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題 名 | Prenatal Diagnosis of Congenital Cystic Adenomatoid Malformation=產前診斷先天肺部腺體囊腫性構造異常 |
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作 者 | 許耀仁; 李信儀; | 書刊名 | 長庚醫學 |
卷 期 | 27:1 2004.01[民93.01] |
頁 次 | 頁61-65 |
分類號 | 417.132 |
關鍵詞 | 產前診斷; 先天肺部腺體囊腫性構造異常; Congenital cystic adenomatoid malformation; Prenatal diagnosis; |
語 文 | 英文(English) |
中文摘要 | 先天肺部腺體囊腫性構造異常乃一種罕見的肺部疾病。此一先天肺部構造異常,嚴重時可導伢胎兒於週產期死亡,亦可能自然於子宮內回復正常而不造成新川兒的罹病。我們報告一例於妊娠23週時經由產前二維及三維超音波診斷胎兒先天肺部腺體囊腫性構造異常。順利自然產後,安排肺部電腦斷層檢查亦發現新生兒右下肺部有多囊腫性構造異常。此新生兒於產後一週後接受右下肺葉切除,術後病理診斷為第二型先天肺部腺體囊腫性構造異常。由於先天肺部腺體囊腫緎構造異常可能導致胎兒於週產期死亡、反覆性肺部感染及轉變為惡性的可能性,故於產前診斷此一先天肺部構造異常並於產後儘快切除肺部病灶,可以得到良好的預後。 |
英文摘要 | Congenital cystic adenomatoid malformation (CCAM) of the lung is a rare pulmonary lesion. The prognosis is variable ranging from perinatal death to spontaneous in utero regression, with no neonatal morbidity. We present a case of CCAM diagnosed at 23 week’s gestation using results of prenatal sonograms. Both prenatal Two-Dimensional and Three-Dimensional sonograms revealed multiople cystic lesions at the right lower lung filed. Regular serial antenatal sonograms revealed the fetus had persistent right lower lung multicyst lesions but had no hydrops fetalis or associated congenital anomalies. The results of the antenatal 50 g glucose diabetic screen at 24 weeks’ gestation were normal. A live male baby was vaginally delivered smoothly at 40 weeks’ gestation. Body weight was 4170 g. Apgar scores were 7 at 1 minute and 8 at minutes. The newborn had no signs of respiratory distress at birth. Postnatal chest computed tomography (CT) also revealed multiple fluid-filled cysts in the right lower lobe compatible with congenital cystic adenomatoid malformation. Because of the potential risk of perinatal death, repeated lung infection and malignant change of CCAM, the newborn received right lower lung lobectomy 1 week after delivery. Pathology confirmed the diagnosis of CCAM type II. The recovery was smooth posoperatively and the baby still receives regular follow-up. We emphasize the importance of prenatal diagnosis of CCAM and early removal of the congenital cystic lesions of the lung to achieve a good outcome. |
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