Indescribable Vision Loss Noushin S. Ahmed, O.D. Ocular Disease Resident Seidenberg Protzko Eye Associates

Chief Complaint � 56 yo Caucasian female presents as a

new patient complaining about decreased vision in the left eye

� She states it has been worsening gradually over the past few months.

� She claims reading has become more difficult and computer work has been tougher.

� LEE 2 months ago

Ocular History � Traumatic penetrating injury OD 1975

�  Cataract surgery OD with BCVA 20/40 2012 �  Iris reconstruction 2012

Medical History � Hypertension

�  Treated with Lisinopril 20mg qd po

� Hypercholesterolemia �  Treated with Lipitor 20mg qd po

� Hayfever

Family History � Unremarkable

� Former smoker x 10 years

�  denies alcohol & recreational drug use

Social History

External Examination BCVA OD 20/40

OS 20/50

Pupils OD Surgical OS PRRL(+)APD

CVF OD temporal abnormal field OS constricted field EOM FROM OU (-)pain, (-) diplopia Adnexa Normal OU

Biomicroscopy Conjunctiva white & quiet OU

Cornea Clear OU

Iris OD post-surgical w/suture @ 8 oclock OS normal

AC deep and quiet OU

Lens OD PCIOL in good position, tr PC haze OS NS 2+

IOP 16mm Hg OU

Additional Tests �  Ishihara

OD 5/7 OS 5/7

� Red Cap Test OS desaturated

� Subjective Brightness OS decreased

HVF 24-2

Fundus Examination OD OS

Vitreous clear Asteroid hyalosis

Optic nerve Flat, sharp, good color

Flat, sharp, good color

CD Ratio 0.2v/0.2h 0.25v/0.25h

Macula Rare drusen Rare drusen

Vessels 2/3 AV ratio 2/3 AV ratio

Periphery (-) holes, tears, detachments

(-) holes, tears, detachments

Differential Diagnoses � Sellar/suprasellar mass

�  Pituitary Adenoma 70% �  Craniopharyngiomas 12.8% �  Meningioma 10.6% �  Gliomas 3.9%

� Germinomas � Sarcoidosis � Lymphocytic infiltration � “Delayed” indirect optic nerve chiasm

injury

1.  Homonymous Hemianopsia, Bilateral

2.  Crystal Deposit, OS

3.  Cataract, Incipient OS

4.  Pupillary abnormalities, OD

5.  After cataract obsucration, OD

Assessment Plan

1.  Urgent Evaluation w/neurosurgery and neuroimaging

2.  Not clinically significant

3.  Incipient—not cause of visual loss

4.  H/o successful iris sutures

5.  Progressing PC haze

MRI Results �  Large Heterogenous

enhancing sellar mass

�  2.5 x 2.2 x 3.2 cm �  Cystic necrosis �  ICA – normal flow �  Cavernous sinuses –

unfiltrated �  Compresses optic

chiasm

http://neurosurgery.ucla.edu/images/Pituitary%20Program/NonFunction_MRI.jpg

Pituitary Adenoma

� Funtional � Non-functional

� Pre-fixed � Post-fixed

� Hardy Wilson Classification system1 �  Stage 0 �  Stage A �  Stage B �  Stage C

Systemic Symptoms

� Headache � Amenorrhea � Galactorrhea �  Impotence � Fatigue

http://2.bp.blogspot.com/-RC9sou7utqE/UYuh1YfpZDI/AAAAAAAAAMQ/l7rZRIPuMbk/s1600/Migraine%2BII.jpg

Visual Complaints � Decreased vision � Loss of depth perception � Difficulties with near tasks � Diplopia � Photophobia � Dyschromatopsia

Visual Loss Determinants

� Tumor size � Growth direction � Rate of Growth �  Individual anatomy

Visual Symptoms �  Chiasm

�  VF defect �  Intermittent diplopia �  Seesaw nystagmus

�  3rd Ventricle �  Hydrocephalus

� Upgaze �  Light-near diss

�  Convergence-retraction Nystagmus

�  Papilledema

� Cavernous sinus �  CN III

�  Upgaze �  Downgaze �  Medial gaze �  Diplopia �  Ptosis �  Mydriasis

�  CN IV & VI � LR & SO palsies

�  CN V1 � Loss of sensation

Visual Pathway

http://www.medicinemcq.com/index.php/journals/sub_details/138/39/FMGE-MARCH-2012-ENT-AND-OPHTHALMOLOGY

VF Defect & Pituitary Adenoma

VF Defect & Pituitary Adenoma

VF Defect & Pituitary Adenoma

Fundus Findings Temporal Pallor Band/Bowtie Atrophy Optic Atrophy Papilledema

http://ophthalmology.stanford.edu/blog/archives/2009/10/bowtie-optic-at.html

Pituitary Apoplexy �  Infarction �  Hemorrhage �  Necrosis

�  Systemic Symptoms �  Headache �  Altered state �  Nausea/vomiting �  Stiff neck �  Endocrine

