British Journal of Ophthalmology 1997;81:415–420 415

LETTERS TO THE EDITOR Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from

Recurrent uveitis in a patient with adult colony stimulating factor (GM-CSF)7 and CASE REPORTS onset cyclic neutropenia associated with granulocyte-colony stimulating factor (G-CSF).8 Although a direct cause-eVect Case 1 increased large granular CH, a 27-year-old white woman presented association between the recurrent uveitis with a 10 day history of deterioration of vision EDITOR,—A 35-year-old white man was re- present in this patient and adult onset cyclic in her left eye. Seven years previously she had ferred with a 1 month history of recurrent neutropenia cannot be definitively estab- been diagnosed as having HSV encephalitis, bilateral uveitis. lished, the existence of diVerent immune confirmed by electron microscopy and viral The patient had a history of cyclic neutro- system abnormalities (for example, prolifera- culture of a brain biopsy specimen. The penia beginning at age 19. Severe neutropenia tion of large granular cells, inver- × sequelae of HSV encephalitis included right (total neutrophil count as low as 68 cells sion of the CD4:CD8 ratio, presence of diVer- 106/l), lasting about 3 days, was documented homonymous hemianopia. Visual acuities ent autoantibodies) in patients with cyclic were 6/5 right eye and light perception left to occur at the time of clinical symptoms. neutropenia and large granular lymphocytosis Prednisone produced a marked diminution in eye. The right eye was normal. Examination of may contribute to the recurrence of uveitis the left eye revealed intense vitritis, and retinal episodes of symptomatic neutropenia. and its refractoriness to conventional therapy. At age 35, the patient developed iritis in his thickening with extensive peripheral retinal left eye during a ‘crisis’. This was treated with ALEJANDRO RODRIGUEZ necrosis and patches of central retinal necro- 0.1% dexamethasone phosphate solution. Immunology and Uveitis Service, Department of sis. The optic disc was swollen and there was The anterior chamber inflammatory reaction Ophthalmology. Massachusetts Eye and Ear Infirmary, an inferior , extending to subsided, but 1+ cells persisted in the vitreous Harvard Medical School. Boston, MA, USA the macula. There was extensive retinal vascu- despite an injection of methylprednisolone 40 ROBERT A YOOD litis and haemorrhage. ARN was diagnosed on mg into the sub-Tenon’s capsule. The fundus Division of Rheumatology, Fallon Clinic, Worcester, clinical grounds and she was treated with was normal. An extensive immunological MA, USA intravenous acyclovir, 10 mg/kg three times examination and HLA-B27 determination THOMAS J CONDON daily for 8 days, and then oral acyclovir 800 were negative or normal. The uveitis resolved Division of Ophthalmology, Fallon Clinic, Worcester, mg five times daily for 12 weeks. After this as the crisis subsided. Recurrent episodes of MA, USA period the dose was reduced to 400 mg five uveitis in the left eye occurred episodically, but C STEPHEN FOSTER times daily, which she is still taking. always at the time of neutropenic crisis and Immunology and Uveitis Service, Department of Vitreous biopsy and lumbar puncture were despite topical and systemic anti- Ophthalmology, Massachusetts Eye and Ear Infirmary, performed. No was found in the CSF; inflammatory therapy. Harvard Medical School. Boston, MA, USA however, using techniques based on polymer- Development of cataract reduced the visual Correspondence to: C Stephen Foster, MD, Massa- ase chain reaction (PCR) the vitreous specimen acuity to counting fingers at 1 foot. Cataract chusetts Eye and Ear Infirmary, 243 Charles Street, revealed the presence of HSV-1 and VZV extraction with posterior chamber lens im- Boston, MA 02114, USA. DNA. The right eye has remained uninvolved, plantation resulted in stable visual acuity of Accepted for publication 11 December 1996 and the patient has retained the left hemifield of 20/30 (5 year follow up) despite continued vision in the right eye with visual acuity of 6/5. episodes of iritis. 1 Wright DG, Dale DC, Fauci AS, WolV SM. Human. cyclic neutropenia: clinical review and Case 2 COMMENT long-term follow-up of patients. Medicine GP, a 17-year-old white man presented with a 1981;60:1–13. 1 week history of blurring of vision in his left Cyclic neutropenia is a rare disorder character- 2 Dale DC, Hammond WP. Cyclic neutropenia: a eye. Vision in the right eye had been poor since ised by regular oscillations in blood neutrophil clinical review. Blood Rev 1988;2:178–85. http://bjo.bmj.com/ counts, as well as other blood cells.1 Cycling 3 Kawaguchi Y, Kobayashi M, Tanabe A, Hara M, childhood as a result of optic atrophy. HSV occurs regularly every 21 days and lasts 3–6 Nishi Y, Usui T, et al. Granulopoiesis in patients encephalitis had occurred at 1 week of age, days in most of the cases.2–4 The patient with congenital neutropenia. Am J Hematol which was diagnosed clinically and confirmed 1985;20:223–34. described here presented with cyclic episodes 4 Loughran TP Jr, Clark EA, Price TH, Hammond on aspiration of fluid from a skin vesicle. of neutropenia occurring every 18–25 days and WP. Adult-onset cyclic neutropenia is associ- Visual acuities, on presentation, were of lasting for 3–5 days. The patient’s neutrophil ated with increased large granular lymphocytes. counting fingers in both eyes. The right eye counts were repeatedly below 800 cells × 106/l Blood 1986;68:1082–7. revealed sector iris atrophy temporally and 5 Wallis WJ, Loughran TP, Kadin ME, Clark EA, at the lowest and no changes in other leucocyte Starkebaum GA. Polyarthritis and neutropenia optic atrophy. The left eye had significant vit- or platelet counts were observed. associated with circulating large granular lym- ritis, peripheral retinal necrosis, and disc on September 30, 2021 by guest. Protected copyright. During neutropenic episodes, patients may phocytes. Ann Intern Med 1985;103:357–62. swelling. No iris atrophy was present in this experience a wide variety of symptoms, from 6 Wright DG, Fauci AS, Dale DC, WolV,SM. eye and there were no records of optic nerve or Correction of human cyclic neutropenia with malaise and low grade fever to severe life prednisolone. N Engl J Med 1978;298:295–300. peripheral retinal involvement before the cur- threatening gastrointestinal infections and, 7 Kurzrock R, Talpaz M, Gutterman JU. Treat- rent presentation. although there is moderate morbidity associ- ment of cyclic neutropenia with very low doses A clinical diagnosis of ARN was made and ated with the disease, its course is usually of GM-CSF. Am J Med 1991;91:317–8. the patient was treated with intravenous 5 1 8 Migliaccio AR, Migliaccio G, Dale DC, Ham- benign. Conjunctivitis has been described. mond WP. Hematopoietic progenitors in cyclic acyclovir 10 mg/kg three times daily and oral Iridocyclitis associated with neutropenic cy- neutropenia: eVect of granulocyte colony- prednisolone (initially 80 mg/day). Unfortu- cling and refractory to therapy has not been stimulating factor in vivo. Blood 1990;75:1951–9. nately a vitreous sample was not available for described previously. Extensive and repeated PCR analysis. laboratory investigations for an underlying Delayed acute retinal necrosis after After 1 week on intravenous acyclovir he cause of the uveitis in our patient were herpetic encephalitis was given a course of oral acyclovir for 12 negative except for large granular lymphocytes weeks (800 mg five times a day) which was and an IgG antineutrophil antibody, both EDITOR,—The herpes virus family, especially then reduced to a maintenance dose of 400 characteristic of cyclic neutropenia. (HSV) and varicella mg five times a day. At his last examination he Many therapeutic strategies have been used zoster virus (VZV), has been associated with had visual acuities of hand movements in his 12 in the management of patients with cyclic acute retinal necrosis (ARN) and these left eye, and there was no inflammatory activ- neutropenia. Splenectomy, testosterone, etio- also represent an important cause of ity in either eye. cholanolone, and endotoxin have been em- encephalitis.3 Cases of in patients with ployed, all to some therapeutic eVect.6 The a history of previous herpetic encephalitis, COMMENT response to alternate day low dose prednisone although rare, have been reported45; a delay Delay between the onset of viral encephalitis therapy has been particularly successful for between the diagnosis of encephalitis and the and ARN has been mentioned previously in patients with the adult onset of the disease.6 onset of ocular symptoms has been reported the literature. Intervals of 17 and 5 months6 Our patient was managed with 5–25 mg of to be as long as from 1 to 5 months,67but an have been reported with the mention in one prednisone every other day, with significantly interval of up to 5 years has been recorded.8 case ofa5yearinterval,8 but without any reduced symptomatology and neutrophil cy- We report here two patients who developed other information regarding the clinical and cling. More recently, correction has been the clinical picture of ARN 7 and 17 years diagnostic aspects of both the encephalitis and reported with granulocyte macrophage- after having had herpetic encephalitis. ARN. In our patients the delays were 7 years 416 Letters and 17 years respectively, and the occurrence 10 Laskin OL. Acyclovir: pharmacology and clinical visual impairment or blindness, if vision did of ARN in these patients was particularly dev- experience. Arch Intern Med 1984;144:1241. not return to normal within 6 days, recovery Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from astating as both had pre-existing visual 11 Palay DA, Sternberg Jr P, Davis J, Lewis H, Hol- was not complete and the vision deteriorated.1 land GN, Mieler WF, et al. Decrease in the risk impairment related to previous encephalitis. of bilateral acute retinal necrosis by acyclovir Other reported cases of methanol intoxication The explanation for such prolonged intervals therapy. Am J Ophthalmol 1991;112:250–5. also demonstrated the guarded prognosis of include the possibility of persistent latent virus visual loss.2 Thus, prolonged visual malfunc- in the CNS with spread to the eye via the optic Late treatment of methanol blindness tion is considered an unfavourable prognostic nerve, or the presence of latent virus in the sign, and vision almost never fully recovers. ocular tissue since the time of primary EDITOR,—Methanol intoxication is a severe Delayed recovery is rare, and usually not com- infection. Cases with asymptomatic peripheral and often blinding disease. Irreversible visual plete. Scrimgeour et al 3 reported a patient chorioretinal lesions have been reported, and loss may occur within hours to days after who, after 12 days of blindness, regained these lesions were felt to be an indication of ingestion. Treatment regimens are aimed at vision and after 12 weeks his vision was hand persistence of the virus in the retina.9 Treat- controlling acid-base balance, removing the movements in one eye and 10/20 in the other ment in the acute phase is by systemic acyclo- toxic agent by haemodialysis, or using ethanol eye. One case, reported by Buzna et al, finally vir, which has a potent antiviral action against solution as a competitive drug. If treatment is recovered to 10/20 in one eye and 2/20 in the varicella zoster and herpes simplex. Acyclovir delayed beyond the first hours after ingestion, other after treatment with ethanol and various is only eVective against actively replicating permanent damage to the optic pathways may vitamins including vitamin B-1.4 The present virus,10 and cannot eradicate latent virus, such occur. We report on successful treatment of case is an extreme exception since the patient that any protective eVect is questionable.11 methanol (methyl alcohol) induced blindness experienced almost absolute blindness (NLP The risk of further reactivation of the virus in with late steroids and vitamin B-1. and CF) for at least 4 days, as late as 15 days our patients is unclear. For this reason, and after methanol was first ingested, and yet especially taking into consideration the severe CASE REPORT recovered to almost perfect vision of 20/20 in visual sequelae from their herpetic encephali- A 40-year-old man was admitted to our eye both eyes. An interval between methanol con- tis and the further loss of vision after the department with sudden bilateral visual loss. sumption and deterioration of vision is typical. episode of ARN, both patients have been He was generally healthy except that he was a Therefore, severe visual loss in a young and maintained indefinitely on low dose acyclovir heavy smoker and consumed alcohol regu- apparently healthy individual should raise a (400 mg five times a day). larly. During a period of 4–10 days before suspicion of methanol intoxication, even if it Considering that significant visual loss usu- admission, the patient consumed pure metha- was consumed several days before presenta- ally results from retinal and optic nerve nol used for cleaning metal electrical wires. tion of symptoms. During hospitalisation our involvement as a late sequela of congenital One day before admission, the patient com- patient was under close supervision, he was HSV infection and, also, potentially from plained of vomiting, nausea, and abdominal practically blind and totally dependent, there- HSV encephalitis, the need for prophylactic pain; however, a complete medical examin- use of systemic antiviral drugs has to be ation in the emergency room was inconclusive considered. The presence of retinal lesions in and his vision was not aVected. On admission, 2 µV/D L 38 ms/D 2 µV/DR 38 ms/D the retinal periphery in cases of congenital physical and neurological examination were 388.8 ms 388.8 ms HSV infection, as described by others,9 may normal. Ophthalmic examination revealed indicate a higher risk for later ocular involve- visual acuity of hand movements in the right ment, and may be used as a marker for use of eye and 50 cm counting fingers in the left eye. prophylactic therapy. The need of prophylaxis Ocular examination was normal except for after encephalitis may be more diYcult to mild dilatation of the pupils which reacted establish, but should be considered in cases of sluggishly to light. No funduscopic abnor- 210 visual loss due to cortical involvement. malities were observed. One day after admis- sion, visual acuity deteriorated to no light per- C E PAVÉSIO A D K CONRAD ception in the right eye and remained as P J MCCLUSKEY counting fingers in the left eye, with a large http://bjo.bmj.com/ S M MITCHELL central scotoma. Relative aVerent papillary HMATOWLER 145 defect was detected in the right eye. Visual 84 78 S LIGHTMAN evoked response (VER) was undetectable in 130 Department of Clinical Ophthalmology, the right eye and prolong latency was detected Moorfields Eye Hospital, Institute of Ophthalmology, City Road, London EC1V 2PD in the left eye (Fig 1A). Computed tomogra- 111 118 phy of the brain and an electroencephalogram Correspondence to: Professor S Lightman, Depart- ment of Clinical Ophthalmology, Moorfields Eye were normal. Blood laboratory tests were nor- mal with no evidence of metabolic acidaemia. B Hospital, City Road, London EC1V 2PD. on September 30, 2021 by guest. Protected copyright. Accepted for publication 11 December 1996 Nine to 15 days after methanol ingestion, no significant recovery was evident. The conven- 160 1 Culbertson WW, Blumenkrantz MS, Pepose JS, Stewart JA, Curtin VT. is a tional treatment with bicarbonate was longer not appropriate because the patient’s acid- 76 cause of the acute retinal necrosis syndrome. 