Diagnosis and treatment of neonatal machete syndrome

Aim: To provide suggestions for clinical diagnosis, treatment and eugenics through the retrospective analysis of a case of neonatal machete syndrome.Methods: The clinical data of a newborn with machete syndrome admitted to the pediatrics department of Baogang Hospital of Inner Mongolia were analyzed, and suggestions of eugenics were provided based on the literatures.Results: The child, male, natural conception, G2P2, gestational age 38+4 weeks, was born by cesarean section (at am 09:24 on November 13th, 2021). He was hospitalized in our department for 9 days and given NCPAP positive pressure ventilation to give oxygen and anti-infection symptomatic treatment. The dyspnea of the child was not significantly alleviated, and the heart murmurs became increasingly obvious. The child was transferred to the neonatology Department of the Seventh Medical Center of the PLA General Hospital for further treatment on 9 days after birth. After admission, piperacillin and tazobactam were treated with anti-infection, high-frequency oscillation mode of invasive ventilator, limited fluid volume and other symptomatic treatments. Fiberbronchoscopy showed atelectasis of right lung and tracheomalacia of left lung. Echocardiography showed partial anomalous pulmonary venous drainage, atrial septal defect (secondary foramina), inferior vena cava dilatation, pulmonary hypertension, initial coronary artery dilatation, and patent ductus arteriosus. CT 3D imaging of pulmonary veins showed: Congenital heart disease: partial anomalous pulmonary venous drainage (subcardial), consideration of "machete syndrome," atrial septal defect, consideration of pulmonary hypertension, right pulmonary artery stenosis, right lower lung receiving abdominal aorta blood supply, right lung tissue dysplasia, and widening of inferior vena cava pulmonary veins. Therefore, the comprehensive diagnosis was "machete syndrome." Considering the high surgical risk of the child, conservative symptomatic treatment was recommended, and the family requested discharge after 22 days of treatment. The patient died at home at 3 months follow-up after discharge.Conclusions: Machete syndrome is a rare congenital property of abnormal partial or complete pulmonary venous drainage from the right or left lung to the inferior vena cava. With an incidence of approximately 2 in 100,000 live births, the syndrome is commonly associated with right lung dysplasia, pulmonary sequestration, persistent left superior vena cava, and right tilt of the heart. In this case, the child had these syndromes after birth, since the primary hospital did not recognize the disease, the child was clearly diagnosed in the higher hospital. Children with machete syndrome can be treated with surgery in the early neonatal period, but the indication of surgical treatment is still controversial, and the need for surgery is evaluated according to the severity of the patient's symptoms and pulmonary circulation blood flow.