Br J Ophthalmol 2000;84:667–672
`
`667
`
`LETTERS TO
`THE EDITOR
`
`Central serous chorioretinopathy may be
`a manifestation of the primary
`antiphospholipid syndrome
`
`EDITOR,—Central serous chorioretinopathy is
`thought to be an idiopathic disease and such
`cases are not usually investigated for an
`underlying cause. Bullous exudative retinal
`detachment is a recognised complication of
`central
`serous
`chorioretinopathy
`(CSR).1
`There are a number of reports of exudative
`retinal detachments occurring in the context
`of generalised coagulopathies.2 3 We present a
`patient with central serous chorioretinopathy
`who on further investigation was found to
`have the primary antiphospholipid syndrome.
`
`CASE REPORT
`A 42 year old white man presented in Decem-
`ber 1992 with diYculty in reading and a grey
`“circle” in his right central field of vision of 6
`weeks’ duration. His past medical history con-
`sisted of widespread burns requiring skin
`grafting and complicated by septicaemia 20
`years previously. Apart from two short courses
`of antimalarial tablets within the past 6 years,
`he had been taking no other medication. He
`was a non-smoker, but had been a heavy
`drinker. Corrected visual acuity was 6/9 N12
`right eye, 6/9 N5 left eye. Funduscopy
`revealed bilateral retinal pigment epithelial
`(RPE) disturbances with no dye leakage on
`fluorescein angiography. Over
`subsequent
`months, transient serous elevation of the neu-
`rosensory retina at
`the right macula was
`noted. In October bilateral pigment epithelial
`detachments were noted with visual acuities of
`right eye 6/24 N10, left eye 6/60 N48. A trial
`of oral prednisolone seemed to halt
`the
`
`Figure 1 Right fundus showing inferior
`exudative retinal detachment.
`
`Figure 2 B-scan ultrasonograph of right eye
`showing retinal detachment (RD) with probable
`subretinal fibrosis (F).
`
`deterioration in vision and subsequently the
`patient was given 1 g daily of intravenous
`methylprednisolone for 3 days. A subsequent
`autoimmune screen revealed a positive lupus
`anticoagulant test (Russell viper venom with
`Cephalin 1.61 (0.95–1.05), Russell viper
`venom with platelets 1.12 (0.95- 1.05)) and
`the presence of anticardiolipin antibodies
`(anticardiolipin IgG 46.3 gpl units (0–13.3)).
`Antinuclear antibodies were weakly positive at
`1:20 with a homogeneous/speckled pattern
`but anti-double stranded DNA, complement,
`extractable nuclear antigens and rheumatoid
`factor were negative. Steroids were stopped
`and the patient was given aspirin.
`On subsequent
`review the patient had
`developed a large bullous inferior exudative
`retinal detachment in the right eye (Fig 1).
`B-scan ultrasound revealed retinal detach-
`ment with presumed subretinal fibrosis (Fig
`2). Eventually the retinal detachment resolved
`spontaneously with the patient taking aspirin
`alone. Visual acuity is currently right eye 1/60,
`left eye 6/36. The patient manages N8 with a
`reading loupe.
`
`COMMENT
`Idiopathic CSR tends to be a self limiting
`disease4 which can be complicated by the
`development
`of
`exudative
`retinal
`detachments.1 The exact aetiology of CSR
`remains obscure. Indocyanine green angio-
`graphy findings
`suggest
`that
`perfusion
`changes
`in the
`choriocapillaris may be
`responsible.5 Occlusive ocular vascular disease
`involving the retinal and choroidal vessels has
`been reported in up to 8% of patients with
`raised anticardiolipin antibodies.6 Serous de-
`tachment of the macula has been reported
`with the primary antiphospholipid antibody
`syndrome; however, other ocular and systemic
`features were always noted.2
`The presence of antiphospholipid antibod-
`ies (anticardiolipin and lupus anticoagulant)
`can result in abnormalities of coagulation
`leading to venous and arterial thrombosis
`although exactly why this happens is not fully
`understood. Putative mechanisms
`include
`inhibition of antithrombin and activated
`protein C dependent anticoagulant systems,
`inhibition of fibrinolysis, and interference with
`the anticoagulant properties of ♢2 glycopro-
`tein I.7
`We postulate that some cases of presumed
`idiopathic CSR may reflect thrombosis of the
`choroidal circulation secondary to the pri-
`mary antiphospholipid antibody syndrome.
`Further studies are warranted to prove or dis-
`prove this hypothesis.
