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Biomedical subjects

G Roger

Publications and source records attributed to G Roger.

At least 37 records · Page 2Linked to original sources

Extensive lymphangioma presenting with upper airway obstruction.

OBJECTIVE: To describe the results of an outcome survey of 18 cases of pediatric lymphangioma with dyspnea from encroachment on the tongue base, parapharyngeal space, and/or larynx. DESIGN: Retrospective review of files from January 1983 to September 1998. SETTING: Pediatric otolaryngology departments from 2 referral centers. PATIENTS: Eighteen patients were treated. The average age at initial surgery was 22 weeks (median, 5 weeks). All presented with at least unilateral suprahyoid and infrahyoid cavernous (microcystic) lymphangioma. The tongue base was involved in 11 patients, the parapharyngeal space in 12, and the larynx in 8. INTERVENTIONS: Neck dissection was performed initially in all patients. Tracheotomy was performed in 9 patients (50%). Macroglossia was treated by V glossoplasty. Parapharyngeal extensions were treated by cervicotomy or endoscopy, and larynx and tongue base extensions by carbon dioxide laser photocoagulation. Supraglottic laryngectomy was performed in 2 patients. MAIN OUTCOME MEASURES: Residual disease, decannulation, duration of tracheotomy, and persistent respiratory symptoms. RESULTS: The average follow-up was 4 years postoperatively. One postoperative death occurred. Sixteen (94%) of the remaining 17 patients had residual lymphangioma. Eight (89%) of the 9 patients with tracheotomy underwent decannulation (average duration, 22 months). Ten patients had persistent symptoms, and 6 were asymptomatic. CONCLUSIONS: Involvement of the upper airway seems to be the determining prognostic factor in extensive lymphangioma. Patients with dyspnea by external compression of cervical lymphangioma on the airway responded well to surgery. Aggressive surgical treatment did not seem to significantly improve the prognosis in patients with intrinsic involvement of the upper airway. The natural evolution of untreated massive lymphangioma has not been documented. Less aggressive, symptomatic therapy may be an alternative to avoid mutilating surgery in patients with intrinsic involvement of the airway.

Airway Obstruction↗

Characterization of inflammatory reaction in upper airways of cystic fibrosis patients.

Inflammatory cell populations have not been yet precisely evaluated in cystic fibrosis (CF) airways. We intended to characterize morphological modifications, inflammatory cell infiltration and cell proliferation in nasal tissues obtained from 15 CF patients and from 6 non-CF patients with nasal polyposis. Morphological analysis showed an intense inflammatory infiltration in CF and non-CF tissues with only few modifications in the epithelium from CF tissues. Inflammatory cell populations characterized by specific immunolabeling were quantified, showing a predominance of macrophages and T- and B-lymphocytes and only moderate numbers of neutrophils in CF tissues; in non-CF polyps, lymphocytes and eosinophils were abundant. Proliferating cell percentages quantified after proliferating cell nuclear antigen immunolabeling were 5.3+/-4.1% (mean +/- SD) in CF polyps and 3.1+/-1.2% in non-CF polyps in epithelium but were very low in lamina propria. Intense inflammation in nasal tissues from CF patients is therefore dominated by macrophages and lymphocytes rather than by neutrophils. While morphology is preserved, proliferation is high in epithelium from CF polyps. These findings should be regarded in the future for a better understanding of inflammation in CF airway disease.

Adolescent↗

Head and neck infantile myofibromatosis--a report of three cases.

Infantile myofibromatosis (IM) is a proliferative disorder characterized by the development of single or multiple nodular lesions arising from cutaneous, subcutaneous, muscular, bone or visceral structures. This proliferation may occur at any anatomical site, but in 30% of the cases it involves the head and neck. We report here three cases of head and neck IM occurring in young children and presenting as solitary lesions. The clinical heterogeneity and the misleading histopathological appearances can make the diagnosis difficult. The treatment is surgical but the low rate of recurrence and the possibility of spontaneous tumoral regression may lead to conservative surgery or therapeutic abstention.

Child↗

Congenital tracheoesophageal fistula without esophageal atresia.

