[Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung].

[Antenatal ultrasound diagnosis and neonatal results of the congenital cystic adenomatoid malformation of the lung].
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DOI:
10.4067/s0370-41062018000200224
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发表时间:
2018-04
期刊:
Revista chilena de pediatria
影响因子:
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通讯作者:
Margarita Gallardo A;Margarita Álvarez de la Rosa R;José F De Luis E;Lorena Mendoza R;Ana Isabel Padilla P;Juan Troyano L
Margarita Gallardo A;Margarita Álvarez de la Rosa R;José F De Luis E;Lorena Mendoza R;Ana Isabel Padilla P;Juan Troyano L
中科院分区:
其他
文献类型:
--
作者:
Margarita Gallardo A;Margarita Álvarez de la Rosa R;José F De Luis E;Lorena Mendoza R;Ana Isabel Padilla P;Juan Troyano L

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引言 先天性囊性腺瘤样畸形(CCAM)是一种罕见的先天性肺部疾病,在大多数情况下,产前诊断是可行的。产前管理和产后治疗存在差异。目的 分析我院医院产前诊断的 CCAM,以评估超声检查结果与胎儿和产后演变的关系。患者和方法 对两家参考医院 2005 年至 2016 年间所有超声产前诊断病例进行回顾性研究。超声检查使用高分辨率超声扫描仪 Toshiba Xario 和 Voluson 730 Expert Pro 进行,并进行从诊断到分娩的跟踪。分析的变量包括诊断时的孕龄、肺部病变的特征、相关畸形、细胞遗传学研究、妊娠进展、分娩类型、呼吸窘迫的存在、补充影像学检查的需要、儿科临床病程和必要的产后治疗。产前超声检查中病变完全消失或产后胸部X光检查未显示病变则视为解决。结果 17例均产前诊断。演变范围从病变的产前消退到出生后的持续存在。三名患者因超声检查结果显示预后不良而自愿决定堕胎。在剩下的 14 例病例中,没有胎儿或新生儿死亡的病例,其中 1 例需要在出生后进行手术,4 例患者在出生后第一年出现轻微症状。报道了 1 例新生儿死亡假阴性病例,尸检报告为 CCAM 0 型。结论:这种肺部畸形预后良好,排除胎儿水肿的病例。二维超声通常足以用于诊断和随访。计算机断层扫描是确认出生后病变消退的首选技术。手术治疗优于保守治疗,尽管尚不清楚这种疾病的潜在并发症(即使无症状)是否证明手术发病是合理的。
INTRODUCTION Congenital cystic adenomatoid malformation (CCAM) is a rare congenital lung di sease, and in the most of cases, prenatal diagnosis is feasible. There are discrepancies regarding pre natal management and postpartum treatment. OBJECTIVE To analyze prenatally diagnosed CCAM in our hospitals, in order to evaluate ultrasound findings with fetal and postnatal evolution. PATIENTS AND METHOD Retrospective study of all cases diagnosed prenatally by ultrasound between 2005 and 2016 in two reference hospitals. The ultrasounds were performed using high-resolution ultrasound scanners, Toshiba Xario and Voluson 730 Expert Pro, with follow-up from diagnosis to delivery. The variables analyzed included gestational age at diagnosis, the characteristics of the lung lesion, associated malformations, cytogenetic study, the evolution of pregnancy, type of delivery, presence of respiratory distress, need for complementary imaging tests, pediatric clinical course, and necessary postnatal treatments. It was considered a resolution the total disappearance of the lesion in the pre natal ultrasound or that the postnatal chest X-ray showed no lesion. RESULTS 17 cases were prenatally diagnosed. The evolution ranges from the prenatal resolution of the lesion to the persistence after bir th. Three patients voluntarily decided to have an abortion due to ultrasound findings of poor progno sis. Of the fourteen remaining cases there were no cases of fetal or neonatal deaths, one case required surgery after birth and four patients had mild symptoms during the first year of life. One case of false negative with neonatal death has been reported which necropsy reported as CCAM type 0. Conclu sions: This pulmonary malformation presents good prognosis, excluding cases with fetal hydrops. Two-dimensional ultrasound is usually enough for diagnosis and follow-up. Computed tomography is the technique of choice to confirm the resolution of lesions after birth. Surgical treatment is pre ferable over conservative management, although it is unknown if the potential complications of this disease, even when asymptomatic, justify surgical morbidity.