Your browser doesn't support javascript.
loading
Mostrar: 20 | 50 | 100
Resultados 1 - 20 de 22
Filtrar
1.
Neonatology ; 121(1): 133-136, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-38035569

RESUMEN

Acinar dysplasia (AcDys) is one of the three main diffuse developmental disorders of the lung. The transcription factor NK2 homeobox 1 (NKX2.1) partly controls the synthesis of surfactant proteins by type 2 alveolar epithelial cells (AEC2), and germline mutations are known to be associated with brain-lung thyroid syndrome. We report the case of a full-term neonate who developed refractory respiratory failure with pulmonary hypertension requiring venoarterial extracorporeal membrane oxygenation. Histological examination of the lung biopsy specimen was consistent with the diagnosis of AcDys. Molecular analyses led to the identification of the missense heterozygous variant in NKX2.1 (NM_001079668) c.731A>G p.(Tyr244Cys), which is predicted to be pathogenic. After 5 weeks, because AcDys is a fatal disorder and the patient's status worsened, life-sustaining therapies were withdrawn, and she died after a few hours. This study is the first to extend the phenotype of NKX2.1 pathogenic variant, to a fatal form of AcDys.


Asunto(s)
Síndrome de Dificultad Respiratoria del Recién Nacido , Insuficiencia Respiratoria , Recién Nacido , Femenino , Humanos , Mutación , Pulmón/metabolismo , Factores de Transcripción/genética , Factores de Transcripción/metabolismo , Insuficiencia Respiratoria/genética , Insuficiencia Respiratoria/terapia , Síndrome de Dificultad Respiratoria del Recién Nacido/diagnóstico , Síndrome de Dificultad Respiratoria del Recién Nacido/genética
2.
Front Pediatr ; 11: 1228248, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37622083

RESUMEN

Introduction: Childhood chronic diseases affect family functioning and well-being. The aim of this study was to measure the impact of caring for a child with PUV, and the factors that most impact the burden of care. Patients and method: We gave a questionnaire on the familial impact of having a child with posterior urethral valves to all parents of a child included in the CIRCUP trial from 2015 onwards. The questionnaire included questions about the parents' demographics, health, professional, financial and marital status and how these evolved since the child's birth as well as the "impact on family scale" (IOFS), which gives a total score ranging from 15 (no impact) to 60 (maximum impact). We then analyzed both the results of the specific demographic questions as well as the factors which influenced the IOFS score. Results: We retrieved answers for 38/51 families (74.5% response rate). The average IOFS score was 23.7 (15-51). We observed that the child's creatinine level had an effect on the IOFS score (p = 0.02), as did the parent's gender (p = 0.008), health status (p = 0.015), being limited in activity since the birth of the child (p = 0.020), being penalized in one's job (p = 0.009), being supported in one's job (p = 0.002), and decreased income (p = 0.004). Out of 38 mother/father binomials, 8/33 (24.2%) declared that they were no longer in the same relationship afterwards. Conclusion: In conclusion, having a boy with PUV significantly impacts families. The risk of parental separation and decrease in revenue is significant. Strategies aiming to decrease these factors should be put in place as soon as possible.

3.
J Urol ; 209(3): 591-599, 2023 03.
Artículo en Inglés | MEDLINE | ID: mdl-36449491

RESUMEN

PURPOSE: Since bladder neck dextranomer/hyaluronic acid copolymer (Deflux) injections can improve urinary incontinence of various etiologies, we hypothesized that incontinent children with bladder exstrophy-epispadias complex would benefit from dextranomer/hyaluronic acid copolymer. We aimed to analyze dextranomer/hyaluronic acid copolymer efficacy and predictors of treatment success in bladder exstrophy-epispadias complex. MATERIALS AND METHODS: Incontinent bladder exstrophy-epispadias complex children aged >4 years undergoing dextranomer/hyaluronic acid copolymer injections in our hospital between October 1997 and January 2021 were included. Medical history, continence, and bladder capacity before injections were recorded. Postoperatively, patients were categorized as "dry," "significantly improved," or "failure." Postoperative complications, bladder emptying mode, and bladder capacity were reported. Failure-free survival was estimated by Kaplan-Meier models. RESULTS: Altogether, 58 patients (27 male epispadias, 9 female epispadias, 22 bladder exstrophy) underwent 105 injections at median age of 8.5 (interquartile range 5.8-12) years. Previous bladder neck reconstruction had been performed in 38 (66%) and 33 (57%) received multiple injections. Complications occurred in 9%. Five-year failure-free survival was 70% (standard error 9.1) in males epispadias compared to 45% (9.0) in females and exstrophy patients (P = .04). Previous bladder neck reconstruction associated with improved 5-year failure-free survival in females and exstrophy patients (58%, SE 11) but not in male epispadias (75%, SE 11). CONCLUSIONS: Dextranomer/hyaluronic acid copolymer injections provided satisfactory and lasting continence in over half of bladder exstrophy-epispadias complex patients. While injections were successful in male epispadias regardless of bladder neck reconstruction timing, results were better in bladder exstrophy and female epispadias after previous bladder neck reconstruction.


