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Measuring scleral thickness with optical coherence tomography in osteogenesis imperfecta: a case report
Author(s) -
Brehon A.,
Jullienne R.,
Kaspi M.,
Perrot J.L.,
Grivet D.,
Peoc'h M.,
Gain P.,
Thuret G.
Publication year - 2016
Publication title -
acta ophthalmologica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.534
H-Index - 87
eISSN - 1755-3768
pISSN - 1755-375X
DOI - 10.1111/j.1755-3768.2016.0356
Subject(s) - sclera , optical coherence tomography , osteogenesis imperfecta , medicine , ophthalmology , choroid , abnormality , anatomy , optics , retina , physics , psychiatry
Purpose The blue sclera appearance is a distinctive but non‐specific clinical sign for osteogenesis imperfecta (OI), a connective tissue disorder caused by an abnormality of type I collagen. It is a challenging diagnosis because multiple bone fractures, occurring in early childhood, can be the only sign of the non‐lethal forms of OI. In these situations, differential diagnosis with child abuse is a major concern for Pediatricians. Aim: to investigate the scleral optical coherence tomography (OCT) and thickness measurement as a diagnostic test for OI. Methods The sclera of a 28‐year‐old man affected by a mild form of OI and presenting a blue scleral hue was examined with the sclera mode coupled with the enhanced depth imaging of the spectral domain OCT equipped with the anterior segment module device (SD‐ASOCT) (Heidelberg Engineering GmbH, Heidelberg, Germany). The scleral thickness was measured at a distance of 2000 μ m from the scleral spur in eight meridians. Three measurements in each meridians were averaged and the mean and the standard deviation of the eight meridians were calculated. The examination of an age matched healthy volunteer served as a control. Results The mean scleral thickness of the OI patient was lower than that of the healthy patient, 277 μ m ± 43 μ m vs. 470 μ m ± 50 μ m. The superotemporal meridian was the thinnest in both patients. Conclusions These findings are consistent with the pathologic features previously described. The blue color results from the visualization of the underlying choroid through the translucent and thinner sclera. The in vivo scleral thickness measurement is a rapid and non‐invasive diagnostic tool that may be useful for challenging cases, especially in children with multiple fractures.