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Introduction: Gastric cancer is not typically a surgical emergency. However, it can evolve into urgent complications such as a perforation. We report a case of a perforated gastric ulcer that turned out to be a cancer. Observation: A 52-year-old man was admitted to the emergency department of Hubert-Koutoukou-Maga National University Medical Center in Cotonou for generalized abdominal pain. He was diagnosed with acute generalized peritonitis with perforated gastric ulcer, establishing a surgical indication. An antral perforation was found and a simple closure was performed. Anatomopathological examination of the surgical piece revealed a gastric adenocarcinoma within the granulation tissue. Following the impact assessment, he underwent a second surgery where a distal gastrectomy was performed with D2 lymphadenectomy followed by gastrojejunostomy. He developed an anastomotic gastrointestinal fistula during the postoperative period but was successfully medically treated. The patient received adjuvant chemotherapy with Epirubicin, Cisplatin and 5-Fluorouracil. The patient is still alive, 3 years after the gastrectomy. Conclusion: When faced with a perforated gastric ulcer, one must also consider a neoplastic cause. The emergency surgical treatment depends on the general condition of the patient and the pre-existing co-morbidities, the choice being made between a one-stage versus two-stage gastrectomy.

Objectif. ��tudier les caract��ristiques ��pid��miologiques et histopathologiques des sarcomes diagnostiqu��s dans les centres hospitaliers de r��f��rence de Cotonou. M��thodes. Il s���agissait d���une ��tude multicentrique, descriptive, r��trospective r��alis��e sur une p��riode de 10 ans. Nous avons recrut�� syst��matiquement toutes les observations m��dicales de patients atteints d���un sarcome histologiquement confirm��. R��sultats. 159 comptes-rendus de sarcome ont ��t�� retenus. L'��ge moyen des patients ��tait de 38,9 ans avec une pr��dominance f��minine (sex-ratio : 0,9). Les sarcomes des tissus mous ��taient les plus fr��quents (65,4 %). La localisation retrouv��e le plus fr��quemment ��tait les membres pelviens (30,2 %). Conclusion. Un meilleur acc��s aux moyens diagnostiques contribuerait �� une meilleure ��valuation du fardeau de cette pathologie au B��nin. Epidemiological and histopathological aspects of sarcomas in Cotonou (Benin) from 2010 to 2020 Aims. To study the epidemiological and histopathological characteristics of sarcomas. Procedure. This was a multi-center, descriptive, retrospective study realized over a 10-year period. We systematically recruited all medical reports of patients with histologically confirmed sarcoma. Results. 159 reports of sarcoma were retained. The average age was 38.9 years. There was a female predominance with a 0.9 estimated sex ratio. Soft tissue sarcomas were the most common (65.4%). The most frequent location was the lower limbs (30.2%). Conclusion. Better access to diagnosis would contribute to a better assessment of the burden of this pathology in Benin., MTSI, Vol. 2 No 1 (2022): MTSI-Revue

Background GISTs are rare tumors but the most frequent mesenchymal tumors of the digestive tract. Diagnosis and treatment are challenging in low-income countries due to relatively poor access to immunohistochemistry and targeted therapy. In Africa, there are few studies about it. Imatinib, an oral targeted therapy, has been available in Benin since 2010 and free since 2016. This study describes the diagnosis and therapeutic management of GIST in Cotonou, Benin. Methods This is a descriptive cross-sectional study, with retrospective data collection over a 10-year period from 2010 to 2020, focused on patients with histological confirmed gastro-intestinal stromal tumor (GIST). Cases were identified using the registry database and the archival files of the Hubert Koutoukou Maga National University Hospital of Cotonou (CNHU-HKM). Results Fifteen GISTs were identified during the study period. The median age was 52 and the sex ratio was 2:1 (10 males and 5 females). The most frequent symptom was abdominal pain (n = 12). Delay in care seeking after onset of symptoms ranged from 24 h to 15 years. The most common site for GISTs was the stomach (n = 8). The median tumor size was 11 cm and the majority (n=10) was metastatic or locally advanced at the time of diagnosis. The tumors were often spindle-shaped at histology (n = 13) and the majority expressed KIT (n = 14). Most of the tumors (n = 12) were at high risk of recurrence according to the Joensuu scoring system. The availability of imatinib has improved the outcome of GIST with response in all cases it was used in neoadjuvant setting (n = 7). Conclusion GISTs are rare tumors and preferentially affect the stomach in Cotonou). Most of the tumors were large, unresectable at the time of diagnosis and at high risk of recurrence. Access to imatinib has revolutionized the management of those tumors in our country.

