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胎儿先天性大叶性肺气肿的诊断与治疗 被引量:8

Diagnosis and management of fetal congenital lobar emphysema
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摘要 目的胎儿先天性大叶性肺气肿(CLE)的产前超声很容易与肺囊腺瘤Ⅲ型(CCAMIH)混淆,因为二者均表现为同一血供的高回声囊性肿块,产前超声难以鉴别。由于二者的临床结局不同,为提高对CLE的认识,结合新生儿的结局探讨胎儿CLE的诊断和治疗。方法回顾性分析广东省妇幼保健院胎儿医学科2011年3月至2013年5月产前诊断和出生后确诊为CLE的诊治经验。结果在胎儿产前超声诊断为胸部囊性病变的基础上,52例确诊为CCAMm,其中6例(11%,6/52例)于出生后CT确诊为CLE,5例于出生后早期手术,术后病理组织诊断。6例中3例出生后即出现急性呼吸道症状,2例于新生儿期急诊手术治愈,1例放弃治疗;另3例分别于出生后第7天、第19天及3个月出现呼吸道症状行手术治愈。所有受累部位以上肺叶为主(83%,5/6例)。手术肺叶切除部位分别为左上肺叶2例,右上肺1例,右上肺叶和右中肺叶1例,右下肺叶1例。手术年龄3~90d,平均20d。手术病例均治愈。结论产前胎儿CLE与CCAMⅢ型在超声上尚无法鉴别,但出生时或出生后较早出现呼吸道症状并导致新生儿急诊和治疗;CT可明确诊断。以往认为的CCAMⅢ预后差是否为将CLE误诊为CCAM Ⅲ,需要重新认识;对于CLE新生儿急性呼吸道症状者行新生儿外科治疗是积极有效的。 Objective Fetal congenital lobar emphysema (CLE) is generally confused with congenital cystic adenomatoid malformation type Ⅲ ( CCAM m) by prenatal ultrasound as both are hyperechoic cystic mass with the same blood supply, which is difficult to differentiate through the prenatal ultrasound. As they have different clinical out- comes, and in order to improve the understanding of CLE,the diagnosis and treatment of neonatal outcome of fetal CLE were discussed. Methods A retrospective analysis was conducted of the experience in prenatal diagnosis and treatment of postnatal CLE at Department of Fetal Medicine, Guangdong Women and Children's Hospital from Mar. 2011 to May 2013. Results On the basis of prenatal ultrasound for fetal chest cystic malformations,there were 52 cases of CCAM Ⅲ in which 6 cases( 11% ,6/52 cases) were confirmed as CLE by CT scan after birth and 5 cases had early operation. Emphysema was detected in all pathological specimens. Of 3 cases presenting symptoms of tachypnea after birth,2 had acute lobectomy and 1 gave up therapy. The other 3 cases underwent lobectomy on 7 day, 19 day and 3 months respec- tively after birth because of tachypnea and respiratory distress. The most common affected lobe was the upper lobe (83 % ,5/6 cases). The lobectomy included 2 left upper lobe, 1 right upper lobe, 1 right upper and middle lobe, and 1 right lower lobe. The mean age of lobectomy was 20 days ( from 3 to 90 days). All the cases were cured through lobecto- my. Conclusions Prenatal CLE can not be differentiated from CCAM Ill on ultrasound, but it exhibited early pulmona- ry distress at birth or after birth leading to neonatal emergency and treatment. It can be confirmed by computed tomo- graphy scan. Previously CCAM Ⅲ prognosis was thought to be poor and it remains unconfirmed whether CLE had been misdiagnosed as CCAM m need to know. For the CLE with neonatal acute respiratory symptoms early surgical treatment is effective.
出处 《中华实用儿科临床杂志》 CAS CSCD 北大核心 2014年第11期818-820,共3页 Chinese Journal of Applied Clinical Pediatrics
关键词 胎儿 先天性大叶性肺气肿 肺囊腺瘤Ⅲ型 新生儿外科 Fetal Congenital lobar emphysema Congenital cystic adenomatoid malformation type Ⅲ Neonatalsurgery
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