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BACKGROUND: Surgical site infections (SSIs) in children represent a common and serious postoperative complication. Surgical skin preparation is an essential preventive measure in every surgical procedure. The most commonly used antiseptic agents for surgical skin preparation are chlorhexidine gluconate and iodophors in alcohol-based solutions. In adult patients the use of chlorhexidine-containing antiseptic solutions for preoperative skin preparation has been advocated to reduce SSI rates. Our objective was to conduct a systematic literature review on use of antiseptic agents for surgical skin preparation in children less than 16 years of age. METHODS: A systematic review of MEDLINE, EMBASE, CINAHL and CENTRAL was performed using both MeSH and free text terms and using the relevant Cochrane filter to identify full text randomized trials (RCTs) and comparative observational studies. Interventions of interest were the choice of main agent in antiseptic solutions (chlorhexidine/povidone-iodine/alcohol) compared with each other or with other antiseptic agents. Primary outcome was the reported rate of surgical site infections. RESULTS: In total 8 studies were included in the review; 2 RCTs and 6 observational studies. Observational studies generally did not primarily investigate the association of different antiseptics with subsequent SSI. The identified randomised controlled trials included only 61 children in total, and were of low quality. Consequently, we did not conduct a formal meta-analysis. Since the publication of a comprehensive systematic review of perioperative measures for the prevention of SSI in 2016, no randomized controlled trials comparing antiseptic agents for surgical skin preparation in paediatric surgery have been conducted. CONCLUSION: Robust evidence on the optimal skin antisepsis to reduce SSIs in children is lacking. Direct extrapolation of effects from trials involving adults is not appropriate as physiologic characteristics and risk factors for SSIs differ between adults and children. It is therefore essential to conduct high quality RCT investigating interventions to identify optimal measures to reduce SSI rates in children. TRIAL REGISTRATION: Prospero registration ( CRD42020166193 ).
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Antiinfecciosos Locales , Clorhexidina , Adulto , Antiinfecciosos Locales/uso terapéutico , Niño , Clorhexidina/uso terapéutico , Humanos , Povidona Yodada , Cuidados Preoperatorios/métodos , Infección de la Herida Quirúrgica/prevención & controlRESUMEN
INTRODUCTION: Multisource feedback (MSF), also called 360-degree assessment, is one form of assessment used in postgraduate training. However, there is an ongoing discussion on its value, because the factors that influence the impact of MSF and the main impact of MSF are not fully understood. In this study, we investigated both the influencing factors and the impact of MSF on residency training. METHODS: We conducted a qualitative case study within the boundaries of the residency training for paediatricians and paediatric surgeons at a University Hospital. We collected data from seven focus group interviews with stakeholders of MSF (residents, raters and supervisors). By performing a reflexive thematic analysis, we extracted the influencing factors and the impact of MSF. RESULTS: We found seven influencing factors: MSF is facilitated by the announcement of a clear goal of MSF, the training of raters on the MSF instrument, a longitudinal approach of observation, timing not too early and not too late during the rotation, narrative comments as part of the ratings, the residents' self-assessment and a supervisor from the same department. We found three themes on the impact of MSF: MSF supports the professional development of residents, enhances interprofessional teamwork and increases the raters' commitment to the training of residents. CONCLUSION: This study illuminates the influencing factors and impact of MSF on residency training. We offer novel recommendations on the continuity of observation, the timing during rotations and the role of the supervisor. Moreover, by discussing our results through the lens of identity formation theory, this work advances our conceptual understanding of MSF. We propose identity formation theory as a framework for future research on MSF to leverage the potential of MSF in residency training.
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Internado y Residencia , Niño , Competencia Clínica , Educación de Postgrado en Medicina , Retroalimentación , Humanos , Investigación CualitativaRESUMEN
Hepatic gene therapy by delivering non-integrating therapeutic vectors in newborns remains challenging due to the risk of dilution and loss of efficacy in the growing liver. Previously we reported on hepatocyte transfection in piglets by intraportal injection of naked DNA vectors. Here, we established delivery of naked DNA vectors to target periportal hepatocytes in weaned pigs by hydrodynamic retrograde intrabiliary injection (HRII). The surgical procedure involved laparotomy and transient isolation of the liver. For vector delivery, a catheter was placed within the common bile duct by enterotomy. Under optimal conditions, no histological abnormalities were observed in liver tissue upon pressurized injections. The transfection of hepatocytes in all tested liver samples was observed with vectors expressing luciferase from a liver-specific promoter. However, vector copy number and luciferase expression were low compared to hydrodynamic intraportal injection. A 10-fold higher number of vector genomes and luciferase expression was observed in pigs using a non-integrating naked DNA vector with the potential for replication. In summary, the HRII application was less efficient (i.e., lower luciferase activity and vector copy numbers) than the intraportal delivery method but was significantly less distressful for the piglets and has the potential for injection (or re-injection) of vector DNA by endoscopic retrograde cholangiopancreatography.
