Surgical care burden in orbito-temporal neurofibromatosis: Multiple procedures and surgical care duration analysis in 47 consecutive adult patients

Pessis, R.; Lantieri, Laurent; Britto, Jonathan A.; Leguerinel, Caroline; Wolkenstein, Pierre; Hivelin, Mikaël

doi:10.1016/j.jcms.2015.06.023

Cited by 7 publications

(5 citation statements)

References 50 publications

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“…Surgical case series, primarily in adults, also have not focused on ophthalmic characteristics or outcomes. 12, 14, 17-20 While the surgical techniques described in these case series are important, without a well-defined indication for treatment or a formal definition of “therapeutic success,” it is difficult to determine if and when intervention is indicated, and whether an intervention is actually beneficial. Improvement in physical appearance and visual outcomes (i.e., avoiding or decreasing amblyopia) are the most common indications for medical and or surgical treatment, yet neither has been well studied nor have they been included in clinical trials.…”

Section: Introductionmentioning

confidence: 99%

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%

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

et al. 2017

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“…However, the focus of studies on skull base dysplasia in NF1 is on the pathognomonic malformation of the sphenoid bone, especially the wings and the sella turcica (18). Individual studies also focus on the deformation of adjacent bones (11,13,54,56), shortening of the anterior skull base (57), and they discuss surgical procedures to correct orbital dysplasia (58)(59)(60)(61)(62). Some authors are concerned with whether the sphenoid bone changes are genuine skeletal malformations or the consequence of a tumour manifestation, but they do not address the pneumatisation of the bone (13,(63)(64)(65).…”

Section: Discussionmentioning

confidence: 99%

Sphenoid Bone Pneumatisation on Lateral Cephalograms of Patients With Neurofibromatosis Type 1

Friedrich

Kersten

Scheuer

2021

In Vivo

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“…Autologous bone grafts from the iliac or calvarial bones were originally adopted as replacement materials for skull base defects, 6,7 but recurrence has been reported in NF1 patients. The absence of neurofibromin in NF1 patients results in the upregulation of osteoclasts and downregulation of osteoblasts, which makes reconstructed autogenous bone grafts susceptible to resorption 3,4 . Conversely, reconstructions with titanium meshes have shown reliable results 7–9 as they can be easily shaped to fit different bony contours and have shown good durability and biocompatibility in craniofacial reconstruction.…”

Section: Discussionmentioning

confidence: 99%

“…2 Rarely, sufficient bony defects may ultimately result in herniation of the temporal lobe into the orbital fossa, causing proptosis and pulsatile exophthalmos with subsequent incomplete eyelid closure, diplopia, vision loss, or even severe facial deformity. 3 In such circumstances, surgical intervention is required to prevent visual complications due to hemimegalencephaly and restore symmetric facial appearance. However, the reconstruction of a complex skull base defect while fulfilling cosmetic needs is challenging.…”

mentioning

confidence: 99%

“…A retrospective study of 52 NF1 images showed that 30 had sphenoid abnormalities of varying degrees 2 . Rarely, sufficient bony defects may ultimately result in herniation of the temporal lobe into the orbital fossa, causing proptosis and pulsatile exophthalmos with subsequent incomplete eyelid closure, diplopia, vision loss, or even severe facial deformity 3 . In such circumstances, surgical intervention is required to prevent visual complications due to hemimegalencephaly and restore symmetric facial appearance.…”

mentioning

confidence: 99%

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Computer-Aided Three-Dimensional Virtual Surgical Planning in Complex Skull Base Reconstruction for Sphenoid Wing Dysplasia in Neurofibromatosis Type 1

Hsu¹,

Wong²,

Lee

et al. 2021

Journal of Craniofacial Surgery

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Unilateral sphenoid dysplasia is a rare but distinctive manifestation of neurofibromatosis type 1, causing pulsatile exophthalmos, decreased vision, and facial deformity. Surgical intervention is required to prevent visual deterioration. However, the reconstruction of a complex cranial base defect while fulfilling cosmetic needs is challenging. The asymmetric anatomy impedes identification and preservation of vital structures, and the use of bone grafts is often unsustainable due to resorption. Here we demonstrate a multimodal technique combining mirror-imagebased virtual surgical planning, stereolithography, and neuronavigation to achieve skull base reconstruction and restore facial symmetry in an neurofibromatosis type 1 patient with sphenoid dysplasia. Preoperative surgical planning involved mirror-image simulation based on the unaffected contralateral counterpart and a stereolithographic skull-base model fabricated to design a patientspecific titanium mesh. Surgical reconstruction via the transcranial approach under intraoperative neuronavigation was performed. Immediate resolution of pulsatile proptosis was observed postoperatively. With the help of virtual surgical planning, stereolithography, and neuronavigation, precise and sustainable reconstruction with patient-specific implants can be tailored for a complex skull base defect.

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Surgical care burden in orbito-temporal neurofibromatosis: Multiple procedures and surgical care duration analysis in 47 consecutive adult patients

Cited by 7 publications

References 50 publications

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

Orbital/Periorbital Plexiform Neurofibromas in Children with Neurofibromatosis Type 1

Sphenoid Bone Pneumatisation on Lateral Cephalograms of Patients With Neurofibromatosis Type 1

Computer-Aided Three-Dimensional Virtual Surgical Planning in Complex Skull Base Reconstruction for Sphenoid Wing Dysplasia in Neurofibromatosis Type 1

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