Dysfunction �  Parasthesia �  Seizures

� Visual Symptoms �  Visual blurring �  VF defects �  Diplopia �  Ophthalmoplegia �  Dorsal Midbrain

syndrome �  Photophobia

Treatment � Goals

�  Control growth �  Normalize function �  Preserve/restore

vision

� Surgery � Medical Therapy � Radiotherapy � Expectant

Observation

Prognosis �  Surgery

�  VA 46.5 – 79% �  VF 70 – 90%

�  Radiotherapy �  VF 16 – 78% �  GKS

� 53% CN � 20% major

improvement � 30% dec tumor

volume

� Bromocriptine �  Improved visual

function �  VF improved

Risks of Treatment � Surgical Removal

�  Direct injury/devascularization of optic apparatus

�  Prolapse into empty sella

�  Orbital Fracture �  Hematoma �  Cerebral

vasospasm

� Bromocriptine �  Visual

hallucinations �  Tumor re-

expansion �  Chiasmal

herniation

Risks of Treatment � Chiasmopathy

�  Delayed radionecrosis of CNS

�  0.25 – 25%2

�  Radiation Optic neuropathy �  Acute, unilateral loss of

vision �  Central scotoma/arc

defects �  Bitemporal hemi �  ON initially normal à

pale �  MRI: enlargement of

enhancement of CNS/chiasmal tract

http://www.healio.com/~/media/Journals/OSLI/2012/1_January/10_3928_15428877_20111129_09/fig4.ashx

Risk Factors � Macroadenoma � Previous Visual

Impairment � Bottleneck/dumb-

bell shaped tumor � Previous surgery � Lumbar

subarachnoid catheter

http://www.neurologyindia.com/viewimage.asp?img=ni_2013_61_2_122_111114_f5.jpg

Predictors of Visual Outcome � > 20/100 pre-op: improvement � < 20/100 pre-op: normalized � Duration of visual complaints � Previous surgery � Age <69 � No frank Optic Atrophy � Tumor Size � Normal Color �  (-) APD

VF Improvement 1.  Early Fast Phase: days – 1 week 2.  Slow Recovery Phase: weeks – 2 months 3.  Late Phase: months – years

**improvement: as long as 5 years post-op

Post-op management � Multidisciplinary

approach

� Baseline Exam �  VA �  Color �  Motility �  VF

� Post-op �  2-3 months �  6-12 months

Conclusion � Multidisciplinary � Patients’ varying symptoms and status � Understanding VF, afferent visual

pathway, anatomy and CN exam

References 1.  Bynke, Olof. "Incidence of Neuro-ophthalmic Manifestations of Pituitary Adenomas in

the Referral Area of Linkoping, Sweden, 1965–1984." Neuro-Ophthalmology 7.3 (1987): 165-73. Print.

2.  Chhabra, Vaninder S., MD, and Nancy J. Newman, MD. "The Neuro-Ophthalmology of Pituitary Tumors." Comprehensive Ophthalmology Update 7.5 (2006): 225-37. Print.

3.  Chiu, Eric K., and Jeffrey W. Nichols. "Sellar Lesions and Visual Loss: Key Concepts in Neuro-ophthalmology." Expert Review Anticancer Therapy 6.9 (2006): S23-28. Print.

4.  Chung, Sophia M., MD. "Neuro-ophthalmic Manifestations of Pituitary Tumors." Neuro-Ophthalmology for Neurosurgeons 10.4 (1999): 717-29. Print.

5.  Dekkers, O. M., S. Hammer, R. J W De Keizer, F. Roelfsema, P. J. Schutte, J. W A Smit, J. A. Romijn, and A. M. Pereira. "The Natural Course of Non-functioning Pituitary Macroadenomas." European Journal of Endocrinology 156.2 (2007): 217-24. Print.

6.  Dhar, Meenakshi Y., MS, and Niranjan K. Pehere, MS. "Unusual Visual Manifestations of Pituitary Tumours." Kerala Journal of Ophthalmology XIX.2 (2007): 147-55. Print.

7.  Elgamal, Ehud, E. Oosman, S. El-Watidy, A. Hazem, N. Al-Khawajah, N. Jastaniyah, and M. Al-Rayess. "Pituitary Adenomas: Patterns of Visual Presentation and Outcome After Transsphenoidal Surgery - An Institutional Experience." The Internet Journal of Ophthalmology and Visual Science 4.2 (2006): n. pag. Print.

References cont. 8.  Fu, Xiangping, and Hongqi Wang. "Ocular Symptoms of Tumors at Sella Turcica Region."

Eye Science 12.3 (1996): 166-68. Print. 9.  Gedik, Sansal, MD, Sirel Gur, MD, Basar Ataly, MD, Meric Colak, Nur Altinors, MD, and

Yonca A. Akova, MD. "Humphrey Visual Field Analysis, Visual Field Defects, and Ophthalmic Findings in Patients with Macro Pituitary Adenoma." Saudi Medical Journal 28.9 (2007): 1380-384. Print.

10.  Kedar, Sachin, Deepta Ghate, and Jamesj Corbett. "Visual Fields in Neuro-ophthalmology." Indian Journal of Ophthalmology 59.2 (2011): 103. Print.

11.  Lee, Jung Pil, In Won Park, and Yun Suk Chung. "The Volume of Tumor Mass and Visual Field Defect in Patients with Pituitary Macroadenoma." Korean Journal of Ophthalmology 25.1 (2011): 37. Print.

12.  Monteiro, Mário L.r., Beatriz K. Zambon, and Leonardo P. Cunha. "Predictive Factors for the Development of Visual Loss in Patients with Pituary Macroadenomas and for Visual Recovery after Optic Pathway Decompression." Canadian Journal of Ophthalmology 45.4 (2010): 404-08. Print.

13.  Schmalisch, Kathrin, Monika Milian, Thilo Schimitzek, Wolf A. Lagrèze, and Juergen Honegger. "Predictors for Visual Dysfunction in Nonfunctioning Pituitary Adenomas - Implications for Neurosurgical Management." Clinical Endocrinology 77.5 (2012): 728-34. Print.

14.  Simon, Sumu, David Torpy, Brian Brophy, Peter Blumbergs, and Dinesh Selva. "Neuro-ophthalmic Manifestations and Outcomes of Pituitary Apoplexy--a Life and Sight-threatening Emergency." The New Zealand Medical Journal 124.1335 (2011): 52-58. Print.