Primary Cutaneous Lymphoma Registry of the Spanish Academy of Dermatology and Venereology (AEDV): Data for the First 5 Years. / Registro de linfomas cutáneos primarios (RELCP) de la AEDV: datos tras 5 años de funcionamiento.

BACKGROUND AND OBJECTIVE: Primary cutaneous lymphomas (PCL) are uncommon. Observations based on the first year of data from the Spanish Registry of Primary Cutaneous Lymphomas (RELCP, in its Spanish abbreviation) of the Spanish Academy of Dermatology and Venereology (AEDV) were published in February 2018. This report covers RELCP data for the first 5 years. PATIENTS AND METHODS: RELCP data were collected prospectively and included diagnosis, treatments, tests, and the current status of patients. We compiled descriptive statistics of the data registered during the first 5 years. RESULTS: Information on 2020 patients treated at 33 Spanish hospitals had been included in the RELCP by December 2021. Fifty-nine percent of the patients were men; the mean age was 62.2 years. The lymphomas were grouped into 4 large diagnostic categories: mycosis fungoides/Sézary syndrome, 1112 patients (55%); primary B-cell cutaneous lymphoma, 547 patients (27.1%); primary CD30+lymphoproliferative disorders, 222 patients (11%), and other T-cell lymphomas, 116 patients (5.8%). Nearly 75% of the tumors were registered in stage I. After treatment, 43.5% achieved complete remission and 27% were stable at the time of writing. Treatments prescribed were topical corticosteroids (1369 [67.8%]), phototherapy (890 patients [44.1%]), surgery (412 patients [20.4%]), and radiotherapy (384 patients [19%]). CONCLUSION: The characteristics of cutaneous lymphomas in Spain are similar to those reported for other series. The large size of the RELCP registry at 5 years has allowed us to give more precise descriptive statistics than in the first year. This registry facilitates the clinical research of the AEDV's lymphoma interest group, which has already published articles based on the RELCP data.

Actitud de los pacientes con tumores cutáneos durante la pandemia de la COVID-19 / Attitudes of Patients With Skin Tumors During the COVID-19 Pandemic

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Divertículo de Meckel como causa de invaginación intestinal / Meckel's diverticulum as a cause of intestinal invagination

INTRODUCCIÓN: La invaginación intestinal representa la causa más frecuente de obstrucción intestinal entre los 6 meses y los 3 años, siendo secundaria en el 0,3-20% de los casos. El divertículo de Meckel es una causa común de invaginación secundaria y su tratamiento es fundamentalmente quirúrgico. MATERIAL Y MÉTODOS: Se revisaron retrospectivamente los casos codificados como invaginación intestinal en el Servicio de Urgencias Pediátricas del Hospital 12 de Octubre de Madrid entre 2013 y 2018. Se incluyeron 130 pacientes. RESULTADOS: En 10 de los 130 pacientes se identificó una causa subyacente: 5 linfomas intestinales, 4 divertículos de Meckel y 1 quiste de duplicación intestinal. Los casos secundarios fueron más frecuentes en varones (4:1), niños mayores de 3 años (60%) e invaginación ileocólica (90%). La invaginación por divertículo de Meckel afectó a 4 niños, 3 varones y 1 mujer, de entre 1 y 4 años. La ecografía diagnosticó la invaginación en los cuatro casos, pero sólo en uno sugirió divertículo de Meckel. En todos los casos, el tratamiento quirúrgico requerido incluyó reducción manual de la invaginación, seguida de diverticulectomía o resección intestinal más anastomosis. CONCLUSIONES: Las invaginaciones secundarias son más frecuentes en niños mayores de 3 años y en quienes fracasa el tratamiento conservador. El divertículo de Meckel es una de las causas más comunes de invaginación secundaria, sus manifestaciones clínicas son inespecíficas y su diagnóstico preoperatorio sigue suponiendo un reto en la actualidad. La utilidad del abordaje laparoscópico/laparoasistido en el tratamiento de las invaginaciones aún es limitada

