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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).
See: Condition Record
Pierson syndrome

Professional guidelines

PubMed

Visual outcome and success of amblyopia treatment in unilateral small posterior lens opacities and lenticonus initially treated nonsurgically.
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
Management of posterior lenticonus.
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
Results of early treatment of unilateral congenital cataracts.
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

Newer insights into the clinical profile of posterior lenticonus in children and its surgical, visual, refractive outcomes.
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
Clinical characteristics of congenital and developmental cataract undergoing surgical treatment.
Nagamoto T, Oshika T, Fujikado T, Ishibashi T, Sato M, Kondo M, Kurosaka D, Azuma N
Jpn J Ophthalmol 2015 May;59(3):148-56. Epub 2015 Jan 23 doi: 10.1007/s10384-015-0370-8. PMID: 25608682
Clinical manifestations of congenital aniridia.
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
Ophthalmological aspects of Pierson syndrome.
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
Management of posterior lenticonus.
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

Pigmented posterior lenticonus in unilateral development cataract.
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
Newer insights into the clinical profile of posterior lenticonus in children and its surgical, visual, refractive outcomes.
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
Bilateral combined anterior and posterior lenticonus in Alport's Syndrome.
Tiwari US, Aishwarya A, Kujur R
Rom J Ophthalmol 2018 Jul-Sep;62(3):228-230. PMID: 30505993Free PMC Article
Ophthalmological aspects of Pierson syndrome.
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
Alport syndrome. A review of the ocular manifestations.
Colville DJ, Savige J
Ophthalmic Genet 1997 Dec;18(4):161-73. doi: 10.3109/13816819709041431. PMID: 9457747

Therapy

Influence of posterior capsule abnormalities in pediatric cataract surgery.
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
Visual outcome and success of amblyopia treatment in unilateral small posterior lens opacities and lenticonus initially treated nonsurgically.
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
Results and complications of hydrophobic acrylic vs PMMA posterior chamber lenses in children under 17 years of age.
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
Pathogenesis of cataracts in patients with Lowe's syndrome.
Tripathi RC, Cibis GW, Tripathi BJ
Ophthalmology 1986 Aug;93(8):1046-51. doi: 10.1016/s0161-6420(86)33622-4. PMID: 3763153

Prognosis

Newer insights into the clinical profile of posterior lenticonus in children and its surgical, visual, refractive outcomes.
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
Surgical outcomes after intraocular lens implantation for posterior lenticonus-related cataract according to preoperative lens status.
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
Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: an autosomal recessive syndrome.
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
The morphology and natural history of childhood cataracts.
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
Management of posterior lenticonus.
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

Novel and known variants in GJA3 and LIM2 in congenital cataract families from North India.
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
Electroretinographic abnormalities in Alport syndrome with a novel COL4A5 truncated variant (p.Try20GlyfsTer19).
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
Clinical manifestations of congenital aniridia.
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
Ophthalmological aspects of Pierson syndrome.
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
Congenital nephrosis, mesangial sclerosis, and distinct eye abnormalities with microcoria: an autosomal recessive syndrome.
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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