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Pathology of uvea
Azza Mohamed Ahmed Said
Ass. Prof. Of Ophthalmology
Ain Shams University
Uveal tract
Uveal tract
• Uveal tract is embryologically derived from mesoderm
and neural crest.
• Firm attachments between the uveal ...
IRIS
• Most anterior part of uveal tract.
• Circular diaphragm e central hole.
• Forms posterior wall of anterior
chamber....
IRIS
• The iris is composed of 5 layers:
1-Anterior border layer
4-Anterior pigment epithelium

2-Stroma
3 - Muscular laye...
CILIARY BODY
6.0- 6.5 mm wide.
Inner surface:2parts
• pars plicata ant.1/3
• pars plana post.2/3
• Lined by double layer o...
CHOROID
consists of 3 principal layers:
• lamina fusca (suprachoroid layer)
• stroma
• choriocapillaris
The choriocapillar...
Common pathological disorders
Congenital
Congenital
Inflammation
Inflammation
Neoplasms
Neoplasms
Degenerative
Degenerativ...
Iris pathology
Abnormal neural crest cell migration

Axenfeld anomaly

Rieger anomaly

Peter's anomaly

Sclerocornea
Abnormal neural crest cell proliferation
Iridocorneal Endothelial Syndrome

Iris naevus (Cogan-Reese) syndrome
Progressive...
Abnormal neural crest cell final
differentiation

Congenital hereditary endothelial dystrophy
Congenital and
developmental anomalies
Coloboma
Aniridia

Partial absence
Wilm’s
Tumor

Synechial angle-closure
glaucoma in 75%
Iris cysts
Unilateral
stationary
Primary epithelial cyst

Primary stromal cyst
Iris stromal cyst lined by stratified squamous
epithelium
Miotic cyst
Implantation cysts

Pearl cyst

Serous cyst
Iris nodules
Causes of iris nodules
• Cysts….
• Solid swellings:
1- Brushfield spots.
2- Lisch nodules.
3- Inflammatory:
• Koeppe's and...
Brushfield spots in Down syndrome

Relatively normal, or mildly hypercellular, iris
stroma (between arrows) surrounded by ...
Lisch nodules in NF1

Benign iris hamartomas
Koeppe's and Busacca's in nodules

Busacca’s nodules:
on the surface of iris

Koeppe’s nodules:
at pupillary margin

Aggre...
FB granuloma
Juvenile Xanthogranuloma




Pediatric skin disorder of non-langerhan's cell
histiocytosis. (benign)
Skin lesions



...
Juvenile Xanthogranuloma


Extracutaneous…
1- The uveal tract, is the m/f site:
 Iris nodules
 Heterochromia
 Uveitis
...
Iris melanoma
 Color: Pigmented or non pigmented.
 Site : inferior half.
 Size : ›1mm X3mm.
 Shape:
– Nodular
– Diffus...
Iris melanoma

Iris mass completely replacing the normal iris stroma, extending into
the anterior chamber, touching the po...
Melanoma cytology
• Spindle-A cells have slender,
elongated nuclei with small
nucleoli.
• A central stripe may be present
...
Melanoma cytology
• Spindle-B cells demonstrate a
higher nuclear to- cytoplasmic
ratio; more coarsely granular
chromatin; ...
Melanoma cytology
• Epithelioid cells resemble
epithelium because of abundant
eosinophilic cytoplasm and
enlarged oval to ...
Histological classification of uveal melanoma

Spindle A cell

Spindle cell (45%)

Mixed cell (45%)

Spindle B cell

Pure ...
Iris Melanoma
1. Very rare - 8% of uveal melanomas
2. Presentation - fifth to sixth decades
3. Very slow growth
4. Low mal...
Iris nevus
•

Benign tumor that arises in the
melanocytes in the iris stroma.

