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J Robot Surg ; 11(2): 255-258, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-27822660


We report the first description of robotic-assisted Heller myotomy in children. The purpose of this study was to improve the safety of Heller myotomy by demonstrating, in two adolescent patients, the contribution of the robot to the different steps of this procedure. Due to the robot's freedom of movement and three-dimensional vision, there was an improvement in the accuracy, a gain in the safety regarding different key-points, decreasing the risk of mucosal perforation associated with this procedure.

Miotomia de Heller/métodos , Procedimentos Cirúrgicos Robóticos/métodos , Adolescente , Criança , Acalasia Esofágica/cirurgia , Doenças do Esôfago/cirurgia , Feminino , Miotomia de Heller/efeitos adversos , Humanos , Masculino , Fatores de Risco , Procedimentos Cirúrgicos Robóticos/efeitos adversos
Orphanet J Rare Dis ; 9: 206, 2014 Dec 11.
Artigo em Inglês | MEDLINE | ID: mdl-25496976


BACKGROUND: The aim of the present national prospective population-based study was to assess the early morbidity of esophageal atresia (EA). METHODS: All 38 multidisciplinary French centers that care for patients with EA returned a specific questionnaire about the 1-year outcome for each patient. This information was centralized, checked, and entered into a database. RESULTS: From the total population of 307 EA patients born in 2008 and 2009, data about the 1-year outcome were obtained from 301 (98%) patients, of whom 4% were lost to follow-up and 5% died. Medical complications occurred in 34% of the patients: anastomotic leaks (8%), recurrent tracheoesophageal fistula (4%), and anastomotic stenosis (22%); all of the latter group needed dilation (median, 2 dilations/patient). A new hospitalization was required for 59% of patients (2.5 hospitalizations/patient) for digestive (52%) or respiratory (48%) reasons. Twelve percent of patients required antireflux surgery at a median age of 164 days (range, 33-398 days), and 1% underwent an aortopexy for severe tracheomalacia. The weight/age Z-score was -0.8 (range, -5.5 to 3.7 months) at 12 months. Fifteen percent of patients were undernourished at 12 months of age, whereas 37% presented with respiratory symptoms and 15% had dysphagia at the last follow-up. Significant independent factors associated with medical complications were anastomotic esophageal tension (p = .0009) and presence of a gastrostomy (p = .0002); exclusive oral feeding at discharge was associated with a decreased risk of complications (p = .007). CONCLUSIONS: Digestive and respiratory morbidities remain frequent during the first year of life and are associated with difficult anastomosis and lack of full oral feeding.

Atresia Esofágica/diagnóstico , Atresia Esofágica/epidemiologia , Vigilância da População , Sistema de Registros , Atresia Esofágica/terapia , Feminino , Seguimentos , França/epidemiologia , Hospitalização/tendências , Humanos , Lactente , Recém-Nascido , Masculino , Vigilância da População/métodos , Fatores de Tempo , Resultado do Tratamento
J Pediatr Gastroenterol Nutr ; 53(3): 296-302, 2011 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-21865978


OBJECTIVE: To retrospectively assess, in a pediatric multicenter cohort, guidelines for the management of familial adenomatous polyposis (FAP). METHODS: Ten centers from the French-speaking Pediatric Gastroenterology Hepatology and Nutrition Group provided follow-up data on patients up to 18 years of age. Clinical records, genetic test results, endoscopy with histopathology examination, and therapeutic modalities were reviewed. RESULTS: A total of 70 children from 47 families were included. When initial consultation resulted from a surveillance program because of an affected family member, 12 of 59 children were already symptomatic. Among 11 patients whose initial consultation was based only on symptoms, families were unaware at the time of a familial FAP history for 7 children, whereas only 4 cases were sporadic. A panel of 27 different pathogenic adenomatous polyposis coli (APC) germ-line mutations and large genomic deletions were identified in 43 families. Extracolonic manifestations were found in half of the patients. As part of the standard practice for initial screening, the entire cohort underwent colonoscopy, which revealed adenoma above an intact rectosigmoid in 8 cases. Prophylactic colectomy was performed in 42 cases; high-grade dysplastic adenoma and 1 invasive carcinoma were detected in 6 children. For timing of surgery, indications were in accordance with recent international guidelines. CONCLUSIONS: Defining optimal screening and therapeutic modalities in pediatric FAP cohorts is a challenge. Specific advice for genetic screening, endoscopy surveillance, and type of surgery based on recent guidelines is recommended.

