Dati normativi dei parametri tomografici OCT Spectral Domain in eta pediatrica.

RIASSUNTO Obiettivo: Riportare i valori normali dello spessore maculare, del volume maculare e dello strato delle fibre nervose (RNFL) dei bambini utilizzando un OCT a dominio spettrale. Metodi: Studio descrittivo in una popolazione preliminare di 94 bambini di età compresa tra i 5 e i 18 anni con anamnesi negativa per patologie sistemiche e locali e con equivalente sferico medio di 0,33 D±1,41 (range ±4 D, astigmatismo <1D). Sono state eseguite scansioni per lo studio maculare e per lo studio del nervo ottico. I dati sono stati analizzati in relazione a età, sesso ed equivalente sferico. Risultati: Valutando i dati in due gruppi (occhio destro e occhio sinistro) non sono state rilevate differenze significative per nessuno dei parametri analizzati (t-test: campioni indipendenti tutti i p>0,17) per cui si è scelto di analizzare l'occhio destro di tutti i pazienti. Lo spessore maculare centrale medio è risultato 274,97±18,28 micron, il volume retinico centrale medio 0,22±0,15 mm3, RFNL inferiore 124,11±16,49 μ, RNFL superiore 126,42±15,78 μ, RNFL nasale 69,96±13,19 μ e RNFL temporale 75,97±11,88 μ. Si è registrata una differenza significativa tra lo spessore maculare centrale e il sesso (t(92)= -2,65; p=0,009) e una correlazione lineare tra lo spessore maculare centrale e l'età dei pazienti (rho=0,37; p<0,001). Conclusioni: Lo studio riporta i valori standard di riferimento per lo spessore maculare, il volume maculare e l'RNFL in età pediatrica

Late spontaneous resolution of a double anterior chamber post deep anterior lamellar keratoplasty

A 31-year-old healthy male underwent deep anterior lamellar keratoplasty with big-bubble technique for treatment of keratoconus in his right eye. One week after surgery, he presented with detachment of the endothelium-Descemet complex with formation of a double anterior chamber, despite the apparent absence of an intraoperative Descemet membrane rupture. A subsequent intervention with the intent to relocate the corneal graft button was not effective, because the detachment appeared again one day later. The authors hypothesized that, at the time of the stromal dissection with big bubble technique, a small amount of air penetrated into the anterior chamber, creating a false pathway through the trabecular meshwork. The aqueous humor then penetrated the graft flowing through the false pathway, causing the endothelium-Descemet detachment. The persistence of that pathway, even after the intervention of graft repositioning, caused the failure of the latter procedure and persistence of the double chamber. We decided to wait and observe. The double anterior chamber spontaneously resolved in approximately three months

Amniotic membrane application in complex cases of penetrating keratoplasty surgery

INTRODUCTION. The aim of the study is to investigate the ability of an amniotic membrane implant combined with penetrating keratoplasty to reduce early and mid-term complications in complex cases such as penetrating burns, infective ulcers, keratitis, or corneal graft failure. MATERIALS AND METHODS. Fifty patients: 12 with keratitis, 24 with infective ulcers, 10 with penetrating trauma, and four with a history of corneal graft failure, were divided into two groups. The first group of 25 patients (Group A) underwent penetrating keratoplasty alone, while the second group of 25 patients (Group B) received penetrating keratoplasty associated with an amniotic membrane implant. Amniotic membrane implantation was performed in a ‘patch modality’, and the membrane was sewn to the graft with the epithelium/basement membrane side facing inwards. All patients were evaluated, respectively, 3, 15, 30, 90, and 180 days after surgery. At each visit a slit-lamp examination was performed together with corneal thickness and endothelial cell count assessment. All the data were subjected to statistical analysis with Student’s t-test. RESULTS. At the slit-lamp examination in Group A 48% of patients showed Descemet folds at one and three months, respectively, while 80% of patients of Group B did not show any Descemet folds. In Group A we registered two cases of early graft failure and two cases of shallow anterior chamber, while none of these complications appeared in Group B. In Group A the average corneal thickness at 15, 30, 90, and 180 days post-operatively was, respectively, 695 ± 43 μm, 658 ± 31 μm, 588 ± 12 μm, and 518 ± 20 μm, while in Group B it was found to be, respectively, 667 ± 12 μm, 632 ± 17 μm, 562 ± 16 μm, and 516 ± 10 μm. Differences in corneal thickness between Group A and Group B were found to be statistically significant (p = 0.05). Endothelial cell count was in Group A 2582 c/mm2 at 15 days, 2500 c/mm2 at one month, 2335 c/mm2 at three months, and 2111 c/mm2 at six months, while the average count for Group B patients was 2607 c/mm2, 2503 c/mm2, 2299 c/mm2, and 2086 c/mm2, respectively. Differences in endothelial cell count between the two groups did not show any statistical significance (p &gt; 0.05). CONCLUSIONS. Amniotic membrane implantation associated with penetrating keratoplasty reduces early and mid-term complications of corneal grafts in patients with high risk of failure. This positive effect may be due to the anti-inflammatory, neurotrophic, and anti-angiogenetic properties of the amniotic membrane. Longer observation and larger case series can be useful in evaluation of the functional outcome of grafts in these patients

