Robert A Avery1, Awais Mansoor2, Rabia Idrees2, Carmelina Trimboli-Heidler2, Hiroshi Ishikawa2, Roger J Packer2, Marius George Linguraru2. 1. From the Center for Neuroscience and Behavior (R.A.A., R.J.P.), The Gilbert Family Neurofibromatosis Institute (R.A.A., C.T.-H., R.J.P.), Sheikh Zayed Institute for Pediatric Surgical Innovation (A.M., M.G.L.), and The Brain Tumor Institute (R.J.P.), Children's National Health System; The George Washington University School of Medicine and Health Sciences (R.I., M.G.L.), Washington, DC; UPMC Eye Center, Eye and Ear Institute (H.I.), Ophthalmology and Visual Science Research Center, Department of Ophthalmology, University of Pittsburgh School of Medicine; and Department of Bioengineering (H.I.), Swanson School of Engineering, University of Pittsburgh, PA. averyr@email.chop.edu. 2. From the Center for Neuroscience and Behavior (R.A.A., R.J.P.), The Gilbert Family Neurofibromatosis Institute (R.A.A., C.T.-H., R.J.P.), Sheikh Zayed Institute for Pediatric Surgical Innovation (A.M., M.G.L.), and The Brain Tumor Institute (R.J.P.), Children's National Health System; The George Washington University School of Medicine and Health Sciences (R.I., M.G.L.), Washington, DC; UPMC Eye Center, Eye and Ear Institute (H.I.), Ophthalmology and Visual Science Research Center, Department of Ophthalmology, University of Pittsburgh School of Medicine; and Department of Bioengineering (H.I.), Swanson School of Engineering, University of Pittsburgh, PA.
Abstract
OBJECTIVE: To determine whether tumor size is associated with retinal nerve fiber layer (RNFL) thickness, a measure of axonal degeneration and an established biomarker of visual impairment in children with optic pathway gliomas (OPGs) secondary to neurofibromatosis type 1 (NF1). METHODS: Children with NF1-OPGs involving the optic nerve (extension into the chiasm and tracts permitted) who underwent both volumetric MRI analysis and optical coherence tomography (OCT) within 2 weeks of each other were included. Volumetric measurement of the entire anterior visual pathway (AVP; optic nerve, chiasm, and tract) was performed using high-resolution T1-weighted MRI. OCT measured the average RNFL thickness around the optic nerve. Linear regression models evaluated the relationship between RNFL thickness and AVP dimensions and volume. RESULTS: Thirty-eight participants contributed 55 study eyes. The mean age was 5.78 years. Twenty-two participants (58%) were female. RNFL thickness had a significant negative relationship to total AVP volume and total brain volume (p < 0.05, all comparisons). For every 1 mL increase in AVP volume, RNFL thickness declined by approximately 5 microns. A greater AVP volume of OPGs involving the optic nerve and chiasm, but not the tracts, was independently associated with a lower RNFL thickness (p < 0.05). All participants with an optic chiasm volume >1.3 mL demonstrated axonal damage (i.e., RNFL thickness <80 microns). CONCLUSIONS: Greater OPG and AVP volume predicts axonal degeneration, a biomarker of vision loss, in children with NF1-OPGs. MRI volumetric measures may help stratify the risk of visual loss from NF1-OPGs.