To study the epidemiological and histopathological characteristics of sarcomas.This was a multi-center, descriptive, retrospective study realized over a 10-year period. We systematically recruited all medical reports of patients with histologically confirmed sarcoma.159 reports of sarcoma were retained. The average age was 38.9 years. There was a female predominance with a 0.9 estimated sex ratio. Soft tissue sarcomas were the most common (65.4%). The most frequent location was the lower limbs (30.2%).Better access to diagnosis would contribute to a better assessment of the burden of this pathology in Benin.

Introduction Bien que les taux d’incidence du cancer du sein en Afrique soient parmi les plus faibles au monde, les taux de mortalite y sont les plus eleves refletant une survie mediocre des patientes. Au Benin, en particulier, selon les donnees du Centre international de recherche sur le cancer, le cancer du sein est le premier cancer de la femme aussi bien en termes d’incidence que de mortalite. L’objectif de notre etude etait d’analyser les facteurs influencant la survie des patientes atteintes d’un cancer du sein a Cotonou. Methodes L’etude a ete realisee dans deux centres de reference, a l’echelle nationale, dans la prise en charge des cancers du sein : le Centre national hospitalier universitaire Hubert KoutouKou Maga (CNHU-HKM) de Cotonou, et le Centre hospitalier universitaire de la Mere et de l’Enfant Lagune (CHU-MEL) de Cotonou. Elle etait descriptive et analytique avec une collecte retrospective des donnees sur une periode allant du 1er janvier 2014 au 30 septembre 2020. Les cas de cancer du sein histologiquement confirmes et disposant d’une immunohistochimie (Recepteurs a l’œstrogene, Recepteurs a la progesterone, HER2, Ki67) ont ete inclus dans l’etude. L’analyse des donnees, a ete conduite a l’aide du logiciel STATA version 15. Le seuil de significativite des tests statistiques a ete fixe a 5 %. La survie, dans les differents groupes pronostiques, a ete evaluee par la methode de Kaplan-Meier ; le test de log-rank a ensuite ete utilise pour la comparaison de la survie entre les groupes ; et les facteurs pronostiques ont enfin ete determines grâce a un modele de Cox. Resultats Nous avons collige au total 326 cas de cancer du sein. L’âge moyen des patientes etait 48,76 ± 11,73 ans. Le plus souvent, elles n’etaient pas instruites ou avaient un niveau d’instruction bas (50,35 %). Le delai moyen entre la decouverte de l’anomalie mammaire et la premiere consultation etait de 6,21 mois. Les tumeurs etaient surtout classees T3 et T4 (65,95 %) avec un envahissement ganglionnaire N1 et N2 (66,57 %) et des metastases cliniquement notees dans 21,78 % des cas. Les tumeurs “luminal B” (32,57 %) et les tumeurs triples negatives (31,29 %) etaient les types moleculaires les plus frequents. Sur le plan therapeutique les patientes ont beneficie de la chimiotherapie (65,33 %), de la mastectomie radicale associee a curage axillaire (65,33 %), de la radiotherapie (19,02 %), de l’hormonotherapie (21,16 %) et de la therapie ciblee (3,68 %). La survie globale a 5 ans etait de 59,22 %. En analyse univariee, les facteurs de mauvais pronostiques etaient le delai de consultation superieur a 6 mois (p = 0,0368), la taille T3-T4 de la tumeur (p = 0,0001), le grade SBR 3 (p = 0,0005), l’atteinte ganglionnaire (p = 0,0003), la presence de metastases (p = 0,0000), le type moleculaire triple negatif (p = 0,0007), le stade UICC avance de la maladie (p = 0,0000), l’absence de chirurgie (p = 0,0063) et d’hormonotherapie (p = 0,0065). En analyse multivariee, les facteurs de mauvais pronostiques retrouves etaient l’envahissement ganglionnaire (HR = 8,38 ; p = 0,038), la presence de metastase (HR = 6,36 ; p = 0,0000) et l’absence d’hormonotherapie (HR = 3,03 ; p = 0,003). Conclusion La survie des patientes atteintes d’un cancer du sein a Cotonou est influencee par de nombreux facteurs. Elle peut etre amelioree par le diagnostic precoce et un meilleur acces aux differentes modalites therapeutiques. Une attention particuliere devra etre portee a certains types.

