Reabilitação auditiva com sistemas Baha® transcutâneo e percutâneo / Hearing rehabilitation with Baha® transcutaneous and percutaneous systems

RESUMO Objetivo Verificar longitudinalmente a influência dos limiares tonais auditivos obtidos com as próteses auditivas ancoradas no osso transcutâneas e percutâneas na percepção da fala em indivíduos com malformação de orelha externa e/ou média e Otite Média Crônica. Método Estudo observacional, retrospectivo, de seguimento longitudinal de 30 indivíduos usuários unilaterais de sistema Baha® transcutâneo e percutâneo, para coleta de dados secundários dos limiares tonais obtidos por meio da audiometria em campo livre e do limiar de reconhecimento de sentenças no silêncio e no ruído nas condições: sem a prótese; no momento de ativação; no primeiro mês de uso (pós 1); e no terceiro mês (pós 2). Resultados Houve diferença significante entre os limiares tonais obtidos nas frequências de 3 e 4kHz, com melhores resultados para o percutâneo em todos os momentos de avaliação. Para os dois sistemas, observou-se melhor desempenho no reconhecimento de sentenças no silêncio e ruído, com diferença significante na ativação (p<0,001), porém manteve-se estável nos demais momentos de avaliação. O sistema percutâneo mostrou melhor benefício no reconhecimento de sentenças no ruído apenas na ativação (p=0,036), quando comparado ao transcutâneo. Conclusão O sistema percutâneo possibilitou melhor audibilidade para as frequências altas; contudo, tal audibilidade não influenciou no reconhecimento de sentenças na situação de silêncio para ambos os sistemas. Para a situação de ruído, melhores respostas foram observadas no sistema percutâneo; porém, a diferença não se manteve no decorrer do tempo.

ABSTRACT Purpose Longitudinally verify the influence of auditory tonal thresholds obtained with transcutaneous and percutaneous bone-anchored hearing aids on speech perception in individuals with external and/or middle ear malformation and chronic otitis media. Methods Observational, retrospective, longitudinal follow-up study of 30 unilateral users of the transcutaneous and percutaneous Baha® system for the collection of secondary data on pure tone thresholds obtained through free field audiometry and sentence recognition threshold in silence and noise in conditions: without the prosthesis; at the time of activation; in the first month of use (post 1); and in the third month (post 2). Results There was a significant difference between pure tone thresholds obtained at frequencies of 3 and 4kHz with better results for the percutaneous technique at all evaluation moments. For both systems, better performance was observed in sentence recognition in silence and in noise, with a significant difference in activation (p<0.001), but it remained stable during the other evaluation moments. The percutaneous system showed better benefit in recognizing sentences in noise only on activation (p=0.036), when compared to the transcutaneous system. Conclusion The percutaneous system provided better audibility for high frequencies; however, such audibility did not influence sentence recognition in the silent situation for both systems. For the noise situation, better responses were observed in the percutaneous system, however, the difference was not maintained over time.

Rol de la laparoscopia en el manejo del síndrome OHVIRA: reporte de un caso y su relación con la endometriosis / Role of laparoscopy in the management of OHVIRA syndrome: a case report and its relation to endometriosis

El síndrome de hemivagina obstruida y anomalía renal ipsilateral (OHVIRA) es producido por una alteración en el desarrollo de los conductos de Müller y Wolff en la vida fetal. El síndrome es poco frecuente, se reporta una prevalencia de 1/2.000 a 1/28.000 casos. La endometriosis se presenta en un 19% de los casos complicando esta patología. El tratamiento del síndrome OHVIRA consiste en resecar el tabique vaginal drenando el hematocolpos. Hasta el momento no existe un consenso en recomendar la realización de una laparoscopia diagnóstica. El objetivo de este estudio es reportar la eventual importancia de la laparoscopia diagnóstica/terapéutica como parte del manejo del síndrome OHVIRA.

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is caused by a defect in the development of Müllerian and Wolffian ducts at fetal life. The syndrome is uncommon, with a reported prevalence of 1/2,000 to 1/28,000 cases. Endometriosis is present in 19% of cases complicating this pathology. Treatment of OHVIRA syndrome consists in resecting the vaginal septum and draining the hematocolpos. Until now there isnt an agreement on recommending diagnostic laparoscopy as part of the treatment. The aim of this study is to report the importance of diagnostic/therapeutic laparoscopy in the management of OHVIRA syndrome.

