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Updated: Mar 30, 2026
04:14
Laparoscopic Choledochal Cyst Excision and Roux-en-Y Choledochojejunostomy in Adults
Published on: February 28, 2025
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Congenital cystic adenomatoid malformation type I
Alice Rebelo de Matos Borges Dos Reis1, Frederico Becker Ribeiro1, Regina Schultz1
1Department of Pathology - Faculty of Medicine - Universidade de São Paulo, São Paulo/SP - Brazil .
Autopsy & Case Reports
|November 12, 2015
Summary
Congenital cystic adenomatoid malformation (CCAM) is a rare lung malformation in newborns. This case highlights a fatal outcome of CCAM Type 1 in a premature infant, emphasizing the importance of understanding its classification and prognosis.
Area of Science:
- Pediatric Surgery
- Neonatology
- Pulmonary Medicine
Background:
- Congenital cystic adenomatoid malformation (CCAM) is a rare hamartomatous congenital pulmonary airway malformation.
- CCAM incidence is estimated between 1:10,000 and 1:35,000 newborns.
- Current classification divides CCAM into five distinct groups based on pathological and clinical features.
Observation:
- A premature male newborn was diagnosed prenatally with CCAM Type 1.
- The infant also presented with cardiac right axis deviation.
- The newborn experienced respiratory failure and died 67 hours after birth.
Findings:
- This report details the autopsy findings of a rare case of CCAM Type 1.
- The study reviews the existing classification system for CCAM.
- The prognosis for CCAM varies significantly based on subtype and extent of lung involvement.
Implications:
- Understanding CCAM subtypes and their associated prognoses is crucial for clinical management.
- This case underscores the potential for severe respiratory complications in CCAM Type 1.
- Further research into CCAM pathogenesis and treatment strategies may improve outcomes for affected newborns.
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