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Band-shaped keratopathy

MedGen UID:
56354
Concept ID:
C0155120
Disease or Syndrome
Synonym: Band keratopathy
SNOMED CT: BK - Band keratopathy (35055000); Band shaped keratopathy (35055000); Band-shaped keratopathy (35055000); Band keratopathy (35055000)
 
HPO: HP:0000585
Monarch Initiative: MONDO:0009016
OMIM®: 217500

Definition

An abnormality of the cornea characterized by the deposition of calcium in a band across the central cornea, leading to decreased vision, foreign body sensation, and ocular irritation. [from HPO]

Clinical features

From HPO
Band-shaped corneal dystrophy
MedGen UID:
1002866
Concept ID:
CN323271
Finding
A type of genetically determined disease of the cornea with corneal lesions with a band-like shape.

Term Hierarchy

Conditions with this feature

Polyglandular autoimmune syndrome, type 2
MedGen UID:
39126
Concept ID:
C0085860
Disease or Syndrome
Autoimmune polyendocrine syndrome type II (APS2), or Schmidt syndrome, is characterized by the presence of autoimmune Addison disease in association with either autoimmune thyroid disease or type I diabetes mellitus, or both. Chronic candidiasis is not present. APS2 may occur at any age and in both sexes, but is most common in middle-aged females and is very rare in childhood (summary by Betterle et al., 2004). See 240300 for a phenotypic description of autoimmune polyendocrine syndrome type I (APS1).
Posterior polymorphous corneal dystrophy 1
MedGen UID:
343836
Concept ID:
C1852555
Disease or Syndrome
A posterior polymorphous corneal dystrophy that has material basis in autosomal dominant inheritance of mutation in the OVOL2 gene on chromosome 20p11.23.
Alagille syndrome due to a JAG1 point mutation
MedGen UID:
365434
Concept ID:
C1956125
Disease or Syndrome
Alagille syndrome (ALGS) is a multisystem disorder with a wide spectrum of clinical variability; this variability is seen even among individuals from the same family. The major clinical manifestations of ALGS are bile duct paucity on liver biopsy, cholestasis, congenital cardiac defects (primarily involving the pulmonary arteries), butterfly vertebrae, ophthalmologic abnormalities (most commonly posterior embryotoxon), and characteristic facial features. Renal abnormalities, growth failure, developmental delays, splenomegaly, and vascular abnormalities may also occur.
Autosomal recessive proximal renal tubular acidosis
MedGen UID:
370883
Concept ID:
C1970309
Disease or Syndrome
A rare autosomal recessive form of proximal renal tubular acidosis characterized by an isolated defect in the proximal tubule leading to the decreased reabsorption of bicarbonate and consequentially to urinary bicarbonate wastage. Presentation is typically with hyperchloremic acidosis, usually occurring in childhood. Extrarenal manifestations include ocular abnormalities (band keratopathy, glaucoma, and cataracts), intellectual disability and severe growth retardation. Other features like dental enamel defects, basal ganglia calcification and pancreatitis are sometimes present.
X-linked endothelial corneal dystrophy
MedGen UID:
413518
Concept ID:
C2749049
Disease or Syndrome
A rare subtype of posterior corneal dystrophy with characteristics of congenital ground glass corneal clouding or a diffuse corneal haze, and blurred vision in male patients. Prevalence of this rare corneal dystrophy is unknown. Males are affected more severely than females. The condition is progressive in males and non-progressive in females. Has been mapped to the long arm of the X-chromosome (Xq25) but the causative gene has not been identified. Transmission is X-linked recessive.
Craniofacial anomalies and anterior segment dysgenesis syndrome
MedGen UID:
481729
Concept ID:
C3280099
Disease or Syndrome
Knobloch syndrome 1
MedGen UID:
1642123
Concept ID:
C4551775
Disease or Syndrome
Knobloch syndrome-1 (KNO1) is an autosomal recessive developmental disorder primarily characterized by typical eye abnormalities, including high myopia, cataracts, dislocated lens, vitreoretinal degeneration, and retinal detachment, with occipital skull defects, which can range from occipital encephalocele to occult cutis aplasia (summary by Aldahmesh et al., 2011). Genetic Heterogeneity of Knobloch Syndrome KNO2 (618458) is caused by mutation in the PAK2 gene (605022) on chromosome 3q29.
Blau syndrome
MedGen UID:
1684759
Concept ID:
C5201146
Disease or Syndrome
Blau syndrome is characterized by the triad of granulomatous arthritis, uveitis, and dermatitis. First described in 1985, it was considered to be distinct from sarcoidosis due to the early age of onset and autosomal dominant inheritance pattern. Published reports of sporadic cases of children with 'early-onset sarcoidosis' (EOS) with granulomatous involvement of different organs, primarily affecting joints, eyes, and skin, were suspected to represent the same disorder because the patients' characteristics were nearly identical. Subsequently, identical NOD2 mutations were identified in patients with Blau syndrome as well as in patients diagnosed with EOS, confirming earlier suspicions that they represented the same disease (summary by Borzutzky et al., 2010). Unlike older children diagnosed with sarcoidosis, these patients have no apparent pulmonary involvement; however, the disease is progressive and may result in severe complications such as blindness and/or joint destruction (Shetty and Gedalia, 1998).

