Br J Ophthalmol 1998;82:1215–1219 1215 LETTERS TO THE EDITOR Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from Idiopathic keratoconus in a patient with leucoma, iris atrophy and hypoplasia, and therapy. His past medical history was signifi- congenital rubella syndrome glaucoma.2 Keratoconus and hydrops have cant for non-insulin dependent diabetes melli- been reported in patients with CRS with a tus, systemic hypertension, hyperlipidaemia, EDITOR,—Keratoconus is very rarely found in history of mental retardation and eye and obesity. patients with congenital rubella syndrome rubbing.1 At presentation, the patient complained of (CRS). The only known aetiology of kerato- The microtrauma associated with eye rub- pain and redness in his left eye. Visual acuity conus associated with CRS is eye rubbing.1 bing is generally thought to be the aetiological was 20/25 in the right eye and light perception We report here a patient with CRS who devel- link between keratoconus and associated in the left eye. Examination of the right eye oped idiopathic keratoconus. systemic and ocular diseases.3-5 All four disclosed only mild diabetic retinopathy. Slit patients with keratoconus and CRS described lamp examination of the left eye showed 1 CASE REPORT by Boger et al were retarded and vigorously hyperaemia of the conjunctiva, and an intense A 24 year old woman with CRS was examined rubbed and poked their eyes. These authors milky flare obstructing the view of the iris and for progressive visual blurring of 2 years’ postulated that keratoconus and acute corneal the fundus (Fig 1). It was diYcult to detect the hydrops probably resulted from chronic trau- duration in her left eye. The diagnosis of CRS presence of cells in the anterior chamber matising mannerisms common in other men- was based on the maternal history of rash and because of the flare. There were neither tally retarded patients5 and is not specific to fever in the first trimester of pregnancy, keratic precipitates nor hypopyon or iris congenital rubella. Thus far, this is the only cataract, and microphthalmia in the right eye, neovascularisation. Intraocular pressures in known aetiology of keratoconus associated bilateral rubella retinopathy, nystagmus, deaf- both eyes were within normal limits. B-scan with CRS. ness, congenital heart disease. ultrasonography was unremarkable. In the case presented here the keratoconus Available medical records dated from the Results of laboratory tests showed hypergly- appeared to be idiopathic. Indeed, there was time she was 1 year old. At that time, caemia (24 mmol/l), a twofold increase in nor- no history of eye rubbing or atopy, or family extracapsular cataract surgery was performed mal cholesterol levels (15 mmol/l), and a history of keratoconus. To the best of our 22-fold increase in normal triglyceride levels in the right eye. She had esotropia in the right knowledge, this report is unique in as far as (38 mmol/l). Results of laboratory examina- eye and poor fixation. Apart from pigmentary idiopathic keratoconus has never been associ- tion including HLA typing, angiotensin con- changes in the fundus, the left eye was normal. ated with CRS. It is likely that in this case verting enzyme, serologies for herpes simplex A follow up examination when she was 15 there is no such association but merely an virus (HSV) and lues, purified protein deriva- documented nystagmus, esotropia, and micro- 67 overlap of two uncommon conditions occur- tive (PPD) skin test, and chest and sacroiliac phthalmia in her right eye, and bilateral ring in the same person. An association rays, were not contributory. A and pre- rubella retinopathy. Visual acuity was RE light x â â between idiopathic keratoconus and CRS may lipoproteinaemia pattern was established by perception; LE 6/9 (with −4.0 −1.0 c × 180). be determined only after other confirmatory serum electrophoresis. Analysis of the aque- Two other examinations at the ages of 18 and cases have been reported in the literature. ous humour of the left eye disclosed no cells, 21 gave similar results. and high levels of proteins (32 g/l), cholesterol On present examination, visual acuity with ANTONIO PINNA FRANCESCO CARTA (3.4 mmol/l), and triglycerides (4.9 mmol/l). spectacles was LE 6/60. Retinoscopy revealed Visual acuity recovered at 20/50 and the high, irregular myopic astigmatism with scis- Institute of Ophthalmology, University of Sassari, Sassari, Italy flare resolved after lipidaemia and diabetes soring of the red reflex. Central keratometry had been controlled by oral fibrates and insu- showed irregular mires which could not be Correspondence to: Dr Antonio Pinna, Istituto di Clinica Oculistica, Universita degli Studi di Sassari, lin, respectively. Fundus examination of the superimposed; 4 dioptre irregular astigmatism left eye showed mild diabetic retinopathy was found. A conical reflection on the nasal Viale San Pietro 43 A, 07100 Sassari, Italy. Accepted for publication 21 April 1998 including microaneurisms, hard exudates, and http://bjo.bmj.com/ cornea was obtained by shining a penlight many paravascular retinal haemorrhages. Six from the temporal side (Rizzuti’s sign). weeks later, fundus examination of the left eye Bulging of the lower eyelid on downgaze was 1 Boger WP III, Petersen RA, Robb RM. Kerato- showed evidence of central retinal venous conus and acute hydrops in mentally retarded also observed (Munson’s sign). Slit lamp occlusion (CRVO). examination revealed an inferiorly located patients with congenital rubella syndrome. Am J Ophthalmol 1981;91:231–3. ectatic protrusion of the central cornea with 2 Wolf SM. The ocular manifestations of congeni- COMMENT reticular anterior stromal scars. As a result, the tal rubella. J Pediatr Ophthalmol 1973;10:101– diagnosis of mild keratoconus was made. 41. Although clinical presentation including pain, 3 Karseras AG, Ruben M. Aetiology of kerato- perilimbal conjunctival hyperaemia, and ante- Treatment with a Softperm contact lens was on October 2, 2021 by guest. Protected copyright. conus. Br J Ophthalmol 1976;60:522–5. rior chamber flare, was consistent with acute successful and visual acuity was again LE 6/9. 4 Rahi A, Davies P, Ruben M, et al. Keratoconus All other ocular findings were the same as in and coexisting atopic disease. Br J Ophthalmol anterior uveitis, many features favoured a earlier observations. 1977;61:761–4. strong relation between the metabolic disor- 5 Pierce C, Eustace P. Acute keratoconus in mon- ders and the occurrence of the flare. These The patient attended a special school for gols. Br J Ophthalmol 1971;55:50–4. the deaf and dumb, learning sign language 6 Feder RS. Noninflammatory ectatic disorders. clues included the milky appearance of the flare, the results of the anterior chamber para- and to read and write. There was no history of In: Krachmer JH, Mannis MJ, Holland EJ, eds. Cornea. St Louis: Mosby-Year Book, 1996: centesis, the lack of response to topical eye rubbing or atopy, or a family history of 1091–106. keratoconus. Serum total IgE (34 IU/ml) and 7 Centers for Disease Control. Rubella and blood eosinophils (1.1%) were normal. congenital rubella—United States, 1984–1986. MMWR 1987;36:664–75. COMMENT Congenital rubella produces a spectrum of Pseudouveitis as a manifestation of ocular and systemic abnormalities. Infection hyperlipidaemia during the first trimester of pregnancy is more serious, with the virus disrupting organogen- EDITOR,—We report a case of unilateral esis and diVerentiation. Many fetal tissues are anterior pseudouveitis in a diabetic retino- susceptible to infection leading to the abnor- pathic eye, as a manifestation of hyperlipidae- malities found in the CRS—deafness, cardiac mia. High levels of lipids were detected in the malformations, bone and dental abnormali- aqueous humour and the anterior flare ties, ocular malformations, and mental retar- resolved only after successful control of dation. diabetes and hyperlipidaemia was obtained. “Salt and pepper” retinopathy is the most common ocular disorder; other ocular mani- CASE REPORT Figure 1 Slit lamp photograph of the left eye festations include microphthalmia, strabis- A 44 year old man was referred for anterior showing an intense milky flare in the anterior mus, congenital cataract, transient corneal uveitis not responsive toa5daytopical steroid chamber. 1216 Letters steroids, and the resolution of the flare only lymphocytic pleocytosis (proteins 0.37 g/l, and Asian indians) and is uncommon in Br J Ophthalmol: first published as 10.1136/bjo.82.10.1215 on 1 October 1998. Downloaded from after the control of hyperlipidaemia. white cells 77×106/l, lymphocytes 96%), with- whites.1 Our patient was Portuguese and had To our knowledge, only one case of out oligoclonal bands on electrophoresis. A these clinical features. Most patients are in pseudoendophthalmitis related to hyperlipi- magnetic resonance cerebral scan was normal. their second to fourth decade of life at onset of daemia has been published.1 Both eyes were Suspecting a herpetic infection, aciclovir (900 the disease; nevertheless, VKH syndrome has involved in a patient with bilateral proliferative mg/8 hours), topical steroids, and prednisone also been reported in children and young diabetic retinopathy. Blood-aqueous barrier (1 mg/kg) were administered on the day of adults.1 breakdown has been demonstrated in diabetic admission. To date no certain aetiological factors has patients, especially those presenting with Further investigations revealed that serol- been reported, but taking into account that diabetic retinopathy,2 and in patients with ogy (cytomegalovirus (CMV), Epstein–Barr this illness aVects various organs (skin, eye, CRVO.3 In the present case, an underlying virus, herpes simplex I/II, varicella zoster central nervous system) a common aetiologi- mild diabetic retinopathy was diagnosed in virus, rubella virus, toxoplasmosis, Borrelia cal factor seems to be involved in the both eyes. The unilateral leakage of lipids in burgdorferi, Treponema pallidum, and HIV) was pathogenesis.