The authors report a series of eight cases of isolated tracheoesophageal fistula without esophageal atresia (or an H type fistula), treated in three pediatric ENT departments. This is a rare malformation whose diagnosis requires investigation for associated anomalies. The clinical signs are mainly respiratory but also digestive and the symptomatology can be severe. The diagnosis can be made with a barium swallow combined with cineradiography, but a tracheoesophageal endoscopy remains the investigation of choice. The treatment is surgical. In most cases, the fistula is accessible by a right or left cervicotomy, depending on the surgeon's practice, with a much lower postoperative morbidity as compared to a thoracotomy. The postoperative management was straightforward in most of our cases. We discuss the role of gastro-esophageal reflux with respect to postoperative morbidity as well as systematic treatment for reflux peri-operatively. The pros and cons of the various surgical approaches are also discussed.

Barium Radioisotopes↗

The CHARGE association: the role of tracheotomy.

OBJECTIVES: To evaluate the need for a tracheotomy and its timing during the evolution of an association of malformations, including coloboma, heart defects, choanal atresia, developmental and growth retardation, genitourinary malformation, and ear anomalies (CHARGE association). DESIGN: Retrospective study from January 1988 through December 1997. SETTING: Four academic tertiary care centers. PATIENTS AND METHODS: Forty-five patients with CHARGE association having at least 3 cardinal malformations (growth retardation excluded) and review of the malformations and respiratory manifestations encountered. All the patients underwent endoscopic exploration on several occasions. We reviewed the nature and the timing of therapeutic interventions performed on the airway. RESULTS: Two patients died (one patient of septicemia, the other of unknown causes). Abnormalities of blood gas levels and/or sleep were found in 30 patients (67%), were responsible for cardiorespiratory arrest in 9 (20%), and required admission to the intensive care unit in 21 (47%). Pharyngolaryngeal anomalies leading to dyspnea (discoordinate pharyngolaryngomalacia, glossoptosis, retrognathia, laryngeal paralysis, cleft, stenosis, and difficult intubation) were found in 26 patients (58%). Tracheobronchial anomalies (esophagotracheal fistula, esophageal atresia, and tracheomalacia) were present in 18 patients (40%). Resection of the aryepiglottic folds was attempted 3 times, but without success. Tracheotomy was necessary in 13 patients (29%) at a median age of 2.4 months (mean duration, 25 months). Among these infants, the posterior nasal choanae were patent in 10 patients at the time of tracheotomy. Gastroesophageal reflux was encountered in 36 patients (80%). Prolonged enteral feeding was necessary in 21 patients (47%), with gastrostomy in 16 (of whom 9 needed a tracheotomy). These feeding difficulties and airway problems were highly correlated. CONCLUSIONS: We encountered multiple, complicated airway abnormalities. Resection of aryepiglottic folds was inadequate. Often, a tracheotomy could not be avoided in these patients, regardless of choanal patency. Tracheotomy needs to be performed early to avoid hypoxic events. In some selected patients, ventilation using bilevel positive airway pressure may be an alternative.

Abnormalities, Multiple↗

Evolution of the bacteriologic features of persistent acute otitis media compared with acute otitis media: a 15-year study.