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INTRODUCTION: Surgical treatment of esophageal atresia (EA) has markedly improved, allowing the focus to shift from short-term complications and mortality to long-term complications and quality of life. Health-related quality of life (HRQoL) is variable and reported to range from reduced to unimpaired in patients with repaired EA. We assessed the HRQoL, determined the prevalence of long-term complications and their possible impact on the HRQoL in patients who had correction of EA in Switzerland. Further, we also investigated in the general well-being of their parents. MATERIALS AND METHODS: Patients with EA repair in Switzerland between 1985 and 2011 were enrolled. Long-term complications were assessed by enquiring disease-related symptoms, standardized clinical examinations, and analysis of radiographs. HRQoL was inquired using different validated questionnaires (KIDSCREEN-27, World Health Organization [WHO]-5, and Gastrointestinal Quality of Life Index [GIQLI]). Patients were grouped according to their age. In underage patients, general well-being of the parents was assessed using the WHO-5 questionnaire. RESULTS: Thirty patients were included with a mean age of 11.3 ± 5.7 years. Long-term complications were present in 63% of all patients. HRQoL in underage patients was comparable to the provided reference values and rated as good, while adult patients reported a reduced HRQoL. The presence of gastroesophageal reflux disease symptoms was associated with reduced HRQoL in underage patients. Parents of underage patients stated a good general well-being. CONCLUSION: Long-term complications among patients with repair of EA in Switzerland are common. HRQoL in underage patients is good and general well-being of their parents is unimpaired. Adult patients reported a reduced HRQoL, consistent with other reports. As long-term complications may manifest only later in life, a structured follow-up of patients with an EA repair during childhood and adolescence is needed.
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Atresia Esofágica , Esofagoplastia , Adolescente , Adulto , Niño , Preescolar , Atresia Esofágica/complicaciones , Atresia Esofágica/cirugía , Humanos , Calidad de Vida , Encuestas y Cuestionarios , Suiza/epidemiologíaRESUMEN
BACKGROUND & AIMS: Hirschsprung's disease (HSCR) is a congenital intestinal motility disorder defined by the absence of enteric neuronal cells (ganglia) in the distal gut. The development of HSCR-associated enterocolitis remains a life-threatening complication. Absence of enteric ganglia implicates innervation of acetylcholine-secreting (cholinergic) nerve fibers. Cholinergic signals have been reported to control excessive inflammation, but the impact on HSCR-associated enterocolitis is unknown. METHODS: We enrolled 44 HSCR patients in a prospective multicenter study and grouped them according to their degree of colonic mucosal acetylcholinesterase-positive innervation into low-fiber and high-fiber patient groups. The fiber phenotype was correlated with the tissue cytokine profile as well as immune cell frequencies using Luminex analysis and fluorescence-activated cell sorting analysis of colonic tissue and immune cells. Using confocal immunofluorescence microscopy, macrophages were identified in close proximity to nerve fibers and characterized by RNA-seq analysis. Microbial dysbiosis was analyzed in colonic tissue using 16S-rDNA gene sequencing. Finally, the fiber phenotype was correlated with postoperative enterocolitis manifestation. RESULTS: The presence of mucosal nerve fiber innervation correlated with reduced T-helper 17 cytokines and cell frequencies. In high-fiber tissue, macrophages co-localized with nerve fibers and expressed significantly less interleukin 23 than macrophages from low-fiber tissue. HSCR patients lacking mucosal nerve fibers showed microbial dysbiosis and had a higher incidence of postoperative enterocolitis. CONCLUSIONS: The mucosal fiber phenotype might serve as a prognostic marker for enterocolitis development in HSCR patients and may offer an approach to personalized patient care and new therapeutic options.