Background Internal hernias and the appendicular tourniquet are two rare pathologies. Case presentation We report here a case of a 68-year-old obese patient, who has acute small bowel obstruction due to strangulated internal hernia through an appendicular tourniquet. This appendicular tourniquet results from the adhesion between the tip of the appendix and its body. This obstruction was complicated by plugged perforation. Resection of the small bowel segment was performed, in addition to appendectomy, lavage, and drainage. The postoperative recovery was uneventful. Conclusion Acute bowel obstruction secondary to strangulation of an internal hernia through an appendicular tourniquet is exceptional.

Resumé:L’hydatidose utérine est une localisation particulière du kyste hydatique. Elle pose un véritable problème diagnostic même en zone d’endémie. Les auteurs rapportent un cas exceptionnel de kyste hydatique utérin se présentant à l’échographie et au scanner comme une masse ovarienne droite. Ils indiquent les difficultés diagnostic, discutent l’etiopathogenie de la localisation du parasite au niveau du pelvis et les difficultés thérapeutiques mettant en jeu le pronostic gynécologique et obstétrical des jeunes patientes., Journal Marocain des Sciences Médicales, Vol. 20, No 2 (2016)

Objective Preliminary assessment of the efficacy of propranolol on subglottic hemangioma in children on a nation-wide scale. Methods Multicentric, retrospective study of clinical files of 14 children; pre- and post-treatment endoscopies. Results Mean age at diagnosis was 2.3 (0.7–4) months. Mean percentage of airway obstruction was 68% (15–90) before propranolol introduction. Propranolol was started at 5.2 (0.7–16) months of age. This treatment was effective in all cases with a mean regression of the stenosis to 22% after 2 weeks and 12% after 4 weeks. Other medical treatments (steroids) could be stopped. In one patient, a side effect of propranolol motivated the switch to another β-blocker. In four patients, treatment was stopped after 5.2 (1–10) months with a relapse in 2 (50%) cases. One of these two patients developed a resistance to propranolol and required a surgical procedure by external approach. Conclusion This preliminary nation-wide survey confirms propranolol high effectiveness against airways’ localization of infantile hemangiomas. Propranolol also allows alleviation or cut-off of previous medical treatments. However, recurrences are possible after early treatment interruption.

OBJECTIVE: To show that noninvasive positive-pressure ventilation by means of a nasal mask may avoid recannulation after decannulation and facilitate early decannulation. DESIGN: Retrospective cohort study. SETTING: Ear-nose-and-throat and pulmonary department of a pediatric university hospital. PATIENTS: The data from 15 patients (age = 2-12 yrs) who needed a tracheotomy for upper airway obstruction (n = 13), congenital diaphragmatic hypoplasia (n = 1), or lung disease (n = 1) were analyzed. Four patients received also nocturnal invasive ventilatory support for associated lung disease (n = 3) or congenital diaphragmatic hypoplasia (n = 1). Decannulation was proposed in all patients because endoscopic evaluation showed sufficient upper airway patency and normal nocturnal gas exchange with a small size closed tracheal tube, but obstructive airway symptoms occurred either immediately or with delay after decannulation without noninvasive positive-pressure ventilation. INTERVENTIONS: In nine patients, noninvasive positive-pressure ventilation was started after recurrence of obstructive symptoms after a delay of 1 to 48 mos after a successful immediate decannulation. Noninvasive positive-pressure ventilation was anticipated in six patients who failed repeated decannulation trials because of poor clinical tolerance of tracheal tube removal or tube closure during sleep. MEASUREMENTS AND MAIN RESULTS: After noninvasive positive-pressure ventilation acclimatization, decannulation was performed with success in all patients. Noninvasive positive-pressure ventilation was associated with an improvement in nocturnal gas exchange and marked clinical improvement in their obstructive sleep apnea symptoms. None of the 15 patients needed tracheal recannulation. Noninvasive positive-pressure ventilation could be withdrawn in six patients after 2 yrs to 8.5 yrs. The other nine patients still receive noninvasive positive-pressure ventilation after 1 yr to 6 yrs. CONCLUSIONS: In selected patients with upper airway obstruction or lung disease, noninvasive positive-pressure ventilation may represent a valuable tool to treat the recurrence of obstructive symptoms after decannulation and may facilitate early weaning from tracheotomy in children who failed repeated decannulation trials.

