Changes in retinal nerve fibre layer, optic nerve head morphology, and visual field after acute primary angle closure.

AIMS/PURPOSE: To determine and correlate the long-term changes in retinal nerve fibre layer (RNFL) thickness, optic nerve head (ONH) morphology, and visual fields after a single episode of acute primary angle closure (APAC). METHODS: This was a cross-sectional comparative study of patients at National University Hospital (Singapore) from 2000 to 2006 after an episode of unilateral APAC. The peripapillary and macular RNFL were measured using Stratus optical coherence tomography (OCT) and ONH configuration was assessed using Heidelberg Retina Tomography (HRT)-III. Humphrey perimetry was also performed, and the presence of disc pallor was noted. APAC eyes were compared with fellow eyes as matched controls. RESULTS: Twenty-five patients were assessed at a median of 33 months (range, 11-85 months) after APAC. OCT showed that there was a reduction in the peripapillary and outer macular RNFL thickness in APAC eyes compared with controls. Humphrey perimetry revealed significantly reduced mean deviation (P=0.006) and increased pattern standard deviation (P=0.045) in APAC eyes compared with controls. HRT-III showed no difference in mean rim area, rim volume, or cup-disc ratio between APAC eyes and controls. Disc pallor was present in nine APAC eyes (36%) but was absent in fellow eyes (P=0.002), and was associated with peripapillary RNFL thinning, visual field loss, and an increased interval between the onset of symptoms and normalization of intraocular pressure (P=0.023). CONCLUSION: APAC results in peripapillary and outer macular RNFL loss, visual field defects, and optic disc pallor, even in cases in which the ONH configuration remains unchanged.

Tuberous sclerosis with visceral organ involvement.

This study is to determine the incidence of visceral organ involvement in tuberous sclerosis (TS). We reviewed 30 cases of TS diagnosed between 1987 to 1997. There were 17 males and 13 females, ages ranged from one day old to 17 years old. Among the 30 cases, 25 patients had seizures and skin manifestations; 24 had cerebral tubercles; 10 had heart involvement (9 rhabdomyoma, 1 dilated cardiomyopathy); 4 had kidney involvement (3 polycystic kidney disease, 1 renal hamartoma); and 3 had retinal astrocytic hamartoma. Based on our study, the most common visceral organs involved were the heart and kidney. Among the ten patients with cardiac rhabdomyoma, six were less than 1 year old (mean age 1.6 +/- 2.2 years old). One newborn presented with a cardiac mass diagnosed by prenatal sonography and another newborn, noted to have tachycardia after birth, underwent surgical intervention due to impending heart failure. Four patients had kidney abnormalities; three were less than 5 years old (mean age 5.2 +/- 5.2 years). During this 10 year period, there was no mortality seen among patients with visceral organ involvement. We suggest that clinicians treating patients with TS should not overlook the visceral organs, especially heart and kidney, which, if involved can have significant morbidity.