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Letters to the Editor

Bilateral Simultaneous Anterior and Posterior Lenticonus in Alport's Syndrome

Vedantham, Vasumathy MS; Rajagopal, Jyothsna MS; Ratnagiri, Praveen Krishna DNB

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Indian Journal of Ophthalmology: Jul–Sep 2005 - Volume 53 - Issue 3 - p 212-213
doi: 10.4103/0301-4738.16691
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Dear Editor,

Anterior lenticonus is considered a characteristic sign of Alport's syndrome.12 However, anterior and posterior lenticonus in the same eye in Alport's syndrome is rare and there have been only a few such reports. We report a case of classic Alport's syndrome with bilateral combined anterior and posterior lenticonus.

A thirty-two year old male patient presented to our tertiary eye care hospital with difficulty in reading and close work for the last five years. Ocular examination revealed a best corrected visual acuity in both eyes to be 6/24, N12. Anterior segment examination revealed normal corneas and anterior and posterior lenticonus in both eyes with posterior subcapsular cataract in the right eye (Figure 1a and b). Distant direct ophthalmoscopy showed the oil droplet reflex in both eyes. Fundus examination was normal with no evidence of a fleck retinopathy. Electroretinography (UTAS 3000, LKC Technologies) was normal in both the eyes. Based on a clinical suspicion of Alport's syndrome, the patient was questioned following which he revealed that he had had progressive deafness and hematuria from childhood and had undergone renal transplant eight years ago for chronic renal failure. Systemic examination revealed perceptive high-tone deafness. A diagnosis of classical Alport's syndrome was made based on the history and clinical features which fulfilled three diagnostic criteria (renal involvement, deafness and lenticonus) of Alport's syndrome, as suggested previously.2 The patient was advised to undergo cataract surgery with intraocular lens implantation in the right eye at first, but the patient refused due to financial constraints. He was referred to the low-visual aids department but was subsequently lost to follow-up.

Figure 1a
Figure 1a:
(Top): Slit lamp photograph (VISUPAC Digital image archiving system, Carl Zeiss Meditech Technologies) of the right eye of the patient showing anterior lenticonus (white arrow) and posterior lenticonus and posterior subcapsular cataract (white arrow head). Figure 1b (bottom): Slit lamp photograph of the left eye of the patient showing anterior lenticonus (white arrow) and posterior lenticonus (white arrow head).

Classic Alport's syndrome is an X linked disorder characterised by a triad of progressive hematuric nephritis, progressive perceptive high-tone hearing loss, and ocular signs1. Although anterior lenticonus, posterior polymorphous dystrophy, and superficial perimacular flecks are the most characteristic ocular signs, rare lenticular abnormalities reported in patients with Alport's syndrome include several other types of cataracts, spherophakia, and posterior lenticonus.1234 However, the occurrence of anterior and posterior lenticonus in the same eye is very rare. Our patient had bilateral simultaneous anterior and posterior lenticonus. MEDLINE search revealed only very few such previous reports.345 While Nielson3 and Bhatnagar et al4 have reported bilateral anterior and posterior lenticonus in classical Alport's syndrome, Sukhija et al5 have reported such a finding in the autosomal recessive variant. In fact, it has been suggested that while lenticonus anterior is part of the Alport's syndrome lenticonus posterior is not associated with systemic disease.6 Our case serves to illustrate the fact that both anterior and posterior lenticonus may be present simultaneously in classical Alport's syndrome, even in the absence of corneal and retinal findings. The importance of cataract surgery in such eyes is the careful capsulorrhexis that has to be carried out as suggested by Sukhija et al5 and John et al.7 A planned cataract surgery would also avoid complications such as spontaneous rupture of the anterior lens capsule as noted previously in such eyes.8

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2. Junk AK, Stefani FH, Ludwig K. Bilateral anterior lenticonus: Scheimpflug imaging system documentation and ultrastructural confirmation of Alport syndrome in the lens capsule Arch Ophthalmol. 2000;118:895–7
3. Nielsen CE. Lenticonus anterior and Alport's syndrome Acta Ophthalmol. 1978;56:518–30
4. Bhatnagar R, Kumar A, Pakrasi S. Alport's syndrome-ocular manifestations and unusual features Acta Ophthalmol (Copenh). 1990;68:347–9
5. Sukhija J, Saini JS, Jain AK. Phacoemulsification and intraocular lens implantation in an Alport's syndrome patient with bilateral anterior and posterior lenticonus J Cataract Refract Surg. 2003;29:1834–6
6. Jacobs K, Meire FM. Lenticonus Bull Soc Belge Ophtalmol. 2000;277:65–70
7. John ME, Noblitt RL, Coots SD, Boleyn KL, Ballew C. Clear lens extraction and intraocular lens implantation in a patient with bilateral anterior lenticonus secondary to Alports syndrome J Cataract Refract Surg. 1994;20:652–5
8. Pelit A, Oto S, Yilmaz G, Akova YA. Spontaneous rupture of the anterior lens capsule combined with macular hole in a child with Alports syndrome J Pediatr Ophthalmol Strabismus. 2004;41:59–61
© 2005 Indian Journal of Ophthalmology | Published by Wolters Kluwer – Medknow