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相關文獻
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題名 | Deterioration of Multiple Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia during Pregnancy=妊娠會導致遺傳出血性毛細管擴張症的多發性肺動脈畸型瘤惡化 |
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作者 | 黃文賢; 鄭丞傑; 朱繼璋; 李新揚; | 書刊名 | 中華民國婦產科醫學會會刊雜誌 |
卷期 | 38:1 1999.03[民88.03] |
頁次 | 頁41-44 |
分類號 | 417.346 |
關鍵詞 | 肺部動靜脈畸型瘤; 遺傳出血性毛細管擴張; 妊娠; Pulmonary arteriovenous malformation; Hereditary hemorrhagic telangiectasia; Pregnancy; |
語文 | 英文(English) |
中文摘要 | 背景:肺動靜脈畸型瘤可見於全身性血管發育異常之自體顯性遺傳疾病, 如遺傳 出血性毛細管擴張,或是因為外傷及腫瘤所引起。而妊娠後的正常生理性改變通常會加重病 情,造成致命性的低血氧現象。在此我們報告一例妊娠合併遺傳出血性毛細管擴張症的多發 性肺動靜脈畸型瘤。 病例報告:一位 25 歲的年輕婦女於例行性職前胸部 X 光體檢中,偶 然發現肺部長有腫瘤,而被轉介至本院胸腔內科接受進一步診治。根據其自幼反覆性流鼻血 現象及相關家族史,診斷為遺傳出血性毛細管擴張症。 肺部血管 X 光攝影檢查發現雙側肺 葉共有 6 處動靜脈畸型瘤存在。4 處較大者經栓塞法治療後,症狀明顯改善。 在本次妊娠 約二十遇時,病人再度出現嚴重的流鼻血及吐血。 當時雖然建議病患接受胸部 X 光檢查但 遭拒絕,而保守性治療不僅未見療效且病程持續惡化。於妊娠滿三十七週時,孕婦接受建議 剖腹生產,生下一名重 2828 公克之健康女嬰。產後不久,其症狀未消。結論:由於事先存 在的肺部動靜脈畸型瘤常會因為妊娠而使症狀加劇,甚至引起反覆性早產現象,危及胎兒。 因此,凡此類病患計劃懷孕時,均應接受產前諮詢,並予以密切追蹤,以確保懷孕及生產安 全。 |
英文摘要 | Background: Pulmonary arteriovenous malformations (PAVMs) result from abnormal communications in pulmonary vasculature either due to congenital dysgenesis of the pulmonary capillary system as part of a syndrome of hereditary hemorrhagic telangiectasia (Osler-Rendu-Weber syndrome) or to acquired events such as trauma or neoplasm. The cardiovascular effects of pregnancy often increase and worsen the intrapulmonary shunt fraction and produce life-threatening hypoxemia. Here, we report on a case with deterioration of existent multiple PAVMs of hereditary hemorrhagic telangiectasia during pregnancy. Case Report: A 25-year-old woman was incidentally found to have a well-circumscribed and noncalcified pulmonary nodule. She had recurrent nasal bleeding since childhood and a positive family history, so hereditary hemorrhagic telangiectasia was diagnosed. A pulmonary angiogram clearly demonstrated 6 arteriovenous malformations throughout both lungs. Four were successfully treated with embolotherapy, resulting in dramatic improvement both in reduction of intrapulmonary shunt and symptom relief. Three years later, when she was 20 weeks pregnant, profuse epistaxis and massive hemoptysis occurred. Because of worsening condition, an elective primary low transverse cesarean section was performed at 37 weeks of gestation and a healthy, 2828g female infant was delivered. Conclusion: The presentation of existent PAVMs in pregnancy is unpredictable and pregnancy often exacerbates the outcome. Patients with multiple PAVMs should receive preconception counselling and close follow-up during pregnancy. |
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