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Two-Year Outcomes in Children Born With a Congenital Pulmonary Malformation

Marie Blanquer1, Isabelle Monier2, Naziha Khen-Dunlop3

  • 1AP-HP, Hôpital Necker-Enfants Malades, Service de Pneumologie et Allergologie Pédiatriques, Paris, France.

Pediatrics
|May 5, 2026
PubMed

Insights

Only 15% of children with congenital pulmonary malformations (CPMs) developed symptoms requiring surgery. A larger CPM Volume Ratio (CVRmax) over 1 cm2 was a key risk factor for symptomatic progression in these pediatric cases.

Area of Science:

  • Pediatric Surgery
  • Pulmonology
  • Medical Imaging

Background:

  • Congenital pulmonary malformations (CPMs) present a diagnostic and management challenge for pediatric patients.
  • Shared decision-making between families and clinicians requires comprehensive data on CPM complications, especially for asymptomatic children.
  • Understanding symptom development is crucial for guiding surgical interventions in pediatric CPM cases.

Purpose of the Study:

  • To prospectively document the occurrence of symptoms in children diagnosed with congenital pulmonary malformations (CPMs).
  • To identify specific markers or indicators associated with the need for surgical intervention due to symptomatic progression in pediatric CPM.
  • To inform clinical practice and family counseling regarding the management of asymptomatic congenital pulmonary malformations.

Main Methods:

  • A prospective, population-based, nationally representative cohort (MALFPULM) was established, including pregnant women with fetuses diagnosed with CPM.
  • Follow-up data were collected from birth to 2 years of age.
  • Outcomes were compared among children who did not undergo surgery, those who had elective surgery, and those operated on due to symptoms.

Main Results:

  • Of 322 children with follow-up, 15% (48) required surgery for symptoms.
  • Children operated on for symptoms exhibited higher prenatal maximal CPM Volume Ratio (CVRmax), neonatal respiratory distress, eating difficulties, pneumothorax, and respiratory hospitalizations compared to elective surgery cases.
  • A CVRmax greater than 1 cm2 was significantly associated with symptomatic surgical intervention (44% vs. 14%).
  • CPM infection occurred in only 3% of cases, with no significant link to the malformation's cystic nature.
  • Symptom frequency did not significantly differ between non-operated and elective surgery groups, with cough and wheezing being most common.

Conclusions:

  • Symptomatic progression necessitating surgery occurred in a minority (15%) of children with CPM.
  • Prenatal CVRmax exceeding 1 cm2 is a significant risk factor for developing symptoms requiring surgical intervention in pediatric CPM.
  • Elective surgery did not appear to alter the incidence of less severe symptoms in children with CPM.
Abstract

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