ABSTRACT
La pentalogía de Cantrell es una rara anomalía congénita caracterizada por la asociación de ectopia cordis con defectos en la pared toracoabdominal, el diafragma, el esternón y pericárdicos, y anomalías cardíacas intrínsecas. En diagnóstico prenatal, la ecografía se utiliza sistemáticamente entre las 11 y 14 semanas de gestación, evaluando marcadores de alteraciones cromosómicas como la sonolucencia nucal, el hueso nasal y la morfología patológica del ductus venoso, entre otros. Además, permite examinar la anatomía fetal y diagnosticar anomalías mayores, como acrania-anencefalia, holoprosencefalia, defectos de la pared abdominal y toracoabdominal, entre los que se incluye la pentalogía de Cantrell. Se reporta un feto con los hallazgos clásicos de pentalogía de Cantrell, que fue expulsado a las 13 semanas de gestación bajo protocolo de interrupción voluntaria del embarazo. Madre de 23 años, G1P0, sin exposiciones teratogénicas, en cuyo feto se encontró ectopia cordis, asas intestinales e hígado por fuera de la cavidad abdominal en las 10 y 12 semanas de gestación. El objetivo de este estudio es aportar a la literatura un reporte de pentalogía de Cantrell, siendo el primero reportado en Colombia en el primer trimestre de gestación, mostrando la importancia de la ecografía sistemática durante este periodo, en el marco de la posibilidad de interrupción voluntaria del embarazo.
Cantrells pentalogy is a rare congenital anomaly characterized by the association of ectopia cordis with intrinsic cardiac anomalies and various anatomical defects found in the thoracoabdominal wall, diaphragm, sternum and pericardium. Ultrasound is used routinely between 11 and 14 weeks of gestation during prenatal diagnosis. It evaluates markers of chromosomal alterations such as nuchal sonolucency, the nasal bone, and the pathological morphology of the ductus venosus, among others. Furthermore, it allows the diagnosis of altered fetal anatomy and major abnormalities such as acrania-anencephaly, holoprosencephaly, abdominal and thoraco-abdominal wall defects including Cantrells pentalogy. In this case report, we present a fetus with the classic findings of Cantrells pentalogy, which was expelled during the 13th week of gestation under the protocol of voluntary interruption of pregnancy. The mother, a 23-year-old woman, G1P0, without teratogenic exposures, in whom during the routine ultrasound of the 10th and 12th weeks of gestation ectopia cordis, intestinal loops and liver outside the abdominal cavity were found on the fetus. The main objective of this study is to contribute to the literature a case report of pentalogy of Cantrell, diagnosed through prenatal ultrasound, being the first reported in Colombia during first trimester of gestation, showing the importance of routine ultrasound, in the context of access to a voluntary termination of pregnancy.
Subject(s)
Humans , Female , Pregnancy , Young Adult , Pentalogy of Cantrell/diagnostic imaging , Pregnancy Trimester, First , Ultrasonography, Prenatal , Abortion, Induced , Ectopia Cordis/etiology , Pentalogy of Cantrell/surgery , Pentalogy of Cantrell/complicationsSubject(s)
Humans , Female , Infant , Ectopia Cordis/diagnostic imaging , Congenital Abnormalities , Radiography, Thoracic , Coronary AngiographyABSTRACT
Abstract Pentalogy of Cantrell (PC) is a rare congenital anomaly characterized by changes in the mesodermal median structures and congenital heart disease, often with a poor prognosis. In 1958, Cantrell et al2 defined the full spectrum of the syndrome with the following anomalies: defects of the anterior diaphragm, of the lower part of the sternum, of the supraumbilical region and the abdominal wall, of the diaphragmatic pericardium, and various intracardiac congenital abnormalities. The present report describes a case of ectopia cordis associated with PC and the importance of the participation of a multidisciplinary team in the treatment of this condition.