OBJECTIVE: To determine whether tumor size is associated with retinal nerve fiber layer (RNFL) thickness, a measure of axonal degeneration and an established biomarker of visual impairment in children with optic pathway gliomas (OPGs) secondary to neurofibromatosis type 1 (NF1). METHODS:Children with NF1-OPGs involving the optic nerve (extension into the chiasm and tracts permitted) who underwent both volumetric MRI analysis and optical coherence tomography (OCT) within 2 weeks of each other were included. Volumetric measurement of the entire anterior visual pathway (AVP; optic nerve, chiasm, and tract) was performed using high-resolution T1-weighted MRI. OCT measured the average RNFL thickness around the optic nerve. Linear regression models evaluated the relationship between RNFL thickness and AVP dimensions and volume. RESULTS: Thirty-eight participants contributed 55 study eyes. The mean age was 5.78 years. Twenty-two participants (58%) were female. RNFL thickness had a significant negative relationship to total AVP volume and total brain volume (p < 0.05, all comparisons). For every 1 mL increase in AVP volume, RNFL thickness declined by approximately 5 microns. A greater AVP volume of OPGs involving the optic nerve and chiasm, but not the tracts, was independently associated with a lower RNFL thickness (p < 0.05). All participants with an optic chiasm volume >1.3 mL demonstrated axonal damage (i.e., RNFL thickness <80 microns). CONCLUSIONS: Greater OPG and AVP volume predicts axonal degeneration, a biomarker of vision loss, in children with NF1-OPGs. MRI volumetric measures may help stratify the risk of visual loss from NF1-OPGs.
Authors: L Weizman; L Ben Sira; L Joskowicz; S Constantini; R Precel; B Shofty; D Ben Bashat Journal: Med Image Anal Date: 2011-07-21 Impact factor: 8.545
Authors: Robert A Avery; Avital Cnaan; Joel S Schuman; Chieh-Li Chen; Natalie C Glaug; Roger J Packer; Graham E Quinn; Hiroshi Ishikawa Journal: Am J Ophthalmol Date: 2014-06-28 Impact factor: 5.258
Authors: L J Balcer; G T Liu; G Heller; L Bilaniuk; N J Volpe; S L Galetta; P T Molloy; P C Phillips; A J Janss; S Vaughn; M G Maguire Journal: Am J Ophthalmol Date: 2001-04 Impact factor: 5.258
Authors: Katherine E Warren; Tina Y Poussaint; Gilbert Vezina; Darren Hargrave; Roger J Packer; Stewart Goldman; Patrick Y Wen; Ian F Pollack; David Zurakowski; Larry E Kun; Michael D Prados; Stefan Rutkowski; Mark W Kieran Journal: Pediatr Blood Cancer Date: 2013-04-26 Impact factor: 3.167
Authors: Robert A Avery; Awais Mansoor; Rabia Idrees; Elijah Biggs; Mohammad Ali Alsharid; Roger J Packer; Marius George Linguraru Journal: Neurology Date: 2016-05-11 Impact factor: 9.910
Authors: Robert A Avery; Rosalie E Ferner; Robert Listernick; Michael J Fisher; David H Gutmann; Grant T Liu Journal: J Neurooncol Date: 2012-07-28 Impact factor: 4.130
Authors: Carlos E Prada; Robert B Hufnagel; Trent R Hummel; Anne M Lovell; Robert J Hopkin; Howard M Saal; Elizabeth K Schorry Journal: J Pediatr Date: 2015-07-29 Impact factor: 6.314
Authors: Peter M K de Blank; Michael J Fisher; Grant T Liu; David H Gutmann; Robert Listernick; Rosalie E Ferner; Robert A Avery Journal: J Neuroophthalmol Date: 2017-09 Impact factor: 3.042
Authors: Margot A Lazow; Martijn T Nievelstein; Adam Lane; Pratiti Bandopadhayhay; Mariko DeWire-Schottmiller; Maryam Fouladi; John W Glod; Robert J Greiner; Lindsey M Hoffman; Trent R Hummel; Lindsay Kilburn; Sarah Leary; Jane E Minturn; Roger Packer; David S Ziegler; Brooklyn Chaney; Katie Black; Peter de Blank; James L Leach Journal: Neuro Oncol Date: 2022-09-01 Impact factor: 13.029
Authors: C E Al-Haddad; M G Sebaaly; R N Tutunji; C J Mehanna; S R Saaybi; A M Khamis; R G Hourani Journal: AJNR Am J Neuroradiol Date: 2017-12-07 Impact factor: 3.825
Authors: Reinhard E Friedrich; Johanna Baumann; Anna Suling; Hannah T Scheuer; Hanna A Scheuer Journal: GMS Interdiscip Plast Reconstr Surg DGPW Date: 2017-03-23