Transmission electron microscopy (TEM) analysis of ciliary ultrastructure is classically used for the diagnosis of primary ciliary dyskinesia (PCD). We report our extensive experience of TEM analysis in a large series of patients in order to evaluate its feasibility and results. TEM analysis performed in 1,149 patients with suspected PCD was retrospectively reviewed. Biopsies (1,450) were obtained from nasal (44%) or bronchial (56%) mucosa in children (66.5%) and adults (33.5%). TEM analysis was feasible in 71.4% of patients and showed a main defect suggestive of PCD in 29.9%. TEM was more feasible in adults than in children, regardless of the biopsy site. Main defects suggestive of PCD were found in 76.9% of patients with sinopulmonary symptoms and in only 0.4% of patients with isolated upper and 0.4% with isolated lower respiratory tract infections. The defect pattern was similar in children and adults, involving dynein arms (81.2%) or central complex (CC) (18.8%). Situs inversus was never observed in PCD patients with CC defect. Kartagener syndrome with normal ciliary ultrastructure was not an exceptional condition (10.2% of PCD). In conclusion, TEM analysis is feasible in most patients and is particularly useful for PCD diagnosis in cases of sinopulmonary syndrome of unknown origin.

Laryngeal clefts are rare congenital anomalies, first described in 1792 by Richter, that allow communication between the tracheal and esophageal axis. The incidence is 1 in 10,000 to 20,000 births, which represents approximately 1.5% of the laryngeal pathology in children. Laryngeal clefts result from a failure of fusion of the posterior cricoid lamina and development of the tracheoesophageal septum. Recent work has further refined our understanding of this complex development.

Lymphangiomas of the mouth and tongue pose considerable therapeutic problems. Their complete exeresis is not feasible, and they can be a major functional impediment and cause face and jaw deformities. A risk of secondary growth is classically described after surgical reduction. Invasion of lingual mucosa, often papillomatous, results in accidental biting, recurrent bleeding and pain. Here, we report on 7 cases of children with haemorrhagic mucosal lingual lymphangiomas, one with noteworthy drop in the haemoglobin, treated by surface radiofrequency reduction. With a short follow-up (3-13 months), a functional improvement was observed in every case, as a complete disappearance of bleeding in 5/7 cases, and a moderate local relapse in 2/7 cases. This technique did not induce progressive regrowth. It provides a new therapeutic tool for the treatment of lingual microcystic lymphangioma.

Annales Francaises d'Oto-Rhino-Laryngologie et de pathologie cervico-faciale - Vol. 125 - N° 1 - p. 46-51

Objectives: To define predictors of residuals and retraction pockets (RP) in children operated on for congenital cholesteatoma (CC). Design and Setting: Retrospective review (1996–2005), academic center. Patients: One hundred seventeen patients treated for CC corresponding to modified Derlacki's criteria were included (median age, 6.5 yr). No case of RP at time of diagnosis, with a mean follow-up of 2.5 years after last surgery. Main Outcome Measures: Clinical and surgical data influencing outcome. Multivariate analysis. Results: Two groups were defined after CC removal: group I (12 cases), no second look required and no case of subsequent re-intervention; group II (105 cases), planned second look always performed (mean delay, 12.1 mo), no difference of sex ratio (M/F = 2). Group I patients were younger than in group II (3.3 vs. 5.9 yr, P < .001). All of them had a normal contralateral eardrum and a disclosure of CC by routine examination (vs. 19% in group II, P < .001). In group I, the mass occupied one or two anterior quadrants (41.6% and 58.4%, respectively) versus more than two quadrants in 46.6% in group II. Residuals and RP rates were 41% and 15%, respectively (only in group II). Predictors for residuals were atticotomy (odds ratio [OR] 2.9, 95% confidence interval [CI] 1.3–6.7) and destruction of stapes (OR 4.3, 95% CI 1.7–10.5). Predictors for RP were eustachian tube extension (OR 6.8, 95% CI 1.7–26.8) and nonreconstructed atticotomy (OR 5.9, 95% CI 1.1–30.9). Conclusions: Young children with small CC had no recurrences. Residuals were more frequent in case of atticotomy and stapes destruction. RP occurred especially in cases of eustachian tube extension and if cartilage tympanoplasty was not performed. Tympanic and canal wall reinforcement should be considered in extensive CC.

