Thiel-Behnke dystrophy

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Corneal dystrophy of Bowman layer, type II
Classification and external resources
OMIM 602082

Thiel-Behnke dystrophy, or Corneal dystrophy of Bowman layer, type II, is a rare form of corneal dystrophy affecting the layer that supports corneal epithelium. The dystrophy was first described in 1967[1] and initially suspected to denote the same entity as the earlier-described Reis-Bucklers dystrophy, but following a study in 1995 by Kuchle et al. the two look-alike dystrophies were deemed separate disorders.[2]

Genetics[edit]

Some cases of it are linked to chromosome 10q24, others stem from a mutation in the TGFBI gene.[3]

Clinical presentation[edit]

To clarify whether Thiel-Behnke corneal dystrophy is a separate entity from Reis-Bucklers corneal dystrophy, Kuchle et al. (1995) examined 28 corneal specimens with a clinically suspected diagnosis of corneal dystrophy of the Bowman layer by light and electron microscopy and reviewed the literature and concluded that 2 distinct autosomal dominant corneal dystrophy of Bowman layer (CBD) exist and proposed the designation CDB type I (geographic or 'true' Reis-Bucklers dystrophy) and CDB type II (honeycomb-shaped or Thiel-Behnke dystrophy). Visual loss is significantly greater in CDB I, and recurrences after corneal transplantation seem to be earlier and more extensive in CDB I.[3]

See also[edit]

References[edit]

  1. ^ Thiel HJ, Behnke H (1967). "[A hitherto unknown subepithelial hereditary corneal dystrophy]". Klin Monatsbl Augenheilkd (in German) 150 (6): 862–74. PMID 5301630. 
  2. ^ Küchle M, Green WR, Völcker HE, Barraquer J (July 1995). "Reevaluation of corneal dystrophies of Bowman's layer and the anterior stroma (Reis-Bücklers and Thiel-Behnke types): a light and electron microscopic study of eight corneas and a review of the literature". Cornea 14 (4): 333–54. doi:10.1097/00003226-199507000-00001. PMID 7671605. 
  3. ^ a b Online 'Mendelian Inheritance in Man' (OMIM) 602082