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Somatostatin Scan Positive Gastrinoma Ocular Metastasis

Somatostatin Scan Positive Gastrinoma Ocular Metastasis

1088 Br J Ophthalmol 1999;83:1088–1095 Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from

Numerous subretinal hypopigmented 1 Beaver PC. Intraocular filariasis: a brief review. tracks with small haemorrhages were noted in Am J Trop Med Hyg 1989;40:40–5. 2 Raymond LA, Gutierrez Y, Strong LE, et al. LETTERS TO the superior , and perivascular haemor- Living retinal nematode (filarial-like) destroyed rhages were observed around the inferior with photocoagulation. 1978;85: THE EDITOR branch of the central retinal vein (Fig 2). 944–9. 3 Ittyerah TP. Nematode in the retina. Indian J angiography showed numerous Ophthalmol 1990;38:178–9. hyperfluorescent tracks without dye leakage. 4 Fitzgerald CR, Rubin ML. Intraocular parasite The patient’s blood test revealed a slightly destroyed by photocoagulation. Arch Ophthal- elevated white blood cell count (10 200 ×106/l; mol 1974;91:162–4. 5 Goodart RA, Riekhof FT, Beaver PC. Subretinal Surgically removed submacular normal <9000) and elevated IgE in the serum nematode: an unusual etiology for and nematode (680 U/ml; normal <250). retinal detachment. Retina 1985;5:87–90. On the following day vitrectomy 6 Funata M, Custis P, De la Cruz Z, et al. Intraocular gnathostomiasis. Retina 1993;13: EDITOR,—Intraocular filariasis is an uncom- was performed and the worm was found par- 240–4. mon disease in civilised countries. In only a tially migrated into the subretinal space of the few cases has the worm been surgically macula. The worm was aspirated successfully removed from the posterior portion of the through a 20 gauge silicone tipped needle and occurring with and identified.1 In this report we describe a submitted for parasitological study; however, daily disposable contact wear patient who had a filaria-like worm in an epi- it was lost during transportation to a diVerent macular and/or submacular lesion, which was laboratory. Parasitological study of the pa- EDITOR,—Up to 92% of cases of acan- successfully removed surgically. tient’s serum detected an antibody to Diro- thamoeba keratitis occur in users 1 filaria. Six months after surgery, the patient’s of all types. Failure to disinfect soft contact CASE REPORT visual acuity was still 20/50. Ophthalmoscopi- lenses and the use of chlorine disinfection sys- tems are major factors accounting for the A 38 year old woman complained of de- cally the hypopigmented tracks had faded, and increase in cases of acanthamoeba keratitis.2 creased visual acuity in her left eye of 5 days’ mildly irregular pigment was observed at the It duration. Her medical history was unremark- deep retina in the macular lesion. Cone and is thought that “daily disposable” contact able. The patient had two pet dogs, and she rod electroretinograms to full field stimuli lenses, which require no disinfection, will have had not travelled to foreign countries in recent were normal bilaterally. a much lower risk for the development of all years. On examination, her corrected visual forms of infectious keratitis. We report a case of acanthamoeba keratitis occurring in a daily acuity was 20/20 in the right eye and 20/100 in COMMENT disposable contact lens wearer. We believe this the left. No inflammation was present in the Our patient owned two dogs, and her ocular is the first such reported case. anterior segment or vitreous cavity bilaterally. findings could be diVerentiated from toxo- Ophthalmoscopic examination disclosed a cariasis, in which only larvae of Toxocara canis white worm, approximately 3 disc diameters can infect humans. The length of the Toxocara CASE REPORT in length, equivalent to about 4.5 mm, moving larva is about 400 µm, and grows no longer. A healthy 21 year old woman, who had been slowly in the macula at the epiretina. Epireti- Although no direct microscopical evidence of wearing daily disposable contact lenses for 1 nal and intraretinal haemorrhages were ob- Dirofilaria infection was obtained in our year, wore her lenses for 4 hours on 1 day and served in and around the macular region. The patient, a positive antibody to Dirofilaria in the then stored the lenses in preserved saline solu- body of the worm was tapered at one end and serum indicated its infection. There have been tion overnight in a new contact lens case. She slightly rounded at the other (Fig 1). A round, many reports of intraocular filariasis; however, rewore the lenses for a further 3 hours the fol- preretinal haemorrhage was observed on a the filariae were removed and identified in lowing day. She reports that this was the first branch of the superonasal retinal artery, which only six of 56 cases reviewed by Beaver in time that she had done this. She then could have been the route of entry into the 1989.1 Direct parasitological microscopic ex- developed a painful left eye. This was initially eye. amination is necessary for accurate diagnosis, treated as by her general prac- but serological study can be helpful, as shown titioner and local accident and emergency in the present case. department. After 2 weeks her local eye unit Various types of management for intraocu- suspected acanthamoeba keratitis and per- lar parasites have been reported. Direct formed an epithelial scrape that “revealed http://bjo.bmj.com/ photocoagulation to the worm body has been species”. She was then referred to our successfully reported in cases with filaria-like unit for our opinion. worms23and in one case with insect parasites.4 On examination her visual acuity was It has been suggested that photocoagulation reduced to 6/18 in the left eye. There was con- denatures the parasite proteins and mitigates junctival injection and a mild . The the immune reaction.24 If the parasite is located in the posterior pole of the retina,

however, photocoagulation may cause perma- on September 25, 2021 by guest. Protected copyright. nent , and surgical removal should be selected. Furthermore, photoco- agulation would make parasitological identifi- Figure 1 photograph of the left cation impossible. Preretinal or subretinal posterior pole demonstrates a white worm and preretinal haemorrhage. parasites were retrieved successfully by pars plana vitrectomy in several reported cases.56 In the present case, the visual acuity was still 20/50 6 months postoperatively. The migrat- ing worm may have caused considerable dam- age to macular function, therefore, we believe V that intraocular parasites should be removed Figure 1 The showing di use punctate staining with linear epithelial infiltrates. as soon as possible.

