- •Contents
- •Preface
- •Abbreviations
- •Introduction
- •Orbit and external eye
- •Extraocular muscles
- •Anterior segment
- •Posterior segment
- •Introduction
- •Ocular examination
- •The ‘red reflex’
- •Assessment of vision and visual acuity
- •Cover test for strabismus
- •Light reflex testing
- •Color vision testing
- •Assessment of stereoacuity
- •Ophthalmoscopy
- •Vision screening
- •Photoscreening
- •Autorefraction
- •Visual evoked potential
- •Strabismus
- •Comitant strabismus
- •Congenital esotropia
- •Accommodative esotropia
- •Congenital exotropia
- •Intermittent exotropia
- •Incomitant strabismus
- •Third cranial nerve palsy
- •Fourth nerve palsy
- •Sixth nerve palsy
- •Strabismus syndromes
- •Duane’s syndrome
- •Brown’s syndrome
- •Monocular elevation deficiency (MED)
- •Möbius syndrome
- •Introduction
- •Conjunctivitis
- •Bacterial conjunctivitis
- •Viral conjunctivitis
- •Herpes conjunctivitis
- •Giant papillary conjunctivitis
- •Allergic conjunctivitis
- •Vernal keratoconjunctivitis
- •Phlyctenular keratoconjunctivitis (phlyctenulosis)
- •Ophthalmia neonatorum
- •Introduction
- •Congenital corneal opacity
- •Embryology
- •Peters anomaly
- •Sclerocornea
- •Congenital dermoid
- •Birth trauma
- •Congenital hereditary endothelial dystrophy
- •Congenital hereditary stromal dystrophy
- •Posterior polymorphous membrane dystrophy
- •Metabolic diseases
- •Mucopolysaccharidosis
- •Hurler’s syndrome (MPS I-H)
- •Scheie’s syndrome (MPS I-S)
- •Hunter’s syndrome (MPS II)
- •Sly’s syndrome (MPS VII)
- •Mucolipidosis
- •Sialidosis (ML I)
- •I-Cell disease (ML II)
- •Pseudo-Hurler dystrophy (ML III)
- •Miscellaneous metabolic diseases
- •Fabry’s disease
- •Cystinosis
- •Tyrosinemia
- •Infectious diseases
- •Herpes simplex virus (HSV)
- •Congenital syphilis
- •Rubella
- •Introduction
- •Structural lens abnormalities
- •Aphakia
- •Spherophakia (microspherophakia)
- •Coloboma
- •Subluxation (ectopia lentis)
- •Lenticonus
- •Persistant fetal vasculature
- •Cataracts
- •Nuclear cataracts
- •Lamellar cataracts
- •Anterior polar cataracts
- •Posterior polar cataracts
- •Sutural cataracts
- •Anterior subcapsular cataracts
- •Posterior subcapsular cataracts
- •Cerulean (blue-dot) cataracts
- •Complete cataracts
- •Etiology of cataracts
- •Genetic and metabolic diseases
- •Trauma
- •Medication and toxicity
- •Maternal infection
- •Diagnosis of cataracts
- •Management/treatment of cataracts
- •Visual significance
- •Surgery
- •Aphakia
- •Pseudophakia
- •Amblyopia
- •Cataract prognosis
- •Introduction
- •Diagnosis of pediatric glaucoma
- •Ocular examination
- •Differential diagnosis of pediatric glaucoma
- •Primary infantile glaucoma
- •Juvenile open-angle glaucoma
- •Primary pediatric glaucoma associated with systemic disease
- •Lowe’s syndrome
- •Sturge–Weber syndrome
- •Neurofibromatosis
- •Axenfeld–Rieger syndrome
- •Aniridia
- •Peters anomaly
- •Secondary childhood glaucoma
- •Trauma
- •Neoplasia
- •Glaucoma following pediatric cataract surgery
- •Other causes of secondary glaucoma in children
- •Treatment of pediatric glaucoma
- •Drug treatment
- •Surgical management
- •Summary
- •Introduction
- •Coats’ disease
- •Leber’s congenital amaurosis
- •X-linked congenital stationary night blindness
- •Achromatopsia
- •Stargardt disease
- •Best’s disease
- •Persistent fetal vasculature
- •X-linked juvenile retinoschisis
- •Albinism
- •Retinal dystrophies with systemic disorders (ciliopathies)
- •Introduction
- •Common clinical features
- •Classification
- •Anterior uveitis
- •Juvenile idiopathic arthritis
- •Juvenile spondyloarthropathies
- •Sarcoidosis
- •Herpetic iridocyclitis
- •Intermediate uveitis
- •Posterior uveitis
- •Toxoplasmosis
- •Toxocariasis
- •Vogt–Koyanagi–Harada syndrome
- •Sympathetic ophthalmia