`
`M T J COSTEN
`Southampton Eye Unit, Tremona Road, Southampton
`SO16 6YD
`J A OLSON
`Eye Department, Aberdeen Royal Infirmary, Aberdeen
`AB25 2ZN
`
`Correspondence to: Mr Costen
`MTJRAC@aol.com
`Accepted for publication 13 January 2000
`
`1 Gass JD, Little H. Bilateral bullous exudative
`retinal detachment complicating idiopathic cen-
`tral serous chorioretinopathy during systemic
`corticosteroid
`therapy.
`Ophthalmology
`1995;102:737–47.
`2 Castanon C, Amigo M-C, Banales JL, et al. Ocu-
`lar vaso-occlusive disease in primary antiphos-
`pholipid syndrome. Ophthalmology 1995;102:
`256–62.
`3 Jabs DA, Hanneken AM, Shachat AP, et al.
`Choroidopathy in systemic lupus erythemato-
`sus. Arch Ophthalmol 1988;106:230–4.
`4 Gilbert CM, Owens SL, Smith PD, et al.
`Long-term follow-up of central serous chorio-
`retinopathy. Br J Ophthalmol 1984;68:815–20.
`
`5 Piccolino FC, Borgia L. Central serous chorio-
`retinopathy and indocyanine green angiography.
`Retina 1994;14:231–42.
`6 Asherson RA, Merry P, Acheson JF, et al.
`Antiphospholipid antibodies: a risk factor for
`occlusive ocular vascular disease in systemic
`lupus
`erythematosus
`and the
`“primary”
`antiphospholipid syndrome. Ann Rheum Dis
`1989;48:358–61.
`7 Greaves M. Thrombosis series: antiphospholipid
`antibodies and thrombosis. Lancet 1999;353:
`1348–53.
`
`Acute retinal necrosis following
`chickenpox in a healthy 4 year old
`patient
`
`EDITOR,—Originally described by Urayama et
`al,1 acute retinal necrosis (ARN) is part of a
`continuous spectrum of necrotising herpetic
`retinopathies where the clinical expression is
`determined by the immune status of
`the
`host.2 3 We report a case of unilateral ARN
`complicated by retinal detachment following
`chickenpox. This is unusual in its severity and
`an extremely rare occurrence in this age
`group. To our knowledge, this patient repre-
`sents the youngest case of chickenpox associ-
`ated ARN.
`
`CASE REPORT
`A previously healthy 4 year old boy presented
`with total retinal detachment secondary to
`ARN of his
`left eye 6 weeks after an
`uncomplicated chickenpox infection. He was
`attending his local ophthalmologist soon after
`chickenpox, having been initially referred by
`an optometrist, where he was treated for
`anisometropic amblyopia. His visual acuity at
`that time was 6/18 right eye and 5/60 left eye,
`and no detachment was noted. However, 4
`weeks later, visual acuity in his left eye had
`dramatically reduced to perception of light
`secondary to a retinal detachment, and he was
`transferred to the vitreoretinal service.
`Examination revealed a dense relative aVer-
`ent pupillary defect (RAPD) and panuveitis in
`the left eye. There was a 250(cid:176) giant retinal
`tear, peripheral retinal necrosis, and a pale
`optic disc (Figs 1 and 2). The retina was com-
`pletely detached. The right eye was normal.
`The patient underwent left vitrectomy with
`silicone oil tamponade and 360(cid:176) endophoto-
`coagulation. Vitreous polymerase chain reac-
`tion (PCR) was negative for herpes simplex
`virus (HSV) and varicella zoster virus (VZV)
`but positive for cytomegalovirus (CMV). A
`repeat CMV PCR on the same vitreous
`sample was negative. Magnetic resonance
`imaging did not
`show any evidence of
`intracranial calcification suggestive of CMV or
`VZV. He had IgM to VZV only. A full
`immunological
`survey was normal. This
`included a normal CD4 count of 1.4 · 109/l
`(range 1.2–2.0 · 109/l).
`He was treated with systemic aciclovir and
`topical steroid and mydriatic following sur-
`gery. Although the operation was anatomically
`successful and the retinitis had resolved,
`acuity remained at perception of light due to
`optic atrophy and was unchanged at 7
`months. His
`right eye remained normal
`throughout. Prophylactic treatment to the fel-
`low eye with systemic aciclovir 400 mg by
`mouth twice daily is planned for 12 months.
`
`COMMENT
`Uncomplicated primary VZV infection (chick-
`enpox) presents initially as maculopapular and
`later vesicular dermal eruptions without any
`ocular or CNS manifestations. ARN is a rare
`consequence this infection in the immuno-
`
`Page 1 of 6
`
`SLAYBACK EXHIBIT 1023
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`

`

`668
`
`Figure 1 Fundus drawing of left eye. Note the giant retinal tear within the necrotic retina.
