- •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
128 CHAPTER 9 Glaucoma
Treatment of pediatric glaucoma
Traditional teaching dictated that childhood glaucoma was a surgical disease. Advances in the pharmacologic management of glaucoma have changed this dictum to some degree. However, surgery is still the primary form of treatment for many types of glaucoma that occur in children.
Angle surgery remains the first line of therapy in primary infantile glaucoma. Medical therapy is often tried first in cases of juvenile open-angle glaucoma, aphakic glaucoma, and in the treatment of secondary glaucoma. However, the implications of lifelong medical therapy (with attendant side-effects) including cost and compliance are significant and need to be considered carefully in choosing therapeutic options. Many promising drugs are now available and approved for use in children, but the long-term side-effects remain unknown. As the routine use of the miotic drugs (i.e. pilocarpine) has been largely supplanted in the treatment of glaucoma, there remain four classes of drug with which the primary care provider should be familiar in the care and treatment of pediatric patients. All four classes of these drugs are used on both a shortand long-term basis.
Drug treatment
CARBONIC ANHYDRASE INHIBITORS
The oral carbonic anhydrase inhibitor acetazolamide has been used safely and effectively for over 50 years in the treatment of pediatric glaucoma. It acts as a suppressant of aqueous humor production. The pressurelowering achieved can be upwards of 30–40% and can in many cases clear the cornea preoperatively to allow safe angle surgery. Sideeffects include decreased appetite, diarrhea, and metabolic acidosis. The latter can be problematic in infants under 12 months of age. Topical carbonic anhydrase inhibitors are now available. Brinzolamide and dorzolamide are widely used in both adult and pediatric patients. They are utilized mostly in an adjunctive role with other topical medication. All carbonic anhydrase inhibitors should be avoided in patients with sulfa allergy.
BETA BLOCKERS
The topical beta blockers have an important role in the treatment of pediatric glaucoma. The mode of action is suppression of aqueous humor production. As in adults, the side-effects include fatigue, lethargy, bradycardia, apnea, and asthma exacerbation. The initial dose may produce an adequate therapeutic effect in many cases and may obviate further topical or surgical therapy for the patient.
ADRENERGIC AGONISTS
Apraclonidine and brimonidine are both approved for the topical therapy of glaucoma in adult patients. The mechanism of action has a dual effect, producing both suppression of aqueous humor production and an increase in uveoscleral outflow. The use of apraclonidine in adults has been limited by the high incidence of topical allergy, which approached 15% in some studies. It is not used routinely to any degree in the treatment of pediatric glaucoma. Topical brimonidine has produced lethargy and somnolence in toddlers and apnea, bradycardia, and hypotension in infants. The use of brimonidine in the treatment of childhood glaucoma should therefore be restricted to older children. It should not be considered first-line therapy and the very real possibility of life-threatening side-effects should be discussed with the patient’s parents prior to the initiation of treatment in all cases.
PROSTAGLANDIN ANALOGS
The prostaglandin analogs include latanaprost, bimatoprost, and travoprost. This class of drugs works by enhancing uveoscleral outflow and can produce dramatic reductions in IOP, approaching 25–30% in many cases. They are dosed once-daily, thereby improving therapeutic compliance. Systemic side-effects are minimal, but excessive eyelash growth and darkening of the iris are frequently noted. The prostaglandin drugs should be used with caution where intraocular inflammation coexists with glaucoma, due to the risk of increasing the inflammatory response.
Treatment of pediatric glaucoma 129
Surgical management
Goniotomy was introduced by Barkan in the 1940s and revolutionized the therapy for primary infantile glaucoma, with cure rates today approaching 80% in experienced hands (162–164). A special lens is placed on the cornea which allows visualization of the angle structures. A knife is passed across the anterior
162
162 Goniotomy demonstrating incision of Barkan’s membrane in a counter-clockwise direction. Modern technique generally includes the use of a viscoelastic agent such as Healon.
chamber and an incision is made into the abnormal tissue blocking fluid passage out of the eye. A relatively clear cornea is needed to perform a goniotomy.
Trabeculotomy is an alternative form of angle surgery in which the canal of Schlemm is cannulated externally with the trabeculotome (or suture material). This is then utilized to tear
163
163 Severely photophobic patient with bilateral corneal opacification.IOP was 35 mmHg right eye and 37 mmHg left eye.Angle surgery was required.
164
164 Patient in Figure 163 1 year following bilateral goniotomy.Corneas are clear and photophobia has resolved.After 14 years of follow-up IOP remains controlled in the right eye with topical lumigan.Vision is 20/25 with correction.Vision in the left eye is poor due to central corneal scarring from Haab’s striae.
