| A Case of Congenital Pulmonary Lymphangiectasia in Noonan Syndrome |
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Dong Hee Lee1, Won Bae Kim1, Jung Hye Choi1, Su Nam Lee2 |
1Department of Pediatrics, Inchon Christian Hospital, Inchon, Korea
2Department of Pathology, Inchon Christian Hospital, Inchon, Korea |
| Noonan 증후군 환자에서 발생한 선천성 폐림프관 확장증 1례 |
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이동희1, 김원배1, 최정혜1, 이수남2 |
1인천기독병원 해부병리과
2인천기독병원 해부병리과 |
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| Abstract |
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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. |
| Key Words:
Congenital pulmonary lymphangiectasia, Noonan syndrome |
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