anterior lacrimal sac, and then the skin incision was closedwith running 7-0 nylon suture.

Transnasal endoscopic examination was performed fol-lowing the DCR procedure. A total of 1 ml of 2% lidocaineand 3 ml of oxymethazoline chloride were sprayed into thenasal cavity using a Jackson-type spray apparatus. Atransnasal endoscope (Machida Co Ltd., Tokyo, Japan)with viewing directions of 0 degrees and 30 degrees wasused. The viewing angle was 110 degrees. A chargedcoupled display camera and the control unit (ELMO Co.Ltd., Tokyo, Japan) were attached to the endoscope. Afterthe E-DCR procedure, massive discharge that was firmly

adherent to the intubated silicone tube was often observed.Recently, Kurihashi5 reported that endoscopic cleaning inthe nasal cavity is very important to increase the successrate of E-DCR. When the aspiration procedure is noteffective, a forceps with an injector tip is useful to wash outand remove any firmly adhered massive discharge. Figure 2(bottom) shows an ostium that seems to be good epithe-lization under TNE examination 1 year postoperatively.All consecutive 28 cases have been successfully observedfor more than 6 months after E-DCR procedure, as shownin Figure 2 (bottom).

In conclusion, these two forceps proved to be usefulduring and after the E-DCR procedure. The success rate ofthe E-DCR procedure may increase with the introductionof these forceps.

REFERENCES

1. Welham RAN, Wulc AE. Management of unsuccessful lacri-mal surgery. Br J Ophthalmol 1987;71:152–157.

2. Tarbet KJ, Custer PL. External dacryocystorhinostomy. Sur-gical success, patient satisfaction, and economic cost. Oph-thalmology 1995;102:1065–1070.

3. Allen KM, Berlin AJ, Levine HL. Intranasal endoscopicanalysis of dacryocystorhinostomy failure. Ophthal Plast Re-constr Surg 1988;4:143–145.

4. Kurihashi K. A new bicanalicular intubation method. Orbit1994;13:11–15.

5. Kurihashi K. Dacryology. Tokyo, Japan: Medical Aoi; 1998:77–79.

Spontaneous RetrobulbarHemorrhage in Type IVEhlers–Danlos SyndromeSaad Shaikh, MD, Maria Braun, MD, andJoseph Eliason, MD

PURPOSE: To describe a case of spontaneous retrobulbarhemorrhage in type IV Ehlers–Danlos syndrome, a dis-order characterized by vascular fragility and associatedsevere bleeding problems.METHODS: Observational case report. Spontaneous uni-lateral retrobulbar hemorrhage in a 22-year-old man withtype IV Ehlers–Danlos syndrome was documented byclinical and radiographic examination.RESULTS: The retrobulbar hemorrhage was self-limitedand resolved without visual sequelae.

Accepted for publication Oct 1, 2001.From the Associated Retinal Consultants and William Beaumont

Hospital, Royal Oak, Michigan (S.S.), the Department of Ophthalmol-ogy, Stanford University Medical Center, Stanford, California (M.B.),and the Division of Ophthalmology, Santa Clara Valley Medical Center,Santa Clara, California (J.E.).

Saad Shaikh, MD is a Heed Ophthalmic Foundation Fellow.Inquiries to Saad Shaikh, Associated Retinal Consultants, William

Beaumont Hospital, 3535 W. 13 Mile Rd #632, Royal Oak, MI 48073;fax: (413) 826-4630; e-mail: saads@earthlink.net

FIGURE 2. (Top) The posterior nasal flap (darkened star)grasped by E-DCR forceps (left side) is sutured during opera-tion. The posterior flap of the lacrimal sac (empty star).(Bottom) An ostium (arrow) in the right nasal cavity observedunder transnasal endoscopy 1 year postoperatively. The nasalseptum (darkened star) and the medial nasal concha (asterisk).

AMERICAN JOURNAL OF OPHTHALMOLOGY422 MARCH 2002

CONCLUSION: Spontaneous retrobulbar hemorrhageshould be recognized as part of the spectrum of type IVEhlers–Danlos syndrome, and clinicians must be alert todiagnosis and treatment of this vision-threatening prob-lem. (Am J Ophthalmol 2002;133:422–424. © 2002by Elsevier Science Inc. All rights reserved.)

