The effect of early radiofrequency turbinate reduction, intranasal steroid, and antihistamine H-1 on persistent allergic rhinitis: a randomized clinical trial

Abstract Objective: We aimed to evaluate the effect of radiofrequency turbinate reduction as an initial treatment on clinical improvement, inflammatory mediators, and remodeling process. Methods: Between July 2018- February 2020, 32 patients with moderate-severe persistent AR were randomly divided into 2 groups. Intervention group received radiofrequency turbinate reduction followed by intranasal steroid and Antihistamine H-1 (AH-1), control group received intranasal steroid and AH-1. Both groups were evaluated for clinical improvement (using visual analogue scale based on total nasal symptoms score, peak nasal inspiratory flow, and turbinate size using imageJ) after 4 and 8 weeks of treatment. Inflammatory mediators (ELISA from nasal secretions was performed to measure ECP, IL-5, and HSP-70) and remodeling markers (nasal biopsy followed by immunohistochemistry examination was performed to evaluate MMP-9, TIMP-1, and PAI-1) were evaluated in week 4. Results: Three patients dropped out of the study, resulting in 16 patients in intervention group and 13 patients in control group. At week 4, clinical response improved significantly in the intervention group compared to control group (Chi-Square test, p<0.05). Compared to control, intervention group experienced a reduction of IL-5 and no significant change in ECP level (Mann Whitney test, p>0.05). Reduction in the ratio of MMP-9/TIMP-1 were significantly higher in intervention group (unpaired t-test, p< 0,05). Meanwhile, increase in HSP-70 in the intervention group was slightly lower than in control group, but the difference with control group was not significant (Mann Whitney test, p>0.05). Conclusion: Early radiofrequency turbinate reduction followed by pharmacotherapy given to persistent moderate-severe AR patients give more improvement only in early clinical symptoms and reduce MMP-9/TIMP-1 ratio, thus it might be suggested as one of the adjuvant therapies for the management of moderate-severe persistent AR. However, further investigation with a larger sample size and longer follow-up period is needed. Level of evidence: 1B.

Cervical necrotizing fasciitis caused by oral cavity infection: a case report considering therapeutic approach and its morphofunctional implications / Fascitis necrosante cervical causada por infección de la cavidad oral. Reporte de un caso considerando el abordaje terapéutico y sus implicaciones morfofuncionales

SUMMARY: Cervical necrotizing fasciitis (NF) is a rare complication of oral cavity infection with high morbi-mortality. Given its low prevalence, adequately reporting cases of NF, its therapeutic management, and associated morphofunctional modifications to the clinical and scientific community is pivotal. To that end, we herein describe a case of cervical NF in a 60-year-old patient with comorbidities and patient presented large, painful cervical swelling associated with a necrotic ulcer lesion in the anterior neck region. Intraoral examination indicated a periodontal abscess in the right mandibular area, while computed tomography indicated the lesion's extension from the right mandibular to the submandibular region. Following empirical intravenous antibiotic treatment, a broad surgical debridement was performed, and the foci of oral infection were removed. Debridement revealed communication between deep and superficial anatomical regions in the submandibular area, where we subsequently placed a Penrose drain. Biopsies showing acute inflammatory infiltrate associated with necrotic and hemorrhagic regions confirmed the diagnosis of NF. When an antibiogram revealed resistance to the empirical treatment, the antibiotic scheme was replaced with an adequate alternative. After a second debridement, we closed the defect with fascio-mucocutaneous advancement flaps with a lateral base while maintaining suction drainage. Having reacted positively, the patient was discharged 10 days after the operation. Despite an extensive morphofunctional change generated in the treated area, the patient showed no difficulties with breathing, phonation, swallowing, or mobilizing the area during control sessions. Altogether, this report contributes to the highly limited literature describing morphological aspects that can facilitate or delay the spread of infection or the morphofunctional disorders associated with the size and depth of surgical interventions for cervical NF, information that is relevant for the comprehensive, long-term prognosis of the treatment of NF.

La fascitis necrosante (FN) cervical es una rara complicación de una infección proveniente de la cavidad bucal asociada a una alta morbimortalidad. Por lo anterior, es fundamental informar a la comunidad clínica y científica los casos de FN, su manejo terapéutico y las modificaciones morfofuncionales asociadas. Se describe un caso de FN cervical en una paciente de 60 años quien presentó una gran tumefacción dolorosa asociada a una lesión ulcerosa necrótica en la región anterior del cuello. El examen intraoral mostró un absceso periodontal en el área mandibular derecha y la tomografía computarizada mostró la extensión de la lesión hacia la región submandibular. Tras el tratamiento antibiótico empírico, se realizó un desbridamiento quirúrgico extenso y se extirparon los focos de infección oral. El desbridamiento reveló comunicación entre las regiones anatómicas profundas y superficiales del área submandibular, donde se colocó un drenaje Penrose. Las biopsias mostraron un infiltrado inflamatorio agudo asociado con regiones necróticas y hemorrágicas, confirmando el diagnóstico de FN. El antibiograma reveló resistencia al tratamiento empírico, por lo que el esquema antibiótico se sustituyó. Tras un segundo desbridamiento, se cerró el defecto con colgajos de avance fascio-mucocutáneos de base lateral manteniendo drenaje aspirativo. El positivo progreso del paciente permitió su alta 10 días después. Aun cuando se generó una gran modificación morfofuncional en el área tratada, la paciente no presentó dificultades para respirar, hablar, deglutir o movilizar el área cervical intervenida durante las sesiones de control. Este informe contribuye a la limitada literatura que describe los aspectos morfológicos que pueden facilitar o retrasar la propagación de la FN y las consecuencias asociadas a los trastornos morfofuncionales provocadas por el tamaño y profundidad de las intervenciones quirúrgicas requeridas por la FN, información relevante para el pronóstico integral a largo plazo del tratamiento de la FN.

Sphenopalatine ganglion block - a new treatment for burning mouth syndrome?: a case report

Abstract Burning mouth syndrome is a poorly understood entity for which current treatment modalities fail to provide effective relieve. Branches of the maxillary and mandibular nerves are responsible for the innervation of the affected area. These are also the nerves involved in trigeminal neuralgia, an entity where sphenopalatine block has proved to be effective. We present a case of a patient with burning mouth syndrome in whom a bilateral sphenopalatine ganglion block was successfully performed for pain treatment. It is an easy and safe technique that can be a valuable treatment option for these patients, although more studies are needed.

