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Achromatic retinal patches

MedGen UID:
348171
Concept ID:
C1860710
Finding
Synonym: Punched out areas of chorioretinal hypopigmentation
 
HPO: HP:0009727

Definition

Areas of the retina lacking pigmentation. Punched out areas of chorioretinal hypopigmentation less than 1 disk diameter in size and tending to be located in the midperiphery of the retina. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAchromatic retinal patches

Conditions with this feature

Tuberous sclerosis 1
MedGen UID:
344288
Concept ID:
C1854465
Disease or Syndrome
Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, intellectual disability / developmental delay, psychiatric illness); kidney (angiomyolipomas, cysts, renal cell carcinomas); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system tumors are the leading cause of morbidity and mortality; renal disease is the second leading cause of early death.
Tuberous sclerosis 2
MedGen UID:
348170
Concept ID:
C1860707
Disease or Syndrome
Tuberous sclerosis complex (TSC) involves abnormalities of the skin (hypomelanotic macules, confetti skin lesions, facial angiofibromas, shagreen patches, fibrous cephalic plaques, ungual fibromas); brain (subependymal nodules, cortical tubers, and subependymal giant cell astrocytomas [SEGAs], seizures, intellectual disability / developmental delay, psychiatric illness); kidney (angiomyolipomas, cysts, renal cell carcinomas); heart (rhabdomyomas, arrhythmias); and lungs (lymphangioleiomyomatosis [LAM], multifocal micronodular pneumonocyte hyperplasia). Central nervous system tumors are the leading cause of morbidity and mortality; renal disease is the second leading cause of early death.

Recent clinical studies

Etiology

Öhnell HM, Kjellström U, Eklund E, Pearsson K, Bekassy Z, Gränse L
Acta Ophthalmol 2024 Jun;102(4):421-427. Epub 2023 Nov 22 doi: 10.1111/aos.15812. PMID: 37991117
Ashkenazy N, Yannuzzi NA, Berrocal AM
Ophthalmic Genet 2022 Feb;43(1):123-125. Epub 2021 Dec 10 doi: 10.1080/13816810.2021.1989599. PMID: 34886749
Martin GC, Dénier C, Zambrowski O, Grévent D, Bruère L, Brousse V, de Montalembert M, Brémond-Gignac D, Robert MP
JAMA Ophthalmol 2017 Oct 1;135(10):1100-1105. doi: 10.1001/jamaophthalmol.2017.3008. PMID: 28837721Free PMC Article
Reuter P, Koeppen K, Ladewig T, Kohl S, Baumann B, Wissinger B; Achromatopsia Clinical Study Group
Hum Mutat 2008 Oct;29(10):1228-36. doi: 10.1002/humu.20790. PMID: 18521937

Diagnosis

Öhnell HM, Kjellström U, Eklund E, Pearsson K, Bekassy Z, Gränse L
Acta Ophthalmol 2024 Jun;102(4):421-427. Epub 2023 Nov 22 doi: 10.1111/aos.15812. PMID: 37991117
Ashkenazy N, Yannuzzi NA, Berrocal AM
Ophthalmic Genet 2022 Feb;43(1):123-125. Epub 2021 Dec 10 doi: 10.1080/13816810.2021.1989599. PMID: 34886749
Wuerger S, Ashraf M, Kim M, Martinovic J, Pérez-Ortiz M, Mantiuk RK
J Vis 2020 Apr 9;20(4):23. doi: 10.1167/jov.20.4.23. PMID: 32347909Free PMC Article
Taylor CP, Shepard TG, Rucker FJ, Eskew RT Jr
Invest Ophthalmol Vis Sci 2018 Sep 4;59(11):4622-4630. doi: 10.1167/iovs.18-24113. PMID: 30242363Free PMC Article
Kim RY, Retsas S, Fitzke FW, Arden GB, Bird AC
Ophthalmology 1994 Nov;101(11):1837-43. doi: 10.1016/s0161-6420(94)31092-x. PMID: 7800366

Prognosis

Öhnell HM, Kjellström U, Eklund E, Pearsson K, Bekassy Z, Gränse L
Acta Ophthalmol 2024 Jun;102(4):421-427. Epub 2023 Nov 22 doi: 10.1111/aos.15812. PMID: 37991117
Wuerger S, Ashraf M, Kim M, Martinovic J, Pérez-Ortiz M, Mantiuk RK
J Vis 2020 Apr 9;20(4):23. doi: 10.1167/jov.20.4.23. PMID: 32347909Free PMC Article
Zawadzki RJ, Cense B, Zhang Y, Choi SS, Miller DT, Werner JS
Opt Express 2008 May 26;16(11):8126-43. doi: 10.1364/oe.16.008126. PMID: 18545525Free PMC Article
Kim RY, Retsas S, Fitzke FW, Arden GB, Bird AC
Ophthalmology 1994 Nov;101(11):1837-43. doi: 10.1016/s0161-6420(94)31092-x. PMID: 7800366

Clinical prediction guides

Wuerger S, Ashraf M, Kim M, Martinovic J, Pérez-Ortiz M, Mantiuk RK
J Vis 2020 Apr 9;20(4):23. doi: 10.1167/jov.20.4.23. PMID: 32347909Free PMC Article
Taylor CP, Shepard TG, Rucker FJ, Eskew RT Jr
Invest Ophthalmol Vis Sci 2018 Sep 4;59(11):4622-4630. doi: 10.1167/iovs.18-24113. PMID: 30242363Free PMC Article
Elliott SL, Cao D
J Vis 2013 Nov 14;13(13):15. doi: 10.1167/13.13.15. PMID: 24233245Free PMC Article
Zawadzki RJ, Cense B, Zhang Y, Choi SS, Miller DT, Werner JS
Opt Express 2008 May 26;16(11):8126-43. doi: 10.1364/oe.16.008126. PMID: 18545525Free PMC Article
Kim RY, Retsas S, Fitzke FW, Arden GB, Bird AC
Ophthalmology 1994 Nov;101(11):1837-43. doi: 10.1016/s0161-6420(94)31092-x. PMID: 7800366

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