MR findings of the brain in children and adolescents with portal hypertension and the relationship with blood manganese levels.

BACKGROUND: Few studies have evaluated abnormalities on brain magnetic resonance imaging (MRI) in children and adolescents with chronic liver disease. AIMS: The aim of this study was to investigate the presence of T1 hyperintensity in the basal ganglia of pediatric patients with portal hypertension and its association with blood manganese levels. METHODS: A case control study of 22 patients with portal hypertension (14 Child-Pugh A cirrhosis, 8 non-cirrhotic portal hypertension) and 15 controls was conducted from 2006 to 2007. Blood manganese levels were measured using atomic absorption spectrophotometry. Brain MRI scans were performed using a 1.5 Tesla (Philips) scanner. RESULTS: Blood manganese levels were 26.01+/-12.82 microg/L for patients with portal hypertension (cirrhotic: 22.73+/-11.67 microg/L, non-cirrhotic: 32+/-13.32 microg/L) and 15.64+/-6.61 microg/L for controls (p=0.003). 14/22 patients with portal hypertension presented T1 hyperintensity in the basal ganglia [6/14 cirrhotic; 8/8 non-cirrhotic (p=0.018); zero controls (p=0.001)]. Mean blood manganese levels of patients with liver disease and normal vs. abnormal brain MRI scans were 18.45+/-8.38 microg/L and 30.47+/-13.07 microg/L, respectively (p=0.04). CONCLUSIONS: Brain MRI showed a high frequency (64%) of T1 hyperintensity in the basal ganglia of patients with portal hypertension, which correlated positively with blood manganese levels. This abnormality was found in 100% of the patients with portal hypertension and in 43% of those with mild cirrhotic disease.

[Evaluation of 3D imaging software to study computed tomography images of middle ear defects]. / Evaluation d'un logiciel de reconstruction 3D à l'étude TDM des malformations de l'oreille moyenne.

OBJECTIVE: Evaluate the contribution of original 3D reconstruction software based on the principle of region growing segmentation of CT images to diagnosis of ossicular malformations of the middle ear. METHOD: Prospective study on a population of ten patients presenting uni- or bilateral transmission deafness. Twenty ears were studied using multislice CT. All the data were treated using original software segmentation based on the principle of region growing segmentation. The 3D images were compared with the original CT slices to judge the software's potential contribution to the management of these patients. RESULTS: All the images obtained were deemed of sufficient diagnostic quality. The two normal ears on the CT slices were also found to be normal on the 3D images. Typical CT images of otospongiosis found in two patients were not visible on the 3D images. The 18 ossicular malformations were well analyzed on the images obtained, which also provided better visualization of the abnormalities and associated lesions (particularly the anomalies in facial nerve position), thus providing quality surgical planning as judged by the surgeon. CONCLUSION: The image reconstruction method studied, using region growing segmentation seems to be, in this limited sample, a useful complement to classical CT images in the study of constitutional abnormalities of the middle ear.

Contribution of CT-assisted navigation and microdebriders to endoscopic sinus surgery in cystic fibrosis.

OBJECTIVE: To demonstrate the interest of CT-assisted navigation and the microdebrider in rhino-sinus surgery for cystic fibrosis. METHODS: A retrospective study included 20 patients with cystic fibrosis who had undergone endoscopic sinus surgery using CT-assisted navigation and microdebrider between 1998 and 2006. RESULTS: Surgery was indicated for the following symptoms resistant to medical management: incapacitating nasal obstruction (n=18, 90%) and headache or periorbital pain (n=14, 70%). At 3 years follow-up, six patients (30%) were symptom-free, six (30%) had required re-operation, and symptoms were well controlled by medication in the other eight (40%). Mean initial bilateral surgery time was 61 min. There were no complications. CONCLUSION: The advent of microdebriders and CT-based navigation has improved endoscopic sinus surgery in cystic fibrosis. It has permitted the control of medication-resistant symptoms, especially nasal obstruction and pain. Precision is satisfactory, with reduced surgery time. No major complications have been observed. Two- or three-step surgery is possible where endoscopic anatomic landmarks have suffered alteration. The resultant control of symptoms encourages extending indications, with repeat procedures, in view of improving patients' quality of life. Further assessment on a larger series will be needed.

