Nassim Calixto

Congenital absence of lacrimal puncta and salivary glands: report of a Brazilian family and review.

Congenital absence of the salivary glands and lacrimal puncta is a rare autosomal-dominant disorder with variable expressivity. Only a few instances of this condition have been reported. We present the first Brazilian observation of this syndrome and a review of the literature.

Oculodentodigital dysplasia: study of ophthalmological and clinical manifestations in three boys with probably autosomal recessive inheritance

Oculodentodigital dysplasia (ODDD) is a rare inherited disorder affecting the development of the face, eyes, teeth, and limbs. The majority of cases of ODDD are inherited as an autosomal dominant condition. There are few reports of probable autosomal recessive transmission. Affected patients exhibit a distinctive physiognomy with a narrow nose, hypoplastic alae nasi, and anteverted nostrils, bilateral microphthalmos, and microcornea. Sometimes iris anomalies and secondary glaucoma are present. There are malformations of the distal extremities such as syndactyly. In addition, there are defects in the dental enamel with hypoplasia and yellow discoloration of the teeth. Less common features include hypotrichosis, intracranial calcifications, and conductive deafness secondary to recurrent otitis media. We describe three brothers with ODDD. Their parents are first cousins and present no features of ODDD. These data are in favor of autosomal recessive inheritance and suggest genetic heterogeneity for this entity.

Espessura central e periférica da córnea: influência na medida da pressão intra-ocular pelo Tonopen

PURPOSE To assess the influence of central and peripheral corneal thickness on the IOP measurement by Tonopen. METHODS The IOP of 58 eyes from suspected and glaucomatous patients was evaluated by Goldmanns tonometry (GT) and, by Tonopen XL on the central (TC) and peripheral (TP) regions of the cornea. Using the DGH 5100e pachymeter, we also measured the corneal thickness in its central (CCT) and peripheral (at 6:00 h) regions (PCT). Patients with any corneal changes were excluded. The mean values of all measurements was comparatively analyzed (GT x TC; GT x TP; TC x TP; CCT x PCT) by the paired t test and by regression and correlation analysis. RESULTS The means (+/- standard deviation) of IOP obtained with GT, TC and TP were respectively 14.6 (+/-) mmHg; 18.9 (+/-2.9) mmHg and 20.7 (+/-3.7) mmHg with a statistical highly significant difference (p<0.001). The means (+/- standard deviation) of central and peripheral corneal thickness were respectively 526 (+/-42.3) and 639 (+/-52.9) microm, also with a statistical highly significant difference (p<0.001). The correlation coefficients (r) between the measurements were 0.71 (p<0.001) for GT and TC; 0.65 (p<0.001) for GT and TP and 0.64 (p<0.001) for TC and TP. CONCLUSIONS There was a positive correlation between the measurements performed with Tonopen XL and Goldmanns tonometer at the central region of the cornea and the mean IOP was higher with Tonopen XL. The mean IOP obtained by TP was higher than that obtained by TC. These data showed that Tonopen XL, besides providing higher IOP readings than those with Goldmanns tonometer, is influenced by the corneal thickness presenting higher IOP readings in the periphery than in the central region of the cornea.

New comparative ultrasound biomicroscopic findings between fellow eyes of acute angle closure and glaucomatous eyes with narrow angle.

PURPOSE To compare morphometric features between fellow acute primary angle-closure (APAC) eyes and glaucomatous or suspect eyes with narrow angle (NA). METHODS Fellow eyes of 30 patients with unilateral APAC and 30 with NA were evaluated by ultrasound biomicroscopy (UBM) under light and dark conditions. UBM parameters such as anterior chamber depth (ACD), angle opening distance at 250 microm/500 microm from the scleral spur (AOD250/AOD500), trabecular ciliary process distance (TCPD) and iris-lens contact distance (ILCD) were measured in the superior (SQ) and inferior (IQ) quadrants. RESULTS Significant differences between APAC fellow and NA eyes were found in ACD, P<0.001; AOD250 at SQ and IQ, P<0.001; AOD500 at SQ and IQ, P<0.001; TCPD light, P=0.010 and TCPD dark at SQ, P=0.031; and TCPD light at IQ, P=0.010. Significant differences between light and dark examinations of APAC fellow eyes were found in ILCD (P=0.009) at SQ and ILCD at IQ (P=0.006), and of NA eyes in ILCD at SQ (P=0.047) and ILCD at IQ (P<0.001). CONCLUSIONS APAC fellow eyes have a more crowded anterior segment and shallower ACD than NA eyes. ILCD decreases in both groups when the illumination conditions change from light to dark.

