First trimester sonographic diagnosis of limb-body wall defect associating both cephalic and thoraco-abdominal defects - a case report and literature update.

We present the first trimester prenatal ultrasonography and pathological assessment of a case diagnosed with limb-body wall complex (LBWC) presenting both exenchephaly and a complex thoraco-abdominal wall defect. Ectopia cordis is demonstrated with a movie showing the heart beating outside the body of the fetus after its expulsion. Also, we discuss the pathogenesis and possible etiology of LBWC and associated malformations and we provide an update of the literature of this very rare anomaly.

Management of Pentalogy of Cantrell with complete ectopia cordis and Double Outlet Right Ventricle.

Pentalogy of Cantrell (PoC) is a rare congenital midline defect. We present a case and its treatment of PoC with complete ectopia cordis and congenital heart disease. Postnatally the congenital heart defect was surgically corrected and the ectopic heart was covered by musculous mobilized flap. Due to cephalic orientation of the heart and limited intrathoracic space, replacement of the heart into the thoracic cavity was initially not performed. After 11 years of follow up our patient now is without relevant limitations solely wearing a thoracic shelter. This case elucidates the complexity of further management. The potential risk of disastrous hemodynamic compromise by intrathoracic shift is to compare with the limited safety of the ectopic heart.

Pentalogía de Cantrell: presentación de caso clínico / Pentalogy of Cantrell. Clinical case

La pentalogía de Cantrell es un síndrome congénito raro que incluye: a) defecto en la pared abdominal ventral (frecuentemente onfalocele); b) ectopia cordis;c) anomalías cardíacas comunicación interauricular (CIA), comunicación interventricular(CIV), tetralogía de Fallot, estenosis pulmonar, divertículo ventricular y/o anomalía del retorno venoso); d) defecto del aporción inferior del esternón y e) defecto del diafragma anterior. La probabilidad de sobrevida está relacionada al grado de afección cardíaca. Se presenta el caso de un recién nacido masculino, hijo de madre de 23 años, primigesta, 35.5 semanas de gestación, sin antecedentes de uso de misoprostol, el nacimiento fue vía vaginal en el Hospital Mario Catarino Rivas de San Pedro Sula, Honduras, con APGAR 7 y 8 al primer y quinto minuto respectivamente. Al examen físico presentaba onfalocele, fue ingresado al área de cuidados intermedios de sala cuna, presentó falla respiratoria desde el primer día de vida, se realizó intubación con tubo endotraqueal número 3.5, trasladándose a la unidad de cuidados intensivos neonatales (UCIN) y se acopló a ventilador mecánico, posteriormente se interconsultó con el cirujano pediatra quien observó: hígado y bazo dentro del defecto abdominal, ectopiacordis, defecto de la porción baja esternal y defecto diafragmático inferior, diagnosticando clínicamente la pentalogía de Cantrell. Al tercer día de vida fue trasladado al hospital del Instituto Hondureño del Seguro Social en San Pedro Sula; continúo su atención en UCIN, fue presentado al cardiólogo pediatra quien realizó ecocardiograma en contrando: tronco arterioso común, obstrucción pulmonar, CIA, CIV grande y persistencia...

[Cardiac malformations in patients with pentalogy of Cantrell and ectopia cordis]. / Malformaciones cardiacas en pacientes con pentalogía de Cantrell y ectopia cordis.

We studied 21 patients who presented with a diagnosis of pentalogy of Cantrell. Their mean age was 40 days. All patients presented with congenital heart disease. Six presented with ectopia cordis. Every patient was subjected to echocardiography. Double outlet right ventricle, an atrial septal defect and dextrocardia were seen in 5 patients (24%). Four patients survived. Seventeen died, 12 from sepsis or septic shock. Autopsies were performed on 10 patients. All of the ectopia cordis patients died. Pentalogy of Cantrell is uncommon, and its association with ectopia cordis indicates poor prognosis. Full English text available from: www.revespcardiol.org.

First trimester sonographic diagnosis of ectopia cordis: a case report and review of the literature.

A case of ectopia cordis (EC) with gastroschisis in a 27-year-old primigravida was diagnosed at 10(3/7) weeks of gestation. The pregnancy was terminated by suction dilatation and curettage. With the increasing use of first trimester ultrasonography, early detection of fetal abnormalities is becoming more frequent. We review other published cases of EC detected in the first trimester and discuss the possible advantages of early diagnosis including options of termination at earlier gestational ages which might decrease the physical and psychological trauma on some patients.

Ectopia cordis with tetralogy of Fallot in an infant with pentalogy of Cantrell: high-pitch MDCT exam.

We report the MDCT findings of a 17-month-old girl with Cantrell's pentalogy, a rare congenital disease characterized by several defects in the ventral thoracoabdominal wall including ectopia cordis, and, in this patient, associated with tetralogy of Fallot. This case provides an example of the utility of a wide volume in coverage and high-pitch MDCT scan in the evaluation of complex cardiovascular anatomy in infants with congenital heart disease without the need of an ECG-gating acquisition.

Fontan operation through a right lateral thoracotomy to treat Cantrell syndrome with severe ectopia cordis.

A median sternotomy would be very difficult for Cantrell syndrome with severe ectopia cordis. For Cantrell syndrome and tricuspid atresia after left modified Blalock-Taussig shunt with severe ectopia cordis, defect in the middle and inferior portion of the sternum, and the closing of ventriculo-peritoneal shunt, we performed extracardiac total cavopulmonary connection through a right lateral thoracotomy after establishing right modified Blalock-Taussig shunt and performing coil embolization of left modified Blalock-Taussig shunt by cardiologists.