SMALL INTESTINAL GANGLIONEUROMATOSIS IN A PATIENT WITH NEUROFIBROMATOSIS TYPE 1: A CASE REPORT

DOI
  • KIDA Yuichi
    Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.
  • SAWADA Tsunaki
    Department of Endoscopy, Nagoya University Hospital.
  • ISHIKAWA Eri
    Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.
  • SAKAKIBARA Ayako
    Department of Pathology and Laboratory Medicine, Nagoya University Hospital.
  • YAMAMURA Takeshi
    Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.
  • MAEDA Keiko
    Department of Endoscopy, Nagoya University Hospital.
  • ESAKI Masaya
    Department of Endoscopy, Nagoya University Hospital.
  • HAMAZAKI Motonobu
    Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.
  • MURATE Kentaro
    Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.
  • NAKAMURA Masanao
    Department of Gastroenterology and Hepatology, Nagoya University Graduate School of Medicine.

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Other Title
  • 小腸内視鏡下生検により診断した神経線維腫症１型に合併した腸管diffuse ganglioneuromatosisの１例

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Abstract

<p>A 50-year-old woman with neurofibromatosis type 1 who hospitalized for chronic diarrhea was transferred to our hospital for evaluation of small intestinal dilatation and thickening. Transoral double-balloon enteroscopy revealed jejunal dilatation and suppressed peristalsis; however, mucosal inflammation, such as ulcers or erosions were not detected. Transanal double-balloon enteroscopy detected the intestinal stenosis with inflammatory polyps and a longitudinal ulcer. Histopathological evaluation of duodenal, jejunal, and ileal specimens by biopsy revealed ganglion cells and Schwannian cells; therefore, the patient was diagnosed with ganglioneuromatosis with neurofibromatosis type 1. Small intestinal dilatation was associated with suppressed peristalsis caused by ganglioneuromatosis and was diagnosed as secondary chronic intestinal pseudo-obstruction. Abdominal distention persisted despite conservative therapy. However, she remained asymptomatic, and oral intake remained unaffected. Ganglioneuromatosis is rare; however, clinicians should be mindful that ganglioneuromatosis is an abdominal complication associated with systemic disease such as neurofibromatosis type 1 and multiple endocrine neoplasia type 2B.</p>

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