116 183 Ophthalmology 1986;93:559–69. base balance was normal. Furthermore, the 2 Lewis ML, Culbertson WW, Post JP, Miller D, treatments with ethanol or haemodialysis were Kokame GT, Dix RD. Herpes simplex virus 107 type 1. A cause of the acute retinal necrosis syn- too late to be eVective. Medical therapy was drome. Ophthalmology 1989;96:875. initiated including prednisone 60 mg/day by 3GriYth JF, Ch’ien LT. Herpes simplex virus mouth and an intramuscular injection of vita- C 145 encephalitis. Diagnostic and treatment consid- min B-1 100 mg/day. Three days after initiat- erations. Med Clin North Am 1983;67:991– ing the treatment, visual acuity, visual field 1008. Figure 1 Visual evoked response (VER) with 4 Johnson BL, Wisotzkey HM. Neuroretinitis analysis, and VER showed evidence of signifi- pattern reversal recorded during the acute phase, associated with herpes simplex encephalitis in cant improvement in both eyes (Fig 1B). The an adult. Am J Ophthalmol 1977;83:381–9. initial therapy, and after recovery. (A) Day 1, 5 Bloom JN, Katz JL, Kaufman HE. Herpes patient was treated with vitamin B-1 injections visual acuity, right eye no light perception, left eye simplex retinitis and encephalitis in an adult. for 20 days and with oral prednisone for 1 counting fingers 50 cm. No structured pattern Arch Ophthalmol 1977;95:1798–9. month with gradual tapering oV. Seven weeks was recorded from the right eye and prolonged 6 Ahmadieh H, Sajjadi H, Azarmina M, Kalani H. later the visual acuity had fully recovered in latency was recorded over the left eye with a very Association of herpetic encephalitis with acute low amplitude. These recordings indicate a retinal necrosis syndrome. Ann Ophthalmol both eyes to the 20/20 level. Visual fields of 1991;23:215–9. both eyes showed only slight concentric non-functioning right optic pathway and severe 7 Gartry DS, Spalton DJ, Tilzey A, Hykin PG. constriction with no scotoma; VER demon- damage to the left optic nerve. (B) Day 7, after 3 days of treatment, visual acuity was right eye Acute retinal necrosis syndrome. Br J Ophthal- strated normal patterns on both eyes (Fig 1C). mol 1991;75:292–7. hand movement and left eye 20/100. A definitive 8 Boer JH, Luyendijk L, Rothova A, Baarsma GS, pattern is evident from both sides, however, de Jong PTVM, Bollemeijer J-G, . Detection et al COMMENT latency is still prolonged corresponding to the of intraocular antibody production to herpes- recovery process of the optic pathways. (C) Seven viruses in acute retinal necrosis syndrome. Am J Methanol intoxication may be associated with 1994;117:201–10. weeks after methanol blindness visual acuity Ophthalmol severe damage to the visual pathways, poten- recovered in both eyes to 20/20. VER over the 9 el Azazi M, Malm G, Forsgren M. Late ophthal- tially leading to permanent blindness. In an mologic manifestations of neonatal herpes right eye demonstrates a residual prolonged simplex virus infection. Am J Ophthalmol 1990; outbreak of methanol intoxication involving latency whereas the VER pattern of the left eye 109:1–7. 320 people, Benton et al stated that in cases of returned to normal. Letters 417 fore we assume that he did not consume alco- hol during this period—that is, ethylism did Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from not play a role in his clinical symptoms. The rationale for using steroids in methanol toxic neuropathy was based on the clinical experience with this drug being eVective in other forms of optic neuropathies. The poten- tial therapeutic eVect of steroids on methanol optic neuropathy might be a reduction in the oedema of the nerve sheaths caused by the histotoxic anoxia as suggested by Sharpe et al.5 Steroids are usually not used to treat ill effects of ethylism. Vitamin B-1 is given for treatment of Wernike–KorsakoV syndrome, following prolonged ethanol consumption. We assumed Figure 2 T1 weighted, gadolinium enhanced, that since vitamin B-1 is highly eVective in axial magnetic resonance image illustrating the ethanol intoxication, it might well be eVective Figure 1 Grey scale representation of the enhancing lesion on the right compressing the in methanol intoxication. Other ocular associ- generalised constriction of the patient’s right eye intracranial optic nerve. (The bright image on central visual field as assessed by a Humphrey the left was non-enhancing and reported as a ated pathologies in which thiamine may be probable old venous thrombosis.) eVective are nutritional amblyopia, and visual field analyser 24-2 full threshold program. cranial nerve palsies; (b) intracranial tumour Leigh’s disease (infantile necrotising encepha- On admission examination revealed no lomyopathy), both irrelevant in this case. syndromes; and (c) intraorbital syndromes. focal neurological deficit and investigation Intracranial tumour syndromes tend to pre- In conclusion, the combination of steroids revealed hypercalcaemia (corrected calcium and vitamin B-1 was highly eVective in sent with multiple neurological symptoms and 2.80 mmol/l), raised serum protein (89 g/l), signs, occasionally with those of a space occu- treating severe methanol optic neuropathy. We raised plasma viscosity (2.08 mPa), a venous do not know whether using only one of the pying lesion and raised intracranial pressure. IgG type ê paraprotein of 40 g/l, and a urine drugs might be suYcient but, since the risk of Intraorbital involvement commonly pre- Bence-Jones monoclonal band of ê light sents with optic neuropathy and this is usually permanent blindness is high, we recommend chains of 0.34 g/l. using this combination in the event of metha- secondary to direct infiltration, a retrobulbar Abdominal ultrasound revealed two mass 2 nol intoxication. tumour, or a hyperviscosity state. Optic lesions in the anterior abdominal wall anterior neuropathy is rare secondary to an intracranial YGAL ROTENSTREICH to the liver. Bone scan showed multiple hot EHUD I ASSIA lesion and is usually associated with other spots, especially in the posterior ribs and 2 Department of Ophthalmology, Meir Hospital, Israel neurological signs in these latter cases. Isolated increased uptake in the sternum and left optic nerve compression from an intracranial ANAT KESLER sacroiliac joint. plasmacytoma with no other focal neurological Department of Neurology, Meir Hospital, Israel A diagnosis of multiple myeloma was made, signs has only rarely been reported in the litera- Correspondence to: Ehud I Assia, Department of the patient was rehydrated intravenously, and ture as individual case reports.34 Two cases Ophthalmology, Meir Hospital, Sapir Medical treated with intravenous steroids, melphalan, Center, Kfar Saba, 44281, Israel. found in the literature were both initially and radiotherapy. She was referred for oph- treated as retrobulbar neuritis (RBN)—both Accepted for publication 12 February 1997 thalmic assessment because of the history of with transient alleviation of symptoms with sys- blurred vision. temic steroid therapy; diagnosis was eventually Best corrected visual acuity was found to be made after a period of months by pneumoen- 1 Benton CD, Calhoun FP. The ocular eVect of 6/60 N 14 right eye, 6/9 N 8 left (noted to be methil alcohol poisoning: report of catastrophe cephalography and craniotomy (in the absence involving three hundred and twenty persons. 6/9 in the right eye in 1986). A right relative of more sophisticated imaging techniques). A aVerent pupillary defect (RAPD) was de- Trans Pa Acad Ophthalmol Otolaryngol further case of intracranial plasmacytoma http://bjo.bmj.com/ 1952; :1677–85. 56 tected and generalised constriction of the cen- presenting with a long history of fluctuating 2 Scrimgeour EM. Outbreak of methanol and iso- tral 24-2 (Humphrey full threshold, Fig 1) propanol poisoning in New Briten, Papua New vision and headache was treated as (biopsy Guinea. Med J Aust 1980;2:36–8. with inferior altitudinal scotoma was noted on unconfirmed) cranial with systemic 3 Scrimgeour EM, Dethlefs RF, Kevau I. Delayed the right. There were minimal cortical lens 2 steroids for 41⁄2 years before diagnosis. recovery of vision after blindness caused by opacities and funduscopy did not indicate any methanol poisoning. Med J Aust 1982;2:481–3. Our case demonstrates a single intracranial 4 Buzna E, Cernea D. [The therapeutic approach optic disc hyperaemia, swelling, or pallor. An deposit, and its magnetic resonance imaging in optic neuropathy due to methyl alcohol.] urgent cranial magnetic resonance image was appearance, resulting in an isolated optic neu- Oftalmologia (Romania) 1991;35:39–42. requested and this revealed a smoothly 5 Sharpe JA, Hostovsky M, Bilbao JM, Rewcastle ropathy. outlined mass in the right middle cranial fossa on September 30, 2021 by guest. Protected copyright. NB. Methanol optic neuropathy: a histological Magnetic resonance imaging is now the study. Neurology 1982;32:1093–100. at the apex of the orbit compressing the optic investigation of choice in patients with atypical nerve and enhancing with gadolinium (Fig 2); optic neuropathies and no obvious orbital this mass appeared to arise from the dura Intracranial plasmacytoma presenting lesion. mater and was the only intracranial mass with optic nerve compression Intracranial lesions may arise from the dura detected. A diagnosis of intracranial plasma- mater, brain parenchyma, or adjacent lytic EDITOR,—Neurological complications are not cytoma secondary to multiple myeloma was skull lesion in which case the dura has been uncommon in patients with multiple mye- made and she was referred for radiotherapy to postulated to prevent invasion.15 loma; however, an isolated intracranial plas- the lesion. Six weeks later visual acuity was 6/9 Systemic agents—for example, melphalan, macytoma is rare. We describe a patient with N5 right and left eyes, the right RAPD was have little proved activity against intracranial multiple myelomatosis and a single intracra- barely detectable, and optic disc appearances lesions and extracorporeal radiotherapy re- nial lesion presenting with optic nerve com- remained normal. mains the treatment of choice combined with pression. chemotherapy for any systemic disease, as in COMMENT this case. CASE REPORT Multiple myeloma accounts for 1% of all R MAINI A 73-year-old woman had had symptoms of malignancies and 10% of haematological C J MACEWEN intermittent blurring of vision in the right eye malignancies. Systemic signs and symptoms Department of Ophthalmology, for several months and had consulted her are due to pathological prolifera- Ninewells Hospital and Medical School, optometrist, who had reassured her. She sub- tion, secondary metabolic alteration, and Dundee DD1 9SY sequently presented to her general associated immunological dysfunction. Plas- Correspondence to: R Maini. practitioner with a 3 month history of left macytomas are tumours comprising a Accepted for publication 28 January 1997 upper arm and right lower chest ‘rib’ pain, proliferation of abnormal plasma cells; they anorexia, weight loss, malaise, and fatigue. demonstrate monoclonal intracellular immu- 1 Clarke E. Cranial and intracranial myelomas. Brain 1954;77:61–81. Skeletal survey revealed multiple extrapleural noglobulins using immunological staining 2 Coppeto JR, Monteiro MLR, Collias J, UphoV lesions bilaterally, with associated lytic bone techniques, distinguishing them from plasma D, Bear L. Foster-Kennedy syndrome caused by destruction at each site. There was also cell granulomas which show a polyclonal pat- solitary intracranial plasmacytoma. Surg Neurol evidence of lysis of the transverse process of tern of several diVerent heavy and light chains. 1983;19:267–72. 3 Kamin DF, Hepler RS. Solitary intracranial plas- T1 and facet complex of C7 on the right. She Neurological complications in multiple mye- macytoma mistaken for retrobulbar neuritis. Am was referred urgently for further investigation. loma were first classified by Clark1 into: (a) J Ophthalmol 1972;73:584–6. 418 Letters