Balance in healthy individuals assessed with Equitest: maturation and normative data for children and young adults.

OBJECTIVE: The purpose of the present prospective study was to obtain normative data for Equitest computerized dynamic posturography (CDP) in children and young adults and to observe the balance maturation process. METHODS: Equilibrium pattern and SOT of 195 healthy children aged from 6 to 14 years were compared to those of 64 young adults aged 20 years, using Equitest. RESULTS: The mean stability scores for children progressively improve with age and are significantly lower than those of young adults. Furthermore, sensory organization scores were lower in children compared to young adults, whereas visual preference scores were similar for the whole studied groups. CONCLUSIONS: According to previous literature, it may be stressed that due to a presumed incomplete development of vestibular system and central nervous system integration, children unlike young adults have lower equilibrium scores, especially when visual information was not available or was incorrect. Our results confirm previous ones obtained in children and young adults with Equitest CDP. This study also provides Equitest data for these age groups, which complete those that have been previously reported.

Vestibular assessment with Balance Quest Normative data for children and young adults.

OBJECTIVE: The purpose of the present study was to compare equilibrium pattern in 12-year-old children with 20-year-old young adults and to obtain normative data for the BQ in both groups. METHODS: Mean stability percentages and synthesis ratios of 29 healthy children aged 12 years were compared to those of 68 young adults aged 20 years, using BQ. RESULTS: The mean stability percentages for children were significantly lower than for young adults. Vestibular ratios were lower in children compared to young adults, whereas somesthesic ratios were similar for the two groups. Visual dependence was significant higher in children. CONCLUSIONS: Children unlike young adults had lower stability percentages when visual information was not available or was incorrect. Ratio synthesis pattern was different in the two groups. Our results on BQ partially confirms previous results obtained in children assessed with Equitest CDP. This study also provides BQ normative data for these two age groups.

Tracheotomy in children: evolution in indications.

OBJECTIVES: To evaluate the evolution of indications for tracheostomy in children. METHODS: A retrospective review of 46 children undergoing tracheostomy between 1996 and 2001. RESULTS: The indications for tracheostomy were classified in two groups:(1) prolonged ventilator dependence (57%), (2) upper airway obstructions (43%). The average age of the tracheostomy was 3 years and 8 months. This was higher in the first group (5.5 years). Decannulation was accomplished in 52%. The indications for tracheostomy were evaluated and were found to decrease in frequency for upper airway obstructions. An increasing indication was for chronic disorders requiring prolonged ventilator dependence. Complications occurred in 50% of children. Overall mortality was 13-2.7% directly related to the tracheostomy. CONCLUSIONS: Evolving indication has been ventilator dependence. Upper airway obstruction as an indication has diminished in frequency, especially with the concomitant progress of endoscopic techniques.

[Aspirin hypersensitivity: characteristics and diagnostic approach]. / Intolérance à l'aspirine : caractéristiques et éléments diagnostiques.

INTRODUCTION: In routine medical practice, the diagnosis of aspirin hypersensitivity (AH) remains difficult. No clinical feature or biomarker is available to reliably confirm this diagnosis and oral provocation tests (OPT) are rarely performed. AIM: To compare asthmatics with and without AH. METHOD: The clinical characteristics of 21 asthmatics with and 24 without AH respectively were determined. AH was defined by a positive OPT. A full blood count was done before and 24 hours after the OPT. RESULTS: The medical history was associated with a weak sensitivity (52%) and a good specificity (96%) for assessing the diagnosis of AH. There was a higher prevalence of AH in women, and a higher frequency of allergic rhinitis in AH, but no characteristic was useful to facilitate the diagnosis of AH in asthmatic patients. Our results demonstrate higher values of platelets in AH patients. Following OPT, in AH patients only, a decrease in blood eosinophils and an increase in neutrophils was observed. CONCLUSIONS: These results confirm that the diagnosis of AH is challenging, with the history having only weak sensitivity. The observation that fluctuations in eosinophils and neutrophils occur following OPT in AH patients only warrants further investigations and suggests a rapid pro-inflammatory role for aspirin.

