Grand Rounds Conference Tala Kassm DO August 21 st, 2015 University of Louisville Department of Ophthalmology and Visual Sciences

Subjective CC: “My side vision is worse.” CC: “My side vision is worse.” HPI: 67 year old white male complains of progressive decreased peripheral vision OU over 3-4 months. He denies flashes, floaters, scotoma or eye pain. HPI: 67 year old white male complains of progressive decreased peripheral vision OU over 3-4 months. He denies flashes, floaters, scotoma or eye pain. Review of Systems: positive for headaches, general fatigue, increased fluid intake, aches and pains. Negative for weight loss or sexual dysfunction. Review of Systems: positive for headaches, general fatigue, increased fluid intake, aches and pains. Negative for weight loss or sexual dysfunction.

History POH: none PMH: hypertension, COPD Family Hx: noncontributory Meds: Albuterol, amlodipine, metoprolol Allergies: azithromycin, niacin, clindamycin

Clinical Exam OD OS OD OS VA(cc,D): 20/2020/25 ( plano +3.25x178) ( x170) Pupils: 4->2 no rAPD 4->2 IOP:1317 EOM:FULL FULL CVF: bilateral temporal defect, worse OD than OS

Clinical Exam SLE: OD OS External/LidsWNL WNL Conj/Sclerawhite & quietwhite & quiet Corneaclear clear Ant Chamber deep & quietdeep & quiet IrisWNLWNL Lens1-2+ NS 1-2+ NS VitreousClearClear

Clinical Exam Dilated Fundus Exam: OD: ON c/d 0.4, pink and sharp M/V/P: WNL M/V/P: WNL OS: ON c/d 0.5, pink and sharp M/V/P: WNL M/V/P: WNL

HVF 24-2 OS

HVF 24-2 OD

HVF 24-2 OU

MRI MRI T1 Sagittal

MRI MRI T1 Transverse

MRI T2 Transverse

Lab results Prolactin Prolactin T3, T4, TSH T3, T4, TSH Cortisol, ACTH stimulation test Cortisol, ACTH stimulation test LH, FSH LH, FSH Serum osmolarity Serum osmolarity Testosterone and IGF-1 all within normal limits Testosterone and IGF-1 all within normal limits

Assessment 67 year old male with progressive temporal vision loss over 3-4 months. MRI revealed a pituitary mass. 67 year old male with progressive temporal vision loss over 3-4 months. MRI revealed a pituitary mass. Diagnosis: Nonsecreting Pituitary Macroadenoma Diagnosis: Nonsecreting Pituitary Macroadenoma Differential includes: meningioma, craniopharyngioma, internal carotid artery aneurysm Differential includes: meningioma, craniopharyngioma, internal carotid artery aneurysm

Plan Given that the tumor is non secreting, medical management with bromocriptine was thought to be ineffective. Given that the tumor is non secreting, medical management with bromocriptine was thought to be ineffective. Patient was referred to neurosurgery for endonasal endoscopic tumor resection. Patient was referred to neurosurgery for endonasal endoscopic tumor resection. Surgery date pending. Surgery date pending.

Pituitary Adenoma Benign epithelial tumor Benign epithelial tumor Most common sellar mass, composing 10-15% of all intracranial tumors Most common sellar mass, composing 10-15% of all intracranial tumors Incidence is 1-7 cases per 100,000 Incidence is 1-7 cases per 100,000 Rare in childhood Rare in childhood Age-linked – increase with each decade Age-linked – increase with each decade By 80 th decade, small adenomas found in more than 20% of pituitary glands By 80 th decade, small adenomas found in more than 20% of pituitary glands

Classification Size Size Microadenoma: <1cm Microadenoma: <1cm Macroadenoma: >1cm Macroadenoma: >1cm Activity Activity Functional (74%) Functional (74%) Non-functional (26%) Non-functional (26%) 95% present as macroadenomas 95% present as macroadenomas 2/3 of these patients have visual field defects 2/3 of these patients have visual field defects

Presentation Functioning Functioning Lactotroph (Prolactin): amenorrhea, galactorrhea Lactotroph (Prolactin): amenorrhea, galactorrhea Somatotroph (Growth hormone): gigantism and/or acromegaly Somatotroph (Growth hormone): gigantism and/or acromegaly Corticotroph (ACTH): Cushing’s syndrome Corticotroph (ACTH): Cushing’s syndrome Thyrotroph (TSH): weight loss, tachycardia, diarrhea Thyrotroph (TSH): weight loss, tachycardia, diarrhea Non-functioning Non-functioning Headache Headache VF defects VF defects

Presentation Impaired vision is the most common symptom that leads a patient with a nonfunctioning adenoma, to seek medical attention. Impaired vision is the most common symptom that leads a patient with a nonfunctioning adenoma, to seek medical attention. Due to suprasellar extension of the adenoma, leading to compression of optic chiasm. Due to suprasellar extension of the adenoma, leading to compression of optic chiasm. Leads to bitemporal hemianopsia Leads to bitemporal hemianopsia Diminished visual acuity with more severe optic chiasm compression Diminished visual acuity with more severe optic chiasm compression

Anatomy

Treatment Observation Observation Medical: Bromocriptine for prolactinomas Medical: Bromocriptine for prolactinomas Surgery: transfrontal or transsphenoidal approach Surgery: transfrontal or transsphenoidal approach Radiotherapy Radiotherapy