INTRODUCTION: Intussusception is the most common cause of intestinal obstruction between 6 months and 3 years, having a pathological lead point in 0,3-20% of cases. Meckel's diverticulum is a frequent cause of secondary intussusception, which treatment is mostly surgical. METHODS: This work is a retrospective review of 130 patients diagnosed as intussusceptions. The samples were taken between 2013 and 2018, at the Department of Pediatric Emergency from the Hospital 12 de Octubre (Madrid). RESULTS: Pathological lead point was identified in ten cases (out of 130 patients): 5 intestinal lymphomas, 4 Meckel's diverticulums and 1 intestinal duplication cyst. Secondary intussusception was more common in males (4:1); children older than 3 years (60%); and patients with ileocolic intussusception (90%). Meckel's diverticulum affected 4 children between 1 to 4 years old, 3 males and 1 female. All were diagnosed for the intestinal invagination by ultrasound, but only in one case it suggested Meckel's diverticulum. Required surgical treatment always included manual reduction of intussusceptions, followed of diverticulectomy or intestinal resection plus anastomosis. CONCLUSIONS: Secondary intussusception is more common in children older than 3 years where conservative treatment fails. Meckel's diverticulum is one of the most common causes of secondary invagination, its clinical manifestations are not specific and, at present, its preoperative diagnosis is challenging. Currently, the use of the laparoscopic/laparoassisted approach is limited for the treatment of invaginations

Application of a novel material in the inguinal region using a totally percutaneous approach in an animal model: a new potential technique?

PURPOSE: To evaluate the feasibility and safety of a new percutaneous image-guided surgery technique to simulate a hernia repair using hydrogel. MATERIALS AND METHODS: A comparative prospective study was conducted in animals, with survival. Five pigs without any hernias were used. A hydrogel was injected at a site corresponding to the preperitoneal inguinal region. This procedure was performed bilaterally. An image-guided needle (ultrasound and computed tomography) was used, through which the material was injected. After survival, the local and systemic inflammatory reaction generated by the new material, was studied. RESULTS: All animals survived the procedure. No hemorrhagic or infectious complications were reported. The solidification of the material occurred as expected. In eight out of ten cases, the material was found in the planned site. No systemic inflammatory reaction secondary to the administration of hydrogel was reported. The adhesion of the material to surrounding tissues was satisfactory. CONCLUSION: The introduction of a liquid material which solidifies after injection in a short time (hydrogel) using a needle is feasible. The combined CT-scan and US image guidance allows for the percutaneous placement of the needle in the required location. The introduced hydrogel remains in this space, corresponding to the inguinal region, without moving. The placed hydrogel compresses the posterior wall composed of the transversalis fascia, supporting the potential use of hydrogel for hernia defects.

The impact of perioperative care on complications and short term outcome in ARM type rectovestibular fistula: An ARM-Net consortium study.

BACKGROUND: The impact of perioperative care interventions on postreconstructive complications and short-term colorectal outcome in patients with anorectal malformation (ARM) type rectovestibular fistula is unknown. METHODS: An ARM-Net consortium multicenter retrospective cohort study was performed including 165 patients with a rectovestibular fistula. Patient characteristics, perioperative care interventions, timing of reconstruction, postreconstructive complications and the colorectal outcome at one year of follow-up were registered. RESULTS: Overall complications were seen in 26.8% of the patients, of which 41% were regarded major. Differences in presence of enterostomy, timing of reconstruction, mechanical bowel preparation, antibiotic prophylaxis and postoperative feeding regimen had no impact on the occurrence of overall complications. However, mechanical bowel preparation, antibiotic prophylaxis ≥48 h and postoperative nil by mouth showed a significant reduction in major complications. The lowest rate of major complications was found in the group having these three interventions combined (5.9%). Multivariate analyses did not show independent significant results of any of the perioperative care interventions owing to center-specific combinations. At one year follow-up, half of the patients experienced constipation and this was significantly higher among those with preoperative mechanical bowel preparation. CONCLUSIONS: Differences in perioperative care interventions do not seem to impact the incidence of overall complications in a large cohort of European rectovestibular fistula-patients. Mechanical bowel preparation, antibiotic prophylaxis ≥48 h, and postoperative nil by mouth showed the least major complications. Independency could not be established owing to center-specific combinations of interventions. TYPE OF STUDY: Treatment study. LEVEL OF EVIDENCE: III.