`
`competent. Previous case reports by Matsuo et
`al 4 5 and Culbertson6 suggest that chickenpox
`associated ARN has good visual prognosis irre-
`spective of the immune status. None of their
`patients had retinal detachments. However, this
`case had an unusually severe retinitis culminat-
`ing in a total retinal detachment and a poor
`visual prognosis.
`Determining the specific cause of ARN is
`important as it has implications as to the
`choice of antiviral therapy. CMV associated
`ARN has
`been
`reported
`in
`healthy
`individuals.7 8 This case had no risk factors,
`clinical
`feature, or serological markers of
`CMV infection. The first vitreous PCR for
`CMV DNA was thus falsely positive, as
`confirmed by a repeat PCR test. Since HSV
`studies were also negative and he had classic
`features of chickenpox, backed by serology;
`the ARN was attributed to primary VZV
`infection. A retinal biopsy was not performed
`in this case because of a clear history of chick-
`enpox infection, although it would have been
`strongly considered had the patient exhibited
`bilateral progressive disease.
`The mode of retinal infection is unclear.
`Possible routes include direct haematogenous
`
`Figure 2 Perioperative photograph of left eye
`showing total retinal detachment.
`
`spread to the retina, or indirectly to the brain
`with subsequent transneural spread to the eye
`shortly after the primary episode.6
`The length of
`therapy is controversial.
`Treatment in the acute phase and prophylaxis
`for the fellow eye is by systemic aciclovir.
`However, since aciclovir
`is only eVective
`against actively replicating virus, and cannot
`eradicate latent virus, any protective eVect is
`debatable.9 Indeed, delayed involvement of
`the fellow eye may be as long as 34 years after
`the initial episode.10
`largely a
`Zoster
`related ARN remains
`disease of adults despite the fact that a major-
`ity of patients acquiring chickenpox are in the
`paediatric age group. Before this case, only
`two patients aged 6 years and 13 years were
`reported.6 10 Since
`chickenpox
`associated
`ARN is extremely rare in children, it would
`not be justified to treat them prophylactically.
`However the physician should be aware of the
`possibility of ARN soon after chickenpox
`infection. Early detection of ARN and prompt
`treatment are required to reduce the risk of
`severe visual loss.
`
`W H LEE
`S J CHARLES
`Vitreo-Retinal Service, Royal Eye Hospital,
`Oxford Road, Manchester M13 9WH
`Correspondence to: Mr Weng H Lee
`whlee@appleonline.net
`Accepted for publication 13 January 2000
`
`1 Uruyama A, Yamada N, Sasaki T, et al. Unilateral
`acute uveitis with periarteritis and retinal
`detachment. Jpn J Ophthalmol 1971;25:607–19.
`2 Guex-Crosier Y, Rochat C, Herbort P. Necrotis-
`ing herpetic retinopathies. Ocular Immunol and
`Inflamm 1997;5:259–65.
`3 Holland GN and executive committee of ameri-
`can uveitis society. Standard diagnostic criteria
`for acute retinal necrosis. Am J Ophthalmol
`1994;117:663–7.
`4 Matsuo T, Nakayama T, Koyama T. A proposed
`mild type of acute retinal necrosis syndrome.
`Am J Ophthalmol 1988;105:579–83.
`5 Matsuo T, Koyama M, Matsuo N. Acute retinal
`necrosis as a novel complication of chickenpox
`in adults. Br J Ophthalmol 1990;74:443–4.
`
`Letters
`
`6 Culbertson WW, Brod RD, Flynn HW, et al.
`Chickenpox associated acute retinal necrosis.
`Ophthalmology 1991;98:1641–6.
`7 Bagliro E, Leuenberger PM, Krause KH. Pre-
`sumed bilateral CMV induced optic neuropathy
`in an immunocompetent person. J Neuro-
`Ophthalmol 1996;16:14–7.
`8 Freeman WR, Stern WH, Gross JG, et al. Patho-
`logic observations made by retinal biopsy. Retina
`1990;10:195–204.
`9 Palay DA, Steinberg P, Davis J, et al. Decrease in
`risk of bilateral acute retinal necrosis by acyclo-
`vir therapy. Am J Ophthalmol 1991;112:250–5.
`10 Falcone PM, Brockhurst RJ. Delayed onset of
`bilateral acute retinal necrosis: a 34 year
`interval. Ann Ophthalmol 1993;25:373–4.
`
`Globe perforation with frameless
`spectacles
`
`EDITOR,—Glasses are perceived to be a
`protective eye shield; however, when lenses
`shatter severe ocular
`injuries may result.
