{"id":5256,"date":"2011-03-02T23:17:31","date_gmt":"2011-03-03T04:17:31","guid":{"rendered":"http:\/\/insidesurgery.com\/?p=5256"},"modified":"2013-12-24T19:54:15","modified_gmt":"2013-12-25T00:54:15","slug":"von-recklinghausen-disease","status":"publish","type":"post","link":"https:\/\/insidesurgery.com\/2011\/03\/von-recklinghausen-disease\/","title":{"rendered":"Von Recklinghausen Disease (neurofibromatosis type 1)"},"content":{"rendered":"
Von Recklinghausen disease is also called neurofibromatosis type 1 and is marked by:<\/p>\n
syndrome secondary to genetic defect marked by benign neural tumors and characteristic skin changes<\/p>\n
1)<\/strong> multiple neurofibromas 2)<\/strong> positive “button hole” sign (tumors invaginate into skin with pressure) 3)<\/strong> six or more cafe-au-lait spots greater than 1.5 cm 4)<\/strong> freckles in nonexposed skin areas, such as axillary and inguinal areas 5)<\/strong> scoliosis 6)<\/strong> pseudoarthrosis of tibia 7)<\/strong> peripheral nerve sheath tumors (Schwannomas) 8)<\/strong> CNS tumors (neurofibrosarcoma, glioma, malignant Schwannoma) 9)<\/strong> Lisch’s nodules (pigmented iris hamartomas) 10) infiltrative lung disease<\/p>\n 1)<\/strong> cafe-au-lait spots in Von Recklinghausen disease are flat and uniformly light brown in color 2)<\/strong> benign neurofibroma may convert to malignant peripheral nerve sheath tumors<\/p>\nHistology\/Gross Pathology<\/h4>\n
Associated Conditions<\/h4>\n