Asunto(s)
Extrofia de la Vejiga , Epispadias , Incontinencia Urinaria , Niño , Humanos , Masculino , Femenino , Preescolar , Vejiga Urinaria/cirugía , Extrofia de la Vejiga/complicaciones , Extrofia de la Vejiga/cirugía , Epispadias/complicaciones , Epispadias/cirugía , Ácido Hialurónico , Incontinencia Urinaria/terapia , Incontinencia Urinaria/cirugía
4.
Front Pediatr ; 10: 971662, 2022.
Artículo en Inglés | MEDLINE | ID: mdl-36186628

RESUMEN

Objective: Boys with posterior urethral valves (PUV) present an increased risk of febrile urinary tract infection (fUTI). Identifying specific risk factors could allow for tailoring UTI prevention. The aim of this study was to use the data from the CIRCUP randomized controlled trial data to identify patient characteristics associated with a higher risk of fUTI. Patients and methods: We performed a secondary analysis of the data from the CIRCUP randomized trial which included boys with PUV, randomized to circumcision and antibiotic prophylaxis vs. antibiotic prophylaxis alone and followed for 2 years. There was only 1 episode of fUTI in the circumcision group vs. 17 in the uncircumcised group. We therefore only studied the antibiotic prophylaxis alone group and compared age at prenatal diagnosis, size and weight at birth, presence of dilating VUR at diagnosis, abnormal DMSA scan at 2 months, and nadir creatinine between children who presented a fUTI and those who did not, as well as age at first episode of fUTI. Results: The study group consisted of 42 patients of which 17 presented at least on fUTI. Presence of dilating VUR was significantly associated with risk of fUTI (p = 0.03), OR: 6 [CI 95% = (1.13-27.52)]. None of the other parameters were associated with increased risk of fUTI. We observed three distinct time periods for presenting a fUTI with a decrease in infection rate after the first 40 days of life, then at 240 days of life. Conclusion: In boys with PUV, presence of high-grade VUR is associated with a higher risk of presenting a fUTI. The rate of febrile UTIs seems to decrease after 9 months.

5.
J Laparoendosc Adv Surg Tech A ; 30(12): 1289-1294, 2020 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-33155851

RESUMEN

Background/Objective: Thoracoscopic repair of esophageal atresia (EA) is gaining popularity, but it is a highly technically demanding procedure. The aim of our study is to evaluate our outcomes in the management of type C EA comparing the thoracoscopic and the open (thoracotomy) approaches. Methods: This is a retrospective bicentric study of two major pediatric surgery centers, reviewing all the patients operated for EA with distal tracheoesophageal fistula. Only patients who underwent primary anastomosis were included. From 2008 to 2018, 187 patients were included. Results: Forty-seven patients were operated thoracoscopically (TS group) and 140 by the open approach (TT group). Mean gestational age was 38 ± 2.4 weeks in TS group and 36.4 ± 3.3 weeks in TT group (P = .005) with a mean birth weight of 2785 ± 654 g and 2404.9 ± 651 g in TS and TT groups, respectively (P = .003). The mean operative time was 127.6 ± 35 minutes in TS group and 105.7 ± 23 minutes in TT group (P = .0005). The mean postoperative ventilation time and the mean length of stay were significantly shorter in the thoracoscopic group (P = .004 and P < .0001, respectively). The incidence of anastomotic leak was 8.9% in TS group versus 16.4% in TT group (P = .33). Anastomotic stenosis occurred in 33.3% of TS group and in 22.4% of TT group (P = .17). Conclusions: Surgical outcome of thoracoscopic repair of EA is comparable to the open repair with no higher complication rate with the expected skeletal and cosmetic benefits. However, possible bias regarding prematurity, weight at surgery, and associated anomalies must be taken into consideration.


