Double anterior chamber in a patient with glaucoma and microspherophakia.

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.

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

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 [Figs. 1 and 2]. Dilated fundus examination revealed near complete cupping of both optic nerves [Figs. 3 and 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 [Fig. 5]. Gonioscopy of the left eye was inconclusive due to the membrane's position [Figs 6 and 7]. Chamber volume was 309 mm3 in the right eye and 240 mm3 in the left eye. Endothelial cell count was 2336 cells/mm2 in the right eye and 1036 cells/mm2 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 [Fig. 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

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

Figure 3

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

Figure 4

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

Figure 5

Gonioscopic photograph of the right eye showing an open iridocorneal angle

Figure 6

Gonioscopic photograph of the left eye

Figure 7

Gonioscopic photograph of the left eye showing part of the membrane

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

Microspherophakia is a rare abnormality that can be isolated or associated with other ocular or systemic disorders such as Weill-Marchesani syndrome, 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 [Fig. 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

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.