OBJECTIVES: To define the epidemiologic features of persistent acute otitis media (PAOM) and modifications of these features during the past 15 years and to investigate for possible differences in bacterial resistance between acute otitis media (AOM) and PAOM. DESIGN: Retrospective patient series. SETTING: Academic tertiary care center. PATIENTS AND METHODS: Persistent acute otitis media was defined as AOM lasting longer than 3 weeks despite 1 or several courses of antibiotic therapy, with the persistence of clinical and otoscopic signs of AOM. From 1982 to 1997, 475 children with PAOM were seen in our department. Every patient had 1 or several specimens of aspirations or swabs of spontaneous otorrhea (or both). Microbiologic characteristics of the isolated strains (including antibiotic susceptibility) were analyzed. Four successive series of specimens were analyzed-group 1: from October 1, 1982, to June 30, 1986 (136 patients); group 2: from January 1, 1987, to December 31, 1989 (165 patients); group 3: from January 1, 1992, to April 30, 1993 (73 patients); and group 4: from January 1, 1994, to January 31, 1997 (101 patients). During the same study periods, the bacteriologic results of patients with AOM in the same geographic region were recorded. MAIN OUTCOME MEASURES: A longitudinal comparison between the groups of patients with PAOM and a cross-comparison within each group between patients with PAOM and those with AOM. RESULTS: Obtaining repeated and multiple specimens from patients with PAOM led to a progressive decrease in the rate of sterile specimens, from 35.3% (group 1, 48 patients) to 14.9% (group 4, 15 patients) (P<.01). During this period, the prevalence of Streptococcus pneumoniae increased in patients with positive culture results, from 18.2% (group 1, 16 of 88 patients) to 44.2% (group 4, 38 of 86 patients) (P<.001). These strains rapidly and dramatically became resistant to penicillin (amoxicillin) (0% through 1989, 76.2% [16 of 21 patients] in 1993, and 97.4% [37 of 38 patients] in 1996) (P = .01). The overall prevalence of Haemophilus influenzae remained stable (between 31.4% [27 of 86 patients] and 45.4% [40 of 88 patients]), but the proportion of beta-lactamase-producing strains increased from 30.0% (group 1, 12 patients) to 55.6% (group 4, 15 patients) (P=.04). The prevalences of Pseudomonas aeruginosa and Staphylococcus aureus did not vary significantly (from 23.1% [group 2, 30 patients] to 10.7% [group 3, 6 patients] and from 10.2% [group 1, 9 patients] to 4.6% [group 4, 4 patients], respectively). Comparing data from patients with PAOM with those with AOM revealed that the increased resistance of H influenzae and, in particular, of S pneumoniae was more rapid and more marked in patients with PAOM than in those with AOM (highest rate of resistance in AOM: 36.0% [271 of 753 specimens] and 50.6% [398 of 787 specimens] for H influenzae and S pneumoniae, respectively; P<.001 for S pneumoniae). CONCLUSIONS: The increase in bacterial resistance frequently encountered during otitis media is even more marked in patients with PAOM. The identification of the organism is essential when the otitis does not resolve, especially in patients with PAOM. Obtaining repeated specimens helps to decrease the rate of sterile cultures.

Acute Disease↗

EPR spectroscopy: a powerful technique for the structural and functional investigation of metalloproteins.

Numerous metal centers in proteins can be prepared in a redox state in which their ground state is paramagnetic. Complementary data provided by EPR, Mössbauer, electron nuclear double resonance, magnetic circular dichroism, and NMR spectroscopies have therefore played a major role in the elucidation of the structure and function of these centers. Among those techniques the most commonly used is certainly EPR spectroscopy. In this article various aspects of the current applications of EPR to the structural and functional study of metalloproteins are presented. They are illustrated by recent studies carried out in our laboratory in the field of metalloenzymes and electron transfer systems. The power of numerical simulation techniques is emphasized throughout this work.

Agaricales↗

Loss-of-function mutations in a human gene related to Chlamydomonas reinhardtii dynein IC78 result in primary ciliary dyskinesia.

Primary ciliary dyskinesia (PCD) is a group of heterogeneous disorders of unknown origin, usually inherited as an autosomal recessive trait. Its phenotype is characterized by axonemal abnormalities of respiratory cilia and sperm tails leading to bronchiectasis and sinusitis, which are sometimes associated with situs inversus (Kartagener syndrome) and male sterility. The main ciliary defect in PCD is an absence of dynein arms. We have isolated the first gene involved in PCD, using a candidate-gene approach developed on the basis of documented abnormalities of immotile strains of Chlamydomonas reinhardtii, which carry axonemal ultrastructural defects reminiscent of PCD. Taking advantage of the evolutionary conservation of genes encoding axonemal proteins, we have isolated a human sequence (DNAI1) related to IC78, a C. reinhardtii gene encoding a dynein intermediate chain in which mutations are associated with the absence of outer dynein arms. DNAI1 is highly expressed in trachea and testis and is composed of 20 exons located at 9p13-p21. Two loss-of-function mutations of DNAI1 have been identified in a patient with PCD characterized by immotile respiratory cilia lacking outer dynein arms. In addition, we excluded linkage between this gene and similar PCD phenotypes in five other affected families, providing a clear demonstration of locus heterogeneity. These data reveal the critical role of DNAI1 in the development of human axonemal structures and open up new means for identification of additional genes involved in related developmental defects.

Amino Acid Sequence↗

Myringoplasty in children: predictive factors of outcome.