Múltiples reacciones adversas graves a fármacos en un paciente luego de la infección por COVID-19: informe de un caso / Drug-induced severe cutaneous adverse reactions in a patient after COVID-19 infection: case report

Durante la pandemia por COVID-19 se observaron diversas reacciones adversas a fármacos. Esto pudo haber estado relacionado con una mayor susceptibilidad inmunológica de los pacientes con SARS-CoV-2 a presentar este tipo de cuadros, así como también con la exposición a múltiples medicamentos utilizados en su tratamiento. Comunicamos el caso de un paciente con una infección respiratoria grave por COVID-19, que presentó 2 reacciones adversas graves a fármacos en un período corto de tiempo. (AU)

During the COVID-19 pandemic, various adverse drug reactions were observed. This could have been related to a greater immunological susceptibility of patients with SARS-CoV-2 to present this type of symptoms, as well as exposure to multiple drugs used in their treatment. We report the case of a patient with a severe respiratory infection due to COVID-19, who presented 2 serious adverse drug reactions associated with paracetamol in a short period of time. (AU)

Comparison Between Surgical and Conservative Treatment for AOSpine Type A3 and A4 Thoracolumbar Fractures without Neurological Deficit: Prospective Observational Cohort Study / Comparação entre tratamento cirúrgico e conservador para fraturas toracolombares AOSpine tipo A3 e A4 sem déficit neurológico: Estudo de coorte prospectivo observacional

Abstract Objective To compare the clinical results between conservative (CS) and surgical treatment (CXS) of A3 and A4 fractures without neurological deficit. Methods Prospective observational study of patients with thoracolumbar fractures type A3 and A4. These patients were separated between the surgical and conservative groups, and evaluated sequentially through the numeric rating scale (NRS), Roland-Morris disability questionnaire (RMDQ), EuroQol-5D (EQ-5D) quality of life questionnaire, and Denis work scale (DWS) up to 2.5 years of follow-up. Results Both groups showed significant improvement, with no statistical difference in pain questionnaires (NRS: CXS 2.4 ± 2.6; CS 3.5 ± 2.6; p> 0.05), functionality (RMDQ: CS 7 ± 6.4; CXS 5.5 ± 5.2; p> 0.05), quality of life (EQ-5D), and return to work (DWS). Conclusion Both treatments are viable options with equivalent clinical results. There is a tendency toward better results in the surgical treatment of A4 fractures.

Resumo Objetivo Comparar os resultados clínicos entre os tratamentos conservador (CS) e cirúrgico (CXS) das fraturas A3 e A4 sem déficit neurológico. Métodos Estudo prospectivo observacional de paciente com fraturas toracolombares tipo A3 e A4. Esses pacientes foram separados entre os grupos cirúrgico e conservador e avaliados sequencialmente através da escala numérica de dor (NRS), do questionário de incapacidade de Roland-Morris (RMDQ), do EuroQol-5D (EQ-5D) e da escala de trabalho de Denis (DWS) até 2,5 anos de acompanhamento. Resultados Ambos os grupos apresentaram melhora significante, sem diferença estatística nos questionários de dor (NRS: CXS 2,4 ± 2,6; CS 3,5 ± 2,6; p> 0,05), funcionalidade (RMDQ: CS 7 ± 6,4; CXS 5,5 ± 5,2; p> 0,05), qualidade de vida (EQ-5D) e retorno ao trabalho (DWS). Conclusão Ambos os tratamentos são opções viáveis e com resultados clínicos equivalentes. Há uma tendência a melhores resultados no tratamento cirúrgico das fraturas A4.

Investigación clínica en homeopatía: revisiones sistemáticas y estudios clínicos aleatorizados / Clinical research in homeopathy: systematic reviews and randomized clinical trials

Se considera que las revisiones sistemáticas y estudios clínicos aleatorizados (RCT) presentan el nivel de evidencia más alto. Objetivo: Realizar una revisión descriptiva de las revisiones sistemáticas y estudios clínicos controlados aleatorizados (RCT, por sus siglas en inglés) sobre la efectividad y eficacia de la homeopatía. Métodos: Los datos del reporte publicado por la Liga Medicorum Homeopathica Internationalis (LMHI) en 2014 fueron actualizados mediante una búsqueda en la base de datos PubMed. Resultados: Se localizaron 7 revisiones sistemáticas con meta-análisis, 6 de las cuales concluyeron que los efectos de la homeopatía no son compatibles con el efecto placebo; sólo una revisión sistemática llegó a la conclusión opuesta, aunque fue severamente criticado debido a sus errores metodológicos. Un total de 19 RCT fueron publicados a lo largo del periodo analizado. El 84.2% tuvo por lo menos un resultado positivo. Conclusiones: Con base en las evidencias disponibles del más alto nivel, no es posible afirmar que los efectos de la homeopatía son exclusivamente un efecto placebo. Por el contrario, se han detectado efectos específicos en varios estudios.

Systematic reviews and randomized clinical trials (RCT) are considered to have the highest level of evidence. Aim: To perform a descriptive review of systematic reviews and RCT on the effectiveness and efficacy of homeopathy. Methods: Data from the report published by Liga Medicorum Homeopathica Internationalis (LMHI) in 2014 were updated by means on a search conducted in database PubMed. Results: 7 systematic reviews with meta-analysis were located, 6 of them concluded that the effects of homeopathy are not compatible with placebo effect; only 1 systematic review arrived to the opposite conclusion, but was severely criticized due to methodological flaws. A total of 19 RCT were published along the analyzed period; 84.2% had at least one positive outcome. Conclusions: Based on the available evidences of the highest level it is not possible to assert that the effects of homeopathic are exclusively placebo effect. On the opposite, specific effects were detected in several studies.

