Fascinating Image of Pentalogy of Cantrell: Navigating Challenges for Optimal Anesthesia Care

Pentalogy of Cantrell is a rare congenital condition with an incidence of one case for every 65,000 to 200,000 live births with around 250 probably underreported cases described in modern literature.  Pentalogy of Cantrell consists of five midline birth defects in their complete form, which include ectopia cordis, supraumbilical abdominal wall defect, sternal cleft, diaphragmatic pericardial defect, and anterior diaphragmatic hernia. It is the result of abnormal development in the ventral mesoderm on the third week after conception with the lateral mesodermal folds failing to migrate to the midline.  A fetal ultrasonography during the first trimester may be used for the diagnosis, and an echocardiography performed at birth would demonstrate cardiac defects that can be missed if performed intrauterine^. 

Figures 1 and 2 show a 2-day-old male, 2.13 kg, born at 33 weeks’ gestation via vaginal delivery without prenatal care with Pentalogy of Cantrell diagnosed after birth. Figure 1 shows that the heart (A), diaphragm (B), liver (C), spleen (D), and stomach and small and large intestines (E) were protruding from the abdominal wall. Multiple surgeries are necessary to reduce the eviscerated organs and correct frequent cardiac malformations such as ventricular or septal defects and left ventricular diverticulae.  Ventricular diverticulum is usually associated with thromboembolic events, and early repair is recommended. Atrial and ventricle septal defects may be treated months later after birth in stable conditions. Anesthetic considerations include hemodynamic variations and arrhythmias related to heart compression. Early intubation with IV midazolam, vecuronium, and sevoflurane can be used for induction. Administration of broad-spectrum antibiotics as well as adequate coverage of the viscera may reduce the risk of sepsis and severe dehydration with electrolyte imbalance. Lung hypoplasia and pulmonary hypertension are common; High-frequency oscillatory ventilation mode with low pulmonary volumes may avoid barotrauma and pneumothorax. Surgical table pads protect against tissue damage, pressure ulcers, and nerve injuries related to impaired blood flow by redistributing pressure evenly across the body during lengthy procedures. A cleft palate or cleft lip increases the risk of a difficult airway. Elevated intraabdominal pressures after abdominal wall closure may compromise cardiovascular function and renal perfusion, therefore postoperative intensive care unit management should be mandatory.