II. Epidemiology

  1. Rare pediatric eye disease
  2. Incidence: 1:12,500 live births
    1. Sporadic: 80-90%
    2. Familial: 10-20%
  3. Onset under age 1 year
  4. Bilateral eyes involved in 75% cases

III. Pathophysiology

  1. Intraocular Pressure abnormally increased
  2. Damages the Optic Nerve

IV. Etiology

  1. Congenital Cataract surgery
  2. Congenital Infections
    1. TORCH Virus (especially Rubella)
  3. Sturge Weber Syndrome
  4. Retinopathy of Prematurity
  5. Neurofibromatosis
  6. Aniridia
  7. Aphakia
  8. Steroid induced
  9. Uveitis

V. Symptoms (presents during first months of life)

  1. Epiphora (chronic tearing) or other Lacrimation
  2. Eye discomfort
  3. Photophobia (light sensitivity)
  4. Excessive Eyelid squeezing (blepharospasm)

VI. Signs

  1. Optic Nerve "cupping"
  2. Buphthalmos
    1. Injected Red Eye
    2. Hazy or "steamy" Cornea (Corneal Opacification)
    3. Swollen eye

VII. Differential Diagnosis

VIII. Management

  1. Surgical Direct communication made between
    1. Anterior chamber
    2. Aqueous Humor draining system (Schlemm's canal)
  2. Long term Ophthalmology follow-up

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Ontology: Hydrophthalmos (C0020302)

Definition (MSHCZE) Zvětšení celého očního bulbu v důsledku zvýšeného nitroočního tlaku u dětí, u nichž se bulbus může zvětšovat (vrozený glaukom). Nitrooční tlak stoupá v důsledku poškození komorového úhlu na podkladě vrozené vady nebo poškození (např. v důsledku zánětu v útlém věku). Léčba je operativní (trabekulotomie). (cit. Velký lékařský slovník online, 2013 http://lekarske.slovniky.cz/ )
Definition (MSH) Congenital open-angle glaucoma that results from dysgenesis of the angle structures accompanied by increased intraocular pressure and enlargement of the eye. Treatment is both medical and surgical.
Concepts Disease or Syndrome (T047)
MSH D006871
ICD9 743.20, 743.2
ICD10 Q15.0
SnomedCT 392368005, 204115008, 392371002, 13832009, 156898000, 204111004, 204112006, 204113001, 204114007, 204121007, 413728006
English Buphthalmos, Glaucoma of childhood, Juvenile glaucoma, Newborn glaucoma, Unspecified buphthalmos NOS, Congenital Glaucoma, Megophthalmos, Hydrophthalmos, GLAUCOMA, CONGENITAL, BUPHTHALMOS, GLC3, congenital glaucoma, congenital glaucoma (diagnosis), Buphthalmos NOS, Glaucoma of newborn, Hydrophthalmos [Disease/Finding], buphthalmos, infantile glaucoma, juvenile glaucoma, Congenital glaucomas, Congenital glaucoma (disorder), Unspecified buphthalmos NOS (disorder), Buphthalmos (disorder), Buphthalmos, unspecified (disorder), Hydrophthalmos (disorder), Buphthalmos NOS (disorder), Buphthalmia, hydrophthalmos, buphthalmia, congenital; glaucoma, glaucoma; congenital, glaucoma; infantile, glaucoma; newborn, infantile; glaucoma, newborn; glaucoma, Buphthalmos, NOS, Congenital glaucoma [Ambiguous], Buphthalmos (finding), Congenital glaucoma, Infantile glaucoma, Buphthalmos, unspecified, Developmental glaucoma, Buphthalmus, Glaucoma congenital, Glaucoma, congenital, Glaucoma, newborn
Dutch ontwikkelingsglaucoom, buphthalmos, glaucoom, congenitaal, buphthalmos, niet-gespecificeerd, kinderglaucoom, infantiel glaucoom, congenitaal glaucoom, congenitaal; glaucoom, glaucoom; congenitaal, glaucoom; infantiel, glaucoom; pasgeborene, infantiel; glaucoom, pasgeborene; glaucoom, glaucomata, congenitaal, Buphthalmus, Congenitaal glaucoom, Hydroftalmie, Hydrophthalmus
French Glaucome du développement, Glaucome congénital, Buphthalmie, Buphthalmie non précisée, Glaucome infantile, Glaucome de l'enfance, Glaucomes congénitaux, Hydrophtalmie, Buphtalmie
Italian Glaucoma della crescita, Glaucomi congeniti, Glaucoma congenito, Glaucoma infantile, Buftalmo, non specificato, Glaucoma dell'infanzia, Buftalmo, Idroftalmo
Portuguese Glaucoma de desenvolvimento, Glaucoma da infância, Glaucoma infantil, Buftalmo NE, Glaucoma congénito, Hidroftalmia, Glaucomas congénitos, Buftalmo
Spanish Glaucoma en el desarrollo, Glaucoma congénito, Buftalmía no especificada, Glaucoma infantil, Glaucoma de la infancia, Hidroftalmía, Buftalmía, hidroftalmía (trastorno), buftalmos (trastorno), buftalmos no especificado, SAI, buftalmía no especificada, SAI (trastorno), buftalmía, no especificada (trastorno), glaucoma congénito, buftalmía no especificada, SAI, buftalmía, no especificada, hidroftalmía, buftalmía, SAI (trastorno), buftalmía (trastorno), buftalmía, SAI, buftalmía, glaucoma congénito (trastorno), glaucoma del recién nacido, no especificado, glaucoma del recién nacido, Buftalmos, Hidroftalmos, Glaucomas congénitos, buftalmos, buftalmos (hallazgo)
German Glaukom kongenital, kindliches Glaukom, kongenitales Glaukom, infantiles Glaukom, Buphthalmus, unspezifisch, Angeborenes Glaukom, Kongenitale Glaukome, Buphthalmus, Hydrophthalmus, entwicklungsbedingtes Glaukom
Japanese 先天性緑内障, 乳児緑内障, 小児緑内障, 詳細不明の牛眼, センテンセイリョクナイショウ, ニュウジリョクナイショウ, ショウニリョクナイショウ, ギュウガン, ショウサイフメイノギュウガン, 牛眼, ハッタツリョクナイショウ, 発達緑内障, 水眼, 水眼症
Swedish Hydroftalmi
Czech hydroftalmus, Vrozené glaukomy, Buftalmus, blíže neurčený, Buftalmus, Vrozený glaukom, Glaukom v dětském věku, Rozvíjející se glaukom, Infantilní glaukom, buftalmus
Finnish Buftalmus
Korean 선천 녹내장
Polish Wodoocze
Hungarian Gyermekkori glaucoma, Veleszületett glaucomák, Buphtalmus, Buphthalmia, Veleszületett glaucoma, Congenitalis glaucoma, Csecsemőkori glaucoma, Buphtalmus, nem meghatározott
Norwegian Hydroftalmus, Buftalmus