Evidence map on the clinical effectiveness of anthroposophic medicine: executive report

The map provides an overview of the evidence on the effects of Anthroposophic Medicine, referred to in the National Policy on Integrative and Complementary Practices (PNPIC) as applied Anthroposophy in Health, due to its multiprofessional nature. Based on an extensive literature search, the map includes 33 review studies that analyzed the effects of interventions with anthroposophic medicines, anthroposophic therapies, and multimodal therapies on clinical outcomes. The map represents 63 associations between 5 types of interventions and 19 clinical outcomes, indicating the reported effect and level of confidence in the evidence reported in the studies. Key Findings: • The interventions were organized into three groups: Anthroposophic Medicines (Viscum album - 22 reviews and Other medicines - 4 reviews), Anthroposophic Therapies (Eurythmy - 2 reviews and Other therapies - 1 review), and Multimodal Therapies (Overall - 4 reviews). • The interventions were associated with 19 outcomes grouped into five categories: Well-being and Quality of Life, Cancer, Healthcare, Physiological and Metabolic Indicators, and other Pathological Conditions. • The majority of associations were found in the Cancer outcome group, followed by the Well-being and Quality of Life group. Notable outcomes included Quality of Life (12 reviews), Survival (9 reviews), Patient Safety (8 reviews), and Chemotherapy and Radiotherapy Symptoms (7 reviews). • Among the interventions, the majority of associations were related to Viscum album (48 associations). • Multimodal interventions, other medicines, eurythmy, and other therapies were associated with outcomes such as Treatment Outcome (5 associations), Patient Satisfaction and Safety (2 associations each), Respiratory Infections (3 associations), Cardiorespiratory Coordination, Gastrointestinal Disorders, and Edema (1 association each). Implications for Practice and Research: • Considering the reported positive effects (in 27 associations), the use of anthroposophic medicines Viscum album (19 associations) stands out for Quality of Life, Patient Safety, and Chemotherapy and Radiotherapy Symptoms outcomes. • Regarding potentially positive effects (in 21 associations), the majority were associated with Viscum album (18 associations) for Cancer Survival, Chemotherapy and Radiotherapy Symptoms, and Quality of Life outcomes. • Inconclusive effects were identified in 15 associations for 11 different outcomes, suggesting a need for further primary studies on interventions with anthroposophic medicines and therapies. • No studies were identified with no effect or negative effects.

Mapa de evidências sobre a efetividade clínica da medicina antroposófica: informe executivo / Evidence map on the clinical effectiveness of anthroposophic medicine: executive report

O mapa apresenta uma visão geral das evidências sobre os efeitos da Medicina Antroposófica, referida na PNPIC como Antroposofia Aplicada à Saúde, em função de sua natureza multiprofissional. A partir de uma ampla busca bibliográfica foram incluídos no mapa 33 estudos de revisão que analisaram o efeito de intervenções com medicamentos antroposóficos, terapias antroposóficas e terapias multimodais para desfechos clínicos. No mapa estão representadas 63 associações entre 5 tipos de intervenções e 19 desfechos clínicos, com indicação do efeito reportado e nível de confiança da evidência reportada nos estudos. Principais Achados: • As intervenções foram organizadas em três grupos: Medicamentos Antroposóficos (Viscum album ­ 22 revisões e Outros medicamentos ­ 4 revisões), Terapias Antroposóficas (Euritmia ­ 2 revisões e Outras terapias ­ 1 revisão) e Terapias Multimodais (Em Geral ­ 4 revisões). • As intervenções foram associadas a 19 desfechos ordenados em cinco grupos: Bem-estar e Qualidade de Vida, Câncer, Atenção à Saúde, Indicadores Fisiológicos e Metabólicos e Outras Condições Patológicas. • A maioria das associações foi para o grupo de desfecho Câncer, seguido pelo grupo Bem-estar e Qualidade de Vida. Dentre os desfechos, destaque para: Qualidade de Vida (12 revisões), Sobrevida (9 revisões), Segurança do Paciente (8 revisões) e Sintomas de Quimioterapia e Radioterapia (7 revisões). • Dentre as intervenções, a maioria das associações foi para Viscum album (48 associações). • As intervenções multimodais, outros medicamentos, Euritmia e outras terapias foram associadas aos desfechos: Resultado do tratamento (5 associações), Satisfação e Segurança do paciente (2 associações cada), Infecções respiratórias (3 associações), Coordenação cardiorrespiratória, Transtornos gastrointestinais e Edema (1 associação cada). Implicações para a prática e pesquisa: • Considerando os efeitos positivos reportados (em 27 associações), destaque para o uso de medicamento antroposófico Viscum album (19 associações) para os desfechos Qualidade de Vida, Segurança do Paciente e Sintomas de Quimioterapia e Radioterapia. • Quanto aos efeitos potencialmente positivos (em 21 associações), a maioria foi associado ao Viscum album (18 associações) para os desfechos: Sobrevida ao câncer, Sintomas de Quimioterapia e Radioterapia e Qualidade de Vida. • Identificaram-se efeitos inconclusivos em 15 associações para 11 diferentes desfechos, o que sugere uma necessidade de novos estudos primários sobre intervenções com medicamentos e terapias antroposóficas. • Não foram identificados estudos com ausência de efeito ou efeitos negativos.

Self-Management Program in Adults with Colorectal Cancer: A Pilot Study / Programa de automanejo en adultos con cáncer colorrectal: estudio piloto / Programa de autogestão em adultos com câncer colorretal: estudo-piloto

Objective: To design and validate a nursing intervention and its effect on improving self-management behaviors in patients with colorectal cancer following surgery within eight weeks after discharge. Method: Pilot study using Sidane and Braden's intervention design proposal, which included determining the guiding theoretical model, characterization of the intervention, validation with eight experts through content validity, and a pilot test with ten patients during the second semester of 2020. Results: The intervention obtained, called the Program for Self-Management Training in Colorectal Cancer (PEACCR, by its acronym in Spanish), is based on the theory of individual and family self-management in the dimensions proposed by Ryan and Sawin. The validity with experts indicates that it meets the criteria of clarity, precision, comprehension, relevance, and pertinence. The pilot reports an increase in self-management behaviors and the dimensions of knowledge, coping, and health personnel-patient alliance in months 1 and 2, with statistical significance. Conclusions: The designed and validated intervention increases the self-management behaviors of patients with colorectal cancer. The proposed scheme is highly acceptable to participants.

Objetivo: diseñar y validar una intervención de enfermería y su efecto para mejorar los comportamientos de automanejo en pacientes con cáncer colorrectal, después de una intervención quirúrgica dentro de las ocho semanas posteriores al alta. Método: estudio piloto que acoge la propuesta de diseño de intervenciones de Sidane y Braden, lo que incluyó determinar el modelo teórico orientador, la caracterización de la intervención, la validación con ocho expertos a través de la validez de contenido, y una prueba piloto con diez pacientes, durante el segundo semestre del año 2020. Resultados: la intervención obtenida, denominada Programa para el Entrenamiento en Automanejo en Cáncer Colorrectal (PEACCR), se basa en la teoría de automanejo individual y familiar en las dimensiones planteadas por Ryan y Sawin. La validez con expertos indica que cumple con los criterios de claridad, precisión, comprensión, relevancia y pertinencia. El piloto reporta un aumento en los comportamientos de automanejo y en las dimensiones de conocimiento, afrontamiento y alianza personal de salud-paciente en el mes 1 y en el mes 2, con significancia estadística. Conclusiones: la intervención diseñada y validada aumenta los comportamientos de automanejo de pacientes con cáncer colorrectal. El esquema propuesto es de alta aceptabilidad para los participantes.