Asunto(s)
Atresia Esofágica/cirugía , Toracoscopía/métodos , Toracotomía/métodos , Fístula Traqueoesofágica/cirugía , Anastomosis Quirúrgica , Femenino , Edad Gestacional , Humanos , Recién Nacido , Masculino , Tempo Operativo , Periodo Posoperatorio , Estudios Retrospectivos , Resultado del Tratamiento
6.
J Robot Surg ; 14(4): 531-541, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-31531753

RESUMEN

The learning curve (LC) of a new technique is fundamental for its application and assessment, and for the training. Literature was analysed to define the LCs of different surgical procedures and the impact of fellowship programs. MEDLINE, EMBASE and paediatric surgical journals' databases from January 1995 to December 2018 were systematically analysed. Two independent residents sought for articles providing description of robotic-assisted procedures' LCs in paediatric age/population. Seventeen articles were selected, describing LC of robotic-assisted pyeloplasty (n = 9), fundoplication (n = 4), cholecystectomy (n = 2), choledochal cyst resection (n = 1) and lingual tonsillectomy (n = 1), with 721 procedures. Ten studies refer to one single surgeon; six to more than one; one does not specify the number of operators. Eleven studies are unicentric retrospective, two multicentric retrospective, three prospective and one is a comparative analysis between a retrospective case series and a prospective cohort. The most recruited parameter is operative time alone in 3 articles, associated with complications in 12, length of hospital stay in 6, blood loss in 3, resolution in 4 and narcotic use in 2. The LC is described as impacting procedural planning (n = 17), training (n = 9) and economic costs (n = 2). To date, operative time is the most reported outcome to measure LC and proficiency. Efforts are needed to consider measures of surgical expertise and patient status. Robotic training should be standardized on targeted programs planned upon well-defined LCs.


Asunto(s)
Educación Médica , Evaluación Educacional , Becas , Curva de Aprendizaje , Tempo Operativo , Procedimientos Quirúrgicos Robotizados/educación , Adolescente , Pérdida de Sangre Quirúrgica/estadística & datos numéricos , Niño , Preescolar , Femenino , Humanos , Lactante , Tiempo de Internación , Masculino , Narcóticos/administración & dosificación , Resultado del Tratamiento
7.
Front Pediatr ; 7: 209, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31192176

RESUMEN

Introduction stating the aim of the study: Robot-assisted laparoscopic pyeloplasty (RALP) is gaining acceptance among pediatric urologists. Few studies have evaluated the retroperitoneal approach for RALP. We share our experience from the first 2 years of a multidisciplinary pediatric robotic program in our center. Patients (or Materials) and Methods: We performed a retrospective analysis of prospectively collected data of children undergoing RALP for ureteropelvic junction obstruction (n = 50). Diagnosis was confirmed by ultrasound and Tc-99m mercaptoacetyltriglycine renal scan or MRI; the same criteria were used to evaluate outcome. Surgical approach was chosen according to a specific algorithm. Transperitoneal approach (n = 13) was reserved for horseshoe kidney, ectopic kidney, and redo surgery. We analyzed the 37 cases performed by a lateral retroperitoneal approach. Dismembered pyeloplasty was done for all cases and anastomosis was performed using a running monofilament 6/0 absorbable suture. All were drained by double J stent. Patient data, operating room parameters and postoperative course were recorded. Results: The median age was 7.9 years (5.1-13.8); the youngest was 2 years old. The median weight was 23 kg (17-41) with the smallest weighing 12.4 kg. Aberrant crossing vessels were present in 18 children. Median set-up time, from skin incision until the end of the 4-port insertion, was 33 min (29-48). Median surgeon's console time was 151 min (136-182). No conversion to an open procedure was necessary. The postoperative course was free of complications, except urinary tract infection in 6 children. All but 4 patients were discharged on day one. Median follow-up was 9 months (5-13). Redo pyeloplasty was not required. Practical training of other colleagues was possible after 10 cases performed by the same surgeon. Conclusion: These preliminary results suggest that retroperitoneal RALP in children is feasible, safe and effective. It is an excellent option with ideal anatomical exposure. Longer term results as well as continued practice will identify and overcome any challenges and enable surgical mastery of this procedure which is still evolving.