4 Kennerdell JS, Jannetta PJ, Johnson BL. A result of applying lead-containing kohl to their steroid sensitive solitary intracranial plasmacy- eyes. In our study, five patients who were Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from toma. Arch Ophthalmol 1974;92:393–8. 5 Jordan DR, Drouin J, Berry G, Watson AG. tested for lead toxicity were normal. The rea- Intracranial plasmacytoma associated with son for this discrepancy is probably related to multiple myeloma. Can J Ophthalmol 1984;19: the dose and site of kohl application. All of our 275–8. adult patients applied the eyeliner to the eyelid margin for cosmetic reasons while in children Conjunctival and lacrimal sac a larger amount of kohl is usually instilled into pigmentation by kohl (eyeliner) the conjunctival sac for therapeutic reasons.6–9 It is interesting to note that a biopsy EDITOR,—Abnormal pigmentation of the con- specimen from the pigmented lacrimal sac junctiva has been associated with various that contained lead showed diVuse radio- pathological conditions including melanosis, opacity when tested radiologically. This find- melanocytic tumours, foreign bodies, systemic Figure 1 Case 1. The tarsal conjunctiva of the ing may be confusing to the radiologists disease, topical and systemic use of medica- left lower lid shows black pigmentary deposit in kohl user. and/or ophthalmologists if they are not aware tions, and industrial exposure to various of this problem. Dacryostenosis in those chemicals.12 patients with lacrimal sac pigmentation is 88/368 Preset = 100 s Vert = 6825 counts Disp = 1 Elapsed = 100 s most probably the result of trachoma, which is CASE REPORTS Pb very common in the Middle East. In this report, abnormal pigmentation of the S Presented at the Association for Research in Vision conjunctiva and lacrimal sac was noted in 10 and Ophthalmology Annual Meeting, Sarasota, patients using kohl (eyeliner, surma) that was Florida 3 May 1990. made in India.3–5 This eyeliner is used by mil- Pb The opinions or assertions contained herein are the private views of the authors and should not be lions in the Middle East, Asia, and Africa. The construed as being oYcial or as representing the median age of nine women and one man was views of the Department of the Army or the Depart- 52 years (range 40–64 years). All patients had ment of Defense. used kohl for many years. The two patients S AHMED A HIDAYAT Pb Pb Armed Forces Institute of Pathology, with conjunctival pigmentation had bilateral, SC O PbPb Pb Pb Pb Washington DC, USA diVuse pigmentation of the fornicial and tarsal 0.000 Range = 10.230 keV 4.490 conjunctiva of the upper and lower eyelids in ROBERT G WEATHERHEAD the form of discrete, punctate black deposits Figure 2 Case 8. Kohl in the wall of lacrimal ALI AL-RAJHI (Fig 1). The other eight patients presented sac shows high contents of lead (Pb) and sulphur King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia with epiphora and had chronic dacryocystitis (S). Energy dispersive analysis of x rays. FRANK B JOHNSON associated with obstruction of the lacrimal also tested and showed the same elements as passages, including the lacrimal canaliculi and Armed Forces Institute of Pathology, detected in their biopsy specimens. Washington DC, USA common lacrimal canaliculus. Three of these Radiographic studies of the biopsy speci- Correspondence to: Ahmed A Hidayat, MD, Depart- patients also had dacryocutaneous fistulas. men from a pigmented lacrimal sac showed a ment of Ophthalmic Pathology, Armed Forces Insti- During dacryocystorhinostomy, focal to dif- diVuse and dense radiopacity, corresponding tute of Pathology, Washington DC 20306-6000, USA. fuse black pigmentation of the lacrimal sac to the high metallic content, mainly lead in the Accepted for publication 13 January 1997 was observed in seven individuals, in tissue. 1 Duke-Elder S, ed. Systems of ophthalmology. Vol tissue obstructing the common canaliculus in 14, part 2 Injuries: non-mechanical injuries. St one, in the subcutaneous tissues around the COMMENT Louis: CV Mosby, 1972:1089–128. fistula in one, and in the periosteum of the 2 Brothers DM, Hidayat AH. Conjunctival pig- In this study, the findings of a similar compo- mentation associated with tetracycline lacrimal sac fossa in another. The black sition in the patient’s tissues and the kohl used medication. Ophthalmology 1981;88:1212–5. pigmentation of the lacrimal sac resembled to in the area of origin strongly support the clini- 3 Al-Hawi SA. Kohl. In: Wafai MZ, Kalaagi MR, http://bjo.bmj.com/ some extent malignant melanoma, except for cal diagnosis of kohl pigmentation in the con- eds. Nur Al-Ugun, Wa Jame Al-Funun. Light of the eyes and the collector of arts. Riyadh, Saudi the absence of a mass. Preoperatively, a dark junctiva and lacrimal drainage system. The Arabia: King Faisal Center for Research and bluish pigmentation was observed around the various elements in kohl that caused tissue Islamic Studies, 1986;142–8. cutaneous fistula site in one patient. Histologi- pigmentation include lead, silver, iron, car- 4 Tabbara KF, Burd EM. Microbial content of cally, the fine to coarse extracellular pigment bon, and magnesium. Intraoperatively, black kohl. Ann Saudi Med 1986;7:177–9. 5 Al-KaV A, Al-Rajhi A, Tabbara KF, El Yazigi A. was often surrounded by a chronic inflamma- pigmentation of the lacrimal sac by kohl may Kohl—the traditional eyeliner: use and analysis. tory infiltrate, mainly lymphocytes with a few be confused with malignant melanoma if the Ann Saudi Med 1993;13:26–30. plasma cells and neutrophils. 6 Warley MA, Blackledge P, Gorman PO. Lead surgeon is not aware of this entity. A more on September 30, 2021 by guest. Protected copyright. poisoning from eye cosmetics. BMJ 1968;1:117. Energy dispersive analysis of x rays of all serious medical problem than pigmentation in 7 Snodgrass GJAI, Ziderman DA, Gulati V, biopsy specimens revealed a high lead content our patients is the finding of lead in their Richards J. Cosmetic plumbism. BMJ 1973;4: in the patient’s tissues (Fig 2). Other less fre- tissues. In the past three decades, several well 230. quent elements included silicon, carbon, documented cases of lead poisoning 8 Betts PR, Astley R, Raine DN. Lead intoxication 6–9 in children in Birmingham. BMJ 1973;1:402–6. sulphur, silver, magnesium, iron, and alu- (plumbism) have occurred in England 9 Ali AR, Smales ORC, Aslam M. Surma and minium. The kohl used by the patients was among Indian and Pakistani children as a lead-poisoning. BMJ 1978;2:215–6.

