CASE REPORTS AND SMALL CASE SERIES

A Reversal of Siderosis

Retained metallic intraocular for- eign bodies (IOFBs) may cause toxic effects such as and retinal damage. We describe an unexpected course of siderosis in a patient who had undergone removal of an IOFB shortly after injury and then had sid- erosis develop, which subsequently disappeared spontaneously. To our B knowledge reversal of siderosis has not been previously reported.

Report of a Case. A 32-year-old man was seen with an IOFB in the left eye after he had hammered a nail. On ad- mission, visual acuity was 20/20 OD and 20/30 OS. Findings from exami- nation of the right eye were unre- markable. Findings from biomicros- copy of the left eye were normal. A 2-mm scleral laceration was noted 3 mm temporally to the limbus. Fun- duscopy disclosed a metallic IOFB im- pacted in vitreal hemorrhage nasally to the optic disc. The entry wound was repaired immediately, and the next day laser photocoagulation was C applied around the IOFB. After 5 days, pars plana vitrectomy and removal of the IOFB via sclerotomy, using a for- eign-body forceps, were performed. Visual acuity returned to 20/20 OS, the remained clear, the retina was attached, and laser scars were seen na- sally to the optic disc. Three years later the patient complained of a gradual decrease of vision in the left eye. Examination findings revealed that the right eye had remained stable, whereas vi- sual acuity in the left eye had dete- riorated to counting fingers. The 2 were of equal size, but the left eye showed a relative afferent pu- pillary defect and heterochromia Figure 1. The course of siderosis. A, Heterochromia is seen with rust-colored discoloration of the left with a rust-colored discoloration of 3 years after injury. B, Rustlike deposits on the anterior surface of the left lens 3 years after injury. the iris (Figure 1, A). Fine rust- C, Rustlike deposits on the anterior surface of the implanted intraocular lens 4 years after injury. like deposits were seen on the left interior lens as well as progressive reduced amplitude even after mod- strated no metal dissolution in the cataract (Figure 1, B). Findings from erate-intensity stimulus. Com- eye. The patient underwent an un- funduscopy through the cataract dis- puted tomographic findings showed eventful left extracapsular cataract closed a flat retina. Results of elec- no IOFB. Results of repeated diag- extraction with intraocular lens im- troretinography revealed markedly nostic x-ray spectrometry demon- plantation, after which visual acu-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Adverse Effects of Latanoprost on Patients With Medically Resistant Glaucoma Latanoprost is a topical ocular hy- potensive medication (prostaglan- din F2␣ analog) that is used for the reduction of (IOP) in patients with glaucoma. Figure 2. Disappearance of the brownish discoloration of the left iris 5 years after injury. To our knowledge, an associa- tion between cystoid macular edema ity improved to 20/30. Fundus- the lens and washing of the anterior (CME) and the use of latanoprost in copy findings showed a slight pallor chamber the siderosis continued to patients with glaucoma has not of the optic disc, attenuation of reti- progress, manifested by an accumu- been investigated nor reported in the nal vessels, and fine yellow depos- lation of rustlike deposits on the im- literature. We report 2 cases of pa- its around the fovea. The visual field planted lens. Siderosis can occur in tients with glaucoma who were diag- examination results were normal, but cases of retained IOFB, especially in nosed as having CME after 1 month findings on electroretinography the absence of clear ocular trauma.3 of treatment with latanoprost. remained unchanged. Although find- However, it is very unusual to have ings from repeated x-ray spectrom- siderosis develop after extrusion of Report of Cases. Case 1. An 83- etry disclosed no metal dissolution the IOFB. Even more unexpected was year-old woman with primary open- in the eye, heterochromia persisted the fact that the siderosis was ar- angle glaucoma was seen in the clinic and new, fine, rustlike deposits were rested and resolved spontaneously. in 1995. The patient underwent