Malformaciones müllerianas: actualización y revisión a propósito de casos clínicos, 2022 / Müllerian malformations: update and review of clinical cases, 2022

Las malformaciones müllerianas (MM) son un grupo de anomalías estructurales originadas por fallas de desarrollo de los conductos paramesonéfricos o de Müller durante las primeras 16 semanas de gestación. Un oportuno diagnóstico y una correcta clasificación permiten ofrecer el mejor manejo y diferenciar aquellas pacientes que requieren tratamiento quirúrgico. Se realizó una revisión de la literatura sobre MM en las bases de datos Epistemonikos, SciELO, Cochrane y PubMed. Se rescataron todas las pacientes ingresadas con diagnóstico de MM. En el año 2021, la American Society of Reproductive Medicine publicó un consenso en el que se estandarizó la nomenclatura, se amplió el espectro y se simplificó la clasificación. La clínica es variada, e incluye pacientes asintomáticas cuyo diagnóstico es un hallazgo por imágenes. Los mejores estudios imagenológicos son la resonancia magnética (RM) y la ultrasonografía 3D, dejando la histeroscopia y la laparoscopia (método de referencia) como procedimiento diagnóstico-terapéutico. Se presentan casos clínicos desarrollados durante el primer trimestre de 2022. Recomendamos la utilización sistemática de la RM para el diagnóstico de anomalías complejas u obstructivas del aparato genital. El tratamiento de estas patologías debe ser realizado por ginecólogos endoscopistas expertos, e incluye tratamiento médico y quirúrgico, el cual debe ser enfocado en cada paciente dependiendo del tipo de MM y de los deseos de fertilidad.

Müllerian malformations (MM) are a group of structural anomalies caused by developmental failure of the paramesonephric or Müllerian ducts during the first 16 weeks of gestation. Timely diagnosis and classification allow us to offer the best management and to differentiate those patients who require surgical treatment. Literature review on MM in Epistemonikos, SciELO, Cochrane and PubMed databases. All patients admitted with a diagnosis of MM were rescued. In 2021, the American Society of Reproductive Medicine publishes a consensus where it standardizes the nomenclature, broadens the spectrum, and simplifies the classification. The clinical picture is varied and includes asymptomatic patients whose diagnosis is an imaging finding. The best imaging studies are magnetic resonance imaging (MRI) and 3D ultrasonography, leaving hysteroscopy and laparoscopy (gold standard) as diagnostic therapeutic. Clinical cases developed during the first trimester 2022 are presented. We recommend the routine use of MRI for the diagnosis of complex and/or obstructive anomalies of the genital tract. The treatment of these pathologies should be performed by expert endoscopic gynecologists and include medical and surgical treatment, which should be focused on each patient, depending on the type of MM and fertility desires.

Óbitos pós-cirúrgicos em neonatos com malformações congênitas do aparelho digestivo ou osteomuscular / Post-surgical deaths in neonates with congenital malformations in the digestive or musculoskeletal systems / Muertes post-quirúrgicas en neonatos con malformaciones congénitas digestivas o musculoesqueléticas

Objetivo: Analisar a ocorrência de óbitos pós-cirúrgicos em recém-nascidos com malformação do aparelho digestivo ou osteomuscular em uma maternidade de referência. Método: Estudo exploratório, retrospectivo, de abordagem quantitativa, realizado em uma maternidade de referência localizada em Teresina ­ PI. Os dados foram coletados do Tabwin e de fichas de investigação de óbito infantil de neonatos nascidos em 2016 e 2017 e analisados no software Statistical Package for the Social Sciences. Resultados: O tipo de malformação mais prevalente do aparelho digestivo e osteomuscular entre os neonatos que foram a óbito após cirurgia foi o ânus imperfurado (41%) e a gastrosquise (64,2%), respectivamente. O choque séptico, seguido da insuficiência renal aguda foram os fatores determinantes dos óbitos analisados. Conclusão: O diagnóstico precoce é o fator primordial para redução da morbimortalidade de neonatos acometidos por malformações congênitas, uma vez que contribui para o direcionamento e planejamento dos cuidados imprescindíveis a esses pacientes

Objective: To analyze the occurrence of post-surgical deaths in newborns with malformation in the digestive or musculoskeletal systems in a reference maternity hospital. Method: This is an exploratory and retrospective study, with a quantitative approach, conducted in a reference maternity located in Teresina ­ PI. Data were collected from Tabwin and infant death investigation forms of neonates born in 2016 and 2017 and analyzed through the Statistical Package for the Social Sciences software. Results: The most prevalent type of malformation of the digestive and musculoskeletal systems among neonates who died after surgery was the imperforate anus (41%) and gastroschisis (64.2%), respectively. Septic shock, followed by acute kidney failure, constituted the determining factors of the analyzed deaths. Conclusion: Early diagnosis is the key factor for reducing morbidity and mortality in neonates affected by congenital malformations, as it contributes to the targeting and planning of care actions essential for these patients

