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