Síndrome de Herlyn-Werner Wünderlich: reporte de dos casos / Herlyn-Werner-Wünderlich syndrome: Two case report

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

Congenital malformations of the posterior fossa: a review of clinical and radiological features through an Illustrated guide / Malformações congênitas da fossa posterior: uma revisão das características clínicas e radiológicas por meio de um guia ilustrado

Congenital malformations of the posterior fossa (PF) encompass a wide spectrum of morphological anomalies that arise during embryonic development. This paper provides a narrative review of these most common disorders from a morphological approach based on the division into cystic (Dandy-Walker malformation, Blake's Pouch cyst, mega cisterna magna, arachnoid cyst, and cerebellar vermis hypoplasia) and non-cystic malformations (Joubert syndrome, rhomboencephalosynapsis, and pontocerebellar hypoplasia). The embryogenesis of PF structures is briefly outlined. While magnetic resonance imaging is the preferred modality for evaluation, computerized tomography and ultrasonography serve complementary roles. In conjunction with clinical aspects, specific radiological features, such as cerebellar vermis morphology, Torcular Herophili position, and the presence/absence of mass effect, are highlighted for accurate diagnosis. We also present some typical radiological findings in non-cystic malformations, such as molar tooth sign, diamondshaped fourth ventricle, and dragonfly-shaped cerebellum. This comprehensive review aims to assist radiologists, neuropediatricians, and general neurologists in recognizing and describing PF malformations, thereby facilitating appropriate management strategies.

As malformações congênitas da fossa posterior (FP) abrangem um amplo espectro de anomalias morfológicas que surgem durante o desenvolvimento embrionário. Este artigo fornece uma revisão narrativa desses distúrbios mais comuns a partir de uma abordagem morfológica baseada na divisão em malformações císticas (malformação de Dandy-Walker, cisto da bolsa de Blake, mega cisterna magna, cisto aracnoide e hipoplasia do vermis cerebelar) e não císticas (syndrome de Joubert, rombencefalossinapse e hipoplasia pontocerebelar). A embriogênese das estruturas da FP é brevemente descrita. Embora a ressonância magnética seja a modalidade preferida para avaliação, a tomografia computadorizada e a ultrassonografia desempenham funções complementares. Em conjunto com os aspectos clínicos, características radiológicas específicas, como a morfologia do vermis cerebelar, a posição da torcula Herophili e a presença/ausência de efeito de massa, são destacadas para um diagnóstico preciso. Apresentamos também alguns achados radiológicos típicos de malformações não císticas, como sinal do dente molar, quarto ventrículo em forma de diamante e cerebelo em forma de libélula. Esta revisão abrangente visa auxiliar radiologistas, neuropediatras e neurologistas gerais no reconhecimento e na descrição das malformações da FP, facilitando, assim, estratégias de manejo adequadas.

Compressive Structures of the Median Nerve at the Elbow / Estructuras Compresivas del Nervio Mediano en el Codo

SUMMARY: The objective of this study was to analyze the potential for compression of the median nerve (MN) caused by the bicipital aponeurosis (BA), the humeral and ulnar heads of the pronator teres muscle (PTM) and the arcade of the flexor digitorum superficialis muscle (FDS) in recently deceased cadavers. In this analysis 20 forearms of 10 recently deceased adult male cadavers were dissected. Dissections were performed in the institution's autopsy room or anatomy laboratory. The short and long heads of the biceps brachii muscle, as well as the BA were identified in all upper upper limbs. The BA received contribution from the short and long heads of the biceps brachii muscle. In 12 upper limbs the BA was wide and thickened and in 8 it was supported by the MN. In 5 upper limbs, the BA was wide but not very thick, and in 3 it was narrow and not very thick. We identified the existence of the FDS muscle arcade in all dissected upper limbs. A fibrous arcade was identified in 4 forearms, a muscular arcade in 14 and a transparent arcade in 2 upper limbs. In all of them, we recorded that the arcade was in contact with the MN. We recorded the humeral and ulnar heads of the PTM in all dissected upper limbs, with the presence of fibrous beams between them along their entire length. The MN was positioned between the humeral and ulnar heads of the PTM in all upper limbs. In eight upper limbs (40 %), we identified that the BA had thickness and contact with the MN with the potential to cause its compression. Compression between the humeral and ulnar heads of the PTM by the fibrous connections has the potential to cause nerve compression in all upper limbs (100 %). We did not identify that the anatomical structure of the FDS arcade had the potential to cause compression in the MN.

El objetivo de este estudio fue analizar la potencial compresión del nervio mediano (NM) causado por la aponeurosis bicipital (AB), las cabezas humeral y cubital del músculo pronador redondo (MPR) y la arcada del músculo flexor superficial de los dedos (MFS). En este análisis se diseccionaron 20 antebrazos de 10 cadáveres masculinos de individuos adultos fallecidos recientemente. Las disecciones se realizaron en la sala de autopsias o en el laboratorio de anatomía de la Institución. En todos los miembros superiores se identificaron las cabezas corta y larga del músculo bíceps braquial, así como la AB. La AB recibió contribución de las cabezas corta y larga del músculo bíceps braquial. En 12 miembros superiores la AB era ancha y engrosada y en 8 estaba sostenida por el NM. En 5 miembros superiores la AB era ancha pero poco gruesa, y en 3 era estrecha y de menor grosor. Identificamos la existencia de la arcada muscular MFS en todos los miembros superiores disecados. Se identificó una arcada fibrosa en 4 antebrazos, una arcada muscular en 14 y una arcada delgada y transparente en 2 miembros superiores. En todos ellos registramos que la arcada estaba en contacto con el NM. Registramos las cabezas humeral y cubital del MPR en todos los miembros superiores disecados, con presencia de haces fibrosos entre ellas en toda su longitud. El NM estaba situado entre las cabezas humeral y cubital del MPR en todos los miembros superiores. En ocho miembros superiores (40 %), identificamos que la AB era gruesa y tenía contacto con el NM con potencial para causar su compresión. La compresión entre las cabezas humeral y ulnar del MPR, por las conexiones fibrosas, tiene el potencial de causar compresión nerviosa en todos los miembros superiores (100 %). No identificamos que la estructura anatómica de la arcada MFS tuviera el potencial de causar compresión del NM.