cata- seen on the intraocular lens (Fig- One possibility is that the short de- ract extraction with phacoemulsifi- ure 1, C). Two years later (5 years lay of 5 days in the removal of the cation of the right eye, complicated from initial admission) he returned IOFB contributed to the develop- by a posterior capsular tear and pro- because vision in that eye had again ment of siderosis. Another possibil- lapsed vitreous that requiredi ante- deteriorated. Reexamination showed ity is that there was a second minute rior vitrectomy and anterior cham- a visual acuity of 20/200 OS due to foreign body that was initially unde- ber intraocular lens placement. opacification of the posterior lens tected and caused siderosis. Al- Over the next year, there was capsule. However, the rust-colored though results of electroretinogra- increasing difficulty in controlling discoloration of the left iris and the phy remained abnormal, the final the patient’s IOPs, and eventually la- resulting heterochromia had disap- outcome in visual acuity was good. tanoprost was added to her thera- peared (Figure 2). The patient Other authors4 have also reported a peutic regimen. She was prescribed underwent Nd:Yag capsulotomy and favorable outcome even in cases with latanaprost eyedrops every night, in visual acuity improved to 20/30. a retained metallic IOFB and have addition to 5% timolol maleate (Ti- elected to do follow-up on the re- moptic XE) every night. Comment. This report describes an tained IOFB with use of serial elec- Findings from the initial visit af- unusual case in which, despite the re- troretinography and spectrometry. ter the administration of latanoprost moval of an IOFB, siderosis unex- Insummary,thisreportdescribes demonstrated a decrease in IOP from pectedly developed but was subse- a favorable outcome of siderosis. 25 to 10 mm Hg OD and 15 mm Hg quently arrested and resolved OS. Snellen visual acuity measured spontaneously. The course this con- Ayala Pollack, MD 20/25-3 in each eye. The slitlamp ex- dition took would appear to contra- Moshe Oliver, MD amination findings were unremark- indicate the commonly recom- Jerusalem, Israel able. The patient was instructed to fol- mended treatment involving IOFB low up in 3 weeks, when results of removal concomitantly with or soon Corresponding author: Ayala Pol- clinical examination revealed an IOP after repair of the entry site,1,2 to avoid lack, Department of , of 14 mm Hg OD and 17 mm Hg OS. the development of siderosis and its Kaplan Hospital, PO Box 1, 76100 Re- Visual acuity, however, had dropped possible devastating effects. Our pa- hovot, Jerusalem, Israel. to 20/200 OD and 20/25 OS. Results tient underwent vitrectomy with 1. Glaser BM. Surgical retina. In: Ryan SJ, ed. Retina. of a dilated fundus examination re- thorough cleansing of dust particles Vol 3. 2nd ed. St Louis, Mo: Mosby–Year Book vealed a glaucomatous disc, and from the eye shortly after injury, and Inc; 1994:2361-2364. macular changes consistent with 2. Hope-Ross M, Mahon GJ, Johnston PB. Ocular the eye exhibited no metal dissolu- siderosis. Eye. 1993;7:419-425. CME. Fluorescein angiography was tion in the eye on repeated diagnos- 3. Scotcher SM, Canning CR, Dorrell D. Siderosis not performed. Treatment with the la- bulbi: an unusual cause of a unilaterally dilated tic x-ray spectrometry testing. He . Br J Hosp Med. 1995;54:110-111. tanoprost eyedrops was discontin- nevertheless developed siderosis 4. Neumann R, Belkin M, Loewenthal E, Goro- ued and the patient was instructed to manifested by heterochromia, cata- detsky R. A long-term follow-up of metallic in- return in 1 week. traocular foreign bodies, employing diagnostic ract, and retinal abnormalities. Fur- x-ray spectrometry. Arch Ophthalmol. 1992;110: At the return examination, vi- thermore, even after the extraction of 1269-1272. sual acuity improved to 20/30-3 OD

