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Posterior synechiae of the anterior chamber

MedGen UID:
488784
Concept ID:
C0152253
Disease or Syndrome
Synonyms: Iridolenticular adhesions; Posterior synechiae
SNOMED CT: Posterior synechiae (111512005)
 
HPO: HP:0011484

Definition

Adhesions between the iris and the lens. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPosterior synechiae of the anterior chamber

Conditions with this feature

Congenital primary aphakia
MedGen UID:
339935
Concept ID:
C1853230
Congenital Abnormality
Anterior segment dysgeneses 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 ASGD2 have been reported with a congenital primary aphakia subtype. Congenital primary aphakia is a rare developmental disorder characterized by absence of the lens, the development of which is normally induced during the fourth to fifth week of human embryogenesis. This original failure leads, in turn, to complete aplasia of the anterior segment of the eye, which is the diagnostic histologic criterion for CPAK. In contrast, in secondary aphakia, lens induction occurs and the lens vesicle develops to some degree, but is progressively resorbed perinatally, resulting in less severe ocular defects (summary by Valleix et al., 2006).
Persistent hyperplastic primary vitreous, autosomal recessive
MedGen UID:
370100
Concept ID:
C1969783
Disease or Syndrome
Persistent hyperplastic primary vitreous (PHPV), also termed 'persistent fetal vasculature,' is a developmental malformation of the eye in which the primary vitreous fails to regress in utero, resulting in the presence of a retrolental fibrovascular membrane with persistence of the posterior portion of the tunica vasculosa lentis and hyaloid artery. This abnormality is usually unilateral and associated with microphthalmia, cataract, glaucoma, and congenital retinal nonattachment (see Haddad et al., 1978; Khaliq et al., 2001; Prasov et al., 2012). PHPV shares phenotypic overlap with Norrie disease (310600). Genetic Heterogeneity of Persistent Hyperplastic Primary Vitreous A dominant form of PHPV has been described (PHPVAD; 611308).
Oculoauricular syndrome
MedGen UID:
393758
Concept ID:
C2677500
Disease or Syndrome
Oculoauricular syndrome (OCACS) is characterized by complex ocular anomalies, including congenital cataract, anterior segment dysgenesis, iris coloboma, and early-onset retinal dystrophy, and dysplastic ears with abnormal external ear cartilage (summary by Gillespie et al., 2015).
Axenfeld-Rieger syndrome type 3
MedGen UID:
394534
Concept ID:
C2678503
Disease or Syndrome
Axenfeld-Rieger syndrome is primarily an eye disorder, although it can also affect other parts of the body. This condition is characterized by abnormalities of the front part of the eye, an area known as the anterior segment. For example, the colored part of the eye (the iris), may be thin or poorly developed. The iris normally has a single central hole, called the pupil, through which light enters the eye. People with Axenfeld-Rieger syndrome often have a pupil that is off-center (corectopia) or extra holes in the iris that can look like multiple pupils (polycoria). This condition can also cause abnormalities of the cornea, which is the clear front covering of the eye.\n\nAbout half of affected individuals develop glaucoma, a serious condition that increases pressure inside the eye. When glaucoma occurs with Axenfeld-Rieger syndrome, it most often develops in late childhood or adolescence, although it can occur as early as infancy. Glaucoma can cause vision loss or blindness.