Resumo A pentalogia de Cantrell (PC) é uma rara anomalia congênita caracterizada por alterações nas estruturas medianas mesodérmicas e doenças cardíacas congênitas, cursando muitas vezes com um mau prognóstico. Em 1958, Cantrell et al2 definiram o espectro completo da síndrome com as seguintes anomalias: defeitos do diafragma anterior, da parte inferior do esterno, da região supraumbilical e parede abdominal, do pericárdio diafragmático, e várias anormalidades congênitas intracardíacas. O presente relato relaciona-se a um caso de ectopia cordis associado à PC e à importância da participação de uma equipe multidisciplinar no acompanhamento da doença.
Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Ultrasonography, Prenatal , Pentalogy of Cantrell/diagnostic imaging , Magnetic Resonance Imaging , Fatal Outcome , Diagnosis, Differential , Ectopia Cordis/diagnostic imaging , Heart Defects, Congenital/diagnostic imagingABSTRACT
@#Ectopia cordis (EC) is a rare congenital malformation characterized by a complete or partial extrathoracic presentation of the heart,1 2 with an incidence of only 6 to 8 in a million live births globally.3 4 It occurs due to failure of maturation of the midline mesoderm and improper formation of the chest and abdomen during embryonic development.5 6 Depending on the heart’s ectopic location, EC is classified into four types—i.e., thoracic (65% of cases), thoracoabdominal (20%), abdominal (10%), and cervical (5%).1 2 4 7 Only two cases of EC, one thoracic and one thoracoabdominal, from the Philippines had been reported in literature.8 Cantrell’s pentalogy—findings of bifid sternum, absence of the diaphragm, defect of anterior diaphragmatic pericardium, defect of the anterior abdominal wall, and intracardiac defects—usually accompanies the thoracoabdominal type of EC.
Subject(s)
Ectopia CordisABSTRACT
Ectopia cordis es el desplazamiento completo o parcial del corazón fuera de la cavidad torácica, producido por un defecto congénito en la fusión de la pared torácica anterior dando como resultado una ubicación torácica adicional del corazón. Su prevalencia estimada es de 5.5-7.9 por millón de nacidos vivos. Se presenta el caso de un recién nacido varón de 15 h de edad, que pesaba 2.25 Kg, con ectopia cordis, con signos de dificultad respiratoria y cianosis periférica. El paciente fallece antes de que cualquier intervención quirúrgica se pudiera llevar a cabo
Ectopia cordis is the complete or partial displacement of the heart outside the thoracic cavity, produced by a congenital defect in the fusion of the anterior chest wall resulting in an additional thoracic location of the heart. Its estimated prevalence is 5.5-7.9 per million live births. We present the case of a 15-h-old male newborn, weighing 2.25 kg, with ectopia cordis, with signs of respiratory distress and peripheral cyanosis. The patient dies before any surgical intervention can be carried out
Subject(s)
Humans , Male , Infant, Newborn , Congenital Abnormalities , Ectopia Cordis , Heart Defects, Congenital , Early Neonatal MortalityABSTRACT
La pentalogía de Cantrell es una enfermedad muy poco frecuente sobre todo en embarazos múltiples. Está caracterizado por presentar malformaciones integradas por defectos de cinco anomalías: tercio inferior del esternón, defecto epigástrico de la línea media abdominal, alteración del segmento anterior del diafragma, defectos pericárdicos y malformaciones cardiacas. Se reporta un caso en una paciente primigesta de 22 años de edad y embarazo gemelar que acude para su control ecográfico a las 27,3 semanas de gestación, en donde se encuentran los siguientes hallazgos ecográficos: anencefalia, anormalidad facial y corazón fuera de la cavidad torácica. Se da el nacimiento de los gemelos por vía cesárea a las 35 semanas: el primero de aspecto normal y el segundo con Pentalogía de Cantrell y otras malformaciones asociadas.