Objetivo: Analizar la ocurrencia de muertes post-quirúrgicas en recién nacidos con malformación digestiva o musculoesquelética en una maternidad de referencia. Método: Estudio exploratorio, retrospectivo, con enfoque cuantitativo, realizado en una maternidad de referencia ubicada en Teresina - PI. Los datos se recopilaron de Tabwin y de registros de investigación de muerte infantil de neonatos en 2016 y 2017 y se analizaron utilizando el programa Statistical Package for the Social Sciences. Resultados: El tipo de malformación digestiva y musculoesquelética más frecuente entre los neonatos que murieron después de la cirugía fue el ano imperforado (41%) y la gastrosquisis (64,2%), respectivamente. El shock séptico, seguido de insuficiencia renal aguda, constituyeron los factores determinantes de las muertes analizadas. Conclusión: El diagnóstico temprano es el factor principal para reducir la morbimortalidad en los neonatos afectados por malformaciones congénitas, ya que contribuye a la dirección y planificación de la atención esencial para estos pacientes

Diagnóstico y manejo de Himen Imperforado en una lactante menor / Diagnosis and management of Imperforated Hymen in an infant

INTRODUCCIÓN: El himen imperforado es la malformación congénita más frecuente del tracto genital femenino. La mayoría de los casos no se pesquisan en la infancia, debido a un examen genital insuficiente. Su diagnóstico y resolución deben ser precoces y definitivas para evitar complicaciones posteriores. OBJETIVO: Presentar el caso de una lactante portadora de himen imperforado, y actualizar la información sobre las técnicas de examen genital para detectar esta patología en forma precoz y su tratamiento. CASO CLÍNICO: Lactante de 3 meses, consultó por aumento de volumen protruyente en la zona del introito. En el examen físico se realizó maniobra de valsalva que dio salida a una masa homogénea, nacarada de superficie lisa, entre los labios mayores, sugerente himen imperforado. Se complementó el estudio con ultrasonido ginecológico, el que demostró la presencia de hidrocolpos, descartando otras anomalías. Se realizó una himenotomía, que dio salida a abundante material seroso, sin mal olor, y luego se completó la himenectomía mediante la resección de la membrana himeneal. En control al 4to mes posterior a la intervención se constató un himen ampliamente permeable. CONCLUSIÓN: Los equipos de atención neonatal, requieren capacitación acerca de la técnica correcta del examen genital externo de la recién nacida y lactante. La himenectomía es la técnica que permite resolver definitiva mente el cuadro evitando complicaciones.

INTRODUCTION: Imperforated hymen is the most frequent congenital malformation of the female genital tract. Most cases are not investigated in childhood, due to an insufficient genital examination. Its diagnosis and resolution must be early and definitive to avoid subsequent complications. OBJECTIVE: A clinical case of an infant with imperforated himen is presented. To update on genital examination technique necessary to detect this pathology in the newborn and infants, and the proper treatment. CLINICAL CASE: 3-months-old infant that consulted due to an increase in bulging volume in the introitus area. In the physical examination, the Valsalva's maneuver was performed allowed the ex pulsion of a homogeneous pearly mass with a smooth surface, between the labia majora, suggesting imperforate hymen. The study was complemented with gynecological ultrasound, which demons trated the presence of hydrocolpos, ruling out other anomalies. A hymenotomy was performed, which allowed for the evacuation of abundant serous material, with no bad smell, and then the hymenectomy was completed by resection of the hymenal membrane. In follow-up monitoring 4 months after the intervention, a widely permeable hymen was found. CONCLUSION: Neonatal care teams require training on the correct technique of external genital examination of the newborn and infant. Hymenectomy is the technique that allows definitely resolving the condition, avoiding complications.

Fisura palatina con paladar hendido. Presentación en un neonato / Cleft lip and palate. Presentation in an infant

Se presentó un recién nacido del sexo femenino, hijo de madre de 31 años con antecedentes patológicos referidos de hipertensión arterial y abuela materna con diabetes mellitus. En el examen físico realizado se comprobó la base de labio y paladar hendidos unilaterales izquierdo completos. Fue atendida en el Hospital Provincial Ginecobstétrico "Fe del Valle Ramos", provincia Granma, por un equipo de salud multidisciplinario. Es importante una vez diagnosticada la patología, desarrollar a través de un equipo multidisciplinario acciones que permitan restaurar la adecuada funcionalidad de la vía digestiva, así como todas las problemáticas estéticas y fonética, mejorando de esta manera la calidad de vida del niño, de sus padres y familiares, lo que permitirá al paciente una mejor integración a la sociedad.

A female newborn was presented, son of a 31-year-old mother with a referred pathological history of high blood pressure and grandmother on mother´s side with diabetes mellitus. The physical examination showed a unilateral complete cleft lip and palate in the left side. She was treated at the "Fe del Valle Ramos" Gynecobstetric Hospital in Granma Province, by a multidisciplinary health team. When pathology has been diagnosed, it is very important a multidisciplinary team to develop actions that allow the restoration of the adequate functionality of the digestive tract, as well as all the aesthetic and phonetic problems, using this way to improve the quality of life of the child, his parents and relatives, which will allow the patient a better integration to society.

Criptotia em paciente ocidental adulto: relato de caso / Cryptotia in a Western adult patient: a case report

Criptotia é uma deformidade auricular congênita comum em orientais e rara em ocidentais, sendo a grande maioria dos estudos de técnicas cirúrgicas orientais e aplicados em crianças. Nesta patologia, a cartilagem do polo superior da orelha encontra-se alojada embaixo da pele na região temporal, o que impossibilita o uso de óculos, devido à falta de apoio e torna o polo superior sem definição estética. O presente estudo tem por objetivo relatar o caso de um paciente adulto com criptotia, submetido ao tratamento cirúrgico com retalho de pedículo subcutâneo mastóideo, revisando as principais técnicas descritas para o tratamento deste acometimento. O retalho de pedículo subcutâneo descrito por Yoshimura, mostrou-se adequado para a correção da criptotia em paciente ocidental e adulto.

