PDF(Pubmed)
Abstract：
BACKGROUND: Congenital pulmonary airway malformation (CPAM) is a rare congenital dysplastic malformation and accounts for 25% of congenital lung lesions. Commonly, it is diagnosed prenatally in ultrasound. The CPAM volume ratio (CVR) is a well-recognized predictor of fetal prognosis, and when the CVR is >1.6 cm2, the fetus is very likely to develop hydrops and even intrauterine deaths. However, the association of CVR with a wide range of complications and neonatal prognosis is unclear.
METHODS: Cystic lesions in the right thorax of the fetus detected by ultrasound at 19 weeks of gestation, with a CVR of 0.88 cm2. The CVR grew progressively with increasing gestational weeks, reaching a maximum of 5.2 cm2 at 35 gestational weeks. However, there were no complications with the fetus other than polyhydramnios.
METHODS: Imaging and pathological findings confirmed the diagnosis of CPAM.
METHODS: During pregnancy, a multidisciplinary team was involved in the management and the prenatal visits increased to weekly from 31 weeks of gestation. During the cesarean section, neonatologists and pediatric surgeons were present for timely evaluation of newborns. The neonate was admitted to the neonatal intensive care unit for monitoring immediately after birth and underwent thoracoscopic right lower lobectomy at 57th days old.
RESULTS: The neonate recovered without any respiratory symptoms and no abnormality on chest computed tomography (CT) at the 3-month postoperative follow-up.
CONCLUSIONS: During pregnancy, in addition to monitoring CVR, a multidisciplinary team should join in the management of CPAM patients. And as for the fetus with increased CVR, a closely monitoring after birth is necessary even if the general condition of the pregnancy is well. In particular, timely intervention should be made at the onset of respiratory symptoms.
METHODS: Cystic lesions in the right thorax of the fetus detected by ultrasound at 19 weeks of gestation, with a CVR of 0.88 cm2. The CVR grew progressively with increasing gestational weeks, reaching a maximum of 5.2 cm2 at 35 gestational weeks. However, there were no complications with the fetus other than polyhydramnios.
METHODS: Imaging and pathological findings confirmed the diagnosis of CPAM.
METHODS: During pregnancy, a multidisciplinary team was involved in the management and the prenatal visits increased to weekly from 31 weeks of gestation. During the cesarean section, neonatologists and pediatric surgeons were present for timely evaluation of newborns. The neonate was admitted to the neonatal intensive care unit for monitoring immediately after birth and underwent thoracoscopic right lower lobectomy at 57th days old.
RESULTS: The neonate recovered without any respiratory symptoms and no abnormality on chest computed tomography (CT) at the 3-month postoperative follow-up.
CONCLUSIONS: During pregnancy, in addition to monitoring CVR, a multidisciplinary team should join in the management of CPAM patients. And as for the fetus with increased CVR, a closely monitoring after birth is necessary even if the general condition of the pregnancy is well. In particular, timely intervention should be made at the onset of respiratory symptoms.
摘要：
背景：先天性肺气道畸形（CPAM）是一种罕见的先天性发育不良畸形，占先天性肺病变的25％。通常,产前超声诊断。CPAM体积比（CVR）是公认的胎儿预后预测指标，当CVR>1.6cm2时，胎儿很可能发生积水，甚至宫内死亡。然而,CVR与多种并发症和新生儿预后的关系尚不清楚.
方法：在妊娠19周时通过超声检查发现胎儿右侧胸部的囊性病变，CVR为0.88cm2。随着孕周的增加，CVR逐渐增长，在35孕周达到最大5.2cm2。然而,除羊水过多以外，胎儿没有其他并发症。
方法：影像学和病理结果证实了CPAM的诊断。
方法：在怀孕期间，一个多学科小组参与管理,产前检查从妊娠31周增加到每周一次.在剖腹产期间,新生儿科医师和儿科医生出席了对新生儿的及时评估.新生儿出生后立即被送往新生儿重症监护病房进行监测，并在第57天大时接受了胸腔镜右下肺叶切除术。
结果：术后3个月随访，新生儿恢复无任何呼吸道症状，胸部计算机断层扫描（CT）无异常。
结论：在怀孕期间，除了监控CVR，多学科团队应参与CPAM患者的管理.至于CVR增加的胎儿，即使怀孕的一般状况良好，出生后也需要密切监测。特别是,呼吸道症状发作时应及时干预。
方法：在妊娠19周时通过超声检查发现胎儿右侧胸部的囊性病变，CVR为0.88cm2。随着孕周的增加，CVR逐渐增长，在35孕周达到最大5.2cm2。然而,除羊水过多以外，胎儿没有其他并发症。
方法：影像学和病理结果证实了CPAM的诊断。
方法：在怀孕期间，一个多学科小组参与管理,产前检查从妊娠31周增加到每周一次.在剖腹产期间,新生儿科医师和儿科医生出席了对新生儿的及时评估.新生儿出生后立即被送往新生儿重症监护病房进行监测，并在第57天大时接受了胸腔镜右下肺叶切除术。
结果：术后3个月随访，新生儿恢复无任何呼吸道症状，胸部计算机断层扫描（CT）无异常。
结论：在怀孕期间，除了监控CVR，多学科团队应参与CPAM患者的管理.至于CVR增加的胎儿，即使怀孕的一般状况良好，出生后也需要密切监测。特别是,呼吸道症状发作时应及时干预。