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Neuronas Colinérgicas/patología , Enterocolitis/etiología , Enfermedad de Hirschsprung/complicaciones , Mucosa Intestinal/inervación , Mucosa Intestinal/patología , Acetilcolinesterasa/metabolismo , Niño , Preescolar , Estudios de Cohortes , Citocinas/metabolismo , Disbiosis/inmunología , Disbiosis/microbiología , Disbiosis/patología , Células Epiteliales/metabolismo , Células Epiteliales/patología , Femenino , Enfermedad de Hirschsprung/patología , Enfermedad de Hirschsprung/cirugía , Humanos , Lactante , Recién Nacido , Inflamación/inmunología , Receptores de Lipopolisacáridos/metabolismo , Macrófagos/metabolismo , Masculino , Fenotipo , ARN Mensajero/genética , ARN Mensajero/metabolismo , Factores de RiesgoRESUMEN
Infants affected by Hirschsprung disease (HSCR), a neurodevelopmental congenital disorder, lack ganglia of the intrinsic enteric nervous system (aganglionosis) in a variable length of the colon, and are prone to developing severe Hirschsprung-associated enterocolitis (HAEC). HSCR patients typically show abnormal dense innervation of extrinsic cholinergic nerve fibers throughout the aganglionic rectosigmoid. Cholinergic signaling has been reported to reduce inflammatory response. Consequently, a sparse extrinsic cholinergic innervation in the mucosa of the rectosigmoid correlates with increased inflammatory immune cell frequencies and higher incidence of HAEC in HSCR patients. However, whether cholinergic signals influence the pro-inflammatory immune response of intestinal epithelial cells (IEC) is unknown. Here, we analyzed colonic IEC isolated from 43 HSCR patients with either a low or high mucosal cholinergic innervation density (fiber-low versus fiber-high) as well as from control tissue. Compared to fiber-high samples, IEC purified from fiber-low rectosigmoid expressed significantly higher levels of IL-8 but not TNF-α, IL-10, TGF-ß1, Muc-2 or tight junction proteins. IEC from fiber-low rectosigmoid showed higher IL-8 protein concentrations in cell lysates as well as prominent IL-8 immunoreactivity compared to IEC from fiber-high tissue. Using the human colonic IEC cell line SW480 we demonstrated that cholinergic signals suppress lipopolysaccharide-induced IL-8 secretion via the alpha 7 nicotinic acetylcholine receptor (a7nAChR). In conclusion, we showed for the first time that the presence of a dense mucosal cholinergic innervation is associated with decreased secretion of IEC-derived pro-inflammatory IL-8 in the rectosigmoid of HSCR patients likely dependent on a7nAChR activation. Owing to the association between IL-8 and enterocolitis-prone, fiber-low HSCR patients, targeted therapies against IL-8 might be a promising immunotherapy candidate for HAEC treatment.
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Colon , Sistema Nervioso Entérico/metabolismo , Células Epiteliales/metabolismo , Enfermedad de Hirschsprung/metabolismo , Interleucina-8/metabolismo , Línea Celular , Colon/inervación , Colon/metabolismo , Femenino , Humanos , Lactante , MasculinoRESUMEN
BACKGROUND: In medical settings, multisource feedback (MSF) is a recognised method of formative assessment. It collects feedback on a doctor's performance from several perspectives in the form of questionnaires. Yet, no validated MSF questionnaire has been publicly available in German. Thus, we aimed to develop a German MSF questionnaire based on the CanMEDS roles and to investigate the evidence of its validity. METHODS: We developed a competency-based MSF questionnaire in German, informed by the literature and expert input. Four sources of validity evidence were investigated: (i) Content was examined based on MSF literature, blueprints of competency, and expert-team discussions. (ii) The response process was supported by analysis of a think-aloud study, narrative comments, "unable to comment" ratings and evaluation data. (iii) The internal structure was assessed by exploratory factor analysis, and inter-rater reliability by generalisability analysis. Data were collected during two runs of MSF, in which 47 residents were evaluated once (first run) or several times (second and third run) on 81 occasions of MSF. (iv) To investigate consequences, we analysed the residents' learning goals and the progress as reported via MSF. RESULTS: Our resulting MSF questionnaire (MSF-RG) consists of 15 items and one global rating, which are each rated on a scale and accompanied by a field for narrative comments and cover a construct of a physician's competence. Additionally, there are five open questions for further suggestions. Investigation of validity evidence revealed that: (i) The expert group agreed that the content comprehensively addresses clinical competence; (ii) The response processes indicated that the questions are understood as intended and supported the acceptance and usability; (iii) For the second run, factor analysis showed a one-factor solution, a Cronbach's alpha of 0.951 and an inter-rater reliability of 0.797 with 12 raters; (iv) There are indications that residents benefitted, considering their individual learning goals and based on their ratings reported via MSF itself. CONCLUSIONS: To support residency training with multisource feedback, we developed a German MSF questionnaire (MSF-RG), which is supported by four sources of validity evidence. This MSF questionnaire may be useful to implement MSF in residency training in German-speaking regions.
