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1.
Prog Urol ; 32(5): 312-318, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34688536

RESUMEN

OBJECTIVE: To report experience and technical refinements with the Koyanagi urethroplasty for proximal hypospadias. METHODS: A retrospective study was conducted between 2004 and 2017. Medical records of patients treated by the Koyanagi technique were investigated. The penile ventral skin was closed either with a Byars flap or using an "Ombredanne's chasuble" (OC). The cohort was divided chronologically into two groups of the same number of patients (early -E- and late experience group-LEG-). Demographics, anatomical findings and surgical outcomes were compared between groups using univariate analysis. A logistic regression was performed to assess factors associated with the occurrence of a postoperative urethrocutaneous fistula or urethroplasty dehiscence (UD). RESULTS: The Koyanagi urethroplasty was performed in 67 patients, 33 in the EEG and 34 in the LEG. The overall surgical complication rate was 64.2% (n=43), including 42 fistula or UD, higher in the EEG (81.8%) than in the LEG (44.1%, P<0.01). However, in the LEG, patients underwent the surgery older and the use of OC more frequent. After multivariate analyses, factors associated with a postoperative fistula or UD were the year of surgery (OR=0.71 [0.53-0.96] P=0.02), the age at surgery (OR=1.11 [1.01-1.22], P=0.03); contrary to the skin coverage method or the stenting duration (P>0.05). An urethral stenosis occurred in 1 patient (1.5%). At last follow-up, 64.2% of patients required a further procedure and 80.6% of urethral meatus were glandular. CONCLUSION: In this study the complication rate, particularly the urethrocutaneous fistula, remained high. Urethral stenosis, were rare but the follow-up was too short to clearly identified them. Considering as a 2 stage procedure koyanagi uretroplasty allows to obtain finally good results. LEVEL OF EVIDENCE: III.


Asunto(s)
Fístula , Hipospadias , Estrechez Uretral , Femenino , Fístula/etiología , Humanos , Hipospadias/complicaciones , Hipospadias/cirugía , Lactante , Masculino , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Uretra/cirugía , Estrechez Uretral/cirugía , Procedimientos Quirúrgicos Urológicos Masculinos/métodos
2.
Prog Urol ; 32(5): 319-325, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34920919

RESUMEN

AIMS: Evaluation of repeated (at least 4) intra-detrusor injections of toxin botulinum A (IDI-TBA) for neurogenic bladder in a pediatric cohort. METHODS: Patients who underwent at least 4 IDI-TBA between 2005 and 2017 for neurogenic bladder related issues were included (detrusor overactivity and low compliance). Clinical and cystometric data were collected before and after the first injection and after the last injection. The primary endpoint was the proportion of patients with non-abnormal cystometry (no detrusor overactivity and normal compliance). Secondary outcomes were the evolution of the observed bladder capacity/expected ratio, surgical complications and acquired kidney impairment. RESULTS: From the 832 patients referred to our institution for neurogenic bladder, 48 underwent IDI-TBA, and 17 at least 4 injections. Among them, a total of 95 procedures were performed (median per patient 5 [4-8]). While the first injection had a significant effect for 82.3% patients, the last injection improved the medical condition for only 53.0% cases. The bladder capacity ratio, initially 36.1%, increased to 80.3% after the first injection but decreased to a level of 57.1% at last. After a median follow-up of 57 [34-102] months, no severe complications were reported but 11.8% of patients presented with repeated pyelonephritis. A bladder augmentation surgery was finally indicated for 35.3% cases. CONCLUSIONS: Despite a low complication rate and impressive cystometric results after the first injection, IDI-TBA efficacy decreased with time and repetition. These findings prone a long-term follow-up and a "à-la-carte" management of this specific population depending on the long-term response to IDI-TBA.


