- 期刊
Delayed Manifestation of Congenital Diaphragmatic Hernia with Intrathoracic Kidney: Report of One Case
遲發性先天性橫膈疝氣合併胸腔內腎臓異位：一病例報告
摘要
胸腔內腎臓異位是一個非常罕見之先天性異常，大約只有五二䨉病例被報告於世界文獻上。它伴隨先天性橫膈疝氣(Bochdalek)之發生率約少於萬分之二十五。 本篇報告一例遲發性先天性橫膈疝氣合併左側胸腔內腎臓異位併有不完全集尿系統複製。然而左側胸腔內腎臓異位合併不完全集尿系統複製之出現較早於橫膈疝氣，且出生時之胸部X光正常。其它並存之先天性異常包括水腦和無肛併有會陰部瘻管。病人於九個月大時在台北馬偕醫院進行橫膈缺陷修補術及肛門整形術。日後，病人屢次因病毒性感染住進本院，最後，一歲大時死於肺炎。 經由此病例衍生出一問題“是否胎兒期之後腎於嬰兒後期在腹腔內仍繼續上昇而影響橫膈膜之發育？”本篇將一一討論之。
並列摘要
Intrathoracic kidney is a very rare congenital anomaly with only about 50 cases reported in the world literature. Incidence of intrathoracic kidney with Bochdalek hernias was reported to be less than 0.25 percent. The relationship between them remains uncertain. We report a case in which the patient had a normal chest x-ray at birth, but at the age of 5 months an isolated left sided intrathoracic kidney with incomplete duplication was discovered. This was followed by ipsilateral diaphragmatic hernia at the age of nine months. Other coexisting malformations included hydrocephalus and an imperforate anus with perineal fistula. A primary repair of the diaphragmatic defect and a minimal posterior sagittal anorectoplasty were carried out at the age of nine months. Since then, the patient has had repeated admissions due to viral infection. Eventually, she succumbed to pneumonia at the age of one year. This case raised the question ‘Does the ascent of metanephron continue postnatally and affect development of the diaphragm?”
延伸閱讀
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