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   Table of Contents      
Year : 2011  |  Volume : 59  |  Issue : 4  |  Page : 319-322

Double anterior chamber in a patient with glaucoma and microspherophakia

1 Khatam-Al-Anbia Eye Hospital, Mashhad University of Medical Sciences, Iran
2 Department of Ophthalmology and Visual Sciences, University of Utah Health Sciences Center, Salt Lake City, UT, USA

Date of Submission29-May-2009
Date of Acceptance20-Mar-2010
Date of Web Publication11-Jun-2011

Correspondence Address:
Majid Moshirfar
John A. Moran Eye Center, 6360 South 3000 East, Suite 200, Salt Lake City, UT 84121
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0301-4738.82006

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We report the case of a 16-year-old woman with microspherophakia and secondary open angle glaucoma. The patient presented with a membrane dividing the anterior chamber into two segments without edema or Descemet's membrane detachment. Slit lamp biomicroscopy, Pentacam, and specular microscopy images were obtained. Double anterior chamber is primarily found in patients with anterior chamber anomalies when there is no history of surgery or trauma.

Keywords: Descemet′s membrane detachment, glaucoma, microspherophakia

How to cite this article:
Khakshoor H, Ansari-Astaneh MR, Shoeib M, Schliesser JA, Moshirfar M. Double anterior chamber in a patient with glaucoma and microspherophakia. Indian J Ophthalmol 2011;59:319-22

How to cite this URL:
Khakshoor H, Ansari-Astaneh MR, Shoeib M, Schliesser JA, Moshirfar M. Double anterior chamber in a patient with glaucoma and microspherophakia. Indian J Ophthalmol [serial online] 2011 [cited 2020 Aug 11];59:319-22. Available from: http://www.ijo.in/text.asp?2011/59/4/319/82006

Microspherophakia can be an isolated finding or associated with other systemic and ocular disorders. [1] Double anterior chamber has been reported as a complication of deep anterior lamellar keratoplasty. [2] However, to our knowledge, this is the first reported case of primary double anterior chamber without Descemet's membrane detachment in a patient with microspherophakia.

  Case Report Top

The patient was a 16-year-old female who presented to Khatam eye center, complaining of decreased vision bilaterally. The best spectacle corrected visual acuity was 20/200 and the refraction was -28.00 diopters sphere in both the eyes. Intraocular pressure (IOP) measurements were 20 mm Hg in the right eye and 35 mm Hg in the left eye. Slit lamp examination demonstrated microspherophakia with dislocated lenses inferonasally with phakodonesis bilaterally. In the left eye, the anterior chamber was divided by a clear membrane which was attached to the cornea superiorly and inferiorly without any visible communications [Figure 1] and [Figure 2]. Dilated fundus examination revealed near complete cupping of both optic nerves [Figure 3] and [Figure 4]. Central corneal thickness was 470 μm in the right eye and 555 μm in the left eye. Oculus-Pentacam measured anterior chamber depth of 3.20 mm in the right eye and 2.32 mm in the left eye with anterior distance to membrane of 1.64 mm. Lens thickness was 4.81 and 5.54 mm in the right and left eyes, respectively. Anterior chamber angle was 45° in the right eye and 28°-35° in the left eye (by Oculus-Pentacam). This measurement was inaccurate in the left eye due to the pseudo angle (between cornea and the membrane). Gonioscopy in the right eye showed an open iridocorneal angle [Figure 5]. Gonioscopy of the left eye was inconclusive due to the membrane's position [Figure 6] and [Figure 7]. Chamber volume was 309 mm 3 in the right eye and 240 mm 3 in the left eye. Endothelial cell count was 2336 cells/mm 2 in the right eye and 1036 cells/mm 2 in the left eye with endothelial cells on the posterior cornea of the left eye. Although clinical findings were suggestive of Descemet's membrane detachment, there was no corneal edema identified in the left eye [Figure 1]. The patient underwent trabeculectomy because of poorly controlled IOP in her left eye, 4 months later. Good IOP control was obtained after surgery and has been maintained for the past 4 years. During follow-up, no cataract formation was observed.
Figure 1: Slit lamp photograph from the left eye

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Figure 2: Photograph of anterior chamber of both the eyes with Pentacam. Left eye anterior chamber is divided by a clear membrane into anterior and posterior parts, and it was attached to cornea superiorly and inferiorly

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Figure 3: Fundus photograph of the right eye showing near complete cupping of the optic nerve

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Figure 4: Fundus photograph of the left eye showing near complete cupping of the optic nerve

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Figure 5: Gonioscopic photograph of the right eye showing an open iridocorneal angle

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Figure 6: Gonioscopic photograph of the left eye

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Figure 7: Gonioscopic photograph of the left eye showing part of the membrane

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Three years later, the patient's sister presented to Khatam Eye Center with a similar clinical appearance. The sister's visual acuity in the left eye was no light perception (NLP) and refraction was -19.00 diopters sphere in both eyes. Other clinical findings were bilateral microspherophakia, phakodonesis, and increased IOP, without double anterior chamber. The similar findings in two patients were suggestive of a genetic background to their disease.