Variability of the influence of a visual task on the active micromechanical properties of the cochlea.

The effect of a visual task on the active micromechanical properties of the cochlea studied by the evoked otoacoustic emissions (EOAEs) has been the subject of only one published study (Brain Research, 44 (1988) 380-383). In order to examine the reliability of this effect, a similar study has been run on 16 subjects. A significant decrease in EOAEs during a visual task was obtained for 3 subjects. The two subjects whose decrease was the most significant were tested again one month later and the same effect was found. This striking interindividual variability is discussed in terms of olivo-cochlear neuronal excitability.

Transiently evoked otoacoustic emission amplitudes change with changes of directed attention.

The effect of a visual and of an auditory task on the cochlea was studied by transiently evoked otoacoustic emissions (TEOAEs). TEOAE amplitude decreased during visual and auditory attention for all 13 subjects tested. The decreased TEOAE activity was noted predominantly within the 960 to 1920 Hz range during visual attention and within the 1920-2880 Hz range during auditory attention. Selective attention, by way of the olivocochlear medial efferent system, modifies cochlear micromechanical properties. Visual and auditory attention act on different areas of the cochlear partition.

Sleep and active cochlear micromechanical properties in human subjects.

In this paper the effect of sleep on the cochlea is studied by transiently evoked otoacoustic emissions (TEOAEs). Amplitude increases considerably (e.g., 4 dB) and the effect of a contralateral noise on the TEOAEs decreases during the night. These modifications can be related to sleep, although there is no link to electroencephalographic sleep stage. During sleep onset, the effect of contralateral noise disappears: this could correspond to a functional rest of the auditory pathway during that period.

Lack of association between transiently evoked otoacoustic emission amplitude and experimentation linked-factors (repeated acoustic stimulation, cerebrospinal fluid pressure, supine and sitting positions, alertness level).

Changes in transiently evoked otoacoustic emissions (TEOAEs) occur during sleep and during tasks requiring attention. This can be due to a central nervous system effect on the cochlea. But, an additional or dominant influence by non-controlled factors is possible. In this paper, the effect of click-stimulus repetition, lying and sitting positions, state of alertness (awake or asleep) and CSF pressure variation on TEOAE features were studied. None of these factors affected TEOAE amplitude. In 2 subjects, TEOAE amplitude increased considerably during the night while remaining stable in 7 subjects during daytime sleep. This may be due to circadian variations of TEOAE amplitude.

A clinical study of human type IV collagen as tympanic membrane grafting material. Preliminary noncomparative study.

OBJECTIVE: To evaluate tolerance for this middle-ear implant and its effectiveness in eardrum healing under various pathologic conditions. SETTING: A 12- to 18-month follow-up case series at an institutional referral center offering hospitalized care. PATIENTS: Twenty-six eligible volunteers suffering from tympanic perforation or tympanic pocket retraction class II and III in Tos' classification. INTERVENTION: Patients underwent implantation with the type IV collagen film (film called "MTY" from the French membrane tympanique). The surgical procedure was similar to those using the temporal aponeurosis or the tragal perichondrium. OUTCOME MEASURE: Anatomic criteria was determined by otoscopy, and functional audiometric criteria was determined by air-borne gap measurements and by effects on cochlear function. RESULTS: Twenty-two of 26 patients were completely healed after 6 months, with no subsequent evolution. Nineteen of 26 subjects underwent a 1-year postoperative audiometric follow-up examination: 13 (68%) had an air-borne gap less than 10 dB, four (21%) had between 11 and 20 dB, and two (11%) had between 21 and 30 dB. Adverse effects included one patient whose MTY fell into the middle ear, three patients with transient myringitis, and one patient with otitis media with effusion. CONCLUSIONS: A human type IV collagen film may be a possible alternative to autologous tissue in tympanic membrane reconstruction. This new biomaterial was tested on patients with various diseases common in clinical otology, and demonstrated a good biocompatibility of MTY in the different pathologic conditions of chronic otitis media. A future randomized, controlled trial will randomly allocate patients to receive either MTY collagen film or tissue from the temporal aponeurosis.

