Long-term recurrence of Dupuytren's disease treated with clostridium histolitycum collagenase. Surgical treatment and anatomopathological study.

OBJECTIVE: To present the functional results obtained and the possible surgical difficulties after the surgical treatment of Dupuytren's disease (DD) recurrence in patients previously treated with Clostridium histolyticum (CCH) collagenase. MATERIALS AND METHODS: In this prospective study, 178 patients with DD were treated with CCH from 2011 to 2018; During long-term postoperative follow-up, 34 patients (19.1%) had recurrence of DD. In all patients injected in the IFP the disease recurred; In patients injected in the MCP, recurrence was highest in grade III and IV of the Tubiana classification, with involvement of the 5th finger and the two-finger Y-chord. Fourteen patients (7,8%) required surgery by partial selective fasciectomy due to recurrence of cord DD infiltration. The clinical and functional results of the patients, the difficulty of the surgical technique and the anatomopathological analysis of the infiltrated cords were evaluated in comparison with those of cords and patients who had had no previous CCH treatment. RESULTS: In all patients, cord rupture was achieved after injection, reducing joint contracture. In 14 patients, we observed during the follow-up the existence of DD recurrence that required surgical treatment by selective partial fasciectomy. There were no major difficulties in surgery and good clinical and functional results at 6 months of follow-up. The anatomopathological study of the resected tissue did not present histological alterations with respect to the samples obtained from patients initially treated by selective partial fasciectomy. CONCLUSIONS: Selective fasciectomy after CCH injection does not lead to important operative difficulties, as long as the CCH injection is performed according to the recommendations. There were no histological changes in the tissue after CCH injection. LEVEL OF EVIDENCE: III.

Matrix Cauterization With Silver Nitrate in the Treatment of Ingrown Toenails in Children: Pilot Study / Cauterización de la matriz con nitrato de plata para el tratamiento de las uñas encarnadas en los dedos de los pies en niños: estudio piloto

Background Although partial onychectomy with chemical matricectomy has been described as the treatment of choice, there is sparse evidence in the literature regarding the use of silver nitrate for matricectomy. Our aim is to describe the effectiveness of silver nitrate for matrix cauterization after partial onychectomy. Methods A prospective observational study was performed on patients with ingrown toenails stage 2–3 who underwent partial onychectomy with silver nitrate chemical matricectomy during 2018–2019 in our institution. All patients were evaluated in the outpatient clinic on the 7th and 30th post-operative day and a telephone evaluation was performed every 6 months after the surgical procedure to date. Results One hundred and twenty-three patients, who underwent 231 partial onychectomies with silver nitrate chemical matricectomy were included, with a median follow-up of 21 months (interquartile range, 12–29). The procedure had an effectiveness of 95.3%, with only 11 recurrences (4.7%) reported so far on follow-up. Postoperative infections were observed in 4 patients (1.7%). Adverse effects, such as pain and postoperative drainage, were irrelevant in most patients. Conclusions Silver nitrate matricectomy after partial onychectomy is an effective and safe alternative for the treatment of ingrown toenail in children, with scarce postoperative morbidity and low recurrence rate (AU)

Antecedentes Aunque la onicectomía parcial con matricectomía química ha sido descrita como tratamiento de elección en los casos de uñas encarnadas en los dedos de los pies, existe escasa evidencia en la literatura en cuanto al uso de nitrato de plata para matricectomía. Nuestro objetivo es describir la efectividad del nitrato de plata para cauterización de la matriz tras onicectomía parcial. Métodos Se realizó un estudio observacional prospectivo en pacientes con uñas encarnadas en los dedos de los pies estadio 2-3, sometidos a onicectomía parcial con matricectomía con nitrato de plata durante los años 2018 y 2019 en nuestra institución. Se evaluó a todos los pacientes en la clínica ambulatoria el 7.° y 30.° días postoperatorios, realizándose una evaluación telefónica cada 6 meses, a contar desde la fecha de la intervención quirúrgica. Resultados Se incluyó a 123 pacientes, a quienes se realizaron 231 onicectomías parciales con matricectomía química con nitrato de plata, con un seguimiento medio de 21 meses (rango intercuartílico: 12-29). El procedimiento tuvo una efectividad del 95,3%, con solo 11 recidivas (4,7%) reportadas hasta el seguimiento. Se observaron infecciones postoperatorias en 4 pacientes (1,7%). Los efectos adversos, tales como dolor y el flujo postoperatorio, fueron irrelevantes en muchos pacientes. Conclusiones La matricectomía con nitrato de plata tras onicectomía parcial es una alternativa efectiva y segura para el tratamiento de las uñas encarnadas en niños, con escasa morbilidad postoperatoria y baja tasa de recidiva (AU)