• Composed of low-grade spindletype cells.
...
Iris leiomyoma
• Benign tumor, arises from smooth
muscles of iris.
• DD :
Iris amelanotic melanoma
– Location :may not pre...
Adenoma of iris pigment epithelium

Benign Pigmented friable nodule in the angle due to
proliferation of iris pigment epit...
Malignant infiltrates

Malignant hypopyon and
hyphema in leukemic patient

Retinoblastoma
infiltrates of iris
CB malignant melanoma invading the
iris
Ciliary Body tumors
Ciliary body tumors
• Stromal tumors:
– Benign : angioma, neurofibroma, leiomyoma.
– Malignant: malignant melanoma, metast...
Ciliary body melanoma
 Clinical picture
 Asymptomatic.
 Blurring of vision.(lenticular
astigmatism, presbyopia)
 Catar...
CB malignant melanoma

Ring melanoma
Malignant melanoma
• Melanomas of the ciliary body have a
similar

histologic

melanomas

in

the

appearance
choroid

to
...
Medulloepithelioma (Diktyoma)
•
•
•
•

Affects children between 6 months and 5 years.
Aises from inner layer of optic cup....
Pseudoadenomatous hypeplasia
(Fuchs' adenoma)
• A glistening, white irregular
tumor arising from the ciliary
body. It cons...
Adenoma and adenocarcinoma
• Adenoma

shows

tubular

proliferation of pigment epithelium
of the ciliary body.
• Adenocarc...
Melanocytoma
• Heavily pigmented tumors with
large polygonal cells and small
vesicular nuclei with abundant
cytoplasm tota...
Leiomyoma

• Leiomyoma
body.

The

of

the

ciliary

tumor

shows

bundles of eosinophilic spindle
cells. The neoplastic c...
Choroidal pathological
changes
Age –related changes: Drusen
Histopathology

Hard

•

•

Small well-defined
spots
Usually innocuous

Soft

•

Larger, ill-...
CNVM
Type 2
In
CNVM
Type 1
In
ARMD

POHS
Aicardi syndrome

Very rare
• X-linked dominant which is lethal in utero for males
• Infantile spasms
• Developmental dela...
Hereditary choroidal dystrophies
1. Choroideremia
2. Gyrate atrophy
3. Central areolar choroidal dystrophy
4. Diffuse chor...
Choroideremia
Inheritance - X-linked recessive
Presents - first decade with nyctalopia
Prognosis - good VA until late
ERG ...
Female carriers of choroideremia

Central patchy atrophy and
mottling of RPE

Peripheral diffuse pigmentary
granularity
Gyrate atrophy
Cause - deficiency of ornithine keto-acid aminotransferase
Inheritance - autosomal recessive
Presents - fir...
Central areolar choroidal dystrophy
Inheritance - dominant
Presents - fifth decade
Prognosis - poor
ERG - normal

•
•

Bil...
Diffuse choroidal atrophy

Inheritance - dominant
Presents - fourth to fifth decades
Prognosis - poor
ERG - reduced

•
•

...
Elschnig's spots
• Elschnig's
spots
are
ischemic infarcts of the
choroid and
RPE that
appear in the posterior pole.
• Caus...
Uveitis
• Def.: inflammation of the uveal tract.
• Classification:
a-a- Anatomical
Anatomical

