1 / 39

GRAND ROUNDS

GRAND ROUNDS. Desirée Ong, M.D. Vanderbilt Eye Institute June 28, 2007. Our Patient. CC: “My appearance has changed” HPI: 57 yo F Left eye has progressively “pulled outward” Binocular diplopia x 3 yrs Lashes rub against her sunglasses

kenny
Download Presentation

GRAND ROUNDS

An Image/Link below is provided (as is) to download presentation Download Policy: Content on the Website is provided to you AS IS for your information and personal use and may not be sold / licensed / shared on other websites without getting consent from its author. Content is provided to you AS IS for your information and personal use only. Download presentation by click this link. While downloading, if for some reason you are not able to download a presentation, the publisher may have deleted the file from their server. During download, if you can't get a presentation, the file might be deleted by the publisher.

E N D

Presentation Transcript

  1. GRAND ROUNDS Desirée Ong, M.D. Vanderbilt Eye Institute June 28, 2007

  2. Our Patient • CC: “My appearance has changed” • HPI: 57 yo F • Left eye has progressively “pulled outward” • Binocular diplopia x 3 yrs • Lashes rub against her sunglasses • Students said they did not know who she was looking at

  3. History • POHX: • Narrow-angle glaucoma OU s/p LPI OU • Eye muscle surgery x 3 within last two years • Revision of lower eyelid OS for post-surgical scarring • PMHX: • Hypothyroid • Arthritis • Depression • Concussion x 2 • FHx: Daughter has strabismus

  4. History • Social Hx: • Married with grown children • No tobacco or ETOH • 8th grade teacher • Meds: • Wellbutrin • Synthroid • Hormone replacement therapy • Alphagan BID OU • Allergies: Darvocet

  5. Exam • BCVA: OD 20/15, OS 20/25-2 • CVF: full OU • Ishihara: 15/15 OU • Hertel: 7 mm of proptosis OS • Pupils: 32mm OU, no RAPD

  6. Motility

  7. Exam • L/L: quiet OD, inferior symblepharon OS • S/C: quiet OU • K: quiet OD, PEE OS • A/C: narrow angles OU • Iris: patent superior LPI OU • Lens: 1+ NSC OU • Ant vitreous: quiet OU

  8. Differential Diagnosis?Proptosis with extraocular motility limitation

  9. Differential DiagnosisProptosis with extraocular motility limitation • Neoplastic • Sphenoid wing meningioma • Lacrimal gland tumor • Schwannoma • Mucocele • Nasopharyngeal tumor • Dermoid cyst • Metastasis • Solitary fibrous tumor • Fibrous histiocytoma • Hemangiopericytoma • Lymphoproliferative • Neurofibroma • Leiomyoma • Glioma

  10. Differential DiagnosisProptosis with extraocular motility limitation • Autoimmune • TED – 60% of all orbital disease • Inflammatory • Pseudotumor • Sarcoid granuloma • Vascular • Cavernous hemangioma • Varix • A-V fistula • Infectious • Orbital cellulitis • Tuberculosis granuloma • Trauma • Retrobulbar hematoma

  11. What is next?

  12. Imaging: MRI • T1: isointense to brain • T2: hyperintense to brain, isointense to CSF • Marked enhancement on T1 with gadolinium • “Peripheral ring” • Cavitary change or heterogeneity

  13. Our Patient Excisional biopsy Underwent anterior orbitotomy with image-guided excision of a well-encapsulated mass 4/20/07

  14. Gross Pathology • Yellow-tan lesion with varicose vessels on the surface • Cone-shaped > dumbbell > oval > round • True capsule composed of perineurium • Eccentric position within capsule Wt. 3.58 g, dimensions: 3.4 x 2 x 1.4 cm

  15. Pathology Verocay bodies: dense whorls • Antoni A pattern: compact palisading nuclei

  16. Pathology

  17. Pathology Antoni B pattern: ovoid clear cells, loose pattern of myxoid stromal degeneration, cystic spaces

  18. Pathology • Most common pattern: mixed Antoni A and B (47%) • No axons present within the substance of the tumor • Fibrosis and hyalinization around blood vessels, vessel walls contain xanthoma cells • “Ancient” lesions can have calcification, cystic changes Luse bodies: cytoplasmic collagen fibrils upload.wikimedia.org/wikipedia/commons/thumb/... ‘Ancient’ http://www.histopathology-india.net/Schwannoma.htm