OBJECTIVES: To assess the results of myringoplasty in children and to determine which factors independently influence the postoperative results. STUDY DESIGN: Retrospective study of the anatomic and functional results of 231 consecutive myringoplasties performed in 188 children between 1988 and 1992. Multivariate analysis of poor prognostic factors by cross-sectional comparison 1 year after surgery. METHODS: Myringoplasties were performed via an endaural approach with a fascia temporalis underlay graft. RESULTS: In 216 of 231 ears (93.5%) the tympanic membrane was closed. A good anatomic outcome was considered to have been achieved in 188 ears (81.6%), although in 18 ears (7.8%) seromucous otitis media occurred, in 8 ears (3.5%) a progressive retraction pocket was encountered, and in 2 ears significant lateralization was present. One hundred thirty-nine (67.5%) of the 206 ears tested in the postoperative period had a postoperative air-bone gap of 10 dB or less. On average, mean bone conduction remained unaltered. The age of the patient and the size and the location of the perforation did not affect the outcome. Three prognostic factors for an abnormal postoperative tympanic membrane were found, with 95% confidence intervals: inflammatory changes in the middle ear mucosa (P < .05), contralateral tympanic perforation (P < .05), and contralateral cholesteatoma (P < .01). CONCLUSIONS: Myringoplasty with underlay grafting of the fascia temporalis in children gives good anatomic and functional results. Inflammatory changes within the middle ear mucosa, contralateral tympanic perforation, and contralateral cholesteatoma independently influence the risk of an abnormal postoperative tympanic membrane. The presence of one of these factors preoperatively should lead to the consideration of alternative, more durable graft material, such as autologous cartilage.

Adolescent↗

Surgical removal of subglottic hemangiomas in children.

OBJECTIVE: To examine the indications and the results of surgical excision of severe subglottic hemangiomas. DESIGN: Retrospective study and case series. SETTING: Four academic tertiary care centers of pediatric otolaryngology. PATIENTS: Twenty children were included from 1991 to 1997. All presented with severe subglottic hemangiomas resistant to classical treatments such as corticosteroids and/or CO2 laser. INTERVENTION: Six children were operated on using laryngotracheal reconstruction and prolonged stenting by a reinforced Silastic roll and 14 children were operated on by a single-stage laryngotracheoplasty and postoperative intubation in a pediatric intensive care unit. RESULTS: All patients were successfully decannulated or extubated and have been free from recurrent hemangiomas. CONCLUSION: The surgery of severe subglottic hemangiomas is a reliable technique in selected patients and should be considered in corticoresistant or corticodependent, circular, or bilateral hemangiomas.

Child, Preschool↗

Pharyngo-laryngeal histoplasmosis: one case in an immunocompetent child.

We report a very rare case of disseminated pharyngo-laryngeal histoplasmosis with systemic spread in a 10-year-old, immunocompetent child from Guyana. The main signs were a marked deterioration in his general condition, hepato-splenomegaly, multiple lymphadenopathy and ulcerated pharyngo-laryngeal lesions. The diagnosis was made from brushings of the ulcerative lesions, a lymph node biopsy and serological tests performed in the national reference center for histoplasmosis. The initial treatment was with amphotericin B, which was then replaced by oral itraconazole. We report here the main epidemiological, clinical and therapeutic characteristics.

Amphotericin B↗

Results of tympanoplasty in children with primary ciliary dyskinesia.

OBJECTIVE: To assess the results of tympanoplasty in children with primary ciliary dyskinesia complicated by tympanic perforation or cholesteatoma with hearing loss and/or recurrent otorrhea. DESIGN: Retrospective study. Postoperative follow-up of 26.3 months in the type 1 tympanoplasty group and 46 months in the child with cholesteatoma. SETTING: Hospitalized care, referral center. PATIENTS: Seven children with primary ciliary dyskinesia, complicated in 6 children by 9 tympanic perforations (3 bilateral perforations) and in 1 child by an attical cholesteatoma. RESULTS: After 9 type 1 tympanoplasties, the grafts were intact in 9 ears, with no recurrence of otorrhea, but serous otitis media was present in 6 of the 9 ears. Auditory improvement was significant, with an average gain of 17-dB hearing level in speech frequencies. After a canal wall-down tympanoplasty with mastoidectomy for attic cholesteatoma in 1 ear, the cavity that was operated on showed no signs of otorrhea or residual cholesteatoma after a follow-up of 46 months. CONCLUSION: In children with primary ciliary dyskinesia, tympanoplasty has a high probability of graft success and auditory improvement, despite the frequent recurrence of serous otitis media.

