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Glaucoma 3, primary congenital, D(GLC3D)

MedGen UID:
416524
Concept ID:
C2751316
Disease or Syndrome
Synonyms: Glaucoma 3, Primary Congenital, D; Glc3d
 
Gene (location): LTBP2 (14q24.3)
 
Monarch Initiative: MONDO:0013122
OMIM®: 613086

Disease characteristics

Excerpted from the GeneReview: Primary Congenital Glaucoma
Primary congenital glaucoma (PCG) is characterized by elevated intraocular pressure (IOP), enlargement of the globe (buphthalmos), edema, and opacification of the cornea with rupture of Descemet's membrane (Haab's striae), thinning of the anterior sclera and iris atrophy, anomalously deep anterior chamber, and structurally normal posterior segment except for progressive glaucomatous optic atrophy. Symptoms include photophobia, blepharospasm, and excessive tearing. Typically, the diagnosis is made in the first year of life. Depending on when treatment is instituted, visual acuity may be reduced and/or visual fields may be restricted. In untreated individuals, blindness invariably occurs. [from GeneReviews]
Authors:
Khaled K Abu-Amero  |  Deepak P Edward   view full author information

Clinical features

From HPO
Photophobia
MedGen UID:
43220
Concept ID:
C0085636
Sign or Symptom
Excessive sensitivity to light with the sensation of discomfort or pain in the eyes due to exposure to bright light.
Corneal opacity
MedGen UID:
40485
Concept ID:
C0010038
Finding
A reduction of corneal clarity.
Ectopia lentis
MedGen UID:
41704
Concept ID:
C0013581
Congenital Abnormality
Dislocation or malposition of the crystalline lens of the eye. A partial displacement (or dislocation) of the lens is described as a subluxation of the lens, while a complete displacement is termed luxation of the lens. A complete displacement occurs if the lens is completely outside the patellar fossa of the lens, either in the anterior chamber, in the vitreous, or directly on the retina. If the lens is partially displaced but still contained within the lens space, then it is termed subluxation.
Primary congenital glaucoma
MedGen UID:
288550
Concept ID:
C1533041
Disease or Syndrome
Primary congenital glaucoma (PCG) is characterized by elevated intraocular pressure (IOP), enlargement of the globe (buphthalmos), edema, and opacification of the cornea with rupture of Descemet's membrane (Haab's striae), thinning of the anterior sclera and iris atrophy, anomalously deep anterior chamber, and structurally normal posterior segment except for progressive glaucomatous optic atrophy. Symptoms include photophobia, blepharospasm, and excessive tearing. Typically, the diagnosis is made in the first year of life. Depending on when treatment is instituted, visual acuity may be reduced and/or visual fields may be restricted. In untreated individuals, blindness invariably occurs.

Professional guidelines

PubMed

Gupta A, Kekunnaya R, Ramappa M, Vaddavalli PK
Br J Ophthalmol 2011 Apr;95(4):477-80. Epub 2010 Oct 8 doi: 10.1136/bjo.2010.184606. PMID: 20935310

Curated

UK NICE Guideline NG81, Glaucoma: diagnosis and management

Recent clinical studies

Etiology

Khairy MA, Kenawy S, Fawzi KM, Al-Nashar HY
Int Ophthalmol 2023 May;43(5):1591-1600. Epub 2022 Oct 20 doi: 10.1007/s10792-022-02558-1. PMID: 36266601
Abdeen W, Esmael AF, Gawdat G, El-Fayoumi D
Eye (Lond) 2022 Jun;36(6):1238-1245. Epub 2021 Jun 11 doi: 10.1038/s41433-021-01583-1. PMID: 34117386Free PMC Article
Mahmoud SRK, Morsy MS, Bayoumi NHL, Saad NSF
J Pediatr Ophthalmol Strabismus 2021 Jul-Aug;58(4):218-223. Epub 2021 Jul 1 doi: 10.3928/01913913-20210211-02. PMID: 34288764
Lee HJ, Kim YK, Jeoung JW, Park KH, Kim SJ
Graefes Arch Clin Exp Ophthalmol 2021 Nov;259(11):3445-3451. Epub 2021 Jun 2 doi: 10.1007/s00417-021-05232-x. PMID: 34076742
Khokhar S, Yadav D, Gupta S, Sihota R, Chaurasia AK, Gupta A, Gupta V
Eye (Lond) 2019 Apr;33(4):542-548. Epub 2018 Oct 31 doi: 10.1038/s41433-018-0253-6. PMID: 30382237Free PMC Article

Diagnosis

Abdeen W, Esmael AF, Gawdat G, El-Fayoumi D
Eye (Lond) 2022 Jun;36(6):1238-1245. Epub 2021 Jun 11 doi: 10.1038/s41433-021-01583-1. PMID: 34117386Free PMC Article
Agarwal R, Sen S, Kashyap S, Dada T, Nag TC, Gupta V, Sihota R
Br J Ophthalmol 2022 Jan;106(1):60-64. Epub 2020 Oct 16 doi: 10.1136/bjophthalmol-2020-316346. PMID: 33067359
Mahmoud SRK, Morsy MS, Bayoumi NHL, Saad NSF
J Pediatr Ophthalmol Strabismus 2021 Jul-Aug;58(4):218-223. Epub 2021 Jul 1 doi: 10.3928/01913913-20210211-02. PMID: 34288764
Lee HJ, Kim YK, Jeoung JW, Park KH, Kim SJ
Graefes Arch Clin Exp Ophthalmol 2021 Nov;259(11):3445-3451. Epub 2021 Jun 2 doi: 10.1007/s00417-021-05232-x. PMID: 34076742
Khokhar S, Yadav D, Gupta S, Sihota R, Chaurasia AK, Gupta A, Gupta V
Eye (Lond) 2019 Apr;33(4):542-548. Epub 2018 Oct 31 doi: 10.1038/s41433-018-0253-6. PMID: 30382237Free PMC Article