[Artículo traducido] Cauterización de la matriz con nitrato de plata para el tratamiento de las uñas encarnadas en los dedos de los pies en niños: estudio piloto / Matrix Cauterization With Silver Nitrate in the Treatment of Ingrown Toenails in Children: Pilot Study

Antecedentes Aunque la onicectomía parcial con matricectomía química ha sido descrita como tratamiento de elección en los casos de uñas encarnadas en los dedos de los pies, existe escasa evidencia en la literatura en cuanto al uso de nitrato de plata para matricectomía. Nuestro objetivo es describir la efectividad del nitrato de plata para cauterización de la matriz tras onicectomía parcial. Métodos Se realizó un estudio observacional prospectivo en pacientes con uñas encarnadas en los dedos de los pies estadio 2-3, sometidos a onicectomía parcial con matricectomía con nitrato de plata durante los años 2018 y 2019 en nuestra institución. Se evaluó a todos los pacientes en la clínica ambulatoria el 7.° y 30.° días postoperatorios, realizándose una evaluación telefónica cada 6 meses, a contar desde la fecha de la intervención quirúrgica. Resultados Se incluyó a 123 pacientes, a quienes se realizaron 231 onicectomías parciales con matricectomía química con nitrato de plata, con un seguimiento medio de 21 meses (rango intercuartílico: 12-29). El procedimiento tuvo una efectividad del 95,3%, con solo 11 recidivas (4,7%) reportadas hasta el seguimiento. Se observaron infecciones postoperatorias en 4 pacientes (1,7%). Los efectos adversos, tales como dolor y el flujo postoperatorio, fueron irrelevantes en muchos pacientes. Conclusiones La matricectomía con nitrato de plata tras onicectomía parcial es una alternativa efectiva y segura para el tratamiento de las uñas encarnadas en niños, con escasa morbilidad postoperatoria y baja tasa de recidiva (AU)

Background Although partial onychectomy with chemical matricectomy has been described as the treatment of choice, there is sparse evidence in the literature regarding the use of silver nitrate for matricectomy. Our aim is to describe the effectiveness of silver nitrate for matrix cauterization after partial onychectomy. Methods A prospective observational study was performed on patients with ingrown toenails stage 2–3 who underwent partial onychectomy with silver nitrate chemical matricectomy during 2018–2019 in our institution. All patients were evaluated in the outpatient clinic on the 7th and 30th post-operative day and a telephone evaluation was performed every 6 months after the surgical procedure to date. Results One hundred and twenty-three patients, who underwent 231 partial onychectomies with silver nitrate chemical matricectomy were included, with a median follow-up of 21 months (interquartile range, 12–29). The procedure had an effectiveness of 95.3%, with only 11 recurrences (4.7%) reported so far on follow-up. Postoperative infections were observed in 4 patients (1.7%). Adverse effects, such as pain and postoperative drainage, were irrelevant in most patients. Conclusions Silver nitrate matricectomy after partial onychectomy is an effective and safe alternative for the treatment of ingrown toenail in children, with scarce postoperative morbidity and low recurrence rate (AU)

Matrix Cauterization With Silver Nitrate in the Treatment of Ingrown Toenails in Children: Pilot Study.

