Obstrucción intestinal por vólvulo de ciego en paciente con malrotación intestinal asociado a situs ambiguous / Intestinal obstruction due to volvulus of the cecum in a patient with intestinal malrotation associated with situs ambiguous

Introducción. La oclusión intestinal, completa o incompleta, es uno de los cuadros de abdomen agudo más frecuentes. Constituye entre 20 % y 35 % de los ingresos urgentes en las áreas quirúrgicas hospitalarias. Caso clínico. Se presenta el caso de un paciente con antecedente de carcinoma de próstata, que consultó con un cuadro de obstrucción intestinal y abdomen agudo. Se le diagnosticó vólvulo de ciego, mal rotación intestinal y situs ambiguous. El tratamiento quirúrgico del paciente fue exitoso. Conclusión. El conocimiento de estas condiciones patológicas es imprescindible para poder brindarle un correcto tratamiento quirúrgico y disminuir la mortalidad que pueden acarrear

Introduction. Complete or incomplete intestinal obstruction is one of the most frequent acute abdomen conditions. It constitutes between 20% and 35% of the urgent admissions of hospital surgical areas. Clinical case. We present a case of a patient with a history of prostate carcinoma, who began with clinical symptoms of intestinal obstruction and acute abdomen. He was diagnosed with volvulus of the cecum, intestinal malrotation, and situs ambiguous. Surgical treatment of the patient was successful. Conclusion. Knowledge of these pathological conditions is essential to be able to provide correct surgical treatment and reduce the mortality that these can lead to

Kartagener's syndrome: a case report

Kartagener's syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive inherited disease, and is characterized by the triad of chronic sinusitis, bronchiectasis, and situs inversus. This paper reports the case of a 27-year-old female presenting with dyspnea on medium exertion, accompanied by chronic cough, non-productive or with clear expectoration. She had recurrent pneumonia until 15 years of age and underwent a lobectomy in the lower lobe of the left lung, probably due to bronchiectasis. Chest computed tomography showed situs inversus totalis, signs of previous surgical manipulation, and mild bronchial thickening. Computed tomography of the paranasal sinuses showed signs of chronic sinusitis due to a probable ciliary kinesis disorder. These finding suggest the diagnosis of Kartagener's syndrome. The prognosis reveals a slow rate of decline in lung function. However, repeated or chronic infections can negatively influence the quality of life of these patients.

Situs inversus totalis asociado a síndrome de preexcitación ventricular: reporte de un caso / Situs inversus totalis and pre excitation syndrome

Resumen: El situs inversus totalis es la inversión congénita completa de órganos torácicos y abdominales. Se presenta el caso de una paciente de 3 años sin antecedentes médicos previos, a quien en atención primaria, y por un cuadro respiratorio agudo, se evidencia el hallazgo de dextrocardia y burbuja gástrica a derecha en la radiografía de tórax, sospechándose situs inversus totalis. Fue derivada a cardiología infantil donde se confirmó el diagnóstico con un ecocardiograma transtorácico, asociado a un hallazgo de preexcitación ventricular en el electrocardiograma. Una vez resuelto el cuadro respiratorio agudo, la paciente se mantiene controlada de manera periódica en atención primaria y por especialista de manera semestral.

Abstract: Situs inversus totalis is the complete congenital inversion of thoracic and abdominal organs. We present the case of a 3-year-old girl with no previous medical history. When seen with an acute respiratory syndrome, dextrocardia and gastric bubble on the right side led to the diagnosis of Situs inversus. She was referred to infant cardiology where the diagnosis was confirmed with a transthoracic echocardiogram. In addition, the electrocardiogram identified the presence of ventricular preexitation. Once the acute respiratory symptoms subsided, the patient remains controlled periodically in primary care and by a specialist every six months. No episodes of tachycardia have been detected.