Cryptotia is a congenital ear deformity common in Easterners and rare in Westerners, with most studies addressing Eastern surgical techniques applied to children. In this pathology, the cartilage of the upper pole of the ear is lodged subcutaneously in the temporal region, which prevents individuals from using glasses due to lack of support and prevents esthetic definition of the upper pole. The present study aimed to report the case of an adult patient with cryptotia undergoing surgical treatment using a mastoid subcutaneous pedicle flap and review the main techniques described for the treatment of this involvement. The subcutaneous pedicle flap described by Yoshimura proved to be adequate for correcting cryptotia in a Western adult patient.

Reporte de serie de casos: neovagina en pacientes con Síndrome Mayer-Rokitansky-Kuster-Hauser / Case series report: neovagina in patients with Mayer-Rokitansky-Kuster-Hauser Syndrome

RESUMEN INTRODUCCIÓN: La ausencia congénita de vagina es una condición poco común, algunas causas son el síndrome de Mayer-Rokitansky-Kuster-Hauser y la insensibilidad periférica a los andrógenos. Múltiples técnicas quirúrgicas y no quirúrgicas se han descrito para el manejo de esta condición, siendo el objetivo principal la creación de un canal vaginal de diámetro y longitud adecuada que permitan restaurar la función coital. El objetivo de este estudio es detallar la experiencia del procedimiento de neovagina con la técnica quirúrgica de McIndoe en pacientes con Mayer-Rokitansky-Kuster-Hauser realizados en la Unidad de Uroginecología de la Clínica Universitaria Bolivariana. METODOLOGÍA: Reporte de 5 casos de pacientes con agenesia de vagina secundarios al síndrome de Mayer-Rokitansky-Kuster-Hauser, a las cuales se les realizó neovagina con la técnica de McIndoe con algunas modificaciones en el molde para el implante de piel. RESULTADOS: Se incluyeron cinco pacientes con diagnóstico de Mayer-Rokitansky-Kuster-Hauser, todas tenían desarrollo de características sexuales secundarias, perfil hormonal normal, y un cariotipo XX. Se utilizó la técnica quirúrgica de McIndoe para la realización de la neovagina sin complicaciones intraoperatorias asociadas y con adecuada evolución posoperatoria, con una longitud vaginal entre 7-9 cm y 3 pacientes con vida sexual activa. El tiempo de estancia hospitalaria fue 7 a 9 días. CONCLUSIÓN: La técnica quirúrgica de McIndoe es una opción para restaurar la función sexual en mujeres con agenesia vaginal con resultados favorables. El tiempo para decidir su realización es electivo, sin embargo, se debe contar con madurez física y emocional para ser llevado a cabo. Las pacientes de nuestro reporte tenían una edad promedio de 18 años.

SUMMARY INTRODUCTION: The congenital absence of the vagina is an uncommon condition, some causes are the Mayer-Rokitansky-Küster-Hauser syndrome and peripheral insensitivity to androgens. Multiple surgical and non-surgical techniques have been described for the management of this condition, being the main objective the creation of a vaginal canal of adequate diameter and length to restore coital function. The objective of this study is to detail the experience of the neovagina procedure with the McIndoe surgical technique performed in patients with Mayer-Rokitansky-Küster-Hauser syndrome at the Clinica Universitaria Bolivariana. METHODOLOGY: Report of five cases of patients with vaginal agenesis secondary to the Mayer-Rokitansky-Kuster-Hauser syndrome, who underwent neovagina with the McIndoe technique and some modifications in the mold for the skin implant. RESULTS: Five patients with diagnosis of Mayer-Rokitansky-Kuster-Hauser were included, all had development of secondary sexual characteristics, normal hormonal profile, and a XX karyotype. The McIndoe surgical technique was used to perform the neovagina without associated intraoperative complications and with adequate postoperative evolution, with a vaginal length between 7-9 cm and three patients with active sexual life. The length of hospital stay was 7 to 9 days. CONCLUSION: The McIndoe surgical technique is an option to restore sexual function in women with vaginal agenesis with favorable results. The time to decide its realization is elective, however, they must have the physical and emotional maturity to be carried out. The patients in our report have an average age of 18 years.