Minimal endoscopic approach to subperiosteal orbital abscess.

OBJECTIVE: To limit endoscopic abscess drainage to the opening of the ethmoid cells involved, adjacent to the subperiosteal orbital abscess. DESIGN: Prospective study. PATIENTS: Twenty children with subperiosteal orbital abscess complicating acute sinusitis. INTERVENTION: Endoscopic opening of the medial wall of the bulla ethmoidalis and of the lamina papyracea. RESULTS: The limited endoscopic approach allowed subperiosteal orbital abscess drainage in all patients with positive clinical outcomes. Extensive ethmoidectomy was not necessary to achieve drainage. CONCLUSIONS: Endoscopic subperiosteal orbital abscess drainage does not require complete ethmoidectomy as was previously performed and can be limited to the opening of the bulla ethmoidalis and the lamina papyracea through the bulla ethmoidalis.

Viral RNA in middle ear mucosa and exudates in patients with chronic otitis media with effusion.

OBJECTIVE: To evaluate viral and cytokine signaling correlates of the persistent inflammation associated with chronic otitis media with effusion (OME). DESIGN: Prospective study. METHOD: Reverse transcriptase-polymerase chain reaction targeting RNA viruses frequently associated with OME (respiratory syncytial virus and parainfluenza virus type 3, the proinflammatory cytokines interleukin 8 and interleukin 1beta, and RANTES [regulated upon activation, normal T cell expressed and secreted]) was performed on mucosal biopsy samples and on samples of the liquid and cellular compartments of inflammatory exudates obtained from 26 children (49 ears) with infected middle ears. Ribonucleic acid extracted from rapidly frozen samples was reverse transcribed by Moloney murine leukemia virus reverse transcriptase and amplified for 35 cycles using previously validated primers. Amplicons were evaluated by molecular size after agarose gel electrophoresis with ethidium bromide. RESULTS: Most children had evidence of the presence of an RNA virus in at least one specimen. Respiratory syncytial virus was present in 40% and parainfluenza virus type 3 in 8% of effusions. Interleukin 8 messenger RNA was present in 21% of inflammatory exudates but never in cells from the mucosal biopsy samples. CONCLUSIONS: Our data support a viral contribution to the cause of OME and suggest that the inflammatory cytokines observed derive more from cells in the inflammatory exudate than from those in the middle ear mucosa.

A selective HPLC/RIA for the determination of budesonide.

A combined HPLC/RIA procedure is described for the selective determination of budesonide (BUD) in plasma. The assay involves the extraction of plasma or serum samples with ethylacetate, consequent HPLC separation of intact budesonide from cross-reacting metabolites on a C8 reversed phase column, collection of the budesonide containing fraction and determination of budesonide immunoreactivity with the budesonide antiserum. The method was accurate, sensitive (IC50 value of 0.9 ng ml-1) and reproducible (intra- and inter-day less than 15%) with a limit of quantification of 0.133 ng ml-1 (RSD < 25%). The evaluation of a limited number of clinical samples after oral administration of budesonide by both the HPLC/RIA procedure and a direct RIA using the same antiserum differed in average by a factor of 2, with the ratio of HPLC/RIA-RIA results declining as a function of time. Thus, this ratio might be a suitable indicator for probing for the ratio of budesonide and overall metabolites on a semi-quantitative level.

Morphometric analyses of Ateles: systematic and biogeographic implications.