Dextrocardia como hallazgo incidental: revisión a partir de un caso / Dextrocardia as incidental finding: review from a case

INTRODUCTION: Dextrocardia is characterized by the positioning of the heart to the right of the thoracic cavity, usually with the apex oriented to the right and its inverse anatomy 1,2. It is a rare congenital pathology that can be associated with other congenital cardiac anomalies, as well as a change in position of all thoracoabdominal structures 1-4. Its diagnosis in adult life is usually incidental. We present the clinical case of a 64-year-old man with an electrocardiogram that showed deviation of the QRS complex axis to the extreme right and a physical examination compatible with dextrocardia, which was later confirmed with images. OBJECTIVES: To review the medical literature related to the diagnosis of dextrocardia through the presentation of a clinical case. METHODS: The clinical data of the case were collected by means of anamnesis, physical examination and specialized exams of the patient, as well as his clinical file. The literary review was made using the MEDLINE® search engine for scientific journals. RESULTS: Dextrocardia affects less than 1% of the general population according to the studies reviewed. Its importance lies in the association with other congenital pathologies such as cardiac and extracardiac structural malformations, which increases morbidity and mortality in this group of patients. It is imperative, when suspected, complete the study with images that allow confirming or ruling out other structural anomalies.

Idiopathic thrombocytopenic purpura in a patient with situs inversus totalis: case report and literature review / Púrpura trombocitopênica idiopática em paciente com situs inversus totalis: relato de caso e revisão da literatura

ABSTRACT Situs inversus totalis is a rare recessive autosomal congenital abnormality in which the mediastinal and abdominal organs are in a mirrored position when compared to the usual topography. The literature reports some cases of situs inversus totalis and concomitant conditions: spinal abnormalities, cardiac malformations and hematological diseases, such as idiopathic thrombocytopenic purpura, which is an autoimmune disease that causes thrombocytopenia due to platelet destruction or suppression of its production. This article aimed to report the coexistence of situs inversus totalis and idiopathic thrombocytopenic purpura.

RESUMO Situs inversus totalis é uma anormalidade congênita autossômica recessiva rara em que os órgãos mediastinais e abdominais encontram-se em posição espelhada em relação à topografia habitual. A literatura relata alguns casos de concomitância do situs inversus totalis com outras condições: anomalias espinhais, malformações cardíacas e doenças hematológicas, como púrpura trombocitopênica idiopática, que é uma doença autoimune com plaquetopenia, devido à destruição dos trombócitos ou supressão da sua produção. Esse artigo teve o objetivo de relatar coexistência de situs inversus totalis e púrpura trombocitopênica idiopática.

Dextrocardia and situs inversus totalis in a Turkish subject: a case report / Dextrocardia y situs inversus totalis en un sujeto turco: reporte de un caso

Dextrocardia with situs inversus is an uncommon anomaly affecting about 1 to 2 per 10,000 in the general population. This report describes an adult male patient with dextrocardia and in a Turkish subject. The photographic illustrations revealed transposition of some of the visceral organs such as the spleen was located right and the liver and gall bladder on the left. The heart was flattened and flipped to the right. Many people with situs inversus totalis are unaware of their unusual anatomy until they seek medical attention for an unrelated condition. So, early detection may lead to a successful surgical management and consequently offer a safer chance of survival. This report showed that dextrocardia and situs inversus can be seen amongst Turkish subjects.

La dextrocardia con situs inversus es una anomalía poco frecuente que afecta aproximadamente de 1 a 2 personas por 10.000 en la población general. Este informe describe un paciente masculino adulto con dextrocardia. Las figuras revelaron que la transposición de algunos de los órganos viscerales, como el bazo, se ubicada a la derecha y el hígado y la vesícula biliar a la izquierda. El corazón fue aplastado y girado hacia la derecha. Muchas personas con situs inversus totalis desconocen su anatomía inusual hasta que buscan atención médica por una afección no relacionada. Por lo tanto, la detección temprana puede llevar a un manejo quirúrgico exitoso y, en consecuencia, ofrecer una posibilidad más segura de supervivencia. Este informe mostró que la dextrocardia y el situs inversus se pueden encontrar entre los sujetos turcos.

Síndrome de Kartagener: diagnóstico neonatal. A propósito de un caso / Kartagener syndrome: neonatal diagnosis. A case report

El síndrome de Kartagener es una enfermedad hereditaria autosómica recesiva caracterizada por la asociación de discinesia ciliar primaria y la tríada situs inversus total, sinusitis crónicas y bronquiectasias. Su prevalencia varía en 1/15 000-1/30 000, pero se estima que muchos pacientes con discinesia ciliar primaria no han sido diagnosticados. Su presentación clínica es inespecífica y heterogénea, y no hay una única prueba gold standard para su diagnóstico. Esto, unido a las limitaciones y no disponibilidad de las pruebas, hace que el diagnóstico se retrase. Un diagnóstico y tratamiento adecuados de forma precoz modifican el pronóstico. En los últimos años, las sociedades han publicado algoritmos diagnósticos para pacientes con clínica sugestiva. Por ello, es importante una puesta al día y enfatizar en la necesidad de una sospecha clínica ante las manifestaciones clínicas de esta enfermedad. Se presenta a un recién nacido con este síndrome diagnosticado por estudio genético en un hospital secundario.

