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Posterior lenticonus

MedGen UID:
83368
Concept ID:
C0344263
Congenital Abnormality
Synonym: Lenticonus posterior
SNOMED CT: Posterior lenticonus (95482000)
 
HPO: HP:0011502

Definition

A conical projection of the posterior surface of the lens, occurring as a developmental anomaly. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVPosterior lenticonus

Conditions with this feature

Pierson syndrome
MedGen UID:
373199
Concept ID:
C1836876
Disease or Syndrome
Pierson syndrome (PIERS) is an autosomal recessive disorder comprising congenital nephrotic syndrome with diffuse mesangial sclerosis and distinct ocular abnormalities, including microcoria and hypoplasia of the ciliary and pupillary muscles, as well as other anomalies. Many patients die early, and those who survive tend to show neurodevelopmental delay and visual loss (summary by Zenker et al., 2004). Mutations in the LAMB2 gene also cause nephrotic syndrome type 5 with or without mild ocular anomalies (NPHS5; 614199).

Professional guidelines

PubMed

Travi GM, Schnall BM, Lehman SS, Kelly CJ, Hug D, Hirakata VN, Calhoun JH
J AAPOS 2005 Oct;9(5):449-54. doi: 10.1016/j.jaapos.2005.06.001. PMID: 16213394
Cheng KP, Hiles DA, Biglan AW, Pettapiece MC
J Pediatr Ophthalmol Strabismus 1991 May-Jun;28(3):143-9; discussion 150. doi: 10.3928/0191-3913-19910501-07. PMID: 1890571
Robb RM, Mayer DL, Moore BD
J Pediatr Ophthalmol Strabismus 1987 Jul-Aug;24(4):178-81. doi: 10.3928/0191-3913-19870701-07. PMID: 3668765

Recent clinical studies

Etiology

Kekunnaya R, Deshmukh AV, Kulkarni S
Eye (Lond) 2022 May;36(5):985-993. Epub 2021 May 6 doi: 10.1038/s41433-021-01564-4. PMID: 33958736Free PMC Article
Singh B, Mohamed A, Chaurasia S, Ramappa M, Mandal AK, Jalali S, Sangwan VS
J Pediatr Ophthalmol Strabismus 2014 Jan-Feb;51(1):59-62. Epub 2014 Jan 3 doi: 10.3928/01913913-20131223-01. PMID: 24369682
Ranchod TM, Quiram PA, Hathaway N, Ho LY, Glasgow BJ, Trese MT
Ophthalmology 2010 Sep;117(9):1843-7. Epub 2010 Apr 24 doi: 10.1016/j.ophtha.2009.12.045. PMID: 20417569
Travi GM, Schnall BM, Lehman SS, Kelly CJ, Hug D, Hirakata VN, Calhoun JH
J AAPOS 2005 Oct;9(5):449-54. doi: 10.1016/j.jaapos.2005.06.001. PMID: 16213394
Cheng KP, Hiles DA, Biglan AW, Pettapiece MC
J Pediatr Ophthalmol Strabismus 1991 May-Jun;28(3):143-9; discussion 150. doi: 10.3928/0191-3913-19910501-07. PMID: 1890571

Diagnosis

Zou Y, Liu Z, Liu Y
Am J Ophthalmol 2022 Aug;240:e3-e4. Epub 2022 May 2 doi: 10.1016/j.ajo.2022.04.014. PMID: 35513032
Kekunnaya R, Deshmukh AV, Kulkarni S
Eye (Lond) 2022 May;36(5):985-993. Epub 2021 May 6 doi: 10.1038/s41433-021-01564-4. PMID: 33958736Free PMC Article
Agarwal R, Jain V, Kaur M, Titiyal JS
BMJ Case Rep 2021 Feb 5;14(2) doi: 10.1136/bcr-2020-238801. PMID: 33547123Free PMC Article
Tiwari US, Aishwarya A, Kujur R
Rom J Ophthalmol 2018 Jul-Sep;62(3):228-230. PMID: 30505993Free PMC Article
Colville DJ, Savige J
Ophthalmic Genet 1997 Dec;18(4):161-73. doi: 10.3109/13816819709041431. PMID: 9457747

