OCULAR PATHOLOGY

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CHAPTER 18 OCULAR PATHOLOGY

Eyelid

Infections
Inflammation
Tumors
Lacrimal drainage system

Dacryocystitis
Canaliculitis
Lacrimal gland
Conjunctiva

Inflammation
Degenerative changes
Tumors
Cornea

Infections
Inflammation
Degenerative
Corneal dystrophies
Metabolic disease involving the cornea
Keratoconus
Corneal dermoids
Pseudophakic bullous keratopathy
Corneal graft rejection and failure
Developmental anomalies

Anterior segmental developmental anomalies
Persistent hyperplastic primary vitreous
Microphthalmia
Orbital pathology

Orbital pseudotumor
Optic nerve pathology

Optic nerve glioma
Meningioma
Intraocular tumors

Non-retinoblastoma intraocular ‘tumors’
Retinoblastoma
Miscellaneous ocular disorders

Epithelial down-growth
Endophthalmitis / panophthalmitis
Phthisis bulbi
Irido-corneal endothelial syndrome

INTRODUCTION

• Two broad groups of surgical pathology are specific to pediatric practice

image developmental anomalies
image tumors

• Most of the remaining pathology is either not specific to children or covered elsewhere in the book

image little detail is given here

EYELID

INFECTIONS

Bacterial

• External hordeolum/stye

image purulent infection of hair follicle of the eyelid
image spreads to adjacent follicles and develops an abscess

• Internal hordeolum

image purulent infection of Meibomian gland

– associated with bacterial infection and obstruction of duct

• Necrotizing fasciitis

image group A Streptococcus with Staphylococcus
image purulent exudate and necrosis
image bacteria

• Granulomatous

image rare in this site

Viral

• Warts
• Molluscum contagiosum

image well circumscribed nodule of epithelial cells
image viral inclusions

INFLAMMATION

Chalazion (Fig 18.1)

• Lipogranuloma
• Obstruction of the Meibomian gland duct
• Free fat surrounded by granulomatous inflammation
image

Fig 18.1 Photomicrograph of a chalazion showing a lipogranuloma consisting of dense active chronic inflammation and epithelioid histiocytes surrounding a lipid vacuole.

TUMORS

Cysts

• Sudoriferous cysts (Fig 18.2)

image translucent cysts
image double layered wall

– cuboidal inner cells
– external myoepithelial cells

• Epidermoid/dermoid
image

Fig 18.2 Photomicrograph of a sudiferous cyst. The cyst has a two-layer wall.

Xanthelasma

• Typically adult
• Foamy cells in dermis

Nevi

• Junctional
• Compound
• Intradermal

Hamartomas

• Cavernoma
• Lymphangioma
• Neurofibroma

Epithelial tumors

• Squamous cell papilloma

image warty lesion
image papillomatous
image squamous cells

• Other epithelial tumors common in adults are rare in childhood

Soft tissue tumors

• Rhabdomyosarcoma

LACRIMAL DRAINAGE SYSTEM

DACRYOCYSTITIS (Fig 18.3)

• Inflammation of the lacrimal sac

image associated with obstruction
image acute or chronic inflammation

• Mucocele
• Dacryolith
image

Fig 18.3 Photomicrograph from a chronic dacryocystitis. The wall of the lacrimal sac is infiltrated by dense chronic inflammation.

CANALICULITIS

• Epiphora
• Conjunctivitis
• Several organisms

image most common is Actinomyces

LACRIMAL GLAND

• Biopsy of lacrimal gland pathology is unusual in pediatric practice
• Inflammatory lesions

image dacryoadenitis
image sarcoidosis

• Tumors

image see orbital tumors
image pleomorphic adenoma may arise at this site but is predominantly a disease of adulthood

CONJUNCTIVA

INFLAMMATION

Non-granulomatous conjunctivitis

• Two patterns:

image Papillary

– subepithelial chronic inflammation forming papillae due to tethering of the epithelium by fibrous tissue

· lymphocytes
· plasma cells
· eosinophils

– central vessels
– causes

· bacteria
· allergy (vernal conjunctivitis)
· foreign body (contact lenses)
· chronic blepharitis

image Follicular (Fig 18.4)

– lymphoid follicles/aggregates
– surrounded by vessels
– causes

· viruses (Adenovirus, Molluscum, HSV)
· Chlamydia
· hypersensitivity
· topical medication

image

Fig 18.4 Photomicrograph showing follicular conjunctivitis.

Trachoma

• Chlamydia trachomatis infection
• Immunotypes A–K
• Diagnosis by scrapings
• Follicular conjunctivitis

image later papillary phase

Ophthalmia neonatorum

• Infection by Chlamydia acquired in the birth canal
• Papillary conjunctivitis

Keratoconjunctivitis sicca

• Inadequate tear film – ‘dry eyes’
• May occur in association with juvenile rheumatoid arthritis
• Conjunctiva may undergo squamous metaplasia

Ocular cicatricial pemphigoid

• Rare in childhood

image usually associated with age

• Irritation/burning sensation
• Scarring
• Dry eyes
• Biopsy

image diagnosis depends on staining for immunoglobulins (esp. IgG) on the epithelial basement membrane
image subepithelial bullae
image chronic inflammation in the stroma

Granulomatous conjunctivitis

• Sarcoid

DEGENERATIVE CHANGES

Pinguecula / pterygium

• Unusual in childhood

image but described occasionally

• Fibrovascular tissue showing elastosis
• Pinguecula

image nodule on the conjunctiva only

• Pterygium

image localized to limbus and extending across the cornea

Pseudopterygium

• Adhesion between conjunctiva and cornea following corneal damage
• Fibrovascular tissue lacking elastosis

TUMORS

Epithelial inclusion cysts (Fig 18.5)

• Benign epithelial cysts
• Lined by conjunctival epithelium
• May undergo squamous metaplasia
image

Fig 18.5 Photomicrograph showing an epithelial inclusion cyst taken from the eyelid.