b- Clinical

anterior inter...
Uveitis

Specific
Fuch’s heterochromic

Non specific
25%
Inflammation
A reaction of microcirculation ccc. By
movement of fluid and cells into the
extravascular tissues in response...
Inflammation
Categories (by type of cells in tissue or exudates)
• Acute
– Polymorphonuclear leucocytes.
– Mast cells and ...
Granulomatous inflammation
Three histologic patterns of granulomatous
inflammation
• Diffuse.
• Discrete.
• Zonal.
1- Diffuse granulomatous inflammation
• Epithelioid histiocytes are
scattered throughout the
involved uveal tissue. There
...
1- Diffuse granulomatous inflammation
• Sympathetic ophthalmitis
– Bilateral diffuse chronic granulomatous panuveitis
– Th...
1- Diffuse granulomatous inflammation
• Sympathetic ophthalmitis
– Histologically, both the traumatized and
sympathizing e...
1- Diffuse granulomatous inflammation
2- Discrete granulomatous inflammation
• Well-circumscribed areas of
epithelioid histiocytes
• Seen in :
Sarcoidosis: Non ...
2- Discrete granulomatous inflammation
• The classic sarcoid nodule is composed of
noncaseating granulomas. These are
coll...
3- Zonal granulomatous inflammation
• A central zone of necrosis and/or
polymorphonuclear
leukocytes
surrounded
by
epithel...
Typical choroidal nevus
• Common - 2% of population
• Round slate-grey with
indistinct margins
• Surface drusen
• Flat or ...
Nevus cells
Four types of nevus cells:
• Plump polyhedral: abundant cytoplasm filled with pigment and a
small, round to ov...
Choroidal melanoma
• Most common primary intraocular
tumour in adults.
• Most common uveal melanoma 80% of cases
• Present...
Predisposing factors
Suspicious choroidal nevus

• Diameter more than 5 mm
• Elevation 2 mm or more
• Surface lipofuscin
•...
Course of malignant melanoma of the choroid
(knapp’s classification)
1. Quiescent stage:
Tumor starts in the outer layers ...
Course of malignant melanoma of the choroid
(knapp’s classification)
Course of malignant melanoma of the choroid
2. Glaucomatous stage:
a) Obstruction of venous outflow.
b)

Pusching of iris ...
Course of malignant melanoma of the choroid
3. Extraocular stage:
a) Scleral foramina… epibular mass
b) direct sclera inva...
Course of malignant melanoma of the choroid
4. Metastasis stage:
Hematogenous spread to liver, lung, bone.
Histological classification of uveal melanoma

Spindle A cell

Spindle cell (45%)

Mixed cell (45%)

Spindle B cell

Pure ...
Malignant melanoma of choroid
• Clinical picture






Asymptomatic.
Photopsia or floaters.
Painless visual loss.
Pai...
Choroidal melanoma (1)

• Brown, elevated, subretinal mass

• Secondary retinal detachment

• Occasionally amelanotic
• Do...
Choroidal melanoma (2)

• Surface orange pigment (lipofuscin) is
common.
• Mushroom-shaped if breaks through
Bruch’s membr...
Poor Prognostic Factors of Uveal Melanomas
1. Histological
• Epithelioid cells
• Closed vascular loops
• Lymphocytic infil...
Differential diagnosis of choroidal melanoma

Large choroidal naevus

Choroidal detachment

Metastatic tumour

Choroidal g...
Circumscribed choroidal haemangioma
• Presentation - adult life
• Dome-shaped or placoid,
red-orange mass
• Commonly at po...
Circumscribed choroidal haemangioma

It is composed of cavernous spaces filled with red blood
cells and is usually demarca...
Diffuse choroidal haemangioma
Typically affects patients with Sturge-Weber syndrome

Can be missed unless compared with no...
Choroidal metastatic carcinoma
Most frequent primary site is breast in women and bronchus in both sexes

• Fast-growing, c...
Choroidal osseous choristoma
Compact bone contain osteocytes, and intratrabecular
spaces are filled with loose connective ...
Melanocytoma (Magnocellular nevus)
• Affects dark skinned
individuals
• Usually asymptomatic
• Most frequently affects
opt...
Choroidal ruptures
• Blunt trauma.
• Semicircular lines circumscribing
the optic nerve head in the
peripapillary region.
•...
Choroidal detachment

• - Associated with hypotony.
• - Unilateral, brown, smooth,
solid and immobile.
Heterochromia iridis
Different colours of the two irides
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Pathology of uvea
Pathology of uvea
Pathology of uvea
Pathology of uvea
Pathology of uvea
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Pathology of uvea