Intraocular pressure and ocular rigidity after LASIK

PURPOSE To assess the intraocular pressure (IOP) and ocular rigidity (E) at 1, 3, 6 and 24 postoperative months in eyes that underwent LASIK. METHODS The IOP measured by Goldmann applanation tonometer (GAT) and by the 5.5 and 10 gm. weights of a standardized Schioetz tonometer (ST) and the E measured by differential tonometry (the 5.5 and 10 gm. of ST) were assessed before and after LASIK (at 1, 3, 6 and 24 month) in 23 eyes. The volume of the corneal indentation (Vc) and IOP with ST resting on the eye (tonometric pressure - Pt) were also evaluated. RESULTS The average IOP (GAT) at 6 and 24 months after LASIK was lower than at 1 and 3 months and before LASIK. A statistically significant difference of E was found at 1, 3, 6 and 24 months. The reduction of the central corneal thickness (CCT) by LASIK was the cause of increasing values of Vc and decreased values of Pt. In turn, the changes in Vc and Pt were responsible for the lower IOP measurements by GAT and E changes. CONCLUSIONS LASIK reduces IOP readings by GAT but not the ones by ST (differential tonometry). LASIK also diminishes E. IOP (GAT) values are lower at 6 and 24 months. IOP evaluation by ST is more accurate than that by GAT. At 6 months, Vc, Pt and E parameters are stabilized (no changes relatively to 24 months measurements.

Plateau iris configuration in eyes with narrow-angle: an ultrasound biomicroscopic study

PURPOSE To investigate, through ultrasound biomicroscopy images, the presence of plateau iris configuration in eyes with narrow-angle from patients with open-angle glaucoma and in eyes with previous acute primary angle-closure and compare the biometric features of eyes with plateau iris configuration with those of normal eyes. METHODS Ultrasound biomicroscopic images from 196 patients with open-angle glaucoma and narrow-angle and 32 patients with acute primary angle-closure were retrospectively analyzed. The inclusion and specific criteria for the diagnosis of plateau iris configuration was the presence of an anterior positioning of the ciliary processes, supporting the peripheral iris so that it was parallel to the trabecular meshwork; the iris root had a steep rise from its insertion point, followed by a downward angulation from the corneoscleral wall; presence of a central flat iris plane; an absent (complete plateau iris configuration) or partially absent (incomplete plateau iris configuration) ciliary sulcus. The ultrasound biomicroscopic parameters were compared between complete and incomplete plateau iris configuration. The same parameters of both groups were compared with those of normal eyes. The following measurements were performed: anterior chamber depth; angle opening distance at 500 microm from the scleral spur; peripheral iris thickness at 500 microm from the scleral spur; iris-ciliary process distance; trabecular-ciliary process distance and angle recess area. RESULTS Plateau iris configuration was found in 33 eyes of 20 (10.2%) out of 196 patients with open-angle glaucoma and narrow-angle and in 4 eyes of 2 (6.3%) out of 32 patients with acute primary angleclosure. Seventeen (77.3%) patients with plateau iris configuration were female and 5 (22.7%) male. Twenty-three (62.2%) out of 37 eyes had complete plateau iris configuration, and 14 (37.8%) had incomplete plateau iris configuration. Complete and incomplete plateau iris configuration presented similar biometric features with the exception of the iris-ciliary process distance. All plateau iris configuration eyes showed biometric parameters completely different from those of normal eyes except for peripheral iris thickness at 500 microm from the scleral spur. CONCLUSIONS Plateau iris configuration was present in 10.2% of patients with open-angle glaucoma and narrow-angle and in 6.3% of patients with acute primary angle-closure. Biometric features were similar in eyes with complete and incomplete plateau iris configuration with the exception of iris-ciliary process distance. Compared to normal eyes, all plateau iris configuration eyes showed biometric parameters completely different except for peripheral iris thickness at 500 microm from the scleral spur.

New considerations on pupillary block mechanim

PURPOSE To study the mechanisms of pupillary block in eyes with occludable angle by ultrasound biomicroscopy. METHODS Initially, a pilot study of 13 eyes with acute primary angle-closure without medication was executed. Ultrasound biomicroscopy measurements of the angle, posterior chamber depth and iris thickness were performed in the temporal quadrant under light and dark conditions. Afterwards, ultrasound biomicroscopy measurements of iris-lens contact distance and iris-lens angle in the temporal quadrant and central anterior chamber depth were made in 32 eyes with acute primary angle-closure or intermittent primary angle-closure without medication, under light and dark conditions before and after laser peripheral iridectomy. RESULTS In the pilot study, a significant decrease in the angle as well as a significant increase in the iris thickness occurred when comparing light to dark conditions. Before and after laser peripheral iridectomy (second study), significant differences were found in iris-lens contact distance (P<0.001) and iris-lens angle (P<0.001) under light and dark conditions. Also, significant differences were found in light and dark conditions, before laser peripheral iridectomy, in iris-lens angle (P=0.005), and after laser peripheral iridectomy, in iris-lens contact distance (P<0.001). No significant change occurred with anterior chamber depth. CONCLUSIONS A decreased angle was correlated to an increase in iris thickness. After laser peripheral iridectomy, acute primary angle-closure or primary angle-closure eyes had an increased iris-lens contact distance and a decreased iris-lens angle. The anterior chamber depth did not change. These findings contradict the theory that pupillary block is the mechanism of acute primary angle-closure.