The authors are grateful to Dr Keizo Yamaguchi for parasitological examination and Mr Shingo Yama- zaki for fundus photographs. SHUICHI YAMAMOTO MASANORI HAYASHI SHINOBU TAKEUCHI Department of Ophthalmology, Toho University Sakura Hospital, Sakura, Japan Figure 2 Fundus photograph of the left eye Correspondence to: Shuichi Yamamoto, MD, shows a white worm at the macula, a round, Department of Ophthalmology, Toho University preretinal haemorrhage along the superonasal Sakura Hospital, 564-1 Shimoshizu, Sakura, Chiba retinal artery, and numerous subretinal 2858741, Japan. Figure 2 The cornea showing diVuse punctate hypopigmented tracks in the superior retina. Accepted for publication 19 July 1999 staining with perineural infiltrates. Letters 1089 Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from cornea had diVuse punctate staining with lin- Correspondence to: Mr J K G Dart. visual disturbance in her left eye. Her ear epithelial and perineural infiltrates (Figs 1 Accepted for publication 22 March 1999 corrected visual acuity was 20/20 in her right and 2). She had a mild anterior uveitis. Acan- eye, and 20/100 in her left. The were thamoeba was strongly suspected. Corneal 1 Radford CF, Lehmann OJ, Dart JKG. Acan- equal and reactive normally. Slit lamp and epithelium was removed for microscopy, thamoeba keratitis: multicentre survey in Eng- fundus examination, computed tomography, culture, and histology. Her contact lens case land 1992–6. Br J Ophthalmol 1998;82:1387– magnetic resonance imaging scan, and general 92. and solutions were also sent for culture. Acan- 2 Radford CF, Bacon AS, Dart JKG, et al. Risk examination were normal. HVF 30-2 demon- thamoeba was cultured from both corneal factors for acanthamoeba keratitis in contact strated blind spot enlargement breaking out to epithelium and lens case, but not the lens lens users: a case control study. BMJ 1995;310: the inferotemporal periphery in the left eye 1567–70. solutions. She was treated with topical poly- 3 Seal D, Stapleton F, Dart JKG. Possible enviro- (Fig 1, top). Full field ERG showed grossly hexamethylene biguanide 0.02% (PHMB) mental sources of acanthamoeba spp in contact reduced A and B waves in the left eye. The FA and prednisolone 0.3 % as well as oral flurbi- lens wearers. A case control study. Br J Ophthal- showed slight leakage from peripapillary profen (Froben, Knoll Ltd, Nottingham). Six mol 1992;76:424–7. capillaries. Indocyanine green (ICG) angio- 4 Bacon AS, Frazer DG, Dart JKG, et al. A review weeks after diagnosis she was asymptomatic of 72 consecutive cases of acanthamoeba kerati- graphy showed slight hypofluorescence of the with a visual acuity of 6/9. The eye was quiet, tis, 1984–1992. Eye 1993; 7:719–25. macular area at a late phase. though some corneal infiltrates remained. 5 Ficker L, Hunter P, Seal D, et al. Acanthamoeba Analysis of the mean deviation in HVF over keratitis occurring with disposable contact lens wear. Am J Ophthalmol 1989;108:453. the clinical course corresponded with the COMMENT visual acuity (Fig 1, bottom). In addition, Acanthamoebae are free living com- m-ERG (Veris III, Tomey, Nagoya, Japan) was Clinical course of acute zonal occult monly found in soil and water, including analysed during the clinical course. In this bathroom tap water.3 Acanthamoeba keratitis outer in visual field and examination, the fundus was divided into four is an uncommon but potentially devastating multifocal electroretinogram foci and the sum of amplitudes in each group corneal infection. The number of cases was measured (Fig 2, top). In the left eye, the diagnosed in the United Kingdom has steadily EDITOR,—Patients with acute zonal occult sum of amplitudes was altered individually risen over the past 20 years owing to increased outer retinopathy (AZOOR) may present with but all of them were aVected during the clini- awareness of the condition and the rise in a normal fundus examination and almost nor- cal course. Only in the inferotemporal area did contact lens wear.4 mal fluorescein angiography (FA), despite it correspond with HVF. The values in the severe loss of visual field and electroretino- right eye were about 5000 µV in each focus. Radford et al found that daily wear dispos- 12 able contact lenses were associated with greatly gram (ERG) abnormalities. The lesion This is almost the same as normal volunteers increased risk of acanthamoeba keratitis com- defined zones of the retinal receptor cells; in our clinic (data not shown). 12 however, the cause of the disease remain pared with other lens types and wear systems. 3 unclear. There is also no established conclu- COMMENT Multivariable analysis showed that this was 1 largely attributable to a lack of disinfection, the sion about progression of visual field loss. AZOOR may be precipitated by various use of non-sterile saline, and the use of chlorine With a multifocal ERG (m-ERG), a large retinal disorders and is characterised by rapid based disinfection rather than alternative number of retinal locations can be stimulated visual field loss which cannot be explained by chemical systems. It was concluded that 80% of simultaneously and local responses can be the ophthalmoscopic changes resulting from cases of acanthamoeba keratitis could be extracted independently in a single recording the initiating disease. The ERG is abnormal, session. High resolution topographic mapping indicating that the field loss is due to retinal prevented by the adequate use of an eVective 4 disinfection system. It was felt that the “low of retinal function also is possible. A previous dysfunction.6 The cause of the acute damage report indicates its eYciency in the diagnosis to sharply defined zones of the retinal receptor care” philosophy of daily wear disposable lenses 5 had become “no care” in practice. of AZOOR. There are no reports about the cells in the absence of visible fundus changes Daily disposable contact lenses, in which clinical course. Thus, using m-ERG and static in patients with AZOOR is unknown.7 In the lens is discarded after 1 day’s wear only, perimetry (Humphrey 30-2), the alteration of some previous reports, an apparent response were introduced in 1995. When used properly retinal function in a clinical course of a patient to corticosteroid therapy suggested that an and discarded after a single day’s wear, they do with AZOOR was investigated. inflammatory and perhaps an immune reac- not carry the risks of inadequate lens disinfec- tion may play a part in the disease; however, tion, contaminated lens solutions, and storage CASE REPORT there is no specific evidence for an immune http://bjo.bmj.com/ cases. A case of acanthamoeba keratitis occur- A healthy 26 year old woman presented to our abnormality.68 An infectious aetiology could ring in an extended wear disposable lens outpatient clinic complaining of acute onset of also be the cause of AZOOR.8 wearer has been reported, illustrating that even without the risks of inadequate lens (i)(ii) (iii) (iv) disinfection and contaminated lens cases the infection can occur.5 However, it is known that contact lens wear increases the susceptibility