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Internado y Residencia , Lenguaje , Competencia Clínica , Retroalimentación , Humanos , Reproducibilidad de los Resultados , Encuestas y CuestionariosRESUMEN
The original version of this article unfortunately contained a mistake in the author name Sasha Job Tharakan. The corrected name is given above.
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The purpose of this single-centre randomized controlled trial was to assess the non-inferiority of buddy taping versus splint immobilization of extra-articular paediatric finger fractures. Secondary fracture displacement was the primary outcome; patient comfort, cost and range of finger motion were secondary outcomes. Ninety-nine children were randomly assigned to taping or splinting. Sixty-nine fractures were undisplaced; 31 were displaced and required reduction before taping or splinting. Secondary displacement occurred in one patient in the taping and three in the splinting group. The risk difference was below the predefined non-inferiority level of 5%. All secondary displacements occurred in the 31 displaced fractures after reduction and were in little fingers. Patient comfort was significantly higher and cost lower in the taping group. We conclude from this study the non-inferiority of buddy taping versus splint immobilization of extra-articular paediatric finger fractures in general. We advise treatment may need to be individualized for patients with displaced fractures because we cannot make any absolute conclusions for these fractures. Level of evidence: I.
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Cinta Atlética , Falanges de los Dedos de la Mano/lesiones , Fracturas Óseas/terapia , Férulas (Fijadores) , Adolescente , Cinta Atlética/economía , Niño , Preescolar , Femenino , Humanos , Masculino , Comodidad del Paciente , Férulas (Fijadores)/economíaRESUMEN
PURPOSE: Needle manometry is a tool to confirm suspected acute compartment syndrome (ACS). There is scarce evidence of normal pressure values of the lower extremities in children. The aim of this study is to assess the normal compartment pressures in non-injured lower extremities of children. METHODS: This prospective study included children up to the age of 16 years with lower extremity fractures that needed reduction. Between June 2009 and August 2015, 20 children were included. We used needle manometry to measure the pressures in the superficial (SPC), deep posterior (DPC) and in the anterior compartments (AC) on both the lower legs. RESULTS: On the healthy leg, the mean compartment pressure was 15.15 mmHg in the AC (range 7-30 mmHg), 14.32 mmHg in the SPC (range 8-24 mmHg) and 13.00 mmHg in the DPC (range 4-21 mmHg). On the injured leg, the mean compartment pressure was 24.07 mmHg in the AC (range 5-40 mmHg), 17.21 mmHg in the SPC (range 7-29 mmHg) and 17.13 mmHg in the DPC (range 6-37 mmHg). We found a perfusion gradient (diastolic blood pressure-compartment pressure) < 30 mmHg in at least one compartment of the fractured and healthy leg in 13 patients. Five patients underwent fasciotomy for suspected ACS and their data was excluded for the injured leg. CONCLUSION: We could show that children have higher normal compartment pressures than adults in the lower leg. They seem to be able to tolerate higher absolute compartment pressures and lower pressure gradients before ACS occurs. More studies are needed to make a final statement on tolerable compartment pressures in children.
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Síndromes Compartimentales/diagnóstico , Fracturas Óseas/cirugía , Extremidad Inferior/lesiones , Presión , Adolescente , Estudios de Casos y Controles , Niño , Preescolar , Síndromes Compartimentales/etiología , Femenino , Fracturas Óseas/complicaciones , Humanos , Masculino , Manometría , Valores de ReferenciaRESUMEN
OBJECTIVES: Di(2-ethylhexyl) phthalate (DEHP) is a plasticizer used in many polyvinylchloride medical devices and is washed out easily. Thereby critically ill infants can become exposed to DEHP concentrations significantly exceeding the recommended threshold. We suspect DEHP to play an important role in the development of intestinal failure-associated liver disease. The aim of this study was therefore to determine the direct influence of DEHP on different liver cell types. METHODS: HepG2, human upcyte hepatocytes, primary murine hepatocytes, LX-2, human upcyte hepatic stellate cells, and liver organoids were cultured with DEHP (0.5-500âµmol/L) and parameters including cytotoxicity, cell-cell interactions, and expression of metabolizing enzymes were investigated. RESULTS: DEHP modulated the expression of xenobiotic metabolizing enzymes, reduced the formation of bile canaliculi and cell polarity, and inhibited Cyp-activity in hepatocytes. DEHP had a toxic effect on LX-2 and induced the fibrogenic activation of hepatic stellate cells. The mode of action of DEHP was different in monolayer cultures compared to 3D-liver organoids, which were more sensitive to DEHP. CONCLUSIONS: This study suggests that DEHP modulates expression and activity of drug-detoxifying liver enzymes in humans at a clinically relevant concentration. Furthermore, it may contribute to the development of cholestasis and fibrosis. These findings strongly support the opinion, that there is a significant potential for serious adverse effects of DEHP derived from medical devices on human health, especially in very young infants with immature livers.
