Intraoperative discovery of lens dislocation in a child with Knobloch syndrome

We present the case of a 14-year-old boy with a known diagnosis of Knobloch syndrome (KS) referred for bilateral cataract evaluation and possible cataract surgery. At time of initial presentation, no lens subluxation was appreciated, and no phacodonesis was detected on slit lamp biomicroscopy. However, 7-weeks later, on the day of surgery, his right eye was found to have a complete lens dislocation into the vitreous cavity, with no zonule attachment. The left eye did not have subluxated lens; however, intraoperatively, after irrigation into the eye, near complete zonular dialysis was observed. This case highlights the importance of regular follow-up of children with KS.