Meckel’s diverticulum is the most common congenital gastrointestinal malformation and is caused by partial or total involution of the omphalomesenteric duct during embryonic development, resulting in a true diverticulum, since it contains all the layers of the small intestine. It has an incidence of 0.6-4% in the general population and is the main cause of lower gastrointestinal bleeding in pediatric patients. Most of them remain asymptomatic until adulthood, and may present clinical symptoms suggestive of acute appendicitis, intestinal obstruction and lower gastrointestinal bleeding. The finding of the diverticulum is usually incidental and only 4-6% of the time it is found as a directed study of this abnormality. There are multiple diagnostic studies available, including computed tomography (CT), Technetium-99m pertechnetate (Tc-99m) scintigraphy, video capsule endoscopy, and laparoscopy/laparotomy. The management of Meckel’s diverticulum is surgical, especially due to its multiple complications, which are detailed in this article. The use of minimally invasive techniques and the best technologies and detection methods have made it possible to reduce the mortality of Meckel’s diverticulum complications. Finally, this pathology is a challenge for any specialist, since the low incidence and symptoms similar to diverticular diseases pose a greater challenge both in diagnosis and management.

Resumen El divertículo de Meckel es la malformación gastrointestinal congènita más frecuente y se produce por la involución parcial o total del conducto onfalomesentérico durante el desarrollo embrionario, resultando en un divertículo verdadero, puesto que contiene todas las capas del intestino delgado. Posee una incidencia de 0,6-4% en la población general y corresponde a la principal causa de hemorragia digestiva baja en pacientes pediátricos. Mayoritariamente, permanece asintomático hasta la adultez, pudiendo presentar cuadros clínicos sugerentes de apendicitis aguda, obstrucción intestinal y hemorragia digestiva baja. El hallazgo del divertículo suele ser incidental y en solo 4-6% de las ocasiones se encuentra como estudio dirigido de esta anormalidad. Existen múltiples estudios diagnósticos disponibles, pasando por la tomografía computarizada (TC), cintigrafía con pertecnetato de Tecnecio-99m (Tc-99m), videocápsula endoscópica y la laparoscopía/laparotomía. El manejo del divertículo de Meckel es quirúrgico, especialmente, debido a sus múltiples complicaciones, siendo detalladas en el presente artículo. El uso de técnicas mínimamente invasivas y las mejores tecnologías y métodos de detección han permitido disminuir la mortalidad de las complicaciones del divertículo de Meckel. Finalmente, esta patología supone un reto para cualquier especialista, puesto que la baja incidencia y el cuadro clínico similar a enfermedades diverticulares significa un mayor reto tanto en el diagnóstico como en el manejo.

ABSTRACT This review aimed to provide an update on the morphological and/or functional abnormalities related to congenital Zika virus (ZIKV) infection, based on primary data from studies conducted in Brazil since 2015. During the epidemic years (2015-2016), case series and pediatric cohort studies described several birth defects, including severe and/or disproportionate microcephaly, cranial bone overlap, skull collapse, congenital contractures (arthrogryposis and/or clubfoot), and visual and hearing abnormalities, as part of the spectrum of Congenital Zika Syndrome (CZS). Brain imaging abnormalities, mainly cortical atrophy, ventriculomegaly, and calcifications, serve as structural markers of CZS severity. Most case series and cohorts of microcephaly have reported the co-occurrence of epilepsy, dysphagia, orthopedic deformities, motor function impairment, cerebral palsy, and urological impairment. A previous large meta-analysis conducted in Brazil revealed that a confirmed ZIKV infection during pregnancy was associated with a 4% risk of microcephaly. Additionally, one-third of children showed at least one abnormality, predominantly identified in isolation. Studies examining antenatally ZIKV-exposed children without detectable abnormalities at birth reported conflicting neurodevelopmental results. Therefore, long-term follow-up studies involving pediatric cohorts with appropriate control groups are needed to address this knowledge gap. We recognize the crucial role of a national network of scientists collaborating with international research institutions in understanding the lifelong consequences of congenital ZIKV infection. Additionally, we highlight the need to provide sustainable resources for research and development to reduce the risk of future Zika outbreaks. andor or (ZIKV 2015 20152016, 20152016 2016 , (2015-2016) defects overlap collapse arthrogryposis clubfoot, clubfoot clubfoot) CZS. . (CZS) atrophy ventriculomegaly calcifications severity cooccurrence co occurrence epilepsy dysphagia deformities impairment palsy metaanalysis meta analysis 4 Additionally onethird third abnormality isolation ZIKVexposed exposed results Therefore longterm long term followup follow up gap outbreaks 201 2015201 (2015-2016 (CZS 20 201520 (2015-201 2 20152 (2015-20 (2015-2 (2015- (2015 (201 (20 (2 (