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 with IOPs of 29 mm Hg and 21 mm Hg OS. The optic nerve exami- tion in high-risk patients who have a mm Hg OD and OS, respectively. Re- nation findings indicated broad rim historyofcomplicatedophthalmicsur- sults of fluorescein angiography at defects in both eyes. Snellen visual gical procedures. The use of latano- this time revealed mild hyperfluo- acuity was 20/25-2 OD and 20/ prost in these patients may exacer- rescence in the right eye, suggest- 40-1 OS. The therapeutic regimen batesubclinicalmacularedema.There ing moderate CME. An area of hy- included betaxolol hydrochloride is a need to conduct further clinical perfluorescence consistent with drops twice daily, 4% pilocarpine epidemiological studies to ascertain window defect was observed infe- drops 4 times a day, and latano- the relationship between latanoprost rior and temporal to the macula, prost drops every night in each eye. and CME. which corresponds with old retinal Findings from the first week fol- pigment changes. No low-up visit were unremarkable. Ini- Arpenik Avakian, MD, PhD other abnormalities were noted. tial IOP was 14 mm Hg OD and 17 Sharon Anderson Renier, MPH An explanation of the risks and mm Hg OS and decreased to 12 Patrick J. Butler, MD benefits of restarting latanoprost mm Hg OD and 14 mm Hg OS 2 Springfield, Ill therapy was discussed with the pa- weeks later. Two months later, the tient. She requested to continue patient’s visual acuity measured Reprints: Patrick J. Butler, Prairie Eye therapy and was reevaluated 2 weeks 20/30-3 OD and 20/200 OS while Center, 2020 W Iles Ave, Springfield, later. Visual acuity decreased to 20/ IOP measurements remained un- IL 62704. 70+2 OD, and 20/25+1 OS with IOP changed. Findings from fluorescein 1. Woodward DR, Regan JW, Lake, Oakline A. The measurements of 18 mm Hg and 17 angiography conducted in the left eye molecular biology and ocular distribution of pros- mm Hg in each eye, respectively. revealed CME. The treatment with la- tanoid receptors. Surv Ophthalmol. 1997;41 (suppl 2):S15-S21. Findings from slitlamp biomicros- tanoprost eyedrops was discontin- 2. Hoyng PFJ, Rulo AH, Greve EL, et al. Fluores- copy of the right fundus showed ued, and 48 hours later, visual acu- cein angiographic evaluation of the effect of la- tanoprost treatment on blood retinal barrier in- CME in the right eye. Fluorescein an- ity measured 20/70 in the affected left tegrity: a review of studies conducted on phakic giography findings revealed in- eye. There was no change in the IOP and aphakic monkeys. Surv Ophthalmol.1997; creased perifoveal leakage in the right measurements. Two months later, vi- 41(suppl 2):S83-S88. eye indicative of CME. Therapy with sual acuity improved to 24/40+2 OS. latanoprost eyedrops was discontin- ued, and the patient was instructed Comment. The results of these case Cystoid Macular Edema to return in 2 weeks. Intraocular pres- studies suggest a possible associa- Associated With sure increased to 27 mm Hg OD and tion between latanoprost therapy and Latanoprost Use 23 mm Hg OS, but there was no CME. Although CME is a common change in visual acuity. Diclofenac clinical side effect of complicated ocu- Latanoprost is a prostaglandin ana- sodium (Voltaren) eyedrops were lar surgeries, the inclusion of latano- log that is being used to reduce in- prescribed for the right eye, and the prost therapy increased the likeli- traocular pressure in patients with patient was instructed to return to the hood of development of CME in the glaucoma. The convenience of its office in 1 month. Subsequent visits cases we reported herein. Visual acu- once-a-day dosing, relative safety, on January 3, 1997, and January 28, ity improved after latanoprost therapy and apparent efficacy have resulted 1997, saw improved visual acuity to was discontinued, and a noticeable in wide acceptance of latanoprost as 20/30-1 and 20/25 OD, respec- increase in IOP was observed in the a valuable adjunct in the treatment tively. Final IOP measurement OD 2 cases, most notably when treat- of glaucoma.1 was 23 mm Hg. ment was rechallenged in case 1. Common adverse effects of la- Case 2. An 84-year-old woman Latanoprost is a prostaglandin- tanoprostuseincludeconjunctivalhy- received a diagnosis of primary open- based therapeutic agent that has been peremia,topicalirritation,andchanges angle glaucoma in both eyes in Janu- shown to be effective in the reduc- in iris pigmentation.2 We report a case ary 1984. Her surgical history involv- tion of IOP in patients with glau- in which cystoid macular edema ing the right eye included cataract coma. The association between pros- (CME) occurred after the initiation of extraction with phacoemulsifica- taglandins and their role in CME has topicallatanoprost.TheCMEresolved tion with posterior chamber intra- been clearly defined by Woodward et afterdiscontinuationofthedrug,with- ocular lens placement in November al.1 Latanoprost has only weak affin- out additional intervention. 