to keratitis independent of factors relating to on September 25, 2021 by guest. Protected copyright. disinfection and cleaning. Our case of acanthamoeba keratitis in a daily disposable lens wearer illustrates that misuse occurs and when it does so inadequate 0 day (–14.99 dB)22 day (–5.74 dB) 120 day (–11.88 dB) 136 day (–5.15 dB) disinfection is almost inevitable leading to greatly increased risk of acanthamoeba infec- tion. Misuse, as in this case, may often be associated with storage in saline or water with- 0 out disinfection. This has been shown by Rad- ford et al to increase the relative risk of acanthamoeba keratitis to 55.86 (10–302) (iv) * (ii) p<0.001. Our case does not, however, indicate –5 * the mechanism of infection. It is possible that 1.0 the infection is unrelated to the lens misuse and could have been acquired from other 0.5 –10 unrelated sources, such as exposure to con- MD (dB) * taminated tap water while wearing lenses. Left visual acuity (iii) However, we would recommend greater edu- –15 MD of perimetry cation of daily disposable lens wearers on the acuity Visual (i) importance of strict adherence to wearing 0.1 their lenses for 1 day only and of the risks of 0 30 60 90 120 150 misuse of their lenses. Clinical course (days) S A WOODRUFF Figure 1 (Top) Raw images of Humphrey 30-2 visual fields in the left eye. (Bottom) The relation J K G DART between clinical course and mean deviation (MD) of Humphrey 30-2 visual field and visual acuity in Moorfields Eye Hospital, City Road, the left eye. Asterisks indicate the day in which multifocal ERG was analysed. The roman numerals London EC1V 2PD correspond with raw images in the upper part of the figure. 1090 Letters Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from Between October 1997 and November 1997 he was presented to our unit on three occasions complaining of further deteriora- tion of vision to 20/200, IOPs between 3–5 mm Hg, and a full blown hypotonous . After 5 months of hypotony of “unknown origin”, a diagnosis was made by a specialist using indentation gonios- copy revealing a dehiscence of the wound. A preoperative UBM study confirmed the presence of an internal wound gap, behind the limbus, superotemporally, 3 mm in circumference. Ultrasonically the wound de- hiscence was depicted as a narrow slit (Fig 1) with a flat inadvertent bleb above which was not apparent clinically. A surgical repair of the wound was decided upon. Intraoperatively no definite dehiscence could be clinically identi- fied. Balanced salt solution through a 30 gauge needle was repeatedly injected under pressure to the anterior chamber but this failed to localise any suspicious area. At this point, based on the ultrasonic study, two 10-0 nylon interrupted sutures were placed through the parallel to the limbus in the suspicious area. These bites were moderately deep in an attempt to engage the internal flap Figure 2 (Top left) In the multifocal ERG, the fundus was divided into four foci. (Top right) Sum of of the cataract wound. the amplitudes in each foci was altered during the clinical course. (Bottom) The three dimensional Two weeks postoperatively, the pressure topography and sum of the amplitudes in each of four foci of the m-ERG were indicated. normalised (IOP 12 mm Hg), maculopathy was reversed, and visual acuity improved to The findings of AZOOR that we observed Hypotonic maculopathy following 20/50. in our patient suggest that the retinal recovery pneumatic retinopexy: a UBM study Hypotonic maculopathy is an unusual and, assessed by m-ERG was diVerent for the to our knowledge, the first reported lesion (main focus was related to the infero- EDITOR,—Pneumatic retinopexy is a promis- complication12 of this kind after pneumatic temporal retina) and was delayed compared ing surgical alternative in selected cases of retinopexy. We hypothesise that the original with visual acuity and HVF. Perhaps this . cataract wound did not heal properly and the delayed retinal recovery reflects a subtle We report a rare , to our additional cryoprobe manipulation caused the microcirculatory disturbance that can not be knowledge the first ever reported, of this clearly detected by FA or ICG. Slight leakage procedure—namely, hypotonic maculopathy, in FA and slight hypofluorescence on the late caused by a wound dehiscence on a patient phase in ICG would suggest such a microcir- who had previously undergone a standard ext- culation disorder. racapsular extraction with posterior chamber When better understanding of the aetiology implant. Ultrasound biomicroscopy (UBM) and pathophysiology of AZOOR is available, proved to be a valuable adjunct in both the the clinical response to appropriate therapy diagnosis and management of this compli- may perhaps best be followed by m-ERG. cated case. http://bjo.bmj.com/ KANAKO YASUDA MASAHIKO SHIMURA CASE REPORT MICHIRU NORO A 63 year old white man, complaining of a MITSURU NAKAZAWA shadow in his right visual field, was referred to MAKOTO TAMAI Department of Ophthalmology, School of Medicine, our department in April 1997 for further Tohoku University, Sendai 980–8574, Japan management. Ocular history was notable for Correspondence to: Masahiko Shimura, MD, De- bilateral extracapsular cataract extraction with posterior chamber lens implantation (PC/ partment of Ophthalmology, School of Medicine, Figure 1 UBM picture of right eye showing the on September 25, 2021 by guest. Protected copyright. Tohoku University, Sendai, 980-8574, Japan. IOL); right eye in January 1997 and left eye in external part of the dehiscent cataract wound, as June 1996. Visual acuities were 20/25 in both a narrow slit, before repair (area between Accepted for publication 26 March 1999 . IOPs were within normal limits. Anterior arrows). There is a full blown hypotonous segment examination was unremarkable with maculopathy in this eye, with VA 20/200. well positioned IOLs. Fundal examination of 1 Gass JDM. Acute zonal occult outer retinopathy. his right eye revealed a superotemporal Donders Lecture: The Netherlands Ophthal- macula on rhegmatogenous retinal detach- mological Society, Maastricht, Holland, 19 June 1992. J Clin Neuro-Ophthalmol 1993;13:79–97. ment, produced by a single horseshoe retinal 2 Lee AG, Prager TC. Acute zonal occult outer break around 10 o’clock. After the risks and retinopathy. Acta Ophthalmol Scand 1996;74: benefits of pneumatic retinopexy were ex- 93–5. 3 Jacobson SG, Morales DS, Sun XK, et al. Pattern plained to the patient he chose to have the of retinal dysfunction in acute zonal occult outer procedure. retinopathy. Ophthalmology 1995;102:1187–98. Cryopexy was applied to the tear and after 4 Sutter EE, Tran DD. The field topography of ERG components in man. I: The photopic an anterior chamber paracentesis was per- luminance response. Vis Res 1992;32:433–46. formed 0.7 ml of 100% SF6 gas was injected 5 Arai M, Nao-i N, Sawada A, et al. Multifocal into the vitreous cavity 3.5 mm behind the electroretinogram indicates visual field loss in limbus inferotemporally. acute zonal occult outer retinopathy. Am J Oph- thalmol 1998;126:466–9. He tolerated the procedure well and made 6 Holz FG, Kim RY, Schwartz SD, et al. Acute an excellent recovery with the retina totally zonal occult outer retinopathy (AZOOR) asso- attached. He maintained a 20/30 visual acuity ciated with multifocal choroidopathy. Eye 1994; Figure 2 UBM picture of right eye showing the 8:77–83. right eye, IOP remained within normal limits until 3 months later. At that time an IOP of 6 same area after repair. Note that the external 7 Gass JDM. Acute zonal occult outer retinopathy.In: part of the wound is totally closed (area between Stereoscopic atlas of macular diseases: diagnosis and mm Hg and a deterioration of visual acuity to arrows) while internal wound gape appears to be treatment. St Louis: Patterson, 1997:682–3. 