Reprodutibilidade intra-observador de medidas morfométricas do segmento anterior em olhos normais pela biomicroscopia ultra-sônica (UBM)

PURPOSE To systematically study the anterior segment morphometric measurements by ultrasound biomicroscopy (UBM) in a large population of normal eyes, to evaluate their intra-observer reproducibility. Two new measurements are proposed and also tested concerning their intra-observer reproducibility. METHODS One hundred ninety eyes of 101 patients with normal ophthalmic examination were studied by UBM. Anterior chamber depth (ACD) along with 11 different morphometric parameters that characterize the anterior segment morphometry were measured in the superior, nasal, inferior and temporal meridians. All measurements were performed twice with a minimum interval of four weeks between them. RESULTS There were no statistical significant differences between the first (M1) and second (M2) measurements and regarding each studied parameter except for 2 variables in 2 meridians in the right eyes (OD) and 2 variables of one meridian in the left eyes (OS). Even in these cases, the largest difference between M1 and M2 was less than 0.006 mm, which was not clinically significant. The new tested parameters also presented a good intra-observer reproducibility. CONCLUSION This study confirmed the good intra-observer reproducibility of the variables that characterize anterior segment morphometry by UBM.

Corneal thickness and daily curve of intraocular pressure in suspected and glaucomatous patients

PURPOSE To evaluate in suspected and glaucomatous patients the correlation between central corneal thickness (CCT) and intraocular pressure (IOP), measured during the daily curve of intraocular pressure (DCPo), including CCT and IOP measurements in bed at 6 am. The ocular axial length was also compared. METHODS 114 eyes from 73 patients, 30 eyes suspected of glaucoma (group I) and 84 eyes with chronic open-angle glaucoma (group II) were prospectively studied. Both groups were subjected to DCPo with measurements of IOP at 9 am, 12 pm, 6 pm, 10:30 pm and on the next day at 6 am in bed and in darkness before the patient gets up. Using the ultrasonic DGH 5100 device, CCT measurements were performed at 9 am, 6 pm, 10:30 pm and the next day at 6 am in bed before the patient gets up. The ocular axial length was also measured. RESULTS In the total sample and, separately in each group, the mean IOP was more elevated in bed at 6 am. There were no significant CCT changes during the day within each group or between both groups. No statistically significant difference was found between the mean ocular axial length of both groups. CONCLUSIONS In suspected and glaucomatous patients, there were no statistically significant CCT variations during the day; therefore, only one CCT measurement is sufficient. Also, there was no statistically significant difference between ocular axial length of both groups.

Síndrome de Sturge-Weber: estudo ultrabiomicroscópico

PURPOSE: To describe the ultrasound biomicroscopy findings in patients with Sturge-Weber syndrome with or without glaucoma. METHODS: Ultrasound biomicroscopy was performed on 11 eyes from 8 patients with Sturge-Weber syndrome with or without glaucoma. RESULTS: Ultrasound biomicroscopy showed images suggestive of dilated intrascleral vessels in all eyes with or without glaucoma. Supraciliar effusion was found in 8 (88.9%) of 9 eyes with glaucoma and in none of those without glaucoma. Sectorial pars plana detachment was present in one eye with and in two eyes without glaucoma. A round image with low to moderate echogenicity in the ciliary body was present in 8 (88.9%) eyes with glaucoma and in 2 eyes without glaucoma. This image is highly suggestive of a dilated vessel or angioma. CONCLUSIONS: UBM showed paradoxically supraciliary effusion in the great majority of the eyes with glaucoma (89%), controlled by previous surgery in the Sturge-Weber syndrome. The images found in the anterior portion of sclera and in the ciliary body are highly suggestive of a scleral dilated vessel and an angioma, respectively, in eyes with or without glaucoma. These findings associated with increased episcleral venous pressure might contribute to explain not only the pathogenesis of the secondary glaucoma but also the peri- and postoperative complications eventually present during and after the antiglaucomatous surgeries in these eyes.