1995, and argon laser trabeculo- ity for the receptor sites which are plasty in January 1996. Surgical pro- known to mediate the vascular ef- Report of a Case. An 81-year-old white cedures on her left eye were cataract fects of prostaglandins; however, it has womanhaddecreasedvisionintheleft extraction with phacoemulsifica- been postulated by Hoyng et al2 that eye 3 weeks after being prescribed la- tion with posterior chamber intra- selected prostaglandin analogs may tanoprostforchronicopen-angleglau- ocular lens placement in June 1996 have a direct or indirect effect on the coma. Her ocular history, in addition and endoscopic cyclophotocoagula- blood-retinal barrier and induce CME. to glaucoma, was remarkable for pseu- tion for glaucoma in July 1996. Use of latanoprost therapy while dophakic bullous keratopathy after The patient was started on managing these patients may have ex- cataract extraction of the left eye in treatment with latanoprost in Octo- acerbated these prostaglandin effects. 1993 with placement of an anterior ber 1996. Baseline IOP measure- Our findings suggest that latanoprost chamber intraocular lens because of ments were 16 mm Hg OD and 19 therapyshouldbeprescribedwithcau- intraoperative capsular rupture. She

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 Figure 1. Presentation. Early phase of fluorescein angiogram showing Figure 2. Presentation. Late phase of fluorescein angiogram from Figure 1 punctate areas of hyperfluorescence in superior and temporal macula. showing cystoid macular edema and staining of the optic nerve. Hyperfluorescence of the temporal aspect of the optic nerve is also present.

Figure 3. Ten-week follow-up. Early phase of fluorescein angiogram Figure 4. Ten-week follow-up. Late phase of fluorescein angiogram from revealing a small amount of hyperfluorescence in nasal macula. Figure 3 shows leakage in nasal macula, markedly decreased from initial angiogram. In addition, the optic nerve no longer stains.

underwent penetrating keratoplasty microscopic fundus examination of after discontinuing latanoprost treat- and intraocular lens exchange with the left eye revealed prominent cys- ment, her visual acuity had improved transscleral suturing in of a posterior toid spaces in the macula. Fluorescein to 20/40 OS and the CME was less chamber intraocular lens in 1994. angiography revealed numerous peri- prominent. Seven weeks later, her vi- The patient’s visual acuity sta- foveolar punctate hyperfluorescent le- sion had recovered to her pre-CME bilized at 20/30 OS, but the intraocu- sions in the left macula in the early levelof20/30OS.Thefundusappeared lar pressure remained in the range of phases (Figure 1). These lesions be- normal. Fluorescein angiography re- the mid-20s mm Hg (despite treat- came increasingly more prominent in vealed mild leakage in the left macula ment with 0.5% timolol twice daily). the mid phase of the angiogram, and (Figure 3 and Figure 4). Latanoprost treatment was started, showed leakage in a petalloid pattern once daily in the left eye. in the late phase (Figure 2). In ad- Comment. Latanoprost is a prosta- The patient was seen 3 weeks dition, there was mild staining of the glandin analog developed to re- later complaining of decreased vision optic disc. The diagnosis of CME was duce intraocular pressure in pa- in the left eye. Examination revealed made. Because of the sudden occur- tients with glaucoma. The ocular best-corrected visual acuity of 20/60 rence of the CME with the initiation adverse effects of latanoprost have OS. Intraocular pressure was 13 of latanoprost therapy, the medication been relatively mild, and have largely mm Hg. Anterior segment examina- was stopped and no additional treat- consisted of topical irritation, con- tion of the left eye showed a clear and ment was initiated. junctival hyperemia, and changes in compact graft, a deep and quiet ante- The patient noted improvement iris pigmentation. riorchamber,andawell-centeredpos- in vision within days of stopping Investigators have speculated terior chamber intraocular lens. Bio- latanoprost treatment. Three weeks that prostaglandins can cause retinal