\n\nResearchers have described at least three types of Axenfeld-Rieger syndrome. The types, which are numbered 1 through 3, are distinguished by their genetic cause.\n\nThe signs and symptoms of Axenfeld-Rieger syndrome can also affect other parts of the body. Many affected individuals have distinctive facial features such as widely spaced eyes (hypertelorism); a flattened mid-face with a broad, flat nasal bridge; and a prominent forehead. The condition is also associated with dental abnormalities including unusually small teeth (microdontia) or fewer than normal teeth (oligodontia). Some people with Axenfeld-Rieger syndrome have extra folds of skin around their belly button (redundant periumbilical skin). Other, less common features can include heart defects, the opening of the urethra on the underside of the penis (hypospadias), narrowing of the anus (anal stenosis), and abnormalities of the pituitary gland that can result in slow growth.
Weill-Marchesani 4 syndrome, recessive
MedGen UID:
416383
Concept ID:
C2750787
Disease or Syndrome
Weill-Marchesani syndrome (WMS) is a connective tissue disorder characterized by abnormalities of the lens of the eye, short stature, brachydactyly, joint stiffness, and cardiovascular defects. The ocular problems, typically recognized in childhood, include microspherophakia (small spherical lens), myopia secondary to the abnormal shape of the lens, ectopia lentis (abnormal position of the lens), and glaucoma, which can lead to blindness. Height of adult males is 142-169 cm; height of adult females is 130-157 cm. Autosomal recessive WMS cannot be distinguished from autosomal dominant WMS by clinical findings alone.
Muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A6
MedGen UID:
461764
Concept ID:
C3150414
Disease or Syndrome
Congenital muscular dystrophy-dystroglycanopathy with brain and eye anomalies (type A), which includes both the more severe Walker-Warburg syndrome (WWS) and the slightly less severe muscle-eye-brain disease (MEB), is an autosomal recessive disorder with characteristic brain and eye malformations, profound mental retardation, congenital muscular dystrophy, and death usually in the first years of life. It represents the most severe end of a phenotypic spectrum of similar disorders resulting from defective glycosylation of DAG1 (128239), collectively known as 'dystroglycanopathies' (Godfrey et al., 2007). For a general phenotypic description and a discussion of genetic heterogeneity of muscular dystrophy-dystroglycanopathy type A, see MDDGA1 (236670).
Familial progressive retinal dystrophy-iris coloboma-congenital cataract syndrome
MedGen UID:
903733
Concept ID:
C4225493
Disease or Syndrome
Retinal dystrophy and iris coloboma with or without cataract (RDICC) is characterized by early-onset chorioretinal dystrophy that is variably associated with other eye anomalies, including iris coloboma and/or congenital or early-onset cataract. Congenital glaucoma has also been observed (Conte et al., 2015; Jedlickova et al., 2023).
Anterior segment dysgenesis 6
MedGen UID:
934590
Concept ID:
C4310623
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). Patients with ASGD6 have been reported with the Peters anomaly subtype. Peters anomaly consists of corneal opacity, defects in the posterior structures of the cornea, and iridocorneal and/or keratolenticular adhesions. Over 50% of patients develop glaucoma in childhood (summary by Vincent et al., 2001).

