Grand Ward Round

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Grand Ward Round
4th October 2007 Jamie Ng

• The Eye Institute Tan Tock Seng Hospital

2
History

• Mr CYO 37/Chinese/Male
• Medical history
• Obstructive sleep apnoea
• Obese
• Chronic sputum 2yrs with occ haemoptysis
• Prev investigated and treated with course of
  anti-biotics, improved
• Currently on TCM
• Multiple skin lumps for many years (Father also
  has similar lumps)

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Presenting Complaints

• Left eye BOV for 2/12
• Prominence of left eyeball for 9/12
• Gradually increasing
• Also noticed that his short-sightedness became
  less since 4/12 ago
• No pain
• Systemic review essentially normal

4
Examination

• VR - 6/6 VL 6/24 ? 6/12

5
Examination

• Fine tremors and warm palms noted
• Left axial proptosis
• Mild chemosis LE
• GAT 14 mmHg OU

6
Examination

• EOM

I I II
I I II
I I II
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Examination

• No RAPD
• Ishihara R 15/15 L 15/15
• Light brightness R 100 L 80
• No red desaturation
• Confrontation - BE no gross field defects
• Anterior Segment BE otherwise normal

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Examination

• Posterior segment

9
Examination
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Differential diagnosis

• Thyroid eye disease
• Tumours intraconal
• Optic nerve meningioma
• Optic nerve glioma
• Orbital lymphoma
• Cavernous hemangioma
• Orbital varix
• Carotid Cavernous Fistula
• Metastatic disease

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Investigations

• Blood investigations
• TSH, T3, T4 and TSH receptor Antibody normal
• FBC, U/E/CR, ESR and syphillis screen - normal
• Imaging
• CT orbits 18/09/07

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Investigations

• MRI Orbits 20/09/07

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Subsequently

• Left grade 1 to 2 RAPD
• Ishihara R 15/15 L 13/15
• Light brightness R 100 L 70
• Left intraconal orbital mass with early
  compression of optic nerve
• Referred to OPLS for biopsy of lesion via lateral
  orbitotomy

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Subsequently

• Review of films and patient

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Ocular Lymphoma

• 1. Lymphoma of ocular adnexa
• 2. Primary intra-ocular CNS lymphoma
• Diffuse large B-cell NHL
• Rare
• A. Solitary or multiple intracranial nodules
• B. Diffuse meningeal lesions
• C. Localized intradural masses
• D. Intraocular involvement
• Poor prognosis

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Ocular Adnexal Lymphoma

• Histological features of ocular adnexal lymphoma
  (REAL classification) and their association with
  patient morbidity and survival.
• Jenkins C, Rose GE, Bunce C, Wright JE, Cree IA,
  Plowman N, Lightman S, Moseley I, Norton A.
• Orbital Clinic, Moorfields Eye Hospital, London.
• Br J Ophthalmol. 2000 Aug84(8)907-13

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Ocular Adnexal Lymphoma

• The biopsies and clinical follow up data for 192
  patients with ocular adnexal lymphoma were
  reviewed
• Classified according to the Revised European
  American Lymphoma (REAL) system
• 1. Marginal Zone Lymphoma
• 2. Lymphoplasmocytic lymphoma
• 3. Follicle center lymphoma
• 4. Diffuse large B cell lymphoma
• 5. Plastocytoma and other rare histologic variants

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Ocular Adnexal Lymphoma
Marginal Zone Lymphoma
Follicle Center Lymphoma
Diffuse Large B-cell Lymphoma
Atypical Mantle Cell
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Ocular Adnexal Lymphoma
20
Ocular Adnexal Lymphoma
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Ocular Adnexal Lymphoma

• Treatment All but 4 patients received local DXT
  and/or various combinations of chemotherapy
• Mean time to relapse in orbital MZL 63 months
• MZL - considered indolent and have a good
  prognosis
• Provides evidence showing good correlation of
  histoloical grade and survival outcome in ocular
  adnexal lymphoma

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Ocular Adnexal Lymphoma

• Management
• Correct histological diagnosis and accurate
  staging
• Localized low grade
• up to 90 controlled with DXT
• DXT is often well tolerated
• Intermediate to high grade combined with
  adjuvant chemotherapy
• Localized orbital lymphoma. Galieni P, Polito E,
  Leccisotti A, Marotta G, Lasi S, Bigazzi C,
  Bucalossi A, Frezza G, Lauria F. Haematologica.
  1997 Jul-Aug82(4)436-9.
• Radiotherapy for Orbital Lymphoma Outcome and
  Late Effects. Bischof M, Karagiozidis M, Krempien
  R, Treiber M, Neuhof D, Debus J, Zierhut D.
  Strahlenther Onkol. 2007 Jan183(1)17-22

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Interesting reports

• Concomitant HCV infection is associated with more
  disseminated disease and aggressive behavior in
  OAL.
• HCV seropositivity is present in 13 of OAL of
  MALT-type.
• Clinical implications of hepatitis C virus
  infection in MALT-type lymphoma of the ocular
  adnexa.Ferreri AJ, Viale E, Guidoboni M, Resti
  AG, De Conciliis C, Politi L, Lettini AA,
  Sacchetti F, Dolcetti R, Doglioni C, Ponzoni M.
  Ann Oncol. 2006 May17(5)769-72. Epub 2006 Mar 8
• Chlamydia psittaci infection may contribute to
  the development of lymphomas, as was also
  supported by the clinical responses observed in
  this study with C. psittaci-eradicating
  antibiotic therapy.
• Evidence for an association between Chlamydia
  psittaci and ocular adnexal lymphomas. Ferreri
  AJ, Guidoboni M, Ponzoni M, De Conciliis C,
  Dell'Oro S, Fleischhauer K, Caggiari L, Lettini
  AA, Dal Cin E, Ieri R, Freschi M, Villa E,
  Boiocchi M, Dolcetti R. J Natl Cancer Inst. 2004
  Apr 2196(8)586-94

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Thank You

• The Eye Institute - Jamie Ng