Expansión Rápida del Maxilar: Revisión actualizada de las técnicas disponibles / Rapid Maxillary Expansion: Updated Review of Available Techniques

La deficiencia transversal del maxilar es una anomalía dentomaxilar capaz de producir problemas funcionales en la oclusión, respiración y estéticos. Su presentación clínica tradicional es la mordida cruzada posterior y una alteración en la relación transversal intermaxilar, la cual impide la correcta erupción de las piezas dentarias. Su manejo corresponde a la expansión rápida del maxilar, la cual puede ser asistida mediante mini-implantes (MARPE), cirugía (SARPE) o una combinación de ambas técnicas (MISMARPE). El objetivo del presente artículo es presentar un contraste entre las técnicas empleadas en la expansión rápida del maxilar, para simplificar la toma de decisiones clínicas. Se elaboró una revisión narrativa en las bases de datos PubMed, Scopus y Epistemonikos contemplando revisiones sistemáticas, metaanálisis, ensayos clínicos aleatorizados y estudios observacionales publicados entre el año 2013 a 2023. Un total de 23 artículos fueron incluidos, los cuales cumplían con los criterios de inclusión y exclusión establecidos. El manejo clínico de la deficiencia transversal del maxilar frecuentemente requiere un abordaje interdisciplinario combinando un enfoque ortopédico y quirúrgico. Según lo encontrado en la actual revisión, tanto el MARPE, SARPE y MISMARPE reportan indicaciones y limitaciones, así como complicaciones asociadas, sin embargo, serían efectivas en la resolución de deficiencias transversales. Se recomienda al clínico considerar dicha información de acuerdo a las necesidades particulares de cada caso clínico, así como profundizar y prolongar el estudio de nuevas técnicas para analizar su estabilidad a largo plazo en comparación a las otras corrientes terapéuticas.

The transverse deficiency of the maxilla is a dentomaxillary anomaly capable of producing functional problems in occlusion, respiration and esthetics. Its traditional clinical presentation is dental crowding, which obstructs the correct eruption of the teeth. Its management corresponds to rapid maxillary expansion, which can be assisted by means of mini-implants (MARPE), surgery (SARPE) or a combination of both techniques (MISMARPE). The objective of this article is to present a contrast between the techniques used in rapid maxillary expansion to simplify clinical decision making. A narrative review was performed in PubMed, Scopus and Epistemonikos databases, including systematic reviews, meta-analyses, randomized clinical trials and observational studies published between 2013 and 2023. A total of 23 articles were included, which met the established inclusion and exclusion criteria. The clinical management of transverse deficiency of the maxilla frequently requires an interdisciplinary management, combining an orthopedic and surgical approach. As found in the current review, all techniques; MARPE, SARPE and MISMARPE, report indications and limitations, as well as associated complications. It is recommended to deepen and prolong the study of new techniques in order to analyze their long-term stability in comparison to other therapeutic currents.

Prevalencia de patología urogenital en la consulta ambulatoria de varones adolescentes. Estudio descriptivo transversal / Prevalence of urogenital disease in male adolescent outpatient visits. A descriptive, cross-sectional study

Introducción. El examen genital en varones es una evaluación clínica simple y rápida para detectar patología urogenital. Los datos sobre prevalencia de patología urogenital en varones adolescentes son limitados. Nuestro objetivo fue describir la prevalencia de patología urogenital en varones adolescentes. Población y métodos. Estudio descriptivo transversal en el Servicio de Adolescencia de un hospital público de la Ciudad de Buenos Aires. Se evaluaron retrospectivamente las historias clínicas (HC) de varones de 9 a 20 años atendidos entre 2008 y 2018; se incluyeron las que tenían examen genital. Se recabaron datos de edad, estadio puberal, orquidometría, patología urogenital antes de la adolescencia y al momento de la consulta. La prevalencia se expresó en porcentaje e intervalo de confianza del 95 % (IC95%). Se estimó necesario incluir 1167 HC como muestra poblacional. Resultados. Se evaluaron 2129 HC; se incluyeron 1429. En 686 casos no se hizo el examen genital. La población tuvo una mediana de edad de 12 años (rango intercuartílico 11-14 años). En 72 varones (5,7 %; IC95% 4,5-7,2), se halló una enfermedad genitourinaria antes de la adolescencia. Se detectó al menos una patología urogenital en 272 adolescentes (14,8 %; IC95% 13,1-16,7); las más frecuentes fueron adherencia balanoprepucial 5,3 % (IC95% 4,2-6,6), varicocele 2,7 % (IC95% 2-3,7) y fimosis 1,8 % (IC95% 1,2-2,6). Conclusiones. El examen genital permitió detectar que el 14,8 % de los varones adolescentes atendidos presentó alguna patología urogenital. Las entidades más frecuentes fueron adherencia balanoprepucial, varicocele y fimosis.