Asunto(s)
Toxinas Botulínicas Tipo A , Fármacos Neuromusculares , Vejiga Urinaria Neurogénica , Vejiga Urinaria Hiperactiva , Administración Intravesical , Toxinas Botulínicas Tipo A/uso terapéutico , Niño , Femenino , Humanos , Inyecciones , Masculino , Fármacos Neuromusculares/efectos adversos , Resultado del Tratamiento , Vejiga Urinaria Neurogénica/tratamiento farmacológico , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Hiperactiva/complicaciones , Vejiga Urinaria Hiperactiva/tratamiento farmacológico , Urodinámica
3.
Pediatr Blood Cancer ; 66(6): e27641, 2019 06.
Artículo en Inglés | MEDLINE | ID: mdl-30746839

RESUMEN

OBJECTIVES: According to the Renal Tumor Study Group (RTSG) of the International Society of Paediatric Oncology (SIOP), diagnostic biopsy of renal tumors prior to neoadjuvant chemotherapy is not mandatory unless the presentation is atypical for a Wilms tumor (WT). This study addresses the relevance of this strategy as well as the accuracy and safety of image-guided needle biopsy. METHODS: Clinical, radiological, and pathological data from 317 children (141 males/176 females, mean age: 4 years, range, 0-17.6) consecutively treated in one SIOP-affiliated institution were retrospectively analyzed. RESULTS: Presumptive chemotherapy for WT was decided for 182 patients (57% of the cohort), 24 (8%) were operated upfront, and 111 (35%) were biopsied at diagnosis. A non-WT was confirmed after surgery in 5/182 (3%), 11/24 (46%), and 28/111 (25%), respectively. Age at diagnosis was the most commonly (46%) used criterion to go for biopsy but a nine-year threshold should be retrospectively considered more relevant. Tumor volumes of clear cell sarcoma of the kidney and WT were significantly higher than those of other tumors (P = 0.002). The agreement between core-needle biopsy (CNB) and final histology was 99%. No significant morbidity was associated with CNB. CONCLUSION: The use of SIOP criteria to identify patients eligible for presumptive WT neoadjuvant chemotherapy or upfront surgery avoided biopsy in 65% of children and led to a 97% rate of appropriate preoperative chemotherapy. Image-guided CNB is a safe and accurate diagnostic procedure. The relevance of SIOP biopsy criteria might be improved by using an older age threshold (9 years instead of 6 years) and by adding initial tumor volume.


Asunto(s)
Carcinoma de Células Renales/diagnóstico , Guías como Asunto , Neoplasias Renales/diagnóstico , Selección de Paciente , Tumor de Wilms/diagnóstico , Adolescente , Biopsia , Carcinoma de Células Renales/cirugía , Niño , Preescolar , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Neoplasias Renales/cirugía , Masculino , Terapia Neoadyuvante , Estadificación de Neoplasias , Pronóstico , Curva ROC , Estudios Retrospectivos , Tumor de Wilms/cirugía
4.
Prog Urol ; 24(1): 39-45, 2014 Jan.
Artículo en Francés | MEDLINE | ID: mdl-24365627

RESUMEN

INTRODUCTION: The urological management of urinary incontinence in neurogenic bladder due to spinal cord lesions in children is intended to achieve social continence while preserving the upper urinary tract, combining clean intermittent catheterization with anticholinergic agents. The objective of this study was to report the results on continence of endoscopic management of bladder and/or sphincter of children with failure or intolerance to first intention therapy. PATIENTS AND METHODS: Of the 364 children followed for neurologic bladder in our institution, 22 required endoscopic management between 2000 and 2012. Urinary incontinence was related to detrusor overactivity in 16 children and sphincter deficiency in 13 children, requiring one or several intradetrusor injections of botulinium toxin-A (BTA) and/or dextranomer/hyaluronic acid (Dx/Ha) injection in the bladder neck. Continence was reassessed between six and eight weeks after the last injection using the Schulte-Baukloh score. RESULTS: At the end of the mean follow-up of four years, 16 children received 54 injections of BTA and 13 children had 24 injections of Dx/Ha. Social continence, defined as a score between 0 and 1, was acquired quickly after injection of BTA and required to repeat the injections every 8.7 months (6-12) with a very low morbidity. After the first injection of Dx/Ha, 69% of the children improved significantly their incontinence score (from 1 to 0 or from 2 or 3 to 1) with better results for girls. CONCLUSION: An appropriate endoscopic management has enabled an improvement of the continence of two-thirds of children who fail first intention treatment for their neurogenic bladder. This is an alternative to delay or avoid major surgery.


