ABSTRACT
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.
Subject(s)
Humans , Female , Adult , Situs Inversus/diagnostic imaging , Kartagener Syndrome/complications , Dextrocardia/diagnosis , Situs Inversus/complications , Kartagener Syndrome/diagnosis , Ciliary Motility DisordersABSTRACT
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.
Subject(s)
Humans , Male , Young Adult , Situs Inversus/complications , Situs Inversus/diagnostic imaging , Purpura, Thrombocytopenic, Idiopathic/complications , Situs Inversus/pathology , Radiography, Panoramic , Tomography, X-Ray ComputedABSTRACT
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.
Subject(s)
Humans , Male , Aged, 80 and over , Situs Inversus/pathology , Abnormalities, Multiple , Dextrocardia/pathology , Situs Inversus/diagnostic imaging , Dextrocardia/diagnostic imagingABSTRACT
Dextrocardia with situs inversus is a rare condition. Situs inversus with dextrocardia is also called as "situs inversus totalis". Transesophageal echocardiography (TEE) views in dextrocardia patient are not discussed in the literature. The cardiac position and the cardiac chambers are mirror image of the normal anatomy. Because of this positional change, certain TEE probe and multiplane angle manipulations are required to obtain the recommended views.
Subject(s)
Dextrocardia/complications , Dextrocardia/physiopathology , Dextrocardia/diagnostic imaging , Echocardiography, Transesophageal , Electrocardiography , Humans , Male , Middle Aged , Situs Inversus/complications , Situs Inversus/diagnostic imagingABSTRACT
El objetivo del presente reporte es dar a conocer un caso de situs inversus y colecistitis aguda diagnosticado y tratado en el Hospital Al Wahda Mabar Thamar de Yemen. Paciente de sexo femenino, de 50 años, con dolor en epigastrio después de la ingestión de alimentos grasos. Dos días antes de su ingreso incrementa su intensidad y se mantiene en hipocondrio izquierdo, y además, presenta vómitos. Utilizando antibioticoterapia perioperatoria, se realiza incisión subcostal izquierda, y se encuentra colecistitis aguda con dilatación moderada del colédoco. Se realiza colecistectomía y coledocotomía, con buena evolución posoperatoria(AU)
The objective of present paper is to present a case of situs inversus and cholecystitis diagnosed and treated in the Al Wahda Mabar Thamar Hospital of Yemen. A female patient aged 50 with epigastric pain after ingestion of fatty foods; two days before its admission increase its intensity and remains in left hypochondrium and also vomiting. With the use of perioperative antibiotic-therapy, a left subcostal incision is made detecting an acute cholecystitis with a choledochal slight dilatation. A cholecystectomy and choledochotomy with a good postoperative evolution were performed(AU)
Subject(s)
Humans , Middle Aged , Situs Inversus/diagnostic imaging , Choledochostomy/methods , Cholecystectomy/methods , Cholecystitis, Acute/diagnosisABSTRACT
Endoscopic procedures are difficult in patients with situs inversus owing to left-right reversal of viscera. Conventionally, reversal of the position of the endoscopist in relation to the patient is advocated to overcome the anatomical difficulty. We describe a patient with chronic calcific pancreatitis and pseudocyst of the pancreas who had situs inversus. ERCP was performed with minor modification of maneuvers with the patient in the usual left lateral decubitus and the endoscopist on the left of the table.
Subject(s)
Adult , Calcinosis/diagnostic imaging , Cholangiopancreatography, Endoscopic Retrograde/instrumentation , Chronic Disease , Equipment Design , Humans , Male , Pancreatic Pseudocyst/diagnostic imaging , Pancreatitis/diagnostic imaging , Situs Inversus/diagnostic imagingABSTRACT
We report a case of carcinoma gall bladder with situs inversus totalis in a 65 years old lady. She presented very late with obstructive jaundice and gastric outlet obstruction. Different imaging tests were done to accurately outline vital structures. At laparotomy the carcinoma was inoperable, hence a palliative percutaneous external biliary drainage and gastrojejunostomy was done. She survived for three months but ultimately died of disseminated cancer