ABSTRACT The presence of fragmented QRS complexes (fQRS) on a routine 12-lead electrocardiogram (ECG) is an interesting marker of depolarization abnormality. There is compelling data suggesting that fQRS represents a conduction delay from inhomogeneous activation of the ventricles due to myocardial scar. However, fQRS is not specific for coronary artery disease and it is also found in other myocardial diseases such as cardiomyopathy and congenital heart disease. fQRS has also been described in other entities such as arrhythmogenic right ventricular cardiomyopathy and Brugada syndrome. The importance and predictive value of the fQRS complex as an ECG marker of cardiovascular events appear to be different in different entities. In patients with stable coronary artery disease and in patients with acute myocardial infarction, fQRS appears to be a good predictor of cardiac events. In patients with nonischemic cardiomyopathy, narrow fQRS appears to correlate with the degree of fibrosis and dyssynchrony, and could influence the response to cardiac resynchronization therapy. In patients with LV dysfunction, there is no clear evidence that the presence of fQRS can predict arrhythmic events. On the other hand, there was a statistically significant difference in mortality associated with the presence of fQRS in patients with acute coronary syndrome and myocardial necrosis. The presence of fQRS has a high specificity and a high positive predictive value for the existence of segmental disorders of myocardial motility in patients with ischemic heart disease.

RESUMEN La presencia de complejos QRS fragmentados (fQRS) en un electrocardiograma (ECG) de 12 derivaciones de rutina es un marcador interesante de anormalidad de la despolarización. El fQRS representa un retraso en la conducción debido a una activación no homogénea de los ventrículos debido a una cicatriz miocárdica. Sin embargo, la fQRS no es específica de la enfermedad de las arterias coronarias y además se encuentra en otras enfermedades del miocardio como la miocardiopatía y la cardiopatía congénita. fQRS también se há descrito en otras entidades como la miocardiopatía arritmogénica del ventrículo derecho y el síndrome de Brugada. La importancia y el valor predictivo del complejo fQRS como marcador ECG de eventos cardiovasculares parecen ser diferentes en diferentes entidades. En pacientes con enfermedad arterial coronaria estable y en pacientes con infarto de miocardio agudo, la fQRS parece ser un buen predictor de eventos cardíacos. En pacientes con miocardiopatía no isquémica, la fQRS estrecho parece correlacionarse con el grado de fibrosis y disincronía, y podría influir en la respuesta a la terapia de resincronización cardíaca. En pacientes con disfunción del VI, no hay evidencia clara de que la presencia de fQRS pueda predecir eventos arrítmicos. Por otro lado, hubo diferencia estadísticamente significativa en la mortalidad asociada a la presencia de fQRS en pacientes con síndrome coronario agudo y necrosis miocárdica. La presencia de fQRS, posee una alta especificidad y un alto valor predictivo positivo debido a la existencia de trastornos segmentarios de la motilidad miocárdica en pacientes con cardiopatía isquémica.

Resumo: A estenose laríngea é uma das causas mais comuns de obstrução das vias aéreas superiores em pediatria. A estenose laríngea congênita é a terceira anomalia laríngea congênita mais comum, depois da laringomalácia e da paralisia cordal. O diagnóstico é baseado nos sintomas clínicos do paciente, avaliação radiológica, laringoscopia flexível e endoscopia rígida das vias aéreas sob anestesia geral. Descrevemos um caso de estenose laríngea congênita de apresentação atípica com pólipo mediano, predominando disfonia e não obstrução respiratória.

Resumen: La estenosis laríngea es una de las causas más habituales de obstrucción de la vía aérea superior en pediatría. La estenosis laríngea congénita es la tercera anormalidad congénita laríngea más frecuente, luego de la laringomalacia y la parálisis cordal. El diagnóstico se sustenta en la clínica del paciente, la evaluación radiológica, la laringoscopía flexible y la endoscopía rígida de la vía aérea bajo anestesia general. Describimos un caso de estenosis laríngea congénita de presentación atípica con un pólipo en línea media, predominando la disfonía en lugar de la obstrucción respiratoria.

