Clinical Course and Prognosis of Trochlear Nerve Schwannomas

Valerie I. Elmalem, Brian R. Younge, Valérie Biousse, Jacqueline A. Leavitt, Mark L. Moster, Judith Warner, Mark J. Kupersmith, Klara Landau, Michael C Brodsky, Larry P. Frohman, Eugene F. May, Robert L. Tomsak, Nancy J. Newman

Research output: Contribution to journalArticle

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Abstract

Purpose: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas. Design: Nonrandomized retrospective case series. Participants: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers. Methods: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI. Main Outcome Measures: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia. Results: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia. Conclusions: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Original languageEnglish (US)
Pages (from-to)2011-2016
Number of pages6
JournalOphthalmology
Volume116
Issue number10
DOIs
StatePublished - Oct 2009

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Trochlear Nerve
Neurilemmoma
Trochlear Nerve Diseases
Diplopia
Magnetic Resonance Imaging
Strabismus
Brain
Neurofibromatoses
Disclosure
Ophthalmology
Brain Stem

ASJC Scopus subject areas

  • Ophthalmology

Cite this

Elmalem, V. I., Younge, B. R., Biousse, V., Leavitt, J. A., Moster, M. L., Warner, J., ... Newman, N. J. (2009). Clinical Course and Prognosis of Trochlear Nerve Schwannomas. Ophthalmology, 116(10), 2011-2016. https://doi.org/10.1016/j.ophtha.2009.03.054

Clinical Course and Prognosis of Trochlear Nerve Schwannomas. / Elmalem, Valerie I.; Younge, Brian R.; Biousse, Valérie; Leavitt, Jacqueline A.; Moster, Mark L.; Warner, Judith; Kupersmith, Mark J.; Landau, Klara; Brodsky, Michael C; Frohman, Larry P.; May, Eugene F.; Tomsak, Robert L.; Newman, Nancy J.

In: Ophthalmology, Vol. 116, No. 10, 10.2009, p. 2011-2016.

Research output: Contribution to journalArticle

Elmalem, VI, Younge, BR, Biousse, V, Leavitt, JA, Moster, ML, Warner, J, Kupersmith, MJ, Landau, K, Brodsky, MC, Frohman, LP, May, EF, Tomsak, RL & Newman, NJ 2009, 'Clinical Course and Prognosis of Trochlear Nerve Schwannomas', Ophthalmology, vol. 116, no. 10, pp. 2011-2016. https://doi.org/10.1016/j.ophtha.2009.03.054
Elmalem VI, Younge BR, Biousse V, Leavitt JA, Moster ML, Warner J et al. Clinical Course and Prognosis of Trochlear Nerve Schwannomas. Ophthalmology. 2009 Oct;116(10):2011-2016. https://doi.org/10.1016/j.ophtha.2009.03.054
Elmalem, Valerie I. ; Younge, Brian R. ; Biousse, Valérie ; Leavitt, Jacqueline A. ; Moster, Mark L. ; Warner, Judith ; Kupersmith, Mark J. ; Landau, Klara ; Brodsky, Michael C ; Frohman, Larry P. ; May, Eugene F. ; Tomsak, Robert L. ; Newman, Nancy J. / Clinical Course and Prognosis of Trochlear Nerve Schwannomas. In: Ophthalmology. 2009 ; Vol. 116, No. 10. pp. 2011-2016.
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AU - Younge, Brian R.

AU - Biousse, Valérie

AU - Leavitt, Jacqueline A.

AU - Moster, Mark L.

AU - Warner, Judith

AU - Kupersmith, Mark J.

AU - Landau, Klara

AU - Brodsky, Michael C

AU - Frohman, Larry P.

AU - May, Eugene F.

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AU - Newman, Nancy J.

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N2 - Purpose: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas. Design: Nonrandomized retrospective case series. Participants: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers. Methods: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI. Main Outcome Measures: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia. Results: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia. Conclusions: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

AB - Purpose: To delineate the disease course and prognosis of patients with mass lesions of the fourth nerve presumed to be schwannomas. Design: Nonrandomized retrospective case series. Participants: Thirty-seven consecutive cases of presumed trochlear nerve schwannoma from 9 tertiary university neuro-ophthalmology centers. Methods: Cases were collected, and their clinical characteristics on presentation and follow-up are described. Inclusion criteria were brain magnetic resonance imaging (MRI) with a lesion suggestive of a schwannoma along the course of the fourth nerve. Exclusion criteria were other causes of fourth nerve palsy, such as congenital, traumatic or microvascular; normal (or lack of) initial brain MRI; lack of adequate clinical information; and disappearance of the lesion on subsequent follow-up brain MRI. Main Outcome Measures: Demographics of patients, presence of neurofibromatosis, symptoms on presentation, vertical deviation, lesion size (on presentation and follow-up), length of follow-up, and outcomes of treatment for lesions or diplopia. Results: Seven patients were excluded and of the 30 patients included in our series, patients were predominantly male (77%) with a mean age of 51 years (range 9-102 years). In contrast with prior case reports, almost all of our cases had a fourth nerve palsy on presentation (29/30), often isolated. Mean follow-up was 3.1 years (range 0.2 months to 11.1 years). There was no significant difference between initial and follow-up lesion size (4.4 vs. 5 mm) for patients who did not receive treatment of lesions (P = 0.36). Only 3 patients underwent neurosurgical resection and an additional patient received gamma-knife radiotherapy. The majority of patients (24/30) did not pursue strabismus surgery for vertical diplopia. Conclusions: Patients with isolated fourth nerve palsy and small lesions of the fourth nerve have a good prognosis and should be followed with serial MRI scans without neurosurgical intervention unless they develop signs of brain stem compression. Most patients with diplopia and benign fourth nerve lesions typical of trochlear nerve schwannoma can adapt with either prism spectacles or no treatment at all, although strabismus surgery can be successful. Financial Disclosure(s): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

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