Ophthalmology Book

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Congenital Glaucoma

Aka: Congenital Glaucoma
  1. 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
  2. Pathophysiology
    1. Intraocular Pressure abnormally increased
    2. Damages the Optic Nerve
  3. 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
  4. 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)
  5. Signs
    1. Optic Nerve "cupping"
    2. Buphthalmos
      1. Injected red eye
      2. Hazy or "steamy" Cornea (Corneal Opacification)
      3. Swollen eye
  6. Differential Diagnosis
    1. Nasolacrimal duct obstruction
  7. Management
    1. Surgical Direct communication made between
      1. Anterior chamber
      2. Aqueous Humor draining system (Schlemm's canal)
    2. Long term Ophthalmology follow-up
  8. References
    1. Berson (1987) Ophthalmology Study Guide, AAO, p. 95-110
    2. Essman (1992) Am Fam Physician 46(4):1243-52
    3. Mills (1999) Am Fam Physician 60(3):907-16
    4. Russell-Eggitt (1997) J Cataract Refract Surg 23:664-8
    5. Simon (2001) Am Fam Physician 64(4):623-8

Hydrophthalmos (C0020302)

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.
Definition (NCI) Abnormally large eyeball.
Concepts Disease or Syndrome (T047)
MSH D006871
ICD9 743.20, 743.2
ICD10 Q15.0
SnomedCT 204111004, 204113001, 156898000, 13832009, 204114007, 392371002, 204112006, 204115008, 413728006, 392368005, 204121007
English Buphthalmos, Buphthalmia, Glaucoma of childhood, BUPHTMALMOS, Congenital glaucoma, GLAUCOMA, INFANTILE, Glaucoma, congenital, Glaucoma, open angle, congenital, Buphthalmos, NOS, Juvenile glaucoma, Newborn glaucoma, Buphthalmos NOS, Buphthalmos, unspecified, Developmental glaucoma, Unspecified buphthalmos NOS, Congenital Glaucoma, Megophthalmos, Hydrophthalmos, GLAUCOMA, CONGENITAL, BUPHTHALMOS, GLC3, congenital glaucoma, congenital glaucoma (diagnosis), GLAUCOMA CONGENITAL, Glaucoma of newborn, Hydrophthalmos [Disease/Finding], buphthalmos, infantile glaucoma, juvenile glaucoma, Congenital glaucomas, hydrophthalmos, buphthalmia, congenital; glaucoma, glaucoma; congenital, glaucoma; infantile, glaucoma; newborn, infantile; glaucoma, newborn; glaucoma, Buphthalmos (disorder), Buphthalmos, unspecified (disorder), Congenital glaucoma (disorder), Congenital glaucoma [Ambiguous], Hydrophthalmos (disorder), Unspecified buphthalmos NOS (disorder), Buphthalmos (finding), Buphthalmos NOS (disorder), Infantile glaucoma, Buphthalmus, 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, Buphthalmie, Buphthalmie non précisée, Glaucome infantile, Glaucome de l'enfance, Glaucome congénital, 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, Hidroftalmia, Hidroftalmía, buftalmía, SAI (trastorno), buftalmía, SAI, buftalmía (trastorno), Congenital glaucoma, Congenital glaucoma [Ambiguous], Buftalmos, Hidroftalmos, Buftalmía, Buftalmia, buftalmos (trastorno), buftalmos no especificado, SAI, buftalmía no especificada, SAI (trastorno), buftalmía no especificada, SAI, buftalmía, no especificada (trastorno), buftalmía, no especificada, buftalmía, glaucoma congénito (trastorno), glaucoma congénito, glaucoma del recién nacido, no especificado, glaucoma del recién nacido, hidroftalmía (trastorno), hidroftalmía, 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, ﾆｭｳｼﾞﾘｮｸﾅｲｼｮｳ, ｼｮｳﾆﾘｮｸﾅｲｼｮｳ, ｷﾞｭｳｶﾞﾝ, ｼｮｳｻｲﾌﾒｲﾉｷﾞｭｳｶﾞﾝ, 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
Finnish Buftalmus
Russian BUFTAL'M, GIDROFTAL'M, БУФТАЛЬМ, ГИДРОФТАЛЬМ
Korean 선천 녹내장
Polish Wodoocze
Hungarian Gyermekkori glaucoma, Veleszületett glaucomák, Buphtalmus, Buphthalmia, Veleszületett glaucoma, Congenitalis glaucoma, Csecsemőkori glaucoma, Buphtalmus, nem meghatározott
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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