Asunto(s)
Toxinas Botulínicas Tipo A/administración & dosificación , Cistoscopía , Dextranos/administración & dosificación , Ácido Hialurónico/administración & dosificación , Fármacos Neuromusculares/administración & dosificación , Incontinencia Urinaria/tratamiento farmacológico , Adolescente , Niño , Femenino , Humanos , Masculino , Enfermedades de la Médula Espinal/complicaciones , Vejiga Urinaria Neurogénica/complicaciones , Incontinencia Urinaria/etiología , Adulto Joven
5.
Rev Stomatol Chir Maxillofac ; 113(6): 442-7, 2012 Dec.
Artículo en Francés | MEDLINE | ID: mdl-22342887

RESUMEN

INTRODUCTION: Foregut duplication is a heterotopy of the digestive mucosa. The tongue localization is relatively uncommon, it presents as a cystic lesion. We report a series of five patients presenting with cysts of the tongue. PATIENTS AND METHODS: This retrospective study was made on cases of foregut duplication of the lingual area, diagnosed between 1977 and 2008. We documented the patient's age, gender, symptoms, lesion localization, clinical radiological and pathological features, treatment, and outcome. RESULTS: Four boys and one girl were included. Two cysts were diagnosed during antenatal screening, two during early infancy, and one at the age of 12 (after infectious complication). In every case, the diagnosis could be confirmed only after surgical removal. DISCUSSION: Foregut duplication of the tongue is rare and its incidence is probably underestimated. This diagnosis should be suggested in case of congenital intraoral cysts. Treatment is surgical excision, and pathological examination confirms the diagnosis.


Asunto(s)
Coristoma/congénito , Quistes/congénito , Mucosa Gástrica/anomalías , Enfermedades de la Lengua/congénito , Niño , Femenino , Enfermedades Fetales/diagnóstico por imagen , Humanos , Lactante , Imagen por Resonancia Magnética , Masculino , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Ultrasonografía Prenatal
6.
Prog Urol ; 22(5): 291-300, 2012 May.
Artículo en Francés | MEDLINE | ID: mdl-22515926

RESUMEN

OBJECTIVE: To clarify bladder and bowel function of children with lipomas of the conus, without, before and after neurosurgery. PATIENTS AND METHODS: Retrospective analysis of 114 children with a lipomas of the conus, followed in our pediatric neuro-urology department from 1993 to 2010. Several data were collected: bladder and bowel symptoms, bladder and anorectal continence, neurosurgical indication and age, clinical modification after neurosurgery, investigations carried out in pre- and post-surgery treatment, associated bladder and bowel treatment. RESULTS: Forty-nine of the 77 children (63.6%) operated on had never been seen before surgery in our neuro-urology department. Seventy-seven children (67.5%) underwent a neuro-surgery, 60% indicated due to a neurogenic bladder. Before neurosurgery, 66 children (85.7%) had spontaneous miction. Five children (6.5%) had bladder intermittent catheterization. Forty of these patients (56.3%) were continent. After neurosurgery and a specialized consultation in neuro-urology, 54 children (70.1%) were continent. Thirty-seven children (48%) had spontaneous miction. Thirty-seven children (48%) had bladder intermittent catheterization and drug of overactive detrusor. Fifty-two children (67.5%) were constipated after surgery. Seventy-seven percent of the treatments for bowel symptoms were effective in terms of continence. CONCLUSION: The existence of a neurogenic bladder was one of the main indications for neurosurgery. These results suggest that the complexity of care requires neurosurgical, urological surgeon and neuro-urology physician to achieve the explorations and urinary and digestive treatment in order to preserve renal function and both continences.


Asunto(s)
Lipoma/complicaciones , Disrafia Espinal/cirugía , Neoplasias de la Columna Vertebral/complicaciones , Vejiga Urinaria Neurogénica/etiología , Niño , Estreñimiento/etiología , Incontinencia Fecal/etiología , Femenino , Humanos , Lipoma/congénito , Lipoma/cirugía , Masculino , Complicaciones Posoperatorias , Estudios Retrospectivos , Disrafia Espinal/etiología , Neoplasias de la Columna Vertebral/congénito , Neoplasias de la Columna Vertebral/cirugía , Cateterismo Urinario , Micción , Trastornos Urinarios/etiología
7.
Pediatr Blood Cancer ; 57(3): 429-34, 2011 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-21370434

