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Ectopia cordis thoracalis with cystic hygroma, syndactyly and cleft lip and cleft palate R. K. Ghritlaharey, Gaurav Gupta, A. S. Kushwaha, R. Chanchlani Department of Pediatric Surgery, Gandhi Medical College and Associated, Kamla Nehru and Hamidia Hospitals Bhopal - 462 001, MP, India Correspondence: Dr. Rajendra K. Ghritlaharey, Department of Pediatric Surgery, Gandhi Medical College and Associated Kamla Nehru and Hamidia Hospitals, Bhopal - 462 001, MP, India. E-mail:
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m o at right side neck offr4 x 3 cm in size. Syndactyly of CASE REPORT little, ring, and middle fingers of right hand also present d nsrevealed no diaphragmatic A full term female baby of 2.3 kg was born to a Gravida [Figure 2]. Investigations a mobilization I Para 0, 22-years-old mother on June12, 2006. She was hernia. Afterlo io of skin around the heart, it repositedtwithin the thoracic cavity and skin brought to us one hour after birth. The pregnancy and was easily n aShe died 2 hours after operation due was done. hospital delivery were uneventful. There was no history closure w c i o l that our patient also had of infection, intake of any teratogens, drugs or exposure to cardiac d arrest. b We presumed intrinsic ucardiac defects, although neither
to radiation, etc. in antenatal period. e P ). nor post-mortem examination was echocardiography e r Clinical examination revealed peripheral cyanosis withf done.w r o om heart rate of 146/min and respiratory rate of 48/min. Her o f of kDISCUSSION n .c heart was lying outside the thoracic cavity and apex e the heart was pointing anterio-superiorly. The heart had wcordis (EC) is defined as complete or partial l vesselsed Ectopia o b pericardial covering and coronary and few great n a right could be seen [Figure 1]. She also had associated displacement of the heart outside the thoracic cavity. It is l M k i sided complete cleft lip and cleft palate,acystic hygroma a rare congenital defect in fusion of the anterior chest d y e v b a si ted w.m F os w D P te h (w is si h T a
Figure 1: Clinical photograph of patient showing thoracic ectopic heart, great vessels, cystic hygroma right side neck
Figure 2: Clinical photograph of patient showing ectopic heart, syndactyly of right fingers, cleft lip and cleft palate of right side
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Ghritlaharey, et al.: Thoracic ectopia cordis
wall resulting in extra thoracic location of the heart. The estimated prevalence of ectopia cordis is 5.5-7.9 per million live births and may occur more in females.[1,2]
severity of the intrinsic cardiac defects and associated other congenital anomalies, rather than the type of surgical approach itself.
Depending upon the location of the heart, it could be classified into five types: cervical, cervicothoracic, thoracic, thoracoabdominal, and abdominal.[2] Thoracic and thoracoabdominal EC accounts for about 85% of the cases. Thoracic EC is a classic naked heart with no overlying somatic structures.
Advances in the fetal ultrasound techniques have aided in the early recognition of such anomalies and termination of pregnancy prior to viability should be considered and discussed with parents.
EC can be diagnosed by routine prenatal ultrasonography as early as in 10-12 weeks of pregnancy.[3,4] Most cases of EC result in stillbirth or die shortly after birth. It is frequently associated with other intrinsic cardiac as well as other congenital defects involving multiple organ systems.[4,5]
1.
REFERENCES Jain PK, Budhwani KS, Ghritlaharey RK. Ectopia cordis thoracalis with facial cleft. Indian J Radiol Imag 2003;13:415-6. 2. Khaled S, Olivier G, Dominique M, Bernard K. Ectopia cordis: A successful single stage thoracoabdominal repair. Int Cardiovasc Thorac Surg 2003;2:611-3. 3. Cuillier F, Avignon MS, Avignon A. Pentalogy of Cantrell, 11 weeks. Pentalogy of Cantrell, 11 weeks © Cuillier. [cited on 2006 Feb 20]. Available from: http://www.thefetus.net. 4. Repondek-Liberska M, Janiak K, Wloch A. Fetal echocardiography in ectopia cordis. Pediatr Cardiol 2000;21:249-52. 5. Meyer WJ, Gauthier DW, Torres W, Donald W, Warsof S. Heart, cordis ectopia. Ectopia cordis © Meyer. [cited on 1991 Nov 1]. Available from: http://www.thefetus.net 6. Saxena AK. Pectus excavatum, pectus carinatum and other forms of thoracic deformities. J Indian Assoc Pediatr Surg 2005;10:147-57. 7. Amato JJ, Zelen J, Talwalkar NG. Single-stage repair of thoracic ectopia cordis. Ann Thorac Surg 1995;59:518-20. 8. Shamberger RC. Congenital chest wall deformities. Pediatric surgery. O’ Neill JA, Rowe MI, et al, editors. 5 th ed. Mosby – Year Book Inc: 1998. p. 787-817. 9. Lall A, Gupta DK. Sternal defects. Text Book of Neonatal Surgery. Gupta DK, editor. 1 st ed. Modern Publishers: New Delhi; 2000. p. 407-11. 10. Alphonso N, Venugopal PS, Deshpande R, Anderson D. Complete thoracic ectopia cordis. Eur J Cardiothorac Surg 2003;23:426-8.
m o Surgical correction of these defects is complex and fr generally requires a staged closure as: d ns 1. Covering of the naked heart by skin a 2. Placement of the heart into the thoracic cavity. lo tio n Correction of the intrinsic cardiac defects is desirable w lica prior to orthotopic location of heart o 3. Sternal/thoracic reconstruction. d ub e P ). Haller first described the term ectopia cordis in 1706. e r The first attempted repair of EC was performed in 1925 f w m r by Cutler and Wilens; however, it was Koop who o no .co achieved first successful repair of thoracic EC in two f k w stages in 1975. Amato et al. reported successful single e l d stage repair of thoracic EC in 1995. b e no a il of 29y M dk Review of literature reported only three survivors a b me attempted repairs of thoracic EC, to whichvfew more a Successful unsuccessful attempts have been added. s tedandw. repair of such anomalies is dictated by ithe presence F os w D P te h (w is si h T a [6,7]
[8-10]
Source of Support: Nil, Conflict of Interest: None declared.
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