8.
Pediatr Blood Cancer ; 66 Suppl 3: e27867, 2019 08.
Artículo en Inglés | MEDLINE | ID: mdl-31136081

RESUMEN

AIM: We present the preliminary results of robotic-assisted laparoscopic (RAL) total and partial nephrectomy for renal malignant tumors in children. METHODS: This is a prospective study of patients operated with RAL between December 2016 and September 2018. Patients with Wilms tumors were treated according to the SIOP-2001 protocol. Patient and tumor characteristics, type of surgery, surgical-related morbidity, and oncologic outcomes were recorded. Results were compared with a series of patients with similar age- and tumor-related characteristics operated during the same period by an open surgical approach. RESULTS: Ten children underwent RAL nephrectomy with a mean age of five years (3.2-14.1 years). Total nephrectomy was done in six cases for Wilms tumor and in one case for renal sarcoma; three cases were converted. Complete removal of tumor without rupture was achieved in all cases. Postoperative course was uneventful, and patients were discharged between days 2 and 7. Neither recurrence nor medium-term complications occurred. Nine patients are alive with a median follow-up of 16 months (6-27 months) and one female died from complications of central nervous system metastases one year after surgery. When compared with the open surgical approach group, median tumor volume was smaller (P = 0.005), hospital stay was shorter (P = 0.01), and operative time was similar (P = 0.20). CONCLUSIONS: RAL total and partial nephrectomy procedure for renal tumor in children may be an option in carefully selected cases. Indication should be discussed at tumor boards and surgery performed while adhering strictly to oncological surgical rules.


Asunto(s)
Neoplasias Renales/cirugía , Laparoscopía/métodos , Tiempo de Internación/estadística & datos numéricos , Nefrectomía/métodos , Complicaciones Posoperatorias , Procedimientos Quirúrgicos Robotizados/métodos , Adolescente , Niño , Preescolar , Femenino , Estudios de Seguimiento , Humanos , Neoplasias Renales/patología , Masculino , Estudios Prospectivos , Resultado del Tratamiento
9.
J Pediatr Surg ; 53(7): 1424-1427, 2018 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-29735202

RESUMEN

PURPOSE: To suggest a novel technique for omphalocele closure which uses the circular base of the umbilical cord, thus allowing for a more physiological healing process and natural-looking scar. METHODS: Among 16 neonates operated for omphalocele between 2011 and 2016, 12 were closed with a one-stage procedure using a Z omphaloplasty (ZORRO). Median gestational age was 36.5 weeks; median birth weight was 3210 g. The umbilical arteries were divided and ligated outside the peritoneal cavity above the parietal musculocutaneous plane. The upper part of the defect was closed vertically in the midline, while the lower part was closed in a circular fashion by imbricating 2 lateral cutaneous Z flaps thus forming a new cordonal base. RESULTS: The postoperative course was uneventful in all cases. The reconstructed cordonal bases healed as would a normal umbilical cord, with central umbilication surrounded by healthy skin. With a median follow-up period of 11 months, the umbilicus was in the normal position, with a 0.6 xyphoumbilical/xyphopubic ratio. CONCLUSIONS: This technique mimics the natural necrosis mechanism and physiological healing of the umbilicus thus allowing for an esthetic and "natural looking" umbilicus.


Asunto(s)
Hernia Umbilical/cirugía , Procedimientos de Cirugía Plástica/métodos , Cicatrización de Heridas , Femenino , Humanos , Recién Nacido , Masculino , Colgajos Quirúrgicos , Resultado del Tratamiento , Ombligo/cirugía
10.
J Laparoendosc Adv Surg Tech A ; 28(9): 1129-1134, 2018 Sep.
Artículo en Inglés | MEDLINE | ID: mdl-29630443