Kartagener Syndrome is an inherited autosomal recessive disorder characterized by primary ciliary dyskinesia and the triad of situs inversus viscerum, chronic sinus disease and bronchiectasis. Its prevalence varies from 1/15 000 to 1/30 000 but it is estimated that a lot of patients with primary ciliary dyskinesia have not been diagnosed as such. Its clinical presentation is non-specific and heterogeneous, and there is not a single, gold standard, diagnostic test. The diagnosis is often delayed because of these reasons and limitations and no availability of diagnostic tests. Early diagnosis and treatment change patient's prognosis. In addition, Scientific Societies have published recent diagnostic algorithm to evaluate the patient with suspected primary ciliary dyskinesia. Therefore, it is important to keep up to date with all the latest articles. We present the case of a newborn with this syndrome diagnosed by genetic analysis in a secondary care hospital.

Manga gástrica laparoscópica en situs inversus total. Reporte de un caso y revisión de la literatura / Laparoscopy sleeve gastrectomy in situs inversus totalis. A case report and literature review

El objetivo de este estudio es revisar una de las opciones quirúrgicas para el tratamiento de la obesidad en pacientes con situs inversus total. Se presenta el caso de una paciente de 39 años de edad con situs inversus total (diagnóstico transquirúrgico) que fue sometida a gastrectomía en manga laparoscópica, con resultados postquirúrgicos óptimos; además, en la revisión de la literatura encontramos 14 casos publicados a nivel mundial de procedimientos quirúrgicos metabólicos.

The objective of this study is to perform a review of one of the surgicaloptions for the treatment of obesity in patients with total situs inversus. We present the case of a female patient of 39 years of age with total situs inversus (trans-surgical diagnosis) and who underwent a laparoscopic sleeve gastrectomy, with optimal postoperative results, in addition a review of the literature is made, finding 14 cases published to global level of metabolic surgical procedures.

Síndrome de Kartagener / Kartagener Syndrome

Introducción: El síndrome de Kartagener es una variación clínica de la discinesia ciliar primaria, se caracteriza por la triada clásica de sinusitis crónica, bronquiectasia y situs inversus (total o parcial), catalogada como enfermedad rara de herencia autosómica recesiva. Objetivo: Analizar las manifestaciones clínicas, análisis complementarios y tratamiento de los pacientes diagnosticados con síndrome de Kartagener en la República del Ecuador. Presentación de caso: Paciente femenina, de nacionalidad ecuatoriana, con manifestaciones clínicas de la tríada del síndrome de Kartagener y rasgo de infertilidad, con antecedente de sinusitis crónica desde 14 años de edad. Los estudios imagenológicos de rayos X de tórax y tomografía axial computarizada de tórax y senos paranasales confirmaron las manifestaciones de síndrome de Kartagener, que representa el séptimo caso reportado en el país. Se analizaronn las características clínicas de la serie de siete casos reportados en el Ecuador hasta el presente, correspondiente al período 2015-2018 y exámenes complementarios realizados para el diagnóstico de certeza y diferencial. Conclusiones: Se presentó el séptimo caso de síndrome de Kartagener diagnosticado en el Ecuador y se analizó la serie de una totalidad de 7 pacientes reportados en el país entre 2015-2018(AU)