Table 1 Clinical data for nine patients with kohl pigmentation

Patient No, age (years), sex Site of pigmentation Clinical diagnosis 1, 60, F Conjunctiva, both eyes Conjunctival pigmentation; cataract 2, 64, F Conjunctiva, both eyes Conjunctival pigmentation; corneal scar; cataract 3, 44, F Common lacrimal Chronic dacryocystitis, both eyes; common canalicular occlusion by pigmented scar, right eye; upper and lower canalicular occlusion, left eye 4, 52, M Lacrimal sac, right eye Chronic dacryocystitis with non-expressible mucocele, right eye 5, 50, F Lacrimal sac, right eye Chronic dacryocystitis with mucocele and dacrocutaneous fistula, right eye; blockage of lower canaliculus right eye; common canaliculus right eye and common canaliculus left eye 6, 50, F Lacrimal sac, left eye Chronic dacryocystitis, both eyes; partial common canicular occlusion, both eyes 7, 40, F Lacrimal sac, left eye; subcutaneous scar tissue Chronic dacryocystitis with fistula, left eye; pigmentation of around the fistula subcutaneous tissue around the fistula left eye 8, 55, F Lacrimal sac, left eye; periosteum of lacrimal sac Blockage of upper and lower canaliculi, right eye, blockage of fossa, left eye common canaliculus; congenital fistula from skin to common canaliculus 9, 56, F Lacrimal sac, left eye Common canalicular obstruction, both eyes 10, 55, F Lacrimal sac, right eye Chronic dacryocystitis, right eye Letters 419

An unusual case of map-dot epithelial dystrophy Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from

EDITOR,—Map-dot epithelial dystrophy, also called Cogan’s microcystic dystrophy,1 is a well known and relatively frequent clinical entity. The microscopic basis for the biomi- croscopic appearance of the typical superficial corneal opacities and epithelial erosions are intraepithelial cysts and a multilaminated or bilaminated aberrant epithelial basement membrane.1–5 Therefore, the term epithelial basement membrane dystrophy has been introduced by some authors.5 We report a patient with Cogan’s corneal dystrophy with histologically proved erythro- cyte inclusions in the intraepithelial cysts and a conspicuous clinical appearance not before described.

