Metaplastic ossification frequently follows ocular scarring. Anterior scleral ossicles are normal in most common fish, e.g. dogfish, and in reptiles, and are said to have accommodative and protective functions. Birds (2I9 out of 532 species: Tiemeier, I950) have an os opticus of highly cancellous texture lying in the posterior sclera and surrounding the optic nerve head in one or several places. Duke-Elder (1958) wrote that "no satisfactory theory for its presence has been put forward". He also mentioned the occurrence of "fatty tissue, cartilage, and even bone" in coloboma of the vitreous in microphthalmic eyes I964). This description may refer to cases now being recognized as examples of the I3-I5 trisomy syndrome. Saraux and Dhermy (I968) found a nodule of well-developed bone in the line of a uveal coloboma but located episclerally near the optic nerve. This was present in a case of D-trisomy with translocation.This paper reports a case of congenital isolated posterior intrascleral ossification, which appears to be a unique finding.
Clinical historyA married woman aged 23 years was delivered at full term of her first baby on May 25, I969. She had had "tablets" for hyperemesis gravidarum in the first trimester and apart from an E. coli pyelitis, the antenatal history was uneventful. She was blood group 0 RH+. The Wassermann reaction and Venereal Disease Reference Laboratory tests were negative. There was no family history of ocular disorder. The baby girl, delivered by vacuum extraction and weighing 3 3 kg., was admitted to the Special Care Unit where she was found to have a left facial palsy and bilateral colobomata. She died 48 hours later after repeated convulsions. Skull and lateral spinal x rays, urinary chromatography, and karyotyping gave normal results. Laboratory tests showed evidence of urinary infection and the serum had low calcium, raised phosphorus, and normal electrolyte levels.
Pathology AUTOPSY FINDINGSThe baby weighed 3 kg. The cranium and face appeared to be normally formed except for atypical dysplasia of the left auricle, which had a large flat helix with a single layer pointing upwards and an absent lobe. The right ear was large but had a tiny lobe. The eyes appeared to lie within normal orbital fossae. No congenital defects were found in the other systems. The cause of death was bilateral bronchopneumonia.
Macroscopical examinationThe left eye measures 20 X I9 x I8 mm. with an indefinite cornea of approximately Io mm. diameter and an inferior nasal iris coloboma. The opened specimen shows a
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.