Clinical and anatomopathological descriptions of dog with arterious Hemitruncus: case report / [Descrições clínicas e anatomopatológicas de cão com hemitruncus arterioso: relato de caso

The anomalous origin of the pulmonary trunk in the ascending aorta, defined as arterious hemitruncus, is a rare congenital malformation in dogs, caused by a defect in the spiral septum. Thus, given the unusual occurrence in the canine species, the systemic severity and the high lethality, the aim of this study was to describe this heart disease in a three-month-old male German Spitz puppy, emphasizing clinical changes of the necropsy and microscopics. The animal had cyanosis, dyspnea and weakness and was forwarded for necropsy after sudden death. Numerous changes were detected in the post-mortem examination, including in the heart, as cardiomegaly and absence of the arterial ligament, which was replaced by the complete fusion between the ascending aorta and the pulmonary trunk, after leaving both the left and right ventricles, respectively and, among the microscopic findings, cardiomyocyte hypertrophy stood out. The association of these findings with the history indicated the diagnosis of arterious hemitruncus followed by cardiorespiratory failure, emphasizing the importance of out complementary cardiological exams in young symptomatic patients for the survival of those affected. Arterious hemitruncus, although rare, must be added in the differential diagnosis of other heart diseases that cause similar clinical signs.(AU)

A origem anômala do tronco pulmonar em aorta ascendente, definida como hemitruncus arterioso, é uma malformação congênita rara em cães, causada por defeito no septo espiral. Assim, diante da ocorrência incomum na espécie canina, da gravidade sistêmica e da alta letalidade, o objetivo deste trabalho foi descrever essa doença cardíaca em um filhote de três meses de idade, macho, Spitz Alemão, enfatizando as alterações clínicas, de necropsia e microscópicas. O animal apresentava cianose, dispneia e fraqueza e foi encaminhado para necropsia após morte súbita. Inúmeras alterações foram detectadas no exame post-mortem, inclusive no coração, como cardiomegalia e ausência do ligamento arterioso, o qual foi substituído pela fusão completa entre aorta ascendente e tronco pulmonar, após a saída de ambas dos ventrículos esquerdo e direito, respectivamente, e, dentre os achados microscópicos, destacou-se a hipertrofia de cardiomiócitos. A associação desses achados com o histórico indicou o diagnóstico de hemitruncus arterioso seguido de insuficiência cardiorrespiratória, ressaltando-se a importância de exames complementares cardiológicos em pacientes jovens sintomáticos na sobrevida dos acometidos. O hemitruncus arterioso, apesar de raro, deve ser acrescido no diagnóstico diferencial de outras cardiopatias que causam sinais clínicos similares.(AU)

Alterações congênitas do coração e dos grandes vasos em cães / Congenital abnormalities of the heart and large vessels of dogs

As alterações congênitas do coração e dos grandes vasos (ACCGV) são definidas como defeitos morfológicos associados ao nascimento e são as anomalias congênitas mais frequentes dos animais domésticos. O objetivo deste trabalho foi determinar a frequência de diagnósticos de ACCGV em cães no Rio Grande do Sul, com ênfase na Região Metropolitana de Porto Alegre, no período de janeiro de 2000 a dezembro de 2016. Do total de 7.903 necropsias de cães, 27 morreram espontaneamente ou foram submetidos à eutanásia devido às ACCGV, representando 0,3% dos casos. Em 11,1% dos cães apresentaram dois distúrbios congênitos no coração ou nos grandes vasos, totalizando 30 alterações. A idade variou de um dia a 12 anos, com a idade mediana de quatro meses. Em 81,5% acometeu cães com raça, e 18,5% sem raça definida. Em relação ao sexo, 51,8% dos cães eram machos e 48,2%, fêmeas. Estenose subaórtica foi a alteração mais frequente, seguido por defeito do septo atrial, persistência do arco aórtico direito, persistência do ducto arterioso, estenose pulmonar e defeito do septo interventricular, e fibroelastose endocárdica. Dos casos múltiplos, as combinações encontradas foram: persistência do ducto arterioso associado com defeito do septo atrial, estenose subaórtica com defeito do septo interventricular, e defeito do septo atrial e ventricular.(AU)