CASE REPORT In March 1995 a 48-year-old white man reported to our clinic with typical symptoms of recurrent epithelial microerosions mainly on his right eye. He complained of early morning pain and reduced vision for a few weeks but could not recall an earlier ocular trauma. He did not wear contact lenses. Biomicroscopically, bright red intraepithelial inclusions in the corneal epithelium were found in the right eye (Fig 1A); there was no fluorescein staining. The left eye showed a few whitish, map-dot-like epithelial opacities. http://bjo.bmj.com/ on September 30, 2021 by guest. Protected copyright.

Figure 1 (A) Slit-lamp photograph of right eye. Note red blood-like colour of the inclusions in the map, dot, and fingerprint-like epithelial alterations of the cornea of the right eye (arrowheads). There was no fluorescein staining suggesting that the erythrocytes had already been entrapped intraepithelially. (B) Lower and (C) Figure 2 (A) Photomicrograph of the corneal epithelium with bilaminated, intraepithelial basement higher magnification photomicrographs of the membrane with folds (arrowhead). Note pale swelling of underlying oedematous basal epithelial cells. corneal abrasion material demonstrating (periodic acid SchiV, × 500). (B) Electron micrograph (× 3240) illustrating basement membrane fold intraepithelial cyst with cell debris, keratin, and with bilaminated, redundant basement membrane. (C) Shows the more loosely arranged superficial numerous erythrocytes (arrowhead) (trichrome layer (1) and the more densely packed second layer (2) beneath it, both containing randomly oriented stain). fibrils in a finely granular matrix (contrasted with uranylacetate and lead citrate, × 9360). 420 Letters

There was no corneal or limbal neovasculari- periodic acid SchiV staining (Fig 2A). Elec- intraepithelial cysts is unknown, although sation and only a slight ciliary flush. The cor- tron microscopy again displayed a redundant, haemorrhage from the eyelid is a possibility. Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from neal stroma, the endothelium, and the ante- bilaminar basement membrane of the epithe- After a few weeks the red inclusions were no rior chamber were without peculiarities. The lial cells with folds (Fig 2B). On microscopy longer visible, probably because the entrapped eyelid margins showed signs of mild chronic there was no hint of a bacterial infection. erythrocytes had been phagocytised by mac- blepharitis, and the tear film was unstable. rophages. The visual acuities were 6/6 left eye and 6/7 COMMENT GREGOR WOLLENSAK right eye. We are not aware of any case of map-dot epi- As the clinical appearance on the right eye Department of Ophthalmology, thelial dystrophy or other corneal disease pub- University of Freiburg i Br, Killianstrasse 5, was unknown to us and to exclude, for exam- lished previously with intraepithelial blood D-79106 Freiburg i Br, Germany ple, a bacterial infection, bacterial cultures, inclusions. It is useful to know of this corneal conjunctival scrapings, and focal superficial JOSEF WOLLENSAK alteration because of the diVerential diagnosis Department of Ophthalmology, Virchow-Hospital, stripping of the corneal epithelium in some of of bacterial infection. It is also interesting the areas of the red alterations were performed Humboldt University of Berlin, Augustenburger Platz 1, because this case shows that blood cells can be D-13344 Berlin, Germany by means of a hockey knife. The bacterial cul- included intraepithelially in the cornea under Correspondence to: Dr G Wollensak. tures were negative. Therefore, we only special circumstances despite regular blinking prescribed artificial tears. and washing of the ocular surface by the tear Accepted for publication 4 November 1996 After a few weeks treatment was stopped film. because the patient no longer had any The microscopic findings are compatible symptoms. Biomicroscopically, the red inclu- with previous histological reports on map-dot 1 Cogan DG, Kuwabara T, Donaldson DD, Collins E. Microcystic dystrophy of the cornea. sions had completely disappeared without epithelial dystrophy.1–5 The aberrant intraepi- leaving . However, there were still typical Arch Ophthalmol 1974;92:470–4. thelial basement membrane may obstruct the 2 Dark AJ. Cogan’s microcystic dystrophy of the map-dot epithelial opacities in both eyes. The normal migration of maturing epithelial cells cornea: ultrastructure and photomicroscopy. Br visual acuity of the right eye had normalised to leading to the disintegration of these cells and J Ophthalmol 1978;62:821–30. 6/6. 3 Rodrigues MM, Fine BS, Laibson PR, Zimmer- the formation of microcysts which may man LE. Disorders of the corneal epithelium: a rupture eventually and form corneal clinicopathologic study of dot, geographic, and PATHOLOGICAL FINDINGS erosions.1–4 fingerprint patterns. Arch Ophthalmol 1974;92: The light microscopic examination of the In our case, initially there must have been 475–82. 4 Ehlers N, Møller HU. Pathology and patho- stripped corneal epithelium revealed intraepi- temporary microerosions, presumably due to mechanisms of epithelial microcystic and base- thelial microcysts containing entrapped cellu- ruptured epithelial microcysts to which eryth- ment membrane abnormalities of the cornea. lar debris, keratin, and numerous red blood rocytes were attached, which were then incor- Acta Ophthalmol 1988;66:318–26. cells (Fig 1B and C). In addition, a thickened porated into the epithelial microcysts when 5 Ehlers N, Møller HU. Dot-map-fingerprint dystrophy—Cogan’s microcystic dystrophy— bilaminar aberrant basement membrane with the corneal epithelium closed again. The site normal reactions of the corneal epithelium? folds was found intraepithelially, visible with of origin of the erythrocytes within the Acta Ophthalmol 1987;65(suppl 182):62–6. http://bjo.bmj.com/ on September 30, 2021 by guest. Protected copyright. British Journal of Ophthalmology 1997;81:421–423 421

I have been using peribulbar anaesthesia for natal and infant welfare clinics, it was possible intraocular procedures for some years now, to pick up younger children with early visual Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from CORRESPONDENCE injecting 1.5 ml of a mixture of 2% lignocaine defect before gross amblyopia had developed. and 0.5% bupivacaine in the upper fornix, This meant that there was early referral, with then 1.5 ml in the lower fornix. The only slight visual suppression, which entailed anaesthetic eVect is very good and suYcient only a short period of occlusion and treat- even for long and complicated operations. By ment, which was cost eVective for hospital Intraocular pressure changes after using these relatively small quantities of local attendances, and for the families indeed, in my peribulbar injections with and without anaesthetics, the IOP rises only slightly, thus region of South West Thames: full blown ocular compression preventing complications and making ocular amblyopia was virtually eliminated at that compression unnecessary. I would recom- time. EDITOR,—Ten years on from Davis and 1 mend this technique for most intraocular Perhaps the pendulum may once again turn Mandel’s description of the technique, and procedures. towards really early assessment and referral, despite the popularity of the method, the exact ISSAK SCHIPPER and hence lead to prevention rather than cure. role of, and rationale for, ocular compression I would urge the authors and all concerned in peribulbar anaesthesia remains somewhat Eye Clinic, Cantonal Hospital, 6000 Lucerne 16, Switzerland with paediatric management to direct their obscure. Whatever the facts of the matter this energies towards this goal and fulfil the aspira- would appear particularly undesirable since a 2 3 tions of the giants of yesteryear such as Mary body of evidence from clinical, cadaveric, 1 Bowman R, Liu C, Sarkies N. Intraocular Sheridan and Ronnie MacKeith. and animal4 work would suggest certain pressure changes after peribulbar injections with and without ocular compression. Br J Ophthal- G V CATFORD potential risks associated with its employ- mol 1996;80:394–7. ment. In their recent paper, published in this St George’s Hospital, London journal in May 1996,5 Bowman et al correctly admit that comparison of their results with Author’s reply 1 Newman DK, Hitchcock A, McCarthy H, those obtained by other workers is diYcult Keast-Butler J, Moore AT. Outcome of children since so many published studies attempt to EDITOR,—We knew that the use of oculocom- referred to the hospital eye service. Br J draw conclusions from surgeons with widely pression after peribulbar anaesthesia is not Ophthalmol 1996;80:1077–82. diVering practice. Final clarification of the universal and we were interested to learn from controversy remains elusive. Ellis and Kearns’s survey of the Scottish Oph- While conducting a study of a separate but thalmic Club that only 62% of the users of First day follow up for routine related aspect of peribulbar anaesthesia, we peribulbar anaesthesia considered oculocom- phacoemulsification? were interested to establish the details of cur- pression essential. We wonder whether those rent consultant practice and surveyed 60 users of peribulbar anaesthesia who do not EDITOR,—We would certainly concur with consultant members of the Scottish Ophthal- employ oculocompression afterwards were Whitefield et al 1 that an important reason why mic Club with 42 (70%) questionnaires aware of our findings of the immediate mean patients decline day case surgery is the need returned. Of consultants surveyed 55% were pressure rise of 11.44 mm Hg with an for an examination the next day. We would using peribulbar, 23% retrobulbar, 17% sub- injectate volume of 10 ml. We disagree with also question this necessity following uncom- Tenon’s, and 5% topical anaesthesia. Of those Ellis and Kearns that the rationale of the tech- plicated sutureless phacoemulsification with using peribulbar techniques, 62% considered nique remains obscure: we have shown that lens implant. However, we feel it may be compression with the balloon essential. In this oculocompression for 20 minutes with the unsafe practice if patients are not examined at group the minimum acceptable time for com- Honan’s balloon after peribulbar anaesthesia all in the early postoperative period. pression was quoted at between 5 and 20 min- produces a mean fall in intraocular pressure of Firstly, intraocular pressure can be raised utes (average 9 minutes). 11.05 mm Hg. following cataract surgery,2 as indeed it was in Despite the theoretical risks from oculo- As Schipper points out, lower volumes of three cases in this study. The maximum pres-