Prevalencia de la arteria umbilical única en gestantes: su relación con otras malformaciones y el bajo peso al nacer

Introducción: La arteria umbilical única tiene una incidencia del 1 % en los recién nacidos. Se le asocia frecuentemente con gemelaridad, malformaciones y crecimiento intrauterino retardado, y constituye un factor de riesgo de prematuridad, muerte fetal y neonatal. Objetivos: Determinar la prevalencia de la arteria umbilical única en gestantes, y la asociación de esta entidad con otras malformaciones y el bajo peso al nacer. Materiales y métodos: Estudio descriptivo retrospectivo, con datos obtenidos de las historias clínicas y del modelo de seguimiento lineal existente en las consultas de genética comunitaria del municipio Matanzas, de enero de 2015 a diciembre de 2019. Resultados: La prevalencia de la arteria umbilical única fue del 0,3 %. Las malformaciones más frecuentes fueron las renales; el 27,7 % de los nacimientos fueron pretérmino y el 33,3 % de los nacidos fue con un peso inferior a 2500 g. Conclusiones: La arteria umbilical única constituye un marcador para otras malformaciones. Cuando coexisten ambas existe riesgo de prematuridad y bajo peso al nacer. Se recomienda realizar examen clínico posnatal a todo recién nacido con arteria umbilical única, pesquisando defectos renales y cardíacos.

Introduction: The single umbilical artery has an incidence of 1% in newborns. It is frequently associated with twinning, malformations and delayed intrauterine growth, and is a risk factor of prematurity, fetal and neonatal death. Objective: To determine the prevalence of single umbilical artery in pregnant women and the association of this entity with other malformations and low birth weight. Materials and methods: Retrospective descriptive study, with data obtained from medical records and the linear follow-up model existing in the community genetic clinics of the municipality of Matanzas, from January 2015 to December 2019. Results: The prevalence of the single umbilical artery was 0.3%. The most frequent malformations were renal ones; 27.7% of births were pre-term and 33.3% of those born weighed less than 2500g. Conclusions: The single umbilical artery is a marker for other malformations. When both coexist there is a risk of prematurity and low birth weight. Postnatal clinical examination is recommended for all newborns with single umbilical artery, checking for renal and heart defects.

Importância do diagnóstico por imagem em pacientes com sangramento uterino anormal / Importance of diagnostic imaging in patients with abnormal uterine bleeding

O sangramento uterino anormal é diagnóstico sindrômico comum no consultório do ginecologista e pode comprometer substancialmente a qualidade de vida. O objetivo no diagnóstico de sangramento uterino anormal é distinguir pacientes com causas estruturais (anatômicas), como pólipo, adenomiose, leiomioma, malignidade e hiperplasia, de pacientes que apresentam anatomia normal, nas quais o sangramento pode ser devido a alteração dos mecanismos de coagulação, distúrbios ovulatórios, distúrbios primários do endométrio, iatrogenia, ou ter outra causa não classificada. O diagnóstico se inicia a partir de anamnese detalhada e exame físico geral e ginecológico completos, seguidos da solicitação de exames complementares (laboratoriais e de imagem), conforme indicado. O exame de imagem de primeira linha para identificação das causas estruturais inclui a ultrassonografia pélvica. Histerossonografia, histeroscopia, ressonância magnética e amostragem endometrial para exame de anatomia patológica são opções que podem ser incluídas no diagnóstico a depender da necessidade. O objetivo deste artigo é apresentar a relevância dos exames de imagem na investigação das causas de sangramento uterino anormal.

Abnormal uterine bleeding is one of the commonest presenting complaints encountered in a gynecologist's office and may substantially affect quality of life. The aim in the diagnosis of abnormal uterine bleeding is to distinguish women with anatomic causes such as polyp, adenomyosis, leiomyoma, malignancy and hyperplasia from women with normal anatomy where the cause may be coagulopathy, ovulatory disorders, endometrial, iatrogenic and not otherwise classified. Diagnosis begins with a thorough history and physical examination followed by appropriate laboratory and imaging tests as indicated. The primary imaging test for the identification of anatomic causes include ultrasonography. Saline infusion sonohysterography, magnetic resonance, hysteroscopy, endometrial sampling are options that can be included in the diagnosis depending on the need. The aim of this article is to present the relevance of imaging exams in the investigation of the causes of abnormal uterine bleeding.

Congenital pulmonary malformations in children in a pediatric hospital in Peru, 2010-2020 / Malformaciones pulmonares congénitas en niños de un hospital pediátrico de Perú, 2010-2020

Abstract Background: Congenital pulmonary malformations (CPMs) are rare in children. This study aimed to describe the clinical, imaging characteristics, and treatment of patients with this pathology. Methods: We conducted a descriptive and retrospective study with data from patients with CPMs diagnosed at Instituto Nacional de Salud del Niño-Breña (Lima-Peru), from January 2010 to December 2020. We described CPM clinical and imaging characteristics, type and treatment. Results: The sample was formed of 70 patients. The median age was 29 months (range 15 days-14 years) and the male/female ratio was 1.4. The chest tomography found parenchymal involvement in 50 (71.4%) cases and mixed involvement (parenchymal and vascular) in 18 (25.7%) cases. Congenital malformation of the pulmonary airway was present in 39 (55.7%) cases, followed by bronchogenic cyst in 10 (14.3%), intralobar pulmonary sequestration in 9 (12.9%), and extralobar pulmonary sequestration in 7 (10%). Lobectomy was performed in 61 (87.1%) cases, cystectomy in 5 (7.1%), segmentectomy in 2 (2.9%), and embolization in 2 (2.9%). The most frequent post-operative complication was pneumonia, found in 9 (12.9%) cases. The median hospital stay was 26 days. No patient died during hospitalization. Conclusions: In our institution, the most frequent CPM was congenital malformation of the pulmonary airway, and lobectomy was the most frequently performed surgical procedure. CPMs represent a diverse group of disorders of lung development with varied imaging patterns and clinical manifestations.

