Ectopia cordis is an extremely rare congenital heart malformation that remains very difficult to treat. Occurrence is .79 in every 10,000 births. In ectopia cordis, the heart forms outside of the chest wall, so it is not protected by the skin or sternum. Other organs may also have formed outside the skin. To further complicate this difficult presentation, the heart itself is generally improperly formed. It may have defects like Tetralogy of Fallot, pulmonary atresia, atrial and ventricular septal defects, or double outlet right ventricle, among others. Along with heart malformations, children with ectopia cordis may also have cleft palates. The spine can also be formed improperly, causing kyphosis, too great of a C curvature in the thoracic spine.
There is some slim evidence that
ectopia cordis may be related to Turner Syndrome. Chromosomal anomalies like Trisomy 18 have also been observed in some children with
ectopia cordis. In general, however, there is no recognizable cause for this condition. In one way, this may be comforting to parents who have had a child with
ectopia cordis. The condition is unlikely to repeat in future children. Sadly, most cases of
ectopia cordis result in
stillbirth or death shortly after birth. A few cases of
ectopia cordis have been treated successfully, but this defect still poses the biggest challenge and rate of failure for pediatric cardiothoracic surgeons.
Most cases of ectopia cordis are identified through routine prenatal ultrasound. When a case is suspected, the mother will be referred to a pediatric or fetal echocardiologist who will confirm the diagnosis. To give the child the best possible chance at survival, the baby will be born at a Level 3 hospital, where experienced pediatric cardiologists and surgeons are on hand to immediately address the problems. Birth may be vaginal or Caesarian, as indicated by the high-risk obstetrician.
Parents who receive early diagnosis of ectopia cordis have the option to induce labor early or to have a therapeutic abortion performed. Parents who choose induced labor expect fatality, but may find comfort in naturally giving birth to a child, having a chance to see and hold that child and to say goodbye. Other parents prefer to end the child’s life without holding or seeing him or her. They find this option less painful than continuing the pregnancy. This difficult decision should be carefully weighed with the help of a skilled counselor.
When parents choose to wait and see if their child can be treated, success of treatment depends on the severity of other heart defects, as well as other related problems. In most cases, cardiothoracic surgeons attempt repair of the heart defects before covering the heart and organs with prosthetic skin. Unfortunately, the valves leading to the heart, once replaced inside the chest wall, can kink and cause death. An overwhelming number of birth defects in a child with ectopia cordis can also make repair impossible, and parents may simply choose to do nothing, called compassionate care, rather than subject their child to serious medical interventions with little hope of success.
Those parents who elect treatment may feel a sense of relief even when treatment fails. Treatment can offer parents a sense that they have done everything possible to save their child. With any option, the death of a child is a weighty emotional burden. Seeking help from organizations like Hospice and working with grief counselors can assist over time with the overwhelming feelings of loss.
Though very rare, treatment of ectopia cordis clearly needs greater research to improve outcome. Fetal surgery is still relatively new, but attempting repair prior to birth may be beneficial, and this should be studied for potential value. (http://www.wisegeek.com)
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