Towards the perfect ARM center: the European Union's criteria for centers of expertise and their implementation in the member states. A report from the ARM-Net.

BACKGROUND: Pediatric surgeons and patient organisations agree that fewer centers for anorectal malformations with larger patient numbers are essential to reach better treatment. The European Union transacts a political process which aims to realize such centers of expertise for a multitude of rare diseases. All the centers on a specific rare disease should constitute an ERN on that disease. ARM-Net members in different countries report on first experiences with the implementation of national directives, identifying opportunities and risks of this process. METHODS: Relevant details from the official European legislation were analyzed. A survey among the pediatric surgeons of the multidisciplinary ARM-Net consortium about national implementation was conducted. RESULTS: European legislation calls for multidisciplinary centers treating children with rare diseases, and proposes a multitude of quality criteria. The member states are called to allocate sufficient funding and to execute robust governance and oversight, applying clear methods for evaluation. Participation of the patient organisations is mandatory. The national implementations all over Europe differ a lot in respect of extent and timeframe. CONCLUSIONS: Establishing Centers of Expertise and a ERN for anorectal malformations offers great opportunities for patient care and research. Pediatric surgeons should be actively engaged in this process.

European consensus meeting of ARM-Net members concerning diagnosis and early management of newborns with anorectal malformations.

The ARM-Net (anorectal malformation network) consortium held a consensus meeting in which the classification of ARM and preoperative workup were evaluated with the aim of improving monitoring of treatment and outcome. The Krickenbeck classification of ARM and preoperative workup suggested by Levitt and Peña, used as a template, were discussed, and a collaborative consensus was achieved. The Krickenbeck classification is appropriate in describing ARM for clinical use. The preoperative workup was slightly modified. In males with a visible fistula, no cross-table lateral X-ray is needed and an anoplasty or (mini-) posterior sagittal anorectoplasty can directly be performed. In females with a small vestibular fistula (Hegar size <5 mm), a primary repair or colostomy is recommended; the repair may be delayed if the fistula admits a Hegar size >5 mm, and in the meantime, gentle painless dilatations can be performed. In both male and female perineal fistula and either a low birth weight (<2,000 g) or severe associated congenital anomalies, prolonged preoperative painless dilatations might be indicated to decrease perioperative morbidity caused by general anesthesia. The Krickenbeck classification is appropriate in describing ARM for clinical use. Some minor modifications to the preoperative workup by Levitt and Peña have been introduced in order to refine terminology and establish a comprehensive preoperative workup.

Diseño y validación de un programa formativo en cirugía laparoscópica pediátrica y neonatal / Design and validation of a training model on paediatric and neonatal surgery

Presentamos nuestra experiencia en la fase de diseño y desarrollo de un programa formativo en cirugía laparoscópica pediátrica y neonatal y su validación subjetiva por parte de los asistentes. Los datos presentados en este trabajo han sido extraídos de las cinco ediciones del Curso de Cirugía Laparoscópica Pediátrica y Neonatal, desarrolladas en nuestro Centro, a las que han asistido 54 cirujanos. El modelo formativo, de 21 horas de duración, comienza con el conocimiento de aspectos generales de la ergonomía y del instrumental, tras lo cual los alumnos adquieren destrezas básicas mediante la práctica en simulador físico. Posteriormente, se acometen diversas técnicas en modelo animal, siempre asistidos por profesorado experto. Al término de las actividades, los asistentes evaluaron diversos aspectos didácticos y organizativos del programa formativo. Hemos obtenido una valoración muy positiva en los diferentes temas y técnicas del programa (≥ 9 puntos sobre 10). El 78,5% de los asistentes estuvo de acuerdo con la duración del curso, mientras que un 21,5% consideró que debería ser de mayor duración. El 79,1% se vio capacitado para realizar en pacientes las técnicas desarrolladas. El modelo formativo presentado ha demostrado poseer una muy alta valoración, aumentando la confianza de los asistentes para realizar las técnicas planteadas en la práctica clínica (AU)