20/70 was noted. Initially this visual loss was 8 Jampol LM, Wiredu A. MEWDS, MFC, PIC, more pronounced postoperatively. However, at AMN, AIBSE, and AZOOR: one disease or attributed to a posterior capsule opacification this point the leak is rectified. IOP and macula many? (Editorial.) Retina 1995;15:373–8. and a YAG capsulotomy was performed. are normal with VA of 20/50. Letters 1091 Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from wound to leak. The UBM study provided us represents the entrance wound for the electri- with an interesting insight into how the scleral cal energy but the lack of an exit wound makes suture repair may have worked to correct the this case particularly peculiar. Both entry and wound leak as shown in Figure 2. The fact exit sites for the electric current have been that hypotony resolved after suturing the reported by all previous authors. wound, indicated that the external part of the The excellent surgical results noted in both wound was secure (no slit is apparent) despite eyes of this patient are in keeping with the the fact that the internal part of the wound similar result reported by Portellos et al.2 This was gaping even more postoperatively. This observation should encourage the ophthal- finding implies, therefore, that only minimal mologist to undertake surgery for electric overall alteration of the wound architecture cataract, where necessary, without any undue postoperatively, suYcient to rectify the leak. concern. We believe that UBM is a valuable adjunct USHA K RAINA in the management of similar cases by clearly DEVEN TULI identifying both the presence and exact Guru Nanak Eye Center, Maulana Azad Medical location of leak. Finally, pneumatic retinopexy College, New Delhi, India should be performed with caution, especially Correspondence to: Dr Usha K Raina, 427 Hawa in cases of previously operated eyes with large Singh Block, Asiad Village Complex, New Delhi-110 incision wounds. 049, India. Accepted for publication 7 April 1999 IOANNIS M ASLANIDES Department of Ophthalmology, Vitreo-retinal Unit, St Michael’s Hospital, University of Toronto, 1 Long JC. Electric cataract: report of three cases. Canada and VEIC (Vardinoyannion Eye Institute of Am J Ophthalmol 1966;61:1235–9. Crete), Greece 2 Portellos M, Orlin SE, Kozart DM. Electric CHARLES J PAVLIN Figure 1 The characteristic anterior . Arch Ophthalmol 1996;114:1022. Department of Ophthalmology, Ocular Oncology Clinic, subcapsular lens opacities. (A) Right eye, (B) left eye. Princess Margaret Hospital, University of Toronto, Monckeberg’s sclerosis in temporal Canada artery biopsy specimens LOUIS R GIAVEDONI opacities in both eyes, right greater than left. In the right eye some of these opacities were Department of Ophthalmology, Vitreo-retinal Unit, EDITOR,—Temporal artery biopsies are per- St Michael’s Hospital, University of Toronto, seen encroaching into the visual axis and formed routinely on patients suspected of Canada additionally a few posterior subcapsular having giant cell arteritis. Of 131 pathology Correspondence to: Dr Ioannis M Aslanides, Vardi- opacities were noticed (Fig 1). specimens examined at University of Illinois noyannion Eye Institute of Crete. University of Crete In view of the history of electrical injury and at Chicago Eye Center from 1975 to 1998, the Medical School, Voutes PO Box 1352, Iraklion- classic location and typical appearance of the most common diagnosis was atherosclerosis Crete 71110, Greece. lens opacities, a diagnosis of bilateral electric Accepted for publication 26 March 1999 with myointimal fibrosis (63%) followed by cataract was made. Extracapsular cataract giant cell arteritis (13%). In about 6% of cases extraction (ECCE) with posterior chamber we encountered calcific sclerosis confined to intraocular lens implantation was undertaken 1 Hilton GF, Tornambe PE. Pneumatic retin- the tunica media which was associated with opexy. An analysis of intraoperative and postop- in both eyes, right eye first followed 3 months mild tissue disorganisation surrounding the erative complications. The Retinal Detachment later in left eye. The intraoperative and calcific plaque and disruption of the internal Group. Retina 1991;11:285–94. postoperative course were uneventful and the elastic lamina (Fig 1). 2 Hilton GF, Tornambe PE, Brinton DA, et al. The patient has achieved corrected visual acuity of complication of pneumatic retinopexy. Trans Am Monckeberg’s sclerosis as seen in these Ophthalmol Soc 1990;88:191–207; discussion 20/20 in both eyes. specimens was first described by Monckeberg 207–10. in 1903. It commonly aVects medium size COMMENT

muscular arteries and is described in femoral, http://bjo.bmj.com/ Involvement of the lens exclusively, sparing tibial, radial, coronary, cerebral, and visceral Bilateral electrical cataract other ocular structures is rare. This case arteries.1 However, its association with the EDITOR,—Electric trauma is not uncommon documents such a possibility and also high- temporal artery is uncommon. The infrequent in India where majority of the population lives lights the salient features involving electric occurrence of this condition in the temporal in the rural setting. Few cases of electric cata- trauma to the lens. The scalp burn in this case artery and the presence of a fragmented inter- ract have been reported in literature probably because few patients survive the high voltage of current that induces cataract formation. Most patients with electric cataract have no on September 25, 2021 by guest. Protected copyright. subjective complaints early on but become aware of the reducing visual function several months later.1

CASE REPORT A 26 year old man complained of gradual and painless diminution of vision in both eyes, right more than left, for the past 2–3 years. There was a history of an overhead high tension electric transmission cable acciden- tally falling on the patient’s head 4–5 years earlier. This had resulted in immediate burn to the scalp. The patient was visually asympto- matic till about 1 year after the mishap, when he began to notice the gradual fall in vision that had progressed to its present state. Corrected visual acuity was 20/200 right eye and 20/60 left eye. A 15 × 2.5 cm linear, sagittal scar extending from the frontal to the occipital region of the head was noticed. The lids, , cornea, and pupils showed no abnormality in either eye. Fundus exam- ination was unremarkable. Slit lamp examina- Figure 1 Cross section through temporal artery showing disrupted internal elastic lamina (arrow) tion revealed multiple, mid-peripheral and large calcific plaques (C) in the tunica media (haematoxylin and eosin; original magnification snowflake-like anterior subcapsular lens ×40). 1092 Letters Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from nal elastic lamina should not be erroneously Complejo Hospitalario Universitario de Santiago, interpreted as sequelae of previous arterial c/Galeras s/n E-15705 Santiago de Compostela, inflammation. The pathophysiology of Mon- Spain. ckeberg’s arteriosclerosis is still unclear, but it Accepted for publication 9 April 1999 can be induced in animal models by injecting adrenalin, nicotine, parathyroid hormone, and 1 Poland A. Deficiency of the pectoral muscles. 2 vitamin D. In addition, lumbar sympathec- Guys Hosp Rep 1841;6:191–3. tomy has been shown to promote occurrence 2 Beer GM, Kompatscher P, Hergan K. Poland’s of Monckeberg’s arteriosclerosis of the lower syndrome and vascular malformations. Br J 3 Plast Surg 1996;49:482–4. extremities in humans. Automatic dysfunc- 3 Gass JD, Blodi BA. Idiopathic juxtafoveolar retinal tion from diabetic neuropathy is thought to be telangiectasis. Update of classification and responsible for the occurrence of Moncke- follow-up study. Ophthalmology 1993;100:1536– 4 46. berg’s in diabetic patients. Unlike atheroscle- 4 Park DW, Schatz H, McDonald R, et al. Grid rosis, Monckeberg’s arteriosclerosis is a be- laser photocoagulation for in nign condition and does not cause vascular bilateral juxtafoveal telangiectasis. Ophthalmol- thrombosis. In conclusion, Monckeberg’s ar- ogy 1997;104:1838–46. 5 Welling LW, Grantham JJ. Cystic and develop- teriosclerosis of the temporal artery may be mental diseases of the kidney. In: BM Brenner, seen occasionally in the temporal artery.5 It is FC Rector Jr, eds. The kidney. 4th ed. an interesting histological diagnosis that has Figure 1 Early venous phase fluorescein Philadelphia: WB Saunders, 1991:1657–94. 