Resume Les Dyskinesies ciliaires primitives (DCP, MIM 242650) constituent un groupe heterogene de maladies respiratoires genetiques (frequence 1/16 000) classiquement transmises selon un mode autosomique recessif. Dans la moitie des cas, la maladie est associee a un situs inversus (syndrome de Kartagener). Les DCP sont caracterisees par un defaut de l'epuration mucociliaire lie a des anomalies des cils, a l'origine d'infections respiratoires recidivantes. L'anomalie ciliaire la plus frequente et la premiere identifiee porte sur les bras de dyneine. Les DCP presentent ainsi une grande heterogeneite genetique et seuls trois genes ont ete identifies jusqu'a present chez quelques patients atteints de DCP avec absence des bras externes de dyneine. Les principales manifestations respiratoires, nasales et bronchiques, les anomalies de la structure et de la fonction ciliaires, ainsi que les bases moleculaires des DCP sont presentees dans cette revue.

Introduction: Primary ciliary dyskinesia (PCD) is a rare disease classically transmitted as an autosomal recessive trait and characterised by recurrent airway infections due to abnormal ciliary structure and function. To date, only two autosomal genes, DNAI1 and DNAH5 encoding axonemal dynein chains, have been shown to cause PCD with defective outer dynein arms. Here, we investigated one non-consanguineous family in which a woman with retinitis pigmentosa (RP) gave birth to two boys with a complex phenotype combining PCD, discovered in early childhood and characterised by partial dynein arm defects, and RP that occurred secondarily. The family history prompted us to search for an X linked gene that could account for both conditions. Results: We found perfect segregation of the disease phenotype with RP3 associated markers (Xp21.1). Analysis of the retinitis pigmentosa GTPase regulator gene ( RPGR ) located at this locus revealed a mutation (631_IVS6+9del) in the two boys and their mother. As shown by study of RPGR transcripts expressed in nasal epithelial cells, this intragenic deletion, which leads to activation of a cryptic donor splice site, predicts a severely truncated protein. Conclusion: These data provide the first clear demonstration of X linked transmission of PCD. This unusual mode of inheritance of PCD in patients with particular phenotypic features (that is, partial dynein arm defects and association with RP), which should modify the current management of families affected by PCD or RP, unveils the importance of RPGR in the proper development of both respiratory ciliary structures and connecting cilia of photoreceptors.

Twenty-one patients, including our two cases, with variable clinical phenotype, ranging from mild learning disability to severe congenital malformations or overlapping features with DiGeorge/velocardiofacial syndromes (DG/VCFS), have been shown to have a chromosome duplication 22q11 of the region that is deleted in patients with DG/VCFS. The reported cases have been identified primarily by interphase FISH and could have escaped identification and been missed by routine cytogenetic analysis. Here we report on two inherited cases, referred to us, to rule out 22q11 microdeletion diagnosis of VCFS. The first patient was a 2-month-old girl, who presented with cleft palate, minor dysmorphic features including short palpebral fissures, widely spaced eyes, long fingers, and hearing loss. Her affected mother had mild mental retardation and learning disabilities. The second patient was a 7½-year-old boy with velopharyngeal insufficiency and mild developmental delay. He had a left preauricular tag, bifida uvula, bilateral fifth finger clinodactyly, and bilateral cryptorchidism. His facial features appeared mildly dysmorphic with hypertelorism, large nose, and micro/retrognathia. The affected father had mild mental retardation and had similar facial features. FISH analysis of interphase cells showed three TUPLE1-probe signals with two chromosome-specific identification probes in each cell. FISH analysis did not show the duplication on the initial testing of metaphase chromosomes. On review, band q11.2 was brighter on one chromosome 22 in some metaphase spreads. The paucity of reported cases of 22q11.2 microduplication likely reflects a combination of phenotypic diversity and the difficulty of diagnosis by FISH analysis on metaphase spreads. These findings illustrate the importance of scanning interphase nuclei when performing FISH analysis for any of the genomic disorders. © 2005 Wiley-Liss, Inc.

