Esferofauia Aislada y Glaucoma

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Hindawi Publishing CorporationCase Reports in MedicineVolume , Article ID ,pageshttp://dx.doi.org/.//

Case ReportIsolated Spherophakia and Glaucoma

Joseph Pikkel1,2 and Epstein Irena1

Ziv Medical Center, Safed, Israel Faculty of Medicine, Bar Ilan University, Safed, Israel

Correspondence should be addressed to Joseph Pikkel; [email protected]

Received March ; Revised June ; Accepted July

Academic Editor: Marco A. Zarbin

Copyright J. Pikkel and E. Irena. Tis is an openaccess article distributed under the Creative Commons AttributionLicense,which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

We report a case o spherophakia that caused glaucoma anddescribe the characteristics andthe ultrasound biomicroscopy ndingsas well as the mechanism and management o glaucoma in spherophakia. We suggest considering lens extraction to manageglaucoma in spherophakia and discuss the surgical considerations and possible complications o such an intervention.

1. Introduction

Spherophakia is a rare condition in which the crystalline

lens assumes a spherical shape with an increased anterior-posterior diameter and a reduced equatorial diameter. Sphe-rophakia can occur as an isolated anomaly or associated witha systemic disorder such as the Weill-Marchesani syndrome,Marans disorder, mandibuloacial dysostosis, Alports syn-drome, and Klineelters syndrome[].

We report a case o spherophakia that caused glaucoma.We describe the characteristics and the ultrasound biomi-croscopy ndings as well as the mechanism and managemento glaucoma in spherophakia.

Te role o lens extraction in the management o glau-coma in spherophakia has not been established; we discussthe surgical considerations and possible complications osuch an intervention.

2. Patient Description

A -year old man was reerred to our outpatient clinicsuspected o suffering rom uveitis in his lef eye. He initiallypresented years ago with acute angle-closure glaucoma inhis right eye that was treated with lowering the intra ocularpressure by local beta blockers and local prostaglandinsollowed by neodymium: YAG laser peripheral iridotomiesin both eyes. Intraocular pressure values were mmHg inthe right eye and mmHg in the lef eye on admission andlowered to mmHg in the right eye and mmHg in his

lef eye beore perorming the iridotomies. Afer that, theIOP was within normal limits or years, and there is nodocumentation o recurrent iritis in either eye in this periodo time.

Te patient had chronic renal ailure, cardiac arrhythmia,and arterial hypertension.

On examination at reerral, best-corrected visual acuitywas / in his right eye and / in his lef eye. Reractionwas . spherical in the right eye and . spherical in thelef eye. Te intraocular pressure was mmHg in his right eyeand mmHg in his lef eye. He had bilateral neodymium:YAG laser peripheral iridectomies. Both eyes had a shallowanterior chamber. In the lef eye, the lens was located slightlyorward causing a pupillary block, and the anterior chamberangle was very narrow on indentation gonioscopy (Figure ).C/D ratio was . in the righteye and . in the lef eye. Retina

was normal in both eyes. Full-threshold visual elds werenormal in both eyes. Te sagittal lens diameter was . mmin the right eye and . mm in the lef eye (the mean sagittallenticular diameter in a young adults eye is . mm . SDand in spherophakia . to . mm) [].

Ultrasonographic A-scan biometry recorded axial lengtho .mm in the right eye and . mm in the lef eye.Ultrasound biomicroscopy with a mHz probe showed aspherophakia lens in both eyes and pupillary block in thelef eye due to orward subluxation o the lens (Figure ). Onthe basis o these ndings, a diagnosis o spherophakia inboth eyes was made. In light o the diagnosis o spherophakiathe patients medical history and examination were reviewed.
http://dx.doi.org/10.1155/2013/516490http://dx.doi.org/10.1155/2013/516490


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F : Photo slit camera image o the anterior segment o the lefeye. Te anterior surace o the lens (L) is in touch with the pos-terior surace o the iris (I), causing pupillary block thus causingintraocular pressure raise.

Te patient had no history o cardiovascular diseases or

skeletal problems and had high intellect. Te patients heightwas cm with normal skeletal proportions. Tere wereno eatures o Marans syndrome, the Weill-Marchesanisyndrome, or homocystinuria.

Chronic pupil block was believed to be responsible ortheuncontrolled glaucoma in the lef eye. At this stage, we per-ormed lens extraction in the lef eye by phacoemulsication,and we implanted a oldable -piece acrylic intraocular lens.Te postoperative course was routine. Te intraocular pres-sure was controlled with no antiglaucoma drugs, the anteriorchamber depth was normal, and the nal uncorrected visualacuity was /.

Te patient had the same surgical procedure in his right

eye with an excellent outcome.

3. Comment

Isolated spherophakia, with no association with systemicdiseases, is a rare condition [,]. Te triad o angle-closureglaucoma, shallow anteriorchamber, and myopia should alertthe clinician to the possible diagnosis o spherophakia [].Myopia is usually high and develops in the second decade[]. Myopia is mainly lenticular in origin, resulting rom theincreased lenticular curvature and orward placement o thelens. Axial myopia may also occur; however, axial lengths

are usually normal []. In our patient, spherophakia occurredas an isolated condition in eyes with moderate myopia andnormal axial lengths.

