Pentalogy of Cantrell

Balderrabano-Saucedo, Norma; Vizcaíno-Alarcón, Alfredo; Sandoval-Serrano, Erika; Segura-Stanford, Begoña; Arévalo-Salas, Luis Alexis; Cruz, Lorenzo Reyes de la; Espinosa-Islas, Gonzalo; Puga-Muñuzuri, Francisco Javier

doi:10.1177/2150135110390528

Cited by 22 publications

(8 citation statements)

References 23 publications

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“…A wide range of intracardiac anomalies have been associated with PC including septal defects, tetralogy/pentalogy of Fallot, total anomalous pulmonary venous drainage, and left ventricular diverticulum [7,8,9,10,11]. Holt-Oram syndrome, which was seen in the family of Case 1, is associated with cardiac anomalies but has never been reported with PC.…”

Section: Discussionmentioning

confidence: 99%

Complete and Incomplete Pentalogy of Cantrell

Kylat

2019

Children

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Section: Discussionmentioning

confidence: 99%

Complete and Incomplete Pentalogy of Cantrell

Kylat

2019

Children

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“…Since it is very rare to have an intact pericardium and ectopia cordis we may have missed the pericardial defect, which would put the patient in the class I form of the syndrome. However, Pachajoa et al had also described a case with an absent diaphragmatic pericardial defect in presence of a midline defect, upper abdominal wall abnormality, lower sternal defect and an anterior diaphragmatic defect [ 32 ]. The intracardiac defects detected in this patient included VSD and ASD.…”

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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“…Cervical, cervicothoracic, thoracic, and thoracoabdominal types of EC have been described. Ectopia cordis can be an isolated defect, but more frequently is a part of a PC [4]. Fetal echocardiography is an excellent tool for EC diagnosis [5]; however, the use of prenatal magnetic resonance imaging was described to enhance the exact anatomy visualization [6].…”

Section: Discussionmentioning

confidence: 99%