Sequestro pulmonar e tratamento endovascular: um relato de caso / Pulmonary sequestration and endovascular treatment: a case report

O sequestro pulmonar refere-se à anomalia congênita definida por massa de parênquima pulmonar não funcionante. É dividido pela existência de envoltório pleural próprio em intralobar, representando cerca de 75% dos casos, ou extralobar, responsável pelos 25% restantes. O diagnóstico é feito através de radiografia e confirmado com tomografia computadorizada, ressonância nuclear magnética e arteriografia. Tradicionalmente, o tratamento proposto é cirúrgico, mas a técnica endovascular tem apresentado bons resultados. É relatado o caso de uma mulher, 29 anos, com quadro clínico de pneumonias de repetição desde os 5 anos. A tomografia de tórax revelou malformação vascular em região inferior de pulmão direito. O tratamento vascular foi realizado através de embolização do ramo anômalo

Pulmonary sequestration is a congenital anomaly defined as a nonfunctioning mass of lung parenchyma. Presence of an independent pleural envelope classifies it as intralobar, accounting for approximately 75% of the cases, while absence classifies cases as extralobar, accounting for the remaining 25%. Diagnosis is made through radiography and confirmed by computed tomography, magnetic resonance, or angiography. The traditional treatment is open surgical repair, but endovascular techniques have been used, with good results. We report the case of a 29-year-old-woman presenting with recurrent pneumonia for 5 years. A CT scan of the chest revealed poor vascular formation in the lower region of the right lung. The pulmonary sequestration was treated by embolization of the anomalous branch

Relato de caso: síndrome de Klippel-Trénaunay- Weber / Case Report: Klippel-Trénaunay-Weber syndrome

Introdução: A síndrome de Klippel-Trénaunay-Weber (SKTW) é caracterizada pelo conjunto de sinais que consiste em malformações capilares, malformações venosas com ou sem malformações linfáticas associado ao supercrescimento de membros. Na maioria das vezes, envolve apenas uma extremidade com malformação arteriovenosa e cerca de 75% dos pacientes manifestam antes dos 10 anos de idade. Relato de Caso: Relatamos um caso de Klippel-Trénaunay- Weber em um paciente de 7 meses em acompanhamento na enfermaria da Cirurgia Plástica do Hospital de Clínicas da Universidade Federal de Uberlândia para o qual foi proposto tratamento cirúrgico da lesão. Conclusão: Como a SKTW é uma doença com morbidade progressiva e grave, o paciente deve ser acompanhado em um centro de referência com experiência e arsenal terapêutico diversificado para atuar da melhor forma possível no tratamento.

Introduction: The Klippel-Trénaunay-Weber syndrome (KTWS) is characterized by several signs, including capillary malformations and venous malformations with or without lymphatic malformations associated with limb overgrowth. In most cases, only one extremity is involved with arteriovenous malformation, and approximately 75% of the patients manifest symptoms before 10 years of age. Case Report: We report a case of a 7-month-old patient with KTWS followed-up at the Plastic Surgery Service of the Hospital de Clínicas, Federal University of Uberlândia; surgical treatment of the lesion was proposed for the patient. Conclusion: Since KTWS is a progressive disease with severe morbidity, the patient must be followed-up at a reference center by experienced staff with diverse therapeutic arsenal.

Tratamiento endoscópico de una hendidura laríngea posterior: reporte de un caso y revisión de la literatura / Endoscopic treatment of a laryngeal cleft: case report and literature review

RESUMEN Las hendiduras laríngeas posteriores son anomalías congénitas de la laringe de baja incidencia que comprometen la región interaritenoidea o la lámina cricoidea. En caso de extenderse hasta la tráquea son denominados clefts laringo-tráqueo-esofágicos. Su clínica es inespecífica y debe sospecharse en todo niño con trastorno de deglución y neumonía aspirativa a repetición. A continuación, presentamos un caso de un cleft laríngeo tipo 2 tratado endoscópicamente.

ABSTRACT The posterior laryngeal clefts are congenital anomalies of the larynx of low incidence that comprise the interaritenoid region or the cricoid lamina. In case of extending to the trachea they are called laryngo-tracheo-esophagic clefts. Its clinic is non-specific and should be suspected in any child with swallowing disorder and aspiration pneumonia. We present a case of an endoscopically treated laryngeal cleft type 2.

Fissura labiopalatina: avaliação do impacto psicológico utilizando a Escala de Autoestima de Rosenberg / Cleft lip and palate: evaluation of the psychological impact using the Rosenberg self-esteem scale

Introdução: A fissura labiopalatina é a malformação mais frequente da região da cabeça e afeta mais de 10 milhões de pessoas no mundo. O objetivo do estudo foi avaliar a autoestima em pacientes portadores de fissura labiopalatina em acompanhamento no Serviço de Cirurgia Plástica Craniomaxilofacial do Hospital de Clínicas de Porto Alegre, comparando-os com indivíduos não fissurados. Métodos: Estudo transversal contemporâneo, com 160 participantes, sendo 80 pacientes com fissura labiopalatina já submetidos a procedimentos cirúrgicos relacionados à afecção e, como grupo controle, 80 alunos e funcionários da rede pública de ensino. Um questionário para caracterizar o grupo e a escala de autoestima de Rosenberg foram utilizados para a coleta de dados. Resultados: Houve diferença significativa entre os grupos quanto ao estado civil, escolaridade e repetência escolar. Os pacientes com fissura labiopalatina apresentam níveis de autoestima menores em relação a indivíduos não afetados. Dentre eles, os subgrupos dos indivíduos com fissura bilateral, fissura completa, do gênero feminino, classe econômica D/E, baixa escolaridade, situação familiar reconstituída na infância e com resultados não satisfatórios em relação à comunicação, dentição e cicatriz de lábio também mostraram níveis de autoestima menores. Conclusão: Houve relação significativa entre fissura labiopalatina e baixa autoestima.

