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
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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