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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

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
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
Stereopsis in children with unilateral pseudophakia.
Kim SH, Plager DA
Br J Ophthalmol 2009 Mar;93(3):333-6. Epub 2008 Sep 18 doi: 10.1136/bjo.2008.139774. PMID: 18801767
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

Diagnosis

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
Atypical findings in three patients with Pai syndrome and literature review.
Lederer D, Wilson B, Lefesvre P, Poorten VV, Kirkham N, Mitra D, Verellen-Dumoulin C, Devriendt K
Am J Med Genet A 2012 Nov;158A(11):2899-904. Epub 2012 Sep 17 doi: 10.1002/ajmg.a.35592. PMID: 22987662
Stereopsis in children with unilateral pseudophakia.
Kim SH, Plager DA
Br J Ophthalmol 2009 Mar;93(3):333-6. Epub 2008 Sep 18 doi: 10.1136/bjo.2008.139774. PMID: 18801767
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

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

Long-term visual function and relative amblyopia in posterior persistent hyperplastic primary vitreous (PHPV).
Schulz E, Griffiths B
Strabismus 2006 Jun;14(2):121-5. doi: 10.1080/09273970600701242. PMID: 16760119
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
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
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
Intraocular lens implantation in children with monocular cataracts. 1974-1983.
Hiles DA
Ophthalmology 1984 Oct;91(10):1231-7. doi: 10.1016/s0161-6420(84)34162-8. PMID: 6514287

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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