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Abnormal iris vasculature

MedGen UID:
477594
Concept ID:
C3275963
Finding
Synonym: Abnormality of iris blood vessels
 
HPO: HP:0007905

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAbnormal iris vasculature

Conditions with this feature

Glaucoma 1, open angle, A
MedGen UID:
333974
Concept ID:
C1842028
Disease or Syndrome
Glaucoma is a group of eye disorders in which the optic nerves connecting the eyes and the brain are progressively damaged. This damage can lead to reduction in side (peripheral) vision and eventual blindness. Other signs and symptoms may include bulging eyes, excessive tearing, and abnormal sensitivity to light (photophobia). The term "early-onset glaucoma" may be used when the disorder appears before the age of 40.\n\nIn most people with glaucoma, the damage to the optic nerves is caused by increased pressure within the eyes (intraocular pressure). Intraocular pressure depends on a balance between fluid entering and leaving the eyes.\n\nUsually glaucoma develops in older adults, in whom the risk of developing the disorder may be affected by a variety of medical conditions including high blood pressure (hypertension) and diabetes mellitus, as well as family history. The risk of early-onset glaucoma depends mainly on heredity.\n\nStructural abnormalities that impede fluid drainage in the eye increase ocular pressure. These abnormalities may be present at birth and usually become apparent during the first year of life. Such structural abnormalities may be part of a genetic disorder that affects many body systems, called a syndrome. If glaucoma appears before the age of 3 without other associated abnormalities, it is called primary congenital glaucoma.\n\nOther individuals experience early onset of primary open-angle glaucoma, the most common adult form of glaucoma. If primary open-angle glaucoma develops during childhood or early adulthood, it is called juvenile open-angle glaucoma.
Anterior segment dysgenesis 3
MedGen UID:
355748
Concept ID:
C1866560
Disease or Syndrome
Anterior segment dysgeneses (ASGD or ASMD) are a heterogeneous group of developmental disorders affecting the anterior segment of the eye, including the cornea, iris, lens, trabecular meshwork, and Schlemm canal. The clinical features of ASGD include iris hypoplasia, an enlarged or reduced corneal diameter, corneal vascularization and opacity, posterior embryotoxon, corectopia, polycoria, an abnormal iridocorneal angle, ectopia lentis, and anterior synechiae between the iris and posterior corneal surface (summary by Cheong et al., 2016). Anterior segment dysgenesis is sometimes divided into subtypes including aniridia (see 106210), Axenfeld and Rieger anomalies, iridogoniodysgenesis, Peters anomaly, and posterior embryotoxon (Gould and John, 2002). Some patients with ASGD3 have been reported with the following subtypes: iridogoniodysgenesis, Peters anomaly, Axenfeld anomaly, and Rieger anomaly. Iridogoniodysgenesis, which is characterized by iris hypoplasia, goniodysgenesis, and juvenile glaucoma, is the result of aberrant migration or terminal induction of the neural crest cells involved in the formation of the anterior segment of the eye (summary by Mears et al., 1996). Peters anomaly consists of a central corneal leukoma, absence of the posterior corneal stroma and Descemet membrane, and a variable degree of iris and lenticular attachments to the central aspect of the posterior cornea (Peters, 1906). In Axenfeld anomaly, strands of iris tissue attach to the Schwalbe line; in Rieger anomaly, in addition to the attachment of iris tissue to the Schwalbe line, there is clinically evident iris stromal atrophy with hole or pseudo-hole formation and corectopia (summary by Smith and Traboulsi, 2012).

Professional guidelines

PubMed

Varrone E, Lenhart P, Rao P, Weil N
J AAPOS 2022 Aug;26(4):207-210. Epub 2022 Jun 11 doi: 10.1016/j.jaapos.2022.04.003. PMID: 35697318
Ababneh OH, Yousef YA, Gharaibeh AM, Abu Ameerh MA, Abu-Yaghi NE, Al Bdour MD
Retina 2013 Apr;33(4):748-55. doi: 10.1097/IAE.0b013e3182721153. PMID: 23296048

Recent clinical studies

Etiology

Saffren B, Price JM, Zhang QE, Hamershock RA, Sharpe J, Levin AV
J AAPOS 2021 Apr;25(2):97.e1-97.e5. Epub 2021 Apr 24 doi: 10.1016/j.jaapos.2020.11.017. PMID: 33901671
Ebrahimiadib N, Maleki A, Fadakar K, Manhapra A, Ghassemi F, Foster CS
Surv Ophthalmol 2021 Jul-Aug;66(4):653-667. Epub 2021 Jan 5 doi: 10.1016/j.survophthal.2020.12.006. PMID: 33412171
Khan AO, Aldahmesh M
J AAPOS 2006 Jun;10(3):273-4. doi: 10.1016/j.jaapos.2006.02.002. PMID: 16814183
Mizener JB, Podhajsky P, Hayreh SS
Ophthalmology 1997 May;104(5):859-64. doi: 10.1016/s0161-6420(97)30221-8. PMID: 9160035
Brown GC, Duker JS, Lehman R, Eagle RC Jr
Int Ophthalmol 1993 Feb;17(1):9-17. doi: 10.1007/BF00918861. PMID: 7686140