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Colestasis/inducido químicamente , Dietilhexil Ftalato/farmacología , Hígado/efectos de los fármacos , Animales , Técnicas de Cultivo de Célula , Dietilhexil Ftalato/efectos adversos , Técnica del Anticuerpo Fluorescente , Humanos , Inmunohistoquímica , Hígado/citología , Hígado/patología , Ratones , Reacción en Cadena en Tiempo Real de la PolimerasaRESUMEN
INTRODUCTION: Evidence-based guidelines or protocols regarding the perinatal management of babies born with gastroschisis are lacking. The aim of this work is to evaluate the different current treatment modalities for newborns with gastroschisis during the perinatal period in the German-speaking countries Germany, Austria, and Switzerland. These data could serve as a starting point for the development of a multicenter randomized controlled trial. MATERIALS AND METHODS: A questionnaire was developed with 30 questions divided into five sections: (1) prenatal diagnosis, (2) fetal therapy, (3) mode and timing of delivery, (4) operative management, and (5) postoperative management. All pediatric surgery institutions that treat newborns with gastroschisis were identified and asked to participate. Data were categorized by country and analyzed using descriptive statistics (frequency and percentage). RESULTS: The return rate of the questionnaire was 95% (89 hospitals). A standard procedure was identified regarding prenatal ultrasound monitoring, interdisciplinary team approach, planned delivery through cesarean section, postnatal coverage of the intestine with a silastic bag, first intervention within the first 6 hours after birth, attempt of primary abdominal wall closure, and perioperative antibiotic treatment. For many crucial parameters, management was not standardized. CONCLUSIONS: There is no gold standard in German-speaking countries on how to manage fetuses and babies with gastroschisis. Moreover, this report unveils some questionable elements of daily practice for which there is no evidence at all and which can jeopardize outcome and even prove fatal (fetal therapy, preterm delivery, lack of abdominal pressure monitoring). Prospective randomized-controlled multicenter studies are needed to set a standard.
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Gastrosquisis/cirugía , Atresia Intestinal/cirugía , Intestinos/cirugía , Austria , Criptorquidismo/complicaciones , Criptorquidismo/cirugía , Parto Obstétrico/estadística & datos numéricos , Femenino , Terapias Fetales , Gastrosquisis/complicaciones , Gastrosquisis/diagnóstico , Alemania , Edad Gestacional , Humanos , Recién Nacido , Atresia Intestinal/complicaciones , Masculino , Cuidados Posoperatorios , Guías de Práctica Clínica como Asunto , Embarazo , Diagnóstico Prenatal , Encuestas y Cuestionarios , SuizaRESUMEN
Plasticizers migrate from polyvinylchloride (PVC) infusion systems into lipid emulsions. The aim of this study was to investigate the leaching of different plasticizers from PVC perfusion lines by a selection of lipid emulsions under clinical conditions. Seven PVC perfusion lines with an equal length of 150cm and three internal diameters were perfused with three lipid emulsions: Intralipid® 20%, ClinOleic® 20% and SMOFlipid® 20%, mimicking clinical conditions. The concentrations of the plasticizers were measured directly in the emulsions by gas chromatography - mass spectrometry. Of the four plasticizers examined in this study, di (2-ethylhexyl) phthalate (DEHP) leached the most and was found, on average, at 46.5µg/ml in the emulsions - around one order of magnitude higher than the other plasticizers. This study demonstrates that the leaching of DEHP by lipid emulsions in conditions of total parenteral nutrition is many times higher than should be accepted and higher when compared to the other plasticizers. There was no significant difference in leaching of plasticizers in relation to the type of lipid emulsion. The influence of tube diameter on the leaching rate of plasticizers should be taken into account especially in particular exposed patients.
