conjunctival injection (2�), presence of mucous strands inthe inferior fornix, and trichiasis, LE.

An area of peripheral corneal thinning (10%) inferona-sally in the right eye was noted (Figure 1). The anteriorchamber was quiet and deep; there was early bilateralcataract formation; and intraocular pressure was normal.Funduscopic examination was within normal limits.

Histopathology of a conjunctival biopsy (Figure 2)revealed impressive microangiopathy with vaso-occlusionand diffuse inflammatory cell (mainly lymphocytic) infil-tration of the conjunctival stroma, features that are nottypical of the pathema seen in ocular cicatricial pemphi-goid biopsy specimens. The epithelial goblet cell popula-tion was normal. But the conjunctival epithelial basementmembrane, on direct immunofluorescence study, was pos-itive for linear deposition of Ig A and C3, findings that arediagnostic of ocular cicatricial pemphigoid.

The patient died of pulmonary complications fromWegener granulomatosis 1 week after our evaluation,before our formal report of the biopsy findings had reachedthe referring physicians.

This case is presented as an example of a complex andrare situation of two concomitant autoimmune diseases.

The immune system dysregulation present in cicatricialpemphigoid leads to progressive scarring of mucous mem-branes, including the conjunctiva. The clinical diagnosisof ocular cicatricial pemphigoid must be confirmed withconjunctival biopsy and immunofluorescence studies, be-cause other disorders may mimic it, and correct treatmentfor one cause of chronic cicatrizing conjunctivitis may beincorrect for another cause.1,2

Although fatal complications from systemic involve-ment of cicatricial pemphigoid rarely occur, about half ofthe patients with ocular involvement may have associatedextraocular lesions, including esophageal. Ocular cicatri-cial pemphigoid is a systemic autoimmune disease thatrequires systemic treatment.1–3

This patient eventually serendipitously received sys-temic immunosuppressive chemotherapy for an unrelatedautoimmune disease, limited Wegener granulomatosis, di-agnosed by biopsy of nasal and laryngeal mucosa. Thefeatures that led the pathologist to diagnose Wegenergranulomatosis from this material, rather than cicatricialpemphigoid involvement of these mucosa, were the ex-treme degree of granulomatous inflammation with vascularinvolvement and negative immunofluorescence study.

Surprisingly, despite therapy with methotrexate, theocular and extraocular mucosal inflammation of the pa-tient persisted, and he developed peripheral ulcerativekeratitis, prompting referral to our Service. Conjunctivalbiopsy disclosed features of both active Wegener granulo-matosis and ocular cicatricial pemphigoid, which indicatea lack of control of both diseases and prompting us torecommend therapy with cyclophosphamide, particularlyin light of the peripheral ulcerative keratitis, a sign of poorprognosis in patients with Wegener granulomatosis.4–6

Regrettably, the patient expired before institution of ther-apy.

This case, we believe, highlights three important pointsworthy of emphasis:

1. Chronic conjunctivitis may be caused by severaldifferent entities, and occasionally by more than onedisease, all of which must be effectively treated if oneis to expect clinical success.

2. Conjunctival biopsy, with effective histopathologicand immunopathologic analysis, is essential if one isto maximize the likelihood that correct diagnosis willbe made.

3. Most cases of chronic cicatrizing conjunctivitis re-quire systemic therapy for successful intervention,whether that be with tetracycline for rosacea, anti-histamine for atopy, or immunosuppressive chemo-therapy for cicatricial pemphigoid, Wegenergranulomatosis, systemic lupus erythematosus, andother systemic autoimmune diseases.

REFERENCES

1. Foster CS, Wilson LA, Ekins MB. Immunosuppressive therapyfor progressive ocular cicatricial pemphigoid. Ophthalmology1982;89:340–353.

2. Power WJ, Neves RA, Rodriguez A, et al. Increasing thediagnostic yield of conjunctival biopsy in patients with sus-pected ocular cicatricial pemphigoid. Ophthalmology 1995;102:1158–1163.

3. Mondino BJ, Brown SI. Immunosuppressive therapy in ocularcicatricial pemphigoid. Am J Ophthalmol 1983;96:453–459.

4. Fauci AS, Haynes BF, Katz P, et al. Wegener’s granulomatosis:prospective clinical and therapeutic experience with 85 pa-tients for 21 years. Ann Int Med 1983;98:76–83.