Pentalogy of Cantrell is a low frequency disease within multiple pregnancy, characterized with malformations with five anomalies defects: lower sternal defect, midline supraumbilical abdominal wall defect, diaphragmatic pericardial defect, anterior diaphragmatic defect and various intracardiac malformations. We report a case in a patient in her first pregnancy of 22 year old, twin pregnancy. She is 27, 3 weeks pregnant, comes for ultrasound control of the gestation, where the following ultrasound results are found: anencephaly, facial abnormalities and the heart out of the chest cavity. The birth of the twins is given via caesarian section at 35 weeks; the first of normal appearance and the second with Pentalogy of Cantrell and other associated malformations
Subject(s)
Humans , Male , Female , Pregnancy , Infant, Newborn , Adult , Young Adult , Ectopia Cordis/diagnostic imaging , Pentalogy of Cantrell/diagnostic imaging , Tetralogy of Fallot/diagnostic imaging , Fatal Outcome , Pregnancy, TwinABSTRACT
Ectopia cordis is a rare congenital malformation, with an estimated incidence of 5 to 8 per million live births. It is defined as a malformation in which the heart is located in an extra-thoracic position. Ectopia cordis may occur as an isolated malformation or associated with other anomalies such as omphalocele, congenital heart disease or integrating Cantrell syndrome. The size and location of the defect influence the prognosis. Description: we report a case of a 24-year-old nulliparous woman, with no relevant family or personal history, in which the prenatal fetal ultrasound, performed at 21 weeks of gestation, revealed adefect of the anterior chest wall with exteriorization of the heart. Discussion: fetal echocardiography revealed a severe congenital heart disease. The parents decided to continue the pregnancy, after being duly informed by a multidisciplinary team. Delivery occurred at 37 weeks of gestation but the female newborn died one hour afterwards. Pathological examination confirmed the sonographic findings...
A ectopia cordis é uma malformação congênita rara, com uma incidência estimada de 5 a 8 por milhão de nados vivos. Define-se como uma malformação em que o coração se localiza numa posição extratorácica. Pode surgir como malformação isolada ou associada a outras anomalias como onfalocelo, doença cardíaca congênita ou integrando o síndrome de Cantrell. A dimensão e o local do defeito influenciam o prognóstico. Descrição: descreve-se um caso de uma mulher de 24 anos, nulípara, sem antecedentes pessoais oufamiliares relevantes, em que a ultrassonografiaobstétrica, realizada às 21 semanas, revelou um defeito da parede torácica anterior com exteriorização do coração. Discussão: o ecocardiograma fetal revelou uma cardiopatia congênita grave. Os pais decidiram continuar com a gravidez, após de devidamente informados por uma equipe multidisciplinar. O partoocorreu às 37 semanas, tendo o recém-nascido falecido cerca de 1 hora após o mesmo. O estudo anatomopatológico confirmou os achados ultrassonográficos...
Subject(s)
Humans , Female , Pregnancy , Heart Defects, Congenital , Prenatal Care , Ectopia Cordis/diagnosis , Ectopia Cordis , Ultrasonography, PrenatalABSTRACT
We report a case of sternal reconstruction using bilateral sternal bar turnover flaps in a 4-year-old boy with an inferior sternal cleft, as part of Cantrell's pentad. When the patient was 10 months old, he underwent sternal reconstruction using a resorbable poly-L-lactic-polyglycolic acid plate in the first stage when there was insufficient autogenous tissue to provide a reliable reconstruction. Bilateral sternal bar turnover was performed in the second stage at 4 years of age. This operative technique is described in this report. This novel technique provides a robust, dynamic, and reliable reconstruction for inferior sternal defects.