  Discussion Top

Microspherophakia is a rare abnormality that can be isolated or associated with other ocular or systemic disorders such as  Weill-Marchesani syndrome More Details, Marfan syndrome, homocystinemia, Alport syndrome or Kleinfelter syndrome. Glaucoma is rarely seen in the isolated form of microspherophakia. [1] The mechanism of glaucoma in microspherophakia is pupillary block or irritation of ciliary body by ectopic lens. [3]

Chronic angle closure from anteriorly dislocated microspherophakic lenses can cause irreversible changes to the trabecular meshwork, resulting in decreased aqueous outflow. [1] Management of glaucoma in microspherophakia is not clear. [4] Some surgeons suggest lensectomy for angle closure glaucoma and trabeculectomy for open angle glaucoma in microspherophakia patients. In our patient, trabeculectomy has resulted in a well-controlled IOP for the past 4 years. Medication management sufficed for controlling IOP in the right eye as well as for the patient's sister.

Although there were no systemic features such as short stature or joint abnormalities in the two patients discussed in this case report, both exhibited ocular findings similar to Weill-Marchesani syndrome. Ocular findings in this syndrome are microspherophakia, ectopic lens, high myopia and glaucoma. [5] On the other hand, these ocular abnormalities coupled with the absence of systemic disorders may suggest one of the many conditions that comprise the wide spectrum of anterior segment dysgenesis. These disorders are the result of impaired migration or development of mesenchymal cells that play a critical role in the formation of the cornea, iris and trabecular meshwork. [6] One of the earliest events in embryogenesis is determination of lens development. Since the lens plays an important role in anterior segment development, these abnormalities may be present with any primary disorder of the lens. [7]

Descemet's membrane detachment may be encountered as a complication of any intraocular surgery. Management of Descemet's membrane detachment depends on the extent and location of the detachment. [8] Clinical findings include corneal edema overlying the area of Descemet's membrane detachment along with a visible retrocorneal membrane. Although our patients presented with clinical features consistent with this condition, the presence of endothelial cells on the posterior cornea and the absence of corneal edema argues for an alternative explanation [Figure 8].
Figure 8: Evaluation of endothelial cells in center of cornea in both eyes with specular microscope. In the left eye there is an abnormality in the shape and density of endothelial cells

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Double or pseudo-anterior chamber (a space between the corneal donor and Descemet's membrane) is one of the most dramatic complications of deep anterior lamellar keratoplasty. [9] We present a case of unilateral primary double anterior chamber in a patient with microspherophakia without a history of surgery or ocular trauma. To our knowledge, this is the first reported case of this condition in association with microspherophakia.

  References Top

Johnson GJ, Bosanquet RC. Spherophakia in a Newfoundland family: 8 years' experience. Can J Ophthalmol 1983;18:159-64.  Back to cited text no. 1
Higaki S, Maeda N, Watanabe H, Kiritoshi A, Inoue Y, Shimomura Y. Double anterior chamber deep lamellar keratoplasty: Case report. Cornea 1999;18:240-2.  Back to cited text no. 2
Probert LA. Spherophakia with brachydactyly; comparison with Marfan's syndrome. Am J Ophthalmol 1953;36:1571-4.  Back to cited text no. 3
Willoughby CE, Wishart PK. Lensectomy in the management of glaucoma in spherophakia. J Cataract Refract Surg 2002;28:1061-4.  Back to cited text no. 4
Faivre L, Dollfus H, Lyonnet S, Alembik Y, Mégarbané A, Samples J, et al. Clinical homogeneity and genetic heterogeneity in Weill-Marchesani syndrome. Am J Med Genet A 2003;123:204-7.  Back to cited text no. 5
Trainor PA, Tam PP. Cranial paraxial mesoderm and neural crest cells of the mouse embryo: Co-distribution in the craniofacial mesenchyme but distinct segregation in branchial arches. Development 1995;121:2569-82.  Back to cited text no. 6
Li HS, Yang JM, Jacobson RD, Pasko D, Sundin O. Pax-6 is first expressed in a region of ectoderm anterior to the early neural plate: Implications for stepwise determination of the lens. Dev Biol 1994;162:181-94.  Back to cited text no. 7
Kim IS, Shin JC, Im CY, Kim EK. Three cases of Descemet's membrane detachment after cataract surgery. Yonsei Med J 2005;46:719-23.   Back to cited text no. 8
Senoo T, Chiba K, Terada O, Hasegawa K, Obara Y. Visual acuity prognosis after anterior chamber air replacement to prevent pseudo-anterior chamber formation after deep lamellar keratoplasty. Jpn J Ophthalmol 2007;51:181-4.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7], [Figure 8]


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