Introduction. The male genital exam is a simple and quick assessment to look for urogenital disease. Data on the prevalence of urogenital disease in male adolescents are limited. Our objective was to describe the prevalence of urogenital disease in male adolescents. Population and methods. Descriptive, cross-sectional study conducted at the Department of Adolescenceof a public hospital in the City of Buenos Aires. The medical records of male patients aged 9 to 20 years seen between 2008 and 2018 were retrospectively reviewed; all those with a genital exam were included. Data on age, pubertal stage, orchidometry, and urogenital disease before adolescence and at the time of consultation were recorded. The prevalence was described as percentage and 95% confidence interval (CI).As per estimations, 1167 medical records had to be included to establish the population sample. Results. A total of 2129 medical records were assessed and 1429 were included. No genital exam had been conducted in 686 cases. The median age of the population was 12 years (interquartile range: 11­14 years). Urogenital disease before adolescence was detected in 72 boys (5.7%; 95% CI: 4.5­7.2). Urogenital disease was found in 272 adolescents (14.8%; 95% CI: 13.1­16.7); the most common conditions were balanopreputial adhesions in 5.3% (95% CI: 4.2­6.6), varicocele in 2.7% (95% CI: 2­3.7), and phimosis in 1.8% (95% CI: 1.2­2.6). Conclusions. A genital exam allowed to detect that 14.8% of adolescent boys had a urogenital diseaseThe most common conditions were balanopreputial adhesions, varicocele, and phimosis.

Eventos adversos em crianças submetidas a cirurgias orofaciais / Adverse events in children submitted to orofacial surgery / Eventos adversos en niños sometidos a cirugía orofacial

Objetivo: Identificar os eventos adversos no pós-operatório imediato de queiloplastia e/ou palatoplastia em crianças e comparar os eventos identificados aos notificados ao Núcleo de Segurança do Paciente. Métodos: Estudo descritivo, retrospectivo e quantitativo, realizado em um hospital público e terciário brasileiro. Os dados foram coletados por meio da descrição nos registros de enfermagem e comparados aos notificados ao Núcleo de Segurança do Paciente, referente a junho e dezembro de 2019. Os resultados foram submetidos a análise estatística descritiva. Resultados: A amostra constou de 203 crianças, das quais 51% (n=103) apresentaram evento adverso. Foram identificados 176 eventos adversos, de 8 tipos, com prevalência da laringite pós-extubação (n=50; 28%), edema de língua (n=34; 19%) e lesão de comissura labial (n=25; 14%). Destes, apenas 5% (n=9) foram notificados ao Núcleo de Segurança do Paciente. Conclusão: Os eventos adversos prevalentes se relacionaram a cavidade oral e tecidos adjacentes, e a subnotificação foi expressiva. (AU)

Objective: To identify adverse events in the immediate postoperative period of cheiloplasty and/or palatoplasty in children and compare the identified events to those notified to the Patient Safety Center. Methods: Descriptive, retrospective and quantitative study, carried out in a Brazilian public and tertiary hospital. Data were collected through descriptions in nursing records and compared to those notified to the Patient Safety Center, referring to June and December 2019. The results were subjected to descriptive statistical analysis. Results: The sample consisted of 203 children, of which 51% (n=103) had an adverse event. A total of 176 adverse events of 8 types were identified, with prevalence of post-extubation laryngitis (n=50; 28%), tongue edema (n=34; 19%) and labral commissure lesion (n=25; 14%). Of these, only 5% (n=9) were notified to the Patient Safety Center. Conclusion: The prevalent adverse events were related to the oral cavity and adjacent tissues, and underreporting was significant. (AU)

Objetivo: Identificar eventos adversos en el postoperatorio inmediato de queiloplastia y/o palatoplastia en niños y comparar los eventos identificados con los notificados al Centro de Seguridad del Paciente. Métodos: Estudio descriptivo, retrospectivo y cuantitativo, realizado en un hospital público y terciario brasileño. Los datos se recolectaron mediante descripciones en registros de enfermería y se compararon con los notificados al Centro de Seguridad del Paciente, referidos a junio y diciembre de 2019. Los resultados fueron sometidos a análisis estadístico descriptivo. Resultados: La muestra estuvo conformada por 203 niños, de los cuales el 51% (n = 103) tuvo un evento adverso. Se identificaron un total de 176 eventos adversos de 8 tipos, con prevalencia de laringitis posextubación (n=50; 28%), edema de lengua (n=34; 19%) y lesión de la comisura del labrum (n=25; 14%). De estos, solo el 5% (n=9) fueron notificados al Centro de Seguridad del Paciente. Conclusion: Los eventos adversos prevalentes se relacionaron con la cavidad bucal y los tejidos adyacentes y el subregistro fue significativo. (AU)

Erupción fija por luz solar, una serie de 13 casos en Bogotá, Colombia / Fixed Sunlight Eruption: A Series of 13 Cases in Bogotá, Columbia

Desde 1975 se han publicado algunos casos que asocian la radiación ultravioleta como un desencadenante de erupciones cutáneas fijas (erupción o exantema fijo por luz solar o síndrome de fotosensibilidad localizada de amplio espectro). Describimos los casos de 13 pacientes con esta dermatosis, 4 varones (30,8%) y 9 mujeres (69,2%), con edades comprendidas entre los 28 y los 56 años, atendidos en un centro de referencia en dermatología en Bogotá, Colombia. Las lesiones se localizaron en la cara interna de los muslos, los glúteos, la región poplítea, la axilar anterior y posterior y el dorso de los pies. La prueba de fotoprovocación logró la reproducción de las lesiones en todos los casos en las áreas afectadas y la histopatología reveló cambios similares a los descritos en los eritemas fijos por medicamentos. Esta enfermedad podría corresponder a un subtipo de erupción fija, aunque no se descarta que sea una dermatosis diferente con una patogenia común.(AU)

