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All issues > Volume 40(6); 1997

Case Report
J Korean Pediatr Soc. 1997;40(6):877-882. Published online June 15, 1997.
A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome
Dong Hee DH Lee1, Won Bae WB Kim1, Jung Hye JH Choi1, Su Nam SN Lee2
1Department of Pediatrics, Inchon Christian Hospital, Inchon, Korea
2Department of Pathology, Inchon Christian Hospital, Inchon, Korea
Abstract
Congenital pulmonary lymphangiectasia is a rare and perphaps underestimated cause of fatal respiratory distress in the neonate or infant. Pathologically, this condition is characterized by wide lymphatic channels in the subpleural and peribronchovascular spaces and the interlobular septa. Noonan syndrome is characterized by a phenotype similar to Turner syndrome but with a normal karyotype. Both pulmonary and intestinal lymphangiectasia have been reported in patients with Noonan syndrome. We have experienced a case of congenital pulmonary lymphangiectasia in Noonan syndrome, who presented at birth with cyanosis and persistent respiratory distress, and died on 93 postnatal days. We report this case, which was diagnosed by open lung biopsy and autopsy, with brief review of the related literature.

Keywords :Congenital pulmonary lymphangiectasia, Noonan syndrome

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