Michael Seear1, Jennifer Townsend2, Amy Hoepker2, Douglas Jamieson3, Deborah McFadden4, Patrick Daigneault5, William Glomb6. 1. Divisions of Respiratory Medicine, British Columbia's Children's Hospital, Room 1C 31, 4480 Oak Street, Vancouver, BC, V6H 3V4, Canada. mseear@cw.bc.ca. 2. Divisions of Respiratory Medicine, British Columbia's Children's Hospital, Room 1C 31, 4480 Oak Street, Vancouver, BC, V6H 3V4, Canada. 3. Divisions of Radiology, British Columbia's Children's Hospital, Vancouver, Canada. 4. Divisions of Pathology, British Columbia's Children's Hospital, Vancouver, Canada. 5. Division of Pediatric Respirology, Centre Mere-Enfant du CHUQ, Universite Laval, Quebec, Canada. 6. Division of Pediatric Pulmonology, University of Texas Medical Branch, Austin, USA.
Abstract
PURPOSE: Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. RESULTS: The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. CONCLUSIONS: Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.
PURPOSE:Congenital lung abnormalities are rare malformations increasingly detected early by prenatal ultrasound. Whether management of these frequently asymptomatic lesions should be surgical or conservative is an unresolved issue. The necessary prospective studies are limited by the absence of a widely accepted practical classification system. Our aim was to develop a simple, clinically relevant system for classifying and studying congenital lung abnormalities. MATERIALS AND METHODS: We based our proposed grouping on a detailed analysis of clinical, radiological, and histological data from well-documented cases, plus an extensive review of the literature. RESULTS: The existence of hybrid lesions and common histological findings suggested a unified embryological mechanism-possibly obstruction of developing airways with distal dysplasia. Malformations could be classified by their anatomical and pathological findings; however, a system based on the prenatal ultrasound plus initial chest X-ray findings had greater clinical relevance: Group 1-Congenital solid/cystic lung malformation, Group 2-Congenital hyperlucent lobe, Group 3-Congenital small lung. CONCLUSIONS: Pathological classification is academically important but is unnecessarily complex for clinical and research use. Our simple radiological-based system allows unambiguous comparison between the results of different studies and also guides the choice of necessary investigations specific to each group.
Authors: J M Laberge; H Flageole; D Pugash; S Khalife; G Blair; D Filiatrault; P Russo; G Lees; R D Wilson Journal: Fetal Diagn Ther Date: 2001 May-Jun Impact factor: 2.587