Introduction: Cleft lip and palate is the most frequent malformation of the head region and affects more than 10 million people worldwide. This study aims at evaluating the selfesteem in patients with cleft lip and palate and comparing that with the self-esteem of non-affected individuals during followup at the Department of Craniomaxillofacial Plastic Surgery of the Hospital de Clínicas of Porto Alegre. Methods: This is a cross-sectional, contemporary study with 160 participants, comprising 80 patients with cleft lip and palate who have already undergone surgical procedures for correcting the condition and 80 non-affected students and employees of the publicschool system as a control group. We used a questionnaire to characterize the group and the Rosenberg self-esteem scale for data collection. Results: There was a significant difference between groups in terms of marital status, schooling, and school retention. Patients with a cleft lip and palate had lower levels of self-esteem than non-affected individuals. Among them, the individuals with bilateral clefts or complete clefts; female gender; economic strata of D/E; low level of schooling; families reconstituted during childhood; and with unsatisfactory results concerning communication, dentition, and lip scar also showed lower levels of self-esteem. Conclusion: There was a significant relationship between cleft lip and palate and low self-esteem.

Mama tuberosa: clasificación y alternativas quirúrgicas en los últimos años / Tuberous breast: classification and surgical alternatives in the last years

La mama tuberosa constituye una anomalía congénita de la mama que afecta a mujeres jóvenes unilateral o bilateralmente, manifestándose en la pubertad con el crecimiento de la mama y presentando un impacto psicológico significativo en las pacientes. Se caracteriza por una deficiencia en las dimensiones horizontal y vertical de la mama, constricción de la base mamaria, hipoplasia parenquimatosa, deficiencia de piel del polo inferior, malposición superior del surco submamario, hernia areolar y asimetría. Debido a la gran variedad de formas de presentación, la mama tuberosa ha sido tratada quirúrgicamente con numerosos tipos de técnicas por diferentes cirujanos a lo largo del tiempo. No se ha llegado a un consenso sobre la mejor técnica quirúrgica. Con una correcta clasificación y evaluación preoperatoria adecuada se puede llegar a un planeamiento quirúrgico más acertado.

Tuberous breast is a congenital anomaly of the breast affecting young women unilaterally or bilaterally, manifesting in puberty with the growth of the breast and presenting a significant psychological impact on patients. It is characterized by a deficiency in the horizontal and vertical dimensions of the breast, contricted base,underdevelopment of the breast, skin deficiency of the lower pole, superiorly malpositioned submammary fold, areolar herniation and asymmetry. Due to the great variety of presentation forms, the tuberous breast has been treated surgically with many types of techniques by different surgeons over time. No consensus has been reached on the best surgical technique. With a correct classification, and adequate preoperative evaluation, it is possible to arrive at a more successful surgical planning.

Ptosis mamaria y su resolución quirúrgica / Breast ptosis and surgical resolution

La ptosis mamaria es una anomalía de la mama relativamente frecuente, que se debe resolver con reconstrucción quirúrgica. Los objetivos de la reconstrucción quirúrgica de esta anomalía son dos: fundamentalmente corregir la posición inestética de la mama, y en segundo plano darle consorte al posible déficit funcional que pueda presentar En general, los déficits funcionales son tratados inmediatamente, mientras que la reparación cosmética puede dividirse en dos momentos vitales del paciente; el final de la pubertad en aquellos casos vinculados al crecimiento excesivo de la glándula y consecuente pérdida de posicionamiento adecuado del CAP para alcanzar la simetría máxima posible después de la maduración completa de la mama con la reducción pertinente de la glándula, o bien según la solicitud del paciente adulto que busca recuperar la imagen estética personal perdida con el curso de los años a partir de la atrofia glandular o elongación excesiva de los tejidos.

Mammary ptosis is a relatively frequent breast anomaly, which must be resolved with surgical reconstruction. The objectives of the surgical reconstruction of this anomaly are two: fundamentally correct the unstatic position of the breast, and in the background give consolation to the possible functional deficit that may present. In general, functional deficits are treated immediately, while cosmetic repair can be divided into two vital moments of the patient; the end of puberty in those cases related to the excessive growth of the gland and consequent loss of adequate positioning of the CAP to reach the maximum possible symmetry after the complete maturation of the breast with the relevant reduction of the gland, or according to the request of the adult patient who seek store cover the lost personal aesthetic image over the years from glandular atrophy or excessive tissue elongation

An unusual presentation of urethral duplication presenting with chronic bladder retention, left scrotal transposition and left renal agenesis