Objectives were 1) to review the presentation, natural history, and management of subglottic hemangioma; 2) to assess the affect of five variables (age, gender, degree of subglottic narrowing, location and extent of subglottic hemangioma, and lack or presence of other hemangioma) and the outcome of six different treatment modalities (conservative monitoring, corticosteroid, laser surgery, tracheotomy, laryngotracheoplasty, and interferon) in the management of subglottic hemangioma; and 3) to present specific guidelines to help determine the best possible treatment modality at the time of initial presentation.Retrospective review in the setting of three tertiary care pediatric medical centers.Methods included 1) extensive review of the literature; 2) a systematic review with respect to age, gender, presentation, associated medical problems, location and degree of subglottic narrowing, initial treatment, need for subsequent treatments, outcome, complications, and prognosis; and 3) statistical analysis to determine the effect of five variables (age, gender, degree of subglottic narrowing, location and extent of subglottic hemangioma, and lack or presence of other hemangioma) and the outcome of six different treatment modalities (conservative monitoring, corticosteroid, laser surgery, tracheotomy, laryngotracheoplasty, and interferon).In all, 116 patients with a mean age of 4.7 months were treated. The most common location of subglottic hemangioma was the left side. The range of subglottic narrowing was 10% to 99% (mean percentage, 65%). Twenty-six patients (22%) were managed with a single treatment modality, which included conservative monitoring (n = 13), corticosteroid (n = 11), and tracheotomy (n = 2). Ninety patients (78%) required multimodality treatments. Overall, the treatments included conservative monitoring (n = 13), corticosteroid (n = 100), tracheotomy (n = 32), CO2 laser (n = 66), interferon (n = 5), and laryngotracheoplasty (n = 25). Complication rates included the following: conservative monitoring (none), corticosteroid (18%), tracheotomy (none), CO2 laser (12%), interferon (20%), and laryngotracheoplasty (20%). The following variables showed statistical significance in the outcome of different treatment modality: 1) degree of subglottic narrowing (P.001), 2) location of subglottic hemangioma (P.01), and 3) presence of hemangioma in other areas (P.005). Gender (P.05) and age at the time of presentation (P.06) did not show any statistical significance on the outcome of the treatments.Each patient should be assessed comprehensively, and treatment should be individualized based on symptoms, clinical findings, and experience of the surgeon. The authors presented treatment guidelines in an attempt to rationalize the management of subglottic hemangioma and to help determine the best possible treatment modality at the time of initial presentation.

Objective: To assess cidofovir plasma concentration after intralesional airway administration for recurrent respiratory papillomatosis. Design: Prospective study. Setting: Tertiary care teaching hospital. Patients and Method: The study comprised 21 patients (10 children and 11 adults). Plasma samples were collected at 10 and 45 minutes (T10, T45) or at 10 and 60 minutes (T10, T60) after injection. The measurements of cidofovir were performed using a high-performance liquid chromatographic method. Results: Plasma samples were collected at T10 and T45 on 19 occasions from the children and on 17 from the adults. A linear relationship was found between plasma concentration and dose in children (mean dose 1.2 mg/kg; mean cidofovir plasma levels 0.91 and 0.81 μg/mL) but not in adults (mean dose 0.2 mg/kg; mean plasma levels 0.21 and 0.31 μg/mL). The same relationships were found between dose and area under the concentration/time curve (AUC). Four plasma samples were taken in children at T10 and T60: mean dose 1.2 mg/kg and mean plasma concentrations 1.11 and 1.24 μg/mL. Maximum plasma concentration averaged 34% (SD 11%) in children and 62% (SD 33%) in adults, with equivalent plasma level after intravenous infusion of the same dose. Conclusions: The cidofovir plasma levels were below those leading to toxicity. The levels and the AUC were dose dependent in children but not in adults. Diffusion from the injected site was greatest in a few adults and unpredictable. Because of the great individual variation in diffusion in adults, cidofovir should be used at less than the recommended intravenous dose to prevent any risk of systemic toxicity.