Introduction: Kartagener syndrome is a clinical variation of primary ciliary dyskinesia, characterized by the classic triad of chronic sinusitis, bronchiectasis and situs inversus (total or partial), classified as a rare autosomal recessive inheritance disease. Objective: To analyze the clinical manifestations, complementary tests and treatment of patients diagnosed with Kartagener syndrome in the Republic of Ecuador. Case presentation: Female patient, of Ecuadorian nationality, with clinical manifestations of the Kartagener syndrome triad and infertility trait, with a history of chronic sinusitis since 14 years of age. Imaging studies of thorax, x-rays and computed tomography of chest and paranasal sinuses confirmed the manifestations of Kartagener syndrome, which represents the seventh case reported in the country. Respiratory evolution and therapeutic management are exposed. In this context, we analyze the clinical characteristics of the series of seven cases reported in Ecuador up to the present, corresponding to the period 2015-2018 and complementary tests performed for the certainty and differential diagnosis. Conclusions: The seventh case of Kartagener syndrome diagnosed in Ecuador is presented, and the series of a totality of 7 patients reported in the country between 2015-2018 is analyzed(AU)

Casuística. Vesícula biliar siniestra (sinistraposición): Caso clínico / Left-sided Gallbladder (Sinistraposition of the gallbladder)

A rare case of left-sided gallbladder (sinistraposition) is reported with review of the literature. Left-sided gallbladder is very unusual, with a frequency of 0.3% of the cases, being generally associated to situs inversus. The aim of this invesigation was to establish the association between left-sided gallbladder and right-sided round ligaments. Left-sided gallbladder is a rare anomaly and has been classified into two situations: 1) gallbladder migration to the left side, and 2) development of a second gallbladder with atrophy of the original one. Left-sided gallbladder were reported to be associated with right-sided round ligaments.

Kartagener syndrome, current data on a classical disease. case report

ABSTRACT Introduction: This article addresses the general aspects (pathophysiology, embryology, clinical presentation and prognosis) of the Kartagener syndrome (KS). Case presentation: 26-year-old male patient, with a history of complicated sinusitis with cerebral abscess and secondary epilepsy, who consulted to the Hospital Universitario Nacional de Colombia due to headache, fever and mucus expectoration. The presence of situs inversus, chronic sinusitis and bronchiectasis suggested a diagnosis of primary ciliary dyskinesia and KS. Discussion: Differential diagnoses of KS should be framed in its possible causal relationship with primary ciliary dyskinesia and other diagnoses associated with secondary ciliary dysfunction, such as cystic fibrosis, immunodeficiency and anatomical-functional conditions with rhinosenopulmonary involvement. Clinical suspicion of KS occurs when the heart is auscultated on the right and the liver is palpated on the left. Confirmation is achieved through imaging methods that prove visceral heterotaxia, indirect methods related to scan of ciliary malfunction (nasal nitric oxide, video microscopy) and ciliary biopsy that demonstrates the defect of the ciliary ultrastructure. Conclusions: Respiratory infectious involvement in patients with KS is explained by the alteration of the cilia, which leads both to the malposition of some organs and to the structural and functional alteration of others.

RESUMEN Introducción. El presente artículo aborda los aspectos generales (fisiopatología, embriología, presentación clínica y pronóstico) del síndrome de Kartagener (SK). Presentación del caso. Paciente masculino de 26 años, con antecedente de sinusitis complicada con absceso cerebral y epilepsia secundaria, quien consulta al Hospital Universitario Nacional de Colombia por cefalea, fiebre y expectoración mucosa. La presencia de situs inverso, sinusitis crónica y bronquiectasias sugieren diagnóstico de discinesia ciliar primaria y SK. Discusión. Los diagnósticos diferenciales del SK deben enmarcarse en la relación de causalidad posible con la discinesia ciliar primaria y de otros diagnósticos asociados a disfunción ciliar secundaria como fibrosis quística, inmunodeficiencia y condiciones anatómicas-funcionales con compromiso rinosenopulmonar. La sospecha clínica del SK se da cuando se ausculta el corazón a la derecha y se palpa el hígado a la izquierda. Su confirmación es mediante métodos de imagen que comprueban la heterotaxia visceral, por métodos indirectos de mal funcionamiento del barrido ciliar (óxido nítrico nasal, video microscopia) y por biopsia ciliar que demuestra el defecto de la ultraestructura ciliar. Conclusiones. El compromiso infeccioso respiratorio presentado por los pacientes que cursan con SK se explica por la alteración en la cilia, que conlleva tanto a la malposición de algunos órganos como a la alteración estructural y funcional de otros.