Pyogenic granuloma

• Relatively common surgical specimen from this site
• Histological features as seen elsewhere

Hamartoma

• Lymphatic cyst / lymphangiectasia
• Capillary hemangioma
• Dermoids (Fig 18.6)

image solid

– mixtures of mesenchymal elements

image cystic

– epidermal and adnexal structures

image

Fig 18.6 Photomicrograph showing a conjunctival dermoid. There is extensive adipose tissue and fibrous tissue together with a few small glands.

Amyloid

• Conjunctival nodules
• Associated with systemic amyloid or local inflammation

Melanocytic tumors (Figs 18.7, 18.8)

• Conjunctival nevi

image junctional
image compound
image intra-stromal
image often associated with secondary epithelial cysts

• Melanoma and primary acquired melanosis are predominantly diseases of adulthood
• Melanosis may be seen as a congenital phenomenon

image oculodermal melanocytosis (nevus of Ota)

– associated dermal nevus

image malignant transformation is rare

image

Fig 18.7 Photomicrograph showing a conjunctival junctional nevus. There are multiple nests of melanocytes.

image

Fig 18.8 Photomicrograph showing an epithelial cyst within a conjunctival nevus.

Epithelial tumors

• Papilloma

image two patterns

– placode/sessile
– pedunculated/papillary

• Premalignant/malignant tumors rare in children

Lymphoma

• Predominantly a disease of adulthood but childhood cases described
• Mostly MALT type

CORNEA

INFECTIONS

Bacterial keratitis

• Neutrophils in the cornea
• Outcomes

image healing
image descemetocele

– epithelial down growth on to the remaining Descemet’s membrane

· more frequent in viral infections

image perforation

– endophthalmitis

Crystalline keratopathy

• Bacterial infection in the context of topical steroids
• Clumps of bacteria
• Lacks a significant vascular or inflammatory response

Fungal keratitis

• Immunocompromise
• Purulent inflammation
• Fungal elements

Acanthameba

• Associated with contact lens use with contaminated disinfection procedures
• Atrophic epithelium
• Reduction in visible keratocytes in the stroma
• Parasitic cysts in stroma

Virus

• HSV

image early stages

– epithelial inclusions

image later stages

– inflammation
– necrosis
– neovascularization
– lipid keratopathy

· granulomatous reaction
· cholesterol clefts
· not specific

• VZV

image similar to HSV

Syphilis

• Interstitial keratitis

image stroma chronic inflammation

• Ghost vessels

image neovascularization of the stroma lacking circulation

INFLAMMATION

• Wegener’s granulomatosis and rheumatoid arthritis can cause corneal ulceration

DEGENERATIVE

Band keratopathy (Fig 18.9)

• Spotty deposition of calcium in Bowman’s membrane
• Causes include

image uveitis
image sarcoid
image topical medication
image hyperparathyroidism/hypercalcemia

image

Fig 18.9 Photomicrograph showing a band keratopathy with fine speckled calcification of Bowman’s membrane.

Spheroidal degeneration

• Deposits of spheroidal structures in superficial stroma

image contain keratin

• As a primary disease in excess UV exposure
• As a secondary phenomenon in inflammatory disease

Amyloid

• May occur in scarred cornea
• See also ‘Lattice dystrophy’ below

Lipid keratopathies

• May occur as secondary phenomena to neovascularization in inflammatory disease
• Crystalline stromal dystrophy (Schneider’s dystrophy)

image see below

Blood staining

• Following bleeding into the anterior chamber (hyphema)
• Blood products seep into the corneal stroma

CORNEAL DYSTROPHIES

• A range of bilateral, hereditary, progressive disorders in most cases limited to the eyes (Klintworth 2009, Poulaki & Colby 2007)
• Variable corneal opacity
• Classified according to the major anatomical layer affected
• Clinical features important in the diagnosis in many cases
• Fuchs’ dystrophy commonest in adults

image does not affect children

• Important considerations in pediatric histopathological practice

image lattice dystrophy
image macular dystrophy
image granular dystrophy
image posterior polymorphous corneal dystrophy
image congenital hereditary endothelial corneal dystrophy (CHED)

Epithelial and superficial dystrophies

Corneal epithelial membrane dystrophy (map dot corneal dystrophy, MDCD)

• Often asymptomatic
• Presentation typically in adults
• Recurrent corneal erosion
• Redundant basement membrane
• Intraepithelial cysts
• Usually not genetic
• Mutations in βig-H3 gene (transforming growth factor inducing gene/TGFBI) described

Meesmann’s epithelial corneal dystrophy

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