Few reports describing an association between UV radiation and fixed skin eruptions have been published since 1975. These reactions have received various names, including fixed sunlight eruption, fixed exanthema due to UV radiation, and broad-spectrum abnormal localized photosensitivity syndrome. We present a series of 13 patients (4 men [30.8%] and 9 women [69.2%]) aged between 28 and 56 years who were evaluated for fixed eruptions induced by UV radiation at a dermatology referral hospital in Bogotá, Colombia. The lesions were located on the inner thighs, buttocks, popliteal region, anterior and posterior axilla, and dorsum of the feet. Photoprovocation reproduced lesions in all the affected areas, and histopathology showed changes similar to those seen in fixed drug eruptions. While these UV-provoked reactions may be a type of fixed skin eruption, we cannot rule out that they may also be a distinct condition that simply shares a pathogenic mechanism with fixed eruptions.(AU)

[Translated article] Fixed Sunlight Eruption: A Series of 13 Cases in Bogotá, Colombia / Erupción fija por luz solar, una serie de 13 casos en Bogotá, Colombia

Desde 1975 se han publicado algunos casos que asocian la radiación ultravioleta como un desencadenante de erupciones cutáneas fijas (erupción o exantema fijo por luz solar o síndrome de fotosensibilidad localizada de amplio espectro). Describimos los casos de 13 pacientes con esta dermatosis, 4 varones (30,8%) y 9 mujeres (69,2%), con edades comprendidas entre los 28 y los 56 años, atendidos en un centro de referencia en dermatología en Bogotá, Colombia. Las lesiones se localizaron en la cara interna de los muslos, los glúteos, la región poplítea, la axilar anterior y posterior y el dorso de los pies. La prueba de fotoprovocación logró la reproducción de las lesiones en todos los casos en las áreas afectadas y la histopatología reveló cambios similares a los descritos en los eritemas fijos por medicamentos. Esta enfermedad podría corresponder a un subtipo de erupción fija, aunque no se descarta que sea una dermatosis diferente con una patogenia común.(AU)

Few reports describing an association between UV radiation and fixed skin eruptions have been published since 1975. These reactions have received various names, including fixed sunlight eruption, fixed exanthema due to UV radiation, and broad-spectrum abnormal localized photosensitivity syndrome. We present a series of 13 patients (4 men [30.8%] and 9 women [69.2%]) aged between 28 and 56 years who were evaluated for fixed eruptions induced by UV radiation at a dermatology referral hospital in Bogotá, Colombia. The lesions were located on the inner thighs, buttocks, popliteal region, anterior and posterior axilla, and dorsum of the feet. Photoprovocation reproduced lesions in all the affected areas, and histopathology showed changes similar to those seen in fixed drug eruptions. While these UV-provoked reactions may be a type of fixed skin eruption, we cannot rule out that they may also be a distinct condition that simply shares a pathogenic mechanism with fixed eruptions.(AU)

Duplicación del colédoco asociado a unión biliopancreática anómala: Reporte de caso / Common bile duct duplication associated with anomalous biliopancreatic junction: A case report

Introducción. La duplicación del colédoco es una anomalía congénita poco frecuente. En la mayoría de los casos este defecto se asocia a cálculos en la vía biliar, unión pancreatobiliar anómala, pancreatitis, cáncer gástrico o colangiocarcinoma. Por esta razón, el diagnóstico y el tratamiento temprano son importantes para evitar las complicaciones descritas a futuro. Métodos. Se presenta el caso de una paciente de 30 años, con antecedente de pancreatitis aguda, con cuadro de dolor abdominal crónico, a quien se le realizaron varios estudios imagenológicos sin claro diagnóstico. Fue llevada a manejo quirúrgico en donde se documentó duplicación del colédoco tipo II con unión pancreatobiliar anómala. Resultados. Se hizo reconstrucción de las vías biliares y hepatico-yeyunostomía, con adecuada evolución postoperatoria y reporte final de patología sin evidencia de tumor. Conclusión. El diagnóstico se hace mediante ecografía endoscópica biliopancreática, colangiorresonancia o colangiopancreatografía retrógrada endoscópica. El tratamiento depende de si está asociado o no a la presencia de unión biliopancreática anómala o cáncer. Si el paciente no presenta patología neoplásica, el tratamiento quirúrgico recomendado es la resección del conducto con reconstrucción de las vías biliares.

Introduction. Double common bile duct is an extremely rare congenital anomaly. This anomaly may be associated with bile duct stones, anomalous biliopancreatic junction, pancreatitis, bile duct cancer, or gastric cancers. Thus, early diagnosis and treatment is important to avoid complications. Clinical case. We report a rare case of double common bile duct associated with an anomalous biliopancreatic junction in a 30-year-old female, with prior history of acute pancreatitis, who presented with chronic abdominal pain. She underwent several imaging studies, without clear diagnosis. She was taken to surgical management where duplication of the type II common bile duct was documented with anomalous pancreatobiliary junction. Results. Reconstruction of the bile ducts and hepatico-jejunostomy were performed, with adequate postoperative evolution and final pathology report without evidence of tumor. Conclusion. Diagnosis is usually performed by an endoscopic ultrasound, magnetic resonance cholangiopancrea-tography, or endoscopic retrograde cholangiopancreatography. Treatment depends on the presence of anomalus biliopancreatic junction or concomitant cancer. In cases without associated malignancy, resection of bile duct and biliary reconstruction is the recommended surgical treatment.

