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

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.