Home | About Us | Current Issue | Ahead of print | Archives | Search | Instructions | Subscription | Feedback | Editorial Board | e-Alerts | Login 
Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
 Users Online:873 
  Print this page Email this page   Small font sizeDefault font sizeIncrease font size


 
HOW I DO IT
Year : 2006  |  Volume : 11  |  Issue : 4  |  Page : 250-252
 

Primary closure of thoraco-abdominal ectopia cordis


Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India

Correspondence Address:
V Bhatnagar
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi - 110029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-9261.29613

Rights and Permissions

 

   Abstract 

Thoraco-abdominal ectopia cordis is an unusual congenital anomaly. The case of a 45-days-old female child, who presented with a defect in the chest and upper abdomen and visible cardiac pulsations and bulge during crying, is being reported. There was an inferior sternal cleft with divarication of the recti up to the umbilicus. The cleft was covered with a thin translucent membrane. Primary repair was accomplished successfully without causing mediastinal compression.


Keywords: Divarication of recti, primary closure, sternal cleft, thoraco-abdominal ectopia cordis


How to cite this article:
Singal A K, Balamourougane P, Bhatnagar V. Primary closure of thoraco-abdominal ectopia cordis. J Indian Assoc Pediatr Surg 2006;11:250-2

How to cite this URL:
Singal A K, Balamourougane P, Bhatnagar V. Primary closure of thoraco-abdominal ectopia cordis. J Indian Assoc Pediatr Surg [serial online] 2006 [cited 2019 Oct 14];11:250-2. Available from: http://www.jiaps.com/text.asp?2006/11/4/250/29613



   Introduction Top


The spectrum of defects in thoraco-abdominal ectopia cordis includes an inferior sternal cleft and diastasis recti. The heart maybe located in the thorax, but is covered by a membrane or thin skin.[1] These anomalies are rare and the challenge lies in closure of the thoraco-abdominal wall defect without causing compression of the heart. Various techniques are available for the repairing of these anomalies but primary closure, if feasible, is the technique of choice.[2],[3],[4] Though easy and less complicated in neonatal age, it often becomes impossible in older children due to loss of compliance of the chest cage. We report a child with thoraco-abdominal ectopia cordis who was successfully managed with primary repair.


   Case report Top


A 45-days-old female child presented with defect in the chest and upper abdomen with visible cardiac pulsations and bulge during crying. There was a cleft of the sternum from manubrium downwards with involvement of the upper abdominal wall to just above the umbilicus. The cleft was covered with a thin translucent membrane in the chest and a somewhat thicker membrane lower down in the abdomen [Figure - 1]. Associated anomalies included left-sided macrochelia, polydactyly in the hand and syndactyly in the foot. Echocardiogram showed a small PDA. During surgical repair, the membrane was found to be densely adherent to the pericardium and peritoneum. A small rent in the pericardium was repaired with fine absorbable sutures. The sternal halves were approximated in midline with 1-0 prolene sutures without much tension. The overlying soft tissues and muscles were closed over the repair with absorbable sutures [Figure - 2]. A suction drain was placed underneath the flaps. Post-operatively the child required ventilatory support for 2 days. Suction drain was removed at 72 hours and the child was discharged uneventfully on day 6 [Figure - 3].


   Discussion Top


The sternum originates from two longitudinal mesenchymal bands, which appears at 6 weeks in the anterior thoracic wall of the embryo, independently of the ribs. These start fusing in a cephalocaudal direction from 7 weeks onwards and this fusion is complete by the 10th week. Similarly ossification starts at 5-6 months of embryonic life and proceeds in the cephalocaudal direction. Thus, it is believed that sternal defects result from the failure of the mesenchymal plate fusion process during the 8th week of gestation.[3],[5]

In 1990 Shamberger and Welch suggested a classification for these patients dividing them into four types: (a) thoracic ectopia cordis, (b) cervical ectopia cordis, (c) thoracoabdominal ectopia cordis, and (d) sternal cleft or bifid sternum.[1],[2] The simple sternal cleft should have an orthotopic heart, an intact pericardium and normal skin coverage. Isolated sternal clefts can also be classified as partial or total.[5] The partial forms can be superior, medium, or inferior. Pure inferior sternal clefts are extremely rare and are generally found in conjunction with thoracoabdominal ectopia cordis. In the present case the heart was in an orthotopic location and there was no major intracardiac anomaly except patent ductus. This association has been reported in literature albeit with a superior sternal cleft.[6],[7] The membrane bridging the defect was thin and was adherent to the pericardium and peritoneum. Another interesting feature was divarication of midline abdominal musculature, which required correction.