Percepción de la mujer con psoriasis en la planificación familiar: Estudio descriptivo, transversal y multicéntrico / Family Planning Concerns Among Women With Psoriasis: A Descriptive, Cross-Sectional, Multicenter Study

Antecedentes y objetivos La psoriasis afecta a un gran porcentaje de mujeres en edad fértil. Nuestro objetivo fue conocer las inquietudes de las mujeres con psoriasis en relación con la planificación familiar. Material y métodos Estudio observacional, descriptivo, transversal y multicéntrico realizado entre marzo del 2020 y octubre del 2021. Se recabaron datos sociodemográficos e inquietudes relacionadas con la planificación familiar de mujeres entre 18-45 años con psoriasis en placas y candidatas a recibir tratamiento sistémico. Resultados Se reclutaron 153 pacientes de 11 centros españoles (edad media: 35,4 ± 8 años, duración media de la enfermedad: 16,7 años); 38,4% de los casos tenían una enfermedad moderada/grave para los médicos, aunque la percepción de la actividad era significativamente superior para las pacientes. En una de cada tres mujeres, la enfermedad limitaba o retrasaba el deseo gestacional. Existía preocupación de que la enfermedad empeorara al tener que retirar o cambiar un fármaco o que los tratamientos perjudicaran al bebé. Alrededor de la mitad de las pacientes no había recibido información sobre planificación familiar en la consulta, especialmente aquellas mujeres sin embarazos previos. Las mujeres con tratamiento biológico (58,7%) tenían mejor situación clínica, mejor calidad de vida y menos alteraciones en la esfera sexual que las pacientes sin tratamiento biológico. Conclusiones Las pacientes con psoriasis tienen numerosas preocupaciones relacionadas con la planificación familiar. En algunos casos, estos miedos podrían llevar a retrasar y/o limitar el deseo gestacional. Sería necesario incrementar la información que se da a las pacientes y mejorar la formación de los dermatólogos en este tema (AU)

Background and objective A significant proportion of women of childbearing age have psoriasis. The aim of this study was to examine family planning concerns in this population. Material and methods Observational, descriptive, cross-sectional, multicenter study conducted between March 2020 and October 2021. We collected sociodemographic data and analyzed responses to a family planning questionnaire administered to women aged 18 to 45 years with plaque psoriasis who were candidates for systemic treatment. Results We studied 153 patients (mean [SD] age, 35.4 [8.0] years; mean disease duration, 16.7 years) being treated at 11 Spanish hospitals. Overall, 38.4% of women were considered to have moderate to severe psoriasis by their physicians; perceived severity ratings were significantly higher among women. Psoriasis affected the women's desire to become pregnant or led to their delaying pregnancy in 1 in 3 respondents. They were concerned that their condition might worsen if they had to discontinue or switch treatment or that the treatment might harm the baby. Approximately half of the women had not received family planning counseling from their physicians, and this was more likely to be the case among never-pregnant women. Women on biologic therapy (58.7%) had better psoriasis control and a better quality of life than women on other treatments. Their sexual health was also less affected. Conclusions Women with psoriasis have numerous family planning concerns, which in some cases can lead them to delay pregnancy or affect their desire to become pregnant. Dermatologists need to receive better training regarding family planning in women with psoriasis so that they can provide their patients with more and better information (AU)

[Translated article] Family Planning Concerns Among Women With Psoriasis: A Descriptive, Cross-Sectional, Multicenter Study / Percepción de la mujer con psoriasis en la planificación familiar: estudio descriptivo, transversal y multicéntrico

Background and objective A significant proportion of women of childbearing age have psoriasis. The aim of this study was to examine family planning concerns in this population. Material and methods Observational, descriptive, cross-sectional, multicenter study conducted between March 2020 and October 2021. We collected sociodemographic data and analyzed responses to a family planning questionnaire administered to women aged 18 to 45 years with plaque psoriasis who were candidates for systemic treatment. Results We studied 153 patients (mean [SD] age, 35.4 [8.0] years; mean disease duration, 16.7 years) being treated at 11 Spanish hospitals. Overall, 38.4% of women were considered to have moderate to severe psoriasis by their physicians; perceived severity ratings were significantly higher among women. Psoriasis affected the women's desire to become pregnant or led to their delaying pregnancy in 1 in 3 respondents. They were concerned that their condition might worsen if they had to discontinue or switch treatment or that the treatment might harm the baby. Approximately half of the women had not received family planning counseling from their physicians, and this was more likely to be the case among never-pregnant women. Women on biologic therapy (58.7%) had better psoriasis control and a better quality of life than women on other treatments. Their sexual health was also less affected. Conclusions Women with psoriasis have numerous family planning concerns, which in some cases can lead them to delay pregnancy or affect their desire to become pregnant. Dermatologists need to receive better training regarding family planning in women with psoriasis so that they can provide their patients with more and better information (AU)

Antecedentes y objetivos La psoriasis afecta a un gran porcentaje de mujeres en edad fértil. Nuestro objetivo fue conocer las inquietudes de las mujeres con psoriasis en relación con la planificación familiar. Material y métodos Estudio observacional, descriptivo, transversal y multicéntrico realizado entre marzo del 2020 y octubre del 2021. Se recabaron datos sociodemográficos e inquietudes relacionadas con la planificación familiar de mujeres entre 18-45 años con psoriasis en placas y candidatas a recibir tratamiento sistémico. Resultados Se reclutaron 153 pacientes de 11 centros españoles (edad media: 35,4 ± 8 años, duración media de la enfermedad: 16,7 años); 38,4% de los casos tenían una enfermedad moderada/grave para los médicos, aunque la percepción de la actividad era significativamente superior para las pacientes. En una de cada tres mujeres, la enfermedad limitaba o retrasaba el deseo gestacional. Existía preocupación de que la enfermedad empeorara al tener que retirar o cambiar un fármaco o que los tratamientos perjudicaran al bebé. Alrededor de la mitad de las pacientes no había recibido información sobre planificación familiar en la consulta, especialmente aquellas mujeres sin embarazos previos. Las mujeres con tratamiento biológico (58,7%) tenían mejor situación clínica, mejor calidad de vida y menos alteraciones en la esfera sexual que las pacientes sin tratamiento biológico. Conclusiones Las pacientes con psoriasis tienen numerosas preocupaciones relacionadas con la planificación familiar. En algunos casos, estos miedos podrían llevar a retrasar y/o limitar el deseo gestacional. Sería necesario incrementar la información que se da a las pacientes y mejorar la formación de los dermatólogos en este tema (AU)