Concomitancia de situs inversus totalis y cáncer de colon: Presentación de un caso / Concomitant Situs Inversus Totalis and Colon Cancer: A Case Report

El situs inversus totalis (SIT) es una rara anomalía congénita en la cual las estructuras de la cavidad toracoabdominal se encuentran en una posición completamente opuesta a la usual. Hasta la fecha, se han informado casos de situs inversus concomitantes con diversas neoplasias intraabdominales, pero existen muy pocos relacionados con adenocarcinoma colorrectal. A pesar de esto, el adenocarcinoma colorrectal es una neoplasia frecuente del tracto gastrointestinal con una elevada tasa de morbimortalidad, dado que su detección en la mayoría de los casos se da en etapas tardías. Se presenta el caso de una mujer de 70 años de edad que asiste a control por seguimiento oncológico de neoplasia de sigmoides asociada a situs inversus totalis. Se resaltan los hallazgos imaginológicos y su valor en la estadificación y seguimiento, así como las consideraciones anatómicas posteriores al tratamiento clínico-quirúrgico.

Situs inversus totalis (SIT) is a rare congenital anomaly in which the thoracoabdominal inner organs are placed in a completely opposite position to the usual. Up to date, there have been reported cases of situs inversus totalis concomitant with different intraabdominal neoplasms, but there are only few cases related with colorectal adenocarcinoma. Besides this, colorectal carcinoma is a frequent gastrointestinal neoplasm with a high morbimortality rate due to its detection in advanced stages. We report a case of a 70-year-old woman in oncologic following up with sigmoid adenocarcinoma and situs inversus totalis. We highlight imaging findings and their value in staging and monitoring, as well as anatomic consideration after clinical and/or surgical treatment.

Apendicitis aguda en paciente adulto con rotación intestinal anormal y defecto cardiovascular: presentación de caso y revisión temática / Acute appendicitis in an adult patient with abnormal intestinal rotation and a cardiovascular defect: case presentation and literature review

La apendicitis aguda es la enfermedad quirúrgica que se presenta con mayor frecuencia en los servicios de urgencias. Su diagnóstico es predominantemente clínico; alrededor de dos terceras partes de los pacientes con apendicitis presentan un dolor típico localizado en el cuadrante inferior derecho. Sin embargo, hay manifestaciones atípicas, infrecuentes, como el dolor en el cuadrante inferior izquierdo. Se hace pues, importante conocer la existencia de ciertas enfermedades que puedan explicar estos síntomas para así tenerlas en cuenta en el momento de abordar un paciente con dolor abdominal. Estas son la rotación intestinal anormal y, con mayor frecuencia, el situs inversus, que explican el dolor abdominal secundario a la apendicitis en una localización diferente a la clásica para hacer el diagnóstico y dar tratamiento oportuno, con el fin de prevenir complicaciones. Además, los estudios complementarios son importantes en estos pacientes para descartar otras alteraciones o malformaciones, como defectos cardiovasculares, los que se han relacionado con anomalías en la formación y la rotación gastrointestinal en 27 % de los casos, aproximadamente. Se presenta el caso de hombre joven, con un cuadro clínico de dolor abdominal que se inició en el epigastrio y después se localizó en el cuadrante inferior izquierdo, acompañado de signos de irritación peritoneal y alteración de los exámenes paraclínicos. Los estudios de imagen confirmaron la presencia de rotación intestinal anormal y apendicitis aguda izquierda. Durante el seguimiento del paciente, se practicó una ecocardiografía transesofágica por dolor torácico, la cual demostró insuficiencia valvular aórtica secundaria a valvulopatía aórtica bivalva

Acute appendicitis is the most frequent surgical pathology at the emergency departments and its diagnosis is predominantly clinical. Around 2/3 of the patients with this pathology present typical pain, located in the right lower quadrant. However, abdominal pain in the left lower quadrant secondary to acute appendicitis is of infrequent presentation. It is important to know the existence of certain pathologies that can explain the cause for this entity, to take them into account when examining a patient. These are the intestinal malrotation and, most frequently, situs inversus. Both of them explain the abdominal pain secondary to appendicitis in a different location from the classic presentation, in order to make an accurate diagnosis and treatment so complications can be prevented. Additionally, it is important to perform complementary studies in these patients to discard other alterations or malformations, such as cardiovascular defects, because they are related to defects in the gastrointestinal formation and rotation in approximately 27% of the cases. We present the case of a young male, with abdominal pain that begins in the epigastrium with posterior localization in the left lower quadrant. He presents signs of peritoneal irritation and abnormal laboratory tests. Imaging studies are performed and confirm the presence of left sided acute appendicitis and intestinal malrotation. During the patient's follow-up, a transesophageal echocardiography was performed due to chest pain, which shows aortic valve insufficiency secondary to aortic bivalve valvulopathy