  1. 1. Pathology of uvea Azza Mohamed Ahmed Said Ass. Prof. Of Ophthalmology Ain Shams University
  2. 2. Uveal tract
  3. 3. Uveal tract • Uveal tract is embryologically derived from mesoderm and neural crest. • Firm attachments between the uveal tract and the sclera exist at only 3 sites: – Scleral spur – Exit points of the vortex veins – Optic nerve
  4. 4. IRIS • Most anterior part of uveal tract. • Circular diaphragm e central hole. • Forms posterior wall of anterior chamber. • Lies in front of crystalline lens. • Colour differs acc. To melanin concentration in melanocytes. • Has 2 surfaces (ant &post). ant.stromal
  5. 5. IRIS • The iris is composed of 5 layers: 1-Anterior border layer 4-Anterior pigment epithelium 2-Stroma 3 - Muscular layer 5-Posterior pigment epithelium Melanocytes Clump cells 2 layers of Cuboidal epith
  6. 6. CILIARY BODY 6.0- 6.5 mm wide. Inner surface:2parts • pars plicata ant.1/3 • pars plana post.2/3 • Lined by double layer of epith.cells: inner non pig. and outer pig
  7. 7. CHOROID consists of 3 principal layers: • lamina fusca (suprachoroid layer) • stroma • choriocapillaris The choriocapillaris is the blood supply for (RPE) and the outer retinal Layers.
  8. 8. Common pathological disorders Congenital Congenital Inflammation Inflammation Neoplasms Neoplasms Degenerative Degenerative Vascular Vascular Traumatic Traumatic Primary Metastasis
  9. 9. Iris pathology
  10. 10. Abnormal neural crest cell migration Axenfeld anomaly Rieger anomaly Peter's anomaly Sclerocornea
  11. 11. Abnormal neural crest cell proliferation Iridocorneal Endothelial Syndrome Iris naevus (Cogan-Reese) syndrome Progressive iris atrophy Chandler syndrome
  12. 12. Abnormal neural crest cell final differentiation Congenital hereditary endothelial dystrophy
  13. 13. Congenital and developmental anomalies
  14. 14. Coloboma
  15. 15. Aniridia Partial absence Wilm’s Tumor Synechial angle-closure glaucoma in 75%
  16. 16. Iris cysts
  17. 17. Unilateral stationary
  18. 18. Primary epithelial cyst Primary stromal cyst
  19. 19. Iris stromal cyst lined by stratified squamous epithelium
  20. 20. Miotic cyst
  21. 21. Implantation cysts Pearl cyst Serous cyst
  22. 22. Iris nodules
  23. 23. Causes of iris nodules • Cysts…. • Solid swellings: 1- Brushfield spots. 2- Lisch nodules. 3- Inflammatory: • Koeppe's and Busacca's in nodules in granulomatous uveitis. • FB granuloma. • Juvenile xanthograuloma. 4- Neoplasms: • Benign: naevus, leiomyoma, neurofibroma, hemangioma, adenoma of iris pigment epithelium. • Malignant: – Primary :iris melanoma – Secondaries : extension from CB melanoma, leukemic deposits.
  24. 24. Brushfield spots in Down syndrome Relatively normal, or mildly hypercellular, iris stroma (between arrows) surrounded by a hypoplastic iris
  25. 25. Lisch nodules in NF1 Benign iris hamartomas
  26. 26. Koeppe's and Busacca's in nodules Busacca’s nodules: on the surface of iris Koeppe’s nodules: at pupillary margin Aggregates of epithelioid cells & mononuclear cells
  27. 27. FB granuloma
  28. 28. Juvenile Xanthogranuloma   Pediatric skin disorder of non-langerhan's cell histiocytosis. (benign) Skin lesions    Firm, slightly raised papulonodules Several millimeters in diameter Tan-orange in color
  29. 29. Juvenile Xanthogranuloma  Extracutaneous… 1- The uveal tract, is the m/f site:  Iris nodules  Heterochromia  Uveitis  Spontaneous hyphema  Secondary glaucoma 2- Eyelid: typical cutaneous lesions. 3- Orbit: Infiltrative soft tissue tumors cause proptosis.
  30. 30. Iris melanoma  Color: Pigmented or non pigmented.  Site : inferior half.  Size : ›1mm X3mm.  Shape: – Nodular – Diffuse (ring sarcoma)  Associated features: Pupil distortion, ectropion uvea, localized cataract, angle invasion, glaucoma, uveitis