Polipose Adenomatosa do Colo/genética , Polipose Adenomatosa do Colo/patologia , Polipose Adenomatosa do Colo/cirurgia , Criança , Colectomia , Colonoscopia , Estudos de Avaliação como Assunto , Feminino , Seguimentos , Genes APC , Estudos de Associação Genética , Mutação em Linhagem Germinativa , Humanos , Masculino , Guias de Prática Clínica como Assunto , Estudos Retrospectivos , Inquéritos e Questionários
Orphanet J Rare Dis ; 5: 3, 2010 Feb 03.
Artigo em Inglês | MEDLINE | ID: mdl-20128925


OBJECTIVE: To document the epidemiological, clinical, histological and radiological characteristics of aggressive vascular abnormalities of bone in children. STUDY DESIGN: Correspondents of the French Society of Childhood Malignancies were asked to notify all cases of aggressive vascular abnormalities of bone diagnosed between January 1988 and September 2009. RESULTS: 21 cases were identified; 62% of the patients were boys. No familial cases were observed, and the disease appeared to be sporadic. Mean age at diagnosis was 8.0 years [0.8-16.9 years]. Median follow-up was 3 years [0.3-17 years]. The main presenting signs were bone fracture (n = 4) and respiratory distress (n = 7), but more indolent onset was observed in 8 cases. Lung involvement, with lymphangiectasies and pleural effusion, was the most frequent form of extraosseous involvement (10/21). Bisphosphonates, alpha interferon and radiotherapy were used as potentially curative treatments. High-dose radiotherapy appeared to be effective on pleural effusion but caused major late sequelae, whereas antiangiogenic drugs like alpha interferon and zoledrenate have had a limited impact on the course of pulmonary complications. The impact of bisphosphonates and alpha interferon on bone lesions was also difficult to assess, owing to insufficient follow-up in most cases, but it was occasionally positive. Six deaths were observed and the overall 10-year mortality rate was about 30%. The prognosis depended mainly on pulmonary and spinal complications. CONCLUSION: Aggressive vascular abnormalities of bone are extremely rare in childhood but are lifethreatening. The impact of anti-angiogenic drugs on pulmonary complications seems to be limited, but they may improve bone lesions.

Doenças Ósseas/epidemiologia , Malformações Vasculares/epidemiologia , Doenças Ósseas/diagnóstico , Doenças Ósseas/tratamento farmacológico , Criança , Difosfonatos/uso terapêutico , Feminino , França/epidemiologia , Humanos , Interferon-alfa/uso terapêutico , Estimativa de Kaplan-Meier , Masculino , Estudos Retrospectivos , Malformações Vasculares/diagnóstico , Malformações Vasculares/tratamento farmacológico
Inflamm Bowel Dis ; 10(6): 745-50, 2004 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-15626892


Infliximab, a monoclonal antibody against tumor necrosis factor-alpha, has been shown to be effective for the treatment of refractory Crohn's disease in adult patients, but experience in pediatrics is limited. This retrospective study included 88 children and adolescents, 39 girls and 49 boys, with a median age of 14 years (range 3.3-17.9). Infliximab was indicated for active disease (66%) and/or fistulas (42%) that were refractory to corticosteroids (70%), and/or other immunosuppressive (82%) agents, and/or parenteral nutrition (20%). Patients received 1 to 17 infusions (median 4) of 5 mg/kg (range 3.8-7.3) of infliximab during a median time period of 4 months (1-17 months). Infusion reaction was noted in 13 patients (15%), with a total of 16 reactions in 450 infusions (4%). At Day 90 after the first infusion of infliximab, symptoms improved in 49% of patients, whereas 29% of patients were in remission and 13% of patients relapsed. From Day 0 to Day 90, Harvey-Bradshaw score decreased from 7.5 to 2.8 (P < 0.001), C-reactive protein from 36 to 16 mg/L (P < 0.01), and 1-hour erythrocyte sedimentation rate from 35 to 17 mm (P < 0.01). Dosage of corticosteroids decreased from to 0.59 to 0.17 mg/kg/d (P < 0.001); 53% of patients could be weaned of corticosteroids and 92% of parenteral nutrition. Treatment with infliximab is well tolerated and effective in most children and adolescents with Crohn's disease that is refractory to conventional immunosuppressive therapy. Nevertheless, long-term efficacy remains to be shown, and further studies are urgently needed to precisely determine the best modality of continuing treatment.

Anticorpos Monoclonais/uso terapêutico , Doença de Crohn/tratamento farmacológico , Fármacos Gastrointestinais/uso terapêutico , Fator de Necrose Tumoral alfa/antagonistas & inibidores , Adolescente , Anticorpos Monoclonais/administração & dosagem , Anticorpos Monoclonais/efeitos adversos , Criança , Pré-Escolar , Doença de Crohn/patologia , Europa (Continente) , Feminino , Fármacos Gastrointestinais/administração & dosagem , Fármacos Gastrointestinais/efeitos adversos , Humanos , Lactente , Infliximab , Infusões Intravenosas , Masculino , Registros Médicos , Estudos Retrospectivos , Resultado do Tratamento