5. Straatsma BR. Ocular manifestations of Wegener’s granulo-matosis. Am J Ophthalmol 1957;44:789–799.

6. Flach AJ. Ocular manifestations of Wegener’s granulomatosis.JAMA 1995;274:1199–1200.

Vitreous Hemorrhage From a CiliaryGranuloma Associated With WegenerGranulomatosisMotohiro Kamei, MD, Toru Yasuhara, MD,Mamoru Tei, MD, Yoichiro Sano, MD,Kazuhito Yoneda, MD, andShigeru Kinoshita, MD

PURPOSE: To report a case of vitreous hemorrhage fromciliary granuloma in Wegener granulomatosis.

Accepted for publication Jun 25, 2001.From the Department of Ophthalmology, Kyoto Prefectural University

of Medicine, Kyoto, Japan.Inquiries to Motohiro Kamei, MD, Department of Ophthalmology,

Kyoto Prefectural University of Medicine, Kawaramachi Hirokoji, Kami-gyo-ku, Kyoto 602-0841, Japan; fax: �81-75-251-5663; e-mail:mkamei@ophth.kpu-m.ac.jp

AMERICAN JOURNAL OF OPHTHALMOLOGY924 DECEMBER 2001

METHODS: Interventional case report. An 18-year-oldwoman with Wegener granulomatosis and episcleralgranuloma in her LE had ultrasound biomicroscopyfindings of a mass in the pars plana of the ciliary body inthe meridian corresponding to the episcleral granuloma.RESULTS: The patient underwent vitrectomy in the LEfor subsequent vitreous hemorrhage. Intraoperatively,the mass was diagnosed as a ciliary granuloma at the parsplana. Dense blood clotting around the ciliary granulomaand subretinal exudation at the ora serrata were ob-served, with no other changes causative for the vitreoushemorrhage.CONCLUSIONS: We report a case of vitreous hemorrhageassociated with a ciliary granuloma that was revealed byultrasound biomicroscopy. Careful observation is neces-sary in eyes with persistent inflammation in Wegenergranulomatosis. (Am J Ophthalmol 2001;132:924–926. © 2001 by Elsevier Science Inc. All rightsreserved.)

WEGENER GRANULOMATOSIS IS A SYSTEMIC, NECRO-

tizing, granulomatous vasculitis of unknown originthat has a clinical predilection to involve the upperairways, lungs, and kidneys. Ocular manifestations are seenin 28% to 87% of patients1; however, vitreous hemorrhageis an unusual complication of this disease. Here, we reporta rare case of vitreous hemorrhage, possibly from a ciliarygranuloma in Wegener granulomatosis. The study design isan interventional case report.

● CASE: The patient, an 18-year-old woman, was firstdiagnosed with Wegener granulomatosis in August 1996on the basis of clinical features of inflammation involvingthe nose and kidneys, with positive increase of antineu-trophil cytoplasmic antibody, biopsy of the concha nasalisinferior showed vasculitis and granuloma formation withinflammatory cell invasion of the mucosa. She was foundto have bilateral episcleritis in January 1997; episcleralgranuloma formation followed with corneal invasion in her

FIGURE 1. (Left) Persistent episcleral granuloma with corneal vascular invasion at the 6 o’clock meridian in the LE. (Right)Intraoperative view of inferior peripheral fundus with scleral indentation. Subretinal exudation at ora serrata (asterisk) iscontinuous with granuloma at pars plana (arrow) at the 6 o’clock meridian.

FIGURE 2. Ultrasound biomicroscopy images. (Left) Normal part of LE. (Right) Mass at ciliary body (arrow heads) adjoinsepiscleral mass (arrow) along same meridian.

BRIEF REPORTSVOL. 132, NO. 6 925

LE (Figure 1). During a clinical course of repeated remis-sion and exacerbation that resisted various anti-inflamma-tory treatments, a vitreous opacity developed in the LE andultrasound biomicroscopy disclosed a mass within the eyeat the pars plana adjacent to the episcleral granuloma(Figure 2). Fluorescein angiography disclosed leakage intothe vitreous cavity at the late phase, but neither vasculitisnor neovascularization was seen. Subsequent vitreous hem-orrhage deteriorated visual acuity to counting fingers andwas treated with a vitrectomy combined with episcleralgranuloma resection and keratoepithelioplasty. Intraoper-atively, dense blood clotting around the mass at the parsplana and subretinal exudation at the ora serrata contin-uous with the mass were observed (Figure 1). Although themass was not wholly exposed because of the risk of aniatrogenic tear during removal of the coagula around it, weconcluded that it was most likely a ciliary granuloma,given its appearance. No neovascularization or otherchanges causative of the vitreous hemorrhage were de-tected during surgery. The surgery was successful, and thepatient recovered with a visual acuity of 20/30 1 monthafter surgery. She is now following at our outpatient clinicafter secondary vitrectomy in 6 months for tractionalretinal detachment associated with persistent inflamma-tion. Her right eye has shown no recurrence after regres-sion of initial episcleritis.