Objetivo: desenhar e validar uma intervenção de enfermagem e seu efeito para melhorar os comportamentos de autogestão em pacientes com câncer colorretal, depois de uma intervenção cirúrgica dentro das oito semanas posteriores à alta. Materiais e método: estudo-piloto que utiliza a proposta de desenho de intervenções de Sidane e Braden, o que inclui determinar o modelo teórico orientador, a caracterização da intervenção, a validação com oitos especialistas por meio da validade de conteúdo e um teste-piloto com dez pacientes, durante o segundo semestre de 2020. Resultados: a intervenção obtida, denominada "Programa para o Treinamento em Autogestão em Câncer Colorretal", está baseada na teoria de autogestão individual e familiar nas dimensões propostas por Ryan e Sawin. A validade com especialistas indica que cumpre com os critérios de clareza, precisão, compreensão, relevância e pertinência. O piloto relata um aumento nos comportamentos de autogestão e nas dimensões de conhecimento, enfrentamento e parceria pessoal de saúde-paciente no mês 1 e no mês 2, com significância estatística. Conclusões: a intervenção desenhada e validada aumenta os comportamentos de autogestão de pacientes com câncer colorretal. O esquema proposto é de alta aceitabilidade para os participantes.

Colonoscopic laxative instillation for the fecal-loaded colon: a case series

Context: Postoperative, critically ill, and elderly patients often have fecal loading or impaction. In a few such patients, disimpaction of fecalomas and colon cleansing are difficult. Bowel obstruction, megacolon, lower gastrointestinal bleeding, and gut perforation are complications that may ensue. Oral laxatives or enemas may only be partially effective. Surgical intervention may be needed for salvage or to treat complications. Series and Design: Fourteen hospitalized cases with defecation disorder due to fecal loading of the colon were enrolled for retrospective analysis. Colonoscopic instillation of mannitol and/or lactulose was undertaken as an intervention when the use of oral laxatives was either ineffective or unfeasible, and enema had yielded poor results. Results: Ten patients had satisfactory outcomes for fecal clearance, whereas four patients with poor or incomplete responses underwent repeat interventions or surgery. No significant complications were encountered due to this therapy. Conclusion: Colonoscopic instillation of mannitol or lactulose in fecal-loaded critically ill patients results in a safe and satisfactory fecal clearance. (AU)

Short-term outcomes of laparoscopic-assisted colectomy versus open colectomy in patients with colonic carcinoma: a prospective randomized study

Introduction: The second most common cause of cancer-related mortality is colorectal cancer, and laparoscopic-assisted colectomy (LAC) has gained popularity among surgeons as an alternative to the conventional approach, which is open colecrtomy (OC). The differences between LAC and OC in terms of short-term outcomes have not been well documented, and the aim of the present work is to compare the short-term outcomes of both procedures. Materials and Methods: The present prospective study comprised 164 participants submitted to LAC (n = 82) and OC (n = 82) at the Helwan and Zagazig University hospitals between January 2018 and January 2022. We collected and analyzed demographic data, surgical data, and the short-term outcomes. Results: The LAC group had a significantly lower estimated amount of blood loss, shorter hospital stay, lower rates of incisional surgical site infection, and fewer cases of burst abdomen postoperatively, but with a considerably longer operative time (30.3 minutes) than the OC group. Conclusions: Our findings show that LAC is favorable option to OC, with superior outcomes. (AU)

Comparação entre os resultados do implante de um segmento de arco longo versus dois segmentos de arco tradicional no tratamento de pacientes com ceratocone / Comparison between the results of implanting a long-arch segment versus two standard arch segments for treatment of keratoconus patients

RESUMO Objetivo: Analisar retrospectivamente as alterações na ceratometria e no astigmatismo corneano obtidas após cirurgia de implante de anel intraestromal, comparando o uso de um segmento de arco longo versus o implante de dois segmentos de comprimento de arco tradicional. Métodos: A partir de um estudo transversal, obtivemos os dados de 94 olhos de pacientes diagnosticados com ceratocone, que foram submetidos ao implante de anel corneano. Eles foram divididos em dois grupos, dependendo do tipo de implante recebido: Grupo A, um segmento de arco longo; Grupo B, dois segmentos tradicionais. Todos os segmentos implantados possuíam 250µ de espessura. Os dados do pré-operatório dos dois grupos foram comparados, para garantir que as amostras eram similares (as diferenças encontradas entre os dois grupos não eram estatisticamente significativas). As variáveis analisadas no pré e no pós-operatório foram acuidade visual com correção, ceratometria, astigmatismo corneano e refração. Resultados: A amostra que recebeu apenas um segmento de arco longo (Grupo A) obteve redução da ceratometria média de 4,42D (8,7%) e do astigmatismo corneano de 2,43D (40,4%). Já na amostra dos olhos que receberam dois segmentos de arco tradicional (Grupo B), houve redução média de 2,66D (5,1%) em relação à ceratometria média e redução média de 2,11D (34,8%) em relação ao astigmatismo corneano. A redução obtida na ceratometria média no Grupo A foi maior que a obtida no Grupo B (diferença estatisticamente significativa). A redução obtida no astigmatismo do Grupo A não foi estatisticamente significante, se comparada com o resultado obtido no Grupo B (considerando p≤0,05). Conclusão: Foi demonstrado que o uso de um segmento de arco longo possui maior capacidade de aplanação corneana, se comparado com o uso de dois segmentos com comprimento de arco tradicional. Em relação à redução do astigmatismo, os dois grupos mostraram resultados equivalentes.

