Bilateral Chandler Syndrome, Nanophthalmos, and Angle Closure Glaucoma: A Complex Presentation, Challenging Diagnosis, and Pathological Insight—A Case Report
Aim and background: Chandler syndrome (CS) is one of the iridocorneal endothelial syndromes (ICEs) with proliferation of abnormal corneal endothelial cells over the anterior chamber (AC) angle and iris, resulting in complications, for example, secondary angle closure glaucoma (SACG). We report an association between CS and nanophthalmos, highlighting diagnostic and therapeutic challenges and pathological insights.
Case description: A 46-year-old female patient presented with bilateral progressive blurring of vision. Examination revealed bilateral (OU) small corneal diameter, shallow AC, closed AC angle, beaten-bronze appearance of corneal endothelium, and mild iris atrophy in the right eye (OD). Intraocular pressure was 48 mm Hg and 22 mm Hg in the OD and left eye (OS), respectively. Fundus examination revealed optic nerve head cupping. Biometry showed short axial length and microcornea OU, that is, nanophthalmos. Optical coherence tomography and visual field revealed structural and functional evidence of glaucomatous optic neuropathy. Specular microscopy demonstrated reduction of corneal endothelial cell density and the light-dark reversal characteristic of ICE. Therefore, a diagnosis of CS with SACG and nanophthalmos was made. The patient was referred to a specialized glaucoma center with recommendation of clear lens extraction and a glaucoma drainage device with retropupillary tube placement.
Conclusion: This is the first report of an association between CS and nanophthalmos. It highlights the possibility of SACG despite evident risk factors for primary angle closure glaucoma (PACG). Furthermore, it provides a hypothesis about the etiology of ICE. The concurrence of CS and nanophthalmos suggests that a common developmental mechanism could be implicated since periocular mesenchyme, the embryological precursor of corneal endothelium, plays a role in the development of optic cup and stalk.
Clinical significance: SACG should be considered even in the presence of evident risk factors for PACG, such as nanophthalmos. Additionally, the association of nanophthalmos and CS warrants revisiting the yet inconclusive etiology of CS, where a developmental mechanism could be considered.
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