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1.
JNMA J Nepal Med Assoc ; 60(246): 183-186, 2022 Feb 15.
Artigo em Inglês | MEDLINE | ID: mdl-35210636

RESUMO

Ectopia cordis is a rare congenital defect with the prevalence of 5 to 8 per million live births. Here we report a rare case of preterm female live birth with ectopia cordis associated with omphalocele, cleft lip, and palate. In this case, 14+ weeks ultrasound did not show any fetal abnormalities and parents were unaware of the condition until 35+ weeks when ultrasound detected the anomaly a few days before delivery. After delivery, they didn't give consent for further intervention which led to neonatal mortality 3 hours after birth. If the condition was diagnosed in time, an earlier intervention could have been done.


Assuntos
Fenda Labial , Fissura Palatina , Ectopia Cordis , Hérnia Umbilical , Fenda Labial/complicações , Fenda Labial/diagnóstico por imagem , Fissura Palatina/complicações , Fissura Palatina/diagnóstico por imagem , Ectopia Cordis/diagnóstico por imagem , Ectopia Cordis/cirurgia , Feminino , Hérnia Umbilical/diagnóstico , Hérnia Umbilical/diagnóstico por imagem , Humanos , Lactente , Recém-Nascido , Palato , Gravidez , Ultrassonografia Pré-Natal
2.
World J Pediatr Congenit Heart Surg ; 13(1): 94-96, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33956542

RESUMO

A neonate with thoracic ectopia cordis presented following an uncomplicated delivery. A mandibular distractor was placed to bridge the sternal cleft and retracted (reverse distractor activation) over 24 days to facilitate sternal closure. Follow-up at five years postoperatively demonstrated a well-healed sternum. This novel approach to ectopia cordis repair facilitates slow, steady physiologic accommodation of the heart without hemodynamic instability or long-term complications.


Assuntos
Ectopia Cordis , Cardiopatias Congênitas , Anormalidades Musculoesqueléticas , Ectopia Cordis/diagnóstico por imagem , Ectopia Cordis/cirurgia , Seguimentos , Cardiopatias Congênitas/diagnóstico por imagem , Cardiopatias Congênitas/cirurgia , Humanos , Recém-Nascido , Esterno/anormalidades , Esterno/diagnóstico por imagem , Esterno/cirurgia
3.
Pan Afr Med J ; 39: 233, 2021.
Artigo em Francês | MEDLINE | ID: mdl-34659606

RESUMO

Sternal agenesis as well as ectopia cordis are extremely rare congenital malformations. We here report a single case treated in the Department of Paediatric Surgery in Benin. The study involved a 3-year-old girl with congenital sternal agenesis associated with ectopia cordis; firstly, she underwent controlled healing. Then thoracoplasty was performed with favourable outcome. Long-term results are good. Now, she is 13 years old, is attending school and has a satisfactory clinical condition. This is one of the few cases reported in the literature. Optimal therapeutic management has been keeping the patient alive in West Africa.


Assuntos
Ectopia Cordis/cirurgia , Esterno/cirurgia , Benin , Pré-Escolar , Feminino , Seguimentos , Humanos , Esterno/anormalidades , Resultado do Tratamento
6.
Fetal Pediatr Pathol ; 40(5): 540-542, 2021 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31914845

RESUMO

BACKGROUND: Ectopia cordis is a complete or partial extrusion of the heart through a ventral defect in the thoracoabdominal wall, either isolated or accompanied by other viscera in instances of pentalogy of Cantrell. Case Report: This six-year-old child has survived with uncorrected ectopia cordis. He is unable to participate in strenuous physical activities and has respiratory limitations. Conclusion: Ectopia cordis most commonly results in stillbirth or neonatal death without surgical treatment. This report highlights the exceptional 6-year survival of a child without surgical correction.


Assuntos
Ectopia Cordis , Cardiopatias Congênitas , Criança , Ectopia Cordis/cirurgia , Humanos , Masculino
7.
J Int Med Res ; 48(12): 300060520980210, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33322994

RESUMO

Body stalk anomaly is a rare abnormality characterized by an abdominal wall defect with evisceration of abdominal organs, severe kyphoscoliosis, and a very short or absent umbilical cord. Ectopia cordis (EC) is a rare, lethal anomaly characterized by complete or partial malpositioning of the heart outside of the thorax. A 28-year-old healthy primigravida was referred to our department to undergo a nuchal translucency thickness scan at 12 weeks' gestation. The scan revealed typical features of body stalk anomaly and EC. Given the lethal condition of the fetus, the patient opted for termination of the pregnancy. Body stalk anomalies, especially those complicated by EC, are universally lethal for the affected fetus. Selective termination should be recommended to avoid possible complications that can arise during pregnancy. Additionally, the future parents should be informed that because the condition is not associated with chromosomal abnormalities, there is no increased risk of recurrence.