pression, especially in eyes with actual or anaesthetic do not produce such a significant sure rise tends to occur 5–7 hours postopera- http://bjo.bmj.com/ potentially impaired optic nerve head blood pressure rise. Oculocompression may not be tively3 and so if patients were examined then flow, thankfully there is a relative paucity of necessary with smaller volumes of anaesthetic many more may well be found to have raised reports of serious adverse eVects in clinical or topical anaesthesia. However, we continue pressure. For most patients this transient ocu- practice. to recommend the use of oculocompression to lar hypertension is not associated with signifi- cant morbidity, but central retinal artery and JOHN ELLIS reduce the ocular pressure if the technique of Ninewells Hospital, Dundee DD1 9SY peribulbar anaesthesia reported by Davis and venous occlusions and non-arteritic anterior Mandel1 is employed. ischaemic optic neuropathy4 have been re- PATRICK KEARNS ported following cataract surgery and this Queen Margaret Hospital, Dunfermline KY12 0SU NICHOLAS SARKIES pressure spike may be a significant factor. on September 30, 2021 by guest. Protected copyright. Wistow, The Green, Hilton, Huntingdon, It may be better, therefore, to examine the patient on the same day as surgery before dis- 1 Davis DB, Mandel MR. Posterior peribulbar Cambs PE18 9NB anaesthesia: an alternative to retrobulbar anaes- charge. The use of a prophylactic ocular anti- thesia J Cataract Refract Surg 1986;12:182–4. hypertensive agent would also be recom- 2 Morgan JE, Chandna A. Intraocular pressure 1 Davis DB, Mandel MR. Posterior peribulbar anaesthesia: an alternative to retrobulbar anaes- mended. An early examination would also after peribulbar anaesthesia: is the Honan allow a significant wound leak to be detected.5 balloon necessary? Br J Ophthalmol 1995;79:46– thesia. J Cataract Refract Surg 1986;12:182–4. 9. In the study by Tufail et al 5 it was concluded 3 McDonnell PJ, Quigley HA, Maumenee AE, that no complications were missed by examin- Stark WJ, Hutchins GM. The Honan intraocu- ing the patients on the same day as surgery lar pressure reducer Arch Ophthalmol 1985;103: Preschool vision screening 422–5. that would have been detected the next day. 4 Jay WM, Aziz MZ, Green K. EVect of Honan EDITOR,—Congratulations to Newman and Iris prolapse and endophthalmitis are both intraocular pressure reducer on ocular and optic colleagues1 for bringing the subject of amblyo- very rare (0.3%)6 and so would not be nerve blood flow in phakic rabbit eyes. Acta pia to general attention once again. expected to be detected by a study of this size Ophthalmol Copenh 1986;64:52–7. 5 Bowman R, Liu C, Sarkies N. Intraocular After years of neglect in the morass of (100 patients). Often these conditions do not pressure changes after peribulbar injections with developmental trends and the shift of respon- present on the first postoperative day, with and without ocular compression. Br J Ophthal- sibility to parents, as advised by the Hall endophthalmitis typically presenting 48–72 1996; :394–7. mol 80 report and so much beloved by the cost hours postoperatively.7 A fibrinous uveitis, cutting administrators, this is a breath of fresh which may be a warning of impending Reply air. endophthalmitis, again is often worse a few My experience over 30 years of paediatric days after surgery rather than at day 1. It is 1 EDITOR,—Bowman and coauthors demon- ophthalmology evolved along similar lines. It possible some of these cases could be screened strated in their well performed study that was satisfying initially to pick up amblyopes for with a visit 3 days after surgery. It is our there is a marked rise in intraocular pressure from whatever cause at 3 and 5 years of age practice, however, to ask the patient to return (IOP) after peribulbar anaesthesia. They and to carry out the treatments with all the as an emergency if symptoms change, espe- injected a total of 10 ml of the anaesthetic, expense that involved. cially increasing pain, rather than seeing all divided, into two diVerent locations. The However, in due time, I found by organising patients at day 3. pressure could be lowered again with the help screening clinics during the sensitive period of Our policy is to examine on the same day as of oculopressure. visual development, with orthoptists at post- surgery, before discharge, and again at 2 weeks 422 Correspondence, Notices and if all is well the patient is discharged for manifestation and abnormal laboratory find- out any developmental anomalies, normal iris refraction by their optician, as no further ings.23 Although an abnormal rise of venous structure, and contour. The optic nerves were Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from intervention is required. If one suture has lactate is observed in patients with Kearns– normal. Topical steroid was immediately been used this is removed at this 2 week visit. Sayre syndrome and other mytochondrial withdrawn and ocular hypotensives adminis- S J TALKS cytopathy, a few reports have demonstrated tered. The IOP fell to 15 mm Hg bilaterally P ROSEN abnormal elevation of lactate and pyruvate in the next day and remained within normal lim- 4 The Eye Hospital, The RadcliVe Infirmary, blood and CSF in LHON. Since 11778 its after all medications were tailed oV over the 5 Woodstock Road, Oxford OX2 6HE mtDNA encodes subunit 4 of complex l, one ensuing days. Both the visual acuity and visual would expect that the 11778 mtDNA muta- field remained normal at the latest follow up, tion causes an amino acid change in mito- 1 Whitefield L, Crowston J, Little BC. First day 1 year after the steroid treatment. follow up for routine phacoemulsification? Br J chondrial respiratory chain and leads to Ophthalmol 1996;80:148–50. impaired catabolism of lactate and pyruvate. COMMENT 2 Fiest RM, Palmer DJ, Fiscella R, Emest JT, The resting lactate and pyruvate levels in Tripathi R, Torczynski E, et al.EVectiveness of blood and CSF and those after aerobic There was no family history of glaucoma and aproclonidine and acetazolamide in preventing both parents were normotensive with normal postoperative intraocular pressure spikes after exercise would give additional information on extracapsular cataract extraction. J Cataract the biochemical abnormalities of LHON. optic nerves. Steroid provocation tests were Refract Surg 1995;21:191–5. initiated but subsequently discontinued at the 3 Bomer TG, Lagreze WD, Funk J. Increased TSUTOMU NINOMIYA parents’ demand when they grew alarmed by intraocular pressure after cataract extraction— HIROKO YAMAZAKI the positive steroid response in their other eVect of surgical technique, surgical procedure Department of Ophthalmology, Kohnodai Hospital, and preventive drug administration. A prospec- child (see below). The parents’ IOP remained National Center of Neurology and Psychiatry, tive, randomised double-blind study. Klin Japan normal 3 days into the provocation test when Monatsbl Augenheilkd 1995;206:13–9. it was aborted. 4 Hayreh SS. Anterior ischaemic optic neuropathy. SHIN MUNAKATA IV Occurrence after cataract extraction. This test was, however, successfully com- Arch Department of Ophthalmology, School of Medicine, Ophthalmol 1980;98:1410–6. pleted in their 3-year-old daughter. It helps to 5 Tufail A, Foss AJE, Hamilton AMP. Is the first Chiba University, day postoperative review necessary after cataract Japan exclude ocular hypertension secondary to squint surgery itself and tests for a familial extraction? Br J Ophthalmol 1995;79:646–8. HIIDE YOSHINO 6 Desai P. The national cataract surgery survey II. TAKESHI SATO predisposition to steroid responsiveness. Ad- Clinical outcomes. Eye 1993;7:489–94. ditionally, we asked if fluorometholone would 7 D’Amico DJ, Noorily SW.Postoperative endoph- Department of Neurology, Kohnodai Hospital, National thalmitis. In: Albert DM, Jakobiec FA, eds. Center of Neurology and Psychiatry, produce less ocular hypertension in the Principles and practice of ophthalmology. Clinical Japan second child. One eye was randomised to practice. Philadelphia: WB Saunders, 1994;Vol receive Maxitrol while the other received 2:1159–69. 1 Leber T. Ueber hereditare und congenital- fluorometholone, both six times a day. Investi- angelegte Sehnervenleiden. Graefes Arch Oph- gators were masked as to the treatments given. A case of LeberQs hereditary optic thalmol 1871;17:249–91. IOP was measured with a Tono-pen (Men- 2 Nikoskelainen EK, Savontaus MJ, Wanne OP, tor), at 5 to 6 pm. The mean of three was neuropathy with elevated blood levels of Katila MJ, Nummelin KU. LeberQs hereditary lactate and pyruvate optic neuroretinopathy. A maternally inherited recorded. Steroids were planned for with- disease. Arch Ophthalmol 1987;105:665–71. drawal when the IOP doubled that of the 3 Newman N. LeberQs hereditary optic neu- baseline IOP. Just as in her brother, IOP rose EDITOR,—LeberQs hereditary optic neuro- ropathy. Arch Neurol 1993;50:540–8. pathy (LHON) is a maternally inherited 4 Uemura A, Osame M, Nakagawa M, Makahara rapidly to double the pretreatment value disease characterised by acute bilateral loss of K, Sameshima M, Ohba N. LeberQs hereditary within 48 hours in the Maxitrol treated eye central vision.1 We report a patient with optic neuropathy: mitochondrial and biochemi- (Table 1). She exhibited a 66% and 33% IOP cal studies on muscle biopsies. Br J Ophthalmol rise after a single day of instillation of Maxitrol LHON who had elevated lactate and pyruvate 1987;71:531–6. levels in blood and cerebrospinal fluid (CSF). 5 Wallace DC, Singh G, Lott MT, Hodge JA, and fluorometholone, respectively. On ceasing Schurr Tg, Lezza AMS. Mitochondrial DNA steroid treatment, the IOP returned to normal