RESUMEN

BACKGROUND: No data exist evaluating the utilization and safety of outpatient thoracoscopy in children. The aim of this study was to investigate the safety of outpatient thoracoscopy and to assess parental opinions on the advantages and disadvantages of a pediatric thoracoscopy outpatient setting. METHODS: A retrospective review of data from patients treated by thoracoscopy for congenital pulmonary malformation between 2013 and 2016 was performed. Study focused on patients who underwent outpatient thoracoscopy. All were placed in a flank position and underwent a three-port (5-mm optical trocar and two 5-mm trocars) thoracoscopy. Insufflation pressure required was 5 mmHg with bilateral lung ventilation. Pain control was provided with multimodal postoperative analgesia and the use of paravertebral block for regional analgesia. No drain was inserted. Outcomes of interest included 30-day overall morbidity, readmission, reoperation, and parental opinions through a phone call questionnaire. RESULTS: A total of 37 thoracoscopies were identified; 11 (30%) with a median age of 5.3 months (4.2-12.3) were performed as an outpatient procedure (10 sequestration, 1 bronchogenic cyst). Median operating time was 51 minutes (34-87). No conversion and no transfusion occurred. No complications occurred (no morbidity, no readmission, and no reoperation). According to parents' view the outpatient setting has no disadvantages. CONCLUSION: This first analysis of a small monocentric dataset demonstrates that pediatric patients can safely undergo thoracoscopy, an outpatient procedure, with a high rate of parental satisfaction.


Asunto(s)
Procedimientos Quirúrgicos Ambulatorios , Quiste Broncogénico/cirugía , Secuestro Broncopulmonar/cirugía , Seguridad del Paciente , Toracoscopía/métodos , Actitud Frente a la Salud , Estudios de Factibilidad , Femenino , Estudios de Seguimiento , Humanos , Lactante , Masculino , Padres , Estudios Retrospectivos , Medición de Riesgo , Resultado del Tratamiento
11.
Surg Endosc ; 32(2): 1027-1034, 2018 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-28840328

RESUMEN

INTRODUCTION: Robotic surgery has seen increasing use in the field of pediatric surgery. Our clinical experience suggested instrument size can impact on the surgical ability. This study aimed to compare the performance of robot-assisted laparoscopic skills in confined spaces using either 5 or 8 mm instruments. METHODS: A preclinical randomized crossover study design was implemented. 24 assessors performed three different reproducible drill procedures (M1: peg transfer, M2: circle cutting, M3: intracorporeal suturing). To assess surgical proficiency in confined working spaces, these exercises were performed with 5 and 8 mm instruments of the da Vinci® Surgical Systems Si in a cubic box with 60 mm-sized edges. Each performance was recorded and evaluated by two reviewers using both objective structured assessment of technical skills (OSATS) and global evaluative assessment of robotic skills (GEARS) scores. Parietal iatrogenic impacts and instrument collisions were specifically analyzed using a dedicated scoring system. RESULTS: Regardless of their experience, trainees performed significantly better when using 8 mm instruments in terms of OSATS scores (20.5 vs. 18.4; p < 0.01) and GEARS scores (23.4 vs. 21.9; p < 0.01) for most items, except for "depth perception" and "autonomy." The 8 mm performances involved significantly less parietal box damage (4.1 vs. 3.4; p < 0.01), and tool collisions (4.1 vs. 3.2; p < 0.01). CONCLUSIONS: In light of the better performances with 8 mm tools for specific tasks and parietal sparing constraints in restricted spaces, this study indicates that 5 mm instruments can be deemed to be less effective for reconstructive procedures in small children.


Asunto(s)
Procedimientos Quirúrgicos Robotizados/instrumentación , Adulto , Tamaño Corporal , Niño , Competencia Clínica , Estudios Cruzados , Femenino , Humanos , Masculino , Pediatría/instrumentación , Procedimientos Quirúrgicos Robotizados/métodos
12.
Int J Urol ; 24(12): 855-860, 2017 12.
Artículo en Inglés | MEDLINE | ID: mdl-29027269

