Discussion:
Cantrell’s Pentalogy is a rare syndrome with a high mortality rate and poor prognosis. The long-term prognosis mainly depends on the severity of the cardiac and accompanying defects and requires multidisciplinary management due to the involvement of multisystem anomalies (5). The syndrome is characterized by a partial sternal cleft, anterior abdominal wall defects, anterior diaphragmatic defect, and intracardiac defects (6). The steps for closure are usually chest wall closure, closure of the sternal defect, closure of the omphalocele, placement of the heart in the thorax, and repair of the intracardiac defect (7). Nearly one-third of the cases with this syndrome have left ventricular diverticulum, which is a life-threatening abnormality that may cause systemic embolization, fatal ventricular arrhythmias, and sudden death due to ventricle rupture. Early detection and surgical intervention for this pathology might be lifesaving (8).
Jaime F. Vazquez-Jimenez et al. published a literature review of 153 Cantrell’s Pentalogy cases in 1998. According to the analysis, ventricular septal defect was the most common cardiac malformation (72%). Atrial septal defect was present in 44 (34.6%) of patients, left ventricular diverticulum in 41 (32.3%), pulmonary stenosis or atresia in 40 (31.5%), Tetralogy of Fallot was present in 22 patients (17.3%), dextrocardia in 19 (15.0%) and it was accompanied by various malformations in lesser numbers (6). The analysis showed 3 cases of Cantrell’s Pentalogy, of which the intracardiac defect is limited with Left Ventricle Diverticulum (6%), without any accompanying intracardiac defects. First of these three cases was a 2-year-old female patient with a lower sternal defect, an umbilical hernia, and a ventral diaphragmatic defect; the second case was a 2-year-old male patient with a short sternum, an omphalocele, and a diaphragmatic defect; and the third case was a 7-month-old male patient with a huge ventral hernia, a diaphragmatic defect and a pericardial defect coherent with the incomplete syndrome. In addition, a case of complete syndrome of which the intracardiac defect is limited to the left and right ventricle along with a bifid sternum, an omphalocele, a ventral hernia, and absent pericardium has been reported (9).
Turbendian et al. reported another incomplete case of Cantrell’s Pentalogy with an intracardiac defect limited to the left ventricular diverticulum along with a PDA, an omphalocele, a pericardial, and a diaphragmatic defect (10).
According to the Toyama classification, our case fulfills all requirements of the complete syndrome with supraumbilical abdominal wall defect, lower sternal cleft, defect of the diaphragm in the central tendon, pericardium defect, and left ventricular diverticulum. According to the previous research, the left ventricular diverticulum is a defect related to Cantrell’s Syndrome almost always accompanies other intracardiac defects (11). However, the intracardiac defect is limited to the left ventricle diverticulum in our patient.
In summary, our case is interesting because of the presence of left ventricular diverticulum alone. Also, isolated ventricular diverticulum often accompanies incomplete Cantrell’s cases, and our case is one of the rarest complete Cantrell’s syndromes with no other intracardiac defects.
AUTHOR CONTRIBUTIONS
All authors have contributed to the concept, critical revision, and approval of the final article.
ORCID
Serdar Basgoze: https://orcid.org/0000-0002-6146-2095
Ersin Erek: https://orcid.org/0000-0003-1433-3538
Begum Ogunc: https://orcid.org/0000-0001-8657-0116