Summary: Laryngeal stenosis is one of the most frequent causes of airway obstruction in pediatrics. Congenital laryngeal stenosis is the third most common laryngeal congenital abnormality, after laryngomalacia and cord paralysis. The diagnosis is based on the patient’s symptoms, radiological evaluation, flexible laryngoscopy and rigid airway endoscopy under general anesthesia. We describe a case of congenital laryngeal stenosis with atypical presentation with a midline polyp, predominating dysphonia instead of respiratory obstruction.

ABSTRACT Thyroid storm is a critical and infrequent state that conditions the dysfunction of multiple organs due to the effect of excess thyroid hormones. This endocrine dysfunction has a high mortality and generates typical manifestations such as tachycardia, fever, gastrointestinal, cardiovascular and heart disorders, and the central nervous system. Pregnancy has been associated with an increased incidence of arrhythmias. They need immediate treatment with antiarrhythmic drugs, electrical cardioversion, or emergency caesarean section. WPW is a congenital cardiac abnormality that consists of the presence of an abnormal bundle (Kent bundle) that prevents the normal conduction system, directly joining the atria and ventricles. We will see the case of a 32-week pregnant woman who presented symptoms of thyroid storm and multiple episodes of paroxysmal supraventricular tachycardia (PST), with a torpid clinical course mediated by an abnormal intermittent Kent bundle. It is evident that the thyroid storm in the context of pregnancy produced changes in the electrophysiological properties of the intermittent Kent bundle, which led to the development of multiple PST refractory to electrical and pharmacological cardioversion. Moreover, it also did not improve with total thyroidectomy, only resolved completely with radiofrequency catheter ablation of the Kent bundle.

RESUMEN La tormenta tiroidea es un estado crítico y poco frecuente que condiciona la disfunción de múltiples órganos por el efecto del exceso de las hormonas tiroideas, esta disfunción endócrina tiene una elevada mortalidad y genera manifestaciones típicas como la taquicardia, fiebre, alteraciones gastrointestinales, cardiovasculares y del sistema nervioso central. El embarazo se ha asociado con un incremento en la incidencia de arritmias. Necesitan un tratamiento inmediato con drogas antiarrítmicas, cardioversión eléctrica o cesárea de urgencia. El WPW es una anormalidad cardiaca congénita que consiste en la presencia de un haz anómalo (Haz de Kent) que evita el sistema normal de conducción uniendo directamente aurículas y ventrículos. Veremos el caso de una gestante de 32 semanas que presenta un cuadro de tormenta tiroidea y múltiples episodios de taquicardia paroxística supraventricular (TPS), de tórpida y sombría evolución clínica mediada por un haz anómalo de Kent intermitente. Es evidente que la tormenta tiroidea en el contexto de la gestación produjo cambios en las propiedades electrofisiológicas del haz anómalo de Kent intermitente lo cual propició el desarrollo de múltiples taquicardias paroxísticas supraventriculares refractarias a la cardioversión eléctrica y farmacológica. Tampoco mejoró con la tiroidectomía total, solamente cedió por completo con la ablación por catéter de radiofrecuencia del haz anómalo de Kent.

Historically, knowledge regarding congenital skeletal malformations in canines is poor. The Nomina Embryologica Veterinaria does not currently list any dysmorphia related to distal femoral fusion, and there is a significant absence of comprehensive descriptions of congenital defects in the veterinary literature. This paper reports on the radiographic and computed tomography (CT) characteristics of a skeletal abnormality in a seven-month-old crossbreed dog that presented for chronic right pelvic limb lameness as a result of secondary developmental patella luxation. Successful resolution of the lameness was achieved through concurrent surgical correction of the patella luxation and distal femoral cleft.

Resumo Introdução: O quilotórax congénito consiste na acumulação de linfa no espaço pleural. O objetivo deste estudo foi analisar os casos de recém-nascidos com diagnóstico de quilotórax congénito admitidos numa unidade de cuidados intensivos neonatais de nível III entre janeiro de 2014 e dezembro de 2020. Resultados: Foram identificados oito casos de quilotórax congénito, todos com diagnóstico pré-natal. Após confirmação pós-natal do diagnóstico, foi iniciado tratamento de suporte e conservador. Seis casos foram submetidos a ventilação mecânica invasiva, dois foram tratados com surfactante e óxido nítrico inalado, quatro tiveram necessidade de suporte inotrópico e um recebeu oxigenação por membrana extracorporal. Cinco casos foram efetivamente tratados com octreótido. Um recém-nascido com hidrópsia fetal faleceu após o nascimento. Discussão: O prognóstico do quilotórax congénito depende da gravidade do derrame pleural e das anomalias associadas. O tratamento inicial deve ser de suporte e conservador. Embora a utilização de octreótido não seja consensual, demonstrou ser uma terapêutica efetiva e segura nesta amostra de doentes.