BACKGROUND: Although partial onychectomy with chemical matricectomy has been described as the treatment of choice, there is sparse evidence in the literature regarding the use of silver nitrate for matricectomy. Our aim is to describe the effectiveness of silver nitrate for matrix cauterization after partial onychectomy. METHODS: A prospective observational study was performed on patients with ingrown toenails stage 2-3 who underwent partial onychectomy with silver nitrate chemical matricectomy during 2018-2019 in our institution. All patients were evaluated in the outpatient clinic on the 7th and 30th post-operative day and a telephone evaluation was performed every 6 months after the surgical procedure to date. RESULTS: One hundred and twenty-three patients, who underwent 231 partial onychectomies with silver nitrate chemical matricectomy were included, with a median follow-up of 21 months (interquartile range, 12-29). The procedure had an effectiveness of 95.3%, with only 11 recurrences (4.7%) reported so far on follow-up. Postoperative infections were observed in 4 patients (1.7%). Adverse effects, such as pain and postoperative drainage, were irrelevant in most patients. CONCLUSIONS: Silver nitrate matricectomy after partial onychectomy is an effective and safe alternative for the treatment of ingrown toenail in children, with scarce postoperative morbidity and low recurrence rate.

Matrix Cauterization With Silver Nitrate in the Treatment of Ingrown Toenails in Children: Pilot Study. / [Artículo traducido] Cauterización de la matriz con nitrato de plata para el tratamiento de las uñas encarnadas en los dedos de los pies en niños: estudio piloto.

BACKGROUND: Although partial onychectomy with chemical matricectomy has been described asthe treatment of choice, there is sparse evidence in the literature regarding the use of silvernitrate for matricectomy. Our aim is to describe the effectiveness of silver nitrate for matrixcauterization after partial onychectomy. METHODS: A prospective observational study was performed on patients with ingrown toenailsstage 2-3 who underwent partial onychectomy with silver nitrate chemical matricectomy during 2018-2019 in our institution. All patients were evaluated in the outpatient clinic on the 7th and 30th post-operative day and a telephone evaluation was performed every 6 months afterthe surgical procedure to date. RESULTS: One hundred and twenty-three patients, who underwent 231 partial onychectomies with silver nitrate chemical matricectomy were included, with a median follow-up of 21 months (interquartile range, 12-29). The procedure had an effectiveness of 95.3%, with only 11 recur-rences (4.7%) reported so far on follow-up. Postoperative infections were observed in 4 patients (1.7%). Adverse effects, such as pain and postoperative drainage, were irrelevant in mostpatients. CONCLUSIONS: Silver nitrate matricectomy after partial onychectomy is an effective and safealternative for the treatment of ingrown toenail in children, with scarce postoperative morbidityand low recurrence rate.

Functional survival of cementless trapeziometacarpal total joint arthroplasty after upper extremity trauma.

INTRODUCTION: Our purpose with this publication is to document the survival of uncemented and unconstrained total trapeziometacarpal arthroplasty after energy trauma to the upper extremity. MATERIAL AND METHODS: From 1999 to the present, ten patients carrying total TMC arthroplasty suffered major traumatic injuries on the hand. Eight patients had fractures of the distal radius, one patient had scapho-lunate dissociation and one patient had a dorsal pullout of the triquetrum. A clinical and radiological examination of the patients after the trauma was carried out and compared with the pre-traumatic prosthesis status. RESULTS: Three patients required surgical intervention for the associated traumatic injury. The postraumatic clinical and functional follow-up of the patients was good, and no differences were documented with respect to mobility, strength and pain at the level of the thumb with respect to the prior to the trauma. No signs of loosening, instability or alteration in the alignment of the components of the protheses were observed in the radiological examinations following the trauma. CONCLUSIONS: There is a high survival rate of uncemented total trapeziometacarpal arthroplasty in the long term, even in the face of energy trauma. It is then a safe implant. Despite of being non-constrained, a good alignment of the prosthetic components is the key to avoid dislocation of the prosthesis.

Plastic adjustments in xylem vessel traits to drought events in three Cedrela species from Peruvian Tropical Andean forests.