Colecistectomía laparoscópica en situs inversus totalis / Laparoscopic cholecystectomy in situs inversus totalis

Situs inversus totalis es una anomalía congénita poco frecuente. Se caracteriza por la transposición de los órganos tóraco-abdominales en sentido sagital al brindar una imagen llamada en espejo. Reportamos paciente de 59 años con diagnóstico de litiasis vesicular y situs inversus totalis resuelto por colecistectomía videolaparoscópica, en el Hospital Universitario Comandante Manuel Fajardo de La Habana. El cuadro clínico tuvo seis meses de evolución, con dolor a tipo cólico en hipocondrio izquierdo acompañado de náuseas, que se relacionaban con la ingestión de alimentos colecistoquinéticos. Los exámenes de laboratorio estuvieron dentro de parámetros normales. La ecografía abdominal informó litiasis vesicular, así como, el situs inversus totalis que se confirmó con Rayos X de tórax. Su evolución posoperatoria, luego de un año, fue satisfactoria, la cual mostró la factibilidad de la técnica quirúrgica empleada(AU)

Situs inversus totalis (SIT) is a rare congenital anomaly characterized by the transposition of the thoracic-abdominal organs towards the sagittal direction, producing an image called in mirror. The case is presented of a 59-year-old female patient, with a diagnosis of vesicular lithiasis and situs inversus totalis for cholelithiasis, treated by laparoscopic cholecystectomy in Comandante Manuel Fajardo University Hospital of Havana. The patient's clinical picture had a natural history of 6 months, with left colic pain in the left hipocondrium, with nauseas, accentuated on ingestion of greasy meals. The laboratory test results showed normal parameters. X-rays of the abdomen confirmed vesicular cholestasis and situs inversus totalis. The patient's postoperative evolution after one year was satisfactory, showing the feasibility of the surgical technique used(AU)

Laparoscopic hemicolectomy for a patient with situs inversus totalis and colorectal cancer / Colectomia laparóscopica para um paciente com situs inversus totalis e câncer colorretal

ABSTRACT Situs inversus totalis is a congenital anatomic anomaly characterized by a complete inversion of thoracic and abdominal organs. We present a case of a 67 year-old patient diagnosed with situs inversus totals in his childhood who was referred for a two-month history of hematoquezia. Ascending colon cancer where found and he underwent a laparoscopic hemicolectomy with radical lymphadenectomy. An exhaustive preoperative study and a detailed planning of laparoscopic surgery including positions of operator and assistants and trocar sites have been performed to be aware of anatomic challenges. The operating time was 120 min and blood loss was minimal. Histologic examination showed a well-differentiated adenocarcinoma with serosal invasion and without lymph nodes metastasis (pT3N0). The patient was discharged on postoperative 6th day without complications. Laparoscopic surgery for colon cancer in patients with situs inversus totalis could be more difficult nevertheless a safe and feasible procedure should be performed successfully.

RESUMO Situs inversus totalis é uma anomalia anatómica consistindo em um investimento de órgãos abdominais. Nesse estudo, descrevemos um paciente, homem 67 anos, que foi diagnosticado com situs inversus totalis na infância. Apresentava sintomas de sangramento retal e foi diagnosticado com câncer de cólon direito e tratado cirurgicamente com receção laparoscópica. Para a realização da colectomia laparoscopica precisamos de um estudo pré-operatória completa e um plano detalhado de cirurgia com localização do trocateres e cirurgiões. A cirurgia durou 120 minutos e perde de sangue foi mínima. O resultado do exame patológico relatou adenocarcinoma (T3N0). Nosso paciente foi admitido por 7 dias e não apresentaram complicações. Para os pacientes com situs inversus totalis e câncer colorretal a receção laparoscópica pode ser mais difícil mas eficaz e segura.