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Emulsiones Grasas Intravenosas/química , Recien Nacido Prematuro , Perfusión/instrumentación , Plastificantes/análisis , Plastificantes/química , Cloruro de Polivinilo/química , Humanos , Recién NacidoRESUMEN
INTRODUCTION: Acute compartment syndrome (ACS) can lead to irreversible damage if fasciotomy is not performed in a timely manner. Needle manometry is a tool to confirm suspected ACS. The threshold for compartment pressures that can be tolerated has been debated. The aim of this study is to assess the normal compartment pressures in noninjured forearms of children. Further, we sought to quantify the maximum tolerable compartment pressures in fractured forearms of children, thus establishing a baseline and providing guidance in evidence-based decision making to evaluate children with suspected ACS. METHODS: This prospective study included children up to the age of 16 years with forearm fractures that needed reduction with or without osteosynthesis. Between June 2009 and March 2013, 41 children were included. Mean age was 9.25 years (range, 4 to 15.4 y). We used needle manometry to measure the pressures in the superficial and deep volar as well as in the dorsal compartments (DCs) on both the forearms. The mean pressures between compartments in healthy versus injured arms were analyzed using a 1-sided, paired t test. RESULTS: On the injured side, the mean compartment pressure was 19.12 mm Hg (range, 3 to 49 mm Hg) in the deep volar compartment, 15.56 mm Hg (range, 5 to 37 mmHg) in the DC, and 14.8 mm Hg (range, 2 to 35 mm Hg) in the superficial volar compartment. On the noninjured side, the mean compartment pressure was 12.9 mm Hg (range, 6 to 31 mm Hg) in the DC, 10.22 mm Hg (range, 3 to 22 mm Hg) in the deep volar compartment, and 9.66 mm Hg (range, 3 to 21 mm Hg) in the superficial volar compartment. We measured an absolute compartment pressure of >30 mm Hg in 15 patients on the fractured side. Three of them had an absolute compartment pressure of >45 mm Hg. Only 1 had ACS. This patient underwent fasciotomy and was excluded for further analysis. On follow-up (mean, 24.84 mo), no patient was found to have any sequelae of ACS. DISCUSSION: This is the first study to report normal compartment pressure measurements in noninjured forearms and in fractured forearms without clinical suspicion of ACS in children.The mean compartment pressure measured in the deep volar compartment (DVC) in healthy children was 10.22 mm Hg (range, 3 to 22 mm Hg) and therefore slightly higher than in adults. Some children with fractures tolerated absolute compartment pressures >30 mm Hg without clinical signs of ACS. Fasciotomy in children under close observation could eventually be delayed despite surpassing the accepted pressure limits for adults. LEVEL OF EVIDENCE: Level I-prognostic.
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Síndromes Compartimentales/fisiopatología , Traumatismos del Antebrazo/fisiopatología , Antebrazo , Fracturas Óseas/fisiopatología , Manometría , Presión , Adolescente , Estudios de Casos y Controles , Moldes Quirúrgicos , Niño , Preescolar , Reducción Cerrada , Síndromes Compartimentales/etiología , Síndromes Compartimentales/terapia , Fasciotomía , Femenino , Traumatismos del Antebrazo/complicaciones , Traumatismos del Antebrazo/terapia , Fijación Interna de Fracturas , Fracturas Óseas/complicaciones , Fracturas Óseas/terapia , Humanos , Masculino , Manipulación Ortopédica , Estudios Prospectivos , Valores de ReferenciaRESUMEN
Conjoined twins are a rare developmental anomaly with a reported prevalence of 1.47 per 100,000 births. We present an uncommon case of a parasitic ischiopagus tetrapus with a parasitic ischiopagus partial twin joined to the complete fetus at the level of the ischium diagnosed in utero by fetal MRI. The correct prenatal diagnosis led to birth by caesarean section. Prenatal MRI findings are presented and corroborated by postnatal imaging delineating the full extent and associated anomalies of this rare malformation. Differential diagnosis of duplicated lower extremities is discussed.
