Adzick N S, Harrison M R, Crombleholme T M, Flake A W, Howell L J
The Center for Fetal Diagnosis and Treatment at the Children's Hospital of Philadelphia, Pennsylvania 19104, USA.
Am J Obstet Gynecol. 1998 Oct;179(4):884-9. doi: 10.1016/s0002-9378(98)70183-8.
Our purpose was to review our experience with fetal congenital cystic adenomatoid malformation and extralobar pulmonary sequestration emphasizing natural history, management, and outcome.
We conducted a retrospective review of 175 fetal lung lesions diagnosed by antenatal ultrasonography at 2 fetal treatment centers.
There were 134 congenital cystic adenomatoid malformation cases. Fourteen women underwent elective abortion, 101 women were managed expectantly, 13 women had fetal surgery, and 6 women had placement of a thoracoamniotic shunt. For the congenital cystic adenomatoid malformation lesions that were not associated with nonimmune hydrops, all babies survived. Of 25 large congenital cystic adenomatoid malformations that had associated hydrops that were followed expectantly, all fetuses died before or shortly after birth. Fetal surgical resection of the tumor (fetal lobectomy) was performed at 21 to 29 weeks' gestation in 13 hydropic fetuses with 8 fetuses continuing gestation with subsequent hydrops resolution, impressive in utero lung growth, and neonatal survival. Six fetuses with a very large solitary cyst underwent thoracoamniotic shunting and 5 survived. There were 41 extralobar pulmonary sequestration cases. Twenty-eight extralobar pulmonary sequestrations dramatically regressed on serial prenatal sonography, were asymptomatic after birth, and were only detectable by imaging studies postnatally (no resection required). Of the remaining 13 extralobar pulmonary sequestration cases, 2 underwent elective abortion, 7 symptomatic lesions were resected after birth with survival, 1 hydropic fetus died, and 3 fetuses had an associated tension hydrothorax with secondary hydrops that was successfully treated by either fetal thoracenteses or thoracoamniotic shunting followed by postnatal resection.
The natural history of prenatally diagnosed lung masses is variable, and associated anomalies are rare. Most congenital cystic adenomatoid malformation lesions can be managed with maternal transport, planned term delivery, and postnatal resection. Many extralobar pulmonary sequestrations dramatically decrease in size before birth and may not need treatment after birth. Fetal therapy is now an option for lung lesions associated with nonimmune hydrops.
我们的目的是回顾我们在胎儿先天性囊性腺瘤样畸形和肺叶外肺隔离症方面的经验，重点关注其自然病史、治疗方法及治疗结果。
我们对两家胎儿治疗中心通过产前超声诊断的175例胎儿肺部病变进行了回顾性研究。
先天性囊性腺瘤样畸形病例有134例。14名女性选择了人工流产，101名女性采取期待治疗，13名女性接受了胎儿手术，6名女性进行了胸腔羊膜分流术。对于与非免疫性水肿无关的先天性囊性腺瘤样畸形病变，所有婴儿均存活。在25例伴有水肿且采取期待治疗的大型先天性囊性腺瘤样畸形病例中，所有胎儿均在出生前或出生后不久死亡。13例水肿胎儿在妊娠21至29周时接受了肿瘤的胎儿手术切除（胎儿肺叶切除术），其中8例胎儿继续妊娠，随后水肿消退，子宫内肺生长显著，新生儿存活。6例患有非常大的孤立囊肿的胎儿接受了胸腔羊膜分流术，5例存活。肺叶外肺隔离症病例有41例。28例肺叶外肺隔离症在系列产前超声检查中显著缩小，出生后无症状，仅在出生后通过影像学检查可检测到（无需切除）。在其余13例肺叶外肺隔离症病例中，2例选择了人工流产，7例有症状的病变在出生后切除并存活，1例水肿胎儿死亡，3例胎儿伴有张力性胸腔积液及继发性水肿，通过胎儿胸腔穿刺或胸腔羊膜分流术成功治疗，随后在出生后切除。
产前诊断的肺部肿块的自然病史各不相同，且相关异常情况罕见。大多数先天性囊性腺瘤样畸形病变可通过产妇转运、计划足月分娩及出生后切除进行治疗。许多肺叶外肺隔离症在出生前体积显著减小，出生后可能无需治疗。胎儿治疗现在是与非免疫性水肿相关的肺部病变的一种选择。
