Diagnosis and treatment of pentalogy of Cantrell / 国际儿科学杂志

ABSTRACT

Pentalogy of Cantrell is a rare congenital anomaly characterized by a defect of the sternum, a deficiency of the diaphragm, a defect in the pericardium, an abdominal wall defect and congenital heart disease, with or without involvement of other organ systems.The incidence is low, and the prognosis is poor with high rates of perinatal mortality.The etiology and pathogenesis are still unknown.The early diagnosis and timely treatment are important.The prenatal diagnosis may be made by ultrasound as early as the first trimester, and for infants, it can be diagnosed with clinical features, ultrasound, CT and other imaging findings.Surgical repair is the main treatment.The survival depends mostly on associated cardiac anomalies, degree of thoraco-abdominal defect, and so on.There is a review that the diagnosis and treatment of the disease are summarized.