Malformación linfática quística gigante del hígado: dos casos pediátricos con diferente abordaje quirúrgico / Giant cystic lymphatic malformation of the liver: two pediatric cases with different surgical approach

Introducción. Las malformaciones linfáticas quísticas, también llamadas linfangiomas quísticos, aparecen muy raramente de forma aislada en el hígado. Casos clínicos. Se presentan dos pacientes femeninas de edad preescolar con marcada hepatomegalia, dependiente de lesiones quísticas multitabicadas, secundarias a malformación linfática quística gigante del hígado, que fueron tratadas en el Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Resultados. En ambos casos el diagnóstico se apoyó en los estudios de imágenes, la laparoscopia y el análisis histopatológico. En un caso el tratamiento fue la hepatectomía derecha, mientras que en el otro se empleó la escleroterapia, ambas con evolución favorable. Conclusión. A pesar de su rareza, este diagnóstico no debe obviarse ante un paciente pediátrico con lesiones hepáticas quísticas. El tratamiento de elección es la resección quirúrgica, pero su indicación y envergadura debe valorarse de forma individualizada

Introduction. Cystic lymphatic malformations, also called cystic lymphangiomas, are very rarely found in the liver. Clinical cases. Two pediatric female preschool-age patients. presented with hepatomegaly due to multi-septated cystic lesions of the liver, who received treatment at Hospital Pediátrico Universitario William Soler, La Habana, Cuba. Results. We report two pediatric cases with giant cystic lymphatic malformation of the liver. In both cases, the diagnosis were based on imaging, laparoscopy and pathology. In one case the treatment was right hepatectomy, whereas in the other, sclerotherapy was performed, both with a favorable outcome. Conclusion. Despite its rarity, this diagnosis should be considered in pediatric patients with hepatic cystic lesions. The recommended treatment is surgical resection, but its indication and extent should be assessed individually for each patient.

[Translated article] Family Planning Concerns Among Women With Psoriasis: A Descriptive, Cross-Sectional, Multicenter Study.

BACKGROUND AND OBJECTIVE: A significant proportion of women of childbearing age have psoriasis. The aim of this study was to examine family planning concerns in this population. MATERIAL AND METHODS: Observational, descriptive, cross-sectional, multicenter study conducted between March 2020 and October 2021. We collected sociodemographic data and analyzed responses to a family planning questionnaire administered to women aged 18 to 45 years with plaque psoriasis who were candidates for systemic treatment. RESULTS: We studied 153 patients (mean [SD] age, 35.4 [8.0] years; mean disease duration, 16.7 years) being treated at 11 Spanish hospitals. Overall, 38.4% of women were considered to have moderate to severe psoriasis by their physicians; perceived severity ratings were significantly higher among women. Psoriasis affected the women's desire to become pregnant or led to their delaying pregnancy in 1 in 3 respondents. They were concerned that their condition might worsen if they had to discontinue or switch treatment or that the treatment might harm the baby. Approximately half of the women had not received family planning counseling from their physicians, and this was more likely to be the case among never-pregnant women. Women on biologic therapy (58.7%) had better psoriasis control and a better quality of life than women on other treatments. Their sexual health was also less affected. CONCLUSIONS: Women with psoriasis have numerous family planning concerns, which in some cases can lead them to delay pregnancy or affect their desire to become pregnant. Dermatologists need to receive better training regarding family planning in women with psoriasis so that they can provide their patients with more and better information.

Family Planning Concerns Among Women With Psoriasis: A Descriptive, Cross-Sectional, Multicenter Study. / Percepción de la mujer con psoriasis en la planificación familiar: Estudio descriptivo, transversal y multicéntrico.

BACKGROUND AND OBJECTIVE: A significant proportion of women of childbearing age have psoriasis. The aim of this study was to examine family planning concerns in this population. MATERIAL AND METHODS: Observational, descriptive, cross-sectional, multicenter study conducted between March 2020 and October 2021. We collected sociodemographic data and analyzed responses to a family planning questionnaire administered to women aged 18 to 45 years with plaque psoriasis who were candidates for systemic treatment. RESULTS: We studied 153 patients (mean [SD] age, 35.4 [8.0] years; mean disease duration, 16.7 years) being treated at 11 Spanish hospitals. Overall, 38.4% of women were considered to have moderate to severe psoriasis by their physicians; perceived severity ratings were significantly higher among women. Psoriasis affected the women's desire to become pregnant or led to their delaying pregnancy in 1 in 3 respondents. They were concerned that their condition might worsen if they had to discontinue or switch treatment or that the treatment might harm the baby. Approximately half of the women had not received family planning counseling from their physicians, and this was more likely to be the case among never-pregnant women. Women on biologic therapy (58.7%) had better psoriasis control and a better quality of life than women on other treatments. Their sexual health was also less affected. CONCLUSIONS: Women with psoriasis have numerous family planning concerns, which in some cases can lead them to delay pregnancy or affect their desire to become pregnant. Dermatologists need to receive better training regarding family planning in women with psoriasis so that they can provide their patients with more and better information.

Fixed Sunlight Eruption: A Series of 13 Cases in Bogotá, Columbia. / Erupción fija por luz solar, una serie de 13 casos en Bogotá, Colombia.