`Several reports discuss the relation of specta-
`cle design to their safety, both the lens and the
`frame contributing spectacle stability.1–4 A
`trend for thin or absent frames may place
`some patients at increased risk of serious ocu-
`lar injury. We present the case of a car driver
`who sustained a perforated globe when glass
`frameless spectacles fractured during a low
`impact collision.
`
`CASE REPORT
`A 37 year old woman wearing, glass, frameless
`spectacles (Rodenstock R244C), collided with
`a lamp-post at around 15 mph (24 km/h). She
`was driving a small hatchback car and the
`impact was suYcient to damage the bonnet and
`windscreen. The car was not fitted with air bags
`but the driver and passenger were wearing three
`point seat belts. The driver’s head fell forwards
`hitting the steering wheel and fracturing her
`spectacles (Fig 1). The force was not suYcient
`to bruise her face. A shard from the spectacles
`lacerated the patient’s left eye.
`When the patient was seen in casualty a 14
`mm, trans-cornea laceration was noted, which
`extended into sclera (Fig 2). The cut was
`clean and linear with no loss of corneal tissue.
`A total hyphaema was present with a brisk
`haemorrhage from the wound. Further exam-
`ination at surgery revealed a clean laceration
`with loss of the crystalline lens and two thirds
`of the inferior iris; no intraocular foreign body
`was found. Primary repair was undertaken,
`prolapsed iris and vitreous were removed. The
`wound was closed with 10/0 nylon to the cor-
`nea and 6/0 Vicryl to the sclera. The patient’s
`postoperative recovery was covered with
`systemic antibiotics,
`topical
`steroids, and
`cycloplegics.
`Following surgery the patient regained hand
`movement vision. Ultrasonography initially
`showed choroidal detachment and vitreous
`haemorrhage. The choroidal eVusions settled
`over 10 days. Postoperative pressure spikes
`were controlled with Iopidine (apraclonidine
`
`Figure 1 The frameless spectacles; fractured by
`a low velocity accident.
`
`Page 2 of 6
`
`SLAYBACK EXHIBIT 1023
`
`

`

`Letters
`
`Figure 2 The patient’s left eye following
`primary repair; note the “clean cut” caused by
`the broken glass.
`
`(0.25% twice
`timolol
`0.1% twice daily),
`daily), Alphagan (brimonidine, 2% twice
`daily) drops, and oral acetazolamide (250 mg
`four times daily).
`At 2 weeks after injury a three port pars pla-
`nar vitrectomy was performed to clear the
`visual axis of haemorrhage, and the retina was
`found to be relatively undamaged. Following
`this procedure the IOP was controlled with
`Iopidine (0.1% twice daily) and timolol
`(0.25% twice daily) and the patient recovered
`to counting fingers vision which was main-
`tained at 6 months.
`
`COMMENT
`The issue of ocular damage from spectacle
`wear has been highlighted by the widespread
`introduction of airbags as a safety feature in
`modern cars.5 The incidence of such injuries
`is diYcult to estimate; however, there are at
`least 140 reported in the literature.1 Myopic
`patients are three times more likely to suVer
`globe perforation than hypermetropes2 owing
`to mechanically weaker spectacle lenses. The
`male to female ratio is 7:1 and one third of
`these eyes become legally blind.6
`Lenses made from polycarbonates are less
`likely to shatter than glass lenses3 4; however,
`even with these lenses spectacle wear presents
`an additional risk for blunt and penetrating
`injury during car accidents.5 Keeney et al
`showed that the tendency of a lens to shatter is
`defined by its stored elastic energy and its ten-
`dency to oscillate after the impact, both higher
`in glass lenses,7 and reduced by compressing
`the lens with a frame.8 Lens breakage may be
`reduced by inserting a 0.1 mm posterior lip in
`
`plastic frames or a plastic liner to metal
`frames. There are several case reports of pen-
`etrating ocular injuries either from the specta-
`cle frame or from shattered glass in patients
`speeds.9 10 Our case
`travelling a modest
`demonstrates
`the eVect of a low speed
`collision on frameless spectacles with glass
`lenses, dramatically increasing the likelihood
`of ocular injury from airbag activation or rela-
`tively minor collisions. Although polycar-
`bonates are often used in the manufacture of
`many frameless spectacles their mechanical
`strength has not been reported in the litera-
`ture. This case raises doubts about the safety
`of these spectacles, especially when made with
`glass lenses.
`
`RICHARD S B NEWSOM
`Southampton Eye Unit, Southampton
`TOM H WILLIAMSON
`St Thomas’s Hospital, London
`Correspondence to: Mr R S B Newsom, Southamp-
`ton Eye Unit, Southampton General Hospital,
`Tremona Road, Southampton SO16 6YD
`Accepted for publication 13 January 2000
`
`1 Keeney AH. Estimating the incidence of specta-
`cle lens injuries. Am J Ophthalmol 1972;73:289–
`91.