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Anomalías Múltiples/diagnóstico , Anomalías Múltiples/cirugía , Imagen por Resonancia Magnética , Diagnóstico Prenatal , Gemelos Siameses/cirugía , Anomalías Urogenitales/diagnóstico , Adulto , Cesárea , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Isquion/diagnóstico por imagen , Isquion/patología , Isquion/cirugía , Extremidad Inferior/diagnóstico por imagen , Extremidad Inferior/patología , Extremidad Inferior/cirugía , Masculino , Tomografía Computarizada Multidetector , Embarazo , Anomalías Urogenitales/cirugía , Sistema Urogenital/patología , Sistema Urogenital/cirugía , UrografíaRESUMEN
Dermatofibrosarcoma protuberans (DFSP) in childhood is a rare tumor with high recurrence rates. Wide local excision can result in disfiguring mutilation, whereas Mohs micrographic surgery (MMS) reduces surgical margins. MMS in children is not performed routinely, as the required infrastructures such as a histopathology lab in close proximity to the operating room is often lacking. We retrospectively reviewed children diagnosed with DFSP treated at our hospital over 2 years. We recorded surgical treatment details, including margins, duration of inpatient stay, outcome, follow-up, and molecular genetic tumor tissue analysis. Four children with a median age of 6.8 years (range 6.0-8.8 years) were identified who had a diagnostic delay of a median of 2.5 years (range 0.5-4.0 years); all underwent complete tumor excision using the slow MMS technique using vacuum-assisted closure systems between repeated excisions and before wound closure. The median maximal safety margins were 1.5 cm (range 1.0-3.0 cm). By using vacuum-assisted closure systems, no dressing changes were needed, pain was limited, and full mobility was maintained in all children. The median total time in the hospital was 11 days (range 10-14 days). No relapses occurred during a median follow-up of 25.8 months (range 11.3-32.6 months). Collagen 1A1/platelet-derived growth factor B (COL1A1/PDGFB) translocation on chromosomes 17 and 22 was detected in all three analyzable specimens. Lesions suspected of being DFSP warrant prompt histologic evaluation; interdisciplinary management is mandatory in particular for children. Micrographic surgery allows smaller surgical margins than wide excision and should be considered as the treatment of choice in children with DFSP. The interim usage of vacuum-assisted closure systems increases patient comfort. Translocations in the COL1A1/PDGFB gene imply susceptibility to targeted treatment modalities for therapy-resistant cases.
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Dermatofibrosarcoma/cirugía , Cirugía de Mohs/métodos , Terapia de Presión Negativa para Heridas/métodos , Neoplasias Cutáneas/cirugía , Niño , Diagnóstico Tardío , Dermatofibrosarcoma/diagnóstico , Femenino , Estudios de Seguimiento , Humanos , Tiempo de Internación , Masculino , Recurrencia Local de Neoplasia/prevención & control , Estudios Retrospectivos , Neoplasias Cutáneas/diagnóstico , Resultado del TratamientoRESUMEN
BACKGROUND/PURPOSE: Malrotation with a common mesentery is the classical pathology allowing midgut volvulus to occur. There are only a few reports of small bowel volvulus without malrotation or other pathology triggering volvulation. We describe three cases of small bowel volvulus in very premature newborns with a perfectly normal intra-abdominal anatomy and focus on the question, what might have set off volvulation. METHODS: In 2005 to 2008, three patients developed small bowel volvulus without any underlying pathology. Retrospective patient chart review was performed with special focus on clinical presentation, preoperative management, intraoperative findings, and potential causative explanations. Mean follow-up period was 46 months. RESULTS: All patients were born between 27 and 31 weeks (mean 28 weeks) with a birth weight between 800 and 1,000 g (mean 887 g). They presented with an almost identical pattern of symptoms including sudden abdominal distension, abdominal tenderness, erythema of the abdominal wall, high gastric residuals, and radiographic signs of ileus. All of them were treated with intensive abdominal massage or pelvic rotation to improve bowel movement before becoming symptomatic. CONCLUSIONS: Properistaltic maneuvers including abdominal massage and pelvic rotation may cause what we term a "manufactured" volvulus in very premature newborns. Thus, this practice was stopped.
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Vólvulo Intestinal/cirugía , Intestino Delgado/cirugía , Masaje/efectos adversos , Abdomen , Anastomosis Quirúrgica/métodos , Diagnóstico Diferencial , Femenino , Estudios de Seguimiento , Humanos , Lactante , Recién Nacido , Vólvulo Intestinal/diagnóstico , Vólvulo Intestinal/etiología , Masculino , Radiografía Abdominal , Estudios Retrospectivos , Factores de TiempoRESUMEN
INTRODUCTION: Failure to detect and treat partial or complete avulsions of the nail bed may lead to severe nail deformity that predisposes to repeat injuries and is cosmetically inacceptable. Treatment of these injuries with split-thickness nail bed grafts (STNBGs) is controversial and no pediatric series has been published. METHODS: A retrospective, single center case series of nine fingers with complex nail bed injuries that were reconstructed primarily with STNBGs was performed. Surgical outcome and patient satisfaction were assessed. RESULTS: For six nail bed reconstructions, the nail bed graft was harvested from the injured finger, and for the remaining three from the great toe. Harvesting of the great toe's nail bed could be performed without removal of the nail plate by only lifting it up distally. Insufficient vascularization required primary flap coverage in six cases with three Moberg flaps, two palmar V-Y flaps, and one thenar flap. No flap was lost and all nail bed grafts had a 100% take. Only one patient required reoperation due to a hook- and split-nail deformity. All other patients were satisfied or very satisfied and the surgical outcome was least satisfactory in all but two patients. CONCLUSIONS: Primary reconstruction of complex nail bed injuries with STNBGs usually gives good cosmetic and functional results in children and prevents secondary nail growth disturbances reliably.
