Indian Journal of Radiology and Imaging Indian Journal of Radiology and Imaging

PAEDIATRIC IMAGING
Year
: 2003  |  Volume : 13  |  Issue : 4  |  Page : 415--416

Ectopia cordis thoracalis with facial cleft


PK Jain, KS Budhwani, RK Ghritlaharey 
 Department of Paediatric Surgery, Gandhi Medical College Bhopal, M.P-462 001, India

Correspondence Address:
R K Ghritlaharey
Department of Paediatric Surgery, Gandhi Medical College, Bhopal, M.P.462001
India




How to cite this article:
Jain P K, Budhwani K S, Ghritlaharey R K. Ectopia cordis thoracalis with facial cleft.Indian J Radiol Imaging 2003;13:415-416


How to cite this URL:
Jain P K, Budhwani K S, Ghritlaharey R K. Ectopia cordis thoracalis with facial cleft. Indian J Radiol Imaging [serial online] 2003 [cited 2019 Nov 20 ];13:415-416
Available from: http://www.ijri.org/text.asp?2003/13/4/415/28723


Full Text

A full term female baby of 2.6 kgs delivered by Caesarean section for obstructed labour to a 30 yrs old mother. There were no history of antenatal infection, drug ingestion , exposure to radiation etc. She was brought to hospital 7 hrs. after birth. Examination revealed peri-oral cyanosis with heart rate of 140/min, regular & respiratory rate was 40/ min. The heart of baby was lying outside the thoracic cavity & devoid of pericardium. The apex of heart was pointing towards anteriorly. The coronary vessels were seen clearly. She also had associated omphalocele, bilateral cleft lip & cleft palate [Figure 1]. Xray of abdomen & chest revealed crowding of ribs ended near mid clavicular line & sternum was absent. Spina bifida at L2-L5 vertebrae were also seen [Figure 2].

Patient unfortunately died within few hours or birth before any surgical intervention could be undertaken. Post mortem examination of non- medicolegal cases is not a routine in our hospital & relatives also refused consent for the same, therefore the nature of intrinsic cardiac defect etc could not be determined.

 Discussion



Ectopia cordis is defined as complete or partial displacement of the heart out side the thoracic cavity. It is a rare congenital defect in fusion of the anterior chest wall resulting in extra thoracic location of the hear[1],[2]. The term ectopia cordis was first described by Haller in 1706. The estimated prevalence for ectopia cordis is 0.079/10,000 births &may occurs more in females[1]. Four types of ectopia cordis has been described according to the location of the heart as -thoracic (65%), Thoraco-abdominal(20%), Abdominal (10%) & cervical (5%). The thoracic ectopia cordis constitute the classic naked heart with no overlying somatic structures [1],[2],[3],[4]. Genesis of ectopia cordis has not been fully explained, although several theories have been offered. Predominent theories includes-[1] Primary failure of descent & mid line fusion of the lateral body folds.[2] Early rupture of chorion & /or yolk sac causing failure of midline fusion, (3) Amniotic band syndrome[1],[3].

The complex of ectopia cordis , craniofacial defects & amniotic band was first reported by Prochaska in 1734. This complex is embryologically distinct from isolated ectopia cordis. It occurs due to mechanical teratogenesis induced by tissue bands adherent to thorax, cranium & face leading to pressure necrosis, incomplete morphogenesis & tethering of amnion, so the complex seems to be a consequence of " early amnion rupture " during third week of gestation[1],[3]. The etiology for rupture of amnion is unknown.[3]

Isolated ectopia cordis has been produced in experimental models involving multiple organ systems. Ventricular septal defects & tetralogy of Fallot are the most common cardiac defects , while omphalocele , cleft lip & palate are other common associations[1],[2],[3],[4].

Cutler & Wilens first attempted repair of ectopia cordis by skin flap Closure in 1925, patient died presumably due to pressure of flap over heart, leading to cessation of cardiac functions[4]. First succesful repair of ectopia cordis was achived by Koop in 1975 as reported by Saxena. He reported of an infant with normal heart, had skin coverage at 5 hrs after birth. At 7 months acrylic resin of Dacron & Malex mesh was used to cover the sternal cleft & primary skin cover was done, infection of prosthesis & necrosis of skin flap resulted in removal of prosthesis. Child survived above complications & reported well at 12 yrs of age[4]

Dobell, Williams & Long in 1982 reported two stage correction , first Skin flap covering in new born male. At 19 months of age rib graft placed over sternal defect & covered by pectoral muscle flap. Patient had no any intrinsic cardiac defects & survived[5]. To bridge the sternal gap bone graft , cartilage & synthetic materials have been used but invairably they get infected , resulting in rejection of grafts & prosthetic materials[1],[4],[5].

It has been noticed that patients who survived , had, no intrinsic cardiac defects[1],[2],[4]. It has been also observed that the ultimate survival of these patients depend more on the presence or absence of intrinsic cardiac defects rather than surgical techniques. Most of infants are stillborn or die within first few hrs or days of life[1],[2],[4]. Obstetrical management should include a careful search for associated anomalies, specially cardiac & assessment of fetal karyotype. The defects can be diagnosed by antenatal ultrasonography & the intrinsic cardiac defects can be detected by prenatal echocardiography. Pregnancy termination prior to viability & a non aggressive management in the third trimester should be considered & discussed with parents. As this is considered a sporadic event, the recurrence risk is not elevated over that of the general population [1],[2],[4]. Despite many surgical failures, it is expected that accumulated experiences from previous cases can guide' to formulate the plan of management for complicated anomalies and will eventually lead to the survival

References

1William J.Meyer.,Daniel W.Gauthier.,Wilfredo Torres. et al; Heart, Cordis-ectopia, Department of Obst. & Gynae, Division of Maternal Fetal Medicine, University of Illinois at Chicago, Chicago.
2T. Humpl, P.Huggan, L K Hornberger, et al. Presentation & outcomes of ectopia cordis. The Can. J. Cardio. 1999,15 (12):1353-1357.
3Van Allen MI, Myhre S.- Ectopia cordis thoracalis with craniofacial defects resulting from early amnion rupture. Teratology 1985,Aug. 32 (1):19-24.
4Robert C. Shamberger- Congenital chest wall deformities in James A.O. Neill Jr., Marc. 1. Rowe, Jay L. Grosfeld et al. Pediatric Surgery (vol. 1) 5th Ed. Mosby year book inc. 1998: 806-817.
5Dobell AR, Williams HBU, Long RW, Staged repair of ectopia cordis. Journal of Pediatric Surg. 1982 Vol. 17 no.4 pp 353-8