Clinical, radiological features and surgical strategies for 23 NF1 patients with intraorbital meningoencephalocele

AbstractIntraorbital meningoencephalocele is a rare manifestation of neurofibromatosis type 1 (NF1) caused by secondary changes in sphenoid dysplasia, and it seriously affects patients ’ vision and facial appearance. We retrospectively analyzed the clinical data of 23 patients with NF1 and intraorbital meningoencephalocele, summarized the surgical strategies employed, and evaluated their clinical efficacy in order to better understand its management in clinical practice, establi sh a reasonable surgical strategy, and assess prognosis. Before surgery, 22 patients had unilateral pulsatile exophthalmos, 18 patients had significant visual impairment, and 13 patients had ptosis associated with an orbital plexiform neurofibroma (PNF). All 23 patients underwent microsurgical crani otomy with skull base reconstruction using a soft titanium mesh. One month after surgery, the degree of exophthalmos in the 22 (95.65%) patients was significantly reduced compared with before surgery (P <  0.001), and ocular pulsation had subsided. The visual acuity did not decrease significantly (P = 0.298) compared with before surgery. Eleven (47.83%) patients received phase-II eyelid PNF resection and/or oculoplastic surgery, and the degree of ptosis was significantly reduced (P <  0.001). There was no recurrence of pulsatile exophthalmos, displacement of titanium mesh, decreased visual acuity, or increased degree of ptosis noted during follow-up. The best strategy is to reconstruct ...
Source: Neurological Sciences - Category: Neurology Source Type: research