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 vasodilation and vascular leakage, re- 5. Warwar RE, Bullock JD, Ballal D. CME and an- 3 terior uveitis associated with latanoprost use: ex- sulting in CME. While some pros- perience and incidence in a retrospective review taglandins are known mediators of of 94 patients. Ophthalmology. 1998;105:263-268. ocular inflammation and can dis- rupt the blood-retinal barrier, other prostaglandins are effective in reduc- ing intraocular pressure and may de- Persistence of Fetal crease rather than potentiate ocular Vasculature in the Eyes inflammation. Animal and human of Patients With studies have suggested that latano- prost falls into the latter category of prostaglandins.3,4 However, the oc- Incontinentia pigmenti is a rare X- currence of CME within days of linked disorder that affects the eyes, initiation of topical treatment with central nervous system, skin, and latanoprost, and the subsequent teeth.1,2 Ocular abnormalities are seen resolution of CME on discontinua- in approximately 35% of patients3 and tion of treatment without addi- include conjunctival pigmentation, tional intervention, is of concern. strabismus, , optic , Warwar et al5 described 2 patients and retinal abnormalities.2 The main with CME who were receiving la- retinal findings include macular cap- tanoprost therapy. Both, however, illary dropout, peripheral avascular- were treated with topical nonsteroi- ity, arteriovenous anastomoses at the dal anti-inflammatory drugs after junction of the vascular and avascu- diagnosis. Our patient did not re- lar retina, preretinal neovasculariza- ceive treatment other than discon- tion,andretinaldetachment.1 Wenow tinuation of latanoprost, resulting in report2casesofpersistenceoffetalvas- full recovery of vision. Patients re- culature (PFV) in the eyes of patients ceiving latanoprost who complain of with incontinentia pigmenti. Figure 1. Case 1. Top, Ultrasonogram of the decreased vision should be evalu- right eye at 8 months of age reveals a threadlike ated for this potential adverse Report of Cases. Case 1. An 8- echogeneic density extending from the disc to effect. Aphakic and pseudophakic month-old female infant was re- the retrolental space (between the arrows), patients with complex prior surgi- representing a hyaloid artery; on indicates optic ferred from the Department of Der- nerve. Bottom, Artist’s depiction. cal histories may be at increased matology with the diagnosis of risk. incontinentia pigmenti having been vitreous, also known as persistent fe- 4 Jeffrey S. Heier, MD made 1 month prior to our initial ex- talvasculature. Thepatientunderwent Fort Wayne, Ind amination. The family history was un- examination under general anesthe- remarkable. She was the product of a sia with fluorescein angiography at 9 Roger F. Steinert, MD full-term vaginal delivery and had no and12monthsofage.Theanteriorseg- Albert R. Frederick, Jr, MD neonatal oxygen therapy. She was ment of the left eye was normal. The Boston, Mass healthy except for the presence of an posterior pole of the left eye appeared Corresponding author: Jeffrey S. Heier, erythematousvesicularrashsincebirth. normal on ophthalmoscopy, although MD, Retina Vitreous Associates, 7305 An irregular pupil had been noted in an irregularity of the foveal avascular W Jefferson Blvd, Fort Wayne, IN the right eye since age 6 months. She zonewaspresentonthefluoresceinan- 46804. did not respond to a bright light shin- giogram.Examinationoftheretinalpe- ing in the right eye but was able to fix riphery of the left eye disclosed wide- 1. Camras CB, Alm A, Watson P, et al. Latano- and follow objects with the left eye. Iri- spread areas of peripheral nonperfu- prost, a prostaglandin analog for glaucoma dohyaloid vessels (signs of PFV)4 were sion,withascallopedpreretinalwhitish therapy: efficacy and safety after 1 year of treat- ment in 198 patients. Ophthalmology. 