RESUMEN

OBJECTIVES: To compare the outcomes of robot-assisted heminephrectomy for duplex kidney in children with those of open heminephrectomy. METHODS: The present retrospective multicentric analysis reviewed the records of robot-assisted versus open heminephrectomy carried out for duplex kidney in children from 2007 to 2014. Demographic data, weight, surgical time, hospital stay, complications and outcome were recorded. Follow up was based on a clinical review, renal sonography and dimercaptosuccinic acid renal scintigraphy. RESULTS: A total of 15 patients underwent robot-assisted heminephrectomy, and 13 patients underwent retroperitoneal heminephrectomy by open approach. All patients weighed <15 kg. The mean age at the time of surgery was 20.2 months (range 7-39 months) in the robotic group, and 18.4 months (range 6-41 months) in the open group. The mean hospital stay was statistically longer for the open surgery group (6.3 days, range 5-8 days vs 3.4 days, range 1-7 days; P < 0.001). Regarding postoperative pain control, total morphine equivalent intake was statistically greater for the open group (0.52 mg/kg/day vs 1.08 mg/kg/day; P < 0.001). No patient lost the remaining healthy moiety. There was no significant difference in terms of operating time, complication rate or renal outcomes. CONCLUSIONS: Robot-assisted heminephrectomy in small children seems to offer comparable renal outcomes with those of its standard open surgery counterpart. Specific technical adjustments are necessary, which typically increase the set-up time.


Asunto(s)
Laparoscopía/métodos , Nefrectomía/métodos , Procedimientos Quirúrgicos Robotizados , Administración de la Seguridad , Preescolar , Femenino , Francia , Humanos , Lactante , Laparoscopía/efectos adversos , Tiempo de Internación , Masculino , Nefrectomía/instrumentación , Tempo Operativo , Complicaciones Posoperatorias , Estudios Retrospectivos , Resultado del Tratamiento
13.
J Pediatr Surg ; 52(11): 1800-1805, 2017 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-28259381

RESUMEN

PURPOSE: Thoracoscopic lung resection for congenital pulmonary airway malformation (CPAM) is a safe technique for children. Our purpose was to evaluate the feasibility of a fast-track protocol in such cases. METHODS: From September 2007 to May 2016, 101 patients underwent a thoracoscopic pulmonary resection of which 83 for CPAM (lobectomy, wedge resection or sequestrectomy). We retrospectively reviewed the characteristics of surgical procedure, postoperative management and complications through three time periods (September 2007-December 2009: n=14, January 2010-March 2013: n=30, April 2013-May 2016: n=39) corresponding to management protocols modifications introducing fast-track pathways. RESULTS: Through the 3 time periods, median postoperative hospital stay decreases (4, 3, 2days successively, P=0.02). In the third time period, 4 patients underwent surgery in day-case surgery. The overall and surgical complication rates, mainly related to air leakage, remain stable through the 3 time periods (14%, P=0.41 and 10%, P=0.52 respectively). Among the 13 patients without postoperative pleural drainage, one required secondary drainage after a partial resection of an emphysema. CONCLUSION: Fast-track protocol for children undergoing uncomplicated thoracic surgery for CPAM seems feasible without extra morbidity. Selected patient undergoing thoracoscopic resection of the lung may benefit from the absence of pleural drainage and can be operated on in day-case surgery. LEVEL OF EVIDENCE: Level III.


Asunto(s)
Procedimientos Quirúrgicos Ambulatorios , Tiempo de Internación , Neumonectomía/métodos , Anomalías del Sistema Respiratorio/cirugía , Adolescente , Niño , Preescolar , Femenino , Humanos , Masculino , Periodo Posoperatorio , Enfisema Pulmonar/cirugía , Estudios Retrospectivos , Cirugía Torácica Asistida por Video/métodos
14.
Health Qual Life Outcomes ; 15(1): 24, 2017 Jan 28.
Artículo en Inglés | MEDLINE | ID: mdl-28129770

RESUMEN

BACKGROUND: The Hirschsprung's disease Anorectal malformation QoL questionnaire (HAQL) is a disease-specific quality of life (QoL) questionnaire for patients with Hirschsprung's disease (HD) or anorectal malformations (ARM). It was originally proposed in Dutch and is currently being translated into other languages to obtain an internationally standardized instrument. In this work we validate a French adaptation of the HAQL for adolescents and adults. METHODS: The questionnaires were translated into French and sent to patients aged 12 years and older, followed for HD or ARM at three French university hospitals. Questionnaires were sent to 147 adolescents and 188 adults. The psychometric properties of the questionnaires were analyzed in terms of reliability and validity. RESULTS: The original HAQL structure was not satisfactory. A new structure was proposed, while aiming to remain close to the original structure. The proposed structure has acceptable reliability and validity properties and reflects both physical, as well as psychosocial aspects. CONCLUSIONS: A French version of the HAQL questionnaire for adults and adolescents is ready for use in France. In particular the score could discriminate between degrees of clinical status based on the Krickenbeck consensus, which can aid clinicians to inform patients about physical and psychosocial challenges they may expect.