mutation associated with LeberQs hereditary within 5 days. This abbreviated course of IOP http://bjo.bmj.com/ CASE REPORT optic neuropathy. Science 1988;242:1427–30. elevation and recovery in this otherwise A 22-year-old man noticed blurred vision in normal child strongly supports steroids as a both his eyes. Corrected visual acuity was 0.04 cause of ocular hypertension in both children. in his right eye and 0.05 in his left. Slit-lamp Accelerated ocular hypertensive The cause of this exaggerated steroid examination and intraocular pressures were response to topical steroids in children response in children remains unknown. The normal. Pupillary light reflex was full in both relatively immature chamber angle in young eyes. EDITOR,—Topical steroids are well recognised children, which has been suggested to be Fundus examination revealed slightly hyper- to elevate intraocular pressure (IOP) in adults who are sensitive to their eVects.12 However, completely developed only at 8 years, may aemic optic discs. There was marked disc pal- on September 30, 2021 by guest. Protected copyright. predispose them to the eVects of topical lor over the next 2 months. Fluorescein less is known of the steroid response in 34 steroids.5 Support for this argument comes angiography showed diVuse hyperfluores- children. We report an accelerated ocular cence in the paramacular area. Goldmann hypertensive response to topical steroids in from a rabbit model, in which only the perimetry showed a large central scotoma in two siblings. younger animals suVered steroid induced glaucoma, while older rabbits were resistant to each eye. No detectable responses of pattern 6 visually evoked potentials were observed. His CASE REPORT its eVects. As the response in young children neurological examination, magnetic reso- A 6-year-old boy underwent bilateral lateral can be both severe and extremely rapid in nance imaging (MRI) of the brain, electromy- rectus recession for left divergent squint. The onset, we suggest close monitoring of IOP in ography, and electroencephalography were operation was uneventful and no subconjunc- the first week of topical steroid treatment normal. The resting blood level of lactate was tival steroid injection was given at the end of under the age of 8. Moreover, with the elevated to 15.7 mg/dl (normal 3.3–14.9 the operation. He was discharged with Max- possibility of strong familial tendency, siblings mg/dl) and that of pyruvate was elevated to itrol (0.1% dexamethasone and neomycin) should also be tested and cautioned accord- 1.51 mg/dl (normal 0.30–0.94). The level of eyedrops, six times per day bilaterally. When ingly. lactate in the CSF was 13.8 mg/dl (normal reviewed on the fourth postoperative day, he DENNISSCLAM 8.7–13.5), and level of the pyruvate was 1.27 complained of and recurrent ALVINKHKWOK mg/dl (normal 0.37–0.75). The blood level of vomiting for the previous 2 days. Examination Department of Ophthalmology and Visual Sciences, lactate was elevated to 38.0 mg/dl, and the revealed mild ciliary injection with IOP of 44 The Chinese University of Hong Kong, pyruvate level was elevated to 2.26 mg/dl after and 46 mm Hg (measured by the applanation Prince of Wales Hospital, Shatin, Hong Kong exercise loading. Biopsy of the biceps brachii tonometry) in the right and left eyes, respec- SEKJIN CHEW muscle showed no ragged red fibres with tively. Gonioscopy showed open angles with- Singapore Eye Research Institute, Singapore Gomori trichrome staining. A point mutation of mtDNA at nt11778 was determined in the Table 1 Ocular hypertensive response to topical steroids in the patient’s 3-year-old sister leucocyte. Neither the mtDNA point muta- tion at nt3243 (MELAS), nt8344 (MERRF), Day 1* Day 2 Day 3 Day 4 Day 8 nor large deletion (KSS, CPEO) was de- Fluorometholone 12 16 18 13 11 tected. Maxitrol 12 20 24† 16 12 Previous reports suggested that LHON is a multisystem disorder with various clinical *Baseline IOP was taken on day 1. †All drugs were withdrawn when the IOP doubled the baseline reading. Correspondence, Notices 423

