ABSTRACT
@#Sternal cleft is a rare congenital anomaly that is known to be associated with other congenital abnormalities in particular coarctation of aorta and vascular anomalies. We present a case of a young teacher who presented with recurrent episodes of ischemic stroke. During the workup to look for the cause of stroke, it was discovered that this patient had complete midline sternal cleft with underlying coarctation of aorta and left subclavian artery aneurysm. We wish to highlight the relation between the presence of sternal cleft and coarctation of aorta as well as vascular anomalies as one of the rare cause of young stroke.
ABSTRACT
Se presentó el caso de un recién nacido masculino de 48 horas, de un parto vaginal extrahospitalario no controlado, de una comunidad indígena, remitido de un hospital público de Maturín, Monagas, Venezuela, al Servicio de Imagenología del Centro de Alta Tecnología Ernesto Che Guevara; por tener malformación de la pared torácica (onfalocele). Se le realizó tomografía axial computarizada (TAC) de tórax en equipo multicortes, con reconstrucciones multiplanares (2D, 3D) y en volumen rendering; con el objetivo de definir otras malformaciones asociadas, con un diagnóstico preciso para la corrección quirúrgica precoz del defecto y el desarrollo normal de la caja torácica. La TAC mostró un defecto de fusión total de las valvas esternales, que se correspondía con una hendidura esternal, asociada a dextrocardia y onfalocele.
A case of a 48-hour male newborn, from an extra-hospital vaginal delivery, with not controlled pregnancy, from an indigenous community, transfered from a public hospital in Maturín, Monagas, Venezuela to the Imaging Service of the Ernesto Che Guevara High Tech Center. He presented chest wall malformation (omphalocele). Thorax Computerized Axial Tomography (CAT) was performed in multislice equipment, with multiplanar reconstructions (2D, 3D) and in rendering volume, to define associated malformations for an accurate diagnosis, early surgical correction which allows normal rib cage development. The CAT showed a total fusion of the sternal leaflets, related to a sternal cleft with dextrocardia and omphalocele.
ABSTRACT
La pentalogía de Cantrell involucra defectos de la línea media abdominal supraumbilical, parte inferior del esternón, diafragma anterior, pericardio diafragmático y malformaciones congénitas intracardiacas (1, 2). Su pronóstico está supeditado al diagnóstico temprano, la severidad de la malformación cardiaca y las anomalías asociadas (3). Se reporta el caso de una gestante de 28 semanas con diagnóstico fetal de pentalogía de Cantrell: ectopia cordis verdadera toraco-abdominal (con anomalía intracardiaca) y fisura esternal, y se resalta el diagnóstico prenatal que permitió definir su pronóstico y planear el manejo.
Pentalogy of Cantrell involves defects of the midlline supraumbilical abdomen, lower sternum, anterior diaphragm, diaphragmatic pericardium and intracardiac congenital defects. Prognosis depends on early diagnosis, severity of cardiac malformation and associated anomalies. We report the case of a 28 weeks pregnant woman with fetal diagnosis of pentalogy of Cantrell: true thoraco-abdominal ectopia cordis (with intracardiac anomaly) and sternal cleft, and highlight prenatal diagnosis that allowed to define prognosis and plan management.
Subject(s)
Ectopia Cordis , Hernia, Umbilical , Prenatal DiagnosisABSTRACT
Ectopia cordis is a rare congenital abnormality characterised by partial or complete displacement of the heart outside the thoracic cavity. It can be associated with other congenital abnormalities. The present case was a two hours old male child and a product of non-consanguineous marriage. Child had thoraco-abdominal ectopia cordis. Condition of child detoriated rapidly and succumbed before any investigation and surgical intervention could be performed.