Pentalogy of cantrell: a case report

Yıl 2019, Cilt: 33 Sayı: 1, 103 - 107, 26.04.2019

Ali Cenk Özay Özlen Emekçi Özay Erkan Çağlıyan Sabahattin Altunyurt

https://doi.org/10.5505/deutfd.2019.14632

Öz

Introduction: Pentalogy of Cantrell is a congenital malformation syndrome characterized by midline thoracoabdominal wall defect, intracardiac anomalies, sternum lower end defect, absence of pericardium on diapraghma side and diapraghma defects.
Case Report: In this article, we report a case of prenatal diagnosed Cantrell Pentalogy in 20th gestational weeks. 
Conclusion: The ethiology of Cantrell syndrome is not certain yet, but probably the syndrome occurs due to the insufficient migration of intraembryonic mesoderm to ventromedialis. Thoracoabdominal type ectopia cordis, one of five different ectopia cordis types, is frequently found in the components of Cantrell pentalogy.

Anahtar Kelimeler

Pentalogy of Cantrell, ectopia cordis, ventricular septal defect

Cantrell pentalojisi: olgu sunumu

Öz

Giriş: Cantrell pentolojisi; orta hat supra-umbilikal karın ön yüzü defekti, sternum alt ucu defekti, perikardın diafragma yüzünün olmayışı, diafragma ön yüz eksikliği ve çeşitli intrakardiyak anomaliler ile tanımlanmıştır.
Olgu: Bu olgu sunumunda 20. gebelik haftasında prenatal olarak tanımlanmış bir Cantrell sendromlu olgu sunularak, Cantrell sendromunun literatür eşliğinde tartışılması amaçlanmıştır.
Sonuç: Cantrell sendromunun sebebi kesin olarak bilinmemekle birlikte, patogenezinde intraembriyonik mezodermin ventromedial yönde migrasyonunda yetersizlik sonucu oluştuğu düşünülmektedir. Beş farklı ektopia kordis tipinden biri olan torakoabdominal tip ektopia kordis durumu Cantrell pentalojisinin de bileşenleri içerisinde sıklıkla yer almaktadır.

Anahtar Kelimeler

Cantrell pentalojisi, ektopia kordis, ventriküler septal defekt

Kaynakça

• Cantrell JR, Haller JA, Ravitch MM. A syndrome of congenital defects involving the abdominal wall, sternum, diaphragm, pericardium, and heart. SurgGynecolObstet. 1958;107:602-614.
• Vazquez-Jimenez JF, Muehler EG, Daebritz S, Keutel J, Nishigaki K, Huegel W, et al. Cantrell's syndrome: a challenge to the surgeon. AnnThoracSurg. 1998; 65:1178-1185.
• Güven MA, Ceylaner G, Ceylaner S, Coşkun A, Bayazıt H. Prenatal tanısı konmuş Cantrell pentalojisi olgusu: Ensefaloselin eşlik ettiği nadir bir varyant. Türk Jinekoloji ve Obstetrik Derneği Dergisi. 2009; 6:123-127.
• Uludağ S, Barbaros ZM, Aksoy F, Erdoğan E,Madazlı R. Cantrell Sendromu. Perinatoloji Dergisi. 1999; 7:324-327
• Parvari R, Carmi R, Weissenbach J, Pilia G, Mumm S, Weinstein Y. Refined genetic mapping of X-linked thoracoabdominal syndrome. Am J Med Genet. 1996; 61:401-402.
• 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.
• Paidas M, Crombleholme TM, Robertson FM. Prenatal diagnosis and management of the fetus with an abdominal wall defect. Sem Perinatol. 1994;18: 196- 214.
• Jeroen HL, Moonen R, Clement J, Huysentruyt R, offermans PM, Mulder A. Pentalogy of Cantrell: two patients and a review to determine prognostic factors for optimal approach. Eur J Pediatr. 2008; 167:29-35.
• Morales JM, Patel SG, Duff JA, Villareal RL, Simpson JW. Ectopia cordis and other midline defects. Ann Thorac Surg 2000;70(1):111-4.
• Abdallah HI, Marks LA, Balsara RK, et al. Staged repair of pentalogy of Cantrell with tetralogy of Fallot. Ann Thorac Surg. 1993;56:979-80.
• Hornberger LK, Colan SD, Lock JE, et al. Outcome of patients with ectopia cordis and significant intracardiac defects. Circulation. 1996;94:II32-7 (suppl).