- •Masquerade syndromes
- •Retinoblastoma
- •Leukemia
- •Introduction
- •Optic nerve hypoplasia
- •Morning glory disc anomaly
- •Optic disc coloboma
- •Peripapillary staphyloma
- •Congenital tilted disc
- •Optic pit
- •Myelinated retinal nerve fibers
- •Papilledema
- •Pseudopapilledema
- •Optic disc drusen
- •Introduction
- •Dacryocele
- •Nasolacrimal duct obstruction
- •Lacrimal sac fistula
- •Decreased tear production
- •Dacryoadenitis
- •Introduction
- •Cryptophthalmos and ankyloblepharon
- •Coloboma of the eyelid
- •Blepharoptosis
- •Epicanthal folds and euryblepharon
- •Lagophthalmos
- •Lid retraction
- •Ectropion, entropion, and epiblepharon
- •Blepharospasm
- •Blepharitis
- •Hordeolum
- •Chalazion
- •Tumors of the eyelid
- •Preseptal and orbital cellulitis
- •Herpes simplex, molluscum contagiosum, and verruca vulgaris
- •Allergic conjunctivitis
- •Trauma
- •Summary
- •Introduction
- •Cystinosis
- •Marfan’s syndrome
- •Homocystinuria
- •Wilson’s disease
- •Fabry disease
- •Osteogenesis imperfecta
- •The mucopolysaccharidoses
- •Sickle cell disease
- •Albinism
- •Congenital rubella
- •Introduction
- •Genetics
- •Malignant potential
- •Formes frustes
- •Neurofibromatosis (von Recklinghausen’s syndrome)
- •Retinocerebellar hemangioblastomatosis (von Hippel–Lindau syndrome)
- •Racemose hemangiomatosis (Wyburn-Mason syndrome)
- •Encephalofacial cavernous hemangiomatosis (Sturge–Weber syndrome)
- •Oculoneurocutaneous cavernous hemangiomatosis
- •Organoid nevus syndrome
- •Introduction
- •Cortical visual impairment
- •Migraine headache
- •Spasmus nutans
- •Opsoclonus
- •Horner’s syndrome
- •Congenital ocular motor apraxia
- •Myasthenia gravis
- •Introduction
- •Eyelid and conjunctiva
- •Intraocular tumors
- •Orbital tumors
- •Diagnostic approaches
- •Eyelid and conjunctiva
- •Intraocular tumors
- •Orbital tumors
- •Therapeutic approaches
- •Eyelid and conjunctiva
- •Intraocular tumors
- •Orbital tumors
- •Eyelid tumors
- •Capillary hemangioma
- •Facial nevus flammeus
- •Kaposi’s sarcoma
- •Basal cell carcinoma
- •Melanocytic nevus
- •Neurofibroma
- •Neurilemoma (schwannoma)
- •Conjunctival tumors
- •Introduction
- •Choristomatous conjunctival tumors
- •Epithelial conjunctival tumors
- •Melanocytic conjunctival tumors
- •Vascular conjunctival tumors
- •Xanthomatous conjunctival tumors
- •Lymphoid/leukemic conjunctival tumors
- •Non-neoplastic lesions that simulate conjunctival tumors
- •Conclusions
- •Intraocular tumors
- •Retinoblastoma
- •Retinal capillary hemangioma
- •Retinal cavernous hemangioma
- •Retinal racemose hemangioma
- •Astrocytic hamartoma of the retina
- •Melanocytoma of the optic nerve
- •Intraocular medulloepithelioma
- •Choroidal hemangioma
- •Choroidal osteoma
- •Uveal nevus
- •Uveal melanoma
- •Congenital hypertrophy of retinal pigment epithelium
- •Leukemia
- •Orbital tumors
- •Dermoid cyst
- •Teratoma
- •Capillary hemangioma
- •Lymphangioma
- •Juvenile pilocytic astrocytoma
- •Rhabdomyosarcoma
- •Granulocytic sarcoma (‘chloroma’)
- •Lymphoma
- •Langerhan’s cell histiocytosis
- •Metastatic neuroblastoma
- •Introduction
- •Eyelid
- •Open globe
- •Ocular surface injury
- •Intraocular trauma
- •Iridodialysis
- •Cataract
- •Retina
- •Optic nerve injury
- •Orbital fracture
- •Other orbital injury
- •Child abuse
- •Shaking injury
- •Index
78 CHAPTER 6 Conjunctiva
Allergic conjunctivitis
DEFINITION/OVERVIEW
Allergic conjunctivitis can be divided into five common forms, two acute ( seasonal allergic conjunctivitis [SAC] and perennial allergic conjunctivitis [PAC]), and three chronic (vernal keratoconjunctivitis [VKC], atopic keratoconjunctivitis [AKC], and giant papillary conjunctivitis [GPC]) (Table 9).27 AKC is usually seen in late adolescents and adults, so will not be discussed here.
