Incomplete pentalogy of Cantrell: single stage repair
- Department of Pediatric cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Bangalore, Karnataka, India
- Correspondence to Dr M K Usha, Assistant Professor Pediatric Cardiology, Department of Pediatric cardiology, Sri Jayadeva Institute of Cardiovascular Sciences and Research, Jayanagar 9th block, Bangalore 560069, India;
Contributors JM: performed echocardiogram and angiogram, diagnosis and referral to surgeon. MKU: compiling and drafting of the article. DNS: performed the single stage complete repair of all the defects.
- Received 19 December 2011
- Accepted 21 February 2012
- Published Online First 11 June 2012
An 11-month-old boy presented with complete bilateral cleft lip and a palpable, pulsating midline abdominal mass since birth. His growth and development were normal. Echocardiography showed a diverticulum arising from left ventricular (LV) apex. Angiography showed a long tubular LV apical diverticulum extending into abdomen anterior to liver in antero-posterior view (figure 1/video 1) and in lateral view (figure 2/video 2). There was no other anomaly and ventricular function was normal. A one-stage repair was undertaken. A true LV apical diverticulum covered by pericardium was observed arising from the inferior aspect of the LV apex and extending down preperitoneally behind the rectus muscle for 6 cm. On a beating heart, the diverticulum was carefully dissected and excised. Closure of the pericardial defect, ventral diaphragmatic defect, umbilical hernia repair and bilateral cleft lip repair was done. This child is an example of incomplete pentalogy of Cantrell.
Pentalogy of Cantrell is a rare midline syndrome.1 The pentad of findings includes defects in lower sternum, abdominal wall, anterior portion of the diaphragm, diaphragmatic portion of the pericardium and cardiac defects.1 There may be associated cardiac lesions like diverticulum, ventricular or atrial septal defect or complex lesions. Ectopia cordis is an exceedingly rare association.1 Congenital diverticulums are usually single and arise most commonly from LV apex.2 The incidence varies from 0.26% to 0.4%. The complications of diverticulum are thrombosis, embolism, and arrhythmia and very rarely rupture. An LV diverticulum should initiate a search for other features of pentalogy of Cantrell. Only occasionally is one-stage repair of all the anomalies possible. The present case highlights the varied system involvement and the need for innovative multidisciplinary management strategies to achieve better outcomes.
Patient consent Obtained.
Ethics approval Ethics approval was provided by Ethics Committee of Sri Jayadeva Institute of Cardiovascular Research and Sciences.
Provenance and peer review Not commissioned; internally peer reviewed.