Professional guidelines

PubMed

Ong AY, McCann P, Perera SA, Lim F, Ng SM, Friedman DS, Chang D
Cochrane Database Syst Rev 2023 Mar 8;3(3):CD015116. doi: 10.1002/14651858.CD015116.pub2. PMID: 36884304Free PMC Article
Yang P, Zhang W, Chen Z, Zhang H, Su G, Cao Q, Zhu Y, Zhong Z, Zhou C, Wang Y, Kijlstra A
Br J Ophthalmol 2022 Dec;106(12):1678-1683. Epub 2021 Jun 9 doi: 10.1136/bjophthalmol-2021-319343. PMID: 34108225Free PMC Article
Sabhapandit S, Murthy SI, Balne PK, Sangwan VS, Sumanth V, Reddy AK
Indian J Ophthalmol 2016 Aug;64(8):555-8. doi: 10.4103/0301-4738.191485. PMID: 27688274Free PMC Article

Recent clinical studies

Etiology

Kawali A, Sriram R, Srinivasan S, Mahendradas P, Shetty R
Indian J Ophthalmol 2024 Jun 1;72(6):885-889. Epub 2024 Feb 5 doi: 10.4103/IJO.IJO_1151_23. PMID: 38317311Free PMC Article
Papaliodis GN, Rosner BA, Dreger KA, Fitzgerald TD, Artornsombudh P, Kothari S, Gangaputra SS, Levy-Clarke GA, Nussenblatt RB, Rosenbaum JT, Sen HN, Suhler EB, Thorne JE, Bhatt NP, Foster CS, Jabs DA, Pak CM, Ying GS, Kempen JH; Systemic Immunosuppressive Therapy for Eye Diseases (SITE) Cohort Study Research Group
Am J Ophthalmol 2023 Oct;254:221-232. Epub 2023 Jul 5 doi: 10.1016/j.ajo.2023.06.021. PMID: 37414328Free PMC Article
Woreta FA, Lindsley KB, Gharaibeh A, Ng SM, Scherer RW, Goldberg MF
Cochrane Database Syst Rev 2023 Mar 13;3(3):CD005431. doi: 10.1002/14651858.CD005431.pub5. PMID: 36912744Free PMC Article
Onouchi H, Hayashi T, Shimizu T, Matsuzawa A, Suzuki Y, Kato N
Cornea 2021 Jun 1;40(6):690-695. doi: 10.1097/ICO.0000000000002535. PMID: 33009090
D'Ambrosio EM, La Cava M, Tortorella P, Gharbiya M, Campanella M, Iannetti L
Semin Ophthalmol 2017;32(6):689-701. Epub 2016 Jul 12 doi: 10.3109/08820538.2016.1170158. PMID: 27404944

Diagnosis

Woreta FA, Lindsley KB, Gharaibeh A, Ng SM, Scherer RW, Goldberg MF
Cochrane Database Syst Rev 2023 Mar 13;3(3):CD005431. doi: 10.1002/14651858.CD005431.pub5. PMID: 36912744Free PMC Article
Fram NR, Bedrood S, Durr GM, Liaboe CA, Samuelson TW, Shah M, McCabe C
J Cataract Refract Surg 2022 Feb 1;48(2):254. doi: 10.1097/j.jcrs.0000000000000882. PMID: 35082239
Chan NS, Chee SP
Clin Exp Ophthalmol 2019 Apr;47(3):320-333. Epub 2018 Nov 13 doi: 10.1111/ceo.13417. PMID: 30345620
Alshamrani AA, Al-Shahwan S
J Glaucoma 2018 Jun;27(6):e110-e112. doi: 10.1097/IJG.0000000000000946. PMID: 29557836
Pathanapitoon K, Dodds EM, Cunningham ET Jr, Rothova A
Ocul Immunol Inflamm 2017 Aug;25(4):569-576. Epub 2016 Jul 18 doi: 10.1080/09273948.2016.1185527. PMID: 27428361

Therapy

Mallat F, Chaaya C, Aoun M, Soutou B, Helou J
J Cutan Med Surg 2023 Jul-Aug;27(4):375-387. Epub 2023 Jun 5 doi: 10.1177/12034754231174852. PMID: 37272371
Woreta FA, Lindsley KB, Gharaibeh A, Ng SM, Scherer RW, Goldberg MF
Cochrane Database Syst Rev 2023 Mar 13;3(3):CD005431. doi: 10.1002/14651858.CD005431.pub5. PMID: 36912744Free PMC Article
Sim HE, Hwang JH
Graefes Arch Clin Exp Ophthalmol 2023 Feb;261(2):555-560. Epub 2022 Aug 8 doi: 10.1007/s00417-022-05798-0. PMID: 35939122Free PMC Article
Terada Y, Kaburaki T, Takase H, Goto H, Nakano S, Inoue Y, Maruyama K, Miyata K, Namba K, Sonoda KH, Kaneko Y, Numaga J, Fukushima M, Horiguchi N, Ide M, Ehara F, Miyazaki D, Hasegawa E, Mochizuki M
Am J Ophthalmol 2021 Jul;227:191-200. Epub 2021 Mar 25 doi: 10.1016/j.ajo.2021.03.020. PMID: 33773985
Alshamrani AA, Al-Shahwan S
J Glaucoma 2018 Jun;27(6):e110-e112. doi: 10.1097/IJG.0000000000000946. PMID: 29557836

