Early corneal and optic nerve changes in a paediatric population affected by obstructive sleep apnea syndrome.

PURPOSE: The relation between OSAS and eye diseases is well known in adults, while very few and contradictory data can be found regarding paediatric ages. The aim of this study is to explore the early corneal, macular and optic nerve changes in paediatric patients with OSAS. METHODS: Prospective study that enrolled children aged ≥ 4 years referred to the Paediatric Pneumology Clinic in Verona for suspected obstructive sleep apnoea syndrome (OSAS) and investigated with the overnight respiratory polygraphy. Patients with apnoea-hypopnea index (AHI) > 1 were classified as OSAS, while those with AHI < 1 were classified non-OSAS. All patients underwent comprehensive eye examination including slit lamp, refraction, intraocular pression (Goldman applanation tonometry), corneal tomography (corneal astigmatism, corneal keratometry at the apex, surface asymmetry index, central corneal thickness and thinnest corneal thickness) and optical coherence tomography (central macular thickness, macular volume and retinal nerve fibre layer). RESULTS: Seventy-two children were enrolled in the study. The overall prevalence of OSAS was 48.6%. Statistically significant differences were found between OSAS and non-OSAS group for corneal asymmetry (0.9 ± 0.5 and 0.6 ± 0.3, respectively; p = 0.02), thinnest corneal thickness (551.8 ± 33.9 and 563.7 ± 32.5; p = 0.04), average retinal nerve fibre layer (102.8 ± 10.5 µm and 98.1 ± 12.3 µm; p = 0.012) and in nasal quadrant (76.2 ± 15.4 µm and 66.5 ± 12.6 µm; p = 0.0002). CONCLUSIONS: A comprehensive eye examination with corneal and optic nerve imaging showed early corneal and optic nerve changes in children newly diagnosed with OSAS. These could be prelude of the known ocular manifestations associated with OSAS in adult patients.

In vivo confocal microscopy study of corneal nerve alterations in children and youths with Type 1 diabetes.

OBJECTIVE: To determine whether children and youths with Type 1 diabetes (T1D) have early alterations of the corneal subbasal nerve plexus detectable with in vivo confocal microscopy (IVCM) and to investigate the role of longitudinally measured major risk factors for diabetes complications associated with these alterations. METHODS: One hundred and fifty children and youths with T1D and 51 age-matched controls were enrolled and underwent IVCM. Corneal nerve fiber length (CNFL), corneal nerve fiber density (CNFD), corneal nerve branch density (CNBD), corneal fiber total branch density (CTBD), and corneal fiber fractal dimension (CNFrD) were measured. Risk factors for diabetes complications (blood pressure, BMI, HbA1c, lipoproteins, urinary albumin-creatinine ratio) were recorded at IVCM and longitudinally since T1D onset. Unpaired t-test was used to compare variables between the groups. Multiple regression models were calculated using IVCM parameters as dependent variables and risk factors as independent variables. RESULTS: All IVCM parameters, except CTBD, were significantly lower in the T1D patients. Glycometabolic control (HbA1c, visit-to-visit HbA1c variability, and mean HbA1c), and blood pressure were inversely correlated with IVCM parameters. Multiple regression showed that part of the variability in CNFL, CNFD, CTBD, and CNFraD was explained by HbA1c, blood pressure percentiles and age at IVCM examination, independent of diabetes duration, BMI percentile and LDL cholesterol. Comparable results were obtained using the mean value of risk factors measured longitudinally since T1D onset. CONCLUSIONS: Early signs of corneal nerve degeneration were found in children and youths with T1D. Glycometabolic control and blood pressure were the major risk factors for these alterations.

Small-incision lenticule addition in ex vivo model of ectatic human corneas.

