CLINICOPATHOLOGIC REPORTS, CASE REPORTS, AND SMALL CASE SERIES SECTION EDITOR: W. RICHARD GREEN, MD Capsulorrhexis in Capsular Delamination Separation of the anterior layer of the lens capsule, also known as true ex- foliation and capsular delamina- tion, has become an increasingly rare clinical finding. Although its patho- genesis is not precisely known, the condition has been associated with age, trauma, and exposure to tox- ins and/or to thermal radiation.1 An association with occupational infra- red radiation exposure was ac- cepted after Elschnig2 described the classic clinical findings in 2 glass- blowers and Kubik3 and others4,5 noted the condition in black- smiths, puddlers, chainmakers, and steelworkers. With improved safety Figure 1. Preoperative appearance of a diaphanous membrane (arrows) in the anterior chamber that is standards, the condition is now re- attached to the lens. ported less frequently in associa- tion with occupational hazards. Cap- pigmentation of the angle, and no sular delamination remains of phacodonesis. Results of tonom- interest particularly because there etry, fundus, and optic nerve exami- may be mild subclinical forms of the nations were all normal. condition,6 and because modern The patient underwent bilat- cataract surgery is dependent on suc- eral cataract extraction with a 2-month cessful anterior capsule removal. interval between procedures. The phacoemulsification technique was Report of a Case. An 81-year-old man standard except for a larger than usual who under treatment for a cardiac ar- capsulorrhexis and the submission of rhythmia and hypertension reported the capsule specimens for histopatho- blur and glare in both eyes. There was logic study. The diaphanous mem- a family history of cataracts and glau- brane was gently teased to the side and coma. His career involved more than the deeper capsular layer was dis- 20 years in a steel mill. During 4 of sected with a bent 30-gauge needle. these years, he experienced frequent There were no complications in either and intense prolonged exposure to operation. Two years postoperatively, the heat of the blast furnaces. the uncorrected vision was 20/25 OU On ophthalmic examination, his with a mild astigmatism with the rule best-corrected visual acuity was 20/70 notedonrefraction.Theposteriorcap- OU. The patient had hyperopia of 4 sules remained clear and the anteri- Figure 2. Electron microscopy ultrastructural diopters. Bilateral cataracts were pres- or capsular edges appeared normal. appearance of a 3-µm-thick layer (asterisk) that is split from the anterior lens capsule. Note ent, having combined cortical and Findings from the histopatho- surface-parallel vacuolization of the anterior nuclear elements. Within the cen- logic examination of the specimens capsule extending beyond the split (original tral anterior chamber in both eyes was revealed delamination of the lens magnification ϫ12000). a folded cellophane-like membrane capsule that was best illustrated by fixed to the anterior lens capsule’s transmission electron microscopy Comment. Multiple reports exist of surface, unassociated with any evi- (Figure 2). The capsule was mod- capsulardelaminationspecimensfrom dence of inflammation (Figure 1). erately electron dense with a lami- successful intracapsular and extracap- The degree of delamination was ap- nated granular appearance. The sular cataract surgery.4,7,8 In this case, proximately symmetrical. There were splitting of the capsule was docu- the curvilinear capsulorrhexis tech- no other abnormal deposits on the mented with the anterior layer thin- nique was successful. With the excep- lens capsule or the iris, no unusual ner than the posterior layer. tion of the manipulation required to (REPRINTED) ARCH OPHTHALMOL / VOL 120, NOV 2002 WWW.ARCHOPHTHALMOL.COM 1581 ©2002 American Medical Association. All rights reserved. Downloaded From: https://jamanetwork.com/ on 09/28/2021 take the surgical specimen, the cases has also been suggested that capsu- most commonly in neonates, and were routine and without complica- lar protein abnormalities may play a they are frequently located in the sa- tion. To our knowledge, no series to role.10 In this case, however, a volun- crococcygeal and presacral areas.1 Al- date has reported a rate of complica- teered history of prolonged expo- though intracranial teratomas are tion in cataract extraction with cap- sure to the heat of a blast furnace pro- rare, they are the most common brain sular delamination, but the true inci- vided the most likely etiologic factor tumors of the neonatal period,2 and denceofcomplicationsassociatedwith related to both the cataract and de- most frequently involve the pineal re- this finding will be difficult to estab- lamination of the anterior capsule. gion, suprasellar region, hypothal- lish because of its rarity. Clinically, diaphanous transparent mus, cerebellar vermis, and ven- While capsular delamination is membranes were similar to those re- tricles.1-3 Teratomas of the optic nerve rare, mild and subclinical forms of the ported as glassblower’s cataracts. For- are