Implications of neuropathy and management of the corneal surface in a patient with Stuve-Wiedemann syndrome.

Abstract

Purpose: To describe the ophthalmological management of a girl diagnosed with Stuve Wiedemann syndrome (SWS). Clinical and in vivo confocal microscopy (IVCM) are described. Methods: Case report of a 6-year-old girl, who presented with neurotrophic keratitis and was treated with intense lubrication including heterologous serum and tear plugs. Results: In the following months, the evolution of the neurotrophic keratitis was good, but a hypertrophic corneal leukoma persisted with mild neovascularization in the left eye. Conclusion: Close ophthalmological follow-up in patients with SWS is needed, given that most of the time they do not present symptoms due to the characteristic neuropathy of their lesions. Abbreviations: SWS = Stuve-Wiedemann syndrome, IVCM = in vivo confocal microscopy, CNTF = ciliary neurotrophic factor, BCVA = best corrected visual acuity, LIFR = Leukemia Inhibitory Factor Receptor, IGF1 = Insulin-like growth factor-1.