Resumen Introducción: Las malformaciones pulmonares congénitas son poco frecuentes en niños. El objetivo de este estudio fue describir las características clínicas, imagenológicas y tratamiento de los pacientes con esta patología. Métodos: Se llevó a cabo un estudio descriptivo y retrospectivo con datos de los pacientes con malformaciones pulmonares congénitas diagnosticados en el Instituto Nacional de Salud del Niño-Breña (Lima-Perú) entre enero 2010 y diciembre 2020. Se describieron las características clínicas, imagenológicas, el tipo de malformación pulmonar congénita y el tratamiento. Resultados: La muestra fue de 70 pacientes. La mediana de edad fue 29 meses, la relación masculino/femenino fue 1.4. En la tomografía de tórax se encontró compromiso parenquimal en 50 (71.4%) casos, y compromiso mixto (parenquimal y vascular) en 18 (25.7%). La malformación congénita de la vía aérea pulmonar se observó en 39 (55.7%) casos, seguida del quiste broncogénico en 10 (14.3%), secuestro pulmonar intralobar en 9 (12.9%) y secuestro pulmonar extralobar en 7 (10%). La lobectomía fue realizada en 61 (87.1%) casos, la quistectomía en 5 (7.1%), segmentectomía en 2 (2.9%) y embolización en 2 (2.9%). La complicación posquirúrgica más frecuente fue la neumonía en 9 (12.9%) casos. La mediana de estancia hospitalaria fue de 26 días. Ningún paciente falleció durante la hospitalización. Conclusiones: En nuestra institución, la malformación pulmonar congénita más frecuente fue la malformación congénita de la vía aérea pulmonar, y la lobectomía el procedimiento quirúrgico más comúnmente realizado. Las malformaciones pulmonares congénitas representan un grupo diverso de trastornos del desarrollo pulmonar con variados patrones imagenológicos y manifestaciones clínicas.

Venous malformation of the conjunctiva and orbit

A 28-year-old male presented with a large conjunctival lobulated, nonpulsatile, red vascular lesion involving a large part of the temporal quadrant of the left globe. There was no proptosis or globe displacement, but the left eye abduction was limited. T2-weighted magnetic resonance imaging scan of the brain and orbit revealed an extensive contrast-enhancing lobulated lesion in the left half of the face involving the upper lip, cheek, oral cavity, extraconal space of left orbit, and nasal cavity. He underwent surgical excision of the conjunctival lesion with amniotic membrane reconstruction.

A Truncus Bicaroticus and an Aberrant Right Subclavian Artery. A Rare Case and Literature Review / Un Tronco Bicarótico y una Arteria Subclavia Derecha Aberrante. Un Caso Raro y Revisión de la Literatura

SUMMARY: An aberrant right subclavian artery described by David Bayford, is rare and one of the aortic arch anomalies. It gives usually incidental findings. We present the case of a 57-year-old woman who was admitted to a neurology outpatient clinic due to a headache and no obvious pathology was detected during a physical examination. In consequence of the brain and chest CT angiography, incidental anomalies of the aortic arch branches were found and asymptomatic aberrant right subclavian artery and bicarotid trunk was diagnosed. The presence of this anomaly is becoming increasingly important in avoiding vascular injuries and cerebral complications in patients undergoing endovascular intervention on the aorta aortic arch surgery, thyroidectomy, or cervicothoracic surgery. So, detailed knowledge of variations of the subclavian artery and bicarotid trunk is paramount for radiologists and surgeons operating on the region of the anterior neck.

Una arteria subclavia derecha aberrante descrita por David Bayford, es rara y es una de las anomalías del arco aórtico. Además, suele ser hallazgos accidentales. Presentamos el caso de una mujer de 57 años que ingresó a una consulta de neurología por dolor de cabeza, sin embargo, no se le detectó patología evidente al examen físico. En el angio-TC de cerebro y tórax, se encontraron anomalías incidentales de las ramas del arco aórtico y se diagnosticó arteria subclavia derecha aberrante asintomática y tronco bicarotídeo. Determinar la presencia de esta anomalía es cada vez más importante, para así evitar lesiones vasculares y complicaciones cerebrales en pacientes sometidos a intervención endovascular de la aorta, cirugía del arco aórtico, tiroidectomía o cirugía cervicotorácica. Por lo tanto, el conocimiento detallado de las variaciones de la arteria subclavia y el tronco bicarotídeo es primordial para los radiólogos y cirujanos que operan en la región anterior del cuello.