Primary ciliary dyskinesia (PCD) is an autosomal recessive disorder, characterized by chronic infections of the upper and lower airways, associated in 50% of cases with situs inversus, therefore, corresponding to Kartagener's syndrome. PCD is suspected on clinical features, including bronchitis, rhinosinusitis and chronic otitis media beginning in early childhood. The recurring infections eventually lead to bronchiectasis. The clinical features of PCD have been ascribed to primary defects in cilia, which lead to impairment of mucociliary clearance. Ciliary investigations looking for abnormalities in ciliary motion and ultrastructure can be easily performed at nasal level. Quantitative ultrastructural study of cilia is performed in cases of abnormal ciliary motion and/or clinical symptoms highly suggestive of PCD. In PCD, all or most of the cilia are abnormal, all bearing the same ultrastructural defects, mainly concerning dynein arms. In older children, the detection of a very low nasal NO output could also be useful for the diagnosis of PCD. As soon as the ciliary investigations are easy to perform at the nasal level, they could help for a better detection of PCD. This strategy could be especially useful in cases of atypical presentations, which are underestimated as a cause of recurrent airway infections. Diagnosis of PCD is important in order to prevent the development of bronchiectasis and to avoid any unnecessary procedure.

Resume But. – Evaluer l’efficacite d’un traitement par vincristine d’hemangiomes graves, compliques et corticoresistants. Patients et methodes. – Neuf nourrissons, huit filles et un garcon, atteints d’hemangiomes compliques ont recu un traitement par vincristine (VCR). Six enfants avaient une atteinte cervicofaciale segmentaire, severe et diffuse (3/6 avec atteinte laryngee et 2/6 tracheale obstructive, et 5/6 avec envahissement palpebral et orbitaire) ; un enfant avait des hemangiomes miliaires cutanes tres necrotiques et hemorragiques et une atteinte hepatique et renale ; deux autres avaient une hemangiomatose hepatique avec defaillance cardiaque. La resistance a des doses elevees de corticosteroides donnees au prealable durant des semaines ou mois etait notee sept fois, dont une fois avec un echappement a un traitement par corticoides puis par interferon α 2b pendant cinq mois. Deux enfants ont recu d’emblee la VCR. La dose etait de 1 mg/m 2 par voie intraveineuse pour chaque injection. Resultats. – Les injections, hebdomadaires au debut, etaient ensuite espacees selon la reponse : cinq a 25 injections (moyenne = 16) ont ete administrees, durant un mois et demi a huit mois (moyenne = 5 1/2 mois). L’efficacite a ete nette apres un mois de traitement dans sept cas, reelle mais plus lente et progressive dans les deux autres cas. Des effets secondaires transitoires moderes ont ete notes chez quatre patients. Discussion. – Les corticosteroides sont consideres comme le traitement de premiere intention. Mais la reponse therapeutique est tres inconstante ou transitoire avec rebond dans les formes graves. En cas d’echec des corticoides, le traitement par interferon α 2a ou 2b a donne un taux de reponse favorable de 90 %, mais la survenue de paraplegie spastique de pathogenie inconnue, a conduit a rechercher d’autres traitements pharmacologiques. La vincristine apparait dans notre etude comme un traitement efficace des hemangiomes compliques corticoresistants.

Resume Objectifs – Nous avons voulu verifier l'adequation aux criteres requis des parametres pharmacocinetiques/pharmacodynamiques d'Augmentin®, forme pediatrique, par mesure des concentrations d'amoxicilline et d'acide clavulanique dans le sang et le liquide auriculaire d'enfants ou de nourrissons. Methodes – Une etude pharmacocinetique, multicentrique, non comparative, ouverte, randomisee en cinq groupes selon l'heure des prelevements, a ete menee chez 36 enfants traites (80 mg/10 mg kg–1 j–1 en 3 prises pendant 3 jours). Les prelevements sanguins et auriculaires ont ete effectues simultanement. Les dosages ont ete realises par HPLC. Nous avons calcule : le pourcentage de penetration des 2 composes dans l'oreille moyenne ; le quotient inhibiteur dans le liquide auriculaire ; le temps pendant lequel les concentrations seriques et auriculaires etaient superieures a la CMI ; celui pendant lequel les quotients inhibiteurs auriculaires etaient superieurs a 5 ; le rapport des aires sous courbe sur la CMI serique et auriculaire. Resultats – Pour l'amoxicilline : les CA etaient de 7,23 ; 7,40 ; 7,08 ; 6,01 et 3,31 mg/l a 1 h, 2 h, 3 h, 8 h et 12 h. Les CS s'echelonnaient de 14,5 mg/l a 0,46 mg/l. Pour une CMI de 1 mg/l, ces valeurs correspondaient a un T > CMI de 66 % dans le sang et de 100 % dans le liquide auriculaire et un quotient inhibiteur maximum de 7,5 dans ce dernier. Pour l'acide clavulanique, les CA etaient de 0,58 ; 0,94 ; 0,62 ; 0,11 et 0,11 mg/l a 1 h, 2 h, 3 h, 8 h et 12 h (valeurs superieures a celles qui inhibent in vitro les betalactamases d'H. influenzae et de M. catarrhalis. Conclusion – Le schema etudie est en adequation avec les prerequis pharmacodynamiques des betalactamines.