Professional guidelines

Recent clinical studies

Etiology

Abdi P, Asadigandomani H, Amirkhani A, Taghizadeh S, Nozarian Z
J Med Case Rep 2024 Mar 10;18(1):98. doi: 10.1186/s13256-024-04429-y. PMID: 38461261Free PMC Article
Das AV, Pillutla LN, Chaurasia S
Indian J Ophthalmol 2022 May;70(5):1582-1585. doi: 10.4103/ijo.IJO_2541_21. PMID: 35502030Free PMC Article
Kumar P, Gupta A, Bansal R, Suri D, Gupta V, Gupta A, Singh S
Indian J Pediatr 2022 Apr;89(4):358-363. Epub 2021 Nov 3 doi: 10.1007/s12098-021-03884-5. PMID: 34731440
Gautam Seth N, Kaur S, Yangzes S, Jugran D, Bansal R, Gupta V, Dogra MR, Suri D, Singh S, Singh R
Ocul Immunol Inflamm 2021 Nov 17;29(7-8):1375-1380. Epub 2020 Jul 10 doi: 10.1080/09273948.2020.1762897. PMID: 32649841
Yang P, Sun M
Cornea 2011 Dec;30(12):1336-40. doi: 10.1097/ICO.0b013e31820f774c. PMID: 21934492

Diagnosis

Das AV, Pillutla LN, Chaurasia S
Indian J Ophthalmol 2022 May;70(5):1582-1585. doi: 10.4103/ijo.IJO_2541_21. PMID: 35502030Free PMC Article
Kumar P, Gupta A, Bansal R, Suri D, Gupta V, Gupta A, Singh S
Indian J Pediatr 2022 Apr;89(4):358-363. Epub 2021 Nov 3 doi: 10.1007/s12098-021-03884-5. PMID: 34731440
Gupta SN, Goel R, Saran RK, Rathie N
Nepal J Ophthalmol 2021 Jul;13(24):225-229. doi: 10.3126/nepjoph.v13i2.30629. PMID: 35996789
Gautam Seth N, Kaur S, Yangzes S, Jugran D, Bansal R, Gupta V, Dogra MR, Suri D, Singh S, Singh R
Ocul Immunol Inflamm 2021 Nov 17;29(7-8):1375-1380. Epub 2020 Jul 10 doi: 10.1080/09273948.2020.1762897. PMID: 32649841
Yang P, Sun M
Cornea 2011 Dec;30(12):1336-40. doi: 10.1097/ICO.0b013e31820f774c. PMID: 21934492

Therapy

Das AV, Pillutla LN, Chaurasia S
Indian J Ophthalmol 2022 May;70(5):1582-1585. doi: 10.4103/ijo.IJO_2541_21. PMID: 35502030Free PMC Article
Gautam Seth N, Kaur S, Yangzes S, Jugran D, Bansal R, Gupta V, Dogra MR, Suri D, Singh S, Singh R
Ocul Immunol Inflamm 2021 Nov 17;29(7-8):1375-1380. Epub 2020 Jul 10 doi: 10.1080/09273948.2020.1762897. PMID: 32649841
Yang P, Sun M
Cornea 2011 Dec;30(12):1336-40. doi: 10.1097/ICO.0b013e31820f774c. PMID: 21934492
Arora R, Shroff D, Kapoor S, Nigam S, Narula R, Chauhan D, Jain P
Indian J Ophthalmol 2007 Jan-Feb;55(1):55-7. doi: 10.4103/0301-4738.29496. PMID: 17189888
Ticho U, Lahav M, Ivry M
J Pediatr Ophthalmol Strabismus 1979 May-Jun;16(3):183-5. doi: 10.3928/0191-3913-19790501-12. PMID: 110915

Prognosis

Gautam Seth N, Kaur S, Yangzes S, Jugran D, Bansal R, Gupta V, Dogra MR, Suri D, Singh S, Singh R
Ocul Immunol Inflamm 2021 Nov 17;29(7-8):1375-1380. Epub 2020 Jul 10 doi: 10.1080/09273948.2020.1762897. PMID: 32649841
Selvan H, Brar AS, Angmo D
Cont Lens Anterior Eye 2019 Aug;42(4):467-469. Epub 2019 May 17 doi: 10.1016/j.clae.2019.04.014. PMID: 31104851
Yang P, Sun M
Cornea 2011 Dec;30(12):1336-40. doi: 10.1097/ICO.0b013e31820f774c. PMID: 21934492
Arora R, Shroff D, Kapoor S, Nigam S, Narula R, Chauhan D, Jain P
Indian J Ophthalmol 2007 Jan-Feb;55(1):55-7. doi: 10.4103/0301-4738.29496. PMID: 17189888
Klaassen-Broekema N, Van Bijsterveld OP
Eur J Ophthalmol 1994 Apr-Jun;4(2):126-9. doi: 10.1177/112067219400400210. PMID: 7950338

Clinical prediction guides

Gupta SN, Goel R, Saran RK, Rathie N
Nepal J Ophthalmol 2021 Jul;13(24):225-229. doi: 10.3126/nepjoph.v13i2.30629. PMID: 35996789
Shah R, Byanju R, Pradhan S
Nepal J Ophthalmol 2018 Jul;10(20):124-129. doi: 10.3126/nepjoph.v10i2.23012. PMID: 31056554
Yang P, Sun M
Cornea 2011 Dec;30(12):1336-40. doi: 10.1097/ICO.0b013e31820f774c. PMID: 21934492
Sharma N, Mannan R, Sinha R, Kaushal S, Titiyal JS, Kumar A, Vajpayee RB
Eye Contact Lens 2011 Sep;37(5):282-5. doi: 10.1097/ICL.0b013e31821269f1. PMID: 21455078
Klaassen-Broekema N, Van Bijsterveld OP
Eur J Ophthalmol 1994 Apr-Jun;4(2):126-9. doi: 10.1177/112067219400400210. PMID: 7950338

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