Ramos-Arroyo syndrome: confirmation of an entity.

In 1987, Ramos-Arroyo et al. described a family with a previously unreported combination of features, which included corneal anesthesia, short stature, sensorineural deafness, learning difficulties, and a characteristic facial appearance. The family was reviewed in 2008 and additional features were noted. The name Ramos-Arroyo syndrome was proposed. The condition can be delineated by corneal anesthesia, absence of the peripapillary choriocapillaris and retinal pigment epithelium, bilateral sensorineural hearing loss, unusual facial appearance, persistent ductus arteriosus, Hirschprung disease, short stature, and intellectual disability. No other patient has been described since. Here, we report on a 5-year-old girl with features consistent with Ramos-Arroyo syndrome. We propose an overlap of this condition with dysautonomia syndromes.

Postoperative surface deposits on intraocular lenses in children.

PURPOSE: To report the clinicopathologic features of 4 intraocular lenses (IOLs) composed of 3 different hydrophilic biomaterials explanted from children who had postoperative opacification of the IOL optic. SETTING: David J Apple, MD, Laboratories for Ophthalmic Devices Research, John A. Moran Eye Center, Department of Ophthalmology and Visual Sciences, University of Utah, Salt Lake City, Utah, USA. METHODS: The IOLs were explanted 20, 11, 22, and 25 months postoperatively from children aged 10, 3, 36, and 20 months old, respectively, at IOL implantation. Clinical data were obtained to correlate the findings with possible associated risk factors. The explanted IOLs were examined by gross and light microscopy. They were further analyzed with a stain for calcium, alizarin red 1%. Scanning electron microscopy and energy dispersive X-ray spectroscopy (EDS) were also performed. RESULTS: The primary reason for cataract surgery in Case 1 and Case 2 was persistent hyperplastic primary vitreous (PHPV); 1 patient received a B-Lens IOL (Hanita) and the other a Centerflex IOL (Rayner). The primary reason in Case 3 was familial bilateral congenital cataract and in Case 4, rubella cataract; both patients received a Hydroview IOL (Bausch & Lomb). All 4 IOLs had surface deposits on the optic, but the morphology of the deposits on the B-Lens and Centerflex IOLs was different than that in previously reported cases. The deposits in all four cases stained positive with alizarin red and consisted of calcium and phosphorus when imaged with EDS. CONCLUSIONS: Calcified deposits on 2 Hydroview IOLs explanted from children were similar to those seen in adults with the same IOL. The deposits on the B-Lens and Centerflex IOLs were probably secondary to a breakdown of the blood-aqueous barrier caused by preexisting PHPV.