Long-term anatomical and functional outcomes of idiopathic macular hole surgery. The yield of spectral-domain OCT combined with microperimetry.

BACKGROUND: To evaluate the correlations between anatomical and functional changes studied with microperimetry (MPM) and spectral-domain OCT (SD-OCT) in patients after successful repair of idiopathic macular hole (MH). METHODS: Monocentric, retrospective, interventional study in 23 eyes of 23 patients who underwent successful surgery for MH defined as closure of the hole, at least 1 year before. Reported data were pre- and postoperative best-corrected visual acuity (BCVA), retinal sensitivity values on MPM, macular and foveal thicknesses, and retinal anatomic lesions on SD-OCT. RESULTS: Macular sensitivity (MS) and foveal sensitivity (FS) were lower and the number of lesions of the outer retinal layers was higher in patients with a poorer postoperative VA (P = 0.029, P = 0.011 and P = 0.003 respectively). Preoperative MH size was lower and MS and FS were better in patients with a preserved junction line between the inner and outer segments of photoreceptors (IS/OS) (P = 0.045, P = 0.001, and P = 0.001 respectively). Better postoperative VA was correlated with better preoperative VA (P = 0.012, r = 0.513). Postoperative VA was correlated with MS and FS (P = 0.032, r = 0.449, and P = 0.019, r = 0.483 respectively). Greater foveal thickness was associated with better postoperative VA (P = 0.020, r = 0.482). CONCLUSION: Postoperative outer retinal layer integrity is associated with better final retinal sensitivity. Further studies are warranted to assess the role of SD-OCT and microperimetry in the pre- and postoperative evaluation of idiopathic macular holes.

Impaired cortical processing of inspiratory loads in children with chronic respiratory defects.

BACKGROUND: Inspiratory occlusion evoked cortical potentials (the respiratory related-evoked potentials, RREPs) bear witness of the processing of changes in respiratory mechanics by the brain. Their impairment in children having suffered near-fatal asthma supports the hypothesis that relates asthma severity with the ability of the patients to perceive respiratory changes. It is not known whether or not chronic respiratory defects are associated with an alteration in brain processing of inspiratory loads. The aim of the present study was to compare the presence, the latencies and the amplitudes of the P1, N1, P2, and N2 components of the RREPs in children with chronic lung or neuromuscular disease. METHODS: RREPs were recorded in patients with stable asthma (n = 21), cystic fibrosis (n = 32), and neuromuscular disease (n = 16) and in healthy controls (n = 11). RESULTS: The 4 RREP components were significantly less frequently observed in the 3 groups of patients than in the controls. Within the patient groups, the N1 and the P2 components were significantly less frequently observed in the patients with asthma (16/21 for both components) and cystic fibrosis (20/32 and 14/32) than in the patients with neuromuscular disease (15/16 and 16/16). When present, the latencies and amplitudes of the 4 components were similar in the patients and controls. CONCLUSION: Chronic ventilatory defects in children are associated with an impaired cortical processing of afferent respiratory signals.

Respiratory muscle testing: a valuable tool for children with neuromuscular disorders.

RATIONALE: Data on respiratory muscle performance in children with neuromuscular disorders are limited. OBJECTIVES: The aim of this study was to assess respiratory muscle strength by volitional and nonvolitional tests and to compare these tests with forced vital capacity. METHODS: Inspiratory muscle strength was assessed by measuring transdiaphragmatic and esophageal pressures generated during volitional and nonvolitional maneuvers, whereas expiratory muscle strength was assessed by measuring the gastric pressure generated during a cough maneuver. Lung volumes were assessed by measuring forced vital capacity. MEASUREMENTS AND MAIN RESULTS: Forty-one patients with Duchenne muscular dystrophy (n = 20), spinal amyotrophy (n = 8), and congenital myopathy (n = 13) were included, aged 2 to 18 yr. All the patients were able to perform the sniff and the cough maneuver. Sniff transdiaphragmatic pressure decreased with age in Duchenne patients, whereas it increased with age in patients with spinal amyotrophy and congenital myopathy. Magnetic stimulation of the phrenic nerves was obtained in all patients. Twenty-five (61%) patients were able to perform forced vital capacity. In the three groups of patients, a positive correlation was observed between volitional, assessed by the sniff maneuver, and nonvolitional respiratory muscle tests, assessed by the magnetic stimulation of the phrenic nerves. Also, forced vital capacity correlated with sniff transdiaphragmatic pressure and cough gastric pressure. CONCLUSIONS: Volitional respiratory muscle tests correlated with nonvolitional tests and with forced vital capacity. Simple volitional respiratory muscle tests constitute a valuable tool for the assessment of respiratory muscle strength in young patients with neuromuscular disorders.

Mechanical limitation during CO2 rebreathing in young patients with cystic fibrosis.

The aim of the study was to determine whether a decrease in the ventilatory response to carbon dioxide (CO2) in children with cystic fibrosis (CF) is related to a mechanical limitation of the respiratory muscle capacity. The ventilatory response during CO2 rebreathing was performed in 15 patients (mean forced expiratory volume in 1 s (FEV1): 37 +/- 21% predicted, mean arterial CO2: 41+/- 5 mmHg). The slope of the minute ventilation normalised for weight per mmHg CO2 increment correlated negatively with respiratory muscle output, assessed by the oesophageal (p = 0.002), the diaphragmatic pressure time product (p = 0.01), and the tension time index (p = 0.005). In addition, this slope was correlated with dynamic lung compliance (p < 0.0001) and FEV1 (p = 0.03) but not with airway resistance and maximal transdiaphragmatic pressure. Therefore, an excessive load imposed on the respiratory muscles explains the blunting of the ventilatory response to CO2 in young patients with CF.

