Ectopia cordis: uma condição rara

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Ectopia cordis in an adult patient with COVID-19: A case report and literature review

Ectopia cordis (EC) is a rare congenital condition characterized by a partial or complete defect of the anterior chest wall. It is associated with ventricular and atrial septal defects (ASD),. This study aimed to explore the cardiac manifestations of EC complicated by coronavirus disease 2019 (COVID-19).

Abdominal ectopia cordis in an aborted calf without chromosomal aberrations

Ectopia cordis is a rare congenital heart disease characterized by partial or complete displacement of the heart out of the thoracic cavity. Apart from cattle, the condition has also been described in humans and is frequently associated with Cantrell’s pentalogy. It is classified into five types: cervical, cervicothoracic, thoracic, abdominal and thoracoabdominal. The prognosis is poor and ectopia cordis may be linked to the presence of unbalanced chromosome alterations. In this report, a case of abdominal ectopia cordis is described in an aborted calf, in which no unbalanced structural chromosomal variants could be identified.

Pentalogy of Cantrell with ectopia cordis: A rare case with review of literature

Pentalogy of Cantrell (PC) is a rare congenital syndrome comprising five characteristic abnormalities that include midline anterior abdominal wall defect, sternal defect, diaphragmatic defect, defect in apical pericardium, and structural heart defect. The exact etiology of this syndrome is still unknown. However, many causative factors and association with other syndromes/aneuploidies (trisomy 18/trisomy 13) have been described in literature. Prognosis is often poor in cases with complete ectopia cordis with large defect. The postnatal surgical correction demands expert care in good resource settings. We are describing a rare case of severe form of PC with complete ectopia cordis and omphalocele, detected at 32 weeks of gestation. The patient had vaginal delivery of live female baby but unfortunately, the baby succumbed on day 2 of life. We are reporting this rare and fatal case to make health-care professionals more aware about importance of timely detection of such anomalies by anomaly scan in the second trimester before the period of viability so that the parents can make informed choice about termination of pregnancy in such lethal cases. Furthermore, the corrective surgeries can be planned beforehand with multidisciplinary approach in cases where affordability is not an issue for parents.

Ectopia Cordis Sternal Cleft Repair With Mandibular Distractors: A Follow-Up Case Report

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.

Limb body wall complex complicating a dichorionic diamniotic twin pregnancy: MRI for demonstration of fetal morphology

Limb body wall complex (LBWC) is a rare, lethal malformation characterised by body wall defects, craniofacial and limb anomalies with or without various other organ anomalies. We report a case of dichorionic diamniotic twin pregnancy discordant for LBWC, diagnosed by ultrasound (US) and confirmed by MRI at 21 weeks’ gestation, managed expectantly and delivered at 35 weeks by emergency caesarean section with a favourable outcome of the unaffected twin. The anomalous twin, who died soon after birth, had a sizeable thoracoabdominal wall defect, eviscerated liver and bowel loops attached to the placenta, short cord, ectopia cordis, lung hypoplasia, kyphoscoliosis, right upper limb amelia and left clubfoot with polydactyly. MRI helps to demonstrate the fetal morphology better when there are limitations to the US due to unfavourable fetal position, multifetal gestation, maternal obesity or reduced liquor. In twin pregnancies, the management will depend on ensuring the survival of the unaffected twin.

Complete Thoracic Ectopia Cordis, Dilemma of the Outcome: A Case Report

Background: Ectopia Cordis (EC) is a rare congenital condition where the heart is partially or completely lies outside the thoracic cavity (extrathoracic), uncovered by pericardium and skin. Many works of literature reported EC is a rare congenital abnormality with an incidence of about 5-8 per 1 million live births and includes about 0.1% of congenital heart diseases. Methods: This was a male baby, weighing 2.900 kg, received with cyanosis with a defect in the anterior chest wall and heart protruding out through it. On initial physical examination, split sternum with complete thoracic EC (beating outside the thoracic cavity with a complete absence of the pericardium with the apex pointing upwards) were reported. Results: The baby’s poor general condition did not allow further radiologic studies and echocardiography could not be performed. By the time, an arrangement had been planned for him to undergo cardiothoracic referral; unfortunately, he ran a downhill course and succumbed within 36 hours of life. Conclusion: The important for submitting such findings will assist our team: pediatric surgeons, obstetricians, pediatricians and, neonatal intensivists to develop future management strategies when they are enrolled or confronted with such cases, by improving the outcome through a precise workup design to provide the optimal evaluation, diagnosis, and management roadmaps of potential cases of EC.