Therapy

Ding Y, Zhang J, Huang Y
J Cataract Refract Surg 2024 Feb 1;50(2):146-152. doi: 10.1097/j.jcrs.0000000000001324. PMID: 37816250Free PMC Article
Travi GM, Schnall BM, Lehman SS, Kelly CJ, Hug D, Hirakata VN, Calhoun JH
J AAPOS 2005 Oct;9(5):449-54. doi: 10.1016/j.jaapos.2005.06.001. PMID: 16213394
Küchle M, Lausen B, Gusek-Schneider GC
Graefes Arch Clin Exp Ophthalmol 2003 Aug;241(8):637-41. Epub 2003 Jul 17 doi: 10.1007/s00417-003-0722-9. PMID: 12883914
Tripathi RC, Cibis GW, Tripathi BJ
Ophthalmology 1986 Aug;93(8):1046-51. doi: 10.1016/s0161-6420(86)33622-4. PMID: 3763153

Prognosis

Kekunnaya R, Deshmukh AV, Kulkarni S
Eye (Lond) 2022 May;36(5):985-993. Epub 2021 May 6 doi: 10.1038/s41433-021-01564-4. PMID: 33958736Free PMC Article
Lee BJ, Kim JH, Yu YS
J Cataract Refract Surg 2014 Feb;40(2):217-23. Epub 2013 Dec 22 doi: 10.1016/j.jcrs.2013.07.048. PMID: 24368116
Travi GM, Schnall BM, Lehman SS, Kelly CJ, Hug D, Hirakata VN, Calhoun JH
J AAPOS 2005 Oct;9(5):449-54. doi: 10.1016/j.jaapos.2005.06.001. PMID: 16213394
Amaya L, Taylor D, Russell-Eggitt I, Nischal KK, Lengyel D
Surv Ophthalmol 2003 Mar-Apr;48(2):125-44. doi: 10.1016/s0039-6257(02)00462-9. PMID: 12686301
Cheng KP, Hiles DA, Biglan AW, Pettapiece MC
J Pediatr Ophthalmol Strabismus 1991 May-Jun;28(3):143-9; discussion 150. doi: 10.3928/0191-3913-19910501-07. PMID: 1890571

Clinical prediction guides

Goyal S, Singh R, Singh JR, Vanita V
BMC Genomics 2024 Jan 4;25(1):31. doi: 10.1186/s12864-023-09880-7. PMID: 38178039Free PMC Article
Mizobuchi K, Hayashi T, Ohira R, Nakano T
Doc Ophthalmol 2023 Jun;146(3):281-291. Epub 2023 May 10 doi: 10.1007/s10633-023-09935-w. PMID: 37162688
Singh B, Mohamed A, Chaurasia S, Ramappa M, Mandal AK, Jalali S, Sangwan VS
J Pediatr Ophthalmol Strabismus 2014 Jan-Feb;51(1):59-62. Epub 2014 Jan 3 doi: 10.3928/01913913-20131223-01. PMID: 24369682
Bredrup C, Matejas V, Barrow M, Bláhová K, Bockenhauer D, Fowler DJ, Gregson RM, Maruniak-Chudek I, Medeira A, Mendonça EL, Kagan M, Koenig J, Krastel H, Kroes HY, Saggar A, Sawyer T, Schittkowski M, Swietliński J, Thompson D, VanDeVoorde RG, Wittebol-Post D, Woodruff G, Zurowska A, Hennekam RC, Zenker M, Russell-Eggitt I
Am J Ophthalmol 2008 Oct;146(4):602-611. Epub 2008 Jul 31 doi: 10.1016/j.ajo.2008.05.039. PMID: 18672223
Zenker M, Tralau T, Lennert T, Pitz S, Mark K, Madlon H, Dötsch J, Reis A, Müntefering H, Neumann LM
Am J Med Genet A 2004 Oct 1;130A(2):138-45. doi: 10.1002/ajmg.a.30310. PMID: 15372515

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