Cantrell syndrome with complex cardiac malformations: a case report

Li, Wen; Liao, Junlin; Jia, Hao; Zhang, Dong; Li-guang, Zou; Shuhua, Dai; Weijin, Liu; Gao, Yunhua

doi:10.1016/j.jpedsurg.2011.03.071

Cited by 11 publications

(7 citation statements)

References 10 publications

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“…Our case could have had a relatively better outcome since she had an incomplete form of the syndrome. Previous reports suggest that, mortality is higher in infants with the complete form and associated extra cardiac anomalies whilst patients with ectopia cordis with intracardiac defects have a favorable outcome [ 31 , 34 ].…”

Section: Discussionmentioning

confidence: 99%

Class II pentalogy of Cantrell

et al. 2015

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BackgroundPentalogy of Cantrell is a rare syndrome, first described by Cantrell and co-workers in 1958. The syndrome is characterized by the presence of five major congenital defects involving the diaphragm, abdominal wall, the diaphragmatic pericardium, lower sternum and various congenital intra-cardiac abnormalities. The syndrome has never been reported in Tanzania, although may have been reported from other African countries. Survival rate of the complete form of pentalogy of Cantrell is as low as 20%, but recent studies have reported normal growth achieved by 6 years of age where corrective surgeries were done; showing that surgical repair early in life is essential for survival.Case presentationThe African baby residing in Tanzania was referred from a district hospital on the second day of life. She was noted to have a huge omphalocele and ectopia cordis covered by a thin membrane, with bowels visible through the membrane and the cardiac impulse visible just below the epigastrium. Despite the physical anomaly, she appeared to saturate well in room air and had stable vitals. Her chest X-ray revealed the absence of the lower segments of the sternum and echocardiography showed multiple intra-cardiac defects. Based on these findings, the diagnosis of pentalogy of Cantrell was reached. On her fifth day of life, the neonate was noted to have signs of cardiac failure characterized by easy fatigability and restlessness during feeding. Cardiac failure treatment was initiated and she was discharged on parents’ request on the second week of life. Due to inadequate facilities to undertake this complex corrective surgery, arrangements were being made to refer her abroad. In the meantime, her growth and development was satisfactory until the age of 9 months, when she ran out of the medications and succumbed to death. Her parents could no longer afford transport cost to attend the monthly clinic visits, where the infant was getting free medication refill.ConclusionsThe case reported here highlights that in resource limited settings; poor outcome in infants with complex congenital anomalies is a function of multiple factors. However, we believe that surgery would have averted mortality in this 9-month-old female infant. We hope to be able to manage these cases better in future following the recent establishment of cardiac surgery facilities at Muhimbili National Hospital.

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Section: Discussionmentioning

confidence: 99%

Class II pentalogy of Cantrell

et al. 2015

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“…Cantrell sendromunun mortalitesi yüksektir (%95'in üzeri) ve yaşayanlarda bile ciddi morbidite gelişmektedir (3,4).…”

Section: Discussionunclassified

Cantrell Sendromlu Bir Yenidoğan

Öztürk¹,

Saygı²,

Güzeltaş³

et al. 2013

haseki

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“…Survival rate has been reported as less than 40% 3 . However, because of its extreme rarity, there are only several isolated case reports and case series, though no large series or prospective studies 3‐7 . To our knowledge, only a few patients who have undergone complete repair of cardiac anomalies fully recovered and achieved long‐term survival 3,4 .…”

Section: Discussionmentioning

confidence: 99%