominal manifestation is rare. To the best of our knowledge, this is the first reported case of a patient with mesenteric involvement of NF-1 manifesting as a volvulus, underscoring the role of surgery in this heterogeneous disease.