Cedrela species occur within the Tropical montane cloud forest (TMCF) and rainforest in North America (Mexico), Central and South America. We assessed the hypothesis that functional xylem hydraulic architecture might be influenced by specific climatic variations. We investigated the effect of climate on tree-ring width and vessel traits (diameter, vessel density, vulnerability index and hydraulic diameter) of three relict-endemic and threatened Cedrela species (Cedrela fissilis, C. nebulosa and C. angustifolia) inhabiting Peruvian Tropical Andean cloud forests. All Cedrela species showed a significant reduction in radial growth and adjusted vessel trait linked with temperature, precipitation, and evapotranspiration. Ring-width and vessel traits showed adaptation within Cedrela species, crucial to understanding a rough indication of the plant's ability to withstand drought-induced embolism or cavitation. Our results provide evidence for hydraulic mechanisms that determine specific wood anatomical functionality to climatic variation and drought responses. Therefore, changing the frequency or intensity of future drought events might exceed the adaptive limits of TMCF tree species, resulting in a substantial reduction of hydraulic functionality in Peruvian Cedrela species.

Understanding climate change impacts on biome and plant distributions in the Andes: Challenges and opportunities.

Aim: Climate change is expected to impact mountain biodiversity by shifting species ranges and the biomes they shape. The extent and regional variation in these impacts are still poorly understood, particularly in the highly biodiverse Andes. Regional syntheses of climate change impacts on vegetation are pivotal to identify and guide research priorities. Here we review current data, knowledge and uncertainties in past, present and future climate change impacts on vegetation in the Andes. Location: Andes. Taxon: Plants. Methods: We (i) conducted a literature review on Andean vegetation responses to past and contemporary climatic change, (ii) analysed future climate projections for different elevations and slope orientations at 19 Andean locations using an ensemble of model outputs from the Coupled Model Intercomparison Project 5, and (iii) calculated changes in the suitable climate envelope area of Andean biomes and compared these results to studies that used species distribution models. Results: Future climatic changes (2040-2070) are projected to be stronger at high-elevation areas in the tropical Andes (up to 4°C under RCP 8.5), while in the temperate Andes temperature increases are projected to be up to 2°C. Under this worst-case scenario, temperate deciduous forests and the grasslands/steppes from the Central and Southern Andes are predicted to show the greatest losses of suitable climatic space (30% and 17%-23%, respectively). The high vulnerability of these biomes contrasts with the low attention from researchers modelling Andean species distributions. Critical knowledge gaps include a lack of an Andean wide plant checklist, insufficient density of weather stations at high-elevation areas, a lack of high-resolution climatologies that accommodates the Andes' complex topography and climatic processes, insufficient data to model demographic and ecological processes, and low use of palaeo data for distribution modelling. Main conclusions: Climate change is likely to profoundly affect the extent and composition of Andean biomes. Temperate Andean biomes in particular are susceptible to substantial area contractions. There are, however, considerable challenges and uncertainties in modelling species and biome responses and a pressing need for a region-wide approach to address knowledge gaps and improve understanding and monitoring of climate change impacts in these globally important biomes.

Incidental appendectomy in surgical treatment of ileocolic intussusception in children. Is it safe to perform? / Apendicectomía incidental en el tratamiento quirúrgico de la intususcepción ileocólica en niños. ¿Es segura?

BACKGROUND: Surgical treatment of ileo-colic intussusception (ICI) has been reported as the second cause of emergency laparotomy in children. The performance of incidental appendectomy after surgical reduction is currently controversial. The aim is to analyse the outcomes of performing incidental appendectomy after surgical ICI reduction with or without associated bowel resection. MATERIALS AND METHODS: A retrospective study was performed in patients with ICI episodes, who underwent surgical treatment in our institution between 2005-2019. Patients were divided in two groups according to the performance of associated appendectomy (AA group) or not (NA group). Subsequently, a stratified analysis was performed according to the need for bowel resection in both groups. Demographic variables, intraoperative findings, surgical time, hospital stay, postoperative complications and recurrences were analysed. RESULTS: A total of 101 patients (77 AA group; 24 NA group) were included, without differences in demographics or intraoperative findings. A total of 36 bowel resections were performed (24 group AA; 10 group NA), with no differences in surgical time (55.7 min in group AA vs. 61.2 min in group NA; p = 0.587) or hospital stay (median 5 days in both groups). There were also no differences in postoperative complications or recurrences between the two groups. Stratified analysis showed that bowel resection increases operative time, hospital stay and postoperative complications, regardless of whether associated appendectomy was performed or not. CONCLUSIONS: Incidental appendectomy during surgical treatment of ICI in children is a safe procedure that does not increase operative time, hospital stay, postoperative complications or recurrence.