ABSTRACT Purpose: To retrospectively analyze the changes in corneal keratometry and astigmatism after intrastromal ring surgery, comparing the use of one long arch segment versus two traditional arc length segments. Methods: A cross-sectional study obtained data from 94 eyes of patients diagnosed with keratoconus that underwent surgical treatment with corneal ring implant. They were divided into two groups according to the type of implant received: one long-arch segment (Group A) or two traditional segments (Group B), both 250 microns thick. Preoperative data from the two groups were compared to ensure that the samples were similar (the differences between the two groups were not statistically significant). The variables (pre and post-operatively) analyzed were: best corrected visual acuity, keratometry, corneal astigmatism and refraction. Results: Group A, which received one long arch segment, showed a Km decrease of 4.42D (8.7%) and a corneal astigmatism reduction of 2.43D (40.4%). Group B, where the eyes acquired two traditional arch segments, showed an average Km decrease of 2.66D (5.1%) and corneal astigmatism reduction of 2.11D (34.8%). The mean keratometry (Km) reduction obtained was statistically significant (p≤0.05) when comparing both groups (A and B). The mean corneal astigmatism reduction was not statistically significant (p≤0.05) when comparing both groups (A and B). Conclusions: One long-arch segment has been demonstrated to have a greater capacity to reduce corneal curvature when compared to the use of two traditional-sized arch segments. No significant differences were found regarding the reduction of corneal astigmatism after comparing the results obtained in both groups.

Primer caso de escisión mesorrectal total transanal (TaTME) para el tratamiento del cáncer de recto medio en un centro docente universitario: descripción de la técnica / First case of transanal total mesorectal excision (TaTME) for middle rectal cancer in a teaching tertiary center: technical aspects / Primeiro caso de excisão total do mesorreto transanal (TaTME) para tratamento de câncer de reto meio em um centro universitário de ensino: aspectos tecnicos

La escisión mesorrectal transanal (TaTME: transanal total mesorectal escision) es la última de una larga lista de desarrollos técnicos y tecnológicos para el tratamiento del cáncer de recto medio y bajo. Incluso para los cirujanos colorrectales experimentados, lograr una escisión mesorrectal total (emt) de calidad en cirugía oncológica no siempre es sencillo, por la dificultad de obtener un adecuado acceso a la pelvis. Los estudios realizados han mostrado resultados comparables al abordaje laparoscópico, con tasas elevadas de escisiones mesorrectales completas y bajo porcentaje de margen circunferencial radial (CRM: circumferential radial margin) y distal positivos, con un adecuado número de ganglios resecados. Como toda técnica nueva, su implementación puede traer consecuencias no intencionales. La complejidad del abordaje, la dificultad en la identificación de nuevos repères y planos anatómicos, ha llevado a complicaciones graves como la lesión uretral o la siembra tumoral pelviana. Por ello, la comunidad quirúrgica ha retrasado la implementación masiva de la técnica y desarrollado estrategias de enseñanza y monitorización de este procedimiento para su realización en centros de alto volumen. El objetivo de esta publicación es presentar el primer caso de TaTME en un centro docente universitario y difundir en nuestra comunidad científica el fundamento de la técnica, sus indicaciones, describir los principales pasos técnicos, complicaciones, resultados oncológicos y funcionales.

Transanal total mesorectal excision (TaTME) is the last of a long list of technical and technological developments for treatment of middle and low rectal cancer. Even for skilled colorectal surgeons, achieving a good quality total mesorectal excision (TME) in oncology surgery is not always simple, due to the difficulty of obtaining optimal access to the pelvis. So far, studies have shown similar results to laparoscopic surgery, with high rates of complete mesorectal excisions and low rate of circumferential radial margin (CRM) and distal margin with an appropriate number of resected lymph nodes. Like every new technique, its implementation can bring unwanted consequences. The complexity of the approach, the difficulty in the identification of new landmarks and anatomic planes, has led to serious complications such as urethral injury or tumoral seeding. This has made slowdown the massive implementation of the technique among the surgical community, addressing the need of developing training programs and mentoring of this procedure that belongs to high volume centers. The aim of this publication is to present the first case of TaTME in a teaching tertiary center and spread, in our scientific community, the principles of the technique, its indications, main technical steps, complications and functional and oncologic results.

A excisão mesorretal transanal (TaTME: transanal total mesorectal escision) é o mais recente de uma longa linha de desenvolvimentos técnicos e tecnológicos para o tratamento do câncer retal inferior e médio. Mesmo para cirurgiões colorretais experientes, nem sempre é fácil obter uma excisão total do mesorreto (EMT) de qualidade em cirurgia de câncer, devido à dificuldade de obter acesso adequado à pelve. Os estudos realizados mostraram resultados comparáveis ​​à abordagem laparoscópica, com altas taxas de excisões completas do mesorreto e baixo percentual de margem radial circunferencial positiva (CRM: circumferential radial margin) e distal, com número adequado de linfonodos ressecados. Como qualquer nova técnica, sua implementação pode ter consequências não intencionais. A complexidade da abordagem, a dificuldade em identificar novos repères e planos anatômicos, levou a complicações graves, como lesão uretral ou semeadura de tumor pélvico. Por esse motivo, a comunidade cirúrgica atrasou a implementação massiva da técnica e desenvolveu estratégias de ensino e acompanhamento desse procedimento para sua realização em centros de alto volume. O objetivo desta publicação é apresentar o primeiro caso de TaTME em um centro de ensino universitário e divulgar em nossa comunidade científica as bases da técnica, suas indicações, descrever as principais etapas técnicas, complicações, resultados oncológicos e funcionais.

Reporte de caso de una niña de 9 años de edad con una fractura acetabular fisaria desplazada tratada quirúrgicamente: 5 años de seguimiento / Case report of an acetabular displaced physeal fracture in a 9-year-old girl treated surgically: follow-up of 5 years / Relato de caso de uma menina de 9 anos com fractura de acetábulo desviada tratada cirurgicamente: seguimento de 5 anos

Las fracturas acetabulares con compromiso del cartílago trirradiado (CTR) en pacientes pediátricos son muy poco frecuentes, difíciles de diagnosticar y complejas de tratar; pudiendo dejar graves secuelas. Generalmente son producto de accidentes de tránsito de alta energía cinética. Debido a su baja prevalencia no hay grandes series en la bibliografía y por ende tampoco existe un consenso terapéutico. Realizamos una puesta a punto del tema a propósito de un atípico caso de una niña de 9 años con una fractura del acetábulo con compromiso del CTR, producido por un traumatismo de baja energía cinética. Registramos su diagnóstico y tratamiento quirúrgico, evaluamos su resultado clínico - radiológico y funcional mediante el Hip Harris Score (HHS) al final de su seguimiento de 5 años.