Facial side effects during noninvasive positive pressure ventilation in children.

OBJECTIVE: The study quantified the side effects of nasal masks use for noninvasive positive pressure ventilation (NPPV) in children. DESIGN AND SETTING: Cross-sectional retrospective study in a tertiary pediatric university hospital. PATIENTS: Patients with obstructive sleep apnea (n=16), neuromuscular disorders (n=14), and cystic fibrosis (n=10). INTERVENTIONS: Clinical evaluation of facial tolerance. MEASUREMENTS AND RESULTS: A skin injury was observed in 19 patients (48%), with a transient erythema in 7 (18%), prolonged erythema in 9 (23%), and skin necrosis in 3 (8%). Skin injury was associated with age over 10 years (OR=16) and use of a commercial mask (OR=15) and was less frequent in patients with obstructive sleep apnea. The change of a commercial mask for a custom-made mask was associated with reduction in the skin injury score. Global facial flattening was present in 68% of the patients. No correlation was observed with age, daily or cumulative use of NPPV, or the type of mask. A maxillary retrusion was present in 37% of patients. No correlation was observed with age or the type of mask or the underlying disease, but an association was found with a longer daily use of NPPV (OR=6.3). CONCLUSIONS: The prevalence of facial side effects is clinically significant in children using NPPV. Systematic maxillofacial follow-up enables these effects to be identified. Remedial measures could include the change of the interface or reducing the daily use of NPPV.

Noninvasive positive pressure ventilation in infants with upper airway obstruction: comparison of continuous and bilevel positive pressure.

OBJECTIVE: This study evaluated the efficacy of noninvasive continuous positive pressure (CPAP) ventilation in infants with severe upper airway obstruction and compared CPAP to bilevel positive airway pressure (BIPAP) ventilation. DESIGN AND SETTING: Prospective, randomized, controlled study in the pulmonary pediatric department of a university hospital. PATIENTS: Ten infants (median age 9.5 months, range 3-18) with laryngomalacia (n=5), tracheomalacia (n=3), tracheal hypoplasia (n=1), and Pierre Robin syndrome (n=1). INTERVENTIONS: Breathing pattern and respiratory effort were measured by esophageal and transdiaphragmatic pressure monitoring during spontaneous breathing, with or without CPAP and BIPAP ventilation. MEASUREMENTS AND RESULTS: Median respiratory rate decreased from 45 breaths/min (range 24-84) during spontaneous breathing to 29 (range 18-60) during CPAP ventilation. All indices of respiratory effort decreased significantly during CPAP ventilation compared to unassisted spontaneous breathing (median, range): esophageal pressure swing from 28 to 10 cmH(2)O (13-76 to 7-28), esophageal pressure time product from 695 to 143 cmH(2)O/s per minute (264-1417 to 98-469), diaphragmatic pressure time product from 845 to 195 cmH(2)O/s per minute (264-1417 to 159-1183) During BIPAP ventilation a similar decrease in respiratory effort was observed but with patient-ventilator asynchrony in all patients. CONCLUSIONS: This short-term study shows that noninvasive CPAP and BIPAP ventilation are associated with a significant and comparable decrease in respiratory effort in infants with upper airway obstruction. However, BIPAP ventilation was associated with patient-ventilator asynchrony.

Anatomical and functional macular changes after rhegmatogenous retinal detachment with macula off.

PURPOSE: To evaluate the correlation between morphologic changes in the outer retina and visual function after successful repair of rhegmatogenous retinal detachment with macula off. DESIGN: Observational case series. SETTINGS: Dijon University Hospital. PATIENTS: Thirty patients (30 eyes) with successful repair of rhegmatogenous retinal detachment after macula off and a minimum 6-month follow-up after surgery. MAIN OUTCOME MEASURES: Spectral-domain optical coherence tomography (SD-OCT) of the outer retina, fundus autofluorescence (FAF), and microperimetry. RESULTS: Twenty of 30 eyes presented microstructural changes within the photoreceptor layer (66.7%). Of these, half of the patients (50%) had more than 1 lesion. Disrupted inner segment/outer segment (IS/OS) junction was noted in 16 out of 30 eyes (53.3%), irregular hyporeflectivity in the photoreceptor outer segments (PROS) was observed in 17 eyes (56.7%), external limiting membrane was discontinued in 10 eyes (33.3%), and hyperreflective spots in the outer nuclear layer were observed in 5 eyes (16.7%). FAF changes were detected in only 5 eyes (16.7%). Abnormalities in the IS/OS junction were significantly associated with lower foveal and macular sensitivity, thinner PROS, and global photoreceptor changes (P = .014, P = .003, P = .006, P < .0001, respectively). Patients with a normal foveal profile showed similar findings. CONCLUSIONS: SD-OCT and microperimetry seem to be appropriate tools to determine the visual and the anatomic recovery of the macula after surgery.

Congenital diaphragm weakness without neuromuscular disease.

We report on two children presenting at birth with respiratory failure, bilateral diaphragmatic eventration, and floppiness. Electrodiagnostic studies of the limbs, and biochemical and DNA studies excluded generalized neuromuscular diseases. Phrenic nerve electrodiagnostic studies and electromyography of the diaphragm suggested isolated diaphragm hypoplasia. Diaphragm muscle biopsy showed a paucity of muscle fibers. Isolated hypoplasia of the diaphragm is a rare cause of neonatal respiratory failure, which may have a favorable outcome with long-term ventilatory support.