OBJETIVO: El tratamiento quirúrgico de la intususcepción ileocólica (IIC) es la segunda causa más frecuente de laparotomía de urgencia en niños. La realización de una apendicectomía incidental tras la reducción quirúrgica sigue siendo motivo de controversia. El objetivo de este trabajo es analizar los resultados obtenidos al llevar a cabo una apendicectomía incidental tras la reducción quirúrgica de una IIC con o sin resección intestinal asociada. MATERIAL Y METODO: Se realizó un estudio retrospectivo en pacientes con episodios de IIC sometidos a tratamiento quirúrgico en nuestro centro entre 2005 y 2019. Los pacientes se dividieron en dos grupos según se llevara a cabo apendicectomía asociada (grupo AA) o no (grupo NA). Posteriormente, se elaboró un análisis estratificado según la necesidad de practicar resección intestinal en ambos grupos. Se analizaron las variables demográficas, los hallazgos intraoperatorios, el tiempo quirúrgico, la estancia hospitalaria, las complicaciones posoperatorias y las recidivas. RESULTADOS: Se incluyeron un total de 101 pacientes (77 en el grupo AA, y 24 en el grupo NA), sin diferencias en las características demográficas ni en los hallazgos intraoperatorios. Se practicaron un total de 36 resecciones intestinales (24 en el grupo AA; 10 en el grupo NA), sin diferencias en el tiempo quirúrgico (55,7 min en el grupo AA frente a 61,2 min en el grupo NA; p = 0,587) ni en la estancia hospitalaria (mediana de 5 días en ambos grupos). Tampoco se registraron diferencias en términos de complicaciones posoperatorias o recidivas entre los dos grupos. El análisis estratificado mostró que la resección intestinal incrementa el tiempo quirúrgico, la estancia hospitalaria y las complicaciones posoperatorias, con independencia de si se lleva a cabo apendicectomía asociada o no. CONCLUSION: La apendicectomía incidental durante el tratamiento quirúrgico de la IIC en niños es un procedimiento seguro que no aumenta el tiempo quirúrgico, la estancia hospitalaria, las complicaciones posoperatorias ni las posibilidades de recidiva.

Apendicectomía incidental en el tratamiento quirúrgico de la intususcepción ileocólica en niños. ¿Es segura? / Incidental appendectomy in surgical treatment of ileocolic intussusception in children. Is it safe to perform?

Objetivo: El tratamiento quirúrgico de la intususcepción ileocólica(IIC) es la segunda causa más frecuente de laparotomía de urgencia enniños. La realización de una apendicectomía incidental tras la reduc-ción quirúrgica sigue siendo motivo de controversia. El objetivo deeste trabajo es analizar los resultados obtenidos al llevar a cabo unaapendicectomía incidental tras la reducción quirúrgica de una IIC cono sin resección intestinal asociada. Material y método: Se realizó un estudio retrospectivo en pacientescon episodios de IIC sometidos a tratamiento quirúrgico en nuestro centroentre 2005 y 2019. Los pacientes se dividieron en dos grupos según sellevara a cabo apendicectomía asociada (grupo AA) o no (grupo NA). Posteriormente, se elaboró un análisis estratificado según la necesidad depracticar resección intestinal en ambos grupos. Se analizaron las variablesdemográficas, los hallazgos intraoperatorios, el tiempo quirúrgico, laestancia hospitalaria, las complicaciones posoperatorias y las recidivas. Resultados: Se incluyeron un total de 101 pacientes (77 en el gru-po AA, y 24 en el grupo NA), sin diferencias en las características demográficas ni en los hallazgos intraoperatorios. Se practicaron untotal de 36 resecciones intestinales (24 en el grupo AA; 10 en el grupoNA), sin diferencias en el tiempo quirúrgico (55,7 min en el grupo AAfrente a 61,2 min en el grupo NA; p = 0,587) ni en la estancia hospi-talaria (mediana de 5 días en ambos grupos). Tampoco se registrarondiferencias en términos de complicaciones posoperatorias o recidivasentre los dos grupos. El análisis estratificado mostró que la resecciónintestinal incrementa el tiempo quirúrgico, la estancia hospitalaria y las complicaciones posoperatorias, con independencia de si se lleva a caboapendicectomía asociada o no.(AU)

