Complete Pentalogy of Cantrell with craniorachischisis: a case report.
نویسندگان
چکیده
BACKGROUND Pentalogy of Cantrell is a rare malformation syndrome consisting of a specific combination of ventral midline defects, uncommonly found to be associated with other anomalies. CASE We report a case of complete Pentalogy of Cantrell with craniorachischisis diagnosed in-utero at 19 weeks of gestation through antenatal ultrasonography. Fetal autopsy following termination of the pregnancy confirmed the presence of the sonographically detected malformations and also revealed associated transposition of great vessels (TGV) in the fetus. CONCLUSION Co-occurrence of such ventral and dorsal midline defects suggests the possibility that common genetic and environmental factors influence the early stages of development of the ventral as well as dorsal embryonic midline.
منابع مشابه
Prenatal diagnosis of pentalogy of cantrell in a case with craniorachischisis
The pentalogy of Cantrell was first described by Cantrell et al in 1958, who reported 5 cases with this anomaly [1]. The findings included a lower sternum defect, a midline supraumblical thoracoabdominal wall defect, a defect of the anterior diaphragm, a defect of the diaphragmatic pericardium and congenital cardiac anomalies [1,2]. The pentalogy is associated with very poor prognosis and there...
متن کاملامفالوسل و قلب بیرون از قفسه سینه درنوزاد با Pentalogy of Cantrell
Background : Pentalogy of Cantrell is a very rare congenital anomaly which is diagnosed with severe thoracoabdominal defect, omphalocele, lower sternal defect, anterior diaphragmatic defect anterior pericardial defect and ectopia cordis.Pathogenesis is unknown, mesodermal developmental defects in 14-18 days after conception maybe responsible . Case Report: The case is newborn with omphalocele ...
متن کاملPrenatal diagnosis of pentalogy of cantrell with craniorachischisis by three-dimensional ultrasonography in the first trimester.
متن کامل
Partial Ectopia Cordis: A Case Report
Background: One-third of all major congenital anomalies are Congenital heart disease (CHD) and Reported CHD prevalence increased over time and in Asian countries is more than western countries. Ectopia cordis (EC) is a rare congenital anomaly with an estimated incidence of 1:100 000 live births in developed countries. EC is characterized by abnormal heart placement outside the thorax, mostly on...
متن کاملPentalogy of Cantrell associated with exencephaly and spinal dysraphism: antenatal ultrasonographic diagnosis. Case report.
The pentalogy of Cantrell, a rare syndrome, consists of the defects in the anterior diaphragm, diaphragmatic pericardium, lower sternum and supraumbilical abdominal wall, along with congenital cardiac abnormalities. Till date, only few patients with full spectrum of this syndrome have been reported with only 2 cases showing associated exencephaly and spinal dysraphism. We report extremely rare ...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- Journal of prenatal medicine
دوره 6 1 شماره
صفحات -
تاریخ انتشار 2012