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Case Report

Posterior approach for giant S1 neurofibroma in Von Recklinghausen's disease: Is total resection realistic?



Abstract

Bilateral sacral neurofibromas are uncommonly seen in neurofibromatosis type 1 (NF1) also known as Von Recklinghausen's disease. They often grow to enormous dimensions before detection owing to bone scalloping. Resections of “giant” S1 neurofibromas are difficult due to the limitations of operative window in sacrum and critical functions associated with S1 nerve root. We report a case of bilateral S1 neurofibromas in a patient of NF1 where she had a giant left-sided neurofibroma with extensive bone erosion and a small fusiform neurofibroma on the right side. The tumor was excised completely on the left and near totally on the right side via posterior approach. There were no postoperative neurological deficits and the patient recovered well. Usually, complete excision harbors the chances of postoperative neurological deficits due to the eloquence of the nerve root involved and complete resection without significant morbidity seems unrealistic. However, excision of giant ones may not result in grave deficits always if the patient is neurologically intact before surgery. Also, in selected patients, only posterior approach may suffice for giant neurofibromas with extensive bone scalloping and complete removal can be attempted successfully despite narrow corridors.


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