The vitreous hemorrhage in this case was suspected tohave originated from the ciliary granuloma. Few reportsexist on the vitreous hemorrhage associated with Wegenergranulomatosis.1 We are unaware of previous reports ofvitreous hemorrhage from granuloma and could find noreference to it in a computer search using Pubmed. Allvitreous hemorrhage in previous reports resulted fromretinal vasculitis or vascular occlusion.

Ciliary granuloma is also an uncommon ocular manifes-tation of Wegener granulomatosis; only three cases havebeen reported.2–4 The ciliary granulomas in those caseswere histopathologically demonstrated to be continuouswith the episcleral granuloma. The ciliary granuloma inour case as well seemed to be continuous with theepiscleral granuloma, because they were joined along thesame meridian revealed by ultrasound biomicroscopy, andtheir activity seemed synchronous. Some sensitizing sub-stances or autoantigens5 causative of the persistent inflam-mation may have been present in the region. Consideringthat scleritis is a common finding in Wegener granuloma-tosis,1 the apparent rarity of ciliary granuloma may becaused by the difficulty of its visualization. Because ultra-sound biomicroscopy is useful in detecting lesions insidethe anterior eye, the incidence of ciliary granuloma mayincrease through ultrasound biomicroscopy examinationwhen episcleral granuloma is present.

The findings in the present case suggest that a scleralgranuloma can progress to both sides of the sclera, thatultrasound biomicroscopy is useful in detecting a ciliarygranuloma, and that vitreous hemorrhage can occur from a

ciliary granuloma. Therefore, ciliary granuloma should besought when a persistent episcleral mass is present, andwhen a ciliary granuloma is revealed inside the eye, thepatient should be informed of the possibility of vitreoushemorrhage. Careful observation is necessary for eyes withpersistent granuloma formation associated with Wegenergranulomatosis.

ACKNOWLEDGMENT

Our thanks to Dr. Tatsuya Iwami, Department of Ophthal-mology, Shiga Medical College, for providing the UBMimages.

REFERENCES

1. Harman LE, Margo CE. Wegener’s granulomatosis. SurvOphthalmol 1998;42:458–480.

2. Cutter WM, Blatt IM. The ocular manifestations of lethalmidline granuloma (Wegener’s granulomatosis). Am J Oph-thalmol 1956;42:21–35.

3. Frayer WC. The histopathology of perilimbal ulceration inWegener’s granulomatosis. Arch Ophthalmol 1960;64:58–64.

4. Janknecht P, Mittelviefhaus H, Loffler KU. Sclerochoroidalgranuloma in Wegener’s granulomatosis simulating a uvealmelanoma. Retina 1995;15:150–153.

5. Reynolds I, Tullo AB, John SL, Holt PJ, Hillarby MC.Corneal epithelial-specific cytokeratin 3 is an autoantigen inWegener’s granulomatosis-associated peripheral ulcerativekeratitis. Invest Ophthalmol Vis Sci 1999;40:2147–2151.

Serial Axial Length Measurements inCongenital GlaucomaSimon K. Law, MD, David Bui, MD, andJoseph Caprioli, MD

PURPOSE: To illustrate the utility of axial length measure-ments in the management of congenital glaucoma.METHODS: Interventional case series. Twelve eyes of sixconsecutive patients with congenital glaucoma were fol-lowed with serial axial length measurements. The axialgrowth patterns were compared with the normal growthcurve.RESULTS: Eight (66.7%) of 12 eyes had initial axiallengths longer than the normal range, and 4 eyes(33.3%) were within the normal range. After surgicalintervention with good intraocular pressure control in 10eyes, 3 eyes showed a decrease of axial length and 7 eyesshowed no decrease of axial length, but the axial growthpattern paralleled the normal growth curve. After surgi-cal intervention with poor intraocular pressure control, 2

Accepted for publication Jul 18, 2001.From the Glaucoma Division, Jules Stein Eye Institute, University of

California at Los Angeles, School of Medicine, Los Angeles, California.Inquiries to Simon K. Law, MD, Glaucoma Division, Jules Stein Eye

Institute/University of California, 100 Stein Plaza, Los Angeles, CA90095-7000; fax: (310) 206-7773.

AMERICAN JOURNAL OF OPHTHALMOLOGY926 DECEMBER 2001