Subject(s)
Child, Preschool , Humans , Male , Ectopia Cordis , Pentalogy of CantrellABSTRACT
A rare congenital defect in fusion of the anterior chest wall resulting in an extrathoracic location of the heart. Cantrell’s pentalogy is a congenital anomaly resulting from embryologic development defect and consists of the following: A deficiency of the anterior diaphragm, a midline supraumbilical abdominal wall defect, a defect in the diaphragmatic pericardium, congenital intracardiac abnormalities, and a defect of the lower sternum. Here
Subject(s)
Adult , Ectopia Cordis/epidemiology , Ectopia Cordis/genetics , Female , Hernia, Umbilical/epidemiology , Hernia, Umbilical/genetics , Hernia, Umbilical/mortality , Humans , Infant, Newborn , Infant, Premature , Male , StillbirthABSTRACT
La ectopia cordis (EC) es una rara malformación congénita, definida por un desplazamiento total o parcial del corazón fuera de la cavidad torácica, con incidencia entre 5,5-7,9 casos por millón de recién nacidos vivos. Objetivo: Divulgar caso clínico de relevancia. Métodos: Caso clínico: Primigesta de 22 años con 22 semanas de amenorrea, quien acudió referida de la consulta prenatal por presentar ecograma morfofenotípico que evidencia útero grávido con feto podálico de sexo masculino, con deformidad de bóveda craneana, dilatación ventricular e hidrocefalia; y corazón localizado fuera del tórax con latidos arrítmicos. La evaluación anatomopatológica reveló feto de sexo masculino, peso: 600 gramos y talla: 33 cms, con: hidrocefalia, corazón extratorácico, comunicación interauricular e interventricular, ausencia de pericardio, defecto del esternón, labio y paladar hendido, y espina bifida. Conclusiones: El diagnóstico prenatal ha tenido enormes avances con el uso del ultrasonido; un ejemplo es la EC, la cual puede ser identificada desde el primer trimestre de la gestación(AU)
Ectopia cordis (EC) is a rare congenital malformation, defined by a total or partial displacement of the heart outside the thoracic cavity; its incidence is within 5,5-7,9 cases per million alive newborns. Objectives: To disclose clinical case of relevance. Methods: Case report: primigravida 22 years old and 22 weeks of amenorrhea, who went referring to the prenatal visit by presenting evidence that echogram uterus gravid with male fetus, who present deformity of cranial vault, ventricular dilatation and hydrocephalus; heart located outside the chest with arrhythmic beats. The assessment revealed pathologic male fetus, weight: 600 grams and size: 33 cm, showing: hydrocephalus, heart extra thoracic, with interventricular and interauricular communication, absence of the pericardium, defects of the sternum, lip and cleft palate, and spina bifida Conclusión: Prenatal diagnosis has been enormous progress with the use of ultrasound; one example is the EC, which can be identified from the first trimester of pregnancy
Subject(s)
Humans , Male , Ultrasonography , Dilatation and Curettage , Ectopia Cordis/physiopathology , Heart Defects, Congenital , Gynecologic Surgical Procedures , Congenital Abnormalities , HydrocephalusABSTRACT
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...
Subject(s)
Humans , Male , Infant, Newborn , Congenital Abnormalities , Hernia, Umbilical , Pentalogy of Cantrell/diagnosis , Ectopia Cordis/complications , Heart Defects, CongenitalABSTRACT
Trata-se de doença congênita, descrita por Cantrell, em 1958, caracterizada por ectopia cordis e malformações da parede abdominal. A prevalência é um em cada 65.000 nascidos vivos. Existem, aproximadamente, 900 casos descritos. O prognóstico é reservado, depende do grau de malformação intracardíaca e malformações associadas, além do grau de exposição do coração. A maioria das crianças falece nas primeiras horas de vida. A criança deste relato apresenta forma completa da síndrome de Cantrell. Infelizmente, a correção cirúrgica da onfalocele não pôde ser realizada, devido ao trabalho de parto precoce e morte da criança.
