Abstract：
UNASSIGNED: Congenital pulmonary airway malformations (CPAMs) are rare sporadic lesions frequently associated with poor fetal prognosis. Type 3 CPAMs are characterized by small hyperechogenic cysts (<5 mm). Hydrops often develops secondarily, and the fetal survival rate is approximately 5% in this setting.
UNASSIGNED: We present a case of a large type 3 CPAM complicated by fetal hydrops. The lesion was detected at 19 gestational weeks (GW) and confirmed by fetal MRI at 29 GW. At 22 GW, a course of maternal steroids was given as a possible treatment of type 3 CPAM. Peritoneal-amniotic shunt was placed twice to reduce fetal ascites, with unsatisfactory results. Similarly, polyhydramnios was relieved by two amnioreductions, but redeveloped soon after. A baby girl was delivered spontaneously at 33 GW and received a two-stage partial lobectomy in the first three months of life. Desaturations necessitated challenging invasive oscillatory ventilation between stages. Her outcome is unexpectedly positive and she may expect a good quality of life. She now approaches one year of age, with near-to-normal growth and developmental milestones.
UNASSIGNED: Type 3 CPAMs complicated by fetal hydrops are associated with high perinatal mortality. While open fetal surgery remains a viable option in select specialist centers, antenatal interventions are typically ineffective. The survival of this infant can be attributed to prenatal management and early postnatal surgical intervention. The lack of guidelines for ventilation in this setting was a significant challenge for neonatal intensivists. Multidisciplinary vigilance and collaboration with frequent specialist follow ups were the key to success for both mother and child.
UNASSIGNED: We present a case of a large type 3 CPAM complicated by fetal hydrops. The lesion was detected at 19 gestational weeks (GW) and confirmed by fetal MRI at 29 GW. At 22 GW, a course of maternal steroids was given as a possible treatment of type 3 CPAM. Peritoneal-amniotic shunt was placed twice to reduce fetal ascites, with unsatisfactory results. Similarly, polyhydramnios was relieved by two amnioreductions, but redeveloped soon after. A baby girl was delivered spontaneously at 33 GW and received a two-stage partial lobectomy in the first three months of life. Desaturations necessitated challenging invasive oscillatory ventilation between stages. Her outcome is unexpectedly positive and she may expect a good quality of life. She now approaches one year of age, with near-to-normal growth and developmental milestones.
UNASSIGNED: Type 3 CPAMs complicated by fetal hydrops are associated with high perinatal mortality. While open fetal surgery remains a viable option in select specialist centers, antenatal interventions are typically ineffective. The survival of this infant can be attributed to prenatal management and early postnatal surgical intervention. The lack of guidelines for ventilation in this setting was a significant challenge for neonatal intensivists. Multidisciplinary vigilance and collaboration with frequent specialist follow ups were the key to success for both mother and child.
摘要：
■先天性肺气道畸形（CPAMs）是罕见的偶发性病变，常与胎儿预后不良相关。3型CPAM的特征在于小的高回声囊肿(<5mm)。积水通常是次要发展的，在这种情况下，胎儿存活率约为5%。
■我们介绍了一例大型3型CPAM并发胎儿积水的病例。病变在19孕周（GW）时检测到，并在29GW时通过胎儿MRI证实。在22GW时，作为3型CPAM的可能治疗方案,我们给予一个疗程的母体类固醇治疗.两次放置腹膜-羊膜分流术以减少胎儿腹水，结果不满意。同样，羊水过多通过两次羊膜减少缓解，但不久之后就重新开发了.一名女婴在33GW时自发分娩，并在生命的前三个月接受了两阶段部分肺叶切除术。去饱和需要在阶段之间具有挑战性的侵入性振荡通气。她的结果出乎意料地积极，她可能期望有良好的生活质量。她现在快一岁了,具有接近正常的生长和发展里程碑。
■3型CPAM并发胎儿水肿与高围产期死亡率相关。虽然开放式胎儿手术在某些专科中心仍然是可行的选择，产前干预通常是无效的。该婴儿的生存可归因于产前管理和产后早期手术干预。在这种情况下缺乏通气指南对新生儿重症监护医师来说是一个重大挑战。多学科的警惕和与频繁的专家随访的合作是母亲和孩子成功的关键。
■我们介绍了一例大型3型CPAM并发胎儿积水的病例。病变在19孕周（GW）时检测到，并在29GW时通过胎儿MRI证实。在22GW时，作为3型CPAM的可能治疗方案,我们给予一个疗程的母体类固醇治疗.两次放置腹膜-羊膜分流术以减少胎儿腹水，结果不满意。同样，羊水过多通过两次羊膜减少缓解，但不久之后就重新开发了.一名女婴在33GW时自发分娩，并在生命的前三个月接受了两阶段部分肺叶切除术。去饱和需要在阶段之间具有挑战性的侵入性振荡通气。她的结果出乎意料地积极，她可能期望有良好的生活质量。她现在快一岁了,具有接近正常的生长和发展里程碑。
■3型CPAM并发胎儿水肿与高围产期死亡率相关。虽然开放式胎儿手术在某些专科中心仍然是可行的选择，产前干预通常是无效的。该婴儿的生存可归因于产前管理和产后早期手术干预。在这种情况下缺乏通气指南对新生儿重症监护医师来说是一个重大挑战。多学科的警惕和与频繁的专家随访的合作是母亲和孩子成功的关键。
