The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations | 66 patients with prenatal CPAM diagnosis were included | Retrospective review 2002 - 2020 | 94% survived | RESULTS | 31% Required Surgery | 69% Conservative management | Risk factors for requiring surgery: | Presence of mediastinal shift on postnatal imaging | High CVR | Large lesion size | Conclusion: Prenatal regression is common among prenatally diagnosed CPAM/BPS. Most of the children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. | https://www.jpedsurg.org/article/S0022-3468(22)00253-6/fulltext | Source: Matilda Karlsson et al. | Department of Pediatric Surgery, Karolinska University Hospital, Department of Women's and Children's Health, Karolinska Institutet, Stockholm, Sweden | @StayCurrentMD | @cecilipstick | Cincinnati Children's | Journal of Pediatric Surgery
The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations
Infographic · Jan 2023 · 1 min read
In brief
In brief
This 18-year retrospective study of 66 prenatally diagnosed CPAM/BPS cases found that 56% regressed during gestation and 69% were managed conservatively with median 4-year follow-up. High CVR, mediastinal shift on postnatal imaging, and large gestational lesion size predicted need for surgery in the 31% who required intervention.
- 56% of prenatally diagnosed CPAM/BPS lesions decreased in size during gestation, with 94% overall survival rate.
- 69% of cases managed conservatively with median 4-year follow-up; only 19% developed symptoms after neonatal period.
- Mediastinal shift on postnatal imaging, high CVR, and large gestational lesion size predict need for surgery.
- Asymptomatic children beyond neonatal period typically remain symptom-free throughout childhood under watchful waiting.
- Prenatal regression is common; conservative management is viable for most prenatally diagnosed CPAM/BPS cases.
Written by the GCMD Library team from the infographic.
A teal and yellow infographic divided into sections with icons including a calendar with checkmark and warning triangle. The layout flows from study design at top left, through patient cohort and survival data in the center, to results displayed as large percentages, risk factors in a bulleted list, and conclusion in a yellow box on the right. Footer contains source citation and institutional logos.
Infographic by Cecilia Gigena
"The natural history of prenatally diagnosed congenital pulmonary airway malformations and bronchopulmonary sequestrations" Matilda Karlson et.al.
https://www.jpedsurg.org/article/S0022-3468(22)00253-6/fulltext
Authors: Matilda Karlsson, Peter Conner, Henrik Ehren, Catarina Bitkover, Carmen Mesas Burgos
Abstract
Background
The natural history of congenital pulmonary airway malformations (CPAM) and bronchopulmonary sequestrations (BPS) is not fully understood, and the management of the newborn with an asymptomatic lesion is a controversial issue. We aimed to study the natural history and outcome of CPAM/BPS at our institution with a policy of watchful waiting, and to investigate if any prognostic factors in the pre- and/or postnatal- period may predict the need for surgery.
Material and methods
A retrospective review study was conducted of children prenatally diagnosed with CPAM and/or BPS during the 18-year period, from 2002 to 2020. Data from the pre and postnatal period was collected and analysed.
Results
Sixty- six patients with prenatally observed lung lesions were entered in the study, with an overall survival rate of 94%. Fifty-six percent of the lesions decreased in size during gestation. Thirty-one percent had surgery and 69% could be managed conservatively with a median follow-up of 4 years. Nineteen percent developed symptoms after the neonatal period. Children with a presence of mediastinal shift on postnatal imaging (p = 0.003), with a high CVR (p = 0.005) and a large lesion size during gestation (p = 0.014) were significantly more likely to require surgery.
Conclusion
Prenatal regression is common among prenatally diagnosed CPAM/BPS and the majority of children that are asymptomatic beyond the neonatal period will remain asymptomatic throughout their childhood. Future analysis with a longer follow-up might give new insights in order to identify children at risk of developing symptoms.