A Clinical Case Of Congenital Bilateral Cataract Associated With Lowe Syndrome

  Many systemic pediatric diseases can be  associated with cogenital cataract. Among them is  Lowe syndrome, wich is an X-linked recessive disorder, characterized by ocular . neurologic and renal abnormalities occurring predominantly in mailes. Ocular manifestations can include congenital cataract, corneal keloid and glaucoma. We would like to underscore the importance of managing congenital cataract associated with systemic pediatric disease and other ocular abnormalities with inccreased risk of complicationes.

During the last years we have met two clinical cases of congenital bilateral cataract associated with Lowe syndrome in pediatric center . According to our approaches early detection, on time surgery, optical correction with aphakic glasses, contact lenses, or IOL implantation, prevent ambliopia.   Due to potential complications these patients require long term follow up. 

A one-year-old boy aditted the  Ofthalmological Center after S.V. Malayan with complains of opacities in both eyes. Clinical examination detected nistagmus, corneal opacity, congenital cataract, ocular hypertension. Due to dense opacification of the lens fundoscopy couldn"t be  done. Axial length measerments by ultrasound biometry were 17,9 mm in the right eye, 18,10 mm in the left eye. Ultrasound examination revealed a remnant of hyaloid artery in the right eye. IOP was 26mm Hg inthe right eye, 27mm Hg in the left eye. The treatment plan included cataract surgery with primary single-piece hydrofob  IOL  implantation  in both eyes, ocular hypotensive drops for managing intraocular pressure, a few months later capsulotomy due to secondary cataract.   Considering and evaluating the risks of congenital anesthesia and systemic codition  of patient was decided  to do primary IOL implantation. After surgery fundoscopy revealed cup-disc ratio 0,5 in both  eyes. The patient is under observation. In addition, the second with Lowe syndrome was left aphakic because of  the age of patient (3-month-old) and incresed risk of glaucoma progrssion. For control of intraocular  pressure was required Preserflo MicroShunt implamtation in one eye. 

The course and visual prognoses of cataracr in patients with Lowe syndrome is highly  variable due to various visual abnormalities.  It is importany to  prevent life treatening complications due renal patholohy, hypotonia or infection.  These patients require long term regular follow up, spcially control of glaucoma.