ABSTRACT Introduction and objective Urethral duplication is a rare congenital anomaly, with roughly 200 cases reported in the literature (1). It is more frequent in males, with few cases reported in females. The clinical presentation differs according to the anatomical variant present. The duplication most commonly occurs in the sagittal plane with one urethra located ventrally and the other dorsally (2). Usually the ventral urethra is the more functional of both. Duplications occurring in the coronal plane are quite rare and they are usually associated with bladder duplication (3). The purpose of this paper was to present a video of a boy with an unusual urethral duplication form. Materials and Methods Patient was born premature due to oligohydramnios at 7 months-gestational age and he has initial diagnosis of hypospadia. Since then, he presented at least 7 febrile UTI and mother complained of difficult micturition and a presence of a mass at lower abdomen. Patient was referred to our institution and we identified urethral duplication with a glandar and scrotal meatus, palpable bladder and left penile-hemiscrotum transposition. US and CT-scan showed left kidney agenesis and overdistended bladder. VCUG and retrograde urethrography showed duplication, presence of contrast in the seminal vesicles and complete catheterizing of both urethras was not possible. Results The topic urethra was dysplastic and not patent to a 4Fr plastic tube so we were unable to access it endoscopically. We performed initially a Mitrofanoff procedure to allow CIC and treat chronic retention. Six months later, we assessed both urethras surgically and concluded that dorsal urethra was dysplastic after 3cm still in the penile area and scrotal urethra was not possible to be catheterized. We excised the ventral urethra because of dribbling complaints up to bulbar area and reconstructed the scrotal transposition, keeping the topic urethra for cosmetic issues. Patient had excellent outcome, performs CIC every 4 hours and has not presented further UTI episodes. Discussion and conclusion The urethral duplication is an anomaly that has multiple anatomical presentations. There are several theories about the etiology, but none can explain all types of presentations. There is also more than one rating available, and the Effmann classification is the most detailed. The case exemplifies this varied spectrum of anatomic urethral duplication. It resembles the urethral duplication type IIa-Y, however, ventral urethra meatus was located in penoscrotal area and both urethras were at least partially hypoplastic/dysplastic associated with obstruction and bladder retention. In determining how to best manage a patient with Y-type urethral duplication, the caliber and quality of the orthotopic urethra must first be assessed. Published reports suggest that best outcomes are those using the ventral duplicated urethra for the reconstruction (4). In this case, none of urethras were functional and a supravesical outlet channel had to be provided. The treatment of this condition requires an individualized planning and a vast technical knowledge of reconstructive surgery.

Avaliação da técnica de Millard associada à zetaplastia no tratamento de portadores de fenda labial unilateral / Evaluation of the Millard technique associated with zetaplasty in the treatment of patients with unilateral cleft lip

Introdução: As fendas labiais são deformidades congênitas muito comuns e acometem em grau variável as partes moles e estruturas ósseas do terço médio da face. O tratamento cirúrgico deve ser precoce e segue um protocolo que varia de acordo com o centro de referência. As técnicas cirúrgicas de queiloplastia são inúmeras e, dentre elas, uma das mais utilizadas é a de Millard. O objetivo é avaliar a técnica de Millard tipo I associada a uma zetaplastia da mucosa (técnica empregada pelo autor) como cirurgia de escolha na queiloplastia primária dos pacientes portadores de fenda labial unilateral, entendendo que a técnica é adequada se o número de cirurgias secundárias (reoperações) for baixo. Métodos: Foram operados 65 pacientes por essa técnica no período de janeiro de 2007 a dezembro de 2012 em Santos. Todos acompanhados por no mínimo quatro anos. Resultados: Dos 65 pacientes, 10 (15%) foram considerados "resultados insatisfatórios" e reoperados. Conclusão: Queiloplastia primária à Millard tipo I associada a zetaplastia é adequada, com um número de reoperações baixo e semelhantes aos da literatura atual.

Introduction: Cleft lips are very common congenital deformities that affect, in varying degrees, the soft tissues and bone structures of the middle third of the face. Surgical treatment should be performed early and a protocol must be followed, which varies according to the reference center. There are numerous surgical techniques for lip repair, and among them, the Millard technique is the most used. The objective is to evaluate the association of the Millard type I with zetaplasty mucosal technique (used by the author) as the surgery of choice for primary lip repair in patients with unilateral cleft lip, and to understand whether the techniques are appropriate when the number of second surgeries (reoperation) is low. Methods: Sixty-five patients underwent operations by this technique from January 2007 to December 2012 in Santos, all of whom were followed for at least four years. Results: Of the 65 patients, 10 (15%) were considered to present "unsatisfactory results" and underwent reoperation. Conclusion: Primary cheiloplasty with zetaplasty-associated Millard type I is appropriate when the number of reoperations is low, and our results agree with the current literature.

Tumor fibroso solitario y síndrome Doege-Potter / Solitary fibrous tumor and Doege-Potter syndrome

El tumor fibroso solitario es raro. Si bien su localización más frecuente es la pleura, han sido informados algunos casos de ubicación retroperitoneal. Es difícil diferenciarlo imagenológicamente de otras neoplasias, por lo que casi siempre el diagnóstico es histológico. Dado que los tumores fibrosos solitarios tienen presentaciones clínicas muy diversas, una mejor compresión de la ubicación y sus características imagenológicas ayudaría a abreviar la lista de diagnósticos diferenciales. Comunicamos un tumor fibroso solitario de localización retroperitoneal cuyo diagnóstico fue confirmado por examen histológico.

The solitary fibrous tumor is uncommon. Even though it frequently locates in the pleura, a few cases have been reported in the retroperitoneum. Differentiation from other neoplasms is difficult, and therefore the diagnosis is always attained through histological examination. Although solitary fibrous tumors have variable clinical behaviors, a better comprehension of the location and its imaging characteristics would help to decrease the list of differential diagnoses. We report a solitary fibrous tumor located in the retroperitoneum whose diagnosis was confirmed by histological examination.

