HOMEPRODUCTSCOMPANYCONTACTFAQResearchDictionaryPharmaSign Up FREE or Login

Ocular treatment of children with Stuve-Wiedemann syndrome.

AbstractPURPOSE:
Stuve-Wiedemann syndrome is a rare condition consisting of bone dysplasia, hypotony, and dysautonomia with ocular and neuropathic features. We present the ocular findings and visual outcome in 4 patients (2 siblings, 1 related cousin, and 1 unrelated patient) with this syndrome.
METHODS:
A retrospective case series review. Clinical notes of 4 patients with Stuve-Wiedemann syndrome were reviewed for findings of systemic features, ocular examination, visual acuity, and ocular procedures.
RESULTS:
The median age at presentation was 16 months (range, 14-72 months). All 4 cases consisted of bilateral plaque-like corneal scarring with reduced corneal sensation. Treatment comprised topical lubrication, punctal plugs, lateral tarsorrhaphies, surgical optical iridectomies when required, and aggressive visual rehabilitation with frequent refraction and occlusion therapy if necessary. Mean best-corrected visual acuity (BCVA) in the eye with best vision was 0.86 logarithm of the minimum angle of resolution (logMAR) (range, 0.72-1.1 logMAR) at presentation and 0.43 logMAR (range, 0.25-0.56 logMAR) at the last follow-up. The BCVA in the eye with worst vision was 0.98 logMAR (range, 0.72-1.3 logMAR) at presentation and 0.68 logMAR (range, 0.47-1.0 logMAR) at the last follow-up. The median follow-up was 3.2 years (range, 1.1-5.8 years).
CONCLUSIONS:
All of our patients showed improved BCVA with the treatment regimen prescribed. Given these children's debilitating orthopedic problems, visual function is important and has hitherto been reported as being poor.
AuthorsAnas M Injarie, Aman Narang, Zubair Idrees, Anand K Saggar, Ken K Nischal
JournalCornea (Cornea) Vol. 31 Issue 3 Pg. 269-72 (Mar 2012) ISSN: 1536-4798 [Electronic] United States
PMID22316651 (Publication Type: Journal Article)
Chemical References
  • Lubricants
  • Silicones
Topics
  • Administration, Topical
  • Child
  • Child, Preschool
  • Exostoses, Multiple Hereditary (pathology, physiopathology, therapy)
  • Female
  • Follow-Up Studies
  • Humans
  • Infant
  • Iridectomy (methods)
  • Lubricants (administration & dosage)
  • Male
  • Osteochondrodysplasias (pathology, physiopathology, therapy)
  • Prostheses and Implants
  • Retrospective Studies
  • Silicones (administration & dosage)
  • Visual Acuity (physiology)

Join CureHunter, for free Research Interface BASIC access!

Take advantage of free CureHunter research engine access to explore the best drug and treatment options for any disease. Find out why thousands of doctors, pharma researchers and patient activists around the world use CureHunter every day.
Realize the full power of the drug-disease research graph!


Choose Username:
Email:
Password:
Verify Password:
Enter Code Shown: