Chou Jadzia Tin-Tsen, Kalantarova Anastasia, Borkowska-Kłos Monika, Kornacki Jakub, Szczapa Tomasz, Wender-Ozegowska Ewa
Division of Reproduction, Department of Obstetrics, Gynecology, and Gynecologic Oncology, Poznan University of Medical Sciences, Poznań, Poland.
Department of Neonatal Infectious Diseases, Poznan University of Medical Sciences, Poznań, Poland.
J Matern Fetal Neonatal Med. 2023 Dec;36(2):2239424. doi: 10.1080/14767058.2023.2239424.
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.
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.
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.
先天性肺气道畸形（CPAM）是罕见的散发性病变，常与胎儿预后不良相关。3型CPAM的特征是小的高回声囊肿（<5毫米）。常继发水肿，在这种情况下胎儿存活率约为5%。
我们报告一例大型3型CPAM合并胎儿水肿的病例。该病变在孕19周时被检测到，并在孕29周时通过胎儿磁共振成像得到证实。在孕22周时，给予母亲一个疗程的类固醇作为3型CPAM的可能治疗方法。两次进行腹膜羊膜分流以减少胎儿腹水，但效果不理想。同样，通过两次羊水减量缓解了羊水过多，但很快又复发。一名女婴在孕33周时自然分娩，并在出生后的头三个月接受了两阶段部分肺叶切除术。在两个阶段之间，因饱和度问题需要进行具有挑战性的有创振荡通气。她的预后出乎意料地良好，可能会有良好的生活质量。她现在快一岁了，生长和发育里程碑接近正常。
合并胎儿水肿的3型CPAM与高围产期死亡率相关。虽然开放性胎儿手术在某些专科中心仍然是一个可行的选择，但产前干预通常无效。这名婴儿的存活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生来说是一个重大挑战。多学科的警惕以及频繁的专科随访合作是母婴成功的关键。
