Causes of Childhood Glaucoma

Glaucoma in the pediatric age group can be divided into roughly 3 subtypes: primary infantile (22%), those with associated systemic conditions (46%), and secondary pediatric glaucoma (32%). Examples of the secondary group are uveitic, post infantile cataract surgery, tumor-related (e.g. retinoblastoma), and angle closure (e.g. retinopathy of prematurity, PHPV). The associated pediatric glaucoma group includes such conditions as Sturge-Weber syndrome, neurofibromatosis (NF-1), Axenfeld-Rieger spectrum, and many other disorders with systemic involvement (Table1).

Primary congenital glaucoma

Patient with Sturge-Weber

Developmental Glaucomas 1. Primary congenital glaucoma (PCG)    W Newborn primary congenital glaucoma    W Infantile primary congenital glaucoma    W Late-recognized primary congenital       glaucoma 2. Juvenile open-angle glaucoma (JOAG) 3. Primary glaucomas associated with systemic     diseases    W 8q23.3 deletion S    W 9p deletion syndrome    W Aicardi-Goutieres syndrome    W Androgen insensitivity, pyloric stenosis    W Brachmann-deLange syndrome    W Caudal regression syndrome    W Cranio-cerebello-cardiac (3C) syndrome    W Cutis marmorata telangiectatica congenita    W Diabetes mellitus, polycystic kidneys,       hepatic fibrosis, hypothyroidism    W Epidermal Nevus syndrome (Solomon S)    W Fetal hydantoin syndrome    W GAPO syndrome    W Glaucoma with microcornea and absent       sinuses    W Hepatocerebrorenal syndrome (Zellweger)    W Infantile glaucoma with retardation and        paralysis    W Kniest syndrome (skeletal dysplasia)    W Linear scleroderma    W Marfan syndrome    W Michel's syndrome    W Moyamoya S    W Mucopolysaccharidosis    W Nail-patella syndrome    W Neurofibromatosis (NF-1)    W Nevoid basal cell carcinoma S.(Gorlin S)    W Nonprogressive hemiatrophy    W Oculocerebrorenal syndrome (Lowe)    W Oculodentodigital dysplasia    W PHACE syndrome    W Phakomatosis pigmentovascularis (PPV)    W Proteus syndrome    W Rieger syndrome    W Roberts' pseudothalidomide syndrome    W Robinow syndrome    W Rothmund-Thomson syndrome    W Rubinstein-Taybi syndrome    W SHORT syndrome    W Soto syndrome    W Stickler syndrome    W Sturge-Weber syndrome    W Trisomy 13    W Trisomy 21 (Down syndrome)    W Warburg syndrome    W Wolf-Hirschhorn (4p-) syndrome 4. Primary glaucomas with associated ocular     anomalies    W Aniridia      o congenital aniridic glaucoma      o acquired aniridic glaucoma    W Axenfeld- Rieger anomaly    W Congenital anterior (corneal) staphyloma    W Congenital hereditary endothelial       dystrophy    W Congenital iris ectropion syndrome    W Congenital microcoria    W Congenital ocular melanosis    W Idiopathic or familial elevated venous       pressure    W Iridotrabecular dysgenesis       (iris hypoplasia)    W Peters' syndrome    W Posterior polymorphous dystrophy    W Sclerocornea

Secondary (Acquired) Glaucomas 1. Traumatic glaucoma    W Acute glaucoma      o Angle concussion      o Hyphema      o Ghost cell glaucoma    W Glaucoma related to angle-recession    W Arteriovenous fistula 2. Glaucoma with intraocular neoplasms    W Aggressive iris nevi    W Juvenile xanthogranuloma (JXG)    W Iris rhabdomyosarcoma    W Leukemia    W Medulloepithelioma    W Melanocytoma    W Melanoma of ciliary body    W Mucogenic glaucoma with iris stromal cyst    W Retinoblastoma 3. Glaucoma related to chronic uveitis    W Angle-blockage mechanisms      o Synechial angle closure      o Iris bombe with pupillary block    W Open-angle glaucoma    W Trabecular meshwork endothelialization 4. Lens-related glaucoma    W Phacolytic glaucoma    W Spherophakia with pupillary block    W Subluxation-dislocation with pupillary block      o Axial-subluxation high-myopia syndrome      o Ectopia lentis et pupillae      o Homocystinuria      o Marfan syndrome      o Weill-Marchesani syndrome 5. Glaucoma following lensectomy for     congenital cataracts    W Infantile aphakic open-angle glaucoma    W Pupillary-block glaucoma 6. Glaucoma related to corticosteroids 7. Glaucoma secondary to rubeosis    W Coats' disease    W Familial exudative vitreoretinopathy    W Medulloepithelioma    W Retinoblastoma    W Subacute/chronic retinal detachment 8. Angle-closure glaucoma    W Central retinal vein occlusion    W Cicatrical retinopathy of prematurity    W Ciliary body cysts    W Congenital pupillary iris-lens membrane    W Laser therapy for threshold ROP    W Microphthalmos    W Nanophthalmos    W Persistent hyperplastic primary vitreous    W Retinoblastoma    W Topiramate therapy 9. Malignant glaucoma 10. Glaucoma associated with increased     venous pressure    W Cavernous or dural A-V shunt    W Orbital disease    W Sturge-Weber syndrome 11. Intraocular infection related glaucoma    W Acute herpetic iritis    W Acute recurrent toxoplasmosis    W Endogenous endophthalmitis    W Maternal rubella infection 12. Glaucoma secondary to unknown etiology    W Iridocorneal endothelial syndrome (ICE) 13. Secondary glaucomas associated with     hereditary ocular conditions    W Ectopia lentis disorders    W Primary angle-closure glaucoma    W Nanophthalmos    W Retinoblastoma

Childhood Glaucoma: Signs, Age of Onset, Prognosis

Any glaucoma appearing before 3 years of age may produce enlargement of the cornea and globe (buphthalmos) and often causes clouding of the cornea and photophobia. After age 3 years, the eye will typically not enlarge, but corneal edema or increased cupping may occur and may be the only signs of glaucoma aside from a decrease in functional vision. Most patients with primary infantile glaucoma will present within the first year. Glaucoma presenting at birth has a poorer prognosis with about 50% of children being legally blind in spite of treatment. Glaucoma developing between 3 and 12 months and receiving successful treatment have up to a 90% chance of obtaining good vision. But, glaucoma can present at any age. Later presentation (e.g. juvenile open angle glaucoma, complicating chronic uveitis) may occur in patients up to 35 years of age or beyond, although it may be confused with adult glaucoma at that point.

Glaucoma associated with Sturge-Weber syndrome, neurofibromatosis, and aniridia may be congenital or present later in childhood or early teens. A thorough knowledge of the natural history of these diseases is necessary to alert the eye care team about the potential onset of glaucoma.

Patient with retinopathy of prematurity (ROP)