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.

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.

[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.

[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.

[Ten years of laparoscope hernia repairs. Indications and results]. / Diez años de herniorrafias laparoscópicas. Indicaciones y resultados.

INTRODUCTION: The inguinal hernia surgery is common in the pediatric population although laparoscopic management today, is not considered the standard technique. The aim of this study is to review the results of our study and evaluate the contralateral side in patients with unilateral hernia. MATERIAL AND METHODS: Retrospective study of laparoscopic herniorraphy at our hospital since the beginning of the art in 1999 with a total of 348 patients. The indications of laparoscopy have been the availability of umbilical hernia associated, doubt diagnosis, recurrence of open surgery, bilateral tumor and casual diagnosis. We analyzed the following parameters: sex, age, weight, associated diagnosis, intra and postoperative complications, recurrence and follow-up. RESULTS: 474 hernias were repaired laparoscopically in 348 patients. A predominance of males against females (30%). In 37 patients (11%) the diagnosis was made during another operation. 39 patients with recidive open surgery were operated by laparoscopy. We had only 2% intraoperative complications and 2% of recurrences. The follow-up period is 10 years. CONCLUSIONS: Laparoscopic herniorraphy may be a routine procedure with results comparable to open surgery. Laparoscopy offers a good overview of the anatomy, easily identifying the defect, with a technique increasingly used in the field of pediatric surgery.

[Use of laparoscopy in neonatal pathology: our experience]. / Uso de la laparoscopia en la patología duodenal: nuestra experiencia.

The neonatal duodenal pathology has been diagnosed and treated with open surgery for many years. The use of minimally invasive techniques is widely use today on pediatric surgery, but its use on neonatal pathology poses a challenge. We have conducted a study of the 8 neonatal patients with duodenal obstruction that were operated with a laparoscopy in our hospital between 2001 and 2007. The analyzed parameters were the gender, prenatal diagnostic, type of duodenal malformation, weight at birth, hospitalization stay, start of feeding, complications and follow-up. In our sample (6 girls and 2 boys) the 62.5% of the cases was diagnosed after a prenatal ultrasound scan. The duodenal malformations discovered were 5 atresias, 2 webs and 1 anular pancreas. In all the cases, the feeding started by means of a trans-anastomotic probe 48 hours postoperatives. There were 3 complications: one re-surgery and 2 stenosis of anastomosis. The average hospitalization stay was of 27 days with a mean follow-up of 3 years. We think that laparoscopy is a good method for the treatment of the neonatal duodenal pathology, although the scarce volume of our sample does not allow us to generalize the technique.

[Thoracoscopic lobectomy in patients with congenital cystic adenomatoid malformation]. / Lobectomía toracoscópica en pacientes con malformación adenomatoidea quística.

BACKGROUND: The management of asymptomatic patients with congenital cystic adenomatoid malformation (CCAM) is controversial. This report evaluates the video-assisted thoracoscopic (VAT) lobectomy in children with this malformation, and the different intraoperative complications and their resolution are discussed. METHODS: Six patients with CCAM underwent video-assisted thoracoscopic lobectomy. All the patients were under one year and all of them were asymptomatic at the diagnosis. The procedures were performed with single lung ventilation and the chest was insufflated with a low flow and pressure to complete collapse of the lung. We used 3 or 4 thoracoscopic ports depending on the difficulty of the dissection. A bipolar sealing device was the preferred mode of vessel ligation and bronchi were closed with interrupted sutures. The following features have been taken into account: age at diagnosis, localization, surgical technique, complications, hospital stay, results, and time of follow-up. RESULTS: Four lesions were on the right lower lobe (66.7%) and two (33.3%) in the middle lobe. All the procedures were completed thoracoscopically. Chest tubes were left in all cases. Two patients (33.3%) showed postoperative hemothorax but it didn't need blood transfusion. Mean hospital stay was 6 days. At the moment all the patients are asymptomatic with and the mean time of follow-up has been 2 years and 8 months. CONCLUSIONS: VAT lobectomy avoids the long-term morbidity associated to an open thoracotomy and therefore it is a safe and efficacious technique in asymptomatic children with CCAM. Moreover, a greater number of cases are necessary to validate and to improve the technique.

[Minimally invasive surgery for the management of congenital diaphragmatic patology]. / Manejo de la patología diafragmáitica congénita con cirugía mínimamente invasiva.

INTRODUCTION: Minimally invasive surgery (MIS) plays a major role in pediatric surgery. We reported our experience in MIS management of congenital diaphragmatic patology (CDP). PATIENTS AND METHODS: The authors collected date on children who underwent a MIS for CDP repair from 1998 until 2006. The following features have been taken into account: lesion type, approach, surgical technique, complications, hospital stay and time of follow-up. RESULTS: From 1998 until 2006 11 patients (age range: 2 days-6 years and 6 months) with CDP had undergone an attempt at MIS repair: 6 patients with posterolateral hernia (36.4%), 4 with Morgagni hernia ( 54.5%) and a congenital diaphragmatic eventration case (9%). Eight patients 8 (72.7%) were treated using laparoscopy and three cases using thoracoscopy. Three patients were treated as newborns (27.2%). Four patients presented complications (36.3%): two patients who were repaired initially laparoscopically were converted to a transabdominally approach and two patients had recurrent herniation, which were repaired with MIS. Actually all cases were asymptomatic with a mean time of follow-up of 1 year and 8 months (range: 3 months-two years). CONCLUSIONS: MIS is a feasible, safe, easy to perform and efficient approach to repair CDP but it needs selection criteria for successful outcome.

