苗勒氏管不發育：一病例報告並描述全人照護

Mullerian Agenesis: A Case Report and Description of Holistic Care

10.7023/TJFM.200509.0181

許維邦(Wei-Pang Hsu)；蕭培靜(Pei-Ching Hsiao)；周思源(Sze-Yuan Chou)；楊日昇(Jin-Sheng Yang)；許維堅(Wei-Jian Hsu)

primary amenorrhea ； mullerian agenesis ； holistic care

台灣家庭醫學雜誌

15卷3期（2005 / 09 / 01）

181 - 189

繁體中文

在原發性無月經症當中，苗勒氏管不發育是僅次於性腺發育異常的第二常見原因。由於胚胎發育異常導致缺少輸卵管、子宮和上三分之一陰道。可能高達40%病患合併有先天腎臟異常，5-10%合併骨骼異常。有正常女性第二性徵發育。血中荷爾蒙檢測顯示正常卵巢功能。染色體檢驗結果是46XX。除了原發性無月經外，病患合併有不孕症、性交不快甚至無法性交。經常在病患過了青春期就被診斷出來。對病患和其它家庭成員造成嚴重身心創傷。本篇探討一位十八歲女性，診斷為苗勒氏管不發育之原發性無月經症。除了給予病患和家屬生物、心理和社會層面照護，並兼顧生命倫理和法律諮詢。希望藉此提醒基層醫師在照顧病患時需謹記人性化醫療之實踐，提昇臨床照護品質以嘉惠病患。

Mullerian agenesis is the second most common cause of primary amenorrhea after gonadal dysgenesis. Due to abnormal embryologic development, there is an absence of fallopian tubes, uterus and upper one-third of the vagina. Congenital renal abnormalities occur in as high as 40% of these individuals and skeletal abnormalities in 5-10%. Female secondary sexual characteristics develop normally. Hormone assay presents normal ovarian function. The data of chromosome analysis is 46XX. In addition to primary amenorrhea, the patient suffers infertility, dyspareunia and even difficulty in intercourse. The diagnosis is often made just after puberty and causes severe psychosomatic trauma to the patient and her family. We present a 18-year-old young lady with primary amenorrhea due to mullerian agenesis. Besides biopsychosocial care, we also offer bioethic and legal consultation to the patient and her family. By presenting this case, we want to remind the primary care doctors to fulfill a humanistic medical care during daily practice, promote quality care and do good for all patients.