Asunto(s)
Malformaciones Anorrectales/psicología , Enfermedad de Hirschsprung/psicología , Calidad de Vida/psicología , Encuestas y Cuestionarios/normas , Adaptación Psicológica , Adolescente , Adulto , Femenino , Francia , Humanos , Masculino , Psicometría , Reproducibilidad de los Resultados , Traducción
15.
Prenat Diagn ; 37(2): 168-175, 2017 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-27981591

RESUMEN

OBJECTIVE: To evaluate prenatal ultrasound parameters as prognostic factors for complex and vanishing gastroschisis. METHODS: Retrospective multicentre study of 200 gastroschisis over 13 years (2000-2013). Collection of prenatal ultrasound evaluation on maternal and fetal growth parameters, intra- and extra-abdominal bowel and stomach dilation, abdominal wall defect diameter and changes in bowel appearance. Correlation of these factors with the presence of mechanical intestinal complications at birth, named 'complex gastroschisis'. RESULTS: Fifty-two patients (26%) had complex gastroschisis (CG), including ten vanishing gastroschisis. The presence of intra-abdominal bowel dilation at the second (T2) or third (T3) trimester ultrasound was predictive for CG, with odds ratios at 6.69 (95%CI 2.41-18.55) and 4.72 (95%CI 2.16-10.28), respectively, with a cut-off value at the last examination of >19 mm. A small abdominal wall defect diameter was also predictive for CG, with cut-off values of <9.2 mm at T2 and <12.5 mm at T3. Vanishing gastroschisis recorded earlier intra-abdominal bowel dilation diagnosis, associated with a small wall defect and no extra-abdominal dilation. CONCLUSION: Intra-abdominal bowel dilation and a small abdominal wall defect diameter accurately predict CG and could be a first sign of vanishing gastroschisis when they occur early. © 2016 John Wiley & Sons, Ltd.


Asunto(s)
Gastrosquisis/diagnóstico , Gastrosquisis/patología , Ultrasonografía Prenatal , Abdomen/diagnóstico por imagen , Abdomen/patología , Adulto , Dilatación Patológica , Femenino , Desarrollo Fetal , Gastrosquisis/epidemiología , Humanos , Recién Nacido , Intestinos/diagnóstico por imagen , Intestinos/patología , Embarazo , Resultado del Embarazo/epidemiología , Pronóstico , Remisión Espontánea , Estudios Retrospectivos , Ultrasonografía Prenatal/estadística & datos numéricos , Adulto Joven
16.
J Robot Surg ; 11(2): 255-258, 2017 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-27822660

RESUMEN

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.


Asunto(s)
Miotomía de Heller/métodos , Procedimientos Quirúrgicos Robotizados/métodos , Adolescente , Niño , Acalasia del Esófago/cirugía , Enfermedades del Esófago/cirugía , Femenino , Miotomía de Heller/efectos adversos , Humanos , Masculino , Factores de Riesgo , Procedimientos Quirúrgicos Robotizados/efectos adversos
17.
Acta Chir Belg ; 116(2): 89-95, 2016 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-27385295

RESUMEN

BACKGROUND: Pyloric atresia (PA) is a rare condition, and may be misdiagnosed and especially confused for duodenal atresia pre-operatively. We looked for clues to avoiding pre-operative misdiagnosis and hence allow the best neonatal medical and surgical management. METHODS: A retrospective case-note review was carried out of the five patients managed in four centres with the diagnosis of isolated PA. We focused on antenatal ultrasound findings, postnatal clinical and radiological features, operative findings, surgical procedures and outcomes. RESULTS: Four patients had polyhydramnios and one double bubble sign on antenatal ultrasound. After birth, non-bilious vomiting and upper abdominal distension were the main symptoms. Gastric decompression showed non-bilious gastric fluid. Radiological findings were a large gastric air bubble with no gas beyond in all cases. The diagnosis of duodenal atresia was postulated at first in all cases. The diagnosis of PA was established peroperatively. One patient referred late, died 13-day post-operatively of cardiopulmonary failure secondary to a severe pneumonia that may be related to aspiration syndrome. Outcomes were otherwise satisfactory. CONCLUSIONS: Even though it is a rare diagnosis, PA has a specific clinical and radiological presentation underlined here that should be kept in mind when managing a neonate with a gastric outlet obstruction.


