Prevalence: 1/182 000. Sporadic but a few X-linked recessive forms (Xq25-q26.1) have been reported. Major form of anomaly of closure of the anterior wall of the trunk with agenesis of:
- the sus-umbilical abdominal wall, generally an omphalocele
- the lower part of the sternum
- the anterior part of the diaphragm
- the diaphragmatic part of the pericardium
and a cardiac malformation (Fallot, VSD, hypoplastic LV) often associated with a diverticulum of the ventricular wall.
Pulmonary hypoplasia is often associated with pulmonary hypertension.
There are 3 types :
- I : the 5 anomalies are present
- II : 4 anomalies are present among which at least the cardiac malformation and the abdominal wall defect
- III : uncomplete expression of the anomalies but agenesis of the lower part of the sternum is present.
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An extreme form is the complete externalization of the heart or ectopia cardis (see this term). In some cases, craniofacial or limb anomalies can be associated (encephalocele, facial cleft).
Often early death.
Anesthetic implications:
surgery is usually necessary in the first days or weeks of life depending on the size of the omphalocele (intact or open ?), the hemodynamic tolerance of the cardiac malformation and the size of the diaphragmatic defect. In addition, the ventricular diverticulum is a very fragile structure. At the time of surgical correction, the combined effects of the cardiac malformation and of the increased intrathoracic pressure caused by closure of the sternal, parietal and diaphragmatial anomaly (Tessier cleft)c defects must be carefuly evaluated. Pulmonary hypoplasia is often associated. Risk of difficult intubation in case of cleft palate or face.
References :
- Laloyaux P, Veyckemans F, Van Dyck M.
Anaesthetic management of a prematurely born infant with Cantrell's Pentalogy.
Paediatr Anaesth 1998; 8: 163-6.
- Suehiro K, Okutani R, Ogawa S, Nakada K, Shimaoka H, Ueda M, Shigemoto T.
Perioperative management of a neonate with Cantrell syndrome.
J Anesth 2009; 23: 572-5
- Nichols JH, Nasr VG.
Sternal malformations and anesthetic management.
Pediatr Anesth 2017 ; 27 : 1084-70.
- Hubbard R, Hayes S, Gillis H, Lindsey S, Malhotra P et al.
Management challenges in an infant with Pentalogy of Cantrell, giant anterior encephalocele, and craniofacial anomalies: a case report.
A&A Practice 2018; 11: 238-40
- Lopez-Rincon RM, Plaza-Lloret M, Munoz-Monaco G, Mavarez AC.
Fascinating image of Pentalogy of Cantrell: navigating challenges for optimal anesthesia care.
Anesthesiology 2024 in press
Updated: March 2024