SPONTANEOUS RETROBULBAR HEMORRHAGES ARE IN-

frequent phenomena, and reported origins include in-traorbital vascular malformations, systemic thrombolytictherapy, as well as other rare origins.1,2 We report a case ofspontaneous retrobulbar hemorrhage secondary to systemicvascular fragility from Ehlers–Danlos syndrome type IV.

● CASE: A 22-year-old man with type IV Ehlers–Danlossyndrome previously confirmed by tissue biopsy was re-ferred to our clinic on an emergent basis for evaluation ofa retrobulbar hemorrhage. The mother and grandfather ofthe patient had previously passed away from vascularcomplications of type IV Ehlers–Danlos syndrome. Themedical history and review of systems of the patient wassignificant for easy bruising and spontaneous bleedingduring childhood and spontaneous rupture of an upperextremity vessel sustained previously while lifting weights.

While bending over to pick up a young child, thepatient noted sudden onset of sharp pain in his left orbitand blurred vision. This was followed by subconjunctivaland eyelid hemorrhage. The patient presented to theemergency room at his local community hospital. Axialand coronal computed tomographic scans revealed prop-tosis of the left globe with retrobulbar hemorrhage (Figure1). The emergency room physician was unable to find anophthalmologist who felt comfortable evaluating the pa-

tient given his systemic bleeding diathesis. During thistime, the patient reported that his left eye had begun toprotrude and he noted more bleeding in his eyelid. Twen-ty-four hours later, the patient was transferred to ourhospital well over 200 miles away.

On presentation, visual acuity of the patient was 20/20in each eye. Pupillary examination was normal withoutafferent pupillary defect, and tonometry was normal toapplanation. External examination revealed 2.5-mm prop-tosis of the left globe by Hertel measurements associatedwith edema and ecchymosis of the upper and lower eyelids(Figure 2). Slit-lamp biomicroscopy of the left eye revealedmoderate subconjunctival hemorrhage inferotemporallywith superficial punctate keratopathy consistent with cor-neal exposure secondary to poor eyelid closure. Externalexamination of the right eye revealed normal findings. Theanterior segment and dilated funduscopic examination

FIGURE 1. (Left) Axial computed tomography (CT) reveals proptosis of the left globe (arrow) as compared with the right. (Right)Coronal section demonstrates hemorrhage in the retrobulbar fat of the left orbit (arrow).

FIGURE 2. External examination reveals eyelid and subcon-junctival hemorrhage (arrows) of the left side.

BRIEF REPORTSVOL. 133, NO. 3 423

were otherwise normal bilaterally without evidence ofoptic nerve edema or hemorrhage. Both globes were soft toretropulsion, and neither retinal arterial nor venous pulsa-tions could be induced by mild pressure on either globe.Extraocular motion was normal without diplopia. Thepatient was reexamined on multiple occasions over thenext 24 hours without change in the clinical findings, andhe was discharged.

Ehlers–Danlos syndrome is a group of nine distinctheritable connective tissue disorders, classified on the basisof clinical, molecular, and genetic differences, that mani-fest in a spectrum of clinical disease marked by skin andvascular fragility, unsightly bruising and scarring, muscu-loskeletal discomfort, and arthritis.3 Type VI, also knownas ocular type or oculus fragilis, is usually associated withprimary ocular pathologic features such as lens subluxa-tion, angioid streaks, thin sclera, and high myopia.3 TypeIV is characterized by vascular fragility from abnormalitiesin type III collagen that lead to dissection, aneurysmalchanges, and rupture of medium-to-large arteries, whichmay have severe and even lethal consequences. Ocularpathologic features in patients with type IV are usuallysecondary to the development of a carotid cavernousfistula4 from the underlying vascular diathesis, but retrob-ulbar hemorrhage has not been previously identified in thisdisorder. Spontaneous retrobulbar hemorrhage has previ-ously been described as a complication of vascular fragilityin scurvy patients with defective collagen formation invessel basement membranes.5 A similar mechanism giventhe abnormalities with collagen synthesis inherent inEhlers–Danlos syndrome can be speculated to have oc-curred in our patient as well.