Subject(s)
Animals , Female , Infant, Newborn , Heart Defects, Congenital/complications , Ectopia Cordis/genetics , Abdominal Wall/abnormalitiesABSTRACT
Supernumerary ectopic limb(s) (SEL) is a congenital anomaly defined as the presence of accessory limb(s) attached to various body regions. This paper describes a case of SEL with ectopic lung and ectopia cordis in a newborn calf, based on macroscopic, microscopic and radiographic findings. External features of multiple congenital anomalies included an ectopic lung growing over the middle of the backbone and covered with normal haired skin. Ectopia cordis was found in the abdominal cavity and attached to the liver. Two extra abnormal limbs originated separately from within the ectopic lung. Most of the abdominal organs were exposed to the outside through the opened abdominal cavity. Microscopically the ectopic lung tissue had edema in the connective tissue around the bronchus and artery. Changes in other organs included congestion of the renal medulla, infiltration of inflammatory cells (lymphocytes and eosinophils) around the hepatic portal tract, and edema surrounding blood vessels and neurons in the brain. The rudimentary humerus of the forelimb was attached to the thoracic spine, as viewed radiographically. The hindlimb was consisted of an irregularly shaped femur, short tibia and fibula, two tarsal bones, one metatarsal bone, and three phalanges. This is the first description of congenital anomalies involving the SEL, ectopic lung and ectopia cordis in a calf.
Subject(s)
Animals , Humans , Infant, Newborn , Abdominal Cavity , Arteries , Blood Vessels , Body Regions , Brain , Bronchi , Connective Tissue , Ectopia Cordis , Edema , Estrogens, Conjugated (USP) , Extremities , Femur , Fibula , Forelimb , Hair , Hindlimb , Humerus , Liver , Lung , Metatarsal Bones , Neurons , Skin , Spine , Tarsal Bones , TibiaABSTRACT
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
La pentalogía de Cantrell es un síndrome que se caracteriza por cinco anomalías: un defecto de la pared abdominal en la línea media, un defecto esternal inferior, un defecto en el diafragma anterior, un defecto pericárdico diafragmático y anormalidades congénitas del corazón. Se presenta con una prevalencia de 1 en 65.000 nacidos vivos y con una baja tasa de supervivencia, la cual depende de la presencia de todos los defectos inherentes a este síndrome o la gravedad de las anomalías cardiacas. El diagnóstico puede hacerse por ecografía obstétrica en el primer trimestre. Para la atención posnatal, las imágenes por tomografía computadorizada y la resonancia magnética se recomiendan para definir claramente la extensión del defecto y para la corrección quirúrgica. Se reporta un caso producto de padres consanguíneos.
Pentalogy of Cantrell is a syndrome evidencing five anomalies: a midline, upper abdominal wall abnormality; lower sternal defect; anterior diaphragmatic defect; diaphragmatic pericardial defect, and congenital abnormalities of the heart. Its prevalence is one in every 65,000 live births and a survival rate that is low if the fall the five defects are present or the gravity of the cardiac anomalies. It may be diagnosed during the first trimester obstetric ultrasound. For postnatal care, emission-computed tomography and magnetic resonance imaging is recommended for a clear definition of the extent of the defect and to design a course of corrective surgery. Herein, a case of pentology of Cantrell is reported for a child offspring of consanguineous parents.
Subject(s)
Humans , Congenital Abnormalities , Ectopia Cordis , Hernia, Diaphragmatic , Inheritance Patterns , Magnetic Resonance Imaging , PericardiumABSTRACT
El misoprostol es un análogo sintético de la prostaglandina E1 y es usada por sus efectos utero-tónicos como abortivo. En los países donde se utiliza como abortivo sin éxito durante el primer trimestre de gestación, se han informado un espectro de malformaciones congenitas asociadas con la exposición prenatal, que incluyen el síndrome de Moebius, defectos de la pared abdominal, artrogriposis y anomalías por reducción de los miembros, entre otros. La pentalogía de Cantrell (PC), con una prevalencia de 1 en 65.000 nacidos vivos, es un síndrome polimalformativo que consta de 5 anomalías: un defecto de la pared abdominal, defecto esternal inferior, defecto del diafragma anterior, defecto pericárdico diafragmático y anormalidades congenitas del corazón. Se reporta un caso de pentalogía de Cantrell asociado al uso de misoprostol en el primer trimestre de la gestación, siendo este reporte el primer registro de esta asociación.