‘If it doesn’t itch, it’s probably not allergy’ is probably the best way to start thinking about allergy as the etiology of an inflamed eye.
ETIOLOGY
Allergic conjunctivitis is usually associated with type 1 hypersensitivity reactions. The main component of ocular allergy is the mast cell along with IgE. When an allergen comes in contact with the conjunctiva of a previously sensitized patient, the mast cell, which is filled with granules of histamine, will degranulate, spilling the contents into the surrounding tissue. The release of histamine causes a cascading release of chemical mediators, which causes vasodilatation, increased vascular permeability, and increased mucoid
production. The main mediators in mast cell degranulation are histamine, tryptase, kininogenase, and eosinophil chemotactic factor of anaphylaxis (ECF-A). If the allergic reaction has a long protracted course, a latephase response can occur. The main mediators of this late-phase response includes leukotriene B4, leukotriene C4, D4, prostaglandins D2, and platelet activation factor.28
Seasonal conjunctivitis is the most common form of ocular allergic disease.26 The ocular manifestations occur either seasonally (SAC) or perennially (PAC). Although the symptoms, including rhinitis and congestion, can be treated quickly by primary care providers, the ocular complaints may not be elucidated during the examination. The symptomatic patient is usually miserable and treatment of their systemic symptoms as well as their ocular symptoms is beneficial. SAC is associated with release of pollens in the early spring from grasses and trees, or in the late summer and early fall secondary to ragweed. PAC can occur at any time throughout the year. This is usually associated with allergens such as pet dander and dust mites. Once an individual is exposed to a sensitizing agent, the symptoms of the allergic response occur quickly. Although PAC sufferers have symptoms year round, they will usually experience seasonal exacerbations.29,30
Table 9 Signs and symptoms of allergic conjunctivitis |
|
|
|
Seasonal |
|
|
|
• |
Bilateral |
• |
Chemosis |
• |
Itching |
• |
Ropey or stringy mucoid discharge |
•Tearing
•Conjunctival injection
Vernal
• |
Severe itching |
• |
Trantas’ dots |
• |
Ropey mucoid discharge |
• |
Giant papillae |
• |
Blurred vision |
|
|
Giant papillary |
|
|
|
• |
Itching |
• |
Giant papillae |
• |
Contact lens intolerance |
• |
Mucoid discharge |
• |
Mild hyperemia |
|
|
|
|
|
|
Allergic conjunctivitis 79
CLINICAL PRESENTATION
Although acute allergic conjunctivitis is usually seen bilaterally, it is not uncommon for a patient to complain of unilateral symptoms, even when bilateral signs are present. Besides the itching, patients will often complain of a stringy or ropey mucoid discharge, lid edema, chemosis (edema of the conjunctiva), and red, hyperemic conjunctiva (81).31 Patients will often show other signs of atopy, with allergic rhinitis being extremely common. Patients with atopic disease will sometimes not have a primary complaint of itching eyes, but instead other symptoms of allergic reactions including wheezing, rhinoconjunctivitis, and eczema.32
MANAGEMENT/TREATMENT
The treatment of acute allergic conjunctivitis centers around symptomatic relief. By reducing the allergic response to the allergen and reducing the inflammatory response of the eye and periorbital region, the patient will be much more comfortable and less symptomatic.33 The most important goal is to reduce the amount of allergic load to the patient. If the child is suffering from PAC, then eliminating the allergen will be the most beneficial approach. Eliminating pet dander or laundry detergent, or reducing contact with dust mites can show significant relief of the patient’s symptoms. If the allergic response is caused by seasonal allergens, this is not an easy task. SAC will also benefit from any reduction in the allergen load, including keeping windows closed and using an air filtration system in the house. When the child is outside exposed to various pollens and other allergens, these allergens will collect in his or her hair, which can be transferred to the pillow at night, putting a significant allergen load in direct contact with the ocular adnexa. By simply washing the child’s hair before they go to bed, the allergen load will not be transferred to the pillow and thus the eyes. Cool or cold compresses are also beneficial for symptomatic relief. The use of topical ophthalmic drops can also help to lower the allergic response, and the patient’s symptoms. These medications are divided into five categories: antihistamines, mast cell stabilizers, antihistamine/mast cell stabilizer combinations, corticosteroids, and nonsteroidal anti-inflammatory drugs (NSAIDs).