Assuntos
Ectopia Cordis , Adulto , Ectopia Cordis/diagnóstico por imagem , Ectopia Cordis/cirurgia , Feminino , Idade Gestacional , Humanos , Gravidez , Primeiro Trimestre da Gravidez , Diagnóstico Pré-Natal , Ultrassonografia Pré-Natal
8.
Cardiol Young ; 30(12): 1951-1953, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-33040739

RESUMO

We present a 31 gestational weeks' premature baby whose fetal echocardiogram showed ectopia cordis, single ventricle and severe pulmonary stenosis. At 31 gestational weeks, an emergency caesarean section was performed, and his birth weight was 1756 g, SpO2 was 80% on 100% O2. Epicardial echocardiogram showed double inlet right ventricle, severe valvular pulmonary stenosis and no ductus arteriosus. The risk of surgery was very high, so we decided to perform balloon valvuloplasty by direct puncture of the heart. We punctured the apex of the ventricle using a 16-gauge needle under echo guidance, advanced the guidewire to the pulmonary artery and performed balloon valvuloplasty. Soon after the procedure, the cyanosis improved dramatically. This is the first report of a transcatheter procedure performed by direct puncture of the heart for ectopia cordis with complex congenital heart disease.


Assuntos
Valvuloplastia com Balão , Ectopia Cordis , Pentalogia de Cantrell , Estenose da Valva Pulmonar , Cesárea , Ectopia Cordis/diagnóstico , Ectopia Cordis/cirurgia , Feminino , Humanos , Lactente , Recém-Nascido de Baixo Peso , Recém-Nascido , Pentalogia de Cantrell/diagnóstico , Pentalogia de Cantrell/cirurgia , Gravidez , Estenose da Valva Pulmonar/diagnóstico , Estenose da Valva Pulmonar/cirurgia , Punções
10.
Fetal Pediatr Pathol ; 39(1): 78-84, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31215820

RESUMO

Introduction: Fetuses with trisomy 18 will occasionally also have ectopia cordis. Case report: A routine ultrasound scan at 12 weeks' gestation revealed a large fetal anterior thoraco-abdominal wall defect with an extrathoracic heart and a liver-containing omphalocele. Chorionic villus sampling revealed a 47,XY,+18 karyotype. Additional anomalies detected after termination of the pregnancy included a cleft lip and palate and left radial agenesis. Conclusions: The prenatal diagnosis of ectopia cordis associated with aneuploidy can be made in the first trimester of pregnancy. An extrathoracic heart located in a liver-containing omphalocoele should be considered a thoraco-abdominal ectopia cordis rather than pentalogy of Cantrell.


Assuntos
Ectopia Cordis/patologia , Pentalogia de Cantrell/patologia , Síndrome da Trissomía do Cromossomo 18/patologia , Adulto , Feminino , Idade Gestacional , Hérnia Umbilical/patologia , Humanos , Pentalogia de Cantrell/diagnóstico , Gravidez , Primeiro Trimestre da Gravidez/metabolismo , Diagnóstico Pré-Natal/métodos , Síndrome da Trissomía do Cromossomo 18/diagnóstico , Ultrassonografia Pré-Natal/métodos
11.
J Pediatr ; 216: 67-72, 2020 01.
Artigo em Inglês | MEDLINE | ID: mdl-31668886

