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Year : 2005  |  Volume : 53  |  Issue : 4  |  Page : 276-277

Isolated foveal hypoplasia detected by optical coherence tomography

Retina–Vitreous Service, Aravind Eye Hospital and Postgraduate Institute of Ophthalmology, Madurai, Tamilnadu, India

Correspondence Address:
Vasumathy Vedantham
Retina–Vitreous Service, Aravind Eye hospital and Postgraduate Institute of Ophthalmology, 1, Anna Nagar, Madurai – 625 020, Tamilnadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.18914

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How to cite this article:
Vedantham V. Isolated foveal hypoplasia detected by optical coherence tomography. Indian J Ophthalmol 2005;53:276-7

How to cite this URL:
Vedantham V. Isolated foveal hypoplasia detected by optical coherence tomography. Indian J Ophthalmol [serial online] 2005 [cited 2020 Oct 20];53:276-7. Available from: https://www.ijo.in/text.asp?2005/53/4/276/18914

Foveal hypoplasia is a condition in which the fovea is underdeveloped and is characterised by the absence of foveal pigmentation and/or the foveal avascular zone. It may occur in isolation or in association with other conditions such as aniridia, albinism, achromatopsia, microphthalmos, retinopathy of prematurity (ROP), myopia and incontinentia pigmenti (IP). [1],[2],[3],[4] The clinical diagnosis might often be missed due to the subtle nature of the fundus findings. This is a case report of isolated foveal hypoplasia in which optical coherence tomography (OCT) was used to confirm the clinical suspicion of foveal hypoplasia in a young girl.

  Case Report Top

A 15-year-old girl was referred to the retina-vitreous service of our tertiary eye care hospital with the complaints of defective vision since childhood. There was no history of day or night blindness, photophobia or premature birth. There was no positive family history. Systemic examination was unremarkable. The best-corrected visual acuity was 6/60, N12 in either eye (+1.00 Dsph right eye, and +1.00 DSph with +0.50 Dcyl 90° left eye). There was pendular nystagmus and the pupillary responses were brisk. There was no iris transillumination suggestive of ocular albinism. Fundus examination revealed normal optic nerve heads and absent foveal reflexes and macular pigmentation along with a large vessel crossing the horizontal meridian across the presumed fovea in the left eye [Figure - 1]A, B. Fundus fluorescein angiography [Figure - 1]C, D revealed an almost absent capillary-free zone in both the eyes, with the vessels running abnormally close to the presumed fovea. The intensity of the choroidal fluorescence emitted from the presumed macular area was similar to that of the other areas, in all probability due to absence of the macular pigments that normally would mask it. Colour vision was normal (as tested with Ishihara's colour plates). Fields could not be tested due to nystagmus.

A provisional diagnosis of foveal hypoplasia was made. The electroretinography (UTAS 3000, LKC Technologies) was within normal limits and this ruled out achromatopsia. OCT ( STRATUS OCTTM Model 3000, Carl Zeiss Meditech technologies) was carried out which showed absence of the foveal depression and persistent inner retinal layers in the fovea, similar to a paramacular scan [Figure - 2]A, B. The scan was carried out after administration of retrobulbar block in both the eyes, on two separate days after informed consent. This was done to ensure a precise focusing of the line scan through the presumed foveal area. Examination of the parents did not show such abnormality. The patient was referred to the low-visual aids department for further management.

  Discussion Top

There were no signs of regressed ROP, myopia, cataract, corneal pannus, ocular albinism or IP as has been reported previously[1],[2],[3],[4] in association with the foveal hypoplasia in our patient. The fact that our patient was a female is also against the possibility of X-linked ocular albinism. In addition, our patient did not have symptoms of photophobia and defective colour vision and flat photopic responses that would suggest achromatopsia.

The role of fluorescein angiography and OCT in the diagnosis of this case was also obvious. There was preservation of the inner retinal layers in the foveae of both the eyes as demonstrated by the OCT. This fact has been corroborated in a previous histopathologic report.[5] In addition, the findings were similar to the only other previous OCT report of this condition by Recchia et al.[2] There was however, no indication of the transient layer of Chievitz (a prominent acellular zone that subdivides the inner nuclear layer and is considered to be the main sign of foveal immaturity)[6] on OCT in this case. We also could not establish any definite pattern of inheritance as has been reported previously.[4]

This report illustrates the importance of careful examination of the foveal area in patients with unexplained visual loss with nystagmus that does not fit into the other well-known dystrophies. The report also highlights the role of OCT in the diagnosis of this condition, by the demonstration of the preservation of the inner retinal layers in the fovea.

  References Top

Oliver MD, Dotan SA, Chemke J, Abraham FA. Isolated foveal hypoplasia. Br J Ophthalmol 1987;71:926-30.  Back to cited text no. 1
Recchia FM, Carvalho-Recchia CA, Trese MT. Optical coherence tomography in the diagnosis of foveal hypolasia. Arch Ophthalmol 2002;120:1587-8.  Back to cited text no. 2
Chen SD, Hanson R, Hundal K. Foveal hypolasia and other ocular signs: A possible case of Incontinentia pigmenti? Arch Ophthalmol 2003;121:921.  Back to cited text no. 3
O'Donnell FE Jr, Pappas HR. Autosomal dominant foveal hypoplasia and presenile cataracts: a new syndrome. Arch Ophthalmol 1982;100:279-81.  Back to cited text no. 4
Duke-Elder S. System of ophthalmology . In : Green WR, editor. Mosby: St.Louis; 1963. p. 652-3.  Back to cited text no. 5
Mann I. The development of the human eyes, 3rd edn. British Medical Association: London; 1964.  Back to cited text no. 6


  [Figure - 1], [Figure - 2]

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