Post Treatment Vision recovery rapid after surgical resection of tumor, even with severe vision loss Vision recovery rapid after surgical resection of tumor, even with severe vision loss Onset of improvement within 24 hours Onset of improvement within 24 hours Slower improvement with medical management of tumor Slower improvement with medical management of tumor Prognosis is poor if retinal nerve fiber layer thickness is less than 75 microns, by OCT scan Prognosis is poor if retinal nerve fiber layer thickness is less than 75 microns, by OCT scan First sign of recurrence may be vision loss First sign of recurrence may be vision loss

Associated Syndromes Multiple Endocrine Neoplasia Type 1 Multiple Endocrine Neoplasia Type 1 Pituitary Adenomas (prolactinomas) Pituitary Adenomas (prolactinomas) Parathyroid Parathyroid Pancreatic Islet cell tumor Pancreatic Islet cell tumor Pituitary Apoplexy Pituitary Apoplexy Acute hemorrhage or infarction of a pituitary tumor Acute hemorrhage or infarction of a pituitary tumor Potentially life threatening Potentially life threatening Sudden onset of severe headache, nausea, altered consciousness, vision loss, diplopia, and/or ptosis Sudden onset of severe headache, nausea, altered consciousness, vision loss, diplopia, and/or ptosis

Other causes of parasellar tumor Meningiomas Meningiomas Middle-aged women Middle-aged women Often produce asymmetric bitemporal vision loss Often produce asymmetric bitemporal vision loss Craniopharyngiomas Craniopharyngiomas Common in children but may present at any age Common in children but may present at any age Second incidence peak in adulthood Second incidence peak in adulthood Arise superiorly, produce inferior bitemporal visual field loss Arise superiorly, produce inferior bitemporal visual field loss Internal carotid artery aneurysms (supraclinoid region ) Internal carotid artery aneurysms (supraclinoid region ) Markedly asymmetric chiasmal syndrome Markedly asymmetric chiasmal syndrome Optic nerve compression on side of aneurysm Optic nerve compression on side of aneurysm

The time course of visual field recovery and changes of retinal ganglion cells after optic chiasmal decompression Investigated the time course of visual field recovery and changes of retinal ganglion cells after chiasmal decompression. Investigated the time course of visual field recovery and changes of retinal ganglion cells after chiasmal decompression. Prospective analysis – 19 patients, pre op, then 1, 3 and 6 months after surgery Prospective analysis – 19 patients, pre op, then 1, 3 and 6 months after surgery Used standard automated perimetry, optical coherence tomography, photopic negative response (PhNR). Used standard automated perimetry, optical coherence tomography, photopic negative response (PhNR). Compared to 20 controls with normal eyes Compared to 20 controls with normal eyes Pre operatively, all parameters worse in affected eyes as compared to control Pre operatively, all parameters worse in affected eyes as compared to control

The time course of visual field recovery and changes of retinal ganglion cells after optic chiasmal decompression After surgery, visual field significantly improved by 3 month. After surgery, visual field significantly improved by 3 month. Retinal nerve fiber layer (RNFL) thickness and ganglion cell complex (GCC) area were significantly reduced at three months Retinal nerve fiber layer (RNFL) thickness and ganglion cell complex (GCC) area were significantly reduced at three months At six months post op: average RNFL thickness, GCC area and PhNR/b-wave ratio showed significant improvement At six months post op: average RNFL thickness, GCC area and PhNR/b-wave ratio showed significant improvement Visual fields were significantly correlated with RNFL thickness and GCC area. Visual fields were significantly correlated with RNFL thickness and GCC area. VF recovery preceded demonstrable retinal regeneration VF recovery preceded demonstrable retinal regeneration

The time course of visual field recovery and changes of retinal ganglion cells after optic chiasmal decompression

References BCSC: Neuro-Ophthlamology. Pgs BCSC: Neuro-Ophthlamology. Pgs Pituitary Tumors: adenoma, craniopharyngioma, cysts. Mayfield Clinic and Spine Institute. Feb pgs 1-6 Pituitary Tumors: adenoma, craniopharyngioma, cysts. Mayfield Clinic and Spine Institute. Feb pgs 1-6 Danesh-Meyer HV, Papchenko T. In vivo retinal nerve fibery layer thickness measured by optical coherence tomography predicts visual recovery after surgery for parachiasmal tumors. Danesh-Meyer HV, Papchenko T. In vivo retinal nerve fibery layer thickness measured by optical coherence tomography predicts visual recovery after surgery for parachiasmal tumors. Ferrante E, Ferraroni M, Castrignano T, Menicatti L, Anagni M, Reimondo G, et al. Non-functioning pituitary adenoma database: a useful resource to improve clinical management of pituitary adenomas. Eur J Endocrinol 155: , Ferrante E, Ferraroni M, Castrignano T, Menicatti L, Anagni M, Reimondo G, et al. Non-functioning pituitary adenoma database: a useful resource to improve clinical management of pituitary adenomas. Eur J Endocrinol 155: , Galal A, Faisal A. Determinants of postoperative visual recovery in suprasellar meningiomas. Galal A, Faisal A. Determinants of postoperative visual recovery in suprasellar meningiomas. Loeffler JS, Shih HA. Radiation therapy in the management of pituitary adenomas. Loeffler JS, Shih HA. Radiation therapy in the management of pituitary adenomas. Moon CH, Hwang SC, Ohn YH, Park TK. The Time course of visual field recovery and changes of retinal ganglion cells after optic chiasmal decompression. Invest Ophthalmol Vis Sci Oct 10;52(11): Moon CH, Hwang SC, Ohn YH, Park TK. The Time course of visual field recovery and changes of retinal ganglion cells after optic chiasmal decompression. Invest Ophthalmol Vis Sci Oct 10;52(11):

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