1996;103: present in the right eye at the 1-o’clock tuft present at the equator in the 2- 1916-1924. and 10-o’clock meridians. The ante- o’clock meridian. The angiographic 2. Watson P, Stjernschantz J, Latanoprost Study Group. A six-month, randomized, double- rior chamber was formed and deep. A evidence of leakage in the area of the masked study comparing latanoprost with posterior synechia was observed at the white preretinal tissue supported the timolol in open-angle glaucoma and ocular 2-o’clock meridian. A dense white ret- clinical impression of the presence of hypertension. Ophthalmology. 1996;103:126- 137. rolental plaque did not allow a view of neovascularization (Figure 2). Find- 3. Hoyng PFJ, Rulo AH, Greve EL, et al. Fluores- the fundus. Findings from ultrasonog- ings from ultrasonography of the right cein angiographic evaluation of the effect of raphy of the right eye revealed a thin eye, repeated at 9 months of age, re- latanoprost treatment on blood-retinal barrier integrity: a review of studies conducted on pseu- threadlike echogeneic density extend- vealed evidence of dense retrolental dophakic glaucoma patients and on phakic and ing from the disc to the retrolental bloodandfibroustissueandatotaltrac- aphakic monkeys. Surv Ophthalmol. 1997;41 Figure 3 (suppl): S83-S88. space, probably representing a hyaloid tional ( ). 4. Toris CB, Camras CB, Yablonski ME, Brubaker arteryoritsremnant(Figure 1).There The axial lengths were 17 mm OD and RF. Effects of exogenous prostaglandins on aque- was no evidence of intravitreal blood 19 mm OS. ous humor dynamics and blood-aqueous barrier function. Surv Ophthalmol. 1997;41(suppl):S69- orretinaldetachment.Adiagnosiswas Results of ultrasound exami- S75. madeofpersistenthyperplasticprimary nation at 12 months of age dis-

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 matogenous retinal detachment was system, prevent or retard its regres- detected inferonasally in the left eye. sion. Such an imbalance between va- The patient underwent a successful soinhibitory and vasostimulatory scleral buckling procedure. growth factors has been proposed as an explanation for the lack of vascu- Comment. These 2 cases illustrate lar regression in some cases of PFV.4 the simultaneous occurrence of 2 If such an explanation proves to be distinct and rare diseases with intra- valid for incontinentia pigmenti, it ocular vascular abnormalities. This suggests that the genetic abnormal- association of incontinentia pig- ity causing retinal ischemia exerts its menti with PFV (in various stages effects at an early stage of gestation of maturation) has been previously before the spontaneous involution of described in 3 patients.5-7 Zweifach5 fetal vessels normally occurs. Many patients with incontinen- Figure 2. Case 1. Fluorescein angiogram of the reported the presence of an iridohya- left eye reveals leakage in an area of scalloped loid artery in a patient with inconti- tia pigmenti develop funnel-shaped preretinal whitish tuft at the equator that is nentia pigmenti. Pollard6 described retinal detachments or phthisic eyes. consistent with neovascularization. a child with incontinentia pigmenti It is usually assumed that such end- and a “combination of anterior and stage manifestations of the disease are posterior PHPV.” A persistent hya- secondary to preretinal neovascular- loid artery in a case of incontinentia ization that leads to vitreous hemor- pigmenti was also described in the rhage and subsequent tractional reti- German literature by von Krummel nal detachment. This proposed and Rausch.7 The PFV in incontinen- sequence of events may well be valid tia pigmenti may thus be more preva- in some patients. However, as may lent than previously believed. have been the course in case 1, a simi- The exact relationship be- lar end point could be reached by tween these rare coexisting diseases bleeding from persistent fetal ves- is not clear, but it may not necessar- sels within the vitreous chamber. Per- Figure 3. Case 1. Ultrasonogram of the right eye ily be one of chance. Persistence of sistence of fetal vasculature, trig- at 9 months of age reveals total retinal fetal vasculature has been associ- gered by retinal