Hallermann-Streiff Syndrome: Should spontaneous cataract absorption be awaited?

Background: As the lens may become absorbed spontaneously extraction of the congenital cataract in the Hallermann-Streiff syndrome is controversial. Patient: A male newborn with Hallermann-Streiff syndrome was referred to the eye clinic because of a bilateral mature cataract. The risks of an operation seemed too serious because the eyes were extremely microphthalmic (a-p diameter approx. 10,5 mm). Instead, it was decided to await spontaneous lens absorption which then occurred slowly and completely between the beginning of the 2(nd) and the end of the 3(rd) year of life without considerable inflammatory responses. In the meantime, the child has a good orientation and is able to paint and to discriminate colours without optic correction. Conclusions: The critical investigation of the literature reveals that the frequency of spontaneous lens absorption in the Hallermann-Streiff syndrome is probably (widely) underestimated. It can be estimated with up to 50% (or even more) if patients which were operated on are excluded and if untreated patients are followed up till the end of the 5(th) year of life. Functional results seem to be quite similar for treated and untreated eyes. Therefore we would recommend to await the spontaneous lens absorption in the Hallermann-Steiff syndrome especially when the cataract is combined with considerable microphthalmia.