Acetabular fractures with compromise of the triradiate cartilage (TRC) in pediatric patients are very rare, difficult to diagnose and complex to treat, also can leave serious consequences. They are generally product of high energy kinematics. Due to its low prevalence, there are no large series in the literature and therefore there is no therapeutic consensus. We carried out a recapitulation of the subject regarding an atypical case of a 9-year-old girl with an acetabulum fracture with compromise of the TRC, produced by a low kinetic energy trauma. We recorded the diagnosis and surgical treatment, and also, we evaluated the clinical-radiological and functional results through the Hip Harris Score (HHS) at the end of their 5-year follow-up.

As fraturas acetabulares com compromisso da cartilagem trirradiada (TRC) em pacientes pediátricos são muito poco frecuentes, de difícil diagnóstico e complexas de tratar; e podem deixar sérias consequências. Geralmente são o produto de acidentes de trânsito de alta energia cinética. Devido à sua baixa prevalência, não há grandes séries na literatura e, portanto, não há consenso terapêutico. Realizamos uma atualização do tema referente a um caso atípico de uma menina de 9 anos com fratura de acetábulo com comprometimento do CTR, produzida por um trauma de baixa energia cinética. Registramos seu diagnóstico e tratamento cirúrgico, avaliamos seus resultados clínico-radiológicos e funcionais por meio do Hip Harris Score (HHS) ao final de seu seguimento de 5 anos.

Compromiso cardíaco en el síndrome inflamatorio multisistémico post SARS-CoV-2 en niños / Cardiac involvement in post-SARS-CoV-2 multisystem inflammatory syndrome in children / Envolvimento cardíaco na síndrome inflamatória multissistêmica pós-SARS-CoV-2 em crianças

Objetivo: describir las características de ocho pacientes pediátricos que se presentaron con síndrome inflamatorio multisistémico (MIS-C) asociado a SARS-CoV-2 y compromiso cardíaco. Material y métodos: estudio descriptivo, retrospectivo de ocho pacientes con edades entre 1 y 13 años, con diagnóstico de MIS-C y compromiso cardíaco, asistidos en el CHPR. Se analiza su historia clínica, evolución y tratamiento. Resultados: los pacientes presentaron fiebre en el 100%, exantema e hiperemia conjuntival en el 88%, síntomas digestivos en el 50%, insuficiencia respiratoria en el 25% y shock en el 50%. Todos requirieron ingreso a cuidados intensivos. La alteración de la contractilidad cardíaca estuvo presente en el 63% de los pacientes, fue leve y segmentaria en el 80%, el 60% requirió soporte inotrópico por 3 días, recuperando una función normal en 7 días. La insuficiencia mitral se presentó en el 25% y el derrame pericárdico en el 38%, ambos de grado leve. Un paciente presentó dilatación de arterias coronarias con Z score < 2. El 85% de los pacientes presentó alteraciones del ECG, en el 29% se trató de alteración en la repolarización, en el 29% intervalo QTc prolongado, en el 15% bloqueo atrioventricular de 1er grado y bloqueo incompleto de rama derecha. Un paciente tuvo fibrilación auricular por 3 días con remisión espontánea a ritmo sinusal. Las troponinas estuvieron altas en el 57% de los pacientes y el ProBNP elevado en el 100%. Todos recibieron inmunoglobulinas, metilprednisolona y aspirina. Conclusiones: se presentaron ocho pacientes pediátricos con MIS-C y compromiso cardíaco, el 50% se presentó en shock, todos requirieron ingreso a cuidados intensivos. El 85% presento alteraciones en el ECG. El 63% presentó compromiso de la contractilidad sectorial y leve, se normalizó en 7 días. El 60% requirió soporte inotrópico por una media de 3 días.

Objective: describe the characteristics of 8 children who presented Multisystem Inflammatory Syndrome associated with SARS-CoV2 infections (MIS-C) and cardiac involvement. Material and methods: descriptive, retrospective study of 8 patients of between 1 and 13 years of age, diagnosed with MIS-C and cardiac involvement, assisted at the Pereira Rossell Children Hospital, analysis of their medical records, evolution and treatment. Results: the patients showed: fever in 100% of the cases, rash and conjunctival hyperemia in 88%, digestive symptoms in 50%, respiratory failure in 25% and shock in 50%. All required admission to Intensive Care. Cardiac contractility alteration was present in 63% of patients, the affectation was mild and segmental in 80%, 60% required inotropic support for 3 days and recovered normal functions in 7 days. Mitral regurgitation was present in 25% of the cases and pericardial effusion in 38%, mild in both cases. One patient had dilated coronary arteries with a Z score <2. 85% of the patients presented ECG abnormalities, 29% present alteration of repolarization, 29% prolonged QTc, 15% 1st degree atrioventricular block and incomplete right bundle branch block. One patient had atrial fibrillation for 3 days with spontaneous remission to sinus rhythm. Troponins were increased in 57% of the patients and ProBNP elevated in 100%. All patients received Immunoglobulins, Methylprednisolone and Aspirin. Conclusions: we present eight pediatric patients with MIS-C and cardiac involvement, 50% suffered shock, all required admission to Intensive Care. ECG abnormalities were found in 85% of the patients. Mild and segmental contractility compromise was found in 63% of the patients and normalized in 7 days. 60% required inotropic support for a mean of 3 days.

Objetivo: descrever as características de 8 pacientes pediátricos que apresentaram Síndrome Inflamatória Multissistêmica (MIS-C) associada ao SARS-CoV-2 e comprometimento cardíaco. Material e métodos: estudo descritivo, retrospectivo, de oito pacientes com idade entre 1 e 13 anos, com diagnóstico de MIS-C e comprometimento cardíaco, assistidos pelo CHPR. Seu prontuário médico, evolução e tratamento são analisados. Resultados: os pacientes apresentaram febre em 100%, erupção cutânea e hiperemia conjuntival em 88%, sintomas digestivos em 50%, insuficiência respiratória em 25% e choque em 50%. Todos necessitaram de internação nos cuidados intensivos. A alteração da contratilidade cardíaca esteve presente em 63% dos pacientes, foi leve e segmentar em 80%, 60% necessitaram de suporte inotrópico por 3 dias, recuperando a função normal em 7 dias. A regurgitação mitral ocorreu em 25% dos pacientes e o derrame pericárdico em 38%, ambos de grau leve. Um paciente apresentou dilatação da artéria coronária com escore Z < 2. 85% dos pacientes apresentaram anormalidades no ECG, 29% foram alterações de repolarização, 29% intervalo QTc prolongado em bloqueio atrioventricular de 1º grau a 15% e bloqueio incompleto do ramo direito. Um paciente apresentou fibrilação atrial por 3 dias com remissão espontânea ao ritmo sinusal. As troponinas foram elevadas em 57% dos doentes e ProBNP elevado em 100%. Todos receberam imunoglobulinas, Metilprednisolona e aspirina. Conclusões: houve oito pacientes pediátricos com SMIM-C e comprometimento cardíaco, 50% em choque, todos necessitaram de internação em terapia intensiva. 85% apresentaram elevações no ECG. 63% apresentaram comprometimento setorial e de contratilidade leve, normalizados em 7 dias. 60% necessitaram de suporte inotrópico por uma média de 3 dias.