  31. 31. Iris melanoma Iris mass completely replacing the normal iris stroma, extending into the anterior chamber, touching the posterior cornea, and occluding the angle.
  32. 32. Melanoma cytology • Spindle-A cells have slender, elongated nuclei with small nucleoli. • A central stripe may be present down the long axis of the nucleus
  33. 33. Melanoma cytology • Spindle-B cells demonstrate a higher nuclear to- cytoplasmic ratio; more coarsely granular chromatin; and plumper, large nuclei. • Nucleoli are prominent and mitoses are present. though not in large numbers.
  34. 34. Melanoma cytology • Epithelioid cells resemble epithelium because of abundant eosinophilic cytoplasm and enlarged oval to polygonal nuclei. • Nuclei have a conspicuous nuclear membrane, very coarse chromatin, and large nucleoli.
  35. 35. Histological classification of uveal melanoma Spindle A cell Spindle cell (45%) Mixed cell (45%) Spindle B cell Pure epithelioid cell (5%) Necrotic (5%)
  36. 36. Iris Melanoma 1. Very rare - 8% of uveal melanomas 2. Presentation - fifth to sixth decades 3. Very slow growth 4. Low malignancy (spindle type) 5. Excellent prognosis (early detection, low grade malignancy, late metastasis)
  37. 37. Iris nevus • Benign tumor that arises in the melanocytes in the iris stroma. • Composed of low-grade spindletype cells. • Asymptomatic pigmented, wellcircumscribed lesion in the iris stroma . • Flat or minimally elevated.
  38. 38. Iris leiomyoma • Benign tumor, arises from smooth muscles of iris. • DD : Iris amelanotic melanoma – Location :may not present in inferior half. – Immunohistochemical analysis with anti-muscle specific actin and electron microscopy.
  39. 39. Adenoma of iris pigment epithelium Benign Pigmented friable nodule in the angle due to proliferation of iris pigment epithelium or pig. or non-pig. epith. Of CB.
  40. 40. Malignant infiltrates Malignant hypopyon and hyphema in leukemic patient Retinoblastoma infiltrates of iris
  41. 41. CB malignant melanoma invading the iris
  42. 42. Ciliary Body tumors
  43. 43. Ciliary body tumors • Stromal tumors: – Benign : angioma, neurofibroma, leiomyoma. – Malignant: malignant melanoma, metastatic carcinoma. • Epithelial tumors: – Benign: adenoma, pseudoadenomatous hyperplasia. – Malignant : Medulloepithelioma, adenocarcinoma.
  44. 44. Ciliary body melanoma  Clinical picture  Asymptomatic.  Blurring of vision.(lenticular astigmatism, presbyopia)  Cataract, sublaxation.  Epibulbar mass  Sentinel vs.  Heterochromia, iris nodule.  Uveitis  Glaucoma  Ch.detachment, ERD.
  45. 45. CB malignant melanoma Ring melanoma
  46. 46. Malignant melanoma • Melanomas of the ciliary body have a similar histologic melanomas in the appearance choroid to and demonstrate spindle- to epithelioidtype cells with variable pigmentation. Mitotic figures may be seen.
  47. 47. Medulloepithelioma (Diktyoma) • • • • Affects children between 6 months and 5 years. Aises from inner layer of optic cup. Teratoid or non teratoid, both subdivied into benign or malignant. Multi-layered sheets or cords of immature neuroepithelial cells/rosettes.
  48. 48. Pseudoadenomatous hypeplasia (Fuchs' adenoma) • A glistening, white irregular tumor arising from the ciliary body. It consists of a benign proliferation of non-pigmented ciliary epithelial accumulation of cells with basement membrane-like material.
  49. 49. Adenoma and adenocarcinoma • Adenoma shows tubular proliferation of pigment epithelium of the ciliary body. • Adenocarcinomas are composed of gland-like accumulations of cells with prominent nuclei, nucleoli and marked pleomorphism. • Pigmented or non-pigmented.
  50. 50. Melanocytoma • Heavily pigmented tumors with large polygonal cells and small vesicular nuclei with abundant cytoplasm totally packed with melanin granules.