This study examined the cranial and dental morphometric patterns among South American spider monkey taxa (Ateles), using various multivariate data reduction techniques to corroborate or falsify the predictions of alternative vicariance or parapatric models for explaining neotropical biodiversity. Data from 284 specimens were standardized to eliminate sex differences and then transformed by factor analysis to obviate the effects of body size on shape relationships. We found the independent discriminant analyses of dental and cranial data to be not at all dissimilar, nor did body size appear to distort geographical relationships. Thus a pooled data set of 50 measurements was further reduced by cluster analysis to produce a dendrogram of taxonomic relationships that is discordant with current classification. Three independent taxa are indicated, with the Guianan samples being most distinct, followed by the animals of northwest Venezuela and adjacent Colombia. Elsewhere, the samples describe a cline of relationships around Amazonia that resembles a ring species. Of particular interest is the apparent clinal intergradation across major tributaries of the Amazon, which is inconsistent with the concept of these rivers as barriers in their present form. Instead these observations, and the congruence between the multivariate clusters and previously identified centers of endemism, support both tierra firme and forest refugia models of historical biogeography. However, the latter model is somewhat falsified within Amazonia by character stability for alternative pelage patterns on opposite sides of the same major rivers that showed morphometric intergradation, but not as predicted within the refugia. This parapatric enhancement of pelage differences, which originated presumably in allopatry, suggests a mechanism of incipient speciation by frequency-dependent assortative mating with no apparent hybrid disadvantage or disruption of widespread gene flow.

Contrasting strategic approaches to the management of subglottic hemangiomas.

The standards of treatment of subglottic hemangiomas are steroids and laser vaporization. If these methods do not provide a sufficient airway, a tracheotomy may be required. Analysis of the data available in the literature shows that there is a place for other therapeutic solutions. Open surgical excision could be one of them but is controversial. So far, it represented a 'last' resort procedure before tracheotomy. Our approach to open surgical excision is different. A prospective decision is taken in the initial therapeutic approach. Infants are considered candidates for excision in cases of large hemangiomas that extend beyond the limits of laser. Laser is, then, avoided in order not to damage the overlying mucosa. Interferon is not utilized because a rapid effect is not expected on the respiratory distress. Open surgical excision without tracheotomy is performed. A cricoid enlargement is associated at the end of the procedure. Risk for post-operative subglottic stenosis is limited. When conceived this way, open surgical excision seems effective to manage large life-threatening hemangiomas.

Long-term airway considerations after treatment of severe pediatric laryngotracheal stenosis in five children.

After initial treatment of severe laryngotracheal stenosis (LTS), we evaluated subsequent airway function. Five children between 2 and 11 years were treated previously for severe LTS by T-tube stenting. One case underwent subsequent laryngotracheal reconstruction. All patients demonstrated dyspnea, the severity of which increased with age and duration of time after completion of stenosis treatment. In the most severe cases, magnetic resonance imaging and endoscopy revealed secondary subglottic stenosis. Ventilatory function tests disclosed obstruction of both extrathoracic and pulmonary origin. These findings raise questions regarding the treatment of the initial stenosing tissue and of the secondary stenoses.

Role of long-term stenting in treatment of pediatric subglottic stenosis.

Twelve cases of childhood subglottic stenosis diagnosed either acquired or congenital were treated using an endolaryngotracheal Montgomery T-tube. Stenting lasted on average 5.6 months. Tracheotomy closure was possible in 75% of cases on average 15.3 months after diagnosis. Tube-linked complications involved the child pulling out, forward migration of the tube out, lower tracheal migration of the tube, clogging and the formation of granulation tissue at its superior extremity. Comparison of outcomes with those for laryngeal surgery (cricoid split, laryngotracheal reconstruction) found in the literature, suggests that long-term T-tube stenting is the optimal treatment for subglottic stenosis where tracheomalacia precludes laryngeal surgery.

Cleft larynx: management and one-stage surgical repair by anterior translaryngotracheal approach in two children.

Cleft larynx is a rare congenital anomaly which is now being reported with increasing frequency. It is characterized by a midline posterior defect. Two children underwent laryngeal cleft repair by an anterior translaryngeal approach. Tracheotomy was avoided and closure of the anterior laryngofissure was carried out over the nasotracheal tube. The patients were cared for in a pediatric intensive care unit until extubation. Extubation was performed on day 8. The older child had few functional problems and did well whereas the younger child did poorly. In this latter case, the initially successful surgical result was impaired by post-operative aspiration, due to numerous possible factors: gastroesophageal reflux secondarily controlled by Nissen fundoplication, disturbed swallowing as a result more of the cleft repair work than of the surgical approach, or else immaturity of the suction-swallowing reflex.