RESUMEN

BACKGROUND: Ovarian teratoma (OT) is the most common ovarian neoplasm in children. Oophorectomy has been the standard treatment but may impair fertility. The aim of this study was to investigate the feasibility and outcome of ovarian-sparing surgery (OSS) for OT. PROCEDURE: We retrospectively studied all children treated for OT at a pediatric teaching hospital in Paris, France, between March 1992 and July 2006. OSS was performed when deemed technically feasible in patients who had no lymphadenopathy by preoperative imaging or surgical exploration, normal tumor marker levels, and calcifications on radiographs. RESULTS: We identified 30 patients, including 29 with unilateral OT and 1 with synchronous bilateral OT. Emergent surgery was performed in five patients, among whom four had ovarian torsion requiring oophorectomy and one underwent OSS. Of the 26 OTs in the 25 remaining patients, 10 were managed with OSS and 16 with oophorectomy. Subsequently, ultrasound monitoring detected OT development in the contralateral ovary in 4 (14%) patients, after a median of 3 years (range, 1-14 years); OSS was performed in all four cases. The patient with bilateral synchronous OT, managed by OSS initially, underwent unilateral oophorectomy 3 years later for a recurrence. Overall OSS was performed for 15 (42%) OTs. CONCLUSIONS: Our results suggest recommendations for preserving fertility whenever possible without compromising the oncological prognosis. In particular, OSS should be reserved for patients who meet all criteria for localized mature teratoma. Long-term follow-up is crucial.


Asunto(s)
Infertilidad Femenina/prevención & control , Neoplasias Ováricas/cirugía , Teratoma/cirugía , Niño , Preescolar , Femenino , Fertilidad , Estudios de Seguimiento , Humanos , Neoplasias Ováricas/complicaciones , Neoplasias Ováricas/diagnóstico por imagen , Estudios Retrospectivos , Tasa de Supervivencia , Teratoma/diagnóstico por imagen , Resultado del Tratamiento , Ultrasonografía
8.
Arch Pediatr ; 28(3): 226-233, 2021 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-33674188

RESUMEN

INTRODUCTION: Multiple intestinal atresia (MIA) is a rare cause of neonatal intestinal obstruction. To provide an overview of the current prenatal, surgical, and nutritional management of MIA, we report our experience and a literature review of papers published after 1990. METHODS: All cases of isolated MIA (non-hereditary, not associated with apple-peel syndrome or gastroschisis) treated at our institution between 2005 and 2016 were reviewed and compared with cases found in the literature. RESULTS: Seven patients were prenatally suspected of having intestinal obstruction and were postnatally diagnosed with MIA, with a mean 1.7 (1-2) resections-anastomoses (RA) and 6 (1-10) strictureplasties performed, resulting in a mean resected bowel length of 15.1cm (15-25 cm). Median time to full oral feed was 46 days (14-626 days). All patients were alive and none had orality disorder after a mean follow-up of 3.1 years (0.2-8.1 years). Three surgical strategies were found in the literature review: multiple RA (68%, 34/50) including Santulli's technique in four of 34 (12%) and anastomoses over a transanastomotic tube (32%, 16/50), with a 98% survival rate, and short-bowel syndrome for only two patients. CONCLUSION: Bowel-sparing surgery and appropriate medical management are key to ensuring a favorable nutritional and gastrointestinal outcome and a good prognosis. Prenatal assessment and standardization of the surgical course of treatment remain challenging.


Asunto(s)
Atresia Intestinal/terapia , Atención Perinatal/normas , Mejoramiento de la Calidad , Terapia Combinada , Procedimientos Quirúrgicos del Sistema Digestivo/métodos , Procedimientos Quirúrgicos del Sistema Digestivo/normas , Femenino , Estudios de Seguimiento , Humanos , Recién Nacido , Atresia Intestinal/diagnóstico , Masculino , Apoyo Nutricional/métodos , Apoyo Nutricional/normas , Atención Perinatal/métodos , Embarazo , Estudios Retrospectivos , Resultado del Tratamiento , Ultrasonografía Prenatal
9.
J Visc Surg ; 157(3 Suppl 2): S77-S85, 2020 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-32331850

RESUMEN

The main objectives of the reform of the 3rd cycle of medical studies in France that was instituted in 2017 after eight years of preparation, are to train future specialists in a consistent and equitable fashion and to replace the previous time-based qualification by training based on the progressive acquisition of skills. This reform was an opportunity for the 13 different French surgical specialty Colleges involved to share reflections on what a surgeon actually was and to define training in surgical sub-specialties. The current reform is well adapted to these specifications and has fostered training models that are consistent with each other. This article discusses the historical construction of this reform, what will change in the training of future surgeons, as well as some points that warrant caution. The third cycle reform has also triggered a reform of the second cycle, which is expected to come into force for the 2020 academic year. Its objective will be to eliminate the guillotine effect created by the National Classifying Examinations and to allow students to better understand and test their desire and skills for a given specialty. It will be up to these same surgical Colleges to determine how to do this for the sub-specialties of the "surgery" discipline.