Background: Surgical treatment of ileo-colic intussusception (ICI)has been reported as the second cause of emergency laparotomy inchildren. The performance of incidental appendectomy after surgicalreduction is currently controversial. The aim is to analyse the outcomesof performing incidental appendectomy after surgical ICI reduction withor without associated bowel resection. Materials and methods: A retrospective study was performed inpatients with ICI episodes, who underwent surgical treatment in ourinstitution between 2005-2019. Patients were divided in two groupsaccording to the performance of associated appendectomy (AA group)or not (NA group). Subsequently, a stratified analysis was performedaccording to the need for bowel resection in both groups. Demographicvariables, intraoperative findings, surgical time, hospital stay, postopera-tive complications and recurrences were analysed.Results. A total of 101 patients (77 AA group; 24 NA group) wereincluded, without differences in demographics or intraoperative findings. A total of 36 bowel resections were performed (24 group AA; 10 groupNA), with no differences in surgical time (55.7 min in group AA vs.61.2 min in group NA; p = 0.587) or hospital stay (median 5 days in bothgroups). There were also no differences in postoperative complicationsor recurrences between the two groups. Stratified analysis showed thatbowel resection increases operative time, hospital stay and postopera-tive complications, regardless of whether associated appendectomy wasperformed or not.(AU)

Impact of the COVID-19 pandemic on acute appendicitis in children.

BACKGROUND AND OBJECTIVES: Since the COVID-19 pandemic confinement was established in Spain on March 9, 2020, the number of visits to the pediatric Emergency Department (ED) has decreased dramatically, probably due to the fear of parents becoming infected in the hospital environment. The aim of this work was to analyze the medium-term consequences during the first 9 months after the onset of the COVID-19 pandemic in children with acute appendicitis (AA). MATERIAL AND METHODS: A retrospective study was performed on children operated on for AA in our institution between 2017 and 2020, who were distributed in two groups according to the date of surgery: COVID-19 group (after March 9, 2020) and control group (before March 9, 2020). Demographic variables, associated symptoms, time from symptoms onset, hospital stay, rate of complicated AA and postoperative complications were analyzed. RESULTS: A total of 1274 patients were included (288 COVID group; 986 control group), without demographic differences. Time from symptom onset was significantly longer in COVID-19 group (34.5 vs. 24.2h; p=0.021), although no differences in associated symptoms were observed between both groups. COVID-19 group presented a higher rate of complicated AA (20.1% vs. 14%; OR: 1.55; CI 95% [1.10-2.18]; p=0.008), a longer hospital stay (3.5 vs. 2.8 days; p=0.042) as well as a higher rate of postoperative complications (21.5% vs. 15.7%; OR: 1.47; CI 95% [(1.06-2.04)]; p=0.008). CONCLUSION: In our experience there was a negative medium-term effects of the COVID-19 pandemic on children with acute appendicitis: delayed ED visits, increased rate of complicated AA, increased hospital stay and increased postoperative complications.

Aberrant muscular hyperplasia of the hand and PIK3CA related overgrowth spectrum disorders. A case series study. / Hiperplasia muscular aberrante de la mano y síndromes de sobrecrecimiento relacionados con el PIK3CA. Serie de casos.