A new counterintuitive training for adult amblyopia

International audienceObjectives: The aim of this study was to investigate whether short-term inverse occlusion, combined with moderate physical exercise, could promote the recovery of visual acuity and stereopsis in a group of adult anisometropic amblyopes. Methods: Ten adult anisometropic patients underwent six brief (2 h) training sessions over a period of 4 weeks. Each training session consisted in the occlu-sion of the amblyopic eye combined with physical exercise (intermittent cycling on a stationary bike). Visual acuity (measured with ETDRS charts), stereoacuity (measured with the TNO test), and sensory eye dominance (measured with binocular rivalry) were tested before and after each training session, as well as in follow-up visits performed 1 month, 3 months, and 1 year after the end of the training. Results: After six brief (2 h) training sessions, visual acuity improved in all 10 patients (0.15 AE 0.02 LogMar), and six of them also recovered stereopsis. The improvement was preserved for up to 1 year after training. A pilot experiment suggested that physical activity might play an important role for the recovery of visual acuity and stereopsis. Conclusions: Our results suggest a noninvasive training strategy for adult human amblyopia based on an inverse-occlusion procedure combined with physical exercise

Amniotic membrane application in complex cases of penetrating keratoplasty surgery

INTRODUCTION. The aim of the study is to investigate the ability of an amniotic membrane implant combined with penetrating keratoplasty to reduce early and mid-term complications in complex cases such as penetrating burns, infective ulcers, keratitis, or corneal graft failure. MATERIALS AND METHODS. Fifty patients: 12 with keratitis, 24 with infective ulcers, 10 with penetrating trauma, and four with a history of corneal graft failure, were divided into two groups. The first group of 25 patients (Group A) underwent penetrating keratoplasty alone, while the second group of 25 patients (Group B) received penetrating keratoplasty associated with an amniotic membrane implant. Amniotic membrane implantation was performed in a 'patch modality', and the membrane was sewn to the graft with the epithelium/basement membrane side facing inwards. All patients were evaluated, respectively, 3, 15, 30, 90, and 180 days after surgery. At each visit a slit-lamp examination was performed together with corneal thickness and endothelial cell count assessment. All the data were subjected to statistical analysis with Student's t-test. RESULTS. At the slit-lamp examination in Group A 48% of patients showed Descemet folds at one and three months, respectively, while 80% of patients of Group B did not show any Descemet folds. In Group A we registered two cases of early graft failure and two cases of shallow anterior chamber, while none of these complications appeared in Group B. In Group A the average corneal thickness at 15, 30, 90, and 180 days post-operatively was, respectively, 695 ± 43 μm, 658 ± 31 μm, 588 ± 12 μm, and 518 ± 20 μm, while in Group B it was found to be, respectively, 667 ± 12 μm, 632 ± 17 μm, 562 ± 16 μm, and 516 ± 10 μm. Differences in corneal thickness between Group A and Group B were found to be statistically significant (p = 0.05). Endothelial cell count was in Group A 2582 c/mm2 at 15 days, 2500 c/mm2 at one month, 2335 c/mm2 at three months, and 2111 c/mm2 at six months, while the average count for Group B patients was 2607 c/mm2, 2503 c/mm2, 2299 c/mm2, and 2086 c/mm2, respectively. Differences in endothelial cell count between the two groups did not show any statistical significance (p > 0.05). CONCLUSIONS. Amniotic membrane implantation associated with penetrating keratoplasty reduces early and mid-term complications of corneal grafts in patients with high risk of failure. This positive effect may be due to the anti-inflammatory, neurotrophic, and anti-angiogenetic properties of the amniotic membrane. Longer observation and larger case series can be useful in evaluation of the functional outcome of grafts in these patients

Benefits of glucocorticoids in non-ambulant boys/men with Duchenne muscular dystrophy: A multicentric longitudinal study using the Performance of Upper Limb test

The aim of this study was to establish the possible effect of glucocorticoid treatment on upper limb function in a cohort of 91 non-ambulant DMD boys and adults of age between 11 and 26 years. All 91 were assessed using the Performance of Upper Limb test. Forty-eight were still on glucocorticoid after loss of ambulation, 25 stopped steroids at the time they lost ambulation and 18 were GC naive or had steroids while ambulant for less than a year. At baseline the total scores ranged between 0 and 74 (mean 41.20). The mean total scores were 47.92 in the glucocorticoid group, 36 in those who stopped at loss of ambulation and 30.5 in the naive group (p <0.001). The 12-month changes ranged between -20 and 4 (mean -4.4). The mean changes were -3.79 in the glucocorticoid group, -5.52 in those who stopped at loss of ambulation and -4.44 in the naive group. This was more obvious in the patients between 12 and 18 years and at shoulder and elbow levels. Our findings suggest that continuing glucocorticoids throughout teenage years and adulthood after loss of ambulation appears to have a beneficial effect on upper limb function. (C) 2015 The Authors. Published by Elsevier B.V