Few reports describing an association between UV radiation and fixed skin eruptions have been published since 1975. These reactions have received various names, including fixed sunlight eruption, fixed exanthema due to UV radiation, and broad-spectrum abnormal localized photosensitivity syndrome. We present a series of 13 patients (4 men [30.8%] and 9 women [69.2%]) aged between 28 and 56 years who were evaluated for fixed eruptions induced by UV radiation at a dermatology referral hospital in Bogotá, Colombia. The lesions were located on the inner thighs, buttocks, popliteal region, anterior and posterior axilla, and dorsum of the feet. Photoprovocation reproduced lesions in all the affected areas, and histopathology showed changes similar to those seen in fixed drug eruptions. While these UV-provoked reactions may be a type of fixed skin eruption, we cannot rule out that they may also be a distinct condition that simply shares a pathogenic mechanism with fixed eruptions.

Dacriocistocele bilateral congénito: reporte de un caso / Congenital bilateral dacryocystocele: case report

Introducción: el dacriocistocele es una malformación congénita rara, secundaria a la obstrucción del conducto nasolagrimal; tiene una incidencia de 0,1 % de pacientes con obstrucción congénita del conducto nasolagrimal y se encuentra bilateralmente hasta en 25 % de casos. Caso clínico: lactante femenina de dos meses con celulitis periorbitaria derecha preseptal no asociada con lesión de entrada y diagnóstico inicial de dacriocistitis derecha. Al examen físico de ingreso, en el ojo derecho se evidencia gran masa abscedada en saco lagrimal; en el ojo izquierdo, un área indurada y leve reflujo a la presión del saco lagrimal. Se realiza tomografía computarizada de órbitas con hallazgos compatibles con dacriocistocele bilateral. Discusión y conclusiones: conocer la presentación y posibles complicaciones asociadas con esta patología previene una morbilidad importante al paciente. La mayoría de los casos de dacriocistocele se pueden manejar médicamente, sin embargo, aquellos asociados con complicaciones requieren de manejo quirúrgico oportuno.

Introduction: Dacryocystocele is a rare congenital malformation secondary to na-solacrimal duct obstruction. It has an incidence of 0.1% of patients with congenital nasolacrimal duct obstruction, being found bilaterally in up to 25% of cases. Case Report: Two-month-old female infant with preseptal right periorbital cellulitis not associated with an entrance lesion, with an initial diagnosis of right dacryocystitis. On physical examination, a large abscessed mass in the lacrimal sac was eviden-ced in the right eye; in the left eye, there was an indurated area and slight reflux to the lacrimal sac pressure. Computed tomography of the orbits was performed with findings compatible with bilateral dacryocystocele. Discussion and conclusions: Knowing the presentation and possible complications associated with this pathology prevents significant patient morbidity. Most cases of dacryocystocele can be mana-ged medically, however, cases associated with complications require timely surgical management.

Litiasis renal derecha en riñón ectópico que simula apendicitis aguda / Right renal lithiasis in ectopic kidney that simulates acute appendicitis / Litíase renal direita em rim ectópico que simula apendicite aguda

Paciente masculino de 45 años que acude por cuadro clínico de 12 horas de evolución caracterizado por dolor abdominal, en flanco y fosa ilíaca derecha, asociado a vómitos de aspecto bilioso.

Origen aórtico anómalo de las arterias coronarias / Abnormal aortic origin of coronary arteries

Resumen Introducción: El origen aórtico anómalo de las arterias coronarias tiene una prevalencia estimada del 0.02-5.7% y están potencialmente involucradas con complicaciones en su evolución natural o aquellos que requieran procedimientos intervencionistas hemodinámico y/o quirúrgicos. Puede asociarse a muerte súbita o dañarse durante intervenciones sobre el anillo mitral, aórtico, pulmonar o el cierre percutáneo de un defecto septal interauricular. Objetivo: identificar estos pacientes por diferentes técnicas de imágenes como el ecocardiograma Doppler color transtorácico (ETT), angiotomografía o angiografía coronaria. Métodos: Las técnicas de imágenes utilizadas para la detección de anomalías coronarias fueron el ETT, angiotomografía coronaria multicorte o angiografía coronaria convencional de acuerdo con lo universalmente aceptado. Resultados: Estudio prospectivo realizado desde enero del 2020 a junio del 2021. Se identificaron 15 pacientes y en 12/15 la sospecha fue por ETT y en los tres restantes por angiotomografía coronaria. La arteria circunfleja fue la más involucrada en forma aislada o asociada a otra anomalía coronaria (12/15 pacientes) y en los tres casos restantes la coronaria anómala tuvo un trayecto interarterial, siendo las arterias coronarias derecha y la descendente anterior las afectadas. Conclusiones: El subdiagnóstico por ETT de las anomalías de las arterias coronarias puede deberse a la dificultad para su visualización, sobre todo en la población adulta. Su detección es crucial, ya que puede generar muerte súbita asociada a isquemia miocárdica y arritmias graves o complicar procedimientos intervencionistas sobre el septum interauricular o sobre los anillos mitral, pulmonar y/o aórtico.

Abstract Introduction: The anomalous aortic origin of the coronary arteries has an estimated prevalence of 0.02-5.7%. It can be associated with sudden death when it has an interarterial or intramural pathway or be damaged during interventions on the mitral, pulmonary and/or aortic annulus or percutaneous closure of an interatrial septal defect. Objective: To identify these patients by imaging techniques such as transthoracic color Doppler echocardiography (TTE), computed tomography (CT) multislice angiography or coronary angiography. Methods: The imaging techniques used for the detection of coronary anomalies were TTE, multislice coronary angiography or coronary angiography according to what is generally accepted. Results: Fifteen patients were identified; in 12 of them the suspicion was due to TTE and in the remaining 3, CT multislice angiography was diagnostic. The circumflex artery was the coronary artery most involved, associated or not with another coronary anomaly (12/15 patients) and in the other three cases, the anomalous coronary artery had an interarterial course, with the right coronary arteries and the anterior descending coronary arteries being involved. Conclusions: The under diagnosis by TTE of coronary artery abnormalities may be due to the difficulty of visualization that is accentuated with age. Their detection is crucial because they can both, lead to sudden death associated with an intramural and/or interarterial pathway and complicate an interventional procedure on the interatrial septum or within the mitral, pulmonary and/or aortic rings.

