先天性多形皮膚萎縮症に合併した食道狭窄の1例 A CASE OF ESOPHAGEAL STENOSIS WITH CONGENITAL POIKILODERMA

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先天性多形皮膚萎縮症は幼少期より種々の皮膚形成異常をきたす極めて稀な疾患である.われわれは先天性多形皮膚萎縮症に合併した食道狭窄の1例を経験した.症例は39歳女性.主訴は小児期からの嚥下困難.既往歴は腟口狭窄と特発性門脈圧亢進症があった.上部消化管造影で食道入口部,上部食道に膜様狭窄を認め,内視鏡検査では披裂後部は膜様となり,食道入口部は狭くscopeの挿入不可能であった.これに対し2回の拡張術で狭窄は改善し, scopeの挿入が可能となり,自覚症状も改善し,経口摂取も良好で外来通院中である.

Congenital poikiloderma is an extremely rare entity which causes various dysplasiae of the skin from chidhood. Recently we experienced a case of esophageal stenosis associated with congenital poikiloderma.<br> A 39-year-old woman was seen at the hospital because of dysphagia which began in her childhood. There were previous histories of stenosis of the vaginal orifice and idiopathic portal hypertension. Upper gastrointestinal series revealed figuratus stenoses at the enterance and upper portion of the esophagus. Endoscopically the arytenoid portoin of the larynx was membranous and the entrance of the esophagus was very narrow, so esophageal bougienage was required. After twice bougienage, the stenosis was improved to permit the insertion of a gastrofiber scope. Her dysphagia disappeared. She can take foods orally and is followed on an ambulant basis.

収録刊行物

  • 日本臨床外科医学会雑誌 = The journal of the Japanese Practical Surgeon Society

    日本臨床外科医学会雑誌 = The journal of the Japanese Practical Surgeon Society 58(2), 369-372, 1997-02-25

    Japan Surgical Association

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各種コード

  • NII論文ID(NAID)
    10008526852
  • NII書誌ID(NCID)
    AN00198696
  • 本文言語コード
    JPN
  • 資料種別
    NOT
  • ISSN
    03869776
  • データ提供元
    CJP書誌  J-STAGE 
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