巨大な腰ヘルニアを合併したレツクリングハウゼン病の1例

摘要

Lumbar hernia is rare, and in the Japanese literature , only less than 100 cases have been reported. We report a case of congenital lumbar hernia associated with von Recklinghausen' s disease, who has presented with scoliosis, costal deformity, and many neurofibromas on the whole body. The lumbar hernia looked like a diffuse plexiform neurofibroma, however X-ray disclosed loops of bowel within the lumbar hernia sac, and CT scan detected spleen within the sac. X-ray and CT scan are useful for the definite diagnosis, and this is the first case report in which the spleen is included in the hernia sac.