6 Bavinck JNB, Weaver DD. Subclavian artery little clinical significance but should be recog- angiogram of the right eye, macular area. Note supply disruption sequence: hypothesis of a vas- nised in temporal biopsy specimens. the easily visible telangiectatic retinal capillaries cular etiology for Poland, Klippel-Feil, and (arrows). These abnormal vessels showed Möbius anomalies. Am J Med Genet 1986;23: BIENVENIDO V CASTILLO JR significant leakage of fluorescein in the late phase 903–18. ELISE TORCZYNSKI of the angiogram. DEEPAK P EDWARD Department of Ophthalmology, University of Illinois at Bilateral oedema associated Chicago, Chicago, IL, USA hard exudates, and retinal swelling, all of them with latanoprost Correspondence to: Dr Deepak P Edward, Depart- more prominent in the temporal perimacular ment of Ophthalmology, University of Illinois at area. Fluorescein angiography clearly showed EDITOR,—Latanoprost is a recent addition to Chicago, 1855 W Taylor Street, Chicago, IL 60612, the vascular abnormalities (Fig 1). The left the medical management of raised intraocular USA. eye fundus was normal. A diagnosis of pressure in chronic open angle glaucoma and Accepted for publication 9 April 1999 juxtafoveal retinal telangiectasis3 was made. . It is a potent ocular hypotensive agent with few ocular or systemic 1 Lachman A, Spray T, Kerwin D, et al. Medial COMMENT side eVects. calcinosis of Monckeberg. A review of the prob- We report a case of bilateral optic disc lem and a description of a patient with involve- Juxtafoveal telangiectasis is diYcult to detect 4 oedema developing soon after commencing ment of peripheral, visceral, and coronary arter- ophthalmoscopically and therefore can be ies. Am J Med 1977;63:615–22. neglected by routine eye fundus examinations treatment with latanoprost which resolved 2 Silbert S, Lippmann H, Gordon E. Moncke- made before the first visual symptoms appear. once therapy was stopped. berg’s arteriosclerosis. JAMA 1953;151:1176– 9. Unilateral renal agenesia occurs in approxi- 3 Goebel F, Fuessel H. Monckeberg’s sclerosis mately 1 per 1000 births and can be CASE REPORT after sympathetic denervaton in diabetic and associated with vascular anomalies.5 An asymptomatic 64 year old woman pre- non-diabetic subjects. Diabetologia 1983;24347– 50. The aetiology of juxtafoveal telangiectasis is sented with raised intraocular pressure. She 4 Gentile S, Bizzaro A, Mario R, et al. Medial arte- unknown. The patient we examined had maintained good general health, had no rial calcification and diabetic neuropathy. Acta retinal vascular malformations that may have significant past medical or family history, and Diabetol Lat 1990;27:243–53. was not on any medication. Snellen visual 5 Albert D. Principles of pathology. In: Albert D, originated in the early stages of life. Indeed, Jakobiec, eds. Principles and practice of ophthal- the deformed capillaries may function for acuities were 6/5 both eyes. The intraocular mology 1994;4:2113. pressures were 28 mm Hg right eye and 26

many years before endothelial decompensa- http://bjo.bmj.com/ tion causes retinal swelling.3 While the exact mm Hg left eye. Ocular examination was oth- pathogenesis of Poland’s syndrome is not well erwise unremarkable with open angles, nor- Retinal vascular abnormality in Poland’s known, it has been postulated that the original mal optic nerves, and full Humphrey 24-2 syndrome cause may be a vascular abnormality at the visual fields. She was thus diagnosed as having embryonic stages.6 The vascular abnormali- ocular hypertension and consented to enter a EDITOR,—Poland’s syndrome is a congenital prospective double masked trial comparing anomaly first described in 18411 consisting of ties, mostly of the diseased hemithorax, reported in this syndrome2 and our finding of some of the intraocular pressure lowering unilateral hypoplasia or aplasia of the pectora- drops. Therapy was commenced with one of lis major muscle and ipsilateral upper extrem- retinal vascular abnormalities in this patient support this hypothesis. the drugs involved in the study and at a 1 on September 25, 2021 by guest. Protected copyright. ity abnormalities which often include ipsilat- month review she reported no problems with eral syndactyly. Since then a number of To our knowledge, this is the first reported case of coexistent juxtafoveal telangiectasis, the drops. The intraocular pressures had low- associated anomalies have been reported. ered to 16 mm Hg in both eyes and the exam- These include absence of the pectoralis minor renal agenesis, and Poland’s syndrome. Al- though we are not aware of direct evidence ination was otherwise unchanged. At her third muscle, absence or atrophy of ipsilateral ribs visit 2 months later, she was again asympto- two to five, aplasia of the ipsilateral breast or reported in the literature indicating any retinal involvement linked to this syndrome, it is matic with visual acuities of 6/5 in both eyes nipple, and simian crease of the aVected and intraocular pressures of 15 mm Hg. How- extremity. Although vascular alterations asso- plausible that the three anomalies found in this patient may had have common original ever, examination of the optic nerves revealed ciated with this syndrome have been bilateral oedema which was more prominent 2 vascular causative factors. Therefore, we described, no involvement of eye vasculature in the left eye. There were no signs of uveitis in has been reported so far. recommend a careful eye fundus examination of patients presenting with this syndrome. either eye, pupillary reflexes were normal, col- our vision and Amsler testing were not CASE REPORT aVected, and the visual fields were full. At this We examined a 39 year old man previously This work was partially supported by grants XUGA- point the code for the trial drug was broken diagnosed with Poland’s syndrome who came 20802B97 and DGESIC PB97–0521. and it was seen that she had been using to our clinic because he had experienced FRANCISCO GOMEZ-ULLA latanoprost 0.005% eye drops at night to both blurred vision in the right eye for 2 months. Servicio de Oftalmologia, Facultad de Medicina, eyes over the 3 month period. A neurological Computed axial tomography, arteriography, Complejo Hospitalario Universitario de Santiago, consultation failed to find any neurological and abdominal echography revealed absence E-15705 Santiago de Compostela, Spain abnormality and all haematological and bio- of the left kidney. There was no history of FRANCISCO GONZALEZ chemical analyses were normal. A computed diabetes or hypertension. The ophthalmologi- Servicio de Oftalmologia and Departamento de tomograph scan with contrast showed no cal examination of the right eye revealed a Fisiologia, Facultad de Medicina, Complejo abnormality and she was discharged from visual acuity of 20/50 and a paracentral Hospitalario Universitario de Santiago, neurological review. Follow up in the eye clinic relative . The right eye fundus showed E-15705 Santiago de Compostela, Spain revealed no change after 72 hours. The perimacular capillary telangiectasis, retinal Correspondence to: Dr Francisco Gomez-Ulla or latanoprost was stopped and the disc swelling vascular distortion, moderate perimacular Dr Francisco Gonzalez, Servicio de Oftalmologia, had largely resolved at 1 week. By 10 weeks Letters 1093 Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from both optic nerves looked normal. Visual acui- Somatostatin scan positive gastrinoma ties were still 6/5 in both eyes and there was no ocular metastasis loss of colour vision or visual field. The intraocular pressures had increased to 22 mm EDITOR,—Symptomatic ocular metastases are Hg in both eyes. uncommon despite the 4% prevalence in patients dying of all types of malignancy in 1 COMMENT postmortem