Health-related quality of life and functional changes in DMD: A 12-month longitudinal cohort study

In Duchenne muscular dystrophy (DMD) little has been reported on the association between clinical outcome measures and patient health-related quality of life (HRQOL) tools. Our study evaluated the relationship between 12 month changes on the Generic Core Scales (GCS), the Multidimensional Fatigue Scale and the Neuromuscular Module of the PedsQL\u2122 with several outcome measures (6 minute walk test, North Star Ambulatory Assessment and timed items) in ambulatory DMD. Ninety-eight ambulatory DMD in a multicentric setting were included in the study. At baseline, the PedsQL\u2122 inventories correlated with almost all the functional measures On the Child Self-Report there was a significant decrease between baseline and 12 months on the PedsQL\u2122 GCS and its first domain, in parallel with the decrement in the functional outcome measures. Correlation between the 12 month changes on the PedsQL\u2122 inventories and functional measures were almost all negligible. Similar results were obtained on the Parent Proxy-Report.In conclusion, PedsQL\u2122 correlates with the level of impairment at baseline, but this does not hold true when 12 month changes are considered. Further studies comparing different tools are needed to better elucidate the complexity of the relationship between HRQOL and functional performances

Reliability of the Performance of Upper Limb assessment in Duchenne muscular dystrophy

Abstract The Performance of Upper Limb was specifically designed to assess upper limb function in Duchenne muscular dystrophy. The aim of this study was to assess (1) a cohort of typically developing children from the age of 3 years onwards in order to identify the age when the activities assessed in the individual items are consistently achieved, and (2) a cohort of 322 Duchenne children and young adults to establish the range of findings at different ages. We collected normative data for the scale validation on 277 typically developing subjects from 3 to 25 years old. A full score was consistently achieved by the age of 5 years. In the Duchenne cohort there was early involvement of the proximal muscles and a proximal to distal progressive involvement. The scale was capable of measuring small distal movements, related to activities of daily living, even in the oldest and weakest patients. Our data suggest that the assessment can be reliably used in both ambulant and non ambulant Duchenne patients in a multicentric setting and could therefore be considered as an outcome measure for future trials

Surgical management of dysthyroid strabismus through muscular weakening procedures with adjustable sutures

Background Autoimmune dysthyroidism may result in a form of restrictive strabismus characterized by a severe reduction of ocular motility and patient’s quality of life. In case of diplopia not manageable with prisms and/or in case of abnormal head position (AHP) not tolerated by patient, surgical treatment is the gold standard procedure. Methods Prospective, monocentric study. All enrolled patients presented a stable thyroidal disease from at least 6 month and were subjected to muscular recession associated with adjustable sutures. Primary endpoints of this study were subjective diplopia (evaluated with Gorman Score), residual deviation (measured in prismatic diopters, PD), field of single binocular vision (evaluated with Sullivan Score) and patients’ quality of life (evaluated with GO-QoL questionnaire). This study also investigated the influence of previous GO treatments (corticosteroids, radiotherapy and orbital decompression) on the baseline deviation and on final results. Results Forty-seven patients (31 females and 16 males with a mean age of 55,91±8,73 years) have been enrolled. Scheduled visits have been performed at baseline, 1 day after surgery, on month, 6 4 and 12 months. All patients completed one year follow-up. At baseline visit all patients presented constant diplopia (Gorman score = 1) with a mean total deviation of 36,70±14,69 PD in primary gaze, a 27,08±16,99 PD mean horizontal deviation and a 23,67±12,01 PD mean vertical deviation. One year after surgery only 2,27% of patients referred constant diplopia not manageable with prisms. Mean residual total deviation at 12 months after surgery was 3,66±6,42 PD in primary gaze while mean residual horizontal deviation was 1,89 ± 4,53 PD and mean residual vertical deviation was 3,47±5,49 PD. Field of binocular single vision expanded from 31,89± 6,67% to 83,7±3,99% while GO-QoL improved from 20,91± 4,50% at baseline to 89,22±3,22 % at one year visit. Previous CS therapy demonstrated a positive effect on vertical deviation both for baseline and final residual deviation. RT demonstrated a good influence on residual final vertical deviation; this result was better when OTD was also performed. Conclusions Muscular weakening with adjustable sutures represents a safe and an effective procedure for dysthyroid strabismus correction and usually determines a significant improvement in patients’ quality of life. Corticosteroids and Radiotherapy have a positive effect on residual deviation at one year followup especially for vertical deviation