. The subject has one brown and one hazel eye.]]
with blue-and-brown eye.]]
In anatomy, '' refers to a difference in Color ation, usually of the Irises but also of Hair or Skin .
Heterochromia (also known as a '' or '''''heterochromia iridium''''') is an Eye condition in which one iris is a different color from the other (''complete heterochromia''), or where part of one iris is a different color from the remainder (''partial heterochromia'' or ''sectoral heterochromia''). It is a result of the relative excess or lack of Pigment within an iris or part of an iris, which may be genetically Inherited or due to Mosaicism , or acquired by Disease or Injury .Imesch PD, Wallow IH, Albert DM. "The color of the human eye: a review of morphologic correlates and of some conditions that affect iridial pigmentation." ''Surv Ophthalmol.'' 1997 Feb;41 Suppl 2:S117-23. PMID 9154287.
Eye Color , specifically the color of the irises, is determined primarily by the concentration and distribution of Melanin pigment within the iris tissuesWielgus AR, Sarna T. "Melanin in human irides of different color and age of donors." ''Pigment Cell Res.'' 2005 Dec;18(6):454-64. PMID 16280011.Prota G, Hu DN, Vincensi MR, McCormick SA, Napolitano A. "Characterization of melanins in human irides and cultured uveal melanocytes from eyes of different colors." ''Exp Eye Res.'' 1998 Sep;67(3):293-9. PMID 9778410. "All About Eye Color" from Larry Bickford. Consequently, anything affecting those factors may result in a difference of color being observed.
The affected eye may be hyperpigmented (hyperchromic) or hypopigmented (hypochromic).Loewenstein, John; Scott Lee (2004). ''Ophthalmology: Just the Facts''. New York: McGraw-Hill. ISBN 0-07-140332-9. An excess of pigmentation is usually associated with ly inherited disorders such as Hirschsprung's Disease and Waardenburg Syndrome .
Although a distinction is frequently made between heterochromia that affects an eye completely or only partially, it is often classified as either Genetic (due to Mosaicism or Congenital ) or acquired, with mention as to whether the affected iris or portion of the iris is darker or lighter.Swann P. "Heterochromia." ''Optometry Today.'' January 29, 1999. Accessed November 1, 2006.
Heterochromia that is congenital is usually inherited as an Autosomal Dominant trait.
; Abnormal iris darker
- Lisch Nodules - iris Hamartoma s seen in Neurofibromatosis .
- Ocular Melanosis - a condition characterized by increased pigmentation of the Uveal Tract , Episclera , and anterior chamber angle.
- Oculodermal Melanocytosis (nevus of Ota)
- Pigment Dispersion Syndrome - a condition characterized by loss of pigmentation from the posterior iris surface which is disseminated intraocularly and deposited on various intraocular structures, including the anterior surface of the iris.
- Sturge-Weber Syndrome - a syndrome characterized by a Port-wine Stain nevus in the distribution of the trigeminal nerve, homolateral meningeal angioma with intracranial calcification and neurologic signs, and angioma of the choroid, often with secondary glaucomavan Emelen C, Goethals M, Dralands L, Casteels I. "Treatment of glaucoma in children with Sturge-Weber syndrome." ''J Pediatr Ophthalmol Strabismus.'' 2000 Jan-Feb;37(1):29-34. PMID 10714693. {Link without Title} .
; Abnormal iris lighter
- Simple heterochromia - a rare condition characterized by the absence of other ocular or systemic problems. The lighter eye is typically regarded as the affected eye as it usually shows iris Hypoplasia . It may affect an iris completely or only partially.
- Congenital Horner's Syndrome Wallis DH, Granet DB, Levi L. "When the darker eye has the smaller pupil." ''J AAPOS.'' 2003 Jun;7(3):215-6. PMID 12825064. - sometimes inherited, although usually acquired
- Waardenburg's Syndrome - a syndrome in which heterochromia presents as a bilateral iris hypochromia in some cases. A Japanese review of 11 albino children with the disorder found that all had sectoral/partial heterochromia.Ohno N, Kiyosawa M, Mori H, Wang WF, Takase H, Mochizuki M. "Clinical findings in Japanese patients with Waardenburg syndrome type 2." ''Jpn J Ophthalmol.'' 2003 Jan-Feb;47(1):77-84. PMID: 12586183.
- Piebaldism - similar to Waardenburg's syndrome, a rare disorder of melanocyte development characterized by a white forelock and multiple symmetrical hypopigmented or depigmented Macules .
- Hirschsprung's Disease - a bowel disorder associated with heterochromia in the form of a sector hypochromia. The affected sectors have been shown to have reduced numbers of melanocytes and decreased stromal pigmentation.Brazel SM, Sullivan TJ, Thorner PS, Clarke MP, Hunter WS, Morin JD. "Iris sector heterochromia as a marker for neural crest disease." ''Arch Ophthalmol.'' 1992 Feb;110(2):233-5. PMID 1736874
- Incontinentia Pigmenti
- Parry-Romberg Syndrome
Heterochromia that is acquired is usually due to injury, inflammation, the use of certain eyedrops, or tumors.
; Abnormal iris darker
; Abnormal iris lighter
- Fuchs' Heterochromic Iridocyclitis - a condition characterized by a low grade, asymptomatic Uveitis in which the iris in the affected eye becomes hypochromic and has a washed-out, somewhat moth eaten appearance. The heterochromia can be very subtle, especially in patients with lighter colored irises. It is often most easily seen in daylight. The prevalence of heterochromia associated with Fuch's has been estimated in various studiesYang P, Fang W, Jin H, Li B, Chen X, Kijlstra A. "Clinical features of Chinese patients with Fuchs' syndrome." ''Ophthalmology.'' 2006 Mar;113(3):473-80. Epub 2006 Feb 3. PMID 16458965.Arellanes-Garcia L, del Carmen Preciado-Delgadillo M, Recillas-Gispert C. "Fuchs' heterochromic iridocyclitis: clinical manifestations in dark-eyed Mexican patients." ''Ocul Immunol Inflamm.'' 2002 Jun;10(2):125-31. PMID 12778348.Tabbut BR, Tessler HH, Williams D. "Fuchs' heterochromic iridocyclitis in blacks." ''Arch Ophthalmol.'' 1988 Dec;106(12):1688-90. PMID 3196209. with results suggesting that there is more difficulty recognizing iris color changes in dark-eyed individuals.Bloch-Michel E. " heterochromic cyclitis: current concepts. " ''J Fr Ophtalmol.'' 1983;6(10):853-8. PMID 6368659.
- Acquired Horner's Syndrome - usually acquired, as in Neuroblastoma ,Mehta K, Haller JO, Legasto AC. "Imaging neuroblastoma in children." ''Crit Rev Comput Tomogr.'' 2003;44(1):47-61. PMID 12627783. although sometimes inherited.
- Neoplasm - Melanoma s can also be very lightly pigmented, and a lighter colored iris may be a rare manifestation of metastatic disease to the eye.
Heterochromia has also been observed in those with Duane Syndrome .Khan AO, Aldamesh M. "Bilateral Duane syndrome and bilateral aniridia." ''J AAPOS.'' 2006 Jun;10(3):273-4. PMID 16814183.Shauly Y, Weissman A, Meyer E. "Ocular and systemic characteristics of Duane syndrome." ''J Pediatr Ophthalmol Strabismus.'' 1993 May-Jun;30(3):178-83. PMID 8350229.
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