series. We report a case of ocular metastasis from a gastrinoma, which was part Latanoprost is a prostaglandin F analogue 2á of the Wermer’s syndrome (multiple endo- which acts by increasing uveoscleral outflow. crine neoplasia (MEN) type 1), diagnosed by Side eVects include increased indium labelled octreotide scanning. pigmentation,1–3 hypertrichosis and increased pigmentation,4 anterior uveitis in patients with complicated glaucoma or in CASE REPORT those having had previous incisional A 57 year old man presented witha1week surgery,56and cystoid macular oedema occur- history of blurring in his peripheral vision in ring soon after beginning latanoprost in pseu- his right eye and severe loss of visual acuity, dophakic or aphakic eyes.6–9 Ocular hypotony worse early morning. He had been diagnosed with choroidal eVusions and facial rash have with MEN type 1, 8 years previously after two also been attributed to latanoprost.9 To the perforated jejunal ulcers (1978, 1990) led to a Figure 2 Image from indium (In-111) labelled best of our knowledge, optic disc oedema diagnosis of Zollinger–Ellison syndrome, and octreotide scan taken 4 hours after injection associated with latanoprost has not previously a hyperplastic parathyroid gland had been showing very avid focal uptake in the right . been described. The mechanism behind this removed for hypercalcaemia (1990). His mother had MEN type 1. association is unclear. One may not be sively seen with neuroendocrine tumours, surprised to see swelling in associ- Ophthalmic examination revealed 6/12 acu- ity in the right eye and 6/5 in the left. His since other tissues have somatostatin recep- ation with signs of posterior uveitis or tors. These include high grade lymphoma, hypotony but in this case it occurred without anterior segments were unremarkable. His right fundus showed a solid amelanotic lesion some small cell lung cancers, occasional any sign of ocular inflammation and the lowest tumours of the breast, and in chronic inflam- recorded intraocular pressure was 15 mm Hg. about one disc diameter above the right disc. Ultrasonography demonstrated a base of 13 matory conditions where there is T cell activa- It may be feasible that the perfusion to the tion including endocrine ophthalmopathy optic nerve heads via the short posterior mm and a height of 8 mm. He also had bilat- eral inferior . One month later the with orbital involvement. Thus, although an ciliary arteries was compromised by a absolute positive diagnosis of metastatic gas- prostaglandin-like action manifesting as disc tumour base measured 14.5 mm and the thickness measured 7.9 mm. The posterior trinoma cannot be made definitively in the oedema. Astin found that latanoprost acid and absence of histology, in the context of this prostaglandin F at high concentrations could edge now practically abutted the optic disc 2á (Fig 1). There was subretinal fluid accumula- clinical case it is highly probable that the cause vasoconstriction of bovine ciliary tion. choroidal tumour is due to ocular metastasis arteries10 and a similar action cannot be A liver ultrasound scan showed multiple from gastrinoma. discounted in this case. The rapid resolution lesions and a tumour biopsy was composed of of the swelling with seemingly no long term small solid islands of polygonal cells with sequelae once latanoprost was stopped would COMMENT granular cytoplasm, diagnostic of metastatic perhaps support this hypothesis. Gastrinoma may occur sporadically or as part neuroendocrine carcinoma (immunostaining of multiple endocrine neoplasia. MEN type 1 OWEN STEWART positive for chromogranin, neuron specific is a rare disorder, usually inherited in an auto- LOUISE WALSH enolase, and NCAM, negative for S-100 and somal dominant fashion with high penetrance MILIND PANDE HMB45 (melanoma markers)). His urinary which aVects multiple endocrine glands (hy- Department of Ophthalmology, 5HIAA was marginally raised at 133 µmol/24 Hull Royal Infirmary, Hull perparathyroid hyperplasia in 80–90%, pan- hours (normal up to 75), and a fasting intesti- creatic islet tumours in 50%, and pituitary Correspondence to: Mr Owen Stewart, Department nal peptide screen, showed a highly elevated adenomas in 40–50%).2 http://bjo.bmj.com/ of Ophthalmology, St James’s University Hospital, gastrin level (on omeprazole 40 mg per day) of Ocular metastasis from gastrinoma has not Leeds LS9 7TF. 343 pmol/l (normal range 0–40 pmol/l) but previously been reported, although it has been Accepted for publication 12 April 1999 normal levels of other polypeptides. reported in other neuroendocrine tumours— No primary tumour or further metastases for example, carcinoid.3 Multiple or metastatic were seen using body computed tomograph gastrinomas (especially if actively secreting) 1 Watson PG. Latanoprost. Two years’ experience and magnetic resonance imaging scans and of its use in the United Kingdom. Latanoprost are best localised by a new radioisotope scan Study Group. Ophthalmology 1998;105:82–7. the I-123 MIBG scan (meta-iodobenzyl gua- using radioactively labelled somatostatin 2 Alm A, Camras CB, Watson PG. Phase III nidine) was also negative. An indium (In-111) 45 analogues. Somatostatin is a peptide elabo- on September 25, 2021 by guest. Protected copyright. latanoprost studies in Scandinavia, the United labelled octreotide scan at 1 and 4 hours rated by the delta cells of the islets of Langer- Kingdom and the United States. Surv Ophthal- +SPET showed focal areas of increased mol 1997;41(Suppl 2):S105–10. hans and the hypothalamus. It inhibits the 3 Bito LZ. Prostaglandins: a new approach to glau- uptake in the right orbit (Fig 2), the nasal secretion of gastrin by gastric mucosa and coma management with a new, intriguing side region, mediastinum, multiple sites in the many other hormones such as insulin, thyro- eVect. Surv Ophthalmol 1997;41(Suppl 2):S1– liver, and possibly other abdomen sites. How- tropin, and corticotropin. Somatostatin recep- 14. ever, a positive octreotide scan is not exclu- 4 Johnstone MA. Hypertrichosis and increased tors are found on neuroendocrine tumours pigmentation of and adjacent hair in (80% of gastrinomas) and indium labelled the region of the ipsilateral of patients octreotide visualises all somatostatin receptor treated with unilateral topical latanoprost. Am J 45 Ophthalmol 1997;124:544–7. positive gastrinomas. Our scans revealed 5 Fechtner RD, Khouri AS, Zimmerman TJ, et al. somatostatin avid liver metastases. Interest- Anterior uveitis associated with latanoprost. Am ingly, the scan also very clearly confirmed the J Ophthalmol 1998;126:37–41. 6 Warwar RE, Bullock JD, Ballal D. Cystoid macu- ocular/orbital disease. Orbital radiotherapy lar edema and anterior uveitis associated with and chemotherapy were recommended.6 latanoprost use. Experience and incidence in a retrospective review of 94 patients. Ophthalmol- KEIR E LEWIS ogy 1998;105:263–8. PAUL B ROGERS 7 Callanan D, Fellman RL, Savage JA. Department of Clinical Oncology Latanoprost-associated cystoid macular edema. JOHN HUNGERFORD Am J Ophthalmol 1998;126:134–5. 8 Gaddie IB, Bennett DW. Cystoid macular edema Department of Ocular Oncology associated with the use of latanoprost. JAm KEITH E BRITTON 1998; :122–8. Optom Assoc 69 Department of Nuclear Medicine 9 Rowe JA, Hattenhauer MG, Herman DC. Adverse side eVects associated with latanoprost. P NICHOLAS PLOWMAN Am J Ophthalmol 1997;124:683–5. Department of Clinical Oncology, St Bartholomew’s 10 Astin M. EVects of prostaglandin E2, F2alpha, Hospital, West Smithfield, London EC1A 7BE and latanoprost acid on isolated ocular blood vessels in vitro. J Ocular Pharmacol Ther Figure 1 Retinal photograph showing Correspondence to: Dr Plowman. 1998;14:119–28. amelanotic neuroendocrine tumour. Accepted for publication 12 April 1999 1094 Letters Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from

