Minimally invasive therapeutic approach to fetal congenital pulmonary airway malformation: Case report and literature review: Fetal congenital pulmonary airway malformation

Alexandre Da Silva Rocha; Juliana Perotoni Dondé; Mariana Morais Pereira; Arthur Martiny; Andre Campos da Cunha; Jorge Bianchi Telles; Vinicius Toniolli

doi:10.28982/josam.8470

Authors

Alexandre Da Silva Rocha Santa Casa De Misericórdia De Porto Alegre and Universidade Federal De Ciências Da Saúde De Porto Alegre (Ufcspa), Porto Alegre, Rio Grande Do Sul, Brazil https://orcid.org/0000-0002-9283-0559
Juliana Perotoni Dondé Santa Casa De Misericórdia De Porto Alegre and Universidade Federal De Ciências Da Saúde De Porto Alegre (Ufcspa), Porto Alegre, Rio Grande Do Sul, Brazil https://orcid.org/0000-0001-5658-5261
Mariana Morais Pereira Santa Casa De Misericórdia De Porto Alegre and Universidade Federal De Ciências Da Saúde De Porto Alegre (Ufcspa), Porto Alegre, Rio Grande Do Sul, Brazil https://orcid.org/0000-0002-8452-5837
Arthur Martiny Santa Casa De Misericórdia De Porto Alegre and Universidade Federal De Ciências Da Saúde De Porto Alegre (Ufcspa), Porto Alegre, Rio Grande Do Sul, Brazil https://orcid.org/0009-0007-3692-7595
Andre Campos da Cunha Presidente Vargas Maternity and Children’s Hospital, Porto Alegre, Brazil https://orcid.org/0000-0002-9893-3348
Jorge Bianchi Telles Presidente Vargas Maternity and Children’s Hospital, Porto Alegre, Brazil https://orcid.org/0000-0002-8108-9638
Vinicius Toniolli Ufpel Teaching Hospital, Pelotas, Brazil https://orcid.org/0000-0002-0585-9872

DOI:

https://doi.org/10.28982/josam.8470

Keywords:

Congenital pulmonary airway malformation, fetal sclerotherapy, ethanolamine, pulmonary sequestration, minimally invasive surgery, hydrops fetalis

Abstract

Congenital pulmonary airway malformation (CPAM) is a rare fetal condition associated with substantial morbidity and mortality, particularly when complicated by hydrops fetalis. Minimally invasive fetal therapies have emerged as alternatives to open fetal surgery, with the goals of reducing lesion volume, improving survival, and minimizing maternal morbidity. We report a hydropic fetus with a solid-appearing left thoracic lesion and a central feeding vessel, initially interpreted as a hybrid CPAM/pulmonary sequestration lesion. After maternal betamethasone failed to control disease progression, ultrasound-guided intralesional sclerotherapy with 5% monoethanolamine oleate (Ethamolin®; 50 mg/mL) was performed at 27 weeks’ gestation. Follow-up ultrasonography showed a reduction in lesion size and a decrease in the CPAM volume ratio (CVR) from 2.58 to 1.29, with complete resolution of hydrops. The pregnancy progressed to term, and postnatal surgical resection and histopathologic examination confirmed pulmonary sequestration. To contextualize this experience, we also reviewed the literature on minimally invasive prenatal treatment of solid-appearing CPAM and pulmonary sequestration. Across the available reports, laser ablation predominated in pulmonary sequestration, whereas sclerotherapy progressively replaced laser therapy in solid CPAM. Among the reported sclerosants, ethanolamine appears to offer the most consistent balance of feasibility, low cost, and favorable fetal response, although careful Doppler-guided injection is essential to avoid intravascular complications.

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