PROGNOSIS
The treatment of SAC is often frustrating for both the physician and patient. Recurrences are common and complete resolution of symptoms without the use, and risk, of steroids is not always possible. However, many patients can be helped significantly. Patients will show a definite increase in the quality of life when their symptoms are controlled.34 Patients with chronic disease can show permanent changes and potential visual impairment.
81
81 Conjunctival chemosis.(Courtesy of Casey Eye Institute,Oregon Health Sciences University.)
80 CHAPTER 6 Conjunctiva
Vernal keratoconjunctivitis
ETIOLOGY
VKC is caused by the exuberant reaction of the body’s immune system towards an allergen. VKC is thought to be mainly a type I hypersensitivity reaction, with eosinophils, as well as mast cells the most involved cells. There has also been some speculation that a type IV hypersensitivity reaction may also play a role in this condition, with mononuclear cells, fibroblasts, and newly secreted collagen seen.35
VKC (82). The superior tarsal conjunctiva will often show large ‘cobblestone-like’ papillae (83). In severe and chronic cases of VKC, sterile corneal ulcers, known as corneal shield (Togby’s) ulcers, may be present (84). They are usually seen at the superior limbus, and can be painful, serious vision-threatening lesions. These ulcers need to be aggressively managed if they do not show rapid re-epithelialization.38 Other symptoms seen in VKC are foreign body sensation, epiphora or excess tearing, photophobia, and ocular burning and stinging (Table 10).
MANAGEMENT/TREATMENT
CLINICAL PRESENTATION
VKC is a chronic form of allergic conjunctivitis. It is bilateral and will usually be seen affecting the superior and limbal conjunctivae. There is a definite predilection for VKC affecting males more than females,36 and it is seen in warmer climates more often than cooler climates. It usually presents before age 10 years, and will often resolve following puberty. There is also a history of eczema or asthma in 75% of patients.37 Although this is a chronic condition, seasonal exacerbations are often seen. The symptoms that are reported are similar to those seen in acute allergic conjunctivitis but are usually more severe and chronic. The primary complaints are severe itching, and a thick, ropey discharge. The patient will usually have a history of allergies and atopy. Along with conjunctival injection, small whitish dots on the cornea at the superior limbus will often be visible with a direct ophthalmoscope. These dots, Trantas or Horner–Trantas dots, are pathognomonic for
Treatment is centered on reducing the patient’s symptoms, controlling the hypersensitivity reaction, and keeping the ocular structures well lubricated to prevent the serious vision-threatening sequelae that can be seen with VKC. The initiation of therapy as early as possible to prevent these sequelae is highly recommended once the diagnosis of VKC is made. The use of cool or cold compresses, artificial tears, as well as bland ophthalmic ointments can help reduce the early symptoms. Systemic as well as topical antihistamines, along with mast cell stabilizers can keep the symptoms under control, but play a less prominent role during an acute exacerbation. The use of NSAIDs as well as topical steroidal anti-inflammatory drops will usually be required during an acute exacerbation.
In patients that have corneal shield ulcers, aggressive therapy is indicated. Since these ulcers are painful, the use of a cycloplegic drop is indicated and will reduce the ciliary spasm and
Table 10 Signs and symptoms of vernal keratoconjunctivitis |
|
|
|
Signs |
Symptoms |
||
• |
Bilateral |
• |
Red injected conjunctiva |
• |
Conjunctival injection |
• |
Severe ocular itching |
• |
Giant cobblestone-like papillae seen on the |
• |
Epiphora (tearing) |
|
superior tarsal conjunctiva (usually larger than those |
• |
Mucoid discharge |
|
seen in giant papillary conjunctivitis) |
• |
Ocular burning |
• |
Superior limbal conjunctivitis |
• |
Photophobia |
•Trantas’ or Horner–Trantas dots
•Corneal shield (Togby’s) ulcers
Vernal keratoconjunctivitis 81
pain that the child may experience. If the ulcer does not rapidly re-epithelialize, the use of topical antibiotics is also indicated. A physical barrier with a low water content hydrogel lens can be used to stop the cobblestone-like giant papillae from re-abrading the corneal epithelium. If a patient is showing these significant symptoms during an acute exacerbation, consultation with an ophthalmologist is highly recommended.
82
PROGNOSIS
VKC is often a chronic and recurring problem. Mild cases can be effectively managed and patients can be made comfortable. More severe cases place the patient at significant risk for vision loss, usually as a result of the disease process itself. However, the overly aggressive use of topical corticosteroids to treat the disease may also cause problems.
83
82 Horner–Trantas dots.(Courtesy of Casey Eye Institute,Oregon Health Sciences University.)
83 Tarsal cobblestone papillae.(Courtesy of Casey Eye Institute,Oregon Health Sciences University.)
84
84 Shield ulcer.(Courtesy of Casey Eye Institute,Oregon Health Sciences University.)
