Macular telangiectasia

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Classification according to ICD-10
H35.0 Retinopathies of the fundus and changes in the retinal vessels
ICD-10 online (WHO version 2019)

The macular telangiectasia (correct: macular telangiectasia), often abbreviated MacTel, synonym : Idiopathic juxtafoveolare telangiectasia , is a very rare malformation with abnormal vessels ( telangiectasia ) in the eye in the region of the macula and resultant restriction of vision .

The disease can be viewed as a variant of the congenital telangiectasia of the retina , Coats' disease . It can easily be confused with other macular diseases.

The distinction was made when it was first described in 1968 by the US ophthalmologist J. Donald M. Gass.

Occurrence

The frequency is given as 0.1% with an average age of 64 years for the most common type 2. This is a relatively rare macular disease. The female gender is affected somewhat more often. The disease is usually diagnosed for the first time between the ages of 50 and 69, but it can also occur sooner or later.

classification

The following classification is common:

  • Type 1 , synonyms: aneurysmal telangiectasia; Visible and exudative idiopathic juxtafoveolar retinal telangiectasis , rare, congenital and mostly unilateral, possibly a variant of Coats' disease
  • Type 2 , synonym: MacTel , acquired and bilateral, most common form, mostly in middle or advanced age
  • Type 3 , synonym: Occlusive idiopathic juxtafoveolar retinal telangiectasis , rarely, primary vascular occlusion

In 2006, Yannuzzi et al. Proposed a somewhat simplified classification into:

  • Type 1 "aneurysmal telangiectasia" (type 1)
  • Type 2: "perifoveal telangiectasia"

root cause

Acquired telangiectasia is more often due to diabetes mellitus (28%) and arterial hypertension (52%).

There are autosomal dominant inherited diseases in which the macula can also be affected.

Symptoms

Typical initial symptoms are above all reading problems despite well-adapted visual aids, perception of distortions ( metamorphopsia ), a blurred visual impression or problems with recognizing faces. The visual acuity is initially not or only slightly reduced, but in later stages it can drop to the range of 0.1. The reading problems are often described as disappearing letters.

literature

  • B. Stoffelns, K. Schoepfer, C. Kramann: Idiopathic macular telangiectasia (IMT) - progress monitoring with and without laser photocoagulation. In: Clinical monthly sheets for ophthalmology. 227, 2010, p. 252, doi: 10.1055 / s-0029-1245187 .
  • Emily Y. Chew et al .: Effect of Ciliary Neurotropic Factor on Retinal Neurodegeneration in Patients with Macular Telangiectasia Type 2. In: Ophthalmology Vol. 126, No. 4, April 2019, pp. 540 - 549, doi: org / 10.2016 / j.ophtha.2018.09.041 ISSN 0161-6420 / 18

Individual evidence

  1. ↑ a b Eyewiki
  2. ↑ a b c d T.FC Heeren, E. Krüger, F. Holz, PC Issa: Functional Characteristics in Macular Telangiectasia Type 2 In: Der Ophthalmologe , Volume 111, Number 9, 2014, pp. 829-833.
  3. ↑ JD Gass: A fluorescein angiographic study of macular dysfunction secondary to retinal vascular disease. V. Retinal telangiectasis. In: Archives of ophthalmology. Volume 80, Number 5, November 1968, pp. 592-605, PMID 5684308 .
  4. ^ A b P. Charbel Issa, MC Gillies, EY Chew, AC Bird, TFC Heeren, T. Peto, FG Holz, HPN Scholl: Macular telangiectasia type 2. In: Progress in Retinal and Eye Research. 34, 2013, p. 49, doi: 10.1016 / j.preteyeres.2012.11.002 .
  5. ↑ a b L. A. Yannuzzi, AM Bardal, KB friend KJ Chen, CM Eandi, B. Blodi: Idiopathic macular telangiectasia. 2006. In: Retina. Volume 32 Suppl 1, February 2012, pp. 450-460, PMID 22451954 .
  6. ↑ JD Gass, BA Blodi: Idiopathic juxtafoveolar retinal telangiectasis. Update of classification and follow-up study. In: Ophthalmology. Volume 100, Number 10, October 1993, pp. 1536-1546, PMID 8414413 .
  7. ^ Idiopathic macular telangiectasia type 1. In: Orphanet (database for rare diseases).
  8. ^ Idiopathic macular telangiectasia type 3. In: Orphanet (database for rare diseases).
  9. ↑ Telangiectasia, hereditary benign.  In: Online Mendelian Inheritance in Man . (English)

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