Previous ultrasonography studies in spherophakia reporteatures similar to our ndings in this case []. Marchesanisuggested that the mechanism o spherophakia is hyperplasiao the ciliary body, resulting in maximum accommodationand lenticular myopia. Te hypoplastic ciliary body oundwith ultrasound was unexpected when rst described, andthereore, it is thought nowthat the etal lens in spherophakia,which is physiologically spherical naturally, has never beensubjected to the orce o properly acting ciliary body andzonular bers [, ]. In our patient, there was evidence

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C L

(a)

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C L

(b)

F : Ultrasound biomicroscopy image o the right eye (a) andthe lef eye (b) showing the orward luxation o the lens (L) and itstouch with the iris (I), thus orming pupillary block, pushing theiris towards the cornea (C), andcausing a shallow anterior chamber.Notice the increased anteroposterior diameter o the lens (L).

F : Ultrasound biomicroscopy image o lef eye showinghyperplasia o ciliary body.

o hyperplasia o the ciliary body on UBM examination(Figure ).

Glaucoma is mainly reported in the literature whenspherophakia is associated with the Weill-Marchesani syn-drome []. Glaucoma in isolated andamilial spherophakiais less common []. Angle-closure glaucoma occurs inspherophakia rom a pupillary block mechanism caused bydislocation o the lens and its orward movement, sometimebeyond the pupil into the anterior chamber, depending onzonular bers integrity[].


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When the zonular bers are intact, the lens moves or-ward and the anterior surace o the lens comes into contactwith the posterior surace o the iris and creates pupillaryblock. Te zonular bers are typically long in the Weill-Marchesani syndrome, and loosening o the zonular bersallows the lens to move orward, producing lens-iris contact

[].Chronic intraocular pressure elevation in spherophakia

can occur by a variety o mechanisms. Unrelieved pupil blockcan lead to peripheral anterior synechies and irreversibletrabecular damage. Chronic pupillary block and posteriorsynechies can occur as well as crowding o the trabeculae[].

Pupillary block is exacerbated with miotics and relievedby mydriatics. Cycloplegic agents relax the ciliary muscle,tighten zonular bers support, and cause posterior lens move-ment[].

Peripheral iridectomy has been suggested as a mean torelieve pupil block; however, the rate o surgical complica-tions is high. Vitreous loss occurs requently as the vitreousace is unprotected by the lens periphery andzonules. Periph-eral iridectomies are hardly done any more, and the usualpreerred way o treatment is to perorm iridotomies byNd:YAG laser. An Nd:YAG laser peripheral iridotomy is asaer initial procedure and i unsuccessul can be ollowed by

a surgical peripheral iridectomy [].

In ourpatient papillary block occurred though the patienthad prior yag laser iridotomies most probably due to the iri-dotomies being not potent. Some o the iridotomies were notperipheral and might be blocked by the anterior movemento the lens.

Te role o lens extraction in the management o sphero-phakia glaucoma has not been established. Surgical removalo the lens may be required to control glaucoma; however,there is a high risk o complications, especially vitreous loss

[]. In spherophakia, the combination o small capsular bagwith a relatively high equatorial diameter and zonular bersinstability predisposes to intraoperative and postoperativecomplications. Shallow anterior chamber, peripheral ante-rior synechias, posterior synechias, and elevated intraocularpressure may cause surgical difficulties too. In our patient, aregular cataract extraction was done by phacoemulsicationthrough a . mm opening, and an aspheric oldable posteriorchamber intraocular lens ( mm optic diameter and mmhepatic diameter) was inserted with no difficulty or compli-cations.

In our case, lens extraction was benecial or the patientwho has now normal intraocular pressure without any needor urther treatment. Te intra- and postoperative courseswere uneventul.

Tis case demonstrates the presentation and pathogenesiso glaucoma in spherophakia and raises several issues aboutthe management o glaucoma in spherophakia. Toughlensectomy in spherophakia can be surgically and postop-eratively challenging, we suggest considering it as a possibletreatment in these cases.

References

[] P. L. Macken, C. J. Pavlin, R. uli, and G. E. rope, Ultrasoundbiomicroscopic eatures o spherophakia,Australian and NewZealand Journal of Ophthalmology, vol. , no. , pp. ,.

[] L. B. Nelson and I. H. Maumenee, Ectopia lentis, Survey of

Ophthalmology, vol. , no. , pp. , .[] C. E. Willoughby and P. K. Wishart, Lensectomy in the man-

agement o glaucoma in spherophakia,Journal of Cataract andRefractive Surgery, vol. , no. , pp. , .

[] M. Willi, L. Kut, and E. Cotlier, Pupillary-block glaucoma inthe Marchesani syndrome,Archives of Ophthalmology, vol. ,no. , pp. , .

[] R. Ritch and M. Wand, reatment o the Weill-Marchesanisyndrome,Annalsof Ophthalmology, vol. ,no. , pp. ,.

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Esferofauia Aislada y Glaucoma