Congenital alveolar capillary dysplasia: Rare cause of persistent pulmonary hypertension

被引:20
作者
Haraida, S [1 ]
Lochbuhler, H [1 ]
Heger, A [1 ]
Nerlich, A [1 ]
Diebold, J [1 ]
Wiest, I [1 ]
MullerHocker, J [1 ]
Lohrs, U [1 ]
机构
[1] UNIV MUNICH,CLIN PEDIAT SURG,MUNICH,GERMANY
来源
PEDIATRIC PATHOLOGY & LABORATORY MEDICINE | 1997年 / 17卷 / 06期
关键词
congenital alveolar capillary dysplasia; extracorporeal membrane oxygenation; misalignment of pulmonary vessels; persistent fetal circulation; persistent pulmonary hypertension;
D O I
10.1080/107710497174417
中图分类号
R36 [病理学];
学科分类号
100104 ;
摘要
We report on a rare case of fatal congenital alveolar capillary dysplasia. The newborn boy of a 37 weeks' normal gestation suffered from persistent pulmonary hypertension without any cardiovascular malformation and died at the age of 4 weeks despite intensive treatment. The autopsy tissue was examined histologically, immunohistochemically, and ultrastructurally. Moreover, a three-dimensional tissue reconstruction based on serial sections was performed comparing the affected lung with normal lung tissue. We observed a unique pattern of pulmonary dysplasia: An extreme decrease of capillaries was localized centrally within thickened intra-acinar septa instead of capillaries intensely neighboring pneumocytes; ectatic veins normally running in the interlobular septa were found to accompany intralobular bronchovascular bundles, denying a clear distinction between pulmonary and bronchial veins; small muscular pulmonary arteries extended to the precapillary level and type 2 pneumocytes exceeded by far the type 1 pneumocytes, inverting the normal ratio. In summary, alveolar capillary dysplasia is assumed to be a primary capillary disorder of unknown origin, which possibly involves the regular differentiation of pneumocytes, according to the close alveolocapillary relationship during pulmonary ontogenesis. We consider the venous alterations as being part of the dysplasia, whereas the arterial phenomena might occur secondarily. Recent reports on affected siblings suggest a genetic component of pathogenesis.
引用
收藏
页码:959 / 975
页数:17
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