d'Orey M C, Mateus M, Guimarães H, Miguel C, Costeira M J, Nogueira R, Montenegro N, Santos N T, Maroteaux P
Neonatal Intensive Care Unit, Department of Paediatrics, Hospital of S. Jõao, Oporto Medical School, Porto, Portugal.
Pediatr Radiol. 1997 Dec;27(12):948-50. doi: 10.1007/s002470050279.
Two different forms of dyssegmental dysplasia, mild and severe, can be distinguished on clinical, radiographic and chondro-osseous morphologic grounds. The milder Rolland-Desbuquois type is frequently characterised by survival beyond the newborn period. The lethal Silverman-Handmaker type has distinctive and more severe findings. In this paper, a rare case of the Rolland-Desbuquois type is presented. The case is unusual because although it was the milder form, the child died on the 10th day of life.