Abstract Introduction: Congenital chylothorax (CC) is the accumulation of lymph in the pleural space. The aim of this study was to review the cases of CC diagnosed in newborns admitted to a level III neonatal intensive care unit between January 2014 and December 2020. Results: Eight cases of CC were identified, all with prenatal diagnosis. After postnatal confirmation of the diagnosis, supportive care and conservative therapy were initiated. Six cases underwent mechanical ventilation, two were treated with surfactant and inhaled nitric oxide, four required inotropic support, and one underwent extracorporeal membrane oxygenation. Five newborns were effectively treated with octreotide. One patient with hydrops fetalis died after birth. Discussion: The prognosis of CC depends on the severity of the effusion and on associated abnormalities. Medical treatment is based on supportive and conservative treatment. Although the use of octreotide is not unanimous, it proved to be an effective and safe adjuvant therapy in these patients.

Resumo A regurgitação congênita da valva atrioventricular direita é definida como displasia tricúspide. A alteração ocorre nos folhetos valvares septais e murais, nas cordas tendíneas ou nos músculos papilares. A malformação causa regurgitação tricúspide e apresentação de sinais clínicos associados à insuficiência cardíaca direita congestiva. As lesões macroscópicas incluem nodulação localizada ou difusa ou espessamento dos folhetos valvares, cordas tendíneas curtas ou alongadas, com desenvolvimento incompleto e fusão ou união parcial, músculos papilares com inserção anormal ou separação incompleta da parede do ventrículo direito ou ausência de um ou mais músculos. O objetivo deste trabalho é fornecer informações e casuística para fins de diagnóstico de uma patologia de baixa prevalência em animais de companhia. Entre 2011 e 2021, foram tratados 24 cães com diagnóstico definitivo de displasia tricúspide. A patologia foi mais frequente em cães de pequeno a médio porte, Poodle (4), Dachshund (3), Bulldog Francês (3), Yorkshire Terrier (2), Bull Terrier (1), Boxer (1) e 10 mestiços , 3 com menos de 12 kg e 7 entre 12 e 30 kg de peso, de tamanho médio a grande. Os achados são diferentes, raça e estatura, aos observados por outros autores. Espessamento nodular associado a cordoalhas tendíneas curtas foi a malformação valvar mais comum. A displasia tricúspide esteve associada a metade (13 de 24) dos casos com estenose pulmonar. Outras anomalias congênitas, defeitos do septo interventricular e interatrial, também foram relatadas. O diagnóstico preciso da anormalidade congênita no aparelho da valva tricúspide só pode ser feito pela ecocardiografia. As alterações valvares, do folheto septal, caracterizadas por nodulação, espessamento e união ao septo interventricular, foram as mais comuns. A exploração contínua com Doppler espectral permitiu determinar a gravidade, as pressões nas câmaras atriais e ventriculares direitas e estabelecer os efeitos hemodinâmicos do processo e suas implicações no prognóstico e tratamento do paciente.