Asunto(s)
Competencia Clínica , Curriculum , Educación Médica/organización & administración , Cirugía General/educación , Especialidades Quirúrgicas/educación , Francia , Humanos
10.
J Pediatr Urol ; 14(6): 558-564, 2018 12.
Artículo en Inglés | MEDLINE | ID: mdl-30126745

RESUMEN

BACKGROUND: The radical soft-tissue mobilization (RSTM, or Kelly repair) is an anatomical reconstruction of bladder exstrophy generally performed as a second part of a two-step strategy, following successful neonatal bladder closure. OBJECTIVE: The objective of this study is to determine the feasibility of a combined procedure of delayed bladder closure and RSTM in one stage without pelvic osteotomy, in both primary and failed initial closure. DESIGN, SETTING, AND PARTICIPANTS: From 11/2015 to 01/2018, 27 bladder exstrophy patients underwent combined bladder closure with RSTM by the same surgical team at four cooperating tertiary referral centers for bladder exstrophy, including 20 primary repairs (delayed bladder closure, median age 3.0m [0.5-37m]) and seven secondary repairs after failed attempt at neonatal closure, median age 10m [8-33m]. INTERVENTION: RSTM included full mobilization of the bladder plate, urogenital diaphragm, and corpora cavernosa from the medial pelvic walls, followed by anatomical reconstruction with antireflux procedure, bladder closure, urethrocervicoplasty, muscle sphincter approximation, and penile/clitoral reconstruction. OUTCOME MEASUREMENTS: The main criteria were bladder dehiscence or prolapse. Secondary outcomes included bladder neck fistula or urethral fistula, urethral stenosis, and parietal hernia. Continence and voiding have not been addressed at this stage. RESULTS AND LIMITATIONS: All bladder exstrophy cases were successfully closed without osteotomy, with no case of bladder dehiscence after 12 m [3-30] follow-up. COMPLICATIONS: Urethral fistula or stenosis occurred in eight patients: 4/5 fistulae closed spontaneously in less than 3 months; four urethral stenoses were successfully treated with 1-3 sessions of endoscopic high-pressure balloon dilatation or meatoplasty; one patient with persistent bladder neck fistula is currently awaiting repair. Although the follow-up is short, it does allow examination of the main outcome criterion, namely bladder dehiscence, which is usually expected to happen very early after surgery. CONCLUSION: The Kelly RSTM can be safely combined with delayed bladder closure without osteotomy in both primary and redo cases in classic bladder exstrophy.


Asunto(s)
Extrofia de la Vejiga/cirugía , Vejiga Urinaria/cirugía , Preescolar , Estudios de Factibilidad , Femenino , Humanos , Lactante , Recién Nacido , Masculino , Estudios Retrospectivos , Resultado del Tratamiento , Procedimientos Quirúrgicos Urológicos/métodos
11.
J Visc Surg ; 154(3): 175-183, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-27888039

RESUMEN

OBJECTIVE: Total small-intestinal volvulus with malrotation (TSIVM) classically presents in the neonatal period; it occurs much less frequently in the adult and is often misdiagnosed. Prognosis is directly related to the degree and duration of intestinal ischemia. Our goal is to describe our experience with TSIVM in the adult, to identify any specific findings and to discuss its management. METHOD: Eleven patients who had undergone surgery for TSIVM at three centers between 1992 and 2012 were included. Surgery was performed as an emergency for five patients and surgery was elective for six. RESULTS: Mean follow-up was 63 months (range: 12-270). Six patients had had previous abdominal surgery. In nine cases, the diagnosis of TSIVM was made preoperatively, mainly by CT scan in eight cases. Seven patients had associated congenital failure of retroperitoneal fixation of the right colon and all of these underwent a Ladd procedure. The mortality rate was zero. Of the five patients who underwent emergency surgery, three required intestinal resections, one of whom developed a short bowel syndrome. The six patients who underwent surgery electively had no surgical complications. CONCLUSION: TSIVM is a very unusual finding in adult patients. The diagnosis can be made by CT scan with IV and oral contrast, but it often comes to light only at the time of surgery, even though the patients have often had recurrent episodes of abdominal symptomatology that dated back to childhood. The Ladd procedure, consisting of division of Ladd's bands, widening of the mesentery, and incidental appendectomy, remains the standard surgical repair. Digestive surgeons who care for adults should be familiar with this procedure, and it should be performed, as often as possible, with the assistance of a pediatric surgeon.


