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腹裂和脐膨出。八年回顾。

Gastroschisis and omphalocele. An eight-year review.

作者信息

Mayer T, Black R, Matlak M E, Johnson D G

出版信息

Ann Surg. 1980 Dec;192(6):783-7. doi: 10.1097/00000658-198012000-00015.

Abstract

Until recently confusion has existed concerning the clinical features and surgical treatment of gastroschisis and omphalocele. Since 1971 75 infants with these abdominal wall defects have been treated at our institution. Significant differences (p equal to 0.001 in all instances) were noted between the two diseases. Gastroschisis occurred twice as often as omphalocele and is increasing in frequency. Prematurity was commonly seen with gastroschisis (65%). While the overall incidence of malformations associated with gastroschisis was low (23%), the vast majority of the additional malformations were jejunoileal or colonic atresias. The mortality rate was 12.7% among gastroschisis patients, with only one death attributable to prematurity. All other deaths were preventable, indicating that even lower mortality rates are feasible. Omphalocele was associated with a 23% incidence of premature birth but associated anomalies were present in 66% of the patients. Major cardiac (52%) and chromosomal defects (40%) predominated. In addition, 17% of omphalocele patients had either Cantrell's pentalogy or cloacal/bladder exstrophy. The mortality rate in omphalocele (34%) was nearly three times that of gastroschisis. Nine of ten patients who died from omphalocele died either from major cardiac or chromosomal disease. However, in patients without cardiac or chromosomal defects the survival rate was 94%.

摘要

直到最近,关于腹裂和脐膨出的临床特征及外科治疗仍存在混淆。自1971年以来,我院已治疗了75例患有这些腹壁缺陷的婴儿。发现这两种疾病之间存在显著差异(所有情况下p均等于0.001)。腹裂的发生率是脐膨出的两倍,且呈上升趋势。腹裂患儿常见早产(65%)。虽然与腹裂相关的畸形总体发生率较低(23%),但绝大多数其他畸形为空肠或结肠闭锁。腹裂患者的死亡率为12.7%,仅1例死亡归因于早产。所有其他死亡均是可预防的,这表明更低的死亡率是可行的。脐膨出与23%的早产发生率相关,但66%的患者存在相关异常。主要是心脏畸形(52%)和染色体缺陷(40%)。此外,17%的脐膨出患者患有坎特雷尔五联症或泄殖腔/膀胱外翻。脐膨出的死亡率(34%)几乎是腹裂的三倍。死于脐膨出的患者中,十分之九死于主要心脏疾病或染色体疾病。然而,在无心脏或染色体缺陷的患者中,存活率为94%。

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