Resumen La insuficiencia congénita de la válvula auriculoventricular derecha se define como displasia tricuspídea. La alteración se presenta en hojuelas valvares septal y mural, en cuerdas tendinosas o en músculos papilares. La malformación provoca regurgitación tricuspídea y presentación de signos clínicos asociados a insuficiencia cardíaca derecha congestiva. Las lesiones macroscópicas incluyen nodulación o engrosamiento localizado o difuso de hojuelas valvares, cuerdas tendinosas cortas o alargadas, con desarrollo incompleto y fusión o unión parcial, músculos papilares con inserción anormal o separación incompleta a la pared ventricular derecha o ausencia de uno o más músculos. El objetivo del presente trabajo es aportar información y casuística con fines diagnósticos de una patología de baja prevalencia en animales de compañía. Entre 2011 y 2021 se atendieron 24 caninos con diagnóstico definitivo de displasia tricuspídea. La patología fue más frecuente en perros de talla pequeña a mediana, Caniche (4), Dachshund (3), Bulldog francés (3), Yorkshire terrier (2), Bull terrier (1), Bóxer (1) y 10 mestizos, 3 menores a 12 kg y 7 entre 12 y 30 kg de peso, de talla mediana a grande. Los hallazgos son distintos, raza y talla, a los observados por otros autores. El engrosamiento nodular asociado a cuerdas tendinosas cortas fue la malformación valvar más común. La displasia tricuspídea se asoció con la mitad (13 de 24) de los casos con estenosis pulmonar. Otras anomalías congénitas, comunicación interventricular e interauricular, también se presentaron relacionadas. El diagnóstico preciso de la anormalidad congénita sobre el aparato valvular tricuspídeo sólo puede ser realizado por ecocardiografía. Las alteraciones valvares, hojuela septal, caracterizados por nodulación, engrosamiento y unión al septum interventricular, fueron las más habituales. La exploración doppler espectral continuo permitió determinar gravedad, presiones en cámaras atrial y ventricular derecha y establecer efectos hemodinámicos del proceso y su implicancia en el pronóstico y tratamiento del paciente.

Abstract Congenital insufficiency of the right atrioventricular valve is defined as tricuspid dysplasia. The alteration occurs in septal and mural valve leaflets, in chordae tendineae or in papillary muscles. The malformation causes tricuspid regurgitation and presentation of clinical signs associated with congestive right heart failure. Macroscopic lesions include localized or diffuse nodulation or thickening of valves leaflets, short or elongated chordae tendineae, with incomplete development and partial fusion or union, papillary muscles with abnormal attachment or incomplete separation to the right ventricular wall, or absence of one or more muscles. The aim of this work is to provide information and casuistry for diagnostic purposes of a pathology of low prevalence in companion animals. Between 2011 and 2021, 24 canines with a definitive diagnosis of tricuspid dysplasia were treated. The pathology was more frequent in dogs of small to medium size, Poodle (4), Dachshund (3), French Bulldog (3), Yorkshire terrier (2), Bull terrier (1), Boxer (1) and 10 mongrels, 3 under 12 kg and 7 between 12 and 30 kg in weight, medium to large size. The findings are different, race and size, to those observed by other authors. Nodular thickening associated with short chordae tendineae was the most common valve malformation. Tricuspid dysplasia was associated with half (13 of 24) of the cases with pulmonary stenosis. Other congenital anomalies, interventricular and atrial septal defect, were also related. The precise diagnosis of the congenital abnormality of the tricuspid valve apparatus can only be made by echocardiography. Valve alterations, septal leaflet, characterized by nodulation, thickening and union to the interventricular septum, were the most common. The continuous spectral Doppler exploration allowed determining severity, pressures in the atrial and right ventricular chambers and establishing hemodynamic effects of the process and its implication in the prognosis and treatment of the patient.

ABSTRACT Tailgut cysts are rare congenital lesions that are remnants of the embryonic hindgut. This abnormality presents with non-specific symptoms or no symptoms; therefore, misdiagnosis is common. Here, we present four cases of tailgut cysts that were successfully removed using a robotic surgical approach. A 42-year-old woman with tenesmus, pain in the right gluteal region, and discomfort in the rectal region during evacuation was referred to our medical center. Another patient was a 28-year-old woman who presented with the same symptoms to our general practitioner. Both patients underwent upper abdominal and pelvic magnetic resonance imaging that revealed a tailgut cyst. Further, a 36-year-old woman was referred with coccyx and hypogastric pain. Magnetic resonance imaging revealed two pararectal cystic formations. She underwent robot-assisted surgery, and after analysis by a pathologist, the conclusion was that the tailgut cyst was associated with scarring fibrosis. A 55-year-old woman with posterior epigastric pelvic pain associated with heartburn underwent robot-assisted surgery to resect a retroperitoneal tumor. These cases highlighted the importance of tailgut cysts in the differential diagnosis of rectal lesions. Surgical treatment is preferred because malignant transformations can occur. The difference between laparoscopic and robotic approaches is the better visualization and stability of the latter, inducing less tissue damage. Robotic resection is a safe procedure, especially in patients with a narrow pelvis, because it reduces tissue damage. hindgut nonspecific non specific therefore common Here approach 42yearold yearold 42 year old tenesmus center 28yearold 28 practitioner Further 36yearold 36 formations robotassisted robot assisted pathologist fibrosis 55yearold 55 tumor occur latter damage procedure pelvis 4 2 3 5

