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

  • Jianxing Niu
  • Jianzhen Wang
  • Daizhong Wang
  • Xin He
  • Zhongming Li
  • Xin Li
  • Fan Su
  • Wang JiaEmail author
Original Article


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.


Neurofibromatosis type 1 Intraorbital meningoencephalocele Sphenoid dysplasia Pulsatile exophthalmos Plexiform neurofibroma 


Compliance with ethical standards

This study was approved by the Ethics Committee of The Third Medical Center of Chinese PLA General Hospital. Written informed consent was obtained from each patient or their legal relatives.

Conflict of interest

The authors declare that they have no conflict of interest.


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Copyright information

© Fondazione Società Italiana di Neurologia 2019

Authors and Affiliations

  1. 1.Department of Neurosurgery, Beijing Tiantan HospitalCapital Medical UniversityBeijingChina
  2. 2.Department of NeurosurgeryThe Third Medical Center of Chinese PLA General HospitalBeijingChina
  3. 3.Department of Pathology, Taihe HospitalHubei University of MedicineShiyanChina
  4. 4.Institute of Orbital Diseases of The Third Medical Center of Chinese PLA General HospitalBeijingChina

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