Hyaluronic Acid Dermal Fillers for Treatment of Congenital and Acquired Lip Asymmetries and Deformities

Hyaluronic acid (HA) dermal fillers are widely used in aesthetic treatments for facial and lip modeling. Despite HA fillers has largely known to be use in procedures for augmentation the lip volume, their application to management lip abnormalities is not widespread. This study reviewed the use of HA fillers for reconstruction of congenital and acquired li p asymmetries and deformities, searching to expand knowledge about this treatment modality. To undertake this narrative review, the Medline-Pubmed, Web of Science, Scopus, Embase, Google Schoolar e Lillacs databases were searched. Several studies have reported positive results in the use of HA fillers for the treatment of lip deformities caused by different factors such as: Insatisfactory dermal fillers, permanent lip implants, excision of lip carcinoma, post-operative scars and electrical burns. HA fillers are also an alternative for the management of lip asymmetries and residual surgical scars in patients with cleft lip. Moreover, injection of HA fillers in individuals with facial paralysis and facioscapulohumeral muscular dystrophy can also improve lip incompetence. Additionally, HA fillers can be used as a complementary treatment in cases of severe malocclusion associated with skeletal changes, helping to maintain the seal and refine the lip appearance. Therefore, HA fillers can be used as alternative treatment for several types of congenital and acquired lip deformities and asymmetries. However, it is necessary to carry out randomized clinical trials with a greater number of patients and follow-up time, in order to investigate the benefits of the HA fillers for carriers patients of lip abnormalities.

Los rellenos dérmicos de ácido hialurónico (AH) son muy utilizados en tratamientos estéticos para el modelado facial y de labios. A pesar de que se sabe en gran medida que los rellenos de HA se utilizan en procedimientos para aumentar el volumen de los labios, su aplicación para el tratamiento de anomalías labiales no está muy extendida. Este estudio revisó el uso de rellenos de HA para la reconstrucción de asimetrías y deformidades labiales congénitas y adquiridas, buscando ampliar el conocimiento sobre esta modalidad de tratamiento. Para realizar esta revisión narrativa, se realizaron búsquedas en las bases de datos Medline-Pubmed, Web of Science, Scopus, Embase, Google Schoolar y Lillacs. Varios estudios han reportado resultados positivos en el uso de rellenos de HA para el tratamiento de deformidades labiales causadas por diferentes factores tales como: rellenos dérmicos insatisfactorios, implantes labiales permanentes, escisión de carcinoma labial, cicatrices postoperatorias y quemaduras eléctricas. Los rellenos de HA también son una alternativa para el manejo de asimetrías labiales y cicatrices quirúrgicas residuales en pacientes con labio hendido. Además, la inyección de rellenos de HA en personas con parálisis facial y distrofia muscular facioescapulohumeral también puede mejorar la incompetencia de los labios. Además, los rellenos de HA se pueden usar como un tratamiento complementario en casos de maloclusión severa asociada con cambios esqueléticos, lo que ayuda a mantener el sellado y refinar la apariencia de los labios. Por lo tanto, los rellenos de HA se pueden utilizar como tratamiento alternativo para varios tipos de deformidades y asimetrías labiales congénitas y adquiridas. Sin embargo, es necesario realizar ensayos clínicos aleatorizados con mayor número de pacientes y tiempo de seguimiento, para investigar los beneficios de los rellenos HA para pacientes portadores de anomalías labiales.

Malformación completa del sistema nervioso central y otras malformaciones asociadas. A propósito de un caso / Complete malformation of the central nervous system and other associated malformations. A case report

La anencefalia es una anomalía gestacional que produce la ausencia de gran parte del cerebro y del cráneo; aunque se desconoce el motivo principal de su aparición, puede estar relacionado con toxinas ambientales, y pobre ingesta de ácido fólico durante el embarazo. Los fetos que padecen de esta malformación, en su mayoría, no llegan a nacer vivos o mueren a las pocas horas. Se presentó una paciente recibida en emergencia en el Centro de Atención Integral Materno Infantil, municipio Ixchiguan, departamento de San Marcos, Guatemala con embarazo a término, según refirió la paciente, sin atención prenatal, dolor en hipogastrio y pérdida de líquido amniótico de un día de evolución. Siendo este un defecto genético multifactorial, para el cual no existe tratamiento, el único recurso para su prevención fue brindar una atención prenatal pormenorizada con énfasis especial en el programa de genética para la detección precoz de anomalías congénitas.

Anencephaly is a gestational anomaly that produces the absence of a large part of the brain and skull; although the main reason for its appearance is unknown, it may be related to environmental toxins and poor intake of folic acid during pregnancy. Most of the fetuses that suffer from this malformation are not born alive or die within a few hours. We present a female patient who was received in the emergency service of the Comprehensive Maternal and Child Care Center in Ixchiguan municipality, San Marcos department, Guatemala, with a full-term pregnancy, as reported by the patient, without prenatal care, hypogastric pain and loss of amniotic fluid of one day of evolution. Since there is no treatment for this multifactorial genetic defect, the only resource for its prevention was to provide detailed prenatal care with special emphasis on the genetics program for the early detection of congenital anomalies.