Adolescent↗

Posterior laryngeal clefts: preliminary report of a new surgical procedure using tibial periosteum as an interposition graft.

OBJECTIVE: To present the preliminary results of a new surgical procedure for posterior laryngeal cleft repair. DESIGN: Retrospective study in an academic tertiary care center. METHOD: The study included three male patients (age at surgery, 2, 13, and 14 mo). One presented with severe aspiration and cyanotic attacks, the two others with aspiration and recurrent chest infections. The types of laryngeal clefts included complete cleft of the cricoid with varying degrees of tracheal involvement but not further than the first six tracheal rings. Associated malformations included one VATER syndrome, one esophageal atresia, and one tracheoesophageal fistula. Surgery was performed under general anesthesia with nasotracheal intubation. A vertical anterior laryngofissure was performed. The mucosal margins of the clefts were incised and then repaired in two layers with polyglactin sutures. The original feature of this procedure was the interposition of a small piece of tibial periosteum between the two layers. This fascia graft is known to be strong and resistant in cleft palate surgery. MAIN OUTCOME MEASURE: Clinical and endoscopic follow-up was used for evaluation of results. RESULTS: The three patients had successful laryngeal repair at a mean follow-up of 6 months (range, 4-14 mo). CONCLUSION: The anterior laryngofissure provides a good surgical access to the cleft. The interposition of tibial periosteum allows durability of the cleft repair. A longer follow-up is needed to confirm these preliminary results. A computed tomography scan study and a study on the rabbit are planned in order to evaluate the outcome of these periosteal grafts.

Adolescent↗

Management of acute otitis media caused by resistant pneumococci in infants.

OBJECTIVES: To assess the clinical outcome and risk of failure after oral vs. intravenous treatment in otitis media caused by penicillin-resistant pneumococci. To determine the possible correlations between pneumococcal minimal inhibitory concentration (MIC) to penicillin and clinical outcome. DESIGN: Retrospective study of 156 cases collected between 1993 and 1995. Mean follow-up: 5 months. Setting. Two tertiary academic medical centers in Paris, France. PATIENTS AND METHODS: Pneumococcus was isolated from 191 of 570 ear samples obtained from children with otitis media and shown to be penicillin-resistant in 156. Medical history, antibiotic therapy during the previous 3 months and day-care center attendance were reviewed. For the current episode microbiologic characteristics of the isolated strains, type of treatment, therapy efficacy and clinical outcome were analyzed. Patients were predominantly young (76.3% were <1 year old) and bacteriologic samples were taken mainly because of previous treatment failure. RESULTS: Among 156 children with pneumococcal penicillin-resistant otitis media, 72.2% attended day-care centers, 71.8% had been previously treated with aminopenicillin and 52.5% with cephalosporins. Failure of previous empirical oral therapy was noted in 84% (one-third of these had been receiving amoxicillin-clavulanate). Patients treated intravenously had had a more protracted otitis but no greater number of previous episodes of acute otitis media than those receiving oral therapy. Acute mastoiditis occurred in 4 infants resulting in mastoidectomy. Oral treatment (mainly with high dose amoxicillin,120 to 150 mg/kg/day) and intravenous therapy (cephalosporin or glycopeptide) had been used in 59 and 41%, respectively. Mean duration of therapy was 10.7 days. Three failures (1.9%) and 10 recurrences (6.4%, average 28 days) occurred. No statistical difference was found between intravenous and oral therapy with respect to risk of recurrence. A high penicillin MIC value was correlated with previous antibiotic treatment but not with clinical outcome. CONCLUSIONS: Oral therapy appears to be as effective as intravenous therapy for the treatment of penicillin-resistant pneumococcal otitis media. Intravenous treatment should not necessarily be dictated by the penicillin susceptibility value but should be considered in cases of failure to thrive, persistent otitis or other complications.

Acute Disease↗

Midline cervical cleft.

Midline cervical cleft is a rare congenital anomaly of the ventral neck and its embryological origin has not been clearly established. Less than 100 cases have been reported in the literature. We present a case of midline cervical cleft operated on at the age of 2 months in order to illustrate its clinical presentation and surgical management.

Branchial Region↗