1 Nelson CC, Hertzberg BS, Klintworth GK. A deletions seen in microphthalmia with linear histopathologic study of 716 unselected eyes in skin defects (MLS) and that of microphthal- patients with cancer at the time of death. Am J Ophthalmol 1983;95:788–93. mia, dermal aplasia, and 2 Thakker RV. Multiple endocrine neoplasia type (MIDAS), which are now considered to be 1. In: Grossman A, ed. Clinical endocrinology. distinct entities.89The wide variation in sever- 2nd ed. Oxford: Blackwell Science, 1998:621– 34. ity of expression is thought to be due to 3 Riddle PJ, Font RL, Zimmerman LE. Carcinoid mosaicism. tumors of the eye and orbit: a clinicopathologic study of 15 cases with histochemical and A A S DUNLOP electron microscopic observations. Hum Pathol Medical Retinal Unit, Moorfields Eye Hospital, 1982;13:459–69. City Road, London 4 McInerney GF, Carmalt H, MaGee M, et al. Detection of multiple hepatic metastases of gas- J I HARPER trinoma not apparent on CT by In-111 Department of Dermatology, Great Ormond Street octreotide scintigraphy. Clin Nucl Med 1996;21: Hospital for Children, London 730–1. A M P HAMILTON 5 Kisker O, Bartsch D, Weinel RJ, et al. The value of somatostatin-receptor scintigraphy in newly Medical Retinal Unit, Moorfields Eye Hospital, diagnosed endocrine gastroenteropancreatic tu- City Road, London mors. J Am Coll Surg 1997;184:487–92. Figure 2 Fluorescein angiogram of same area Correspondence to: Dr A A S Dunlop, 66 King 6 Minatel E, Trozo MG, Forrer L, et al. The showing peripheral non-perfusion and eYcacy of radiotherapy in the treatment of Street, Newcastle, NSW 2300, Australia. haemorrhage arising from the neovascular Accepted for publication 19 April 1999 intraocular metastases. Br J Radiol 1993;66: complex. The arborisation of the retinal vessels 699–702. appears normal till the transitional zone. 1 Goltz RW, Peterson WC, Gorlin RJ, et al. Focal Retinal neovascularisation in Goltz vessels and haemorrhage. Fluorescein angio- dermal hypoplasia. Arch Derm 1962;86:708–17. graphy showed bilateral peripheral retinal 2 Thomas JV, Yoshizumi MO, Beyer CK, et al. syndrome (focal dermal hypoplasia) Ocular manifestations of focal dermal hypopla- non-perfusion and right temporal neovascu- sia syndrome. Arch Ophthalmol 1977;95:1997– EDITOR,—This is the first reported case of larisation (see Figs 1 and 2). This was treated 2001. Goltz syndrome with documented peripheral conservatively for 2 years; however, after six 3 Warburg M. Focal dermal hypoplasia: ocular and retinal non-perfusion with subsequent retinal bleeds within 3 months indirect retinal photo- general manifestations with a survey of the literature. Acta Ophthalmol 1970;48:525–36. neovascularisation and vitreous haemorrhage. coagulation to the areas of non-perfusion was 4 Hall EH, Terezhalmy GT. Focal dermal hypopla- In the eye this represents solely a mesodermal performed with subsequent new vessel sia syndrome: case report and literature review. J disturbance compared with the more common regression within weeks. Am Acad Dermatol 1983;9:443–51. 5 Marcus DM, Shore JW, Albert DM. Anophthal- cases which present with both mesodermal mia in the focal dermal hypoplasia syndrome. and neuroectodermal disturbances, such as Arch Ophthalmol 1990;108:96–100. colobomata or microphthalmia. COMMENT 6 Holden JD, Akers WA. Goltz’s syndrome: focal Focal dermal hypoplasia is a rare disorder of dermal hypoplasia. A combined mesoectoder- ectodermal and mesodermal dysplasia origi- mal dysplasia. Am J Dis Child 1967;114:292– CASE REPORT nally described by Goltz et al.1 It is character- 300. Goltz syndrome was diagnosed shortly after 7 Temple IK, MacDowall P, Baraitser M, et al. ised by congenital atrophic skin changes often Focal dermal hypoplasia (Goltz syndrome). J birth in a girl with linear lesions of atrophic associated with herniation of the subcutan- Med Genet 1990;27:180–7. skin following Blaschko’s lines on the trunk eous fat; skeletal anomalies, in particular syn- 8 Van den Veyver IB, Cormier TA, Jurecic V, et al. and symmetrical syndactyly of the third to Characterization and physical mapping in dactyly, polydactyly, or adactyly as well as sco- human and mouse of a novel RING finger gene fourth fingers and second to third toes. Initial liosis, kyphosis, spina bifida occulta, rib and in Xp22. Genomics 1998;15:251–61. ophthalmic screening revealed no ocular scapula anomalies; and dental anomalies, 9 Happle R, Daniels O, Koopman RJ. MIDAS anomalies. Dental screening revealed the con- especially hypodontia. syndrome (microphthalmia, dermal aplasia and genital absence of one deciduous incisor. 2 sclerocornea): an X-linked phenotype distinct Ocular anomalies occur in 40% of cases. from Goltz syndrome. Am J Med Genet 1993;47: Screening of the parents, brother, and sister Colobomata have been reported in one third 710–3. was negative for skin, skeletal, and ocular of cases, then less frequently microphthalmia, anomalies. When she was aged 5 years, , , and .3 http://bjo.bmj.com/ routine ophthalmic review showed a vision of Other reported ocular anomalies include ano- sclopeteria associated with right eye 6/9 and left eye 6/5, with a minor phthalmia, corneal clouding, , hetero- airbag inflation right . chromia, and optic atrophy. Rarely Age 7 years, she complained of a brief and may occur as well as lid margin or EDITOR,— sclopeteria is a se- episode of photopsia and floaters in the right conjunctival papillomatous lesions (histologi- vere form of blunt trauma, caused by a high eye. Vision was right eye 6/20 and left eye 6/6. cal angiofibromas).1245 Only one case of velocity object grazing the but not pen- Examination revealed a right vitreous haemor- cloudy vitreous has been reported. This was in etrating it. It is a concussion injury, which rhage, telangiectatic vessels temporal to the usually manifests as severe choroidal and reti- association with microphthalmia, aniridia, and on September 25, 2021 by guest. Protected copyright. macula, and temporal equatorial fibrotic 6 nal rupture associated with haemorrhage but lens subluxation. Retinal sclerosis or hypopig- 1 mentation was reported in Goltz’s original an intact sclera. We report a case of case review.1 No attempt was made to explain chorioretinitis sclopeteria resulting from de- these findings. In our case, there was periph- ployment of an airbag in a stationary motor eral retinal non-perfusion and temporal retinal vehicle. telangiectasia with subsequent neovascularisa- tion and vitreous haemorrhage. CASE REPORT The diVerential diagnosis of retinal vascular A 32 year old pregnant woman was the driver anomalies associated with skin lesions in- in a car accident in which the airbag failed to cludes incontinentia pigmenti (IP) and deploy at the time of impact but inflated when Cockayne’s syndrome. Although focal skin she returned to sit in the driver’s seat approxi- atrophy may occur in IP, the initial skin lesions mately 5 minutes after the accident occurred. are vesicles and bullae which may later The patient was not wearing spectacles and become pigmented maculae. The skin lesions there was no evidence of injury from other in Cockayne’s syndrome are pigmented scars sources. due to light sensitivity and trauma. Neither At presentation the visual acuity was hand syndrome is associated with digital anomalies. movements in the left eye and 6/6 in the right The genetic anomaly in Goltz syndrome eye. Examination revealed evidence of swollen remains to be determined. Most cases are eyelids and marked chemosis and subconjunc- sporadic. It is generally thought to be X linked tival haemorrhages on the left, with no bony dominant with lethality in males, like IP; how- injury and a full range of eye movements. Figure 1 Colour fundus photograph of right ever, 9% of