Asunto(s)
Obstrucción Duodenal/cirugía , Obstrucción de la Salida Gástrica/etiología , Obstrucción de la Salida Gástrica/cirugía , Píloro/anomalías , Anastomosis Quirúrgica , Diagnóstico Diferencial , Obstrucción Duodenal/diagnóstico , Femenino , Estudios de Seguimiento , Obstrucción de la Salida Gástrica/complicaciones , Obstrucción de la Salida Gástrica/diagnóstico , Humanos , Recién Nacido , Atresia Intestinal , Laparotomía/métodos , Masculino , Diagnóstico Prenatal , Píloro/cirugía , Estudios Retrospectivos , Medición de Riesgo , Muestreo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Ultrasonografía Prenatal/métodos
18.
Pediatr Surg Int ; 32(11): 1047-1052, 2016 Nov.
Artículo en Inglés | MEDLINE | ID: mdl-27344585

RESUMEN

PURPOSE: To compare the learning curves for mastering OP and LP surgical techniques, in terms of effects on completion times and postoperative outcomes/complications. METHODS: A retrospective analysis was performed for 198 patients with hypertrophic pyloric stenosis. The learning curves were in regard to two groups of surgeons: three of whom performed 106 OPs while three others performed 92 LPs. Treatment-related complications were divided into two categories: specific complications relating to the pyloromyotomy and non-specific complications. A logistic regression model with repeated data was used to explore the occurrence of complications. RESULTS: The overall postoperative complication rates were not significantly different between the OP (15.1 %) and the LP (11.8 %) groups. Specific complications were more frequent in the LP group (6.4 versus 2.8 %), while non-specific complications were more frequent in the OP group (12.1 versus 5.3 %). The occurrence of complications exhibited a statistically decreasing risk with each supplementary procedure that was performed (p = 0.0067) in the LP group, but not in the OP group (p = 0.9665). CONCLUSION: From a learning process perspective, laparoscopy is mainly associated with a significantly higher risk of specific complications. This risk decreases in line with the surgeon's level of experience, whereas non-specific complications remain stable in open procedures.


Asunto(s)
Laparoscopía/educación , Curva de Aprendizaje , Complicaciones Posoperatorias/epidemiología , Estenosis Hipertrófica del Piloro/cirugía , Cirujanos/educación , Adulto , Femenino , Humanos , Laparoscopía/métodos , Masculino , Píloro/cirugía , Estudios Retrospectivos , Resultado del Tratamiento
19.
J Robot Surg ; 10(2): 117-21, 2016 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-26994773

RESUMEN

Robotic technology allows for the management of complex surgical cases with a minimally invasive approach. The aim of this study was to communicate our experience using robotic technology for non-scheduled pediatric procedures (NSP). We performed a prospective study over the last 5 years including all consecutive cases where surgery was performed with a robot. NSP procedures were defined as a time to surgery of <24 h. Preoperative time, operative time, overall completion rate, and postoperative course were analyzed. Of the 85 cases recorded, five corresponded to robot-assisted NSP with a mean weight of 10 kg (3-36 kg). The mean time before surgery was 19 h (11-24 h). Conversion rate to open procedure was 40 %. Fifteen NSP had to be performed without robotic plateform. Robotic surgery is a potentially relevant option for most pediatric thoracic or abdominal procedures performed in a non-scheduled setting and offers technical advantages.


Asunto(s)
Procedimientos Quirúrgicos Robotizados/estadística & datos numéricos , Abdomen/cirugía , Citas y Horarios , Niño , Conversión a Cirugía Abierta/estadística & datos numéricos , Tratamiento de Urgencia/estadística & datos numéricos , Humanos , Tempo Operativo , Pediatría/estadística & datos numéricos , Estudios Prospectivos , Procedimientos Quirúrgicos Torácicos/estadística & datos numéricos , Tiempo de Tratamiento
SELECCIÓN DE REFERENCIAS
DETALLE DE LA BÚSQUEDA
...