`2 Keeney AH, Fintlemann EW, Estlow BR. Refrac-
`tive correction and associated factors in specta-
`cle glass. Trans Am Ophthalmol Soc 1971;60:
`321–35.
`3 Classe JG. An appeal for polycarbonate [edito-
`rial]
`[see comments]. J Am Optom Assoc
`1997;68:141–2.
`4 John G, Feist RM, White MF, et al. Field evalua-
`tion of polycarbonate versus conventional safety.
`South Med J 1988;81:1534–6.
`5 Vichnin MC, Jaeger EA, Gault JA, et al. Ocular
`injuries related to air bag inflation. Ophthalmic
`Surg Lasers 1995;26:542–8.
`6 Parver LM, Dannenberg AL, Blacklow B, et al.
`Characteristics and causes of penetrating inju-
`ries
`reported to the national eye trauma
`registery, 1985–1991. Public Health Rep 1993;
`108:625–32.
`7 Keeney AH, Renaldo D. Newly defined lens and
`frame factors in the reduction of eye injuries.
`Eye, Ear, Nose & Throat Monthly 1973;52:19–
`24.
`8 Keeney AH, Fintelmann E, Estlow B. Refractive
`correction and associated factors in spectacle
`glass. Arch Ophthalmol 1972;88:2–8.
`9 Gault JA, Vichnin MC, Jaeger EA, et al. Ocular
`injuries associated with eyeglass. J Trauma
`1995;38:494–7.
`10 Molia LM, Stroh E. Airbag injury during low
`impact collision [letter]. Br J Ophthalmol 1996;
`80:487–8.
`
`669
`
`Ocular ectodermal syndrome of
`epibulbar dermoid and cutaneous
`myxovascular hamartoma
`
`EDITOR,—Ocular ectodermal syndrome is a
`congenital disorder manifesting classically
`with epibulbar dermoids and aplasia cutis
`congenita. Systemic abnormalities may also
`accompany ocular ectodermal syndrome.1–4
`We describe the case of a newborn boy who
`presented with unilateral epibulbar dermoid
`and myxovascular hamartomas of the scalp.
`We believe that the association of epibulbar
`dermoid and myxovascular hamartoma of the
`skin represents an unusual variant of ocular
`ectodermal syndrome.
`
`CASE REPORT
`A newborn boy was noted at birth to have
`multiple serous purple blisters on the left scalp
`region, measuring 0.5 cm to 2.0 cm in base
`(Fig 1). Anterior fontanelle asymmetry was
`also recognised. The skin lesions were thought
`to be aplasia cutis congenita and a skin biopsy
`was performed. Histopathological examina-
`tion of
`the biopsied skin revealed loose
`connective tissue in the dermis with an appar-
`ent absence of mature collagen, multiple
`blood vessels with thrombi, intimal hyperpla-
`sia and chronic inflammation, and few hair
`follicles, most consistent with pigmented
`myxovascular hamartoma.
`At the 2 week examination, a corneal lesion
`in the left eye was noted and the child was
`referred to the oncology service at Wills Eye
`Hospital for evaluation. On examination, the
`vision was fix light in both eyes. The anterior
`segment examination of the left eye revealed
`an elevated, pink vascular conjunctival mass in
`the superotemporal quadrant measuring 12.0
`mm · 4.0 mm in base and extending onto the
`cornea. The epibulbar mass was not biopsied
`as it appeared consistent with a choristoma,
`most probably dermoid, and it demonstrated
`no change in appearance and no eVect on
`visual acuity (Fig 2). The right eye and the
`fundi of both eyes were normal.
`The patient was the product of a normal
`vaginal delivery and an uncomplicated preg-
`nancy. His birth weight was 2863 g, length
`49.7 cm, and occipitofrontal circumference
`(OFC) 43.2 cm. There was no maternal
`history of tobacco, alcohol, or drug use. The
`
`Table 1 Clinical findings documented in six reported patients with the ocular ectodermal syndrome
`
`Toriello et al (1993)
`
`Gardner and Viljoen (1994)
`
`Birth weight (kg)
`Birth OFC*
`(cm)
`(centile)
`Postnatal macrocephaly
`Craniofacial anomalies
`Parietal bossing
`Frontal bossing
`Skull defect
`(location)
`Prominent eyes
`Epicanthal folds
`Flat nasal bridge
`Ocular anomalies
`Epibulbar dermoid
`Other findings
`
`Ectodermal anomalies
`Aplasia cutis congenita
`Skin hyperpigmentation
`Skin myxovascular hamartoma
`
`*Occipitofrontal circumference.