Diagnóstico prenatal de malformaciones del tracto urinario: evaluación posnatal y resultado clínico / Prenatal diagnosis of urinary tract malformations: postnatal evaluation and clinical outcome

Resumen Antecedentes: Las malformaciones del tracto urinario representan el 15-20% de las anomalías por ecografía prenatal; la prevalencia oscila entre 3 y 6 por cada 1,000 nacimientos. Objetivo: Conocer el desenlace clínico de los recién nacidos diagnosticados prenatalmente con malformación del tracto urinario. Material y métodos: Observacional, analítico, ambispectivo. Recién nacidos con diagnóstico prenatal de malformación urinaria. Evaluación posnatal con pruebas diagnósticas, analizamos días de estancia hospitalaria, intervención quirúrgica y seguimiento. Resultados: 45 pacientes, 55% varones, 65% de término, 77% cesárea. El diagnóstico prenatal más frecuente fue hidronefrosis, 100% se realizó ultrasonido renal posnatal, 27% cistograma miccional, 42% gammagrama renal; 60% requirió estancia hospitalaria prolongada, 27% intervención quirúrgica, 60% seguimiento. 89% de los casos diagnosticados prenatalmente se corroboró algún tipo de malformación del tracto urinario, solo en el 75% de los casos fue el mismo diagnóstico. Conclusión: La evaluación posnatal siempre requiere la realización de un ultrasonido renal y no en todos los casos realización de cistograma miccional o gammagrama renal.

Abstract Background: Urinary tract malformations represent 15-20% of abnormalities in prenatal ultrasound; the prevalence oscillates between 3-6 per 1000 births. Objective: To know the clinical outcome of newborns diagnosed prenatally with some malformation of the urinary tract. Material and methods: Observational, analytical, ambispective. Newborns with prenatal diagnosis of malformation urinary. Postnatal evaluation diagnostic tests performed, we analyzed the days of hospital stay, surgical intervention, follow-up. Results: 45 children, 55% male, 65% term, 77% c-section. Prenatal diagnosis with higher frequency was hydronephrosis. In all cases postnatal renal ultrasound was performed, 27% voiding cystogram, 42% renal gammagram. A 60% of the cases required prolonged hospital stay, 27% required a surgical intervention, 60% were follow-up. 89% of the cases diagnosed prenatally corroborated some type of malformation of the urinary tract, only in 75% of the cases was the same diagnosis. Conclusion: Postnatal evaluation always requires the performance of a renal ultrasound and not in all cases the performance of voiding cystogram or renal gammagram.

A rare case of Syringomyelia with Arnold � Chiari Malformation Type 1 - A case report

Syringomyelia is a pathologic cystic cavity within the spinal cord containing cerebrospinal fluid (CSF). It is commonly seen as a complication of an Arnold-Chiari type 1 malformation, which is the herniation of cerebellar tonsils through foramen magnum into cervical spinal canal. Syringomyelia can also occur as complication of hemorrhage, tumor, meningitis, arachnoiditis, or trauma. Symptoms usually begin to appear in early and middle life. These symptoms usually consist of pain, dissociated sensory loss and weakness that present and progress gradually. We herewith report a rare case of syringomyelia and associated Chiari I malformation presenting with dissociated sensory impairment in neck region with headache and neck pain Treatment in these cases is surgical decompression. Recovery with significant decrease in sensory loss and relief in headache and neck pain.

A rare case of sirenomelia

Sirenomelia also called mermaid syndrome is a congenital, rare, lethal, multisystemic human malformation of unknown etiology. The characteristic feature of sirenomelia is the fusion of the lower limbs, resulting in the appearance of a mermaid’s tail, and thus the name “mermaid syndrome.” This condition is also characterized by various severe urogenital abnormalities and the presence of a singular umbilical cord blood vessel, and it is more common in infants of diabetic mothers and in monozygotic twins. The incidence is around 1 in 60,000–70,000 pregnancies. The majority of affected fetuses are stillborn, whereas the rest of the live-born die in the early neonatal period due to complications of the gastrointestinal and urogenital systems. We are reporting a case of sirenomelia in a neonate born to an unregistered multipara mother. The baby had perinatal asphyxia, sirenomelia, dextrocardia, low set ears, lymphatic malformation, bilateral renal agenesis, absent external genitalia and anus, single umbilical artery, and congenital corneal clouding. There was no antenatal ultrasonography done and the baby died at 6 h of life.

Evaluation of Anaesthetic Challenges in Primary Cleft Lip and Palate Surgeries in a Single Center Study

Background: One of the most prevalent congenital malformations and the most frequent craniofacial defects in children is cleft lip and palate. The aim of the study was to investigate the anaesthetic procedures employed and to determine the challenges and postoperative complications associated with cleft lip and palate surgery. Material & Methods: A retrospective review of the anaesthetic procedures carried out from January 2022 to December 2022 at the Dhaka Dental College and Hospital. The hospital records were reviewed in order to learn about every occurrence of orofacial cleft surgery. In this study, a total number of 120 cases were reviewed. Results: Among 120 cases, 53 (44.2%) of the patients were female and 67 (55.8%) were male. The bulk of patients came after their first year of life. About 54(45%) of them were younger than five years old. 68(56.67%) underwent cleft lip surgery, 37(30.83%) for cleft palate surgery and rest 15(12.5%) patients under went for combined cleft lip and cleft palate surgeries. Upper Respiratory Tract Infection (URTI) was reported as the most common pre-existing morbidity. Bronchospasm was the main intraoperative complications occurred in 8 (6.67%) of the cases with URTI. After surgey 6(5%) patients experienced bleeding, 3(2.5%) patients needed to be reintubated. In addition, 2 (1.67%) instances had trouble controlling their pain, only 1(0.83%) patient had trouble swallowing and 1(0.83%) recovered slowly. Conclusion: Anaesthesia for surgical repair of cleft lip or palate in children is challenging. After a thorough preoperative evaluation, a trained anaesthesiologist should administer anaesthesia under strict supervision to minimize postoperative complications.

Análisis retrospectivo de perfil microbiológico y resistencia antimicrobiana en infección urinaria pediátrica de hospitales públicos de Quito-Ecuador

Objetivo: Determinar el perfil microbiológico y resistencia antimicrobiana en infección urinaria en niños. Materiales y métodos: Estudio descriptivo, transversal, observacional y multicéntrico. Se estudiaron 445 urocultivos procesados y los resultados de antibiogramas en tres hospitales públicos de Quito (Ecuador). En relación con los agentes causales se establecieron frecuencias absolutas y proporciones. En el análisis bivariable entre el antecedente de malformación renal o de la vía urinaria y el riesgo de infección, se aplicó el test Chi2 (p < 0,05) y la RP [IC 95 %; p < 0,05]. Resultados: Se evidenció una resistencia ante aminopenicilinas del 73,5 %; ampicilina más sulbactam 31,8 %; trimetoprim-sulfametoxazol 55,5 %; cefalosporinas de primera y segunda generación hasta 33 %; cefalosporinas de tercera y cuarta generación del 21,3 al 47 %. Ante malformación urinaria y aislamiento de bacterias diferentes a Escherichia coli, se identificó a Klebsiella pneumoniae RP 2,66 [IC 95 %, 1,9-3,6; p < 0,05] y Pseudomonas aeruginosa RP 2,07 [IC 95 %, 1,2-3,5; p < 0,05]. Conclusiones: En nuestro medio, ante el diagnóstico de infección urinaria, no parece adecuado iniciar tratamiento antibiótico con aminopenicilinas, trimetoprim-sulfametoxazol ni cefalosporinas de primera a cuarta generaciones por su elevada resistencia. La presencia de malformación urinaria se asocia a infección por bacterias diferentes de Escherichia coli.

Objective: Determine the microbiological profile and antimicrobial susceptibility in urinary infection in children. Materials and methods: Descriptive, cross-sectional, observational, and multicenter study. 445 urine cultures and the results of antibiograms were studied in three public hospitals in Quito (Ecuador). In relation to the causal agents, absolute frequencies and proportions were established. In the bivariate analysis, Chi-squared test (p < 0.05) and PR [CI 95 %; p < 0.05] were applied between history of kidney or urinary tract malformation and risk of infection. Results: There was evidence of resistance to aminopenicillins of 73.5 %; ampicillin plus sulbactam 31.8 %; trimethoprim-sulfamethoxazole 55.5 %; first and second generation cephalosporins up to 33 %; resistance to third and fourth generation cephalosporins from 21.3 to 47%. In relation to urinary malformation and the isolate of a bacteria different from Escherichia coli, Klebsiella pneumoniae PR 2,66 [CI 95 %, 1.9-3.6; p < 0.05] and Pseudomonas aeruginosa PR 2.07 [CI 95 %, 1.2-3.5; p < 0.05] were identified. Conclusions: In our locality it wouldn't be appropriate to start antibiotic treatment with aminopenicillins, trimethoprim-sulfamethoxazole or first to fourth generation cephalosporins in urinary tract infection due to their resistance. The presence of urinary malformation is associated with infection by bacteria other than Escherichia coli.

