Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung
Outcome and treatment in an antenatally diagnosed congenital cystic adenomatoid malformation of the lung
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DOI：
10.1007/s00383-009-2431-x
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发表时间：
2009-09-01
影响因子：
1.8
通讯作者：
Taguchi, Tomoaki
中科院分区：
文献类型：
--
作者：
Nagata, Kouji;Masumoto, Kouji;Taguchi, Tomoaki
产前诊断的先天性肺囊性腺瘤样畸形（CCAM）患者的自然史仍然完全未知。对于有呼吸窘迫症状的患者，在新生儿期进行手术。然而，在无症状的患者中，手术的最佳时机仍然存在争议。在1977年至2007年期间，我们经历了14例产前诊断的CCAM患者。因此，我们调查了产前诊断的CCAM患者的结局，以阐明这类患者的最佳治疗方法。本文回顾了14例患者的产前超声检查结果和产后临床过程。根据临床表现将患者分为三组。A组伴有胎儿水肿（n = 2）， B组出生后刚出现呼吸道症状（n = 6）， C组出生时无症状（n = 6）。回顾三组患者的产后临床过程。A组2例胎儿水肿患者均在出生后不久死亡。B组6例患儿在新生儿期出现严重呼吸窘迫并行手术治疗。在C组，6例患者中有5例无症状，在婴儿早期接受择期手术。在剩下的一名患者中，病变在出生后一段时间内自然消失。B组和C组手术时平均年龄分别为4.5天和4.5个月。C组几乎所有患者在前6个月内都进行了手术。在此观察期间，我们没有遇到任何与CCAM相关的并发症。对于胎儿水肿的患者，胎儿干预被认为是必要的。对于无症状的CCAM患者，建议在婴儿早期进行选择性手术，以防止6个月前CCAM相关并发症的风险。此外，我们建议采用腋窝皮肤折痕切口进行部分肺切除术，以获得良好的术后生活质量。
The natural history of patients with antenatally diagnosed congenital cystic adenomatoid malformation of the lung (CCAM) is still fully unknown. In symptomatic patients with respiratory distress, an operation is performed during the neonatal period. However, in asymptomatic patients, the optimal timing of the operation remains controversial. During the period from 1977 to 2007, we experienced 14 CCAM patients diagnosed antenatally. Therefore, we investigated the outcome of antenatally diagnosed CCAM patients to clarify the optimal treatment for such patients.Fourteen patients were reviewed regarding the antenatal ultrasonography findings and postnatal clinical course. They were then classified into three groups according to the clinical manifestations. Group A was associated with hydrops fetalis (n = 2), group B had respiratory symptoms just after birth (n = 6), and group C was asymptomatic at birth (n = 6). The postnatal clinical courses in three groups were reviewed.In group A, all two patients with hydrops fetalis died just after birth. In group B, six patients had a severe respiratory distress and underwent operation during the neonatal period. In group C, five out of six patients were asymptomatic and received elective operation during the early infant period. In the remaining one patient, the lesion spontaneously disappeared over time after birth. The mean age at the time of operation in group B and group C was 4.5 days and 4.5 months of age, respectively. In almost all patients in group C, we performed an operation within the first 6 months. During this observation period, we did not experience any complications associated with CCAM.In patients with hydrops fetalis, fetal intervention is thought to be needed. In patients with asymptomatic CCAM, an elective operation during the early infant period is recommended to prevent the risk of complications associated with CCAM before 6 months of age. In addition, we recommend the performance of a partial lung resection using an axillary skin crease incision in order to obtain a good postoperative quality of life.