Diagnosis

Yang JF, Roohipourmoallai R, Straughn PE, Sherwood MB, Agarwal-Sinha S, Zori RT, Iyer SSR
J AAPOS 2021 Oct;25(5):309-311. Epub 2021 Sep 25 doi: 10.1016/j.jaapos.2021.05.014. PMID: 34582953
Ebrahimiadib N, Maleki A, Fadakar K, Manhapra A, Ghassemi F, Foster CS
Surv Ophthalmol 2021 Jul-Aug;66(4):653-667. Epub 2021 Jan 5 doi: 10.1016/j.survophthal.2020.12.006. PMID: 33412171
Santos-Bueso E, Muñoz-Hernández AM, De-Nova E, Calvo-González C, Díaz-Valle D, Gegúndez-Fernández JA, Benítez-Del-Castillo JM
Arch Soc Esp Oftalmol 2016 Sep;91(9):453-6. Epub 2016 Feb 18 doi: 10.1016/j.oftal.2016.01.020. PMID: 26900041
Lasky JB, Sandu M, Balashanmugan A
J AAPOS 2004 Oct;8(5):495-8. doi: 10.1016/j.jaapos.2004.06.014. PMID: 15492745
WEISE EH
J Natl Malar Soc 1946 May-Jun;2(3):78 passim. PMID: 20992908

Therapy

Chang M, Ancona-Lezama D, Shields CL
Indian J Ophthalmol 2019 Oct;67(10):1704-1705. doi: 10.4103/ijo.IJO_311_19. PMID: 31546520Free PMC Article
Yakes W, Huguenot M, Yakes A, Continenza A, Kammer R, Baumgartner I
J Vasc Surg 2016 Nov;64(5):1478-1482. Epub 2015 Dec 31 doi: 10.1016/j.jvs.2015.10.092. PMID: 26749478
Shinohara G, Morita K, Uno Y, Yamashiro M, Hashimoto K
Gen Thorac Cardiovasc Surg 2010 Oct;58(10):524-7. Epub 2010 Oct 13 doi: 10.1007/s11748-009-0567-1. PMID: 20941566
Dorrell M, Uusitalo-Jarvinen H, Aguilar E, Friedlander M
Surv Ophthalmol 2007 Jan;52 Suppl 1:S3-19. doi: 10.1016/j.survophthal.2006.10.017. PMID: 17240254
Lubinsky MS
Am J Med Genet A 2006 Oct 1;140(19):2080-4. doi: 10.1002/ajmg.a.31303. PMID: 16770809

Prognosis

Saffren B, Price JM, Zhang QE, Hamershock RA, Sharpe J, Levin AV
J AAPOS 2021 Apr;25(2):97.e1-97.e5. Epub 2021 Apr 24 doi: 10.1016/j.jaapos.2020.11.017. PMID: 33901671
Khan AO, Aldahmesh M
J AAPOS 2006 Jun;10(3):273-4. doi: 10.1016/j.jaapos.2006.02.002. PMID: 16814183
Lasky JB, Sandu M, Balashanmugan A
J AAPOS 2004 Oct;8(5):495-8. doi: 10.1016/j.jaapos.2004.06.014. PMID: 15492745
Mizener JB, Podhajsky P, Hayreh SS
Ophthalmology 1997 May;104(5):859-64. doi: 10.1016/s0161-6420(97)30221-8. PMID: 9160035
Brown GC, Duker JS, Lehman R, Eagle RC Jr
Int Ophthalmol 1993 Feb;17(1):9-17. doi: 10.1007/BF00918861. PMID: 7686140

Clinical prediction guides

Saffren B, Price JM, Zhang QE, Hamershock RA, Sharpe J, Levin AV
J AAPOS 2021 Apr;25(2):97.e1-97.e5. Epub 2021 Apr 24 doi: 10.1016/j.jaapos.2020.11.017. PMID: 33901671
Santos-Bueso E, Muñoz-Hernández AM, De-Nova E, Calvo-González C, Díaz-Valle D, Gegúndez-Fernández JA, Benítez-Del-Castillo JM
Arch Soc Esp Oftalmol 2016 Sep;91(9):453-6. Epub 2016 Feb 18 doi: 10.1016/j.oftal.2016.01.020. PMID: 26900041
Utsugi N, Takahashi K, Kishi S
Retina 2004 Dec;24(6):915-9. doi: 10.1097/00006982-200412000-00012. PMID: 15579990
Brown GC, Duker JS, Lehman R, Eagle RC Jr
Int Ophthalmol 1993 Feb;17(1):9-17. doi: 10.1007/BF00918861. PMID: 7686140
Eagle RC Jr, Font RL, Fine BS
Arch Ophthalmol 1979 Mar;97(3):510-5. doi: 10.1001/archopht.1979.01020010254014. PMID: 420639

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