Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature
Background: Congenital pulmonary airway malformations (CPAMs) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The natural history of prenatally diagnosed CPAM varies from in utero resolution to progression to hydrops and intrauterine death. The majority of fetuses (90%...
| Main Authors: | , , , , |
|---|---|
| Format: | Article |
| Language: | English |
| Published: |
Wolters Kluwer Medknow Publications
2023-01-01
|
| Series: | Indian Pediatrics Case Reports |
| Subjects: | |
| Online Access: | http://www.ipcares.org/article.asp?issn=2772-5170;year=2023;volume=3;issue=3;spage=154;epage=157;aulast= |
| _version_ | 1797245329920229376 |
|---|---|
| author | Dalia Gobbi Elisa Filippi Gabriella Bracalente Francesca Baciorri Paola Midrio |
| author_facet | Dalia Gobbi Elisa Filippi Gabriella Bracalente Francesca Baciorri Paola Midrio |
| author_sort | Dalia Gobbi |
| collection | DOAJ |
| description | Background: Congenital pulmonary airway malformations (CPAMs) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The natural history of prenatally diagnosed CPAM varies from in utero resolution to progression to hydrops and intrauterine death. The majority of fetuses (90%) have a good outcome. However, in a minority (10%), the mass may grow so large that it is associated with complications and becomes life-threatening to the fetus. Clinical Description: Prenatal diagnosis of giant left CPAM type 1 was made at 24 weeks of gestation in a 29-year-old primigravida woman. The lesion consisted of multiple large cysts with a CPAM volume ratio of 4.7. Since the diagnosis, the mass was associated with nonimmune fetal hydrops and polyhydramnios which got worse throughout the following 4 weeks of gestation. Doppler studies remained normal until term. Management: The family received prenatal counseling. Parents refused termination of pregnancy and any other invasive or pharmacological therapy in light of the bad prognosis. The pregnancy was strictly monitored using standard fetal lung parameters. By 31 weeks' gestation, hydrops spontaneously improved to resolution but without any change in the size of the cysts. A baby girl was delivered at term, with birth asphyxia, who, despite all efforts by a multidisciplinary team, could not be revived. Autopsy and histopathological findings were compatible with type 1 CPAM. Conclusion: Hydrops is a well-known complication in fetuses with congenital cystic adenomatoid malformation. The present case reminds the possibility of its spontaneous resolution during pregnancy, even though the cyst size remains unaltered. The eventual neonatal survival depends not on the presence of hydrops but on the cyst volume and underlying lung hypoplasia. |
| first_indexed | 2024-04-24T19:25:11Z |
| format | Article |
| id | doaj.art-549903fa94f24ed5bb9369fbdc863fb5 |
| institution | Directory Open Access Journal |
| issn | 2772-5170 2772-5189 |
| language | English |
| last_indexed | 2024-04-24T19:25:11Z |
| publishDate | 2023-01-01 |
| publisher | Wolters Kluwer Medknow Publications |
| record_format | Article |
| series | Indian Pediatrics Case Reports |
| spelling | doaj.art-549903fa94f24ed5bb9369fbdc863fb52024-03-25T15:39:50ZengWolters Kluwer Medknow PublicationsIndian Pediatrics Case Reports2772-51702772-51892023-01-013315415710.4103/ipcares.ipcares_252_22Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literatureDalia GobbiElisa FilippiGabriella BracalenteFrancesca BaciorriPaola MidrioBackground: Congenital pulmonary airway malformations (CPAMs) comprise a spectrum of anatomical anomalies of the lungs and respiratory tree. The natural history of prenatally diagnosed CPAM varies from in utero resolution to progression to hydrops and intrauterine death. The majority of fetuses (90%) have a good outcome. However, in a minority (10%), the mass may grow so large that it is associated with complications and becomes life-threatening to the fetus. Clinical Description: Prenatal diagnosis of giant left CPAM type 1 was made at 24 weeks of gestation in a 29-year-old primigravida woman. The lesion consisted of multiple large cysts with a CPAM volume ratio of 4.7. Since the diagnosis, the mass was associated with nonimmune fetal hydrops and polyhydramnios which got worse throughout the following 4 weeks of gestation. Doppler studies remained normal until term. Management: The family received prenatal counseling. Parents refused termination of pregnancy and any other invasive or pharmacological therapy in light of the bad prognosis. The pregnancy was strictly monitored using standard fetal lung parameters. By 31 weeks' gestation, hydrops spontaneously improved to resolution but without any change in the size of the cysts. A baby girl was delivered at term, with birth asphyxia, who, despite all efforts by a multidisciplinary team, could not be revived. Autopsy and histopathological findings were compatible with type 1 CPAM. Conclusion: Hydrops is a well-known complication in fetuses with congenital cystic adenomatoid malformation. The present case reminds the possibility of its spontaneous resolution during pregnancy, even though the cyst size remains unaltered. The eventual neonatal survival depends not on the presence of hydrops but on the cyst volume and underlying lung hypoplasia.http://www.ipcares.org/article.asp?issn=2772-5170;year=2023;volume=3;issue=3;spage=154;epage=157;aulast=congenital cystic adenomatoid malformation of lungcounselingfetal hydropsprenatal diagnosis |
| spellingShingle | Dalia Gobbi Elisa Filippi Gabriella Bracalente Francesca Baciorri Paola Midrio Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature Indian Pediatrics Case Reports congenital cystic adenomatoid malformation of lung counseling fetal hydrops prenatal diagnosis |
| title | Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature |
| title_full | Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature |
| title_fullStr | Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature |
| title_full_unstemmed | Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature |
| title_short | Resolution of Fetal hydrops-not a favorable outcome in congenital pulmonary malformation type 1: A case report with review of literature |
| title_sort | resolution of fetal hydrops not a favorable outcome in congenital pulmonary malformation type 1 a case report with review of literature |
| topic | congenital cystic adenomatoid malformation of lung counseling fetal hydrops prenatal diagnosis |
| url | http://www.ipcares.org/article.asp?issn=2772-5170;year=2023;volume=3;issue=3;spage=154;epage=157;aulast= |
| work_keys_str_mv | AT daliagobbi resolutionoffetalhydropsnotafavorableoutcomeincongenitalpulmonarymalformationtype1acasereportwithreviewofliterature AT elisafilippi resolutionoffetalhydropsnotafavorableoutcomeincongenitalpulmonarymalformationtype1acasereportwithreviewofliterature AT gabriellabracalente resolutionoffetalhydropsnotafavorableoutcomeincongenitalpulmonarymalformationtype1acasereportwithreviewofliterature AT francescabaciorri resolutionoffetalhydropsnotafavorableoutcomeincongenitalpulmonarymalformationtype1acasereportwithreviewofliterature AT paolamidrio resolutionoffetalhydropsnotafavorableoutcomeincongenitalpulmonarymalformationtype1acasereportwithreviewofliterature |