In congenital glaucoma, larger degrees of corneal distention are better tolerated by the epithelium and stroma than by the Descemet membrane. A sequential ultrasound biomicroscopical (UBM) examination of the cornea helps demonstrate both the pathophysiological mechanism for the development of breaks in the Descemet membrane as well as an accompanying clefting of stromal tissue causing acute corneal hydrops. Such examinations can provide warning of impending ruptures affecting visual prognosis.
As recently demonstrated via UBM by Nakagawa and colleagues,1 rupture of the Descemet membrane in keratoconus is also often accompanied by clefting of the stromal tissue. This leads to acute corneal opacification as well as a permanent scar after healing by secondary intention. A similar final pathway, but with minimal stromal cicatrization due to healing by primary intention, may be present in congenital glaucoma with megalocornea. Imaging with UBM has proved reliable in delineating different corneal layers when compared with histopathological sections and allows for in vivo anatomical studies.2 We performed UBM image acquisition under general anesthesia in a patient with congenital glaucoma, both prior to surgical treatment and during routine follow-up. Images obtained (Figures 1, 2, 3, 4, and 5) demonstrate mechanical stretching of the cornea leading to rupture of the Descemet membrane accompanied by heretofore unrecorded clefting of the stromal tissue. Imaging of the cornea with UBM can provide information regarding the presence, position, and evolution of potential breaks in the Descemet membrane along with clefting of the stromal tissue in congenital glaucoma.
The corneal epithelium, Bowman membrane, stroma, Descemet membrane, and corneal endothelium are all clearly delineated. Note the significant epithelial edema at age 2 weeks without any detachment of the Descemet membrane or endothelial layer. A trabeculectomy procedure was then performed.
Visible separation of the Descemet membrane 5 weeks later during a standard follow-up examination reveals decreased extensibility of the Descemet membrane vis-à-vis the stroma and anterior corneal tissue layers. Centered beneath where the overlying epithelium edema is greatest, there appears to be a decreased reflectivity of both the posterior stromal surface (arrow) and the endothelium with the Descemet membrane. Topical pressure-lowering medications were prescribed while the contralateral eye underwent a trabeculectomy procedure.
The patient did not receive the prescribed topical medications; 2 weeks later, an image was obtained approximately 6 hours after endothelium and Descemet membrane rupture had occurred, producing acute corneal hydrops. The detached portion of the Descemet membrane is slack with rolled edges, and a communication between the anterior chamber and the stroma is clearly visible. A tear and irregularity of the posterior stromal surface are also evident (arrow) where decreased reflectivity was previously noted in Figure 2, indicating stromal clefting that exacerbates corneal edema. Epithelial edema is visibly more significant as a consequence of the break both in the endothelium and Descemet membrane as well as within the stroma. A repeat trabeculectomy procedure was performed.
Two weeks after the break, complications in the contralateral eye necessitated another repeat trabeculectomy. Subsequent to normalization of intraocular pressure and reduction of corneal distention, the posterior stromal discontinuity is no longer apparent with the cleft now already sealed by primary intention (arrow). There no longer is cleavage visible between the Descemet membrane and the stroma on the left side, and endothelial cells can migrate over the stroma.
Three months after the break, during standard follow-up examination. The epithelial layer remains moderately thickened despite continued migration of endothelial cells and laying down of new basement membrane material, now filling the previously noticeable cleavage space between the Descemet membrane and the stroma on the right. Clinically, a barely visible diffuse white scar is present, corresponding to the area of previous stromal clefting (arrow), with the surrounding cornea now transparent.
Correspondence: Dr Parsa, MD, Wilmer Ophthalmological Institute, Johns Hopkins University School of Medicine, 600 N Wolfe St, Wilmer 233, Baltimore, MD 21287-9028 (firstname.lastname@example.org).
Financial Disclosure: None reported.
Additional Information: Drs Roche and Dufier are with the Hôpital Necker-Enfants Malades, Paris, France, and Dr Beby is with the Service d'Ophtalmologie, Hôpital Edouard Herriot, Lyon, France.
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