exceedingly rare, with few re- condition may be more prevalent than tunately, occupational safety stan- ports in the English literature.4 currently recognized. In a series of 10 dards and protective engineering have We report a neonate with an cases, Wollensak and Wollensak6 re- made true exfoliation from infrared optic nerve teratoma, an odonto- ported the appearance of a double exposure rare. genic orbital dermoid cyst, and per- contour visible at the capsulorrhexis James S. Kelley, MD sistent fetal vasculature (PFV). We edge. Pathologic analysis of the cap- Tony Tsai, MD are not aware of any previous case sulorrhexis specimens by light and Mary B. Kansora, MD in which these entities have coex- electron microscopy revealed the W. Richard Green, MD isted, and, to our knowledge, this is double contour to result from a char- Baltimore, Md the first report in which any 2 of the acteristic step formation at the cap- 3 have been documented as occur- sulorrhexis edge. In 7 of 10 of these The authors have no financial inter- ring in the same patient. cases, these authors also noted surface- est in this article. parallel splits in the outer third of the Corresponding author: James S. Report of a Case. A healthy 2-day- capsule. They postulated that the Kelley, MD, 6565 N Charles St, Suite old white boy was referred for man- double contour and microscopically 302, Baltimore, MD 21204 (e-mail: agement of PFV of the right eye. The evident surface-parallel splits may rep- [email protected]). patient was carried to term and had resent a subclinical form of true ex- no family history of any unusual eye 1. Duke-Elder S. System of Ophthalmology: Dis- foliation that results from zonular trac- eases of the Lens and Vitreous; Glaucoma and Hy- disease. On examination under an- tion on the superficial capsule over less potony. St Louis, Mo: CV Mosby; 1969. esthesia, the patient appeared enoph- elastic deeper layers in older pa- 2. Elschnig A. Abhlo¨sung der Zonulalamelle bei thalmic on the right side, and had right Glasblasern. Klin Monatsbl Augenheilkd. 1922; tients. These findings suggest that true 69:732-734. microcornea (corneal diameters: 6.0 exfoliation may represent one ex- 3. Kubik J. Ablo¨sung der zonulalamelle bei Glasbla- mm OD, 10.0 mm OS). Intraocular treme of a continuum representing sern. Klin Monatsbl Augenheilkd. 1923;70. pressures were 5 mm Hg OD and 10 4. Burde RM, Bresnick G, Uhrhammer J. True different degrees of capsular delami- exfoliation of the lens capsule: an electron mm Hg OS. Biomicroscopy of the right nation. Although Wollensak and Wol- microscopic study. Arch Ophthalmol. 1969;82: eye (Figure 1) revealed patches of 651-653. lensak reported anecdotally that the 5. Holloway TB, Cowan A. Concerning lamellar band keratopathy at the 4-o’clock and incidence of radial capsular tears ap- membranes of the anterior surface of the lens. 8-o’clock positions, with peripheral peared lower when a double contour Am J Ophthalmol. 1931;14:189-195. corneal neovascularization extend- 6. Wollensak G, Wollensak J. Double contour of was seen, no evidence currently ex- the lens capsule edges after continuous curvi- ing 360°. Dense fibrotic membranes ists regarding the relative strength or linear capsulorhexis. Graefes Arch Clin Exp Oph- extended from the pupillary margin weakness of the capsulorrhexis with thalmol. 1997;235:204-207. and anterior iris stroma into the an- 7. Fukuo Y, Takeda N, Hirata H et al Histologi- the double contour. Likely, the find- cal findings of capsular delamination of the lens. terior chamber angle. A yellow mush- ing goes unnoticed in most cases. No Jpn J Ophthalmol. 1994;38:87-91. room-shaped mass protruded through 8. Kuchle M, Iliff WJ, Green WR. Kombinierte double contour was observed after Feuerlamelle und Pseudoexfoliation der vor- the pupil. B-scan ultrasonography re- curvilinear capsulorrhexis in our case. deren Linsenkapsel. Klin Monatsbl Augen- vealed a funnel-shaped stalk that ex- Perhaps this is because the delami- heilkd. 1996;208:127-129. tended from an area surrounding the 9. Cashwell LF Jr, Holleman IL, Weaver RG, van nation did not extend to the capsu- Rens GH. Idiopathic true exfoliation of the lens optic nerve to the retrolenticular re- lorrhexis edge, although evidence for capsule. Ophthalmology. 1989;96:348-351. gion, as is consistent with PFV. Axial shearing of the capsular layers be- 10. Anderson IL, van Bockxmeer FM. True exfolia- lengths were 15.8 mm OD and 22.9 tion of the lens capsule: a clinicopathological re- yond the edge of true exfoliation is port. Aust N Z J Ophthalmol. 1985;13:343-347. mm OS. Examination results from the suggested by the vacuolization of the left eye were normal. capsule seen ultrastructurally be- At age 1 month, the patient un- yond the split (Figure 2). Optic Nerve Teratoma and derwent attempted anterior segment Although associations with Odontogenic Dermoid Cyst reconstruction, but the dense fi- trauma, toxins, inflammation, and in a Neonate With brotic membranes precluded lensec- heat are well recognized, the under- Persistent Fetal Vasculature tomy.