  51. 51. Leiomyoma • Leiomyoma body. The of the ciliary tumor shows bundles of eosinophilic spindle cells. The neoplastic cells are immunoreactive with musclespecific actin.
  52. 52. Choroidal pathological changes
  53. 53. Age –related changes: Drusen Histopathology Hard • • Small well-defined spots Usually innocuous Soft • Larger, ill-defined spots May enlarge and coalesce • Increased risk of AMD •
  54. 54. CNVM Type 2 In CNVM Type 1 In ARMD POHS
  55. 55. Aicardi syndrome Very rare • X-linked dominant which is lethal in utero for males • Infantile spasms • Developmental delay • CNS malformations and early demise • Multiple ‘chorioretinal lacunae’ Disc coloboma and pigmentation
  56. 56. Hereditary choroidal dystrophies 1. Choroideremia 2. Gyrate atrophy 3. Central areolar choroidal dystrophy 4. Diffuse choroidal atrophy
  57. 57. Choroideremia Inheritance - X-linked recessive Presents - first decade with nyctalopia Prognosis - good VA until late ERG - reduced Progression • Circumscribed atrophy of RPE and choroid • Starting in periphery • Gradual central spread • Fovea spared until late
  58. 58. Female carriers of choroideremia Central patchy atrophy and mottling of RPE Peripheral diffuse pigmentary granularity
  59. 59. Gyrate atrophy Cause - deficiency of ornithine keto-acid aminotransferase Inheritance - autosomal recessive Presents - first decade with axial myopia and nyctalopia Prognosis - usually good VA until late ERG - severely reduced Progression • Mid-peripheral, circular patches of chorioretinal atrophy • Enlargement and confluence Central and peripheral spread • Late retinal vascular attenuation • Fovea spared until late •
  60. 60. Central areolar choroidal dystrophy Inheritance - dominant Presents - fifth decade Prognosis - poor ERG - normal • • Bilateral, circumscribed, atrophic maculopathy Prominent large choroidal vessels
  61. 61. Diffuse choroidal atrophy Inheritance - dominant Presents - fourth to fifth decades Prognosis - poor ERG - reduced • • Diffuse atrophy of RPE and choriocapillaris Prominent large choroidal vessels
  62. 62. Elschnig's spots • Elschnig's spots are ischemic infarcts of the choroid and RPE that appear in the posterior pole. • Causes: – – – – – Malignant hypertension. Temporal arteritis. Sickle cell disease. Toxemia of pregnancy. Disseminated intravascular coagulopathy
  63. 63. Uveitis • Def.: inflammation of the uveal tract. • Classification: a-a- Anatomical Anatomical b- Clinical anterior intermediate posterior pan Acute symp. iritis pars planitis c- Etiological Chronic Asymp. choroditis iridocyclitis < 8 wks >3m (iris+ pars plicata ) Acute Gradual
  64. 64. Uveitis Specific Fuch’s heterochromic Non specific 25%
  65. 65. Inflammation A reaction of microcirculation ccc. By movement of fluid and cells into the extravascular tissues in response to foreign particles, micro-oganisms or antigens.
  66. 66. Inflammation Categories (by type of cells in tissue or exudates) • Acute – Polymorphonuclear leucocytes. – Mast cells and eosinophils. • Chronic – Non granulomatous • Lymphocytes and plasma cells. – Granulomatous • Epithelioid,histiocytes ± giant cells.
  67. 67. Granulomatous inflammation Three histologic patterns of granulomatous inflammation • Diffuse. • Discrete. • Zonal.
  68. 68. 1- Diffuse granulomatous inflammation • Epithelioid histiocytes are scattered throughout the involved uveal tissue. There may be an accompanying background of lymphocytes and plasma cells. • Seen in sympathetic ophthalmitis and VogtKoyanagi-Harada (VKH) disease.