Malformación de Arnold-Chiari tipo I con siringomielia

Introducción: Las malformaciones de Arnold-Chiari, son un grupo heterogéneo de desórdenes neurológicos caracterizados por alteraciones dentro de las regiones del cerebelo, tallo cerebral y unión cráneo-cervical; todas resultan en un desplazamiento inferior del cerebelo hacia el canal espinal por el foramen magno, sea en conjunto con la médula inferior o no. Objetivo: Describir los hallazgos clínicos, estudios complementarios, criterios diagnósticos, conducta terapéutica y evolución, en un caso con malformación de Chiari tipo I con siringomielia. Presentación de caso: Se presenta a una paciente con cefalea de curso insidioso a la que se le diagnostica malformación de Chiari tipo I con siringomielia, que requirió tratamiento neuroquirúrgico. La evolución posoperatoria fue favorable. Conclusiones: La malformación de Arnold-Chiari es una entidad rara; la aparición de la resonancia magnética nuclear ha permitido que su diagnóstico aumente y por tanto, su incidencia. El caso presentado tuvo un curso clínico clásico.

Introduction: Arnold-Chiari malformations are a heterogeneous group of neurological disorders characterized by alterations within the regions of the cerebellum, brain stem and craniocervical junction; all result in inferior displacement of the cerebellum into the spinal canal through the foramen magnum, whether in conjunction with the inferior cord or not. Objective: To describe the clinical findings, complementary studies, diagnostic criteria, therapeutic approach and evolution, in a case with type I Chiari malformation with syringomyelia. Case report: A patient with an insidious course of headache is reported. She was diagnosed with type I Chiari malformation with syringomyelia, which required neurosurgical treatment. The postoperative evolution was favorable. Conclusions: Conclusions: Arnold-Chiari malformation is a rare entity. the appearance of nuclear magnetic resonance has allowed it to be diagnosed and therefore its incidence has increased. The reported case had a classic clinical course.

Concordance between referral and final diagnoses of pediatric patients with vascular malformations / Concordancia entre el diagnostico de referencia y final de pacientes pediátricos con malformaciones vasculares

Abstract Background: Vascular malformations (VaM) are a heterogeneous group of disorders resulting from the dysmorphogenesis of blood vessels. Although correct classification is relevant to providing adequate treatment according to evidence-based medicine, diagnostic terminology may be misused or need clarification. Methods: We conducted a retrospective study to measure agreement and concordance between referral and final confirmed diagnoses of 435 pediatric patients with VaM newly referred to the multidisciplinary Vascular Anomalies Clinic (VAC) using Fleiss kappa (κ) concordance analysis. Results: We found fair concordance between referral and confirmed diagnoses of VaM (κ 0.306, p < 0.001). Lymphatic malformations (LM) and VaM associated with other anomalies showed moderate diagnostic concordance (κ 0.593, p < 0.001 and κ 0.469, p < 0.001, respectively). Conclusions: Continuing medical education strategies are required to improve physician knowledge and diagnostic accuracy in patients with VaM.

Resumen Introducción: Las malformaciones vasculares (MVa) son un grupo heterogéneo de trastornos resultantes de la dismorfogénesis de los vasos sanguíneos. A pesar de que la correcta clasificación es relevante para brindar un adecuado tratamiento de acuerdo con la medicina basada en la evidencia, la terminología diagnóstica podría resultar confusa o utilizarse de manera inapropiada. Métodos: En este estudio retrospectivo se midieron el acuerdo y la concordancia entre los diagnósticos de referencia (o derivación) y los diagnósticos finales confirmados de 435 pacientes pediátricos con MVa recién remitidos a la Clínica de anomalías vasculares (CAV) multidisciplinaria, mediante el análisis de concordancia kappa de Fleiss (κ). Resultados: Se encontró una buena concordancia entre los diagnósticos de referencia (o derivación) y los diagnósticos confirmados de MVa (κ 0.306, p < 0.001). Las malformaciones linfáticas (LM) y las MVa asociadas con otras anomalías presentaron concordancias diagnósticas moderadas (κ 0.593, p < 0.001 y κ 0.469, p < 0.001, respectivamente). Conclusiones: Se requiere de estrategias de educación médica continua para mejorar el conocimiento de los médicos y la precisión diagnóstica de los pacientes con MVa.

Experiencia en patología cervical alta en el Hospital Guillermo Almenara Irigoyen 2016 - 2021

Introducción : El complejo C0-C1-C2 es responsable de la transición de la carga axial, con función biomecánica única, siendo afectada por múltiples patologías, que por lo general la literatura no las considera como un solo ítem, sino que lo desarrolla según su etiología, pero en nuestro estudio se ha considerado en 5 grupos: traumática, congénita, inflamatoria reumática, neoplásica y degenerativa. Objetivo : Determinar las características epidemiológicas, clínicas y del tratamiento en la patología cervical alta. Materiales y métodos : Se incluyeron a todos los pacientes con diagnóstico clínico radiológico de alguna patología cervical alta que hayan sido sometidos a tratamiento quirúrgico entre 2016 y 2021 en el Hospital Almenara. Se usó el test "t" de student y de chi cuadrado. Se dividió a los pacientes en alguno de los 5 grupos antes mencionados. Resultados : Se consideraron 31 pacientes, con una edad media de 51.16 años. La patología cervical alta más frecuente fue la traumática con el 35.48%. El déficit motor se presentó en el 51.61% y el déficit sensitivo se presentó en el 54.84%. La cirugía más frecuente fue la fijación cervical alta con el 43.89%. La tasa de complicaciones fue del 16.13% con una mortalidad del 0%. Conclusiones : La patología cervical alta es rara, siendo la del tipo traumática la más frecuente, pero un manejo oportuno y adecuado permite un mejor pronóstico funcional del paciente.