Abstract Sinus of Valsalva aneurysm is a rare cardiac abnormality which can be acquired or of congenital origin. A spontaneous rupture into the right atrium is possible and, if not adequately treated, may result in a progressive heart failure due to the left-to-right intracardiac shunt. If ruptured sinus of Valsalva aneurysm is diagnosed, surgical repair is indicated, and different surgical techniques have been reported. If concomitant aortic regurgitation is present, aortic valve replacement is usually performed. Herein, we describe an uncommon clinical presentation of a ruptured sinus of Valsalva aneurysm which has been corrected by aortic valve reimplantation.

Resumen: La malformación congénita de la vía aérea pulmonar (MVAP), antes llamada malformación adenomatoidea quística pulmonar, es una rara anormalidad del desarrollo de las vías respiratorias terminales. Las lesiones son de distribución y tamaños variables, usualmente unilaterales. El diagnóstico puede realizarse desde el período prenatal mediante ecografía gestacional, encontrándose, en ocasiones, graves repercusiones fetales. En los recién nacidos la enfermedad puede manifestarse con dificultad respiratoria aguda. En niños y adultos puede diagnosticarse ante infecciones pulmonares recurrentes u otras complicaciones. En pacientes sintomáticos está indicado el tratamiento quirúrgico para prevenir infecciones y la transformación neoplásica; sin embargo, sigue siendo controversial el tratamiento profiláctico frente al tratamiento expectante en pacientes asintomáticos. Se presenta el caso clínico de una lactante de 2 meses, que en el curso de una bronquiolitis se realizó una radiografía de tórax que evidenció una imagen radiolúcida del lóbulo medio. La tomografía computada visualizó gran imagen quística en pulmón derecho, que podría corresponder a una MVAP. Se decidió tratamiento quirúrgico coordinado. Se realizó una segmentectomía, confirmándose con anatomía patológica una MVAP tipo IV. Evolucionó favorablemente.

Summary: Congenital pulmonary airway malformation (CPAM), formerly called pulmonary cystic adenomatoid malformation, is a rare developmental abnormality of the terminal airways. Lesions are of variable size and distribution, usually unilateral. The diagnosis can be made from the prenatal period by means of gestational ultrasound, occasionally causing serious fetal repercussions. In newborns, the disease may manifest itself through acute respiratory distress. In children and adults it can be diagnosed through recurrent lung infections or other complications. In symptomatic patients, surgical treatment is indicated to prevent infections and neoplastic transformation. However, prophylactic versus expectant management in asymptomatic patients remains controversial. We present the clinical case of a 2-month-old infant who, during the course of bronchiolitis underwent a chest X-ray that revealed a radiolucent image of the middle lobe. The computed tomography scan showed a large cystic image in the right lung, which could be linked to an CPAM. The surgical treatment was carried out and the segmentectomy confirmed a pathological type IV CPAM. The patient evolved favorably.

Resumo: A malformação congênita das vias aéreas pulmonares (MVAP), anteriormente chamada de malformação adenomatoide cística pulmonar, é uma anormalidade rara do desenvolvimento das vias aéreas terminais. As lesões são de tamanho e distribuição variáveis, geralmente unilaterais. O diagnóstico pode ser feito desde o pré-natal por meio da ultrassonografia gestacional, podendo encontrar repercussões fetais graves. Em recém-nascidos, a doença pode se manifestar com dificuldade respiratória aguda. Em crianças e adultos, pode ser diagnosticada com infecções pulmonares recorrentes ou outras complicações. Em pacientes sintomáticos, o tratamento cirúrgico é indicado para prevenir infecções e transformações neoplásicas; no entanto, o tratamento profilático versus expectante em pacientes assintomáticos permanece controverso. Apresentamos o caso clínico de uma criança de 2 meses que foi submetida a radiografia de tórax durante bronquiolite, que revelou imagem radiotransparente do lobo médio. A tomografia computadorizada mostrou grande imagem cística em pulmão direito, que poderia corresponder a uma MVAP. O tratamento cirúrgico foi realizado e a segmentectomia confirmou um tipo IV de MVAP com patologia. A criança evoluiu favoravelmente.