We present our experience in the design and development of a training program in paediatric and neonatal laparoscopic surgery, and the determination of face validity by the attendants. Data included in the present study was obtained from five consecutive editions of our Neonatal and Paediatric Laparoscopic Surgery Course. Our training model, with a total duration of 21 hours, begins with acquisition of knowledge in ergonomics and instrument concepts, after which the attendants develop basic laparoscopic dexterity through the performance of hands-on physical simulator tasks. During the second and third days of the course, surgeons undertook various surgical techniques hands-on animal model. At the end of the training program, a subjective evaluation questionnaire was handed out to the attendants, in which different didactic and organizational aspects were considered. We obtained a highly positive score on all questions concerning the different topics and techniques included in the training program (≥9 points over 10). 78,5% of the 54 attendants was in accordance with the course total duration, whilst 21,5% considered that it should be of longer duration. Regarding abilities’ self assessment, 79,1% considered themselves capacitated to perform trained procedures on live patients. The presented training model has obtained a very positive valuation score, leading to an increase in the attendants’ self confidence in the application of learned techniques to their clinical practice (AU)

[Design and validation of a training model on paediatric and neonatal surgery]. / Diseño y validación de un programa formativo en cirugía laparoscópica pediátrica y neonatal.

We present our experience in the design and development of a training program in paediatric and neonatal laparoscopic surgery, and the determination of face validity by the attendants. Data included in the present study was obtained from five consecutive editions of our Neonatal and Paediatric Laparoscopic Surgery Course. Our training model, with a total duration of 21 hours, begins with acquisition of knowledge in ergonomics and instrument concepts, after which the attendants develop basic laparoscopic dexterity through the performance of hands-on physical simulator tasks. During the second and third days of the course, surgeons undertook various surgical techniques hands-on animal model. At the end of the training program, a subjective evaluation questionnaire was handed out to the attendants, in which different didactic and organizational aspects were considered. We obtained a highly positive score on all questions concerning the different topics and techniques included in the training program (> or = 9 points over 10). 78,5% of the 54 attendants was in accordance with the course total duration, whilst 21,5% considered that it should be of longer duration. Regarding abilities' self assessment, 79,1% considered themselves capacitated to perform trained procedures on live patients. The presented training model has obtained a very positive valuation score, leading to an increase in the attendants' self confidence in the application of learned techniques to their clinical practice.

[Endoscopic cholangiopancreatography in the elderly: a prospective comparative study in northern Mexico]. / Colangiopancreatografía endoscópica en la tercera edad; un estudio prospectivo y comparativo en el norte de México.

BACKGROUND: Endoscopic retrograde cholangiopancreatography (ERCP) is a widely used technique for the diagnosis and treatment of bilio-pancreatic diseases. According to Mexican Statistics, there is increasing life expectancy in Mexican population. The incidence of biliary tract pathologies is also increasing, leading to an increased demand of ERCP. AIM: Compare the utility and safety of ERCP in elderly and younger patients. METHODS: Prospective and comparative study including 450 patients who underwent ERCP during 2007. Patients were divided into two groups: 65 years age and older (group A) and less than 65 years old (group B). We registered gender, age, indication and length of the endoscopic procedure, morbidity and mortality. RESULTS: Mean patient age was 74.5 ± 6.9 and 43.0 ± 13.5 years old in groups A and B respectively. Choledocholithiasis was the more frequent diagnosis in both groups (48.62 %), followed by benign biliary stenosis (22.02 %) and malignant biliary obstruction (16.28 %). In 428 patients (98.16%) therapeutic procedures were performed. Endoscopic complications occurred in 1.37 % and there were not significant differences between groups (p = 0.218). There was no mortality. CONCLUSION: ERCP is a safe procedure in elderly patients with a very low rate of complications and excellent therapeutic efficacy.

[Difficulty to cannulate papilla of Vater in the presence of periampullary duodenal diverticulum]. / Grado de dificultad para canular papila de Vater en presencia de divertículo duodenal periampular.