PURPOSE: To investigate the feasibility of intrastromal lenticule insertion to restore corneal shape in a model of ectatic human cornea. METHODS: For this experimental ex vivo study on 34 human corneas unsuitable for transplantation, 17 corneas were thinned by decentralized posterior excimer laser ablation to 200 µm thickness and 6.5 mm diameter and then inflated up to 100 mm Hg to expose the ectasias (recipient corneas). Pachimetry and topography were obtained. Stromal lenticules of the same diameter and thickness as the ectasias were shaped with a femtosecond laser from the remaining 17 donor corneas. An intrastromal pocket was created with femtosecond laser within the ectatic recipient corneas and the donor lenticule was inserted inside it. Changes in corneal architecture and profile were evaluated by means of corneal topography and anterior segment optical coherence tomography. RESULTS: All stromal lenticules were successfully implanted. Tomography confirmed regularity of the lenticule profile within the stromal pocket. Corneal thickness was significantly increased after the procedure (P < 0.0001). Maximal posterior elevation from the best-fitted toric ellipsoid was significantly reduced (P < 0.0001). Significant flattening of posterior K1 and K2 was also obtained (P = 0.041 and P = 0.004, respectively). Anterior and posterior astigmatism, anterior and posterior asphericity, and spherical aberration did not differ significantly after the procedure. CONCLUSIONS: Femtosecond laser-assisted stromal lenticule addition is feasible for restoring corneal thickness to an ectatic area and for regularizing posterior corneal elevation. The technique opens new perspectives for the treatment of corneal ectasias.

Efficacy of plasmapheresis as treatment for bilateral hyperviscosity syndrome related retinopathy in multiple myeloma.

PURPOSE: To report a case of good clinical response to plasmapheresis as therapy for a bilateral hyperviscosity syndrome related retinopathy in a young patient with undiagnosed multiple myeloma (MM). METHODS: Case report. RESULTS: A 48-year-old caucasian man, previously diagnosed with monoclonal gammopathy of undetermined significance (MGUS), presented for medical attention for back ache and vision decrease, worst in the left eye. Ophthalmological examination revealed presence of bilateral CRVO-like retinopathy which raised the hypothesis of hyperviscosity syndrome (HVS). After confirmation of MM diagnosis, plasmapheresis were promptly begun. The right eye regained a good visual acuity and an anatomical restoration already notable 5 days from the first plasmapheresis. CONCLUSIONS: Fundoscopy lead to diagnosis of HVS, it is therefore advisable in all patients with MM in order to perform plasmapheresis as soon as possible if necessary. In case of atypical CRVO, haematological and inflammatory causes, should be always ruled out.

In Vivo Confocal Microscopy of the Corneal-Conjunctival Transition in the Evaluation of Epithelial Renewal after SLET.

Examination of the corneal surface by in vivo confocal microscopy (IVCM) allows for objective identification of corneal and conjunctival cell phenotypes to evaluate different epithelialization patterns. Detection of a corneal-conjunctival epithelial transition could be considered as a sign of restored epithelial function following simple limbal epithelial transplantation (SLET). This is a prospective, interventional case series. We assessed patients with limbal stem cell deficiency (LSCD) by IVCM, preoperatively and at monthly intervals following SLET. Sectors in the central and peripheral cornea were scanned. Immediately upon detection of multi-layered cells with the epithelial phenotype in the central cornea and confirmation of epithelial transition in all corneal sectors, the decision for keratoplasty was taken. Ten patients were enrolled. After SLET, epithelial phenotype in the central cornea and an epithelial transition were identified within six and nine months in seven and one patients, respectively. One patient was a partial success and one failed. Five patients underwent keratoplasty, with stable results up to 12 months. Identification of the epithelial transition zone by IVCM permits assessment of the efficacy of SLET, enabling subsequent planning of keratoplasty for visual rehabilitation. The stability of the corneal surface following keratoplasty confirms that the renewal of the corneal epithelium was effectively retained.

Reduced minimum rim width of optic nerve head: a potential early marker of retinal neurodegeneration in children and adolescents with type 1 diabetes.

