Perioperative management of a neonate with Cantrell syndrome

Abstract

Cantrell syndrome is a congenital malformation with a pentalogy characterized by defects involving the abdominal wall, lower sternum, anterior diaphragm, and diaphragmatic pericardium, as well as congenital cardiac anomalies. We recently managed anesthesia in a patient with this syndrome and herein report our experience. The patient was a 14-day-old male neonate, who had been diagnosed with Cantrell syndrome, including ventricular septal defect, left ventricular diverticulum, abdominal wall defect, omphalocele, and sternal hypoplasia. Surgical interventions to close the ventricular septal defect, resect the left ventricular diverticulum, and close the omphalocele were scheduled. After cardiac surgery, the hernial contents were returned to their original compartment and, subsequently, an attempt was made to suture the abdominal wall. However, blood pressure fell markedly and the attempt was discontinued. The chest was left open postoperatively and the patient was transferred to the intensive care unit (ICU), during which time circulatory and respiratory management was very complex. Issues requiring particular attention in the management of anesthesia for patients with this syndrome include complications of diverse cardiac malformations, pulmonary hypertension, pulmonary hypoplasia, and respiratory and circulatory failure associated with increased intraabdominal pressure due to primary closure of the omphalocele. Accordingly, extreme caution must be taken to restore respiratory and circulatory control.

Cantrell syndrome with complex cardiac malformations: a case report

“…This recommendation has been adopted by most surgeons who report Cantrell syndrome [7,8]. Onestage definitive repair has been attempted with success [9,10] but has also resulted in rapid death [11]. Unsuccessful 1-stage repair is likely underreported.…”

Pentalogy of Cantrell and anaesthesia: a case report

“…11 Monitoring for acute respiratory and cardiac failure is essential because of the potential for raised intra-abdominal pressure with reduction of the omphalocele 4 and resultant herniation of the reduced abdominal contents through the diaphragmatic hernia.…”

“…2 There is a risk of hypothermia and fluid/electrolyte losses due to exposure of the heart and viscera. 4 Other anomalies may include: hydrocephalus 11 or anencephaly, 2 cleft lip/palate 11 with resultant potentially difficult airway, renal or skeletal abnormalities and sternal defects.…”

“…The intracardiac associations are typically ventricular septal defects, atrial septal defects, pulmonary stenosis, Tetralogy of Fallot and left ventricular diverticulae. 1,4,5 By identifying the typical features on ultrasonography, POC can usually be diagnosed at the end of the first trimester. 3 The degree of severity varies and was classified by Toyama in 1972 based on a review of 61 cases of POC.…”

“…2 It is due to a developmental abnormality at about 3-4 weeks post-conception in the ventral mesoderm. 4,9 A multidisciplinary approach is essential in planning the management of these cases. 5,7 Should they survive, these cases require multiple surgeries.…”