`
`Patient 1
`
`3.17
`
`36
`(>98th)
`−
`
`−
`−
`−
`
`+
`−
`−
`
`+
`chorioretinal atrophy;
`corneal opacity;
`strabismus
`
`+
`+
`−
`
`Patient 2
`
`Patient 3
`
`4.7
`
`unknown
`
`40.5
`(>98th)
`+
`
`unknown
`unknown
`+/−
`
`+
`−
`−
`
`−
`+
`+
`
`+
`
`+
`+
`−
`
`+
`−
`−
`
`−
`+
`+
`
`+
`abnormal retinal pigment epithelium;
`hypermetropic astigmatism; strabismus;
`small disc right eye; large disc left eye
`
`+
`−
`−
`
`Evers et al
`(1994)
`Patient 5
`
`Present case
`(1998)
`Patient 6
`
`Patient 4
`
`2.74
`
`4.09
`
`34
`(10–25th)
`−
`
`38
`(>98th)
`+
`
`+
`−
`+
`(vertex)
`+
`+
`+
`
`+
`esotropia
`
`+
`−
`−
`
`−
`+
`−
`
`−
`−
`−
`
`+
`
`+
`−
`−
`
`2.86
`
`43.2
`(>98th)
`−
`
`−
`−
`+
`(parietal)
`−
`−
`−
`
`+
`
`−
`−
`+
`
`Page 3 of 6
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`SLAYBACK EXHIBIT 1023
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`

`

`We believe that the association of atypical
`epibulbar dermoid and myxovascular hamar-
`toma of the skin represents a diVerent and
`extremely unusual variant of ocular ectoder-
`mal syndrome.
`
`Supported by the Paul Kayser International Award
`of Merit
`in Retina Research, Houston, TX (J
`Shields) and Eye Tumor Research Foundation,
`Philadelphia, PA.
`
`KAAN GÜNDÜZ
`CAROL L SHIELDS
`YELENA DOYCH
`Oncology Service, Wills Eye Hospital,
`Philadelphia, PA, USA
`BRUCE SCHNALL
`Pediatric Ophthalmology Service
`JERRY A SHIELDS
`Oncology Service
`Correspondence to: Carol L Shields, MD, Oncology
`Service, Wills Eye Hospital, 900 Walnut Street,
`Philadelphia, PA 19107, USA
`Accepted for publication 28 January 2000
`
`1 Frieden IJ. Aplasia cutis congenita: a clinical
`review and proposal for classification. J Am
`Acad Dermatol 1986;14:646–60.
`2 Toriello H, Lacassie Y, Droste P, et al. Provision-
`ally unique syndrome of ocular and ectodermal
`defects in two unrelated boys. Am J Med Genet
`1993;45:764–6.
`3 Gardner J, Vilijoen D. Aplasia cutis congenita
`with epibulbar dermoids: further evidence for
`syndromic identity of the ocular ectodermal
`syndrome. Am J Med Genet 1994;53:317–20.
`4 Evers MEJW, Dijkman-Neerincx RHM, Hamel
`BCJ. Oculo-ectodermal syndrome: a new case.
`Am J Med Genet 1994;53:378–9.
`5 Shields JA, Shields CL. Atlas of eyelid and orbital
`tumors. Philadelphia: Lippincott, Williams and
`Wilkins, 1999.
`
`Foveal translocation with secondary
`confluent laser for subfoveal CNV in
`AMD: 12 month follow up
`
`EDITOR,—Foveal relocation surgery for the
`treatment of subfoveal choroidal neovasculari-
`sation (CNV) secondary to age related macu-
`lar degeneration (AMD) has received signifi-
`cant publicity in the lay press and has given
`rise to some controversy reported in the BJO.1
`We describe the 12 month follow up of a case
`reported in the press in December 1998.
`
`Letters
`
`CASE REPORT
`Mr JB, a 70 year old retired dentist was
`referred from another unit on 31 August 1998
`with AMD and a history of poor vision for 5
`years in the right eye and 5 months in the left.
`Refraction visual acuity was right eye 5/60
`(+1.50/+1.50), left eye 6/60 (+2.25 sphere),
`Snellen equivalent. Figure 1A shows a sub-
`foveal pure classic CNV of less than 1 disc
`area in extent in the left eye. The right eye was
`aVected by end stage fibrotic scar. After full
`discussion of risk/benefit of confluent laser
`and the experimental nature of surgery he
`underwent surgery on 24 September 1998
`(DW). Scleral plication was achieved by using
`14 radially disposed sutures applied to the
`superotemporal quadrant of the globe. A three
`port pars plana vitrectomy was carried out
`and a subtotal retinal detachment was in-
`duced by subretinal infusions via three poste-
`rior retinotomies. The retina was reattached
`with a fluid/air exchange and the fovea was
`manipulated to its final position with a small
`bore flute needle. A radial fold formed in the
`upper nasal aspect of the fundus.
