肺动脉供血的先天性叶外型肺隔离症1例

Congenital extralobar pulmonary sequestration supplied by pulmonary artery:a case report

本文报道了1例肺动脉供血的左上叶先天性肺隔离症的临床特点。该患儿母亲孕24周产前B超发现胎儿左侧胸腔可见一高回声均匀密度肿块,病变体积与头围比值为1.16,供血来源于左肺动脉。孕27周完善MRI检查提示胎儿左肺体积增大,容积约48.52 ml,右肺容积约8.56 ml,总肺容积约57.08 ml。产前诊断考虑左侧先天性肺气道畸形,左侧支气管闭锁或先天性肺叶过度膨胀待排。孕38周^(+1)经产钳助产娩出,新生儿1、5、10 min Apgar评分均9分,生后胸部CT和MRI均考虑先天性肺气道畸形,左上叶支气管闭锁待排。生后第2天完善纤维支气管镜检查排除支气管闭锁,考虑叶外型肺隔离症。生后呼吸促,先后予吸氧、无创及有创呼吸机辅助通气等保守治疗2周后症状无缓解,予胸腔镜探查术,术中证实为左侧肺隔离症,异常肺组织基底处可见左肺动脉供血,予胸腔镜下左侧病变肺组织切除术,术后病理证实为肺隔离症,术后恢复好,顺利出院。

We report the clinical features of a case of congenital extralobar pulmonary sequestration in the left upper lobe supplied by the pulmonary artery.Prenatal ultrasound examination at 24 weeks of gestation revealed a high echogenic and uniform density mass in the fetal left thoracic cavity with the congenital pulmonary airway malformation volume vatio(CVR)of 1.16,which was supplied by pulmonary arterial vessels.MRI examination at 27 weeks indicated that the left lung volume increased to about 48.52 ml,while the right lung volume was about 8.56 ml giving the total lung volume of 57.08 ml.The congenital pulmonary airway malformation in the left upper lobe was suspected to be congenital bronchial atresia(CBA)or congenital lobar overinflation(CLO).The baby boy was born through vaginal delivery assisted by forceps at 38^(+1) weeks without neonatal asphyxia.Postnatal CT and MRI were both indicated suspicious bronchial atresia in the left upper lobe.Bronchofibroscopy on postnatal day 2 excluded CBA or CLO and extralobar pulmonary sequestration was considered.Thoracoscopic surgery was performed due to continuous shortness of breath after birth,despite two-week conservative treatment including oxygenation,invasive and non-invasive mechanical ventilation,etc,and congenital extralobar sequestration was diagnosed.Blood supply from the left pulmonary artery was observed at the base of abnormal lung tissue.Resection of the pathogenic tissue of the left lung was performed thoracoscopically.The boy recovered and was discharged after the operation.Pulmonary sequestration was confirmed by histopathology.