OBJECTIVE: To report on the outcome of subsequent interventions after failed initial intervention for primary congenital glaucoma (PCG).
METHODS: Retrospective chart review of children presenting with PCG and failed the initial glaucoma surgery in Alexandria Main University Hospital from 2005 to 2017. The data included demographics, preoperative, operative and postoperative clinical characteristics. Success was defined as IOP<16mmHg and <20% from the presenting IOP and C/D ratio like or better than presentation.
RESULTS: The charts of 531 (260 right, 49%) eyes of 360 (224 males, 62%) children presenting with PCG and failed the initial glaucoma surgery (58 [31 right, 53%] eyes [10.9%] of 50 [28 males, 56%] children) revealed that at initial presentation, the mean±standard deviation (range, median) of the age of the children subjected to one successful glaucoma procedure and the study children was 8.6±11.7 (0.6-109, 5.2) and 4.9±6.1 (0.5-34.4, 3.0) months, respectively. Each of the study eyes was subjected to 2.6±0.8 (2-5.2) glaucoma procedures. The mean±standard deviation (range, median) duration between the initial and second glaucoma surgery and the duration of follow-up was 6.9±7.8 (0.7-39,3.9) and 61.5±32.1 (12.0-139.1, 60.1) months, respectively. At the final follow-up visit success was reported in 41 (70.1%) eyes by IOP<16mmHg criterion, 39 (67.2%) eyes by IOP>20% reduction criterion and in 28 (out of 35 eyes, 80.0%) by the optic nerve condition (C/D ratio) criterion. Success by all 3 criteria was reported in 25 (out of 35 eyes, 71%) eyes.
CONCLUSIONS: A repeat glaucoma surgical procedure maybe needed in up to 11% of operated PCG eyes, with the subsequent glaucoma surgical procedures being successful by about 70%.

OBJECTIVE: To examine the reproducibility of spectral domain optical coherence tomography (SD-OCT) segmented ganglion cell complex and circumpapillary retinal nerve fiber layer (cpRNFL) measurements in children with primary congenital glaucoma (PCG) in comparison with healthy children.
METHODS: 12 children with PCG (G1) and 24 healthy children (G2) were recruited. The following SD-OCT measurements (Spectralis, Heidelberg Engineering) were made in one eye per child: total macular thickness (MT), thicknesses in several subfields and volumes of the three inner macular layers, macular retinal nerve fiber layer (mRNFL), ganglion cell layer (GCL), and inner plexiform layer (IPL) and cpRNFL thickness. In a single day, an expert operator obtained 3 circumpapillary and 3 macular measurements in each participant to determine intraoperator variability. Intraoperator repeatability was defined by the coefficient of variation (CoV) and intraclass correlation (ICC).
RESULTS: ICC was excellent in both groups for cpRNFL measurements (G1 ICC=0.950 and G2 ICC=0.995) and for MT was excellent in G1 (ICC=0.957) and moderate in G2 (ICC=552). For the inner macular layer measurements, all ICCs were better in PCG group (mRNFL-ICC: 0.915 vs. 0.765; ICC-GCL: 0.584 vs. 0.263 and ICC-IPL: 0.979 vs. 0.742; G1 and G2 respectively). Greater CoV were recorded for macular measurements (from 0.71% to 9.82%) compared to cpRNFL measurements (from 0.52% to 1.50%).
CONCLUSIONS: In children with PCG, Spectralis SD-OCT showed excellent intrasession repeatability for cpRNFL, MT, mRNFL and IPL measurements and moderated for GCL measurements. For all macular measurements, ICC were higher in children with PCG than healthy children.

OBJECTIVE: To compare corneal densitometry and topography variables in patients with primary congenital glaucoma (PCG) and healthy subjects.
METHODS: Cross sectional study, consecutive recruitment with gender- and age-matched control group. Forty eyes of 40 patients in each group were studied with Pentacam corneal topography. The variables compared between the two groups were: intraocular pressure (IOP), visual acuity (VA) and Pentacam (Oculus, Wetzlar, Germany) corneal topography measurements: mean and maximum keratometry (Km, Kmax), cylinder (Cyl), anterior elevation apex (AEA), central anterior elevation (CAE), maximum anterior elevation (MAE), posterior elevation apex (PEA), central posterior elevation (CPE), maximum posterior elevation (MPE), pachymetry and anterior, mid-stromal and posterior corneal densitometry in the 0-2mm, 2-6mm, 6-10mm zones.
RESULTS: Significant differences between patients and healthy controls were detected in the topographic variables MAE (P=0.002) and MPE (P<0.001), and in all the densitometry variables (anterior, mid-stromal, posterior for the 0-2mm, 2-6mm and 6-10mm zones) (P<0.001 each). In the PCG group, negative correlation was observed between VA and total densitometry (r=-0.49; P=0.004).
CONCLUSIONS: Patients with PCG and healthy subjects display differences in corneal densitometry and topographic measurements. PCG patients show greater corneal density with an inverse relationship between visual acuity and higher elevation (anterior and posterior values).

OBJECTIVE: To study Haab striae in adults with primary congenital glaucoma and describe them by anterior segment optical coherence tomography, as well as analyze their evolution over the patient\'s lifetime.
METHODS: Three patients with primary congenital glaucoma were recruited, aged between 17 and 48 years old, with incidentally noted Haab striae on glaucoma consultation. They were studied by: slit lamp; anterior segment optical coherence tomography, Heidelberg Engineering Inc., Germany, (with anterior segment module) and Cirrus HD-OCT, Carl Zeiss Meditec, Dublin, California, USA (with 3D reconstruction); and specular biomicroscopy. The results obtained were contrasted with other studies performed in children.
RESULTS: Rounded excrescences protruding towards the anterior chamber were found, as well as dense, irregular tissue between them. In addition, endothelial abnormalities (polymegathism and polymorphism) were found. These findings fit the evolution of those obtained in pediatric studies and remain stable in adult life.
CONCLUSIONS: Through noninvasive methods such as optical coherence tomography and specular biomicroscopy, it is possible to make the diagnosis and follow up these patients. The findings suggest posterior corneal topographic changes; additional studies on this subject may be necessary.