`Figure 2A shows the retina on the 7th post-
`operative day with the superonasal fold and
`Figure 1B the midphase fluorescein angio-
`gram (FA) showing the CNV now located
`superior to the fovea. On 8 October 1998 con-
`fluent laser was applied to the CNV (SPH)
`(Fig 2B). On 5 November 1998 refraction VA
`was 6/15; a small zone of persistent CNV was
`treated. On 8 January 1999 refraction VA was
`6/12. Figure 2C shows a flattening retinal fold
`and Figure 1C a zone of recurrent CNV which
`received repeat confluent laser. On 12 Febru-
`ary 1999 the post-laser follow up FA (Fig 1D)
`shows no persistent
`leakage; the VA had
`improved to 6/9. After 9 and 12 months VA
`was 6/18, N6 (−0.75/+0.50; +3.00 add) with
`no further evidence of CNV recurrence; the
`clinical appearance on 1 October 1999 is
`shown in Figure 2D.
`
`COMMENT
`At present, foveal relocation surgery is the
`only therapeutic option which aims to im-
`prove the vision for patients with CNV caused
`by AMD. The case we report gained a
`
`670
`
`Figure 1 Small purple blistered lesions on the
`scalp consistent with myxomatous hamartomas.
`
`parents were healthy, non-consanguineous,
`and with no history of congenital skin or ocu-
`lar disorders.
`Based on the systemic and ocular findings, a
`diagnosis of ocular ectodermal syndrome was
`suspected. Brain computed tomography re-
`vealed a left parietal bone defect separate from
`the anterior fontanelle abnormality. No other
`calvarial, cerebral, or ventricular
`system
`anomalies were found.
`
`COMMENT
`Aplasia cutis congenita, which is the hallmark
`of ocular ectodermal syndrome, is the con-
`genital absence of skin presenting heterogene-
`ously as an ulcerated, eroded, scarred, or blis-
`tered area with alopecia, usually on the scalp
`near the vertex.1 Our patient was initially felt
`to have aplasia cutis congenita. However,
`pathological examination revealed pigmented
`myxovascular hamartoma of the scalp, charac-
`terised by the sporadic onset of asymmetric
`scalp lesions presenting with subcutaneous
`nodules. Our search of the literature failed to
`disclose any previous reports on the associ-
`ation of cutaneous myxovascular hamartoma
`and epibulbar dermoid, as described in our
`patient. We speculate that our case represents
`an extremely unusual variant of ocular ecto-
`dermal syndrome.
`In addition to the epibulbar dermoid, other
`ocular findings in ocular ectodermal syn-
`drome supported by the literature include
`corneal opacity and strabismus (Table 1).2 3
`Besides the skin and eye findings, associated
`systemic findings in ocular ectodermal syn-
`drome are numerous and can occur in several
`systems including the musculoskeletal system
`(Table 1) Cardiac anomalies in ocular ecto-
`dermal syndrome include atrial septal defect
`and neurological findings include psychomo-
`tor retardation, and seizures.1–5 Although the
`skin and eye findings are both relatively
`innocuous,3 knowledge of the more serious
`associated systemic disorders, such as sei-
`zures, psychomotor retardation and various
`congenital defects, should be recognised.1 In
`addition to the skin and ocular findings, our
`patient had parietal bone defect and anterior
`fontanelle asymmetry.
`
`Figure 2 Oblong vascular corneoscleral
`dermoid in the superotemporal quadrant.
`
`Figure 1 Fluorescein angiogram images before and after foveal relocation surgery (A)
`preoperatively, showing pure classic subfoveal CNV less than 1 disc area in extent; (B) 7 days
`postoperatively showing fovea located below CNV; (C) 5 months postoperatively showing recurrence;
`(D) 4 weeks after confluent laser to recurrence shown in (C) showing complete closure of CNV.
`
`Page 4 of 6
`
`SLAYBACK EXHIBIT 1023
`
`

`

`Letters
`
`Figure 2 Colour fundus photographs of follow up after foveal relocation surgery: (A) 7 days
`postoperatively showing fold extending from disc into superonasal retina; (B) 1 week after (A) showing
`confluent laser applied to CNV and fovea located inferiorly to laser; (C) 5 months postoperative CNV
`recurrence with blood (VA = 6/9); (D) 12 months postoperatively (VA = 6/18).