ABSTRACT Introduction: Situs inversus totalis is a rare congenital malformation, characterized by an inverted position of the thoracic and abdominal organs, negatively influencing the diagnostic approach and surgical treatment. Report: Acute appendicitis was diagnosed in a 28-year-old male patient who knew his anatomical abnormality and was corroborated with tomographic images and laparoscopic exploration. He was admitted to the emergency room with 30 hours of abdominal pain with onset in the epigastrium and subsequent radiation to the left iliac fossa. The tomography confirmed his condition of situs inversus totalis and pathological signs in localized appendicitis in FII; The laparoscopic intervention was performed without complications. Discusions: In patients with pain in the left lower quadrant, an adequate differential diagnosis is important and prior knowledge of an anatomical abnormality communicated in a timely manner, that makes feasible an adequate diagnosis and successful surgical resolution.

RESUMEN Introducción: El situs inversus totalis es una malformación congénita rara, caracterizado por una posición invertida de los órganos torácicos y abdominales, influyendo negativamente en la aproximación diagnóstica y en el tratamiento quirúrgico. Reporte: Se diagnosticó apendicitis aguda en un paciente varón de 28 años que conocía su anormalidad anatómica y se corroboró con imágenes tomográficas y la exploración laparoscópica. Ingresó a emergencia con 30 horas de dolor abdominal con inicio en epigastrio y posterior irradiación hacia la fosa iliaca izquierda. La tomografía confirmó su condición de situs inversus totalis y signos patológicos en la apendicitis localizada en FII; se realizó la intervención por laparoscopia sin complicaciones. Discusión: En pacientes con dolor en el cuadrante inferior izquierdo es importante un adecuado diagnóstico diferencial y el conocimiento previo de una anormalidad anatómica comunicada oportunamente hace viable un diagnóstico adecuado y una resolución quirúrgica exitosa.

Congenital high airway obstruction syndrome (CHAOS) is a rare condition that can be diagnosed antenatally by ultrasound. It is usually lethal without immediate intervention at delivery. A 24-year-old woman was diagnosed with fetal CHAOS at 27 weeks' gestation. The couple declined termination of pregnancy. A multidisciplinary team including obstetricians, geneticists, paediatric surgeons, neonatologists and anaesthetists was constituted to plan an ex utero intrapartum treatment (EXIT) procedure. After several simulations, a caesarean section was performed at 38 weeks' gestation under deep inhalational anaesthesia. The fetus was fully delivered with placenta remaining in utero to maintain perfusion. A surgical airway was established via tracheostomy in approximately 5 minutes. The operation was then completed with no maternal complications. The child remains well at 3 years of age. To our knowledge, this is the first EXIT procedure performed for CHAOS in the public sector. This procedure can be lifesaving and is possible with proper planning.

Abstract Heterotaxy syndrome is a rare congenital condition with multifactorial inheritance in which the internal organs are abnormally arranged in the chest and abdomen. It can be classified into two main subcategories: heterotaxy syndrome with polysplenia (left isomerism) and heterotaxy syndrome with asplenia (right isomerism). Such a distinction between these subcategories offers better means of indicating the genetic alterations responsible for the syndrome. Individuals with this condition have complex congenital abnormalities, affecting various organs such as the heart - interferes in blood transport - and lung - altering the number of pulmonary lobes. We report a case of heterotaxy syndrome with polysplenia without abnormality in the thoracic organs in an adult patient.

Resumo A síndrome heterotáxica é uma condição congênita rara com herança multifatorial em que os órgãos internos estão arranjados anormalmente no tórax e abdome, podendo ser classificadas em duas subcategorias principais: síndrome heterotáxica com poliesplenia (isomerismo esquerdo) e síndrome heterotáxica com asplenia (isomerismo direito). Indivíduos com esta condição têm complexas anormalidades congênitas acometendo diversos órgãos, como o coração - dificultando o transporte sanguíneo - e o pulmão - alterando o número de lobos. Relatamos um caso de síndrome heterotáxica com poliesplenia sem anormalidade nos órgãos torácicos em paciente adulta.

Congenital abnormalities of the biliary system are a consideration in children with biliary symptomatology. The preoperative diagnosis rate is still not satisfactory, despite progresses made in imaging technology, with the potential of biliary tract injury if surgery is indicated. The double gallbladder is a rare developmental abnormality of the biliary tract with several anatomical variations. This abnormality was accurately delineated in a 7-year-old child by MRI/MRCP, allowing the ductal anatomy to be accurately identified and safe laparoscopic cholecystectomies to be performed.