RESUMO

OBJECTIVES: To utilize a large multicenter neonatal cohort to describe survival and clinical outcomes of very low birth weight (VLBW) or preterm infants with ectopia cordis. STUDY DESIGN: Data were prospectively collected on 2 211 262 infants (born 2000-2017) from 845 US centers. Both VLBW (401-1500 g or 22-29 weeks of gestation) and non-VLBW (>1500 g and >29 weeks) infants had diagnoses or anatomic descriptors consistent with ectopia cordis and/or pentalogy of Cantrell. The primary outcome was neonatal survival, defined as hospital discharge or initial length of stay of ≥12 months. RESULTS: In total, 180 infants had ectopia cordis, 135 (76%) with findings of pentalogy of Cantrell. VLBW infants comprised 52% of the population. VLBW mortality was 96% with 79% dying within 12 hours, compared with 59% and 36%, respectively, for non-VLBW. One-third of VLBW infants received life support compared with 65% of non-VLBW. Surgery was reported for 34% of VLBW and 68% of non-VLBW infants. Congenital heart disease was reported in 8% of VLBW and 36% of non-VLBW, with conotruncal abnormalities most common. Survival exceeded 50% for infants >2500 g and >37 weeks of gestation. CONCLUSIONS: Survival of VLBW infants with ectopia cordis was poor and substantially worse compared with non-VLBW, with notable discrepancies in resuscitative efforts and surgical interventions. Although gestational age and weight strongly influence current survival, more detailed information regarding the severity of cardiac and noncardiac abnormalities is required to fully determine prognosis and inform counseling.


Assuntos
Ectopia Cordis/mortalidade , Tempo de Internação/estatística & dados numéricos , Estudos de Casos e Controles , Pré-Escolar , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Recém-Nascido de muito Baixo Peso , Estudos Prospectivos , Estados Unidos/epidemiologia
13.
Rev. bras. ginecol. obstet ; 41(5): 352-356, May 2019. graf
Artigo em Inglês | LILACS | ID: biblio-1013614

RESUMO

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.


Assuntos
Humanos , Feminino , Gravidez , Recém-Nascido , Adulto , Ultrassonografia Pré-Natal , Pentalogia de Cantrell/diagnóstico por imagem , Imageamento por Ressonância Magnética , Evolução Fatal , Diagnóstico Diferencial , Ectopia Cordis/diagnóstico por imagem , Cardiopatias Congênitas/diagnóstico por imagem
14.
Rev Bras Ginecol Obstet ; 41(5): 352-356, 2019 May.
Artigo em Inglês | MEDLINE | ID: mdl-30912088

RESUMO

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.


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.


Assuntos
Ectopia Cordis/diagnóstico por imagem , Pentalogia de Cantrell/diagnóstico por imagem , Ultrassonografia Pré-Natal , Adulto , Diagnóstico Diferencial , Evolução Fatal , Feminino , Cardiopatias Congênitas/diagnóstico por imagem , Humanos , Recém-Nascido , Imageamento por Ressonância Magnética , Gravidez
15.
Fetal Pediatr Pathol ; 38(2): 127-137, 2019 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-30600745

RESUMO

BACKGROUND: Ectopia cordis (EC) is a congenital anomaly associated with heart defects and extracardiac malformations. OBJECTIVES: We determined the various presentations of EC diagnosed in our center between 2010 and 2017. RESULTS: Seven fetuses from six pregnancies with EC were detected, five during the first trimester. Three were from multiple pregnancies, and both twins had EC in one monochorionic-monoamniotic pregnancy. Abdominal wall defects were detected in six fetuses. Kyphoscoliosis, cephalocele, clubfoot and short umbilical cord were other abnormalities. Five fetuses were terminated, one fetus died in utero, and one baby died on day two of life. Postnatal evaluation performed in all cases additionally detected cleft lips/palates in two fetuses and tetralogy of Fallot in one. CONCLUSION: Outcome is poor for these fetuses, EC can occur in a multiple pregnancy, most of the abnormalities can be identified in the first trimester and fetopsy continues to add information to the intrauterine diagnosis.


Assuntos
Anormalidades Múltiplas/patologia , Ectopia Cordis/patologia , Feto/patologia , Cardiopatias Congênitas/patologia , Parede Abdominal/anormalidades , Ectopia Cordis/diagnóstico , Feminino , Cardiopatias Congênitas/diagnóstico , Humanos , Gravidez , Primeiro Trimestre da Gravidez , Diagnóstico Pré-Natal/métodos , Ultrassonografia Pré-Natal/métodos
16.
Arq. bras. cardiol ; 111(3 supl.1): 204-204, set., 2018. tab.
Artigo em Português | Sec. Est. Saúde SP, Sec. Est. Saúde SP, SESSP-IDPCPROD, Sec. Est. Saúde SP | ID: biblio-1046134