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Traumatismos de los Dedos/cirugía , Uñas/lesiones , Trasplante de Piel/métodos , Adolescente , Niño , Preescolar , Femenino , Humanos , Lactante , Masculino , Uñas/cirugía , Satisfacción del Paciente , Estudios Retrospectivos , Colgajos QuirúrgicosRESUMEN
INTRODUCTION: The transplantation of neural crest derived stem cells (NCSC) is a potent alternative for the treatment of Hirschsprung's disease (HSCR). Cells to be transplanted should find an appropriate microenvironment to survive and differentiate. Influences of HSCR-smooth-muscle-protein extracts upon isolated myenteric plexus cells, dissociated dorsal root ganglia and NCSC were studied in vitro to investigate the quality of this microenvironment effects. METHODS: Postnatal human gut from children undergoing colonic resection due to HSCR was divided in segments. Smooth muscle was dissected and homogenized. Glial-cell-line-derived-neurotrophic-factor (GDNF) and transforming-growth-factor-ß-1 (TGFß-1) concentration were measured in the homogenates from the individual segment using ELISA. Myenteric plexus and dissociated dorsal root ganglia (DRG) cultures, as well as NCSCs were exposed to protein extracts derived from ganglionic and aganglionic HSCR segments, and their effect upon neurite outgrowth, survival, and branching was evaluated. RESULTS AND CONCLUSIONS: The amount of the factors varied considerably between the individual segments and also from patient to patient. Four major expression patterns could be detected. While all extracts tested lead to a significant increase in neurite outgrowth compared to the control, extracts from proximal segments tended to have more prominent effects. In one experiment, extracts from all individual segments of a single patient were tested. Neurite outgrowth, neuronal survival, and branching pattern varied from segment to segment, but all HSCR-muscle-protein extracts increased neuronal survival and network formation. Smooth muscle protein from aganglionic bowel supports the survival and outgrowth of myenteric neurons and NCSCs and is so an appropriate target for neural stem cell treatment.
Asunto(s)
Microambiente Celular , Tracto Gastrointestinal/patología , Enfermedad de Hirschsprung/patología , Plexo Mientérico/crecimiento & desarrollo , Plexo Mientérico/patología , Agregación Celular , Diferenciación Celular , Proliferación Celular , Supervivencia Celular , Células Cultivadas , Preescolar , Ensayo de Inmunoadsorción Enzimática , Ganglios Espinales/metabolismo , Ganglios Espinales/patología , Regulación de la Expresión Génica , Factor Neurotrófico Derivado de la Línea Celular Glial/genética , Factor Neurotrófico Derivado de la Línea Celular Glial/metabolismo , Enfermedad de Hirschsprung/genética , Enfermedad de Hirschsprung/cirugía , Humanos , Lactante , Recién Nacido , Proteínas Musculares/metabolismo , Neuritas/metabolismo , ARN Mensajero/genética , ARN Mensajero/metabolismo , Extractos de Tejidos , Factor de Crecimiento Transformador beta/metabolismoRESUMEN
OBJECTIVE: Open dismembered pyeloplasty is considered the gold standard to treat ureteropelvic junction obstruction (UPJO) in children. Laparoscopic pyeloplasty (LP) and robot-assisted pyeloplasty (RAP) are increasingly popular. Our present protocol consists of using minimally invasive techniques for all children with UPJO. Here, we report our first 40 cases operated under this protocol. PATIENTS AND METHODS: Retrospective chart review of patients who underwent LP and RAP for UPJO between 2006 and 2010 was performed. Children younger than 4 years of age underwent LP and children aged 4 years and older with robot assistance. Results were assessed comparing pre- and postoperative imaging studies, operating time, hospital course and complications. RESULTS: Thirty-nine patients underwent 41 dismembered pyeloplasties (20 patients LP, 19 patients RAP). No conversions to open surgery were performed. The difference in operative time was statistically significant. The average hospital stay was 7 days (LP) and 6 days (RAP). All patients showed significant decrease of hydronephrosis and the overall success rate was 100%. The complication rate was 25% in the LP and 28% in the RAP group. CONCLUSION: Our data show that RAP and LP are effective to correct UPJO with similar outcomes and complication rates. None of the patients in this series required re-intervention to correct obstruction and the results are comparable with open surgery.