Situs inversus totalis: revisión de tema con aproximación a la Genética y reporte de casos / Situs inversus totalis, topic review approach to Genetics and case report

Resumen Objetivo: Efectuar una revisión actualizada del situs inversus totalis y presentar casos clínicos. Métodos: Se hizo una revisión de las principales bases de datos y se incluyeron los estudios más relevantes correspondientes a 20 a˜nos previos, en espa˜nol e inglés, en todos los grupos etáreos y étnicos, incluidos: metaanálisis, revisiones sistemáticas, ensayos clínicos, estudios de cohorte y casos clínicos relevantes. Se consultaron bases de datos como EBSCO, Medline, PubMed y SciELO entre otras, para obtener información actualizada. Los términos de búsqueda fueron situs inversus, totalis. Resultados: El situs inversus totalis es una variante anatómica poco frecuente que involucra estructuras toraco-abdominales, hace parte de otras variantes de situs, tiene un patrón de herencia poligénico, sin una relación directa establecida; es además una entidad asintomática, reconocida desde tiempos antiguos. Conclusiones: Aunque es una patología de baja incidencia en la población, y un hallazgo incidental, es importante reconocerla como una variante anatómica que puede cursar con patologías concomitantes. A pesar de que existen estudios, se requieren otros con alta significancia estadística. Se encuentra relación de los casos con la información de la literatura que concluyen un estudio acertado.

Abstract Objective: To conduct an updated review of situs inversus totalis and present clinical cases. Methods: A review of the main databases was conducted, and the most relevant studies over the last 20 years were included, both in Spanish and English, in all age and ethnic groups, including: meta-analysis, systematic reviews, clinical trials, cohort studies and relevant clinical cases. Databases such as EBSCO, Medline, PubMed and SciELO were consulted among others to obtain updated information. Search terms were ''situs inversus'', ''totalis''. Results: Situs inversus totalis is a rare anatomical variant that involves thoracoabdominal structures, takes part in other situs variants, has a polygenic inheritance pattern, without a direct established relationship; is also an asymptomatic entity, recognised since ancient times. Conclusion: Despite being a condition with a low incidence in the population and having an incidental finding, it is important to recognise it as an anatomical variant that can coexist with concomitant diseases. Although studies exist, more are required with a higher statistical significance. A relationship is found between the cases with the information found in the literature, which leads to a strong research</org>.

Síndrome de Kartagener: reporte de un caso y revisión de la literatura / Kartagener syndrome: case report and literature review

El síndrome de Kartagener es una enfermedad autosómica recesiva poco frecuente (uno de cada 32.000 nacimientos), caracterizada por la tríada de bronquiectasias, sinusitis crónica y situs tnversus. El artículo presenta el caso de un hombre de veinticuatro años de edad con dicha enfermedad, a partir del cual se revisa su fisiopatología, las estrategias diagnósticas y terapéuticas y su pronóstico.

Kartagener syndrome us a rare autosomal recessive disease (one ¿n every 32,000 births), oharactenzed by a triad of bronchiectasis, chroníc sinusitis and situs mversus. We present the case of a 24'veai'old male with this disease and we review the pathophysiology, prognosis as well as the main diagnostic and therapeutic strategies.

Pectus Excavatum e Situs Inversus Totalis: um relato de caso / Pectus excavatum and situs inversus totalis: a case report

O Pectus Excavatum consiste em uma deformidade óssea torácica caracterizada, basicamente, pela depressão do esterno e das cartilagens costais inferiores, podendo cursar com alterações da extremidade anterior das costelas na sua articulação com as cartilagens costais. Ademais, o Situs Inversus Totalis (STI) é caracterizado pelo desenvolvimento das vísceras no lado oposto de sua topografia habitual acompanhada de dextrocardia; ápice cardíaco voltado para a direita. Os autores relatam um caso combinado de Pectus Excavatum e Situs Inversus Totalis em um paciente jovem, do sexo masculino, diagnosticado no período da puberdade e discutem brevemente acerca de uma revisão literária do caso(AU)

Pectus excavatum is a chest bone deformity characterized primarily by depression of the sternum and the lower costal cartilages, which may be accompanied by changes in the anterior end of the ribs in its articulation with rib cartilage. In addition, the situs inversus totalis (SIT) is characterized by the development of the viscera on the opposite side of its usual topography accompanied by dextrocardia; cardiac apex facing the right. The authors report a combined case of pectus excavatum and situs inversus totalis in a young male patient diagnosed in the puberty period and briefly discuss about a literature review of the case(AU)