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01-06-2019 | Ptosis | Original Article

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

Authors: Jianxing Niu, Jianzhen Wang, Daizhong Wang, Xin He, Zhongming Li, Xin Li, Fan Su, Wang Jia

Published in: Neurological Sciences | Issue 6/2019

Abstract

Intraorbital 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, establish 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 craniotomy 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 the skull base under microscopy to relieve pulsating exophthalmos and preserve existing visual function. In cases of ptosis caused by an eyelid PNF, surgical resection should be performed as soon as possible to remove the tumor, and/or oculoplastic surgery should be performed to improve the cosmetic outcome.

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Title: Clinical, radiological features and surgical strategies for 23 NF1 patients with intraorbital meningoencephalocele
Authors: Jianxing Niu
Jianzhen Wang
Daizhong Wang
Xin He
Zhongming Li
Xin Li
Fan Su
Wang Jia
Publication date: 01-06-2019
Publisher: Springer International Publishing
Keywords: Ptosis
Neurofibromatosis Type 1
Published in: Neurological Sciences / Issue 6/2019
Print ISSN: 1590-1874
Electronic ISSN: 1590-3478
DOI: https://doi.org/10.1007/s10072-019-03826-2

At a glance: The STEP trials

Springer Medicine

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

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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