Swarray-Deen Alim, Seffah Joseph D, Antwi-Agyei Deliverance A
Korle Bu Teaching Hospital - Obstetrics & Gynaecology Department, Korle Bu, Accra, Ghana.
University of Ghana Medical School - Obstetrics & Gynaecology Department, Accra, Ghana.
Ghana Med J. 2017 Dec;51(4):191-195.
Pentalogy of Cantrell is a rare congenital malformation syndrome that may be associated with other congenital anomalies. The syndrome is said to affect males and females in equal numbers and the prognosis is generally poor. We report two cases of Cantrell's Pentalogy diagnosed antenatally at the Korle Bu teaching Hospital. The 2 cases described in this report demonstrate the complete and incomplete variant of the syndrome. The cases were referred as an omphalocoele and a detailed anomaly scan confirmed the diagnosis of Pentalogy of Cantrell. Both cases were females and neither survived beyond 24 hours. Although this condition is associated with a poor prognosis, there have been some reported cases of survival in the literature. Thus, accurate prenatal diagnosis and a multidisciplinary approach to the management may offer improved outcomes.
坎特雷尔五联症是一种罕见的先天性畸形综合征,可能与其他先天性异常有关。据说该综合征在男性和女性中的发病率相等,且总体预后较差。我们报告在科勒布教学医院产前诊断出的两例坎特雷尔五联症病例。本报告中描述的这两例病例展示了该综合征的完整型和不完全型变体。病例最初被诊断为脐膨出,详细的畸形扫描确诊为坎特雷尔五联症。两例均为女性,均未存活超过24小时。尽管这种情况预后较差,但文献中已有一些存活病例的报道。因此,准确的产前诊断和多学科管理方法可能会改善预后。