Asunto(s)
Anomalías del Sistema Digestivo/cirugía , Vólvulo Intestinal/cirugía , Intestinos/anomalías , Laparoscopía , Adolescente , Adulto , Anciano , Anomalías del Sistema Digestivo/diagnóstico por imagen , Anomalías del Sistema Digestivo/etiología , Procedimientos Quirúrgicos Electivos/métodos , Femenino , Estudios de Seguimiento , Francia , Humanos , Vólvulo Intestinal/diagnóstico por imagen , Vólvulo Intestinal/etiología , Laparoscopía/métodos , Tiempo de Internación , Masculino , Persona de Mediana Edad , Tempo Operativo , Estudios Retrospectivos , Resultado del Tratamiento
13.
Ann Urol (Paris) ; 40(1): 28-38, 2006 Feb.
Artículo en Francés | MEDLINE | ID: mdl-16551004

RESUMEN

Pyelo-ureteral junction abnormalities in children are most frequently discovered by prenatal ultrasound investigation. Most pyeloplasties by resection-anastomosis of the pyelo-ureteral junction are performed in young infants, usually with a posterior approach, patient prone. The indication and type of urinary pyelic drain remain debated: simple nephrostomy or double J drain. Ureteral-caliceal anastomosis is an interesting approach to rare surgical reoperations, particularly with major pyelo-ureteral dilatation. Laparoscopic surgery may prove usefulness in less small children. Endopyelotomy gives good results for post-operative stenoses.


Asunto(s)
Anomalías Múltiples/cirugía , Pelvis Renal/anomalías , Pelvis Renal/cirugía , Uréter/anomalías , Uréter/cirugía , Anomalías Múltiples/diagnóstico , Niño , Preescolar , Humanos , Lactante , Procedimientos Quirúrgicos Urológicos/métodos
14.
Gynecol Obstet Fertil ; 33(4): 228-31, 2005 Apr.
Artículo en Francés | MEDLINE | ID: mdl-15894207

RESUMEN

With the progress of foetal ultrasonography, diagnosis of foetal tumors can be made before birth. We report five cases of prenatally detected neuroblastoma of the adrenal glands. Diagnosis was made during the third trimester in all cases. At birth no clinical sign related to the tumor was present, and urinary catecholamines were within normal ranges. On ultrasound scans the tumor was cystic in 1 case, solid in 2 cases and of mixed echogenicity in 2 cases. All children were operated on without pre or postoperative chemotherapy. In each setting the tumor was a stage I neuroblastoma according to the Evans classification. All children are alive and disease-free with a follow-up of 32 months at 14 years.


Asunto(s)
Neoplasias de las Glándulas Suprarrenales/diagnóstico por imagen , Enfermedades Fetales/diagnóstico por imagen , Neuroblastoma/diagnóstico por imagen , Ultrasonografía Prenatal , Neoplasias de las Glándulas Suprarrenales/cirugía , Femenino , Humanos , Masculino , Neuroblastoma/cirugía , Embarazo
15.
Arch Pediatr ; 12(4): 391-6, 2005 Apr.
Artículo en Francés | MEDLINE | ID: mdl-15808427

RESUMEN

UNLABELLED: Achalasia of the cardia is rare in children. We report our experience in the management of 20 children with achalasia. PATIENTS: Twenty children (seven girls including two sisters) with achalasia were studied, seven of them had a morbid association. Age at diagnosis ranged from eight months to 18 years (med: 6.4 yrs). Duration of symptoms prior to diagnosis ranged from one to 62 months (med: 8 months). Regurgitations, weight loss and recurrent pneumonias were the most common presenting symptoms. Diagnosis was established using esophageal manometry, which showed aperistalsis throughout the esophageal body with impaired relaxation of lower esophageal sphincter in all children, and chest x-ray and barium esophagram, which were abnormal in 11 and 18 children respectively. OUTCOME: Nifedipine used in nine children was ineffective. Two balloon dilatations were performed in one child with a poor result. Surgery with Heller's myotomy combined with an antireflux procedure was performed in 19 children. One child died six months later due to an hypoglycemic coma. During the follow-up (6 months-21 years) symptoms reappeared in five children. Seventy-two percent of the children had no symptoms one year after the surgery and 45%, five years after. Esophageal manometry performed after surgery in seven children showed a normal lower esophageal sphincter pressure but with impaired relaxation, and an aperistalsis throughout the esophageal body. These results justify prolonged follow-up of children with achalasia of the esophagus.


