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先天性右侧膈疝:一种异质性病变。

Congenital right-sided diaphragmatic hernia: a heterogeneous lesion.

作者信息

Khwaja M S, al-Arfaj A L, Dawoodu A H

出版信息

J R Coll Surg Edinb. 1989 Aug;34(4):219-22.

PMID:2810186
Abstract

A clinical and radiological analysis of 13 right-sided diaphragmatic hernias is described. Four anatomically distinct though clinically similar variants (Bochdalek; paraoesophageal; eventration and Morgagni) of equal frequency make it a heterogeneous disease. Associated anomalies, found in ten out of 13 (77%), are a strong diagnostic indicator. The presence of congenital hypertrophic pyloric stenosis in 25% of infants suggests a degree of predisposition. Delay in pre-admission diagnosis was a median of 23.5 weeks from onset of symptoms and median age of diagnosis was 9 months, despite the onset of symptoms in the first week of life. A significant perinatal morbidity emphasizes the need for early diagnosis. Possible diagnostic indicators are: multiple anomalies, including chromosomal disease; an early recurrence of apparently 'cured' respiratory infection; and early onset of gastric outlet obstruction. Barium studies are the mainstay of diagnosis and were needed in ten patients. Fundoplication is a useful deterrent to recurrence in paraoesophageal hernias.

摘要

本文描述了对13例右侧膈疝的临床和影像学分析。四种解剖结构不同但临床症状相似的类型(博赫dalek型;食管旁型;膈膨出和莫尔加尼型)出现频率相同,使其成为一种异质性疾病。13例中有10例(77%)存在相关异常,这是一个强有力的诊断指标。25%的婴儿患有先天性肥厚性幽门狭窄,提示存在一定程度的易患倾向。尽管症状在出生后第一周就已出现,但入院前诊断延迟的中位数为症状出现后23.5周,诊断时的中位年龄为9个月。围产期发病率较高,强调了早期诊断的必要性。可能的诊断指标包括:多种异常,包括染色体疾病;看似“治愈”的呼吸道感染早期复发;以及胃出口梗阻的早期发作。钡剂造影是诊断的主要手段,10例患者需要进行此项检查。胃底折叠术对预防食管旁疝复发很有用。

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