microspherophakia

microspherophakia

A congenital, usually bilateral, condition in which the crystalline lens is smaller than normal and spherical in shape. It may give rise to lenticular myopia, subluxation or glaucoma. It may occur independently or it may be associated with the Weill-Marchesani syndrome or more rarely with Marfan's syndrome, Peter's anomaly or congenital rubella.
References in periodicals archive ?
Microspherophakia is a rare entity in which there is a small, spherical crystalline lens with increased antero-posterior thickness.
Her brother also has myopia and microspherophakia. Her brother's IOP was normal but appositional angle closure was observed in both eyes.
Microspherophakia is usually associated with systemic disorders such as Weill-Marchesani syndrome (WMS), homocystinemia, Marfan syndrome, Alport syndrome and Klinefelter syndrome.
Characteristic eye abnormalities of WMS are microspherophakia and ectopia lentis which causes high myopia (mostly dislocates either inferiorly or anteriorly).
Her condition might be familial in origin, because her brother also has microspherophakia. Genetic counseling could not be performed due to the patient's health insurance problem.
Glaucoma in isolated microspherophakia is not common.
Thus, uncontrolled IOP with medical therapy and resolution of her condition with laser iridotomy confirmed our diagnosis that microspherophakia induced pupillary-block glaucoma.
Based on available data, we suggest that a stepwise treatment protocol would be more safe and effective in the management of the patients with glaucoma secondary to microspherophakia. According to this treatment protocol, laser iridotomy should be performed first.
In conclusion, optimal management of glaucoma in microspherophakia is still uncertain.
Dominant microspherophakia. Arch Ophthalmol, 1971;85:534-537.
Histopathological study of microspherophakia in the Weill-Marchesani syndrome.
* Small spherical lens only seen on full dilation (microspherophakia see Figure 4)