The Surgical Management of Orbitopalpebral Neurofibromatosis

Morax, S; Ml, Herdan; Hurbli, T.

doi:10.1097/00002341-198804040-00002

Cited by 21 publications

(7 citation statements)

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“…PNs can result in substantial morbidity due to their appearance and proclivity to cause functional and neurologic deficits, and are at risk for malignant transformation. 2 PNs involving the eyelid, orbit, periorbital and facial structures have been described using a variety of names including orbitotemporal PNs, 7, 9 orbitopalpebral neurofibromatosis, 10, 11 orbitotemporal neurofibromatosis, 5, 12-14 orbital neurofibromas, 15 and orbitofacial neurofibromatosis. 8, 16 PNs in these areas are most appropriately labeled as orbital-periorbital PN (OPPN) to encompass all locations where they occur.…”

Section: Introductionmentioning

confidence: 99%

“…5, 8, 54-56 Strabismus may develop from mechanical restriction of the globe by tumor infiltration or compression of orbital tissues and extraocular muscles, by globe displacement from increased axial length, and globe displacement secondary to orbital and sphenoid wing dysplasia. 10, 55 Further, in the setting of severe vision loss, a sensory strabismus may also develop. In their detailed examination of ocular motility in 49 patients with OPPNs, Oystreck et al 55 found that the mechanisms for eye misalignment were multifold and that no specific factor was highly predictive.…”

Section: Introductionmentioning

confidence: 99%

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Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

et al. 2017

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Children and adults with Neurofibromatosis type 1 (NF1), a common autosomal dominant condition, manifest a variety of ophthalmologic conditions. Plexiform neurofibromas involving the eyelid, orbit, periorbital and facial structures (termed OPPN) can result in significant visual loss in children. Equally important, OPPNs can cause significant alteration in physical appearance secondary to proptosis, ptosis, and facial disfigurement, leading to social embarrassment and decreased self-esteem. Despite NF1 being a relatively common disease in which routine ophthalmologic examinations are required, no formal recommendations for clinical care of children with OPPNs exist. While medical and surgical interventions have been reported, there are no agreed upon criteria for when OPPN require therapy and which treatment produces the best outcome. Since a multi-disciplinary team of specialists (oculofacial plastics, pediatric ophthalmology, neuro-ophthalmology, medical genetics and neuro-oncology) direct management decisions, the absence of a uniform outcome measure that represents visual and or aesthetic sequelae complicates the design of evidence based studies and feasible clinical trials. In September 2013, a multi-disciplinary task force, composed of pediatric practitioners from tertiary care centers experienced in caring for children with OPPN, was convened to address the lack of clinical care guidelines for children with OPPN. This consensus statement provides recommendations for ophthalmologic monitoring and outlines treatment indications, forthcoming biologic therapy, while also discussing challenges to performing clinical trials in this complicated condition.

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Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

et al. 2017

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“…were able to successfully elevate the affected lid above the visual axis in all cases . Care must be taken to prevent overcorrection, particularly if ptosis surgery is undertaken at the time of other orbitofacial procedures . In our series, one patient presenting with moderate amblyopia from ptosis developed severe exposure keratitis, requiring conjunctival autograft and leaving her with ‘light perception’ vision.…”

Section: Discussionmentioning

confidence: 87%

“…As in ptosis, the outcomes of strabismus surgery performed in childhood are likely to be unstable because of increased disease activity in adolescence. Furthermore, conventional squint surgery may not be appropriate in OTNF where strabismus is secondary to a combination of factors including infiltration of extraocular muscles and orbital fat, and displacement of the globe owing a grossly abnormal bony orbit …”

Section: Discussionmentioning

confidence: 99%

Long‐term visual outcomes in patients with orbitotemporal neurofibromatosis

Greenwell

Anderson

Madge

et al. 2013

Clinical Exper Ophthalmology

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“…According to Morax et al 7 % of patients with neurofibromatosis type 1 show skeletal changes of partial or complete absence of the greater wing of sphenoid bone [8]. This may lead to an enlargement of the superior orbital fissure, which results in herniation of the temporal lobe into the orbit, causing exophthalmos and ocular dystopia [9–12].…”

Section: Discussionmentioning

confidence: 99%

Surgical correction in orbitotemporal neurofibromatosis with dystopia

Choi

Lee

2016

BMC Ophthalmol

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BackgroundOrbitotemporal neurofibromatosis is a challenging disease for orbital surgeons. Ptosis correction may be needed following correction of orbital dystopia.Case presentationA 34-year-old man, who underwent excision of a neurofibroma on the right eyelid in our clinic, returned to our clinic four years later complaining of dystopia and bulkiness of the protruding mass in the right eyelid and eyebrow. Computed tomographic imaging showed dysplasia and deformity in the sphenoid bone and orbit. A large mass was found in the superior portion of the orbit, protruding towards the temporal lobe, which in turn displaced the orbit downwards. A bicoronary incision and transcranial approach were performed, followed by the excision of the superior orbital space and temporal lobe mass by uncovering certain portions of the frontal, temporal, and zygomatic bones. After the excision of the mass, a calvarial bone graft was used to remodel the longitudinal widened orbit to correct the dystopia. While primary surgery was successful in the correction of dystopia, secondary surgery was performed to correct the exacerbated ptosis by levator muscle resection.ConclusionsCorrection of orbitotemporal neurofibromatosis with dystopia involves three steps: removal of the mass in the orbit to eliminate the effect of downward dislocation of the orbit, placement of a bone graft in the orbit floor after repositioning the orbit for suspension and remodeling of the orbit, and following the correction of dystopia, ptosis may be corrected if needed.

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The Surgical Management of Orbitopalpebral Neurofibromatosis

Cited by 21 publications

References 0 publications

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

Long‐term visual outcomes in patients with orbitotemporal neurofibromatosis

Surgical correction in orbitotemporal neurofibromatosis with dystopia

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