[Laparoscopic inguinal herniorrhaphy in children. First results]. / Resultados iniciales del tratamiento de la hernia inguinal por vía laparoscópica.

INTRODUCTION: The inguinal hernia repair in the child has a new alternative, the laparoscopic repair. We study our initial results obtained with this technique. We evaluate the indications and the efficiency. MATERIAL AND METHODS: 150 patients have been operated with an initial diagnosis of hernia inguinal or crural. Usually this technique is realized with three ports: we put the umbilical one (5mm) for the scope and two ports (3 mm) placed in flanks. The repair was realized in purse string fashion or interrupted or noninterrupted suture with non-absorbable suture (polipropylen) 3-4/0. RESULTS: Indications of the herniorraphy were: (12%) recurrent hernia, bilateral hernia (28%), association inguinal hernia inguinal and umbilical (39%), crural hernia (4%) and in 16% incidental hernia. Most of them (137) was treated in purse string fashion, (11) non interrupted suture and 12 interrupted suture. No conversion to open was require in any case. Haematoma for puncture of the spermatic vessels was seen in 6 cases (1,4%). Today we do this surgery in ambulatory way. We have had 2 recurrent hernias (1%) that were treated again laparoscopicaly. CONCLUSIONS: We can't extract conclusion from this study because of the number of patients but we think that the laparoscopic treatment of inguinal hernias can offer advantages in selected cases.

[Results of laparoscopic treatment of complicated appendicitis]. / Resultados del tratamiento laparoscópico en la apendicitis complicada.

Laparoscopic appendectomy in children is a generally accepted procedure for the treatment of non-complicated acute appendicitis. Nevertheless, the role of laparoscopy in complicated cases is controversial. We show our experience with 40 cases of complicated acute appendicitis treated by means laparoscopy between February 2000 and October 2002. In every case we used 3 ports, one umbilical and the other two in both lower quadrants. The appendix was gangrenous in 31 patients and in the other 9 was perforated. Appendectomy was performed in an extracorporeal way in most of the cases. Average surgical time was 71 minutes and mean hospital stay was 8.8 days. Intraoperative complications occurred in 8 cases (20%) and postoperative complications were observed in 9 patients (22.5%). Four patients were reoperated (2 open and 2 laparoscopic) in order to drain two abscesses and treat two obstructions. The results of this serie is compared with another group of 40 complicated appendicitis operated in a classic open way in the same period of time. Postoperative complications were less often in the laparoscopic group. Oral intake, need for analgesia and hospital stay are more favorable in the laparoscopic group.

[Alternative treatment of gastroesophageal reflux: endoluminal gastric plication]. / Tratamiento alternativo del reflujo gastroesofágico: plicatura gástrica endoluminal.

Gastroesophageal reflux is a common chronic disorder that is presented in infants under different clinical pictures. Adolescents with heartburn, dependent on acid supression therapy is a group of patients refered with increased frequency to pediatric surgeons. To date, the accepted methods of management are long term medication or surgery. Recently, different endoscopic techniques have been described to treat gastroesophageal reflux. Endoscopic antireflux suturing is one of this developed methods. In this paper, we present the preliminary results in the treatment of pediatric patients with an endoscopic suturing device. The procedure was accomplished in six patients and the results were analized 6 months after the placement of the sutures. We have obtained an improvement in clinical symptoms reduction, in terms of frequency as well as in intensity. Acid supression therapy was also reduced significantly. There were no complications during the endoscopic procedure, neither in the postoperative course. Patients, were agree with the technique and the clinical results obtained. Endoscopic suturing is a safe procedure that can be an alternative in the treatment of selected patients with gastroesophageal reflux, in the pediatric age group.

[Thoracoscopic repair of esophageal atresia]. / Tratamiento toracoscópico de la atresia de esófago.

We present the first three cases of esophageal atresia with tracheoesophageal fistula treated with a thoracoscopic approach in our Service. The technique was accomplished through three trocar inserted in the right hemithorax. Carbon dioxide insufflation was required for lung retraction Primary correction was carried out in all the cases without major perioperative complications. The mean surgical time was 240 minutes. One patient had an anastomotic leak, resulting in a complicated postoperative course. The leak healed on conservative treatment. The others two patients were fed at the seventh postoperative day. Mean hospital stay in these cases was 12 days. Although thoracoscopic repair of esophageal atresia with fistula is a currently feasible technique that offers different advantages on the conventional open technique, a greater number of cases is needed in order to advance in the learning curve and determine the exact place that this technique can have in the future treatment of esophageal atresia.

[Jejunal diaphragm. Laparoscopic treatment in newborns]. / Atresia yeyunal membranosa. Tratamiento laparoscopico en periodo neonatal.

Intestinal obstructions in newborns secondary to atresias or membranes have been treated until the current moment through laparotomy and resection with anastomosis. Recently, it has been reported the use of minimally invasive techniques to correct this congenital anomaly. We present a case of jejunal diaphragm treated in our Service. Diagnosis and treatment by mean of endoscopic techniques was achieved in the fourth day of life. Operative time was 180 minutes. Postoperative course was uneventful. Results in terms of function and cosmetic were excellent. Laparoscopic management in selected cases of intestinal atresia has proven to be safe and effective and represents an alternative to neonatal open surgery.