Breathing pattern, gas exchange, and respiratory effort were assessed in five awake children with chronic stridor caused by laryngomalacia during spontaneous breathing (SB) and noninvasive mechanical ventilation (NIMV). During SB, the youngest children were able to maintain normal gas exchange at the expense of an increased work of breathing as assessed by calculated diaphragmatic pressure-time product (PTPdi), whereas the opposite was observed in the older children. NIMV increased tidal volume, from 8.77 +/- 2.04 ml/kg during SB to 11.67 +/- 2.52 ml/kg during NIMV, p = 0.04, and decreased respiratory rate, from 24.4 +/- 5.6 breaths/ min during SB to 16.6 +/- 0.9 breaths/min during NIMV, p = 0.04. NIMV unloaded the respiratory muscles as reflected by the significant reduction in PTPdi, from a mean value of 541.0 +/- 196.6 cm H(2)O x s x min(-1) during SB to 214.8 +/- 116.0 cm H(2)O x s x min(-1) during NIMV, p = 0.04. Therefore, NIMV successfully relieves the additional load imposed on the respiratory muscles. Long-term home NIMV was provided to a total of 12 children with laryngomalacia (including these five) and was associated with clinical improvement in sleep and growth.

Summary Introduction Fetal rhabdomyomas are very rare benign tumors with infrequent head and neck involvement. We present the first report of a case of a 3-year-old child with an infratemporal fossa location. Material and methods Case study. Discussion Given the volume of the tumor and its difficult-to-access location, medical treatment associating methotrexate and Velbe ® was proposed, but was not effective on the tumor volume after four courses. Surgical intervention via a combined intraoral and preauricular temporal approach allowed monoblock excision. There were no postoperative complications. Conclusion Medical treatment was unsuccessful. Surgical excision controlled the lesion with no recurrence since surgery. The main differential diagnosis is rhabdomyosarcoma.

Resume Introduction Les rhabdomyomes fœtaux sont des tumeurs benignes tres rares. Les atteintes cephaliques sont peu frequentes. Nous presentons le cas d’un enfant de trois ans avec une localisation a la fosse infratemporale, ce qui n’avait jamais ete decrit auparavant. Materiel et methode Etude d’un cas. Discussion Compte tenu du volume de la tumeur et de sa localisation d’acces difficile, un traitement medical associant methotrexate et Velbe ® a ete propose, sans efficacite sur le volume tumoral apres quatre cures. Une intervention par voie combinee endobuccale et temporale preauriculaire a ete pratiquee, permettant une exerese en monobloc. Les suites operatoires ont ete simples. Conclusion Le traitement medical n’a apporte aucun benefice. L’exerese chirurgicale a permis le controle de la lesion sans recidive a ce jour. Le principal diagnostic differentiel est le rhabdomyosarcome.

The aim of this study is to evaluate the prognostic factors of audiophonological results in cochlear implant in congenitally deaf patients. Between 1991 and 1996. 40 congenitally deaf children underwent cochlear implantation in our department, at an average age of 7 years (median: 5 years). The results of speech therapy were evaluated with a mean follow-up of 2 years and were classified according to four criteria: perception of sound, speech perception, speech production and the level of oral language. For each criterion, a score was established ranging from zero to four. These scores were weighted according to age such that the results before and after implantation only reflected the changes related to the implantation. The prognostic factors for good results were: a good level of oral communication before implantation, residual hearing, progressive deafness and implantation at a young age. On the other hand, poor prognostic factors were: the presence of behavioral disorders and poor communication skills prior to implantation. Overall, the major prognostic factor for a good outcome appeared to be the preoperative level of oral language, even if this was rudimentary.

To examine the indications and the results of surgical excision of severe subglottic hemangiomas.Retrospective study and case series.Four academic tertiary care centers of pediatric otolaryngology.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.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.All patients were successfully decannulated or extubated and have been free from recurrent hemangiomas.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.

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.