RESUMO

INTRODUÇÃO: Os beta-bloqueadores (BB) são os medicamentos de escolha para tratar a ectopia ventricular (EV), mas não há estudos que comparem a diferença entre a resposta de Succinato de Metoprolol e Atenolol no controle EV. MÉTODOS: Ensaio de crossover duplo-cego randomizado com 2 grupos. O primeiro começou com Atenolol e mudou para Succinato de Metocrolol (A-S). O segundo começou com Succinato de Metocrolol e mudou para Atenolol (S-A). Cada etapa teve um intervalo de tempo mínimo de 15 dias, para garantir o washout adequado da medicação anterior. Foram incluídos pacientes ≥ 18 anos, com fração de ejeção do ventrículo esquerdo ≥40% e uma taxa de VE ≥5%. O desfecho primário foi o número absoluto e a porcentagem de EV observada em Holter de 24h. O questionário de qualidade de vida (QVFAv2) e a preferência do paciente foram os desfechos secundários. O teste de Mann-Whitney foi utilizado para variáveis quantitativas e o teste exato de Fisher para variáveis qualitativas...(AU)


Assuntos
Humanos , Atenolol , Ectopia Cordis/tratamento farmacológico , Metoprolol
17.
Arch Gynecol Obstet ; 298(4): 841-842, 2018 10.
Artigo em Inglês | MEDLINE | ID: mdl-30145686

RESUMO

We report images of the 7th case worldwide of a twin fetus with a complete isolated ectopia cordis in a dichorionic diamniotic twin gestation. There is no consanguinity, family history of abnormalities, IVF, or first trimester medication. The diagnosis of this isolated anomaly was done at 23 weeks in one fetus, while the other had normal morphology; the pregnancy was complicated by preterm labor and a cesarean delivery followed at 34 weeks. The affected baby weighed 1800 g and had an outcome complicated by episodes of desaturation and deceased at day 8. Parents did not wish either in utero termination of pregnancy or postnatal surgery for reintegration in the thorax and chose instead palliative care.


Assuntos
Doenças em Gêmeos/diagnóstico , Ectopia Cordis/diagnóstico , Diagnóstico Pré-Natal , Adulto , Feminino , Humanos , Recém-Nascido , Gravidez
19.
Rev. ecuat. pediatr ; 19(1): 25-27, enero 2018.
Artigo em Espanhol | LILACS | ID: biblio-996426

RESUMO

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


Assuntos
Humanos , Masculino , Recém-Nascido , Anormalidades Congênitas , Ectopia Cordis , Cardiopatias Congênitas , Mortalidade Neonatal Precoce
20.
J Plast Reconstr Aesthet Surg ; 71(3): 384-393, 2018 03.
Artigo em Inglês | MEDLINE | ID: mdl-29029959

RESUMO

BACKGROUND: Repairing body wall defects is a critical step in the treatment of some congenital deformities, and this procedure may need the help from plastic surgeons. Although there are many articles about congenital deformities, body wall defects of these malformations are rarely studied as independent targets. METHODS: In this article, the authors present an LDT classification for congenital body wall defects according to the position of the defects, the tissue layers involved, and the surgical urgency, each of which is represented by letters L, D, and T, respectively. That is, the defects in different areas (L), full-thickness (D1), or partial (D0A, D0B) defects, defects needing instant repair (T2), semi-elective repair (T1), or elective repair (T0). Based on this classification system, the authors have performed body wall reconstruction on two pairs of thoraco-omphalopagus twins, one pair of ischiopagus tetrapus twins, and an infant and an adult, both of whom were diagnosed with pentalogy of Cantrell associated with ectopia cordis. RESULTS: Except for one pair of thoraco-omphalopagus twins who died after emergency separation, all the other patients survived. Another pair of thoraco-omphalopagus twins suffered from wound dehiscence and partial flap necrosis, respectively, after surgery. An expanded polytetrafluoroethylene mesh in one sister of the ischiopagus twins was removed because of infection. CONCLUSIONS: LDT classification not only can help doctors categorise different congenital body wall defects rapidly and easily, but can also guide the reconstruction of these defects. It may have clinical value to plastic surgeons to some extent.


Assuntos
Ectopia Cordis/cirurgia , Pentalogia de Cantrell/cirurgia , Procedimentos Cirúrgicos Reconstrutivos/métodos , Procedimentos Cirúrgicos Torácicos/métodos , Gêmeos Unidos/cirurgia , Adulto , Feminino , Humanos , Lactente , Masculino , Complicações Pós-Operatórias , Resultado do Tratamento
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