Relato de Caso: mama tuberosa / Case report: tuberous breast

Descrever um caso de mama tuberosa com importante assimetria mamária, descrever a técnica e avaliar o resultado de caso. Diferentes técnicas foram usadas em cada mama, apesar de na literatura dizer que é importante empregar uma estratégia semelhante em ambas as mamas. A paciente evoluiu sem complicações e apresenta, com 6 meses de pós-operatório, resultado extremamente satisfatório, sem estigmas de mama tuberosa e com melhora importante da assimetria mamária. Na literatura é estabelecido que não há uma única técnica cirúrgica adequada para a correção dos diferentes tipos de malformações. A mama tuberosa representa um desafio nas cirurgias plásticas da mama e se tornam mais complexas quando a assimetria é mais exacerbada. Porém, o cirurgião que dominar as mais diversas técnicas mamárias estéticas e reconstrutoras poderá alcançar um resultado satisfatório.

To report a case of tuberous breast with significant breast asymmetry, describe the technique used and evaluate the outcome of the case. Different techniques were used on each breast, although studies in literature recommend the use of similar strategy in both breasts. The patient progressed without complications and had, 6 months after the surgery, extremely satisfactory result without tuberous breast stigmas, and significant improvement of breast asymmetry. The literature establishes that not only one surgical technique is adequate to correct different types of malformations. Tuberous breast constitutes a challenge in breast plastic surgery and it becomes more complex when the asymmetry is more severe. However, surgeons who is trained in a variety of aesthetic and breast reconstructive techniques can achieve a satisfactory result.

Impacto na qualidade de vida em pacientes usuários de medicação retroviral e submetidos à gluteoplastia: estudo de coorte histórica / Gluteal contour restoration in antiretroviral users and its quality-of-life related impacts: a historical cohort study

INTRODUÇÃO: Desde a introdução do tratamento do HIV com uso da terapia antirretroviral altamente ativa, a mortalidade por essa doença foi reduzida drasticamente em todo o mundo. Um dos parefeitos relacionados à utilização desses fármacos é a lipodistrofia glútea. O objetivo deste trabalho é verificar o impacto da correção dessa deformidade na qualidade de vida de pacientes com HIV. MÉTODOS: Foi conduzido um estudo de coorte histórica com 23 pacientes submetidos à gluteoplastia com implante intramuscular, entre janeiro de 2010 e dezembro de 2014, avaliando a qualidade de vida por meio do em Nottingham Health Profile em. As informações foram coletadas de julho a agosto de 2015. A análise estatística foi feita utilizando-se o em Related-Samples McNemar Test em. RESULTADOS: strong Houve diferença significativa entre o pré-operatório e pós-operatório em 19 das 38 perguntas. CONCLUSÃO: É possível afirmar que a reconstrução glútea melhora a qualidade de vida de pacientes HIV positivos acometidos por lipodistrofia glútea relacionada a antirretrovirais.

INTRODUCTION: Since the introduction of highly active antiretroviral therapy for the treatment of human immunodeficiency virus (HIV), disease mortality has been dramatically reduced worldwide. One related side effect from the use of these drugs is gluteal lipodystrophy. The aim of this study is to assess the quality-of-life impact of correcting this deformity in HIV patients. METHODS: A historical cohort study was conducted between January 2010 and December 2014 with 23 patients, assessing the quality of their lives using the Nottingham Health Profile. A statistical analysis was performed using the McNemar test for related samples. RESULTS: There was a significant difference between preoperative and postoperative response in 19 of the 38 questions. CONCLUSION: We may say that gluteal reconstruction plays a role in improving quality of life for HIV patients who have been affected by antiretroviral related gluteal lipodystrophy.

Tratamiento quirúrgico de las deformidades en Boutonniere y cuello de cisne del pulgar en la artritis reumatoidea / Traitement chirurgical des déformations du pouce en boutonnière et en col de cygne dans l'arthrite rhumatoïde / Surgical Treatment of Boutonniere and Swan-Neck Deformities in Rheumatoid Arthritis Thumb

Introducción: la artritis reumatoidea es una enfermedad sistémica, en la que son comunes las deformidades articulares, estas se pueden observar en el pulgar donde las de mayor frecuencia son las de Boutonniere y en cuello de cisne. Objetivo: el objetivo principal fue evaluar los resultados de la aplicación de las técnicas quirúrgicas en estas deformidades. Método: se realizó un estudio longitudinal prospectivo de intervención. Se trataron un total de 20 pacientes con este diagnóstico, atendidos en el Servicio de Miembro Superior Minifijación Externa y Microcirugía del Complejo Científico Ortopédico Internacional Frank País en el período comprendido desde enero 2011 a diciembre 2012. La evaluación de los pacientes se realizó según la escala confeccionada para este grupo particular en estudio. Resultados: se obtuvieron resultados excelentes en el 10 por ciento, buenos en el 75 por ciento y regulares en el 15 por ciento de los casos. Conclusiones: las técnicas quirúrgicas empleadas en el tratamiento de esta afección mostraron resultados alentadores(AU)

Introduction: Rheumatoid arthritis is a systemic disease, in which joint deformities are common; these deformities are observed in the thumb where the most frequent are Boutonniere and swan-neck deformities. Objective: Assess the results of the application of surgical techniques in these deformities. Method: A longitudinal prospective intervention study was conducted at Frank País International Orthopedic Scientific Complex from January 2011 to December 2012. Twenty patients with this diagnosis were treated in the Upper Limb Service for External Miniaturization and Microsurgery. The evaluation of the patients was performed according to the scale prepared for this particular study group. Results: 10 of the cases obtained excellent results, 75 percent obtained good results and 15 percent had moderate results. Conclusions: The surgical techniques used in the treatment of this condition showed encouraging results(AU)