  69. 69. 1- Diffuse granulomatous inflammation • Sympathetic ophthalmitis – Bilateral diffuse chronic granulomatous panuveitis – There is an exciting eye (previously traumatized) sympathizing eye. – Anterior segment findings: and • Mutton fat KPS – Posterior segment findings: • Vitreous inflammatory cells • Papillitis • Dalen-Fuchs nodules (yellow-white lesions beneath the RPE), choroidal granulomas • ERD.
  70. 70. 1- Diffuse granulomatous inflammation • Sympathetic ophthalmitis – Histologically, both the traumatized and sympathizing eyes show diffuse granulomatous inflammation made up of nests of epithelioid cells and giant cells mixed with lymphocytes. This inflammation does not extend to involve the choriocapillaris. XVKH – Dalen-Fuchs nodules are composed of: Nodular clusters of epithelioid cells lying between the RPE and Bruch's membrane .
  71. 71. 1- Diffuse granulomatous inflammation
  72. 72. 2- Discrete granulomatous inflammation • Well-circumscribed areas of epithelioid histiocytes • Seen in : Sarcoidosis: Non caseating granuloma of of the lungs, liver, lymph nodes, skin, and even the central nervous system.
  73. 73. 2- Discrete granulomatous inflammation • The classic sarcoid nodule is composed of noncaseating granulomas. These are collections of epithelioid histiocytes, sometimes accompanied by multinucleated giant cells, that have a surrounding cuff of lymphocytes. • The multinucleated giant cells may demonstrate: – Asteroid bodies • (star-shaped, acidophilic bodies) – Schaumann bodies • (spherical, basophilic, calcified bodies).
  74. 74. 3- Zonal granulomatous inflammation • A central zone of necrosis and/or polymorphonuclear leukocytes surrounded by epithelioid histiocytes which is in turn surrounded by a zone of nongranulomatous inflammation consisting of granulation tissue, lymphocytes and plasma cells. • Seen in: Phacoanaphylactic uveitis
  75. 75. Typical choroidal nevus • Common - 2% of population • Round slate-grey with indistinct margins • Surface drusen • Flat or slightly elevated • < 2 mm X 5 mm • Location - anywhere • Asymptomatic
  76. 76. Nevus cells Four types of nevus cells: • Plump polyhedral: abundant cytoplasm filled with pigment and a small, round to oval nucleus with bland appearance. • Slender spindle : cytoplasm contains scant pigment and a small, dark, elongated nucleus. • Plump fusiform dendritic: morphology is intermediate between plump polyhedral and slender spindle. • Balloon cells: abundant, foamy cytoplasm that lacks pigment and has a bland nucleus.
  77. 77. Choroidal melanoma • Most common primary intraocular tumour in adults. • Most common uveal melanoma 80% of cases • Presentation - sixth decade • More common in males , white race
  78. 78. Predisposing factors Suspicious choroidal nevus • Diameter more than 5 mm • Elevation 2 mm or more • Surface lipofuscin • Posterior margin within 3 mm of disc Oculodermal melanocytosis Nevus of Ota • May have symptoms due to serous fluid
  79. 79. Course of malignant melanoma of the choroid (knapp’s classification) 1. Quiescent stage: Tumor starts in the outer layers of choroid. ( lens –shaped) deep to Bruch’s membrane--- the membrane then raised by tumor and resists invasion----then Broken and tumor extends through hole – -collar -stud appearance which is formed of : A- Broad base in choroid. B- Neck embraced by Bruch’s membrane. C- Head internal to Bruch’s membrane.
  80. 80. Course of malignant melanoma of the choroid (knapp’s classification)
  81. 81. Course of malignant melanoma of the choroid 2. Glaucomatous stage: a) Obstruction of venous outflow. b) Pusching of iris lens dighragm forward closing the angle of Ach. c) Melanomalytic glaucoma. Angle blocked by large number of macrophages containing phagocytosed melanin pigments. d) Iris neovascularization.
  82. 82. Course of malignant melanoma of the choroid 3. Extraocular stage: a) Scleral foramina… epibular mass b) direct sclera invasion: late.. c) Along optic nerve and sheaths: not common. d) Orbit ----Proptosis