Misoprostol is a synthetic analogue of prostaglandin E1 and is used for effects such as uterotonic aborti-facient. In countries where abortion is used as unsuccessful in the first trimester of gestation, reported a spectrum of congenital malformations associated with prenatal exposure, which include Moebius syndrome, abdominal wall defects, and anomalies as artrogriposis reduction members, among others. The pentalogy of Cantrell with a prevalence of 1 in 65,000 live births, is a syndrome consisting of 5 abnormalities: a defect in the abdominal wall, lower sternal defect, defect above the diaphragm, diaphragmatic pericardial defect, and congenital abnormalities of the heart. We report a case of Pentalogy of Cantrell associated with the use of misoprostol in the first trimester of pregnancy, this report is the first record of this association.
Subject(s)
Humans , Female , Pregnancy , Infant, Newborn , Adult , Abnormalities, Drug-Induced , Abortifacient Agents, Nonsteroidal/adverse effects , Misoprostol/adverse effects , Prenatal Exposure Delayed Effects , Abnormalities, Multiple/chemically induced , Ectopia Cordis/chemically induced , Sternum/abnormalities , Fatal Outcome , Intestines/abnormalities , Abdominal Wall/abnormalitiesABSTRACT
La pentalogía de Cantrell es una enfermedad rara, con características clínicas, anatómicas y embriológicas peculiares. Esta entidad es una variedad toracoabdominal de la ectopia cordis en la que se asocian cinco anomalías: defecto epigástrico de la línea media abdominal supraumbilical, defecto del tercio inferior del esternón, deficiencia del segmento anterior del diafragma, defectos pericárdicos y malformaciones cardíacas congénitas. La comunicación interventricular e interauricular se encuentra en la mitad de los casos y puede asociarse a otras anomalías extracardíacas como espina bífida, diversas malformaciones del sistema nervioso central, divertículo de Meckel, poliesplenia, entre otros. El diagnóstico se hace mediante ultrasonografía fetal en el primer trimestre de la gestación; en casos incompletos la tomografía helicoidal y la resonancia magnética complementan el diagnóstico. El tratamiento es quirúrgico y en varias etapas, lo primordial es proteger las vísceras expuestas y corregir la cardiopatía congénita. Los resultados aún son controversiales. El pronóstico de la enfermedad depende de la extensión del defecto, el diagnóstico temprano y el manejo perioperatorio. Nosotros consideramos que la pentalogía de Cantrell es una enfermedad compleja en la que es necesaria la intervención multidisciplinaria del equipo de salud para llegar al diagnóstico preciso y decidir el mejor tratamiento.
The pentalogy of Cantrell is a rare disease, with uniqueclinical, anatomical and embryological characteristics. This disease is a thoraco-abdominal variety of ectopia cordis. Five anomalies are associated: epigastric defect of theover umbilical abdominal mean line, defect of inferiorbreastbone third, defect of the anterior segment of the diaphragm, pericardial defects and congenital cardiac malformations. Ventricular septal defect and atrial septal defects are in half of the cases and it could be associatedto other extra cardiac anomalies as bifid spine, several malformations of central nervous system, meckeldiverticulum and polispleenia, among others. The diagnosis is made by fetal ultrasonography in the first trimesterof pregnancy. In incomplete cases, helical tomography and magnetic resonance imaging complete the diagnosis.The treatment is surgical and is carried out in severalstages. It is fundamental to protect the exposed viscera and to correct the congenital heart disease. The results still are controversial. The prognosis of the disease depends on the defect extension, time of diagnosis and peri-operative handling. We considered that the pentalogyof Cantrell is a complex pathology. A multidisciplinary health team intervention is necessary to reacha precise diagnosis and to decide the best treatment.
Subject(s)
Ectopia Cordis , Pentalogy of Cantrell/diagnosis , Heart Diseases , Magnetic Resonance Spectroscopy , Rare Diseases , Ultrasonography, PrenatalABSTRACT
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.