Introduction: L'arthrite rhumatoïde est une maladie systémique caractérisée par des atteintes articulaires, surtout au niveau du pouce, telles que les déformations en boutonnière et en col de cygne. Objectif: Évaluer les résultats de l'application des techniques chirurgicales dans le traitement de ces déformations. Méthodes: Une étude longitudinale prospective interventionnelle a été réalisée. Un total de vingt patients, diagnostiqués d'arthrite rhumatoïde, ont été traités au Service d'orthopédie de membres supérieurs, mini-fixation externe et microchirurgie, au Complexe scientifique international d'orthopédie Frank Pais, dans la période comprise entre janvier 2011 et décembre 2012. Les patients de ce groupe ont été évalués selon une échelle élaborée pour cette étude. Résultats: On a obtenu des résultats excellents (10 pourcent), bons (75 pourcent), et passables (15 pourcent). Conclusions: Les techniques chirurgicales utilisées dans le traitement de cette affection ont montré des résultats encourageants(AU)

Manejo da deformidade auricular de Stahl: um relato de caso / Management of Stahl's ear: a case report

Descrita no século XIX, a deformidade auricular de Stahl consiste em uma má formação auricular rara, caracterizada por hipoplasia da raiz da anti-hélice com o alargamento de sua base e uma terceira raiz da anti-hélice conectando-a à parte posterior da hélice, deformando a porção posterossuperior do pavilhão auditivo. A correção cirúrgica é o tratamento definitivo, porém, pela diversidade de apresentações clínicas, não há uma técnica padrão para todos os casos. O método descrito neste relato é mais uma opção de tratamento e consiste na ressecção da terceira cruz e confecção da raiz superior da anti-hélice.

Stahl's ear deformity was first described in the 19th century and it consists of a rare auricular deformity characterized by hypoplasia of the antihelix crus with enlargement of its base and a third crus of the antihelix connected to posterior portion of helix crus, which deforms the posterolateral wall of the external auditory canal. Reconstructive surgery is the definitive treatment, however, because of the diversity of clinical presentations, no standard technique exist for all cases. The method described in this report is another treatment option and entails the resection of the third crus and reconstruction of superior crus of the antihelix.

Avaliação computadorizada digital das medidas auriculares em otoplastias / Digital computerized analysis of auricle measures in otoplasties

Introdução: Nos últimos anos, a literatura vem se enriquecendo de medidas quantitativas, principalmente pelo advento do processamento digital de imagens, procedimento não invasivo e bastante preciso. O objetivo do presente estudo é avaliar as medidas em visão anterior no pré e pós-operatório de pacientes submetidos à otoplastia através de análise computadorizada de fotografias digitais. Métodos: O estudo retrospectivo foi realizado numa série consecutiva de casos no período de fevereiro de 2014 a abril de 2015. Foram realizadas medidas no terço superior, médio e inferior das orelhas. Resultados: Do total de 103 pacientes, 47 eram do sexo masculino e 56 do sexo feminino. A técnica de enfraquecimento do pavilhão, pela confecção de ilhas cartilaginosas múltiplas, associada à sutura com fio inabsorvível incolor, foi utilizada em 56 pacientes; a ressecção de cartilagem conchal isolada em um paciente e a combinação das técnicas anteriormente descritas em 47. O resultado da medida auricular em visão frontal nos pontos previamente descritos, realizada através da análise computadorizada, demonstrou uma redução média importante no terceiro mês de pós-operatório. Conclusão: No presente estudo, demonstrou-se que a avaliação fotográfica digital pode estimar medidas da orelha com alta reprodutibilidade, corroborando o emprego da fotografia digital na estimativa não invasiva dessas variáveis, como opção de custo reduzido e alta disponibilidade na prática clínica. Em conclusão, a análise antropométrica computadorizada em visão frontal, utilizando fotografias digitais pré e pós-operatórias é uma alternativa aos métodos de medidas tradicionais utilizados para avaliação de resultados em otoplastias.

Introduction: Recently a number of studies have been published on quantitative measures mainly for the prominence of digital imaging processing, which is a non-invasive and precisely procedure. This study assessed measures in anterior view before and after surgery of patients who underwent otoplasty using computer analysis of digital images. Methods: A retrospective study was performed on a consecutive series from February 2014 to April 2015. Measures were done in the upper third, middle and bottom of the ears. Results: Of 103 patients, 47 were men and 56 women. We used the technique of weakening the cartilage by making multiple posterior islands associated with non-absorbable suture in 56 patients, an isolated conchal cartilage resection in 1 patient and combination of all techniques in 47 patients. The result of auricle measure in frontal viewing at previous measured points performed using computer analysis showed a significant mean reduction in the third month after surgery. Conclusion: Digital image assessment can estimate auricle measure with high repeatability, corroborating with the use of digital images in noninvasive procedures as less expensive and highly available option in clinical practice. The computed anthropometric analysis of digital images in direct frontal view using images taken before and after surgery is an alternative method for traditional measurements to evaluate results in otoplasties.