Asunto(s)
Acalasia del Esófago/diagnóstico , Acalasia del Esófago/terapia , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Factores de Tiempo
16.
Arch Pediatr ; 22(11): 1182-7, 2015 Nov.
Artículo en Francés | MEDLINE | ID: mdl-26412327

RESUMEN

The objective of this article is to inform health-care personnel, especially those of pediatric hospitals (pediatricians, surgeons, anesthetists, etc.), about the incidence of latex allergy in children and the adverse effects that may result if avoidance measures are not taken. The prevalence of this allergy is increasing because of repeated exposure to this ubiquitous material (in medical equipment or household products such as bottle teats or balloons). The risks are allergic reactions, ranging from benign local dermatitis to anaphylactic shock. This problem is well known and progress has been made: there is increased production of latex-free products and screening for latex-related allergy or sensitization during the pre-anesthetic evaluation to take preventive actions when planning surgery. The younger the patient exposed to latex, the higher the risk of sensitization. Therefore, the main issue raised in this article is the potential benefit of moving to latex-free health-care facilities so as to minimize the risk of allergic incidents. Many studies showed a significant decrease in the risk of allergy (sensitization and allergic reaction) when avoiding latex, but we must also consider the feasibility, the cost, and the effectiveness of such a policy.


Asunto(s)
Equipos y Suministros de Hospitales , Hospitales Pediátricos , Hipersensibilidad al Látex/prevención & control , Política Organizacional , Humanos , Hipersensibilidad al Látex/diagnóstico , Hipersensibilidad al Látex/epidemiología , Prevención Primaria , Factores de Riesgo
17.
Eur J Hum Genet ; 9(6): 409-18, 2001 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-11436121

RESUMEN

Beckwith-Wiedemann syndrome (BWS) is an overgrowth disorder involving developmental abnormalities, tissue and organ hyperplasia and an increased risk of embryonal tumours (most commonly Wilms tumour). This multigenic disorder is caused by dysregulation of the expression of imprinted genes in the 11p15 chromosomal region. Molecular diagnosis of BWS is currently difficult, mostly due to the large spectrum of genetic and epigenetic abnormalities. The other difficulty in managing BWS is the identification of patients at risk of tumour. An imprinted antisense transcript within KCNQ1, called KCNQ1OT (also known as LIT1), was recently shown to be normally expressed from the paternal allele. A loss of imprinting of the KCNQ1OT gene, associated with the loss of maternal allele-specific methylation of the differentially methylated region KvDMR1 has been described in BWS patients. The principal aim of this study was to evaluate the usefulness of KvDMR1 methylation analysis of leukocyte DNA for the diagnosis of BWS. The allelic status of the 11p15 region and the methylation status of the KCNQ1OT and H19 genes were investigated in leukocyte DNA from 97 patients referred for BWS and classified into two groups according to clinical data: complete BWS (CBWS) (n=61) and incomplete BWS (IBWS) (n=36). Fifty-eight (60%) patients (39/61 CBWS and 19/36 IBWS) displayed abnormal demethylation of KvDMR1. In 11 of the 56 informative cases, demethylation of KvDMR1 was related to 11p15 uniparental disomy (UPD) (nine CBWS and two IBWS). Thirteen of the 39 patients with normal methylation of KvDMR1 displayed hypermethylation of the H19 gene. These 13 patients included two siblings with 11p15 trisomy. These results show that analysis of the methylation status of KvDMR1 and the H19 gene in leukocyte DNA is useful in the diagnosis of 11p15-related overgrowth syndromes, resulting in the diagnosis of BWS in more than 70% of investigated patients. We also evaluated clinical and molecular features as prognostic factors for tumour and showed that mosaicism for 11p15 UPD and hypermethylation of the H19 gene in blood cells were associated with an increased risk of tumour.


