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A Case Report of Vogt's Limbal Girdle and Retinitis Pigmentosa in a Thirteen-Year-Old Boy: A Rare and Unusual Association.

Vignesh AP, Srinivasan R, Karanth S, Vijitha S - Case Rep Ophthalmol (2015)

Bottom Line: Vogt's limbal girdle is a corneal degeneration usually seen in elderly individuals.It has also never been reported at such a young age.This gives further insight into the pathogenesis of the disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.

ABSTRACT

Aim: To describe a rare case of Vogt's limbal girdle in a boy with retinitis pigmentosa.

Methods: A 13-year-old boy from India presented to us with progressive diminution of vision and nyctalopia for 5 years. On examination, he had the characteristic features of retinitis pigmentosa with the fundus showing disc pallor, bony spicules and arteriolar attenuation. His anterior segment examination showed Vogt's limbal girdle in both eyes.

Results: Vogt's limbal girdle is a corneal degeneration usually seen in elderly individuals. This is the first time it is seen in association with retinitis pigmentosa. It has also never been reported at such a young age.

Conclusion: We report a rare case where Vogt's limbal girdle was observed in a 13-year-old boy with retinitis pigmentosa. This gives further insight into the pathogenesis of the disease.

No MeSH data available.


Related in: MedlinePlus

Fundus photograph showing features of retinitis pigmentosa.
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Figure 1: Fundus photograph showing features of retinitis pigmentosa.

Mentions: A 13-year-old Indian boy presented to us with progressive diminution of vision in both the eyes for 5 years. He also complained of poor vision at night. No associated systemic anomaly or syndromic association was found. His father was a diagnosed case of retinitis pigmentosa. The patient was positive for a RHO gene mutation. His vision was 20/200 in both eyes. On examination, his fundus showed typical features of retinitis pigmentosa with disc pallor, bony spicules and arteriolar attenuation (fig. 1). The findings were bilateral and symmetrical. His electroretinogram showed extinguished cone and rod responses in both the eyes (fig. 2). Visual fields were severely constricted (fig. 3). On examination of his anterior segment, we found a bilateral symmetrical crescentic white band in the interpalpebral limbus suggestive of Vogt's limbal girdle type 2 (fig. 4). He also had posterior subcapsular cataract in both eyes. Optical coherence tomography revealed a mild degree of foveal atrophy in the right eye (fig. 5). The patient's loss of vision could be attributed to the reduced photoreceptor function, cataract and foveal atrophy.


A Case Report of Vogt's Limbal Girdle and Retinitis Pigmentosa in a Thirteen-Year-Old Boy: A Rare and Unusual Association.

Vignesh AP, Srinivasan R, Karanth S, Vijitha S - Case Rep Ophthalmol (2015)

Fundus photograph showing features of retinitis pigmentosa.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4608640&req=5

Figure 1: Fundus photograph showing features of retinitis pigmentosa.
Mentions: A 13-year-old Indian boy presented to us with progressive diminution of vision in both the eyes for 5 years. He also complained of poor vision at night. No associated systemic anomaly or syndromic association was found. His father was a diagnosed case of retinitis pigmentosa. The patient was positive for a RHO gene mutation. His vision was 20/200 in both eyes. On examination, his fundus showed typical features of retinitis pigmentosa with disc pallor, bony spicules and arteriolar attenuation (fig. 1). The findings were bilateral and symmetrical. His electroretinogram showed extinguished cone and rod responses in both the eyes (fig. 2). Visual fields were severely constricted (fig. 3). On examination of his anterior segment, we found a bilateral symmetrical crescentic white band in the interpalpebral limbus suggestive of Vogt's limbal girdle type 2 (fig. 4). He also had posterior subcapsular cataract in both eyes. Optical coherence tomography revealed a mild degree of foveal atrophy in the right eye (fig. 5). The patient's loss of vision could be attributed to the reduced photoreceptor function, cataract and foveal atrophy.

Bottom Line: Vogt's limbal girdle is a corneal degeneration usually seen in elderly individuals.It has also never been reported at such a young age.This gives further insight into the pathogenesis of the disease.

View Article: PubMed Central - PubMed

Affiliation: Department of Ophthalmology, Jawaharlal Institute of Postgraduate Medical Education and Research (JIPMER), Pondicherry, India.

ABSTRACT

Aim: To describe a rare case of Vogt's limbal girdle in a boy with retinitis pigmentosa.

Methods: A 13-year-old boy from India presented to us with progressive diminution of vision and nyctalopia for 5 years. On examination, he had the characteristic features of retinitis pigmentosa with the fundus showing disc pallor, bony spicules and arteriolar attenuation. His anterior segment examination showed Vogt's limbal girdle in both eyes.

Results: Vogt's limbal girdle is a corneal degeneration usually seen in elderly individuals. This is the first time it is seen in association with retinitis pigmentosa. It has also never been reported at such a young age.

Conclusion: We report a rare case where Vogt's limbal girdle was observed in a 13-year-old boy with retinitis pigmentosa. This gives further insight into the pathogenesis of the disease.

No MeSH data available.


Related in: MedlinePlus