AIMS: To determine whether early retinal neurodegenerative changes in pediatric patients with type 1 diabetes (T1D) can be detected by spectral domain-optical coherence tomography (SD-OCT) and whether such changes are associated with risk factors for T1D complications. METHODS: A total of 147 T1D children/adolescents and 51 healthy controls underwent SD-OCT. Spherical refractive error (SRE), macular total retinal thickness (TRT), ganglion cell layer (GCL), retinal nerve fiber layer (RNFL), minimum rim width (MRW), and Bruch's membrane opening area (BMOA) were measured. Clinical and biochemical parameters were recorded at the time of SD-OCT and starting at T1D onset. Multiple regression models were calculated using SD-OCT parameters as dependent and risk factors as independent variables. RESULTS: MRW was significantly thinner in the T1D patients (global MRW:361.58vs386.33 µm; p = 0.009), while RNFL and macular parameters were similar for both groups. MRW was inversely correlated with mean HbA1c (r ≥ -0.180, p < 0.05). Multiple regression showed that part of the variability in MRW was explained by HbA1c and BMOA (R2 = 0.21; p < 0.001), independent of other cardiometabolic risk factors. CONCLUSIONS: MRW reduction could be a potential early marker of retinal neurodegeneration detectable in pediatric patients with T1D. The association between MRW and mean HbA1c suggests that glucometabolic control may affect early retinal neurodegeneration starting in childhood.

In vivo confocal microscopy of verticillata-like paraproteinemic keratopathy in a patient with monoclonal gammopathy of uncertain significance evolving into smoldering multiple myeloma.

PURPOSE: To highlight the utility of in vivo confocal microscopy (IVCM) in the microstructural characterization of corneal deposits resembling vortex keratopathy in a case of secondary deposition keratopathy due to an evolving monoclonal gammopathy. OBSERVATIONS: A 56-year-old Caucasian woman previously diagnosed with monoclonal gammopathy of undetermined significance (MGUS), exhibited bilateral diffuse sub-epithelial and anterior stromal opacities distributed in a whorl-shaped pattern. IVCM examination permitted analysis of the microstructural features and distribution of the opacities in different corneal layers. Deposits resembling those reported in multiple myeloma rather than MGUS were detected, and the patient's hematologist was subsequently advised to re-evaluate the earlier diagnosis. Two months later, a bone marrow biopsy led to a diagnosis of plasma cell myeloma. CONCLUSIONS AND IMPORTANCE: It is suggested that cornea verticillata-like paraproteinemic keratopathy (PPK) in MGUS might be predictive of disease evolution to plasma cell myeloma. Characteristic deposits and morphological features revealed by IVCM may be helpful for non-invasive assessment or screening in patients with hematological disorders.

SD-OCT Choroidal Thickness in Advanced Primary Open-Angle Glaucoma.

PURPOSE: To assess the choroidal thickness in advanced primary open-angle glaucoma (POAG) comparing patients affected by advanced glaucoma and normal subject using spectral domain optical coherence tomography (SD-OCT). METHODS: In total, 35 eyes of 35 patients affected by POAG with advanced perimetric defect (mean deviation worse than -12 dB) were prospectively enrolled and underwent a complete ophthalmologic examination, including enhanced depth imaging SD-OCT. One eye of 35 healthy subjects served as control group. Choroidal thickness was measured at the subfoveal location, at 0.5, 1.0, and 2.0 mm nasal and temporal to the fovea. Primary outcome measure was the identification of different choroidal thickness between advanced glaucoma patients and normal subjects. RESULTS: Glaucoma and control group were homogenous for age, sex, and axial length (P>0.05 in all cases). A significant difference was found in the subfoveal choroidal thickness (SFCT) adjusted for age and axial length between glaucoma and control eyes (P=0.042); in glaucoma patients the mean adjusted SFCT was 209.90 µm compared with 234.78 µm in control subjects. Both temporal and nasal choroidal thickness measurements followed the same SFCT trend, even if only temporal ones were statistically significant. We also found that SFCT decreased with age and increased axial length (P=0.007 and 0.001, respectively). Sex and intraocular pressure did not significantly influence the choroidal thickness (P=0.87 and 0.35, respectively). CONCLUSIONS: Patients affected by advanced POAG damage have a thinner choroidal thickness compared with normal subjects, using SD-OCT. We also confirmed that age and axial length were the main factors affecting choroidal thickness in these patients.