  83. 83. Course of malignant melanoma of the choroid 4. Metastasis stage: Hematogenous spread to liver, lung, bone.
  84. 84. Histological classification of uveal melanoma Spindle A cell Spindle cell (45%) Mixed cell (45%) Spindle B cell Pure epithelioid cell (5%) Necrotic (5%)
  85. 85. Malignant melanoma of choroid • Clinical picture      Asymptomatic. Photopsia or floaters. Painless visual loss. Pain….Glaucoma, uveitis. Epibulbar mass.   Unilateral / Unifocal collar button shaped pigmented or non pigmented (amelanotic) ± Exudative RD
  86. 86. Choroidal melanoma (1) • Brown, elevated, subretinal mass • Secondary retinal detachment • Occasionally amelanotic • Double circulation • Choroidal folds
  87. 87. Choroidal melanoma (2) • Surface orange pigment (lipofuscin) is common. • Mushroom-shaped if breaks through Bruch’s membrane • Ultrasound - acoustic hollowness, choroidal excavation and orbital shadowing
  88. 88. Poor Prognostic Factors of Uveal Melanomas 1. Histological • Epithelioid cells • Closed vascular loops • Lymphocytic infiltration 2. Large size 3. Extrascleral extension 4. Anterior location 5. Age over 65 years 6. Chromosomal abnormalities Cell type 5ys mortality Spindle A 5% Spindle B Necrotic and mixed Epithelioid 25% 50% 60%
  89. 89. Differential diagnosis of choroidal melanoma Large choroidal naevus Choroidal detachment Metastatic tumour Choroidal granuloma Localized choroidal haemangioma Dense sub-retinal or sub-RPE haemorrhage
  90. 90. Circumscribed choroidal haemangioma • Presentation - adult life • Dome-shaped or placoid, red-orange mass • Commonly at posterior pole • Between 3 and 9 mm in diameter • May blanch with externa globe pressure • Surface cystoid retinal degeneration • Exudative retinal detachment • Treatment - radiotherapy if vision threatened
  91. 91. Circumscribed choroidal haemangioma It is composed of cavernous spaces filled with red blood cells and is usually demarcated from normal choroid
  92. 92. Diffuse choroidal haemangioma Typically affects patients with Sturge-Weber syndrome Can be missed unless compared with normal fellow eye as shown here Diffuse thickening, most marked at posterior pole
  93. 93. Choroidal metastatic carcinoma Most frequent primary site is breast in women and bronchus in both sexes • Fast-growing, creamy-white, placoid lesion • Most frequently at posterior pole • Deposits may be multiple • Bilateral in 10-30%
  94. 94. Choroidal osseous choristoma Compact bone contain osteocytes, and intratrabecular spaces are filled with loose connective tissue containing large and small blood vessels. • Very rare, benign, slowgrowing ossifying tumor. • Typically affects young women • Orange-yellow, oval lesion. • Well-defined, scalloped, geographical borders. • Most commonly peripapillary or at posterior pole. • Diffuse mottling of RPE. • Bilateral in 25%.
  95. 95. Melanocytoma (Magnocellular nevus) • Affects dark skinned individuals • Usually asymptomatic • Most frequently affects optic nerve head (inferotemporal) • Black lesion with feathery edges. • Deeply pigmented polyhedral cells with a small nuclei.
  96. 96. Choroidal ruptures • Blunt trauma. • Semicircular lines circumscribing the optic nerve head in the peripapillary region. • Sub retinal NV can occur as a late complication. • More severe injury may cause rupture of both the choroid and the retina, a condition termed chorioretinitis sclopetaria.
  97. 97. Choroidal detachment • - Associated with hypotony. • - Unilateral, brown, smooth, solid and immobile.
  98. 98. Heterochromia iridis Different colours of the two irides
  99. 99. THANK YOU

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