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ABSTRACT
The Pentalogy of Cantrell is a congenital anomaly characterized by the lower sternum, a midline supra umbilical thoracoabdominal wall defect, a deficiency of the diaphragmatic pericardium, a deficiency of the anterior diaphragm and congenital cardiac anomalies. We report a case of newborn who presented at birth an ectopia cordis associated with an omphalocele type 2 according to the Aiteken classification. After investigations the diagnosis of pentalogy of cantrell was made. The outcome of newborn is unfavorable because he died after 12 hours before surgery. The prevention of Pentalogy of Cantrell goes through early antenatal diagnosis and appropriate counseling to decide the outcome of the pregnancy. The prognosis after birth is poor despite early surgical management by trained teams.
RÉSUMÉ
La pentalogie de Cantrell est une anomalie congénitale caractérisée par le sternum inférieur, une anomalie de la paroi thoraco-abdominale supra-ombilicale médiane, une déficience du péricarde diaphragmatique, une déficience du diaphragme antérieur et des anomalies cardiaques congénitales. Nous rapportons un cas de nouveau-né qui a présenté à la naissance une ectopie cordienne associée à une omphalocèle de type 2 selon la classification d’Aiteken. Après investigations, le diagnostic de pentalogie du cantrell a été posé. L’issue du nouveau-né est défavorable car il est décédé après 12 heures avant l’opération. La prévention de la pentalogie de Cantrell passe par un diagnostic prénatal précoce et des conseils appropriés pour décider de l’issue de la grossesse. Le pronostic après la naissance est sombre malgré une prise en charge chirurgicale précoce par des équipes formées.
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References
- Cantrell Jr, Haller JA, ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium,and heart. Surg gynecol obstet. 1958;107:602
- Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome. Pediatrics 1972;50:778-92.
- Williams AP, Marayati R, Beierle EA. Pentalogy of Cantrell . Semin Pediatr Surg. 2019;28(2): 106–110
- Jnah AJ, Newberry DM, England A. Pentalogy of Cantrell: Case Report With Review of the
- Literature. Adv Neonatal Care. 2015;15(4):261–8
- Bagabir HA, Azhar AS. Pentalogy of Cantrell: frst case reported in Saudi Arabia. Ann Saudi Med 2014; 34(1): 75-77
- Oumarou H, Amadou MI, Harissou A et al. Ectopia Cordis: A Case Report and Review of Literature. Journal of Case Reports 2017;7(2):143-145
- Chishugi JB, J Franke TJ. Thoraco-abdominal Ectopia Cordis in Southwest Cameroon. Pan African Medical Journal. 2014; 18:124
- carmi r, Meizner I, Katz M. Familial congenital diaphragmatic defect and associated midline
- anomalies: Further evidence for an X-linked midline gene? Am J Med genet. 1990;36:313-5.
- Steiner MB, Vengoechea J, Collins RT. Duplication of the ALDH1A2 gene in association
- with pentalogy of Cantrell: a case report. Journal of Medical Case Reports 2013;7:287
- Nayak S, Dash SP, Khatua M. Fetal Anomaly: Pentalogy of Cantrell. IOSR Journal of Dental and Medical Sciences. 2015;14:52-55
- Marginean C, Cristina Oana Marginean CO, Gozar L et al. Cantrell Syndrome—A Rare Complex Congenital Anomaly: A Case Report and Literature Review. Front. Pediatr. 2018;6:201
- Yi-Jen H, Fong-Lin C, Yan-Yan N et al. Trisomy 18 Syndrome with Incomplete Cantrell Syndrome. Pediatr Neonatol 2008;49(3):84−87
- Hoorn J , Moonen R , Clément J. R. Huysentruyt C et al. Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr (2008) 167:29–35
References
Cantrell Jr, Haller JA, ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium,and heart. Surg gynecol obstet. 1958;107:602
Toyama WM. Combined congenital defects of the anterior abdominal wall, sternum, diaphragm, pericardium, and heart: A case report and review of the syndrome. Pediatrics 1972;50:778-92.
Williams AP, Marayati R, Beierle EA. Pentalogy of Cantrell . Semin Pediatr Surg. 2019;28(2): 106–110
Jnah AJ, Newberry DM, England A. Pentalogy of Cantrell: Case Report With Review of the
Literature. Adv Neonatal Care. 2015;15(4):261–8
Bagabir HA, Azhar AS. Pentalogy of Cantrell: frst case reported in Saudi Arabia. Ann Saudi Med 2014; 34(1): 75-77
Oumarou H, Amadou MI, Harissou A et al. Ectopia Cordis: A Case Report and Review of Literature. Journal of Case Reports 2017;7(2):143-145
Chishugi JB, J Franke TJ. Thoraco-abdominal Ectopia Cordis in Southwest Cameroon. Pan African Medical Journal. 2014; 18:124
carmi r, Meizner I, Katz M. Familial congenital diaphragmatic defect and associated midline
anomalies: Further evidence for an X-linked midline gene? Am J Med genet. 1990;36:313-5.
Steiner MB, Vengoechea J, Collins RT. Duplication of the ALDH1A2 gene in association
with pentalogy of Cantrell: a case report. Journal of Medical Case Reports 2013;7:287
Nayak S, Dash SP, Khatua M. Fetal Anomaly: Pentalogy of Cantrell. IOSR Journal of Dental and Medical Sciences. 2015;14:52-55
Marginean C, Cristina Oana Marginean CO, Gozar L et al. Cantrell Syndrome—A Rare Complex Congenital Anomaly: A Case Report and Literature Review. Front. Pediatr. 2018;6:201
Yi-Jen H, Fong-Lin C, Yan-Yan N et al. Trisomy 18 Syndrome with Incomplete Cantrell Syndrome. Pediatr Neonatol 2008;49(3):84−87
Hoorn J , Moonen R , Clément J. R. Huysentruyt C et al. Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr (2008) 167:29–35