Introduction : The C0-C1-C2 complex is responsible of axial load transition, and its biomechanical function is unique, it is affected by multiple pathological conditions; and generally speaking, the literature does not consider these conditions as a single item, it describes them according to etiology. For our study we considered five groups: trauma-related, congenital, rheumatic-inflammatory, neoplastic, and degenerative. Objective : To determine epidemiological, clinical, and therapy-related characteristics in upper cervical pathological conditions. Materials and methods : All patients with a clinical-radiological diagnosis of any upper cervical pathological condition that had undergone surgery between 2016 and 2021 in Guillermo Almenara Hospital were included. Student's t test and chi square methods were used. patients were divided into one of the five aforementioned groups. Results : Thirty-one patients were included in the study; their mean age was 51.16 years. The most frequent upper cervical pathological condition was trauma-related, with 35.48%. Motor deficit occurred in 51.61% of all patients, and sensitive deficit occurred in 54.84%. The most frequently surgical procedure performed was upper cervical fixation, in 43.89% of all patients. Complication rate was 16.13%, and mortality was 0%. Conclusions : Upper cervical pathological conditions are rare, trauma-related conditions are most frequent, but timely and adequate management allow us to achieve better functional prognosis for these patients.

Ligadura temporal laparoscópica de arterias uterinas en un caso de vasculatura miometrial aumentada relacionada con el embarazo / Laparoscopic temporary uterine artery ligation in a case of pregnancy-related increased myometrial vasculature

Resumen ANTECEDENTES: La vasculatura miometrial aumentada es una afección poco común, con alto riesgo de hemorragia masiva. Su fisiopatología se relaciona con una remodelación inadecuada del endometrio y miometrio, posterior a un evento obstétrico. El tratamiento convencional, en caso de sangrado masivo, es la histerectomía. En la actualidad, los tratamientos con enfoque conservador que permiten el embarazo espontáneo, ofrecen una opción segura para estas pacientes. CASO CLÍNICO: Paciente de 20 años, primigesta, con deseo gestacional a futuro, llevada a la sala de urgencias debido a una hemorragia uterina profusa, con datos clínicos de bajo gasto, antecedente de aborto completo de 10 semanas de gestación un mes antes. En el ultrasonido Doppler se observó una imagen anecoica irregular en el fondo uterino que interrumpía la interfase endometrio-miometrial asociada con flujo sistólico alto. Para el control vascular se indicó cirugía conservadora de útero, con ligadura temporal laparoscópica de las arterias uterinas; además aspiración uterina. Estos procedimientos trascurrieron sin complicaciones. El reporte histopatológico del material aspirado fue de tejido trofoblástico asociado con ectasia vascular. CONCLUSIÓN: La ligadura temporal laparoscópica de las arterias uterinas es un procedimiento eficaz, en casos seleccionados, de control vascular durante la extracción del tejido trofoblástico remanente, en casos de vasculatura miometrial aumentada, relacionada con el embarazo, con recuperación completa de la irrigación uterina y preservación del útero.

Abstract BACKGROUND: Enlarged myometrial vasculature is a rare condition with a high risk of massive haemorrhage. Its pathophysiology is related to inadequate remodelling of the endometrium and myometrium following an obstetric event. The conventional treatment for massive haemorrhage is hysterectomy. Currently, conservative management approaches that allow spontaneous pregnancy offer a safe option for these patients. CLINICAL CASE: 20-year-old primigravida with future pregnancy aspirations, presented to the emergency department with heavy uterine bleeding, clinical data of low output, history of complete abortion at 10 weeks' gestation one month earlier. Doppler ultrasound showed an irregular anechoic image in the uterine fundus interrupting the endometrial-myometrial interface associated with high systolic flow. For vascular control, uterine-sparing surgery with laparoscopic temporary ligation of the uterine arteries and uterine aspiration was indicated. These procedures were performed without complications. The histopathological report of the aspirated tissue was trophoblastic tissue associated with vascular ectasia. CONCLUSION: Temporary laparoscopic ligation of the uterine arteries is an effective procedure in selected cases for vascular control during removal of the remaining trophoblastic tissue, in cases of pregnancy-related increased myometrial vasculature, with complete recovery of uterine irrigation and preservation of the uterus.