Therapy

Khairy MA, Kenawy S, Fawzi KM, Al-Nashar HY
Int Ophthalmol 2023 May;43(5):1591-1600. Epub 2022 Oct 20 doi: 10.1007/s10792-022-02558-1. PMID: 36266601
Ren J, Lyu J, Ye H, Liu J, Peng J, Zhao P
Int Ophthalmol 2022 Sep;42(9):2871-2879. Epub 2022 Apr 13 doi: 10.1007/s10792-022-02277-7. PMID: 35419635
Ramtohul P, Chardavoine M, Beylerian M, Aziz A, Matonti F, Denis D
BMC Ophthalmol 2018 Sep 5;18(1):240. doi: 10.1186/s12886-018-0906-z. PMID: 30185152Free PMC Article
Khalil DH, Abdelhakim MA
Acta Ophthalmol 2016 Nov;94(7):e550-e554. Epub 2016 Apr 1 doi: 10.1111/aos.13018. PMID: 27037905
Lloyd IC, Ashworth J, Biswas S, Abadi RV
Eye (Lond) 2007 Oct;21(10):1301-9. doi: 10.1038/sj.eye.6702845. PMID: 17914433

Prognosis

Khairy MA, Kenawy S, Fawzi KM, Al-Nashar HY
Int Ophthalmol 2023 May;43(5):1591-1600. Epub 2022 Oct 20 doi: 10.1007/s10792-022-02558-1. PMID: 36266601
Lee HJ, Kim YK, Jeoung JW, Park KH, Kim SJ
Graefes Arch Clin Exp Ophthalmol 2021 Nov;259(11):3445-3451. Epub 2021 Jun 2 doi: 10.1007/s00417-021-05232-x. PMID: 34076742
Tran HT, Tran HT, Luong LH, Nguyen TS, Nguyen HQ, Vu TT, Ta TD, Dao TMA, Bui TH, Ta TV, Tran VK
Ophthalmic Genet 2019 Jun;40(3):286-287. Epub 2019 May 31 doi: 10.1080/13816810.2019.1616304. PMID: 31149863
Khokhar S, Yadav D, Gupta S, Sihota R, Chaurasia AK, Gupta A, Gupta V
Eye (Lond) 2019 Apr;33(4):542-548. Epub 2018 Oct 31 doi: 10.1038/s41433-018-0253-6. PMID: 30382237Free PMC Article
Khalil DH, Abdelhakim MA
Acta Ophthalmol 2016 Nov;94(7):e550-e554. Epub 2016 Apr 1 doi: 10.1111/aos.13018. PMID: 27037905

Clinical prediction guides

Khairy MA, Kenawy S, Fawzi KM, Al-Nashar HY
Int Ophthalmol 2023 May;43(5):1591-1600. Epub 2022 Oct 20 doi: 10.1007/s10792-022-02558-1. PMID: 36266601
Young TL, Whisenhunt KN, Jin J, LaMartina SM, Martin SM, Souma T, Limviphuvadh V, Suri F, Souzeau E, Zhang X, Dan Y, Anagnos E, Carmona S, Jody NM, Stangel N, Higuchi EC, Huang SJ, Siggs OM, Simões MJ, Lawson BM, Martin JS, Elahi E, Narooie-Nejad M, Motlagh BF, Quaggin SE, Potter HD, Silva ED, Craig JE, Egas C, Maroofian R, Maurer-Stroh S, Bradfield YS, Tompson SW
Invest Ophthalmol Vis Sci 2020 Oct 1;61(12):6. doi: 10.1167/iovs.61.12.6. PMID: 33027505Free PMC Article
Khokhar S, Yadav D, Gupta S, Sihota R, Chaurasia AK, Gupta A, Gupta V
Eye (Lond) 2019 Apr;33(4):542-548. Epub 2018 Oct 31 doi: 10.1038/s41433-018-0253-6. PMID: 30382237Free PMC Article
Azar G, Dureau P, Barjol A, Edelson C, Bergès O, Caputo G
Eur J Ophthalmol 2013 Jul-Aug;23(4):597-600. Epub 2013 Mar 6 doi: 10.5301/ejo.5000264. PMID: 23483508
Lu Y, Ji YH, Luo Y, Jiang YX, Wang M, Chen X
Graefes Arch Clin Exp Ophthalmol 2010 May;248(5):681-6. Epub 2010 Feb 17 doi: 10.1007/s00417-010-1310-4. PMID: 20162296

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NICE, 2022
      UK NICE Guideline NG81, Glaucoma: diagnosis and management

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