Sternal defects should be corrected as lack of bony cage makes the mediastinal structures vulnerable to trauma and unsightly appearance. Later, the correction is difficult and there is a disturbance in respiratory and cardiac functions.[3] Many methods for repair of cleft sternum have been described ranging from sliding chondrotomies, repair of the defect with autologous grafts (costal cartilage, ribs, parietal bone) and prosthetic materials (Marlex mesh, teflon, silicone prosthesis). Direct approximation of the sternal halves or primary repair is preferable, because this technique reduces the problems with the prosthetic material and the difficulties in the osteocartilaginous cicatrisation, which occurs in the chondrocostal division procedures.[2],[3],[4],[6],[7],[8] However, the age of the patients is the prime determinant for the feasibility of this procedure. During the neonatal period, the chest wall is more flexible and this allows an easy approximation of the sternal halves without mediastinal compression. After the neonatal period and certainly after one year of life, primary repair is difficult. In the present case the primary repair was accomplished successfully in a 45 days old child with a rare type of sternal defect.

Thoraco-abdominal ectopia cordis is an unusual congenital anomaly. Optimal correction can be obtained by primary closure of the defect in young patients, especially during the neonatal period, with an easy technique. In children above that age, more extensive techniques may be applied, although the results are satisfactory from a functional and aesthetic point of view.

 
   References Top

1.Shamberger RC. Congenital chest wall deformities. In : O'Neill JA Jr, Rowe MI, Grosfeld JL, Fonkalsrud EW, Coran AG, editors. Pediatric Surgery. Mosby Year Book Inc: St Louis; 1998. p. 787-817.  Back to cited text no. 1    
2.Shamberger RC, Welch KJ. Sternal defects. Pediatr Surg Int 1990;5:156-64.  Back to cited text no. 2    
3.Fokin AA. Cleft sternum and sternal foramen. Chest Surg Clin N Am 2000;10:261-76.  Back to cited text no. 3    
4.Acastello E, Majluf R, Garrido P, Barbosa LM, Peredo A. Sternal cleft: A surgical opportunity. J Pediatr Surg 2003;38:178-83.   Back to cited text no. 4    
5.O'Neal ML, Dwornik JJ, Ganey TM, Ogden JA. Postnatal development of the human sternum. J Pediatr Orthop 1998;18:398-405.  Back to cited text no. 5    
6.Knox L, Tuggle D, Knott-Craig CJ. Repair of congenital sternal clefts in adolescence and infancy. J Pediatr Surg 1994;29:1513-6.  Back to cited text no. 6    
7.Ishikawa N, Hiranuma C, Sato H, Ueno Y, Seki M, Yamamoto M. Congenital sternal cleft with patent ductus arteriosus: Report of a case. Surg Today 2002;32:66-8.  Back to cited text no. 7    
8.Domini M, Cupaioli M, Rossi F, Fakhro A, Aquino A, Chiesa PL. Bifid sternum: Neonatal surgical treatment. Ann Thorac Surg 2000;69:267-9.   Back to cited text no. 8    


    Figures

[Figure - 1], [Figure - 2], [Figure - 3]


This article has been cited by
1 A rare congenital cardiac anomaly: Ectopia cordis | [Nadir rastlanan doǧumsal kalp anomalisi: Ektopia kordis]
Keçeligil, H.T., Demiraǧ, M.K., Yücel, S.M., Yüksel, A.
Anadolu Kardiyoloji Dergisi. 2009; 9(2): 144-146
[Pubmed]



 

Top
Print this article  Email this article
Previous article Next article

    

 
  Search
 
   Next article
   Previous article 
   Table of Contents
  
 
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (177 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  


    Abstract
    Introduction
    Case report
    Discussion
    References
    Article Figures

 Article Access Statistics
    Viewed4602    
    Printed162    
    Emailed0    
    PDF Downloaded130    
    Comments [Add]    
    Cited by others 1    

Recommend this journal


Contact us | Sitemap | Advertise | What's New | Copyright and Disclaimer 

 © 2005 - Journal of Indian Association of Pediatric Surgeons | Published by Wolters Kluwer - Medknow 

Online since 1st May '05