INTRODUCTION: Congenital muscular hyperplasia of the hand is a rarely described entity, characterized by the presence of aberrant or accessory muscles in the hypothenar eminence, which has been always reported as sporadic anomaly in the medical literature. The aim of this paper is to report a series of cases with a phenotype of aberrant muscle hyperplasia associated with PIK3CA mutations. MATERIAL AND METHODS: We describe a retrospective series of cases followed at our institution between 2008 and 2020, with a unique phenotypein the context of PIK3CA mutations. RESULTS: A total of 6 patients were included (4 males and 2 females), who presented with an enlargement of the hypothenar eminence of the hand and peculiar wrinkling of the overlying skin, associated with ulnar deviation of the metacarpo-phalangeal joints. In all cases, variable degrees of congenital overgrowth of the ipsilateral limb were observed. Skin punch biopsy (5 mm diameter and 3-5 mm depth) was performed on 4 patients, where striated muscle hyperplasia and the presence of striated muscular fibers in the dermis and hypodermis were observed in all of them. Genetic studies revealed PIK3CA mutation in 3 of the 4 patients whose affected tissue was analyzed, in a mosaic state ranging from 3 to 8% (His1047Arg, Glu542Lys, and Cys420Arg, 1 case each). CONCLUSION: Aberrant muscular hyperplasia of the hand is a well-recognized entity scarcely described in association with PIK3CA spectrum disorder in the pediatric population. The role of PIK3CA in muscle overgrowth is not yet well understood.

INTRODUCCION: La hiperplasia muscular congénita de la mano es una entidad rara, caracterizada por la presencia de músculos aberrantes o accesorios en la eminencia hipotenar, que siempre se ha descrito en la literatura médica como una anomalía esporádica. El objetivo de este artículo es informar de una serie de casos con fenotipo de hiperplasia muscular aberrante asociada a mutaciones del PIK3CA. MATERIAL Y METODOS: Serie retrospectiva de casos atendidos en nuestro centro entre 2008 y 2020, con un fenotipo único en el contexto de las mutaciones del PIK3CA. RESULTADOS: Se incluyeron un total de 6 pacientes (4 masculinos y 2 femeninos) con ensanchamiento de la eminencia hipotenar de la mano y arrugamiento peculiar de la piel suprayacente, asociado a desviación ulnar de las articulaciones metacarpofalángicas. En todos los casos se observaron grados variables de sobrecrecimiento congénito del miembro ipsilateral. Se realizó biopsia cutánea por punch (5 mm de diámetro y 3-5 mm de profundidad) en 4 pacientes con hiperplasia muscular y presencia de fibras musculares estriadas en dermis e hipodermis. Los estudios genéticos revelaron mutación del PIK3CA en 3 de los 4 pacientes, cuyo tejido afectado fue enviado a analizar, con mosaicismo de entre el 3 y el 8% (His1047Arg, Glu542Lys y Cys420Arg respectivamente). CONCLUSION: La hiperplasia muscular aberrante de la mano es una entidad reconocida pero escasamente descrita en asociación con síndromes relacionados con el PIK3CA en la población pediátrica. A día de hoy sigue desconociéndose el papel del PIK3CA en el sobrecrecimiento muscular.

Hiperplasia muscular aberrante de la mano y síndromes de sobrecrecimiento relacionados con el PIK3CA. Serie de casos / Aberrant muscular hyperplasia of the hand and PIK3CA related overgrowth spectrum disorders. A case series study

Introducción: La hiperplasia muscular congénita de la mano esuna entidad rara, caracterizada por la presencia de músculos aberranteso accesorios en la eminencia hipotenar, que siempre se ha descrito enla literatura médica como una anomalía esporádica. El objetivo de esteartículo es informar de una serie de casos con fenotipo de hiperplasiamuscular aberrante asociada a mutaciones del PIK3CA. Material y métodos: Serie retrospectiva de casos atendidos ennuestro centro entre 2008 y 2020, con un fenotipo único en el contextode las mutaciones del PIK3CA. Resultados: Se incluyeron un total de 6 pacientes (4 masculinos y2 femeninos) con ensanchamiento de la eminencia hipotenar de la manoy arrugamiento peculiar de la piel suprayacente, asociado a desviaciónulnar de las articulaciones metacarpofalángicas. En todos los casos seobservaron grados variables de sobrecrecimiento congénito del miembroipsilateral. Se realizó biopsia cutánea por punch (5 mm de diámetro y 3-5mm de profundidad) en 4 pacientes con hiperplasia muscular y presenciade fibras musculares estriadas en dermis e hipodermis. Los estudiosgenéticos revelaron mutación del PIK3CA en 3 de los 4 pacientes, cuyotejido afectado fue enviado a analizar, con mosaicismo de entre el 3 y el8% (His1047Arg, Glu542Lys y Cys420Arg respectivamente). Conclusión: La hiperplasia muscular aberrante de la mano es unaentidad reconocida pero escasamente descrita en asociación con síndromes relacionados con el PIK3CA en la población pediátrica. A día dehoy sigue desconociéndose el papel del PIK3CA en el sobrecrecimientomuscular.(AU)

