新生儿大砍刀综合征的诊断和治疗

Huimin Li
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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. 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引用次数: 0

摘要

目的:通过对1例新生儿大砍刀综合征病例的回顾性分析,为临床诊断、治疗和优生优育提供建议:方法:分析内蒙古包钢医院儿科收治的一例患有大砍刀综合征的新生儿的临床资料,并根据文献资料提出优生建议:患儿,男,自然受孕,G2P2,胎龄38+4周,剖宫产(2021年11月13日上午09:24)。患儿在我科住院 9 天,给予 NCPAP 正压通气吸氧和抗感染对症治疗。患儿的呼吸困难没有明显缓解,心脏杂音越来越明显。患儿于出生后 9 天转入解放军总医院第七医学中心新生儿科接受进一步治疗。入院后给予哌拉西林、他唑巴坦抗感染、有创呼吸机高频振荡模式、限制液体量等对症治疗。纤维支气管镜检查显示右肺无气,左肺气管瘘。超声心动图显示肺静脉引流部分异常、房间隔缺损(继发孔)、下腔静脉扩张、肺动脉高压、初始冠状动脉扩张和动脉导管未闭。肺静脉 CT 3D 成像显示先天性心脏病:部分肺静脉引流异常(心包下),考虑 "弯刀综合征",房间隔缺损,考虑肺动脉高压,右肺动脉狭窄,右下肺接受腹主动脉供血,右肺组织发育不良,下腔静脉肺静脉增宽。因此,综合诊断为 "大砍刀综合征"。考虑到患儿手术风险较高,建议保守对症治疗,治疗22天后家属要求出院。出院后随访3个月,患者在家中死亡:弯刀综合征是一种罕见的先天性肺静脉引流异常,部分或全部肺静脉从右肺或左肺引流至下腔静脉。该综合征的发病率约为十万分之二,通常伴有右肺发育不良、肺栓塞、左上腔静脉持续存在和心脏右倾。在本病例中,患儿出生后就出现了这些综合征,由于基层医院没有发现该病,患儿在上级医院得到了明确诊断。马氏综合征患儿可在新生儿早期进行手术治疗,但手术治疗的指征尚存争议,需根据患儿症状的严重程度及肺循环血流情况评估是否需要手术治疗。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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.
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