Asymptomatic Massive Dural Ectasia Associated with Neurofibromatosis Type 1 Threatening Spinal Column Support

Abstract

We report the case of a girl with a dystrophic neurofibromatosis lumbar scoliosis with asymptomatic progression and spread of dystrophic characteristics over 18 years. Resorption of almost the entire anterior column of L1-L4 had occurred despite a previous posterior instrumentation and fusion. A vascularized fibula bone graft wrapped in titanium mesh was used as an anterior structural graft. With close follow-up, the need for this type of salvage surgery might have been averted. Further follow-up showed no graft resorption and unchanged sagittal alignment after 3 years.

Eight-year follow-up findings of surgical treatment for severe dystrophic changes in the cervical spine associated with neurofibromatosis type I: a case report

“…Successful posterior fusion with little progression of dural ectasia and spinal deformities, as observed in our case, has been reported [9]. The progression of dural ectasia and bone erosion despite surgical treatment has also been documented [6]. The best treatment method for dural ectasia has not been established.…”

“…However, Kleuver et al [6] reported a case of dystrophic neurofibromatosis scoliosis with asymptomatic progression and spread of dystrophic changes in the lumbar spine that occurred over 18 years, despite previous posterior instrumentation and fusion. Therefore, we plan to continue further follow-up examinations of our patient to prevent a poor outcome in his adult life.…”

“…Earlier studies have reported various treatments for spinal deformities in the thoracic or lumbar spine [6][7][8][9][10][11][12][13], but reports pertaining to cervical spine deformities have been rare [14,15]. Furthermore, few studies have assessed the long-term follow-up findings of dural ectasia postoperatively.…”

Dural Ektazi ve Dev Lateral Meningoseller ile Prezente Olan Nörofibromatozis Tip 1 Olgusu

“…Ayrıca progresif vertebral erozyona neden olarak vertebralarda fraktür ve dislokasyonlara neden olabilirler. Ancak spinal kanal genişlediğinden nörolojik defisit gelişmesi nadirdir (16).…”

Dural ectasia: a manifestation of type 1 neurofibromatosis

“…Neurologic deficit rarely occurs because the spinal canal is widened. 4 These deformities have been known to progress and evolve during the course of disease, a phenomenon known as "modulation. "…”