Resultados de la neurólisis como tratamiento del dolor neuropático en pacientes con lesión del nervio ciático. Reporte de casos / Results of neurolysis for the treatment of neuropathic pain in patients with sciatic nerve Injury. Case report

Introducción: La lesión del nervio ciático puede ocasionar desde parestesias e hipoestesias, hasta dolor neuropático severo y parálisis. La neurólisis suele mejorar la función, el dolor y la calidad de vida de los pacientes. El objetivo de este estudio fue evaluar los resultados clínico-funcionales de una serie de pacientes con dolor neuropático por lesión del nervio ciático que no respondieron al tratamiento conservador y fueron sometidos a neurólisis. Materiales y métodos: Se analizó retrospectivamente una serie de pacientes sometidos a neurólisis del nervio ciático mayor entre marzo de 2009 y junio de 2018. El dolor pre- y posoperatorio se evaluó mediante la escala analógica visual y la escala de Likert. El tipo de dolor posoperatorio se evaluó con el cuestionario DN4 y la calidad de vida relacionada con la salud, con el cuestionario SF-36.Resultados:Se incluyó a 8 pacientes. A los 32 meses de seguimiento promedio (rango 14-66), el dolor había mejorado notablemente (promedio de 3 y 1,88 puntos en las escalas analógica visual y de Likert, respectivamente). El cuestionario DN4 arrojó un promedio de 3,75 puntos (rango 2-7). Según el SF-36, la "salud física" fue la variable con peores resultados (promedio 30,15).Conclusiones: La neurólisis, cuando se indica a pacientes con mala respuesta al tratamiento conservador, es un método que alivia el dolor y mejora la calidad de vida de los pacientes con dolor neuropático secundario a una lesión del nervio ciático. Nivel de Evidencia: IV

Introduction: Sciatic nerve injury may cause paresthesias and hypoesthesias, severe neuropathic pain, and paralysis. Neurolysis can improve function, pain, and quality of life for these patients. The objective of this paper is to evaluate the clinical-functional outcomes of a series of patients with neuropathic pain due to sciatic nerve injury that was refractory to conservative treatment in whom neurolysis was performed. materials and methods:A retrospective case series of patients operated on for neurolysis of the greater sciatic nerve between March 2009 and June 2018 was analyzed. Preoperative and postoperative pain were evaluated using the visual analog scale (VAS) and the Likert scale. The type of postoperative pain was evaluated using the DN4 questionnaire, and the health-related quality of life was measured with the SF-36 questionnaire. Results: Eight patients were included. All patients evolved with a notable improvement in pain, with an average of 3 and 1.88 points on the VAS and Likert scales, respectively. The mean follow-up was 32 months (range 14­66). The DN4 questionnaire showed an average of 3.75 points (range 2­7). According to the SF-36, "Physical Health" was the variable with the worst results, with an average of 30.15. Conclusions: Sciatic nerve neurolysis in patients with neuropathic pain due to sciatic nerve injury and poor response to conservative treatment may improve pain and quality of life. Level of Evidence: IV

Reemplazo unicompartimental bilateral de rodilla en un tiempo quirúrgico. Resultados a mediano plazo de 86 prótesis con un seguimiento promedio de 6.2 años / Bilateral simultaneous unicompartmental knee arthroplasty. Medium-term outcomes in 86 arthroplasties with an average follow-up of 6.2 years

Objetivo: Evaluar los resultados funcionales, las complicaciones y la supervivencia a mediano plazo de la prótesis unicompartimental bilateral medial o lateral de rodilla en un tiempo quirúrgico. Materiales y Métodos: Estudio retrospectivo de pacientes con prótesis unicompartimental bilateral medial o lateral de rodilla colocada en un tiempo quirúrgico por gonartrosis entre abril de 2004 y abril de 2020, seguimiento mínimo 1 año. Se evaluaron los resultados clínico-funcionales con el KSS 2011. Se determinaron los tiempos quirúrgico total y de internación, y el requerimiento de transfusiones. Se analizaron las complicaciones a corto y mediano plazo, y las tasas de revisión y de supervivencia de la prótesis. Resultados: Se evaluaron 86 prótesis unicompartimentales en 43 pacientes (seguimiento promedio 6.1 años). El KSS clínico y funcional aumentó de 46,1 ± 10,2 a 80,9 ± 15,9 y de 22,8 ± 11,9 a 89,8 ± 18,9, respectivamente. La flexión máxima mejoró de 106,3° ± 5,2° a 125,1° ± 4,2° y la contractura en flexión, de 7,5° ± 2,2° a 2,3° ± 1,6°. La cirugía duró 178.6 min y la internación, 39.8 h. Dos pacientes requirieron transfusión. La tasa de complicaciones fue del 6,9%, todas menores. Tres rodillas tuvieron un aflojamiento mecánico aséptico y requirieron revisión a prótesis total de rodilla o nueva prótesis unicompartimental tras 12, 8.6 y 7 años. La supervivencia de la prótesis fue del 96,5%. Conclusión: La prótesis unicompartimental bilateral medial o lateral en un tiempo quirúrgico para la gonartrosis unicompartimental de rodilla proporciona excelentes resultados clínico-funcionales, con bajas tasas de complicaciones. Nivel de Evidencia: IV