`
`substantial improvement of vision which was
`at its best 6 months postoperatively but still
`maintained at a good level after 12 months.
`The importance of close angiographic moni-
`toring is illustrated by the development of
`recurrent CNV, which is not unexpected in
`laser treated CNV.2
`Mr JB was one of the first patients to
`undergo foveal relocation as part of a pilot
`study performed with the approval of the
`Liverpool research ethics committee. The
`results and complications of a small consecu-
`tive series of patients treated without scleral
`plications or 360 retinotomies are reported in
`the BJO.3 The reports of our patients and
`those of other series in the literature clearly
`indicate that the vision can be made worse as
`well as better.3–6 Whether the overall surgical
`results are better than natural history remains
`unknown. A prospective randomised control-
`led trial is needed and we are in the process of
`mounting such a trial. We are encouraged by
`the fact that improvements in surgical tech-
`niques are reducing the risks of retinal detach-
`ment and proliferative vitreoretinopathy,3 7
`and also by the long term benefit of a surgical
`approach to AMD as illustrated by this
`patient.
`
`DAVID WONG
`SIMON HARDING
`IAN GRIERSON
`St Paul’s Eye Unit, Royal Liverpool University
`Hospital, Prescot Street, Liverpool L7 8XP
`
`Correspondence to: Mr Wong
`shdwong@mail.msn.com
`Accepted for publication 8 February 2000
`
`1 Leaver PK. Trends in vitreoretinal surgery—time
`to stop and think. Br J Ophthalmol 1999;83:385–
`6.
`2 Macular Photocoagulation Study Group. Persist-
`ent and recurrent neovascularization after laser
`photocoagulation for subfoveal choroidal neo-
`vascularization of age-related macular degenera-
`tion. Arch Ophthalmol 1994;112:489–99.
`3 Wong D, Lois N. Foveal relocation by redistribu-
`tion of the neurosensory retina. Br J Ophthalmol
`2000;84:352–7.
`4 Machemer R, Steinhorst UH. Retinal separation,
`retinotomy, and macular relocation: II. A surgi-
`cal approach for age-related macular degenera-
`tion? Graefes Arch Clin Exp Ophthalmol 1993;
`231:635–41.
`
`5 Wolf S, Lappas A, Weinberger AW, et al. Macular
`translocation for surgical management of sub-
`foveal choroidal neovascularizations in patients
`with AMD: first results. Graefes Arch Clin Exp
`Ophthalmol 1999;237:51–7.
`6 Lewis H, Kaiser PK, Lewis S, et al. Macular
`translocation for subfoveal choroidal neovascu-
`larization in age-related macular degeneration: a
`prospective study. Am J Ophthalmol 1999;128:
`135–46.
`7 Eckardt C, Eckardt U, Conrad H. Macular rota-
`tion with and without counter-rotation of the
`globe in patients with age-related macular
`degeneration. Graefes Arch Clin Exp Ophthalmol
`1999;237:313–25.
`
`The teardrop sign: a rare dermatological
`reaction to brimonidine
`
`EDITOR,—Brimonidine is a potent, highly
`selective ♡
`2 adrenergic agonist used for the
`treatment of open angle glaucoma and ocular
`hypertension. It lowers intraocular pressure by
`decreasing aqueous humour production and
`increasing uveoscleral outflow.1 2 The most
`common ocular and periocular side eVects of
`
`Figure 1 Linear skin blanching (arrows) after
`administration of brimonidine eyedrops.
`
`671
`
`brimonidine include ocular hyperaemia, itch-
`ing, burning, or stinging; foreign body sensa-
`tion, blurred vision, allergic, toxic, or follicular
`conjunctivitis, and lid hyperaemia.3 The fol-
`lowing case describes a previously unreported
`periocular reaction to this medication.
`
`CASE REPORT
`A 23 year old white man with cerebral palsy,
`autism, and chronic open angle glaucoma had
`been treated with brimonidine 0.2% ophthal-
`mic solution for 5 months when erythematous
`cheeks were first noted. The patient’s grand-
`mother questioned the schoolteachers about
`his sun exposure and requested that sunblock
`be applied each day before outdoor activities
`to prevent what she thought was a sunburn.
`Later, after bilateral application of brimoni-
`dine eyedrops, the patient’s cheeks blanched
`in a streak pattern where runoV of the excess
`eyedrop occurred (Fig 1). His cheeks became
`red and his conjunctiva became hyperaemic.
`These eVects lasted throughout the day. They
`recurred with subsequent brimonidine admin-
`istration and later resolved upon discontinua-
`tion of the drug.
`To further investigate this unusual dermato-
`logical reaction, a brimonidine eyedrop was
`placed on the patient’s cheek in a circular
`pattern. The contac