Ann Clin Case Rep | Volume 7, Issue 1 | Case Report | Open Access

Multidisciplinary Management of a Large Microcystic Congenital Pulmonary Airway Malformation

Jadzia Tin-Tsen Chou1*, Anastasia Kalantarova1, Monika Borkowska-Kłos2, Jakub Kornacki1 and Ewa Wender-Ozegowska1

1Department of Obstetrics, Gynecology, and Gynecologic Oncology, Division of Reproduction, Poznan University of Medical Sciences, Poland 2Department of Neonatal Infectious Diseases, Poznan University of Medical Sciences, Poland

*Correspondance to: Jadzia Tin-Tsen Chou 

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Introduction: 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. Case Report: 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. She now approaches one year of age, with near-to-normal growth and developmental milestones. Discussion: The survival of this infant can be attributed to prenatal management, accurate diagnostic imaging, and early postnatal surgical intervention. Her outcome is unexpectedly positive and she may expect a good quality of life. Multidisciplinary vigilance and collaboration with frequent specialist follow ups was the key to success for both mother and child.


CPAM; CVR; Peritoneal-amniotic shunt; Polyhydramnios; Fetal hydrops


Tin-Tsen Chou J, Kalantarova A, Borkowska-Kłos M, Kornacki J, Wender-Ozegowska E. Multidisciplinary Management of a Large Microcystic Congenital Pulmonary Airway Malformation. Ann Clin Case Rep. 2022; 7: 2197..

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