1 Armaly MF. Statistical attributes of the steroid Mullaney. (Tel: (301) 498-4100; email: hypertensive response in the clinically normal [email protected]) Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from eye. I. The demonstration of three levels of response. Invest Ophthalmol 1965;4:187–97. NOTICES 2 Becker B, Hahn KA. Topical corticosteroids and heredity in primary open-angle glaucoma. Am J Conferences on Angiography in Créteil Ophthalmol 1964;57:543–51. 3 Biedner B-Z, David R, Grudsky A, Sachsa U. Intraocular pressure response to corticosteroids Glaucoma Society (UK & Eire) A conference on clinical cases in ICG will be in children. Br J Ophthalmol 1980;64:430–1. held on 9 June 1997 at the University of 4 Ohji M, Kinoshita S, Ohmi E, Kuwayama Y. Créteil. Further details: Professor Gisèle Sou- Marked intraocular pressure response to instil- DAVID COLE TRAVEL FELLOWSHIP brane, Clinique Ophtalmologique Universi- lation of corticosteroids in children. Am J Oph- thalmol 1991;112:450–4. taire de Créteil, 40 Avenue de Verdun, 94010 5 Reme C, d’Epiphany SL. Periods of development The David Cole Travel Fellowship, instituted Créteil Cédex, France. (Tel: 45 17 52 22.) of the normal human chamber angle. Doc Oph- by Merck Sharp and Dohme in memory of thalmol 1981;51:241. 6 Knepper PA, Breen M, Weinstein HG, Black LJ. Professor David Cole, will assist a visit to a Intraocular pressure and glycosaminoglycan hospital or research centre during the aca- British Council International Seminar distribution in the rabbit eye: eVect of age and demic year starting 1 October 1997. The dexamethasone. Exp Eye Res 1978 27:567–75. award will be equivalent to £2000. The A British Council international seminar purpose of the award is to enable the success- (number 97031) entitled ‘Corneal and exter- Feasibility of automated visual field ful applicant to gain experience and knowl- nal eye disease: new surgical techniques’ with Professor D L Easty as director will be held on examination in children edge in pursuit of a specific project related to glaucoma. 29 June to 5 July 1997 in Bristol, UK. The EDITOR,—We have read with great interest the seminar will be of particular interest to all 1 young eye surgeons from the developing and recent paper written by Safran et al on the THE GLAUCOMA SOCIETY (UK & EIRE) feasibility of automated visual field examina- developed world. Further details: Promotions RESEARCH GRANT tions in children between 5 and 8 years of age. Manager, International Seminars, The British It is very important to know that all the Council, 1 Beaumont Place, Oxford OX1 The Glaucoma Society (UK & Eire) research children at this age did remarkably well 2PJ, UK (Tel: +44 (0) 1865 316636; grant, sponsored by the International Glau- regarding both the duration of the automated fax: +44 (0) 1865 557368/516590; email: coma Association will be available for a visual field examination and the reliability of [email protected]) the answers, using an Octopus 2000R peri- research project clinically orientated to glau- meter with a two level strategy. However, a coma for 1997. The award will be equivalent preliminary familiarisation with a specially to £2500. European Association for the Study of designed adaptation program was found to be The grant may be used towards salary or Diabetic Eye Complications (EASDEC) mandatory with children aged 7 years or project expenses or for buying equipment. under. These awards are available to both medical The 7th meeting of EASDEC will be held on I would like to point out our good graduates and non-medical scientists resident 18–19 July 1997 at the Okura Hotel, Amster- experience with the Humphrey 120 full field in the United Kingdom or Ireland. They may dam, the Netherlands, as a pre-congress sym- screening test in children with previous retin- be held concurrently with other awards. posium of the 16th International Diabetic opathy of prematurity at the age of 10 years. For further details and application forms Federation (IDF) congress. Further details: There was no need for a preliminary adapta- please contact: Dr S Nagasubramanian, Sec- Professor BCP Polak, Rotterdam Eye Hospi- tion phase, and this test was found to be reli- retary, The Glaucoma Society (UK & Eire), tal, PO Box 70030, 3000 LM Rotterdam, the able and reproducible in all these children. Glaucoma Unit, Moorfields Eye Hospital, Netherlands. (Fax: (31) 10 4017655.) The data are presented in our paper, pub- City Road, London EC1V 2PD. lished in the BJO in 1995.2 The closing date for applications is the 31 May 1997. The successful candidate will be Continuing Medical Education http://bjo.bmj.com/ ISRAEL KREMER informed by August 1997. YUVAL YASSUR The 17th annual current concepts in ophthal- Department of Ophthalmology, mology will be held on 25–27 July 1997 at the Rabin Medical Center, Beilinson Campus, San Diego Marriott Mission Valley, San Petah Tiqva, 49100, Israel Association for Research in Vision and Ophthalmology (ARVO) Diego, California, USA. Further details: Marie Krygier, Medical Education Coordina- 1 Safran AB, LaY GL, Bullinger A, Viviani P, de Weisse C, Tschopp C, et al. Feasibility of The Association for Research in Vision and tor, San Diego Eye Bank, 9444 Balboa automated visual field examination in children Ophthalmology (ARVO) is holding its annual Avenue, Suite 100, San Diego, CA 92123, between 5 and 8 years of age. Br J Ophthalmol USA. (Fax: (619) 565-7368.) on September 30, 2021 by guest. Protected copyright. 1996;80:515–8. meeting on 11–16 May 1997 at the Fort 2 Kremer I, Nissenkorn I, Lusky M, Yassur Y. Late Lauderdale Convention Center, Fort Lauder- visual field changes following cryotherapy for dale, Florida, USA. Further details: ARVO, ROP stage 3. Br J Ophthalmol 1995;79:267–9. Tübingen Practical Angiography Course 9650 Rockville Pike, Bethesda, MD 20814- 3998. (Tel: (301) 571-1844; fax: (301) Reply The Tübingen Practical Angiography Course 571-8311.) (International Faculty) will take place on 6 EDITOR,—I very much appreciate the interest September 1997 at the Auditorium, Univer- sity Dental Clinic, Osianderstrasse 2–8, Tü- shown by Kremer and Yassur in our article on 30th Panhellenic Ophthalmological the feasibility of automated perimetry in chil- bingen, Germany. Further details; F Gelisken, Congress dren between 5 and 8 years of age.1 Iam MD, Congress Secretariat Dept III, Univer- pleased that their own observations with sity Eye Clinic, Schleichstrasse 12, 72076 The 30th Panhellenic Ophthalmological Con- 10-year-old children2 were in accordance with Tübingen, germany. (Tel: +49 (0) 7071 some of our findings. Moreover, I believe that gress organised by the Hellenic Ophthalmo- 2987448; fax: +49 (0) 7071 293746; email: their excellent paper illustrated well the logical Society will be held at the Astir Palace [email protected]) clinical value of computerised perimetry in Hotel, Vouliagmeni on 28 May to 1 June children. There is an obvious need for assess- 1997. Further details: T Kouris, CT Con- ing the limits of such procedures in very young gress, Creta Travel, 19 Amerikis 106 72 5th International Symposium on Ocular patients, and defining reference values. Athens, Greece. (Tel: (01) 3607 120, 3635 Circulation and Neovascularisation 104; fax; 3603392.) AVINOAM B SAFRAN The 5th International Symposium on Ocular Geneva, Switzerland Circulation and Neovascularisation will be American Institute of Ultrasound in held on 15–19 September 1997 in Kyoto, 1 Safran AB, LaY GL, Bullinger A, Viviani P, de Medicine Japan. Further details: Professor Dr Masa- Weisse C, Desangles D, et al Feasibility of auto- mated visual field examination in children nobu Uyama, Secretary General of the between 5 and 8 years of age. Br J Ophthalmol The second annual bilingual America’s con- Organising Committee, Department of Oph- 1996;80:515–8. ference on ultrasound will be held on 1–3 June thalmology, Kansai Medical University, 2 Kremer I, Nissenkorn I, Lusky M, Yassur Y. Late visual field changes following cryotherapy for 1997 in Miami Beach, Florida, USA. Further Moriguchi, Osaka 570, Japan. (fax: 81-6-997- ROP stage 3. Br J Ophthalmol 1995;79:267–9. details: Stephanie Reisberg or Kimberly 3475.) 424 British Journal of Ophthalmology 1997;81:424

INSTRUCTIONS FOR AUTHORS Br J Ophthalmol: first published as 10.1136/bjo.81.5.415 on 1 May 1997. Downloaded from

Adherence to the following guidelines is ophthalmology. Review articles will be References in the text must be cited in essential if eYcient and expeditious process- approximately 3000-5000 words in length numerical order of first appearance. ing of your manuscript is to be achieved. including references and may contain display References in the list must be given in the Manuscripts will be returned to authors for items (Figures and Tables). Most review arti- numerical order in which they first appear in revision before peer review if they are submit- cles are commissioned but uninvited reviews the text, not in alphabetical order of authors’ ted in incorrect format. Please indicate in a are welcomed. Prior discussion with the names. References with one to six authors covering letter which category of paper your Editor is recommended. All reviews are must include all authors’ names; for references article represents. subject to independent refereeing. with more than six authors the first six should The British Journal of Ophthalmology is an be given and then et al. Titles of journals international journal covering all aspects of should be abbreviated in accordance with the LETTERS TO THE EDITOR 1 clinical ophthalmology and the visual/ Case reports will be published as ‘Letters to Index Medicus or given in full. References to ophthalmic sciences. Contributors should the editor’. These are normally 500–600 books must include names of editor(s) if there consider the widely varying readership and is one, town where published, name of words written in the form of a letter with a 2 write clear, simple articles with the minimum maximum of two display items (Figures and publisher, year, volume, page numbers. of technical detail. Space in the journal is lim- Tables). The letter should include an ited and articles should therefore be as concise introductory section (without heading), the 1 Kaye SB, Shimeld C, Grinfield E, Maitland NJ, as possible. One page of text is approximately case report (heading: Case report) and a com- 1000 words. Hill TJ, Easty DL. Non-traumatic acquisition of ment (heading: Comment), plus a maximum herpes simplex virus infection through the eye. Manuscripts should be sent to the editor of 10 references. Br J Ophthalmol 1992; 76: 412-8. who selects them on the basis of their suitabil- 2 Jakobiec FA, Font RL. Orbit. In: Spencer WB, ed. ity for the journal and of reports from Ophthalmic pathology: an atlas and textbook. 3rd independent referees. Manuscripts are ac- CORRESPONDENCE ed. Philadelphia: Saunders, 1986: 2461-76. Letters are normally constructed in the form knowledged on receipt and the majority References will not be checked in the edito- of scientific correspondence and are usually (>80%) are sent for review. 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REVIEW ARTICLES REFERENCES Substantive review articles will be included In accordance with the Vancouver agreement Reprints under the section ‘Perspective’ and will references are cited by the numerical system. These may be ordered from the OVprint address any aspect of clinical or laboratory They must be typed double spaced. OYce when proofs are returned.