DISEASE


Straatsma syndrome is a rare disease entity characterized by the traditional triad of unilateral myelinated retinal nerve fibers (MNRF), axial myopia, and amblyopia.


Slit Lamp Adapter

Slit Lamp Adapter

Slit Lamp Adapter - Get

Straatsma syndrome

The original description of the condition also included strabismus. Variations of the triad include a “reverse Straatsma syndrome ”, in which patients exhibit hyperopia instead of myopia.

Even though nystagmus and strabismus have not been prominently associated with Stratsma syndrome, either may be present as complimentary findings without precluding one from the diagnosis.

Even though Straatsma syndrome has traditionally been considered a unilateral disorder, cases of bilateral Straatsma syndrome and bilateral reverse Straatsma syndrome have also been reported.

Diagnosis


MRNF appears as a white lesion usually contiguous with the optic nerve head. The distribution is along the retinal nerve fiber layer and typically feather-like appearance is noted at the margin.

The MRNF does not cross the horizontal raphe. MRNF may be separate from the optic disc and may be present in the peripheral retina.

Straatsma syndrome

Despite some controversy in its diagnostic features, it should be included in the differential diagnosis of leukocoria and must be suspected in the presence of refractive errors with anisometropia.

MANAGEMENT


The management includes providing proper refractive correction and ruling our systemic associations.

Prognosis


The visual prognosis of amblyopia associated with myelination of retinal nerve fibers and anisometropia is poorer than anisometropic amblyopia without myelination.

Straatsma syndrome

It is well known that the former is refractory to occlusive therapy. Despite having a poor prognosis, visual rehabilitation should be attempted.

Would you have interest in taking retina images by smartphone?

Fundus photography is superior to fundus analysis as it enables intraocular pathologies to be photo-captured and encrypted information to be shared with colleagues and patients.

Recent technologies allow smartphone-based attachments and integrated lens adaptors to transform the smartphone into a portable fundus camera and Retinal imaging by smartphone.

RETINAL IMAGING BY YOUR SMARTPHONE

REFERENCES


  1.  Juhn, A. T., Houston, S. K., & Mehta, S. (2015). BILATERAL STRAATSMA SYNDROME WITH NYSTAGMUS. Retinal Cases & Brief Reports, 9(3), 198–200. doi:10.1097/icb.0000000000000137 
  2.  Straatsma BR, Heckenlively JR, Foos RY, Shahinian JK. Myelinated retinal nerve fibers associated with ipsilateral myopia, amblyopia, and strabismus. Am J Ophthalmol. 1979 Sep;88(3 Pt 1):506-10. doi: 10.1016/0002-9394(79)90655-x. PMID: 484678. 
  3.  Shenoy R, Bialasiewicz AA, Al Barwani B. Bilateral hypermetropia, myelinated retinal nerve fibers, and amblyopia. Middle East Afr J Ophthalmol 2011;18:65–66
  4.  Levy NS, Ernest JT. Retinal medullated nerve fibers. Arch Ophthalmol 1974;91:330–331
  5.  Congenital optic disk anomalies [Internet]. W.B. Saunders; 2013 [cited 2021 May 19]. Available from: https://www.sciencedirect.com/science/article/pii/B9780702046919000510
  6.  Rosen B, Barry C, Constable IJ. Progression of myelinated retinal nerve fibers. Am J Ophthalmol. 1999 Apr;127(4):471-3. doi: 10.1016/s0002-9394(98)00377-8. PMID: 10218709.
  7.  Quezada-Del Cid NC, Zimmermann-Paiz MA, Ordoñez-Rivas AM, Burgos-Elías VY, Marroquin-Sarti MJ. Straatsma syndrome: Satisfactory amblyopia treatment. Report of two cases. Arch Soc Esp Oftalmol. 2018 Jun;93(6):300-302. English, Spanish. doi: 10.1016/j.oftal.2017.12.010. Epub 2018 Feb 15. PMID: 29398227.

RETINAL IMAGING BY YOUR SMARTPHONE

RETINAL IMAGING BY YOUR SMARTPHONE

RETINAL IMAGING BY YOUR SMARTPHONE

View More Details