  19. Immunohistochemistry Diffuse S-100 positivity • S-100 positive • Vimentin positive • Leu7 positive • Progesterone-receptor positive • May account for increased proptosis during pregnancy • Possible place for hormonal tx http://www.emedicine.com/derm/topic285.htm

  20. Diagnosis Orbital Schwannoma

  21. Schwannoma(Neurilemoma) • Benign tumor of Schwann cell sheaths of peripheral sensory nerves • First described by Verocay in 1910 • Well-defined, slowly progressive and non-invasive with low malignant potential

  22. Schwannoma • No racial predilection • Female predominance • 2nd to 5th decades (mean: 40 yrs) • 18% associated with neurofibromatosis

  23. Schwannoma • 55% of all peripheral nerve tumors • Predilection for the head/neck, flexor surfaces of extremities • Solitary except for in NF-1 or 2

  24. Orbital Schwannoma • 1-2% of orbital tumors • Extraconal > intraconal • Superior > medial superior > apex

  25. Orbital Schwannoma • Most common: sensory branches of CN V1 (supratrochlear/supraorbital) • Rarely from motor nerves: CN III (superior division, ciliary nerve), CN VI • Optic nerve never involved since an extension of the CNS • Rarely intramuscular, epibulbar or intraosseus

  26. Symptoms • Initially asymptomatic • Diplopia • Headache • Decreased vision • Direct optic nerve compression • Induced hyperopia • Numbness or pain in distribution of involved nerve – rare unless large tumor

  27. Signs • Proptosis – most common • Lid swelling • Restricted ocular motility • Globe displacement • Afferent pupillary defect • Choroidal folds • Optic atrophy/disc edema http://content.lib.utah.edu/cgi-bin/showfile.exe?CISOROOT=/EHSL-WFH&CISOPTR=174

  28. Diagnosis and Management • MRI with/without contrast: imaging method of choice • Observation vs excision • Radiation • Evaluation for NF with bilateral vestibular schwannomas and age < 30

  29. Surgical approach • Governed by location of tumor within the orbit • Complete removal is recommended • Aspiration or piecemeal excision • Small risk of recurrence with incomplete removal • One reported case of recurrence and malignant transformation after incomplete excision in a pt with neurofibromatosis • May strip tumor from nerve of origin via microsurgical technique

  30. Our Patient at Follow-Up (6/19/07) • Pt still complains of diplopia in primary gaze • Motility: • Plan: strabismus surgery to improve her diplopia

  31. Teaching points • Early diagnosis and accurate evaluation of the extent of an orbital schwannoma is critical for restoration of vision and preserving extraocular eye movements • Complete excision, if possible, is recommended to prevent recurrence and malignant transformation

  32. References • Chang BY, Moriarty P, Cunniffe G, Barnes C, Kennedy S. Accelerated growth of a primary orbital schwannoma during pregnancy. Eye. 2003 Oct;17(7):839-41. • Cockerham KP, Cockerham GC, Stutzman R et al. The clinical spectrum of schwannomas presenting with visual dysfunction: a clinicopathologic study of three cases. Surv Ophthalmol. 1999 Nov-Dec;44(3):226-34. • Gunduz K, Shields CL, Gunalp I, Erden E, Shields JA. Correlation of magnetic resonance imaging and pathologic findings. Graefe’s Arch Clin Exp Ophthalmol 2003; 241: 593-597. • Rawlings NG, Brownstein S, Robinson JW, Jordan DR. Orbital schwannoma: histopathologic correlation with magnetic resonance imaging. Can J Ophthalmol. 2007 Apr;42(2):326-8. • Rootman J. Diseases of the Orbit. Philadelphia: J.B. Lippincott Company, 1988: 319-325. • Shields JA, Shields, CL. Atlas of Orbital Tumors. Philadelphia: Lippincott Williams and Wilkins, 1999: 76-78. • Wang Y, Xiao LH.Orbital schwannomas: findings from magnetic resonance imaging in 62 cases. Eye. 2007 Apr 20; epublication.

More Related