cases are male. These are Anterior segment examination revealed a clear temporal retina showing perfused retina (photo proposed to be the result of half chromatid left cornea and a quiet anterior chamber with right) to peripheral non-perfusion (photo left) 7 with fibrotic vessels and neovascular complex in mutations. Deletions in the region of the a microhyphaema. The lens was clear and the transition zone. (Inferior discoloration is chromosome Xp22 are a suggested site, there was no evidence of subluxation. There artefact.) though these must be diVerentiated from the was no angle recession and the intraocular Letters 1095 Br J Ophthalmol: first published as 10.1136/bjo.83.9.1088h on 1 September 1999. Downloaded from associated with road traYc accidents, they are associated with a number of injuries directly attributable to their inflation. Some of these are serious ocular injuries and it is important for ophthalmologists and others involved with trauma cases to be aware of these complica- tions. A full ophthalmic assessment is manda- tory in all cases and this should include inden- tation ophthalmoscopy. A variety of posterior segment injuries have been reported in the lit- erature but this is the first report of retinitis sclopeteria resulting from airbag deployment. R H Y ASARIA A ZAMAN P M SULLIVAN Moorfields Eye Hospital, City Road, Figure 1 Airbag injury with retinitis London EC1V 2PD sclopeteria, an intragel haemorrhage, and attached retina. Correspondence to: Mr Asaria. Accepted for publication 19 April 1999 pressure was recorded at less than 4 mm Hg. Fundal examination revealed evidence of a retinal tear although details were obscured by 1 Richards RD, West CE, Meisels AA. Chorio- adiVuse vitreous haemorrhage. An ultrasound retinitis sclopeteria. Am J Ophthalmol 1968;852–60. examination showed a vitreous haemorrhage 2 Whitacre MM, Pilchard WA. Air bag injury pro- and large retinal tear but no evidence of a ducing retinal dialysis and detachment [letter]. scleral perforation. Arch Ophthalmol 1993;111:1320. Figure 1 Specular microscopic findings. (A) An examination under anaesthesia per- 3 Lesher MP, Durrie DS, Stiles MC. Corneal Many crystalline deposits are observed at the edema, , and angle recession after air limbus. (B) Specular microscopic findings formed the following day confirmed that there bag inflation [letter]. Arch Ophthalmol 1993;111: was no scleral rupture and indirect ophthal- re-examined after 1 year. The change of the 1320–2. locations and forms of crystalline deposit from the moscopy confirmed the findings of diVuse vit- 4 Manche EE, Goldberg RA, Mondino BJ. Air same vessels can be seen. reous haemorrhage and a retinal tear. bag-related ocular injuries. Ophthalmic Surg 1997; :246–50. At the 2 week postoperative clinic visit, Lasers 28 5 Rosenblatt MA, Freilich B, Kirsch D. Air deposits were found in the patients with more visual acuity had improved to 6/18. There was bag-associated ocular injury [letter; comment]. advanced retinopathy. It is supposed that cor- 2+ cells in the anterior chamber and the Arch Ophthalmol 1993;111:1318. neal deposits and fundus deposits are essen- intraocular pressure was 24 mm Hg. Posterior 6 Scott IU, John GR, Stark WJ. Airbag-associated ocular injury and periorbital fractures [letter]. tially the same and it is suggested that crystal- segment findings were an intragel haemor- Arch Ophthalmol 1993;111:25. line retinopathy is caused by systemic rhage and retinitis sclopeteria with no evi- 7 Han DP. Retinal detachment caused by airbag abnormality. Although the exact pathogenesis dence of retinal detachment. injury. Arch Ophthalmol 1993;1991:1333–4. of crystalline deposits is still uncertain, it is Four months later, the best corrected visual 8 Vichnin MC, Jaeger EA, Gault JA, et al. Ocular injuries related to air bag inflation. possible that destroyed fibroblasts appear to acuity was 6/36. The anterior chamber activity Ophthalmic Surg Lasers 1995;26:542–8. glitter or fibroblasts with crystalline-like de- had settled; the intraocular pressure was 10 posits look glittering during breakdown. Fur- mm Hg, and fundal examination was un- ther biochemical or cellular biological studies changed. In view of the persistent vitreous Specular microscopic findings of corneal are needed to clarify these possibilities. haemorrhage the patient was listed for routine deposits in patients with Bietti’s pars plana vitrectomy in the postpartum crystalline corneal retinal dystrophy YUKO WADA period. Department of Ophthalmology, Tohoku University

1 http://bjo.bmj.com/ EDITOR,—In 1937, Bietti first described three School of Medicine, Sendai Japan COMMENT cases of tapetoretinal degeneration character- MITSURU NAKAZAWA Airbags are designed to protect the driver ised by yellowish glistening retinal crystals, Department of Ophthalmology, Hirosaki University from direct impact from the steering wheel, tapetoretinal degeneration with choroidal School of Medicine, Hirosaki, Aomori, Japan dashboard, and windscreen. They are de- sclerosis, and marginal crystalline deposits of TOSHIAKI ABE signed to inflate in 10 ms in response to sud- the cornea. Although more than 100 cases of TAKASHI SHIONO den deceleration and during deployment, the crystalline retinopathy have been reported, MAKOTO TAMAI airbag is propelled out of its storage compart- crystalline deposits of the corneal limbus have Department of Ophthalmology, Tohoku University School of Medicine, Sendai Japan 2 been observed in only four out of 52 Japanese ment at speeds of more than 100 mph. on September 25, 2021 by guest. Protected copyright. Following inflation the airbag deflates slowly patients with crystalline retinopathy. Recently, Correspondence to: Yuko Wada, MD, Department 23 of Ophthalmology, Tohoku University School of within seconds. observation using specular microscopy has been reported to be useful in detecting Medicine, 1-1 Seiryo-machi, Aoba-ku, Sendai, 980- Facial and ocular injuries associated with 8754, Japan. airbags have been reported in the literature. crystalline deposits at the limbus of patients Accepted for publication 19 April 1999 Skin abrasions, burns, and ecchymoses with crystalline corneal retinal dystrophy. are the most common facial injuries. Reported Therefore, in this study, we examined four ocular injuries include orbital fractures, kera- patients with crystalline retinopathy using 1 Bietti G. Ueber familiares Vorkmmen von titis, corneal abrasions, hyphaemas, angle specular microscopy under a “con-surface” Retinitis punctata albescence (Verbunden mit “Dystrophia marginalis cristallinea cornea). recession, and lens subluxation. In the poste- mode, which is used for the observation of the Glitzen des Glaskopers und andeen degenera- rior segment, vitreous and retinal haemor- corneal surface, and we detected the deposits tiven Augenveranderungen Klin Monatsbl Au- rhages, commotio retinae, retinal tears and at the limbus. genheilkd 1937;99:737–56. 2 Morimoto M, Shiragami F, et al. A case of crys- dialyses, and choroidal ruptures have been Twelve months after the initial specular talline retinopathy with crystalline deposits in 3–8 reported. To our knowledge, this is the first microscopic examination, we reinspected the the cornea. Nippon Ganka Gakkai Zasshi reported case of retinitis sclopeteria secondary crystalline deposits of two cases. Interestingly, 1990;94:321–7. to airbag inflation. the changes in the locations and forms of the 3 Takikawa C, Miyake Y, Yagasaki K. Re- evaluation of crystalline retinopathy based on Although airbags have clearly been shown crystalline deposits in the corneal limbus were corneal findings. Folia Ophthalmol Jpn 1992;43: to reduce serious morbidity and mortality exposed over time (Fig 1). More crystalline 969–78.

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