Objective: The purpose of this article is to examine the functional outcomes, complications, and medium-term survivorship of medial or lateral bilateral simultaneous unicompartmental knee arthroplasty (bUKA) for the treatment of bilateral knee osteoarthritis. Materials and Methods: Retrospective report of patients who underwent a medial or lateral bUKA for treatment of bilateral knee osteoarthritis between April 2004 and April 2020, with a minimum follow-up of 1 year. The KSS 2011 was used for the clinical-functional evaluation of each patient. The duration of surgery, length of hospital stay, and transfusion requirements were determined. The short-term and medium-term complications were analyzed, as well as the revision rate and the prosthesis survivorship. Results: We evaluated 86 bUKAs in 43 patients with a mean follow-up of 6.1 years. The clinical and functional KSS improved from 46.1 ± 10.2 to 80.9 ± 15.9 and 22.8 ± 11.9 to 89.8 ± 18.9 respectively. Postoperative maximal flexion improved from 106.3º ± 5.2º to 125.1º ± 4.2º and flexion contracture improved from 7.5º ± 2.2º to 2.3º ± 1.6º. The mean surgical time was 178.6 minutes and the hospital stay was 39.8 hours. Two patients required transfusions. The complication rate was 6.9%. Three knees (3.5%) required revision surgery for aseptic loosening after 7, 8.6 and 12 years. The survivorship rate was 96.5%. Conclusion: Simultaneous medial or lateral bUKA provides excellent clinical-functional outcomes with a low rate of complications in patients with bilateral knee osteoarthritis. Level of Evidence: IV

Seudoartrosis después de una osteotomía en chevron: incidencia, tratamiento, seguimiento y evolución / Nonunion after Chevron osteotomy. Incidence, treatment, follow-up, and outcomes

Introducción: El hallux valgus es el trastorno más común del primer dedo del pie. Provoca dolor, discapacidad funcional y altera los patrones de la marcha. Las deformidades leves o moderadas se han corregido con osteotomías distales del primer metatarsiano, como la osteotomía en chevron, un procedimiento seguro, pero no exento de complicaciones. Los objetivos de este estudio fueron determinar la incidencia de seudoartrosis por dicha osteotomía y comunicar nuestro método terapéutico, el seguimiento y la evolución. materiales y métodos: Se realizó un estudio multicéntrico, retrospectivo que incluyó a pacientes operados entre 2009 y 2018. Se evaluaron 1156 osteotomías en chevron como tratamiento del hallux valgus leve o moderado en 1017 pacientes (rango etario 16-83 años; promedio 57.5) realizadas por 4 cirujanos experimentados. El criterio de inclusión fue que el paciente contara con estudios por imágenes compatibles con seudoartrosis a los 6 meses de la cirugía. Resultados: Se evaluó a 5 pacientes con diagnóstico de seudoartrosis después de una osteotomía en chevron para tratar el hallux valgus. Los puntajes promedio de la AOFAS fueron 51 antes del tratamiento del hallux valgus y 87,8 después del tratamiento de la seudoartrosis. Conclusiones: La incidencia de seudoartrosis fue del 0,4% en el posoperatorio alejado. Nuestro abordaje y el tratamiento de la seudoartrosis lograron una excelente mejoría clínica y funcional en todos los pacientes operados. Nivel de Evidencia: III

Introduction: Hallux valgus is the most common disorder of the first toe. It causes pain, functional impairment, and alters gait patterns. Mild to moderate deformities are tipically corrected with distal osteotomies of the first metatarsal, such as the chevron osteotomy, a safe procedure, but not without complications. The objectives of this study were to determine the incidence of pseudarthrosis following this osteotomy and report our therapeutic method, follow-up, and outcomes. Materials and methods: A retrospective multicenter study was carried out, which included patients operated on between 2009 and 2018. A total of 1156 chevron osteotomies were evaluated as a treatment for mild to moderate hallux valgus in 1017 patients (age range 16 -83 years; average 57.5 years) performed by 4 experienced surgeons. The inclusion criterion was that the patient had imaging studies compatible with pseudarthrosis six months after surgery. Results: We evaluated five patients who met our criterion. The average AOFAS (American Orthopedic Foot and Ankle Society) scores were 51 before hallux valgus treatment and 87.8 after pseudarthrosis treatment. Conclusion: The incidence of pseudarthrosis was 0.4% in the distant postoperative period. Our approach and treatment of pseudarthrosis achieved excellent clinical and functional improvements in all operated patients. Level of Evidence: III

Mucopolisacaridosis tipo VI: a propósito de un caso / Mucopolysaccharidosis type VI. Case report

La mucopolisacaridosis tipo VI o síndrome de Maroteaux-Lamy se produce por la deficiencia de la enzima arilsulfatasa B que ocasiona la acumulación intracelular de dermatán sulfato. El riesgo de compresión medular es particularmente elevado y muy frecuente en la unión occipito-cervical. La terapia de reemplazo enzimático ha sido esencial para los pacientes con esta enfermedad; sin embargo, no tiene efecto sobre las alteraciones esqueléticas, y su impacto sobre la estabilidad espinal está aún en estudio. Se sugiere un examen anual (evaluación neurológica, radiografías, resonancia magnética y potenciales provocados somatosensitivos) y, en caso de anomalías, cada 6 meses. Pese al alto riesgo anestésico, la mielopatía y los síntomas progresivos indican la necesidad de una descompresión quirúrgica. Presentamos a una niña de 12 años con mucopolisacaridosis tipo VI tratada con terapia de reemplazo enzimático desde los 7 años, que acude a la consulta con síntomas compatibles con mielopatía cervical alta progresiva. Fue sometida a una descompresión y artrodesis occipito-cervical con ampliación del foramen magno. Esta enfermedad es infrecuente; por lo tanto, es imperativo el seguimiento multidisciplinario del paciente, así como conocer el riesgo de compresión medular y su oportuno tratamiento quirúrgico a cargo de cirujanos espinales. Nivel de Evidencia: IV

Mucopolysaccharidosis type VI, also known as Maroteaux-Lamy syndrome, is caused by a deficiency of the arylsulfatase B enzyme, which causes intracellular accumulation of dermatan sulfate. The risk of spinal cord compression is particularly high and frequent at the occipitocervical junction. Enzyme replacement therapy has been essential for patients with this disease; however, it has no effect on skeletal abnormalities, and its impact on spinal stability is still under study. An annual examination (neurological evaluation, radiography, magnetic resonance imaging, and somatosensory evoked potentials) is recommended. In case of anomalies, it should be repeated every 6 months. Despite the high anesthetic risk, myelopathy and progressive symptoms indicate the need for surgical decompression. We present the case of a 12-year-old girl with mucopolysaccharidosis type VI treated with enzyme replacement therapy since the age of 7, who came to the consultation with symptoms compatible with progressive high cervical myelopathy. She underwent occipitocervical decompression and fusion with enlargement of the foramen magnum. This disease is rare; therefore, multidisciplinary patient follow-up is imperative, as well as knowing the risk of spinal cord compression and its timely surgical treatment by spinal surgeons. Level of Evidence: IV