ischemia, may be a detachment surrounding Cloquet canal (arrow) and subretinal dispersed blood (asterisk). ated with various neonatal states of previously unnoticed mechanism of abnormal or deficient retinal vascu- blindness in patients with inconti- closed total retinal detachment and larization. These disorders include nentia pigmenti. dense organization of the vitreous in Norrie disease, of pre- the right eye. The axial length of the maturity, prematurity per se (with- Arman K. Fard, MD right eye had decreased to 16.1 mm, out retinopathy of prematurity), and Morton F. Goldberg, MD consistent with phthisis. The exami- familial exudative vitreoretinopa- Baltimore, Md nation of the left eye remained un- thy.4 Retinal vascular insufficiency is changed at 12 months of age. a feature common to all of these dis- This study was supported in part by Case 2. A 23-year-old woman orders, including incontinentia pig- an unrestricted research grant from with a diagnosis of incontinentia pig- menti.1,3 The poorly vascularized, is- Research to Prevent Blindness, Inc, menti was referred for the manage- chemic retina in these conditions may New York, NY, The Guerrieri Fund for ment of retinal detachment in her left possibly elaborate angiogenic fac- Retinal Research, Baltimore, Md, and eye. Her mother and sister also had tors that facilitate PFV, which, in the Core grant 5P30EY01765-22 from the incontinentia pigmenti. She was the absence of such factors, would ordi- National Eye Institute, Bethesda, Md. product of a full-term uncompli- narily involute prior to birth. Reprints: Morton F. Goldberg, cated vaginal delivery and had no neo- Ischemic retina in various dis- MD, 727 Maumenee Bldg, 600 N natal oxygen therapy. The patient had eases, including retinopathy of pre- Wolfe St, Baltimore, MD 21287. been noted to have persistent hyper- maturity, induces the production of 8 1. Goldberg MF, Custis PH. Retinal and other Mani- plastic primary vitreous in the right vascular endothelial growth factor. festations of incontinentia pigmenti (Bloch- eye at 3 months of age. The visual acu- During intrauterine development, Sulzberger syndrome). Ophthalmology. 1993; ity, at the present examination, was retinal ischemia in patients with in- 100(11):1645-1654. 2. Carney RGJ. Incontinentia pigmenti: a world sta- no light perception OD and 20/20 OS. continentia pigmenti, and in the other tistical analysis.Arch Dermatol. 1976;112:535-542. Findings from slitlamp examination conditions noted above, may trigger 3. Goldberg MF. The blinding mechanisms of in- of the right eye disclosed an iridohya- the production, up-regulation, or ab- continentia pigmenti. Trans Am Ophth Soc. 1994; 4 92:167-179. loid and a white pupil normal persistence of an angiogenic 4. Goldberg MF. Persistent fetal vasculature (PFV): with a cataractous lens. Iris bombe factor such as vascular endothelial an integrated interpretation of signs and symptoms associated with persistent hyperplastic primary vit- was present for 360°. A Mittendorf dot growth factor. The endothelial reous (PHPV): LIV Edward Jackson Memorial Lec- 4 (a common sign of PFV) was pres- growth factor may then directly sup- ture. Am J Ophthalmol. 1997;124:587-626. ent in the left eye. The anterior seg- port PFV, or, by down-regulating 5. Zweifach PH. Incontinentia pigmenti: its asso- ciation with . Am J Ophthalmol. ment in the left eye was otherwise other factors responsible for the at- 1966;62:716-722. normal. A macula-attached rheg- rophy of the fetal hyaloid vascular 6. Pollard ZF. Results of treatment of persistent

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©1998 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/24/2021 hyperplastic primary vitreous. Ophthalmic Surg size, shape, and pattern of spinous ber, or October2 with the typical le- Laser. 1991;22:48-52. 7. von Krummel H, Rausch L. Anomalien des Au- rings. Oral cephalexin was pre- sion that does not respond to antibi- ges bei der sogenannten Incontinentia Pig- scribed for possible secondary infec- otic therapy. Treatment involves menti. Opthalmologica. 