BACKGROUND: There are conflicting results in the literature regarding the impact of duodenal diverticula on the technical success and complications of endoscopic retrograde cholangiopancreatography (ERCP). AIM: To evaluate if the presence of periampullary duodenal diverticulum increases the risk of failure of ampulla cannulation. METHODS: Patients who underwent ERCP between January 2008 and December 2009 were evaluated. They were divided in group A (without duodenal diverticulum) or group B (with duodenal diverticulum). Gender, age, endoscopic and radiological diagnosis, difficulty to cannulate, endoscopic sphincterotomy, precut technique, therapeutic procedure and complications were documented. RESULTS: 1159 patients were included: 1100 in group A and 59 in group B. A successful cannulation was obtained in 1061 patients of group A and 53 of group B (96.46 vs. 89.83%, p < 0.0001, OR 0.03). The failure of cannulation was observed in 39 patients of group A and 6 of group B (3.54 vs. 10.17%, p= 0.021, OR 2.94). The presence of intradiverticular papilla was the cause of failure in all cases. The therapeutic procedures showed statistical differences in choledocholithiasis clearance as well as endoscopic sphincterotomy and biliary stents insertion, but there was no significant difference in complications. We found statistical significant differences in biliary lithiasis, malignant stenosis, mechanical lithotripsy and insertion of biliary stents. CONCLUSIONS: Periampullary duodenal diverticula increase the risk of failure for cannulation of ampulla. However, it should not be considered as contraindication for ERCP.

[Does the use of fentanyl make Vater's ampulla cannulation difficult? A prospective and comparative study]. / ¿Dificulta la canulación del ámpula de Vater el uso de fentanilo? Un estudio prospectivo y comparativo.

BACKGROUND: Fentanyl is a synthetic opioid with excellent results in perioperative analgesia. It is commonly used for proximal and distal gastrointestinal endoscopic procedures, but its contracting action on the sphincter of Oddi, similar to that of morphine, makes its use for endoscopic cholangiopancreatography (ERCP) controversial. OBJECTIVE: To determinate if intravenous fentanyl as part of deep sedation hinders the cannulation of Vater's papilla during ERCP. MATERIAL AND METHODS: Prospective, comparative, randomized and double-blind trial that enrolled patients undergoing ERCP in 2008, > 18 years old, without previous endoscopic or surgical procedures related with Vater s papilla. Patients were randomized into two groups: patients in whom ERCP was performed with intravenous propofol (group A), and patients in whom the procedure was performed with intravenous fentanyl and propofol (group B). Gender, age, comorbid conditions, reasons for referral, difficulty of cannulation, diagnosis, therapeutic procedures, procedure time and endoscopic complications were all documented. RESULTS: 432 were included: 214 in group A and 218 in group B. Both groups were similar in relation with demographic characteristics, time of sedation and endoscopic procedure. Difficulty in cannulation had not a statistical significance (p = 0.163). The administered dose of propofol were less for group B (p < 0.001). No procedure-related mortality was documented. CONCLUSION: The combination of fentanyl and propofol may be used during ERCP, since it does not hinder the cannulation of Vater's papilla.

[Foreign body reaction to biodegradable esophageal prosthesis. A case report]. / Reacción a cuerpo extraño de una prótesis esofágica biodegradable. Informe de un caso.

Although benign esophageal stricture induced by various factors can often be managed with dilatations using hydrostatic balloons or different dilators, some patients have esophageal stenosis that is refractory to such treatment. Endoprothesis have facilitated the palliation of malignant esophageal strictures. However, the indications for permanent esophageal stenting in patients with benign esophageal strictures have not been established. Everyday, the use of plastic self-expanding endoprosthesis is more common in esophageal strictures because of their advantages over metallic stents, ease of placement and retrieval, and limited local tissue reaction. More recently, biodegradable stents have been used to manage benign esophageal stenosis. We report a case of a 72 years woman who was attended because of the presence of esophageal stenosis secondary to caustic ingestion refractory to dilatation, was placed a biodegradable stent and developed a foreign body reaction.