Prognosis

Kawali A, Sriram R, Srinivasan S, Mahendradas P, Shetty R
Indian J Ophthalmol 2024 Jun 1;72(6):885-889. Epub 2024 Feb 5 doi: 10.4103/IJO.IJO_1151_23. PMID: 38317311Free PMC Article
Papaliodis GN, Rosner BA, Dreger KA, Fitzgerald TD, Artornsombudh P, Kothari S, Gangaputra SS, Levy-Clarke GA, Nussenblatt RB, Rosenbaum JT, Sen HN, Suhler EB, Thorne JE, Bhatt NP, Foster CS, Jabs DA, Pak CM, Ying GS, Kempen JH; Systemic Immunosuppressive Therapy for Eye Diseases (SITE) Cohort Study Research Group
Am J Ophthalmol 2023 Oct;254:221-232. Epub 2023 Jul 5 doi: 10.1016/j.ajo.2023.06.021. PMID: 37414328Free PMC Article
Lim YJ, Byon I, Kim HW, Park SW, Kwon HJ, Kim E
Korean J Ophthalmol 2023 Aug;37(4):299-306. Epub 2023 Jul 3 doi: 10.3341/kjo.2023.0017. PMID: 37400085Free PMC Article
Pathanapitoon K, Dodds EM, Cunningham ET Jr, Rothova A
Ocul Immunol Inflamm 2017 Aug;25(4):569-576. Epub 2016 Jul 18 doi: 10.1080/09273948.2016.1185527. PMID: 27428361
Nicholson AD, Menon S
J Postgrad Med 1995 Jan-Mar;41(1):22-3. PMID: 10740699

Clinical prediction guides

Kawali A, Sriram R, Srinivasan S, Mahendradas P, Shetty R
Indian J Ophthalmol 2024 Jun 1;72(6):885-889. Epub 2024 Feb 5 doi: 10.4103/IJO.IJO_1151_23. PMID: 38317311Free PMC Article
Mallat F, Chaaya C, Aoun M, Soutou B, Helou J
J Cutan Med Surg 2023 Jul-Aug;27(4):375-387. Epub 2023 Jun 5 doi: 10.1177/12034754231174852. PMID: 37272371
Onouchi H, Hayashi T, Shimizu T, Matsuzawa A, Suzuki Y, Kato N
Cornea 2021 Jun 1;40(6):690-695. doi: 10.1097/ICO.0000000000002535. PMID: 33009090
Alshamrani AA, Al-Shahwan S
J Glaucoma 2018 Jun;27(6):e110-e112. doi: 10.1097/IJG.0000000000000946. PMID: 29557836
D'Ambrosio EM, La Cava M, Tortorella P, Gharbiya M, Campanella M, Iannetti L
Semin Ophthalmol 2017;32(6):689-701. Epub 2016 Jul 12 doi: 10.3109/08820538.2016.1170158. PMID: 27404944

Recent systematic reviews

Woreta FA, Lindsley KB, Gharaibeh A, Ng SM, Scherer RW, Goldberg MF
Cochrane Database Syst Rev 2023 Mar 13;3(3):CD005431. doi: 10.1002/14651858.CD005431.pub5. PMID: 36912744Free PMC Article
Ong AY, McCann P, Perera SA, Lim F, Ng SM, Friedman DS, Chang D
Cochrane Database Syst Rev 2023 Mar 8;3(3):CD015116. doi: 10.1002/14651858.CD015116.pub2. PMID: 36884304Free PMC Article
Rouse B, Le JT, Gazzard G
Cochrane Database Syst Rev 2023 Jan 9;1(1):CD012270. doi: 10.1002/14651858.CD012270.pub3. PMID: 36621864Free PMC Article
Schmidt DC, Kessel L, Pedersen KB, Villumsen JE, Bach-Holm D
Acta Ophthalmol 2021 May;99(3):251-259. Epub 2020 Aug 25 doi: 10.1111/aos.14580. PMID: 32840056

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