Introduction: Congenital muscular hyperplasia of the hand is ararely described entity, characterized by the presence of aberrant oraccessory muscles in the hypothenar eminence, which has been alwaysreported as sporadic anomaly in the medical literature. The aim of thispaper is to report a series of cases with a phenotype of aberrant musclehyperplasia associated with PIK3CA mutations. Material and Methods: We describe a retrospective series of casesfollowed at our institution between 2008 and 2020, with a unique phe-notypein the context of PIK3CA mutations. Results: A total of 6 patients were included (4 males and 2 females),who presented with an enlargement of the hypothenar eminence ofthe hand and peculiar wrinkling of the overlying skin, associated withulnar deviation of the metacarpo-phalangeal joints. In all cases, variabledegrees of congenital overgrowth of the ipsilateral limb were observed.Skin punch biopsy (5 mm diameter and 3-5 mm depth) was performedon 4 patients, where striated muscle hyperplasia and the presence ofstriated muscular fibers in the dermis and hypodermis were observedin all of them. Genetic studies revealed PIK3CA mutation in 3 of the 4patients whose affected tissue was analyzed, in a mosaic state rangingfrom 3 to 8% (His1047Arg, Glu542Lys, and Cys420Arg, 1 case each). Conclusion: Aberrant muscular hyperplasia of the hand is a well-recognized entity scarcely described in association with PIK3CA spec-trum disorder in the pediatric population. The role of PIK3CA in muscleovergrowth is not yet well understood.(AU)

The E LiBANS project: Thermal and epithermal neutron sources based on a medical Linac.

The E_LIBANS project (INFN) aims at producing neutron facilities for interdisciplinary irradiation purposes among which pre-clinical research for BNCT. After the successful setting-up of the thermal neutron source based on a medical LINAC, a similar apparatus for epithermal neutrons has been developed. Both structures are based on an Elekta 18 MV coupled with a photoconverter-moderator system which deploys the (γ,n) reaction to convert the X-rays into neutrons. This communication describes the two neutron sources and the results obtained in their characterization.

A new detector for the beam energy measurement in proton therapy: a feasibility study.

The proof of concept of a new device, capable of determining in a few seconds the energy of clinical proton beams by measuring the time of flight (ToF) of protons, is presented. The prototype consists of two thin ultra fast silicon detector (UFSD) pads, aligned along the beam direction in a telescope configuration and readout by a digitizer. The method developed for extracting the energy at the isocenter from the measured ToF, validated by Monte Carlo simulations, and the procedure used to calibrate the system are also presented and discussed in detail. The prototype was tested at the Centro Nazionale di Adroterapia Oncologica (CNAO, Pavia, Italy), at several beam energies, covering the entire clinical range, and using different distances between the sensors. The measured beam energies were benchmarked against the nominal CNAO energy values, obtained during the commissioning of the centre from the measured ranges in water. Deviations of few hundreds of keV have been achieved for all considered proton beam energies for distances between the two sensors larger than 60 cm, indicating a sensitivity to the corresponding beam range in water smaller than the clinical tolerance of 1 mm. Moreover, few seconds of irradiation were necessary to collect the required statistics. These preliminary results indicate that a telescope of UFSDs could achieve in a short time the accuracy required for the clinical application and therefore encourage further investigations towards the improvement and the optimization of the present prototype.