Malformación arteriovenosa uterina como causa de hemorragia puerperal tardía / Uterine arteriovenous malformation as a cause of late puerperal hemorrhage

Resumen ANTECEDENTES: Las malformaciones arteriovenosas uterinas constituyen una causa infrecuente de hemorragia puerperal, en la bibliografía solo se encontraron 150 casos. Su conocimiento es importante porque son potencialmente mortales y porque se ha registrado un aumento en los últimos años. Suelen estar relacionadas con intervenciones en el útero: legrados, cesáreas o miomectomías o asociadas con neoplasias (enfermedad trofoblástica gestacional o adenocarcinoma endometrial), aunque otras son congénitas. CASO CLÍNICO: Paciente de 32 años, con una conización cervical y ablación de un mioma uterino mediante radiofrecuencia como únicos antecedentes de interés. A los 15 días posteriores a un parto eutócico, que fue el primero en su historial, tuvo un episodio de abundante metrorragia. En el puerperio inmediato tuvo una hemorragia abundante que requirió la transfusión de dos concentrados de hematíes. En la ecografía transvaginal el útero se observó de 22 x 44 mm, que podría corresponder a un cotiledón retenido. Ante la persistencia del sangrado se colocó un taponamiento intracavitario con una sonda de Foley con lo que se consiguió el cese del sangrado. Luego de descartar la embolización de las arterias uterinas debido al gran tamaño de la malformación arteriovenosa, se procedió a la histerectomía total simple por vía laparotómica. CONCLUSIÓN: Las malformaciones arteriovenosas uterinas, aunque son una causa infrecuente de hemorragia puerperal, deben ser sospechadas en virtud de ser potencialmente mortales.

Abstract BACKGROUND: Uterine arteriovenous malformations are a rare cause of puerperal hemorrhage, with only 150 cases reported in the literature. Their knowledge is important because they are life-threatening and because there has been an increase in recent years. They are usually related to interventions in the uterus: curettage, caesarean section or myomectomy or associated with neoplasms (gestational trophoblastic disease or endometrial adenocarcinoma), although others are congenital. CLINICAL CASE: A 32-year-old patient with a cervical conization and ablation of a uterine myoma by radiofrequency as the only history of interest. Fifteen days after a euthecological delivery, which was the first in her history, she had an episode of abundant metrorrhagia. In the immediate postpartum period, she had a heavy hemorrhage that required the transfusion of two red blood cell concentrates. Transvaginal ultrasound showed a uterus measuring 22 x 44 mm, which could correspond to a retained cotyledon. In view of the persistent bleeding, intracavitary tamponade was placed with a Foley catheter, which led to cessation of bleeding. After ruling out embolization of the uterine arteries due to the large size of the arteriovenous malformation, a simple total hysterectomy by laparotomy was performed. CONCLUSION: Uterine arteriovenous malformations, although an infrequent cause of puerperal hemorrhage, should be suspected because they are potentially fatal.

Juvenile scoliosis as the first manifestation of syringomyelia associated with Chiari malformation type I / Escoliosis juvenil como primera manifestación de siringomielia asociada con malformación de Chiari tipo I

Abstract Background: Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26% of scoliosis initially classified as idiopathic show neurological alterations when studied with nuclear magnetic resonance, such as syringomyelia and Chiari malformation, among the most frequent. Chiari malformations are characterized by descending herniation of the cerebellar tonsils, brainstem, and IV ventricle into the spinal canal. Case report: We present the case of a patient whose first symptomatic manifestation was early-onset scoliosis. The overlapping of some physical examination signs, such as postural lateralization and scoliosis, reinforced the active suspicion of neuroaxis alterations. Conclusions: Early childhood-onset scoliosis should raise a high degree of suspicion for association with neuro-spinal diseases. Although the frequency of Chiari malformation is low, its early approach could reduce the progression of associated comorbidities. An early detection could change the prognosis of the disease.

Resumen Introducción: La escoliosis es una deformidad de la columna que usualmente se manifiesta como una curva estructural que determina una deformidad variable del tronco. Las malformaciones de Chiari se caracterizan por la herniación descendente de las amígdalas cerebelosas, tronco encefálico y IV ventrículo hacia el canal espinal. Según algunas series publicadas, del 4 al 26% de las escoliosis inicialmente catalogadas como idiopáticas muestran alteraciones neurológicas al ser estudiadas con resonancia magnética nuclear, como la siringomielia y malformación de Chiari, dentro de las más frecuentes. Caso clínico: Se presenta el caso de un paciente cuya primera manifestación sintomática fue escoliosis de comienzo temprano. El solapamiento de algunos signos del examen físico, como la lateralización de la postura y la escoliosis, reforzaron la sospecha activa de alteraciones del neuroeje. Conclusiones: La escoliosis de inicio temprano en la infancia debe despertar un alto grado de sospecha de asociación con enfermedades neuro-espinales. Dentro de este contexto, aunque malformación Chiari es de baja frecuencia, su abordaje precoz permitiría disminuir la progresión de comorbilidades asociadas. La detección temprana podría cambiar el pronóstico de la enfermedad.