Asunto(s)
Síndrome de Beckwith-Wiedemann/genética , Metilación de ADN , ADN/sangre , Leucocitos/metabolismo , Canales de Potasio con Entrada de Voltaje , Canales de Potasio/genética , ARN no Traducido/genética , Tumor de Wilms/genética , Adolescente , Adulto , Alelos , Niño , Preescolar , Cromosomas Humanos Par 11 , Supervivencia sin Enfermedad , Salud de la Familia , Padre , Femenino , Impresión Genómica , Genotipo , Humanos , Lactante , Recién Nacido , Canales de Potasio KCNQ , Canal de Potasio KCNQ1 , Masculino , Madres , Linaje , Fenotipo , Pronóstico , ARN Largo no Codificante , Factores de Tiempo
18.
Ann Thorac Surg ; 56(2): 323-7, 1993 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-8347016

RESUMEN

Right pneumonectomy can lead to severe respiratory impairment due to stenosis of the left main bronchus. This syndrome is usually treated by inserting a fixed-volume prosthesis but, in children, expandable prostheses have the advantage of being adaptable to growth and permit progressive recentering of the mediastinum. We report 3 such cases, with the results of pulmonary function tests. The patients were aged 11, 17, and 22 years at the time of implantation and had undergone pneumonectomy during childhood for either bronchiectasis or complete pulmonary sequestration. All 3 patients are doing well, with a follow-up of 1 to 3 1/2 years. Pulmonary function tests have shown a substantial improvement in the obstructive syndrome in 2 patients whereas, in the third patient, in whom the contralateral lung was not perfectly healthy, the functional improvement was only moderate.


Asunto(s)
Neumonectomía/efectos adversos , Prótesis e Implantes , Cirugía Torácica , Adolescente , Adulto , Bronquios/patología , Niño , Constricción Patológica , Femenino , Humanos , Masculino , Radiografía Torácica , Trastornos Respiratorios/diagnóstico por imagen , Trastornos Respiratorios/etiología , Trastornos Respiratorios/cirugía , Síndrome , Tomografía Computarizada por Rayos X
19.
J Pediatr Surg ; 36(9): 1425-7, 2001 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-11528621

RESUMEN

BACKGROUND/PURPOSE: Pneumonectomy in children can be complicated by a severe mediastinal shift, which leads to bronchial stretching resulting in severe respiratory failure. This postpneumonectomy syndrome can be corrected by inserting a prosthesis in the empty side of the chest. METHODS: Forty-two children, from 6 months to 15 years old, underwent a pneumonectomy. Seven of these patients were treated surgically for severe manifestations of postpneumonectomy syndrome. First insertion of an expandable prosthesis was followed up in 5 cases by its replacement with a breast prosthesis in adolescence. The expandable prosthesis was injected periodically with saline solution to maintain the mediastinum in a midline position as the children grew. RESULTS: The mean delay between pneumonectomy and first prosthesis implantation was 5 years (range, 11 months to 8 years). Pulmonary function tests showed a substantial improvement in the obstructive syndrome in all patients except one, in whom the functional improvement was moderate. The mean follow-up after the expandable prosthesis implantation was 6 years (range, 6 months to 10 years) and all patients are doing well. CONCLUSIONS: The insertion of an intrathoracic prosthesis can dramatically improve the clinical symptoms and reduce the functional obstructive syndrome. The expandable prosthesis allowed for progressive, well-tolerated recentering of the mediastinum and adjustment for growth.


Asunto(s)
Neumonectomía/efectos adversos , Prótesis e Implantes , Insuficiencia Respiratoria/cirugía , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Neumonectomía/métodos , Complicaciones Posoperatorias/cirugía , Diseño de Prótesis , Pruebas de Función Respiratoria , Insuficiencia Respiratoria/etiología , Medición de Riesgo , Síndrome , Factores de Tiempo , Resultado del Tratamiento
20.
J Pediatr Surg ; 30(9): 1302-5, 1995 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-8523230

RESUMEN

Collagen-coated Vicryl mesh (C.C.V.M.) was used in 28 children who needed repair of thoracic and abdominal wall defects. Herein the authors report the clinical experience, surgical technique, and outcome in these cases.


Asunto(s)
Músculos Abdominales/anomalías , Músculos Abdominales/cirugía , Neoplasias Óseas/cirugía , Costillas/cirugía , Mallas Quirúrgicas , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Hernia Umbilical/cirugía , Humanos , Recién Nacido , Masculino , Poliglactina 910
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