1955;130:31-53. tion; the cellulitis resolved in 1 week. removal of the larva and the inflam- 8. Aiello LP, Avery RL, Arriff PG, et al. Vascular en- dothelial growth factor in ocular fluid of patients mation is generally resolved within with diabetic retinopathy and other retinal disor- Comment. Most reported cases of 1 week. ders. N Engl J Med. 1994;331:1480-1487. ocular myiasis have been external to The patient in this report lives the (ophthalmomyiasis ex- near a wooded area of North Caro- 1 Palpebral Myiasis Causing terna). Ophthalmomyiasis externa lina where Cuterebra is endemic. We Preseptal Cellulitis has been associated with larvae of Cu- believe the patient was infected terebra species (rodent or rabbit bots), through the skin at the site of the ini- Human cases of myiasis (infesta- Oestrus ovis (sheep nasal bot), Hypo- tial stinging sensation on the cheek, tion by fly larvae) are rarely ob- derma bovis (cattle grub), and Der- with migration of the larva to the site served in North America. We re- matobia hominis (human bot),1 and of presentation. Previously reported port a case of palpebral myiasis that can be differentiated based on larval cases of ophthalmomyiasis externa appeared as periorbital cellulitis. characteristics and the clinical pre- have involved primary infection of sentation.2 Opthalmomyiasis in- the ocular surface or adnexa. Our case Report of a Case. A 32-year-old man terna (involvement within the globe) is unique in that the larva migrated was seen in August 1997 with red- presents with pathognomonic sub- from a distant site to produce peri- ness and swelling around his right retinal tracks or an intraocular larva.3 ocular involvement simulating pre- eye. The patient had noted a “sting- Human infection with Cutere- septal orbital cellulitis. ing” sensation in his right cheek 2 bra larvae, although rare, is the most weeks earlier. Periorbital edema and frequent cause of myiasis in North Nicholas E. Engelbrecht, MD erythema developed 4 days before American patients who have not trav- R. Patrick Yeatts, MD presentation in association with a eled abroad.2 In contrast, patients Winston-Salem, NC site of serosanguineous drainage in with myiasis who have recently re- Frank Slansky, PhD the right medial canthus. turned from Latin America typically Gainesville, Fla Examination results revealed a harbor D hominis.2 Rodents and rab- track of erythema between the ini- bits are natural hosts of Cuterebra lar- Reprints: R. Patrick Yeatts, MD, Wake tial stinging site and the site of drain- vae. Humans contact the tiny (about Forest University Eye Center, Medi- age, in addition to periorbital ery- 1-mm-long) infective larvae on veg- 2 cal Center Boulevard, Winston- thema and edema (Figure 1). etation or outdoor pets. Larvae typi- Salem, NC 27157-1033 (e-mail: Results of the ocular examination cally enter the host’s body through [email protected]). were otherwise normal, including mucous membranes or skin lacera- normal visual acuity, extraocular mo- tions.2,4 However, a larva may pen- 1. Rodrigues MM, Weiss CB, Muncy DW. Opthal- tility, and slitlamp and fundus find- etrate directly through the skin and momyiasis of the caused by Cuterebra larva. Am J Ophthalmol. 1974;78:1024-1026. ings. A golden, gelatinous foreign develop at this site, or may some- 2. Baird JK, Baird CR, Sabrosky CW. North Ameri- body observed at the site of drain- times migrate to another site.2 Once can cuterebrid myiasis. J Am Acad Dermatol. 1989; 21:763-772. age was removed using forceps and a site is established, the larva cuts a 3. Slusher MM, Holland WE, Weaver RG, Tyler ME. was revealed to be an insect larva hole through the skin, producing a Ophthalmomyiasis interna posterior: subreti- measuring 6ϫ2mm(Figure 2). The furuncle-type lesion, through which nal tracks and intraocular larvae. Arch Ophthal- 4 mol. 1979;97:885-887. larva was identified as that of a bot- it obtains oxygen and excretes. Most 4. Catts EP. Biology of New World bot flies: Cute- fly (Cuterebra species) based on its patients are seen in August, Septem- rebridae. Ann Rev Entomol. 1982;27:313-338.

Figure 1. Initial presentation. Note the site of initial “stinging” sensation (black Figure 2. Cuterebra larva removed from the patient. arrow), track of erythema (arrowheads) and site of larval removal (white arrow).

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