Although spontaneous retrobulbar hemorrhages arerarely life threatening, they can have severe visual se-quelae. Indications for intervention resulting from a tensedorbit include visual loss or an afferent pupillary defectusually associated with poor retropulsion, decreased ex-traocular motion, and increased intraocular pressure.Treatment includes administration of oral hyperosmoticagents and topical intraocular pressure lowering agents,followed when necessary by a lateral cantholysis with anincision directed laterally, superiorly, or inferiorly into thecrux of the lateral canthal tendon. Rarely after suchmeasures is an emergent orbital decompression necessary.Fortunately, our patient suffered no permanent complica-tions as a result of his hemorrhage, but general practitio-ners and ophthalmologists should be alert to isolatedretrobulbar hemorrhage as a consequence of Ehlers–Danlossyndrome.

REFERENCES

1. Moin M, Kersten RC, Bernardini F, et al. Spontaneoushemorrhage in an intraorbital arteriovenous malformation.Ophthalmology 2000;107:2215–2219.

2. Chorich LJ, Derick RJ, Chambers RB, et al. Hemorrhagic

ocular complications associated with the use of systemicthrombolytic agents. Ophthalmology 1998;105:428–431.

3. Pollack JS, Custer PL, Hart WM, Smith ME, Fitzpatrick MM.Ocular complications in Ehlers-Danlos syndrome type IV.Arch Ophthalmol 1997;115:416–419.

4. Schievink WI, Piepgras DG, Earnest IVF, Gordon H. Spon-taneous carotid-cavernous fistulae in Ehlers-Danlos syndrometype IV. J Neurosurg 1991;74:991–998.

5. Sloan B, Kulwin DR, Kersten RC. Scurvy causing bilateralorbital hemorrhage. Arch Ophthalmol 1999;117:842–843.

Lymphomatous Meningitis of theBurkitt Type Presenting With MultipleCranial NeuropathiesMichael A. Grassi, MD, and Andrew G. Lee, MD

PURPOSE: To describe diplopia as the initial manifestationof the Burkitt lymphoma.DESIGN: Observational case report.METHODS: Retrospective chart review.RESULTS: A 53-year-old human immunodeficiency virus(HIV)-positive man presented with a severe headacheassociated with binocular diplopia. Flow cytometric anal-ysis of the cerebrospinal fluid demonstrated a monoclonalB-lymphoid cell population consistent with the Burkittlymphoma.CONCLUSION: Ophthalmologists should be aware thatdiplopia may be the presenting manifestation of Burkittlymphoma in an immunocompromised patient. (Am JOphthalmol 2002;133:424–425. © 2002 by ElsevierScience Inc. All rights reserved.)

IN 1958, BURKITT DESCRIBED A MANDIBULAR MALIG-

nancy in African children that later proved to be anoncleaved B-cell lymphoma.1 In endemic areas, thedisease is associated with exposure to the Epstein–Barrvirus and a translocation t(8:14) mutation resulting in thederegulation of the oncogene, c-myc.1

The increasing frequency of the AIDS, immunosuppres-sion therapy, and organ transplantation has led to anincreased incidence of the nonendemic Burkitt lympho-ma.2 The postulated mechanism is secondary to defectiveregulation of Epstein–Barr virus–infected B-cells leading touninhibited differentiation.3 Extranodal involvement isthe most common presentation with ultimate centralnervous system spread in greater than 50% of cases.4

Accepted for publication Oct 16, 2001.From the Department of Ophthalmology (M.A.G., A.G.L.), the

Department of Neurology (A.G.L.), and the Department of Neurosurgery(A.G.L.), the University of Iowa Hospitals and Clinics, Iowa City, Iowa.

Supported in part by an unrestricted grant from Research to PreventBlindness, Inc., New York.

Inquiries to Andrew G. Lee, MD, Department of Ophthalmology,University of Iowa Hospitals and Clinics, 200 Hawkins Dr PFP, IowaCity, Iowa 52246; fax: (319) 353-7996; e-mail: Andrew-lee@uiowa.edu

AMERICAN JOURNAL OF OPHTHALMOLOGY424 MARCH 2002