Congenital alterations of the heart and large vessels (CAHLV) are defined as morphological defects associated with birth and are the most frequent congenital anomalies of domestic animals. The aim of this study was to determine the frequency of CAHLV in dogs in Rio Grande do Sul, with emphasis in the Metropolitan Region of Porto Alegre, from January 2000 to December 2016. Of the 7,903 necropsied dogs, 27 died spontaneously or were submitted to euthanasia due to CAHLV, representing 0.3% of the cases. In 11.1% of the dogs, there were two congenital disorders in the heart or in the great vessels, totaling 30 changes. The age of affected dogs ranged from one day to 12 years, with the median age of four months. In 81.5% it affected dogs with breed, and 18.5%, without breed defined. In 51.8% were males, and 48.2%, females. Subaortic stenosis was the most frequent alteration, followed by atrial septal defect, persistent right aortic arch, patent ductus arteriosus, pulmonic stenosis, ventricular septal defect, and endocardial fibroelastosis. Of the multiple cases, the combinations found were: patent ductus arteriosus associated with atrial septal defect, subaortic stenosis with ventricular septal defect, and atrial and ventricular septal defect.(AU)

Alterações congênitas do coração e dos grandes vasos em cães / Congenital abnormalities of the heart and large vessels of dogs

As alterações congênitas do coração e dos grandes vasos (ACCGV) são definidas como defeitos morfológicos associados ao nascimento e são as anomalias congênitas mais frequentes dos animais domésticos. O objetivo deste trabalho foi determinar a frequência de diagnósticos de ACCGV em cães no Rio Grande do Sul, com ênfase na Região Metropolitana de Porto Alegre, no período de janeiro de 2000 a dezembro de 2016. Do total de 7.903 necropsias de cães, 27 morreram espontaneamente ou foram submetidos à eutanásia devido às ACCGV, representando 0,3% dos casos. Em 11,1% dos cães apresentaram dois distúrbios congênitos no coração ou nos grandes vasos, totalizando 30 alterações. A idade variou de um dia a 12 anos, com a idade mediana de quatro meses. Em 81,5% acometeu cães com raça, e 18,5% sem raça definida. Em relação ao sexo, 51,8% dos cães eram machos e 48,2%, fêmeas. Estenose subaórtica foi a alteração mais frequente, seguido por defeito do septo atrial, persistência do arco aórtico direito, persistência do ducto arterioso, estenose pulmonar e defeito do septo interventricular, e fibroelastose endocárdica. Dos casos múltiplos, as combinações encontradas foram: persistência do ducto arterioso associado com defeito do septo atrial, estenose subaórtica com defeito do septo interventricular, e defeito do septo atrial e ventricular.(AU)

Congenital alterations of the heart and large vessels (CAHLV) are defined as morphological defects associated with birth and are the most frequent congenital anomalies of domestic animals. The aim of this study was to determine the frequency of CAHLV in dogs in Rio Grande do Sul, with emphasis in the Metropolitan Region of Porto Alegre, from January 2000 to December 2016. Of the 7,903 necropsied dogs, 27 died spontaneously or were submitted to euthanasia due to CAHLV, representing 0.3% of the cases. In 11.1% of the dogs, there were two congenital disorders in the heart or in the great vessels, totaling 30 changes. The age of affected dogs ranged from one day to 12 years, with the median age of four months. In 81.5% it affected dogs with breed, and 18.5%, without breed defined. In 51.8% were males, and 48.2%, females. Subaortic stenosis was the most frequent alteration, followed by atrial septal defect, persistent right aortic arch, patent ductus arteriosus, pulmonic stenosis, ventricular septal defect, and endocardial fibroelastosis. Of the multiple cases, the combinations found were: patent ductus arteriosus associated with atrial septal defect, subaortic stenosis with ventricular septal defect, and atrial and ventricular septal defect.(AU)

Use of total venous inflow occlusion to enable correction of an atrial septal defect in a dog

Background: Atrial septal defects are generally classified into three types: ostium secundum, ostium primum, and sinus venosus. Diagnose is normaly confirmed with Doppler echocardiography, which can identify and classify atrial septal defects types. This cardiac anomaly may be corrected by different surgical approaches, such as cardiopulmonary bypass or transvenous approaches. Therefore, we chose to close the atrial septal defect using a total venous inflow occlusion technique (TIVO), which has been successfully used in other procedures without major postoperative complications, and are notably cheaper and requires no specialized equipment, been able to be done in different places.Case: An American Pit Bull Terrier was referred to our surgical service for ostium secundum atrial septal defect correction, by the time of surgery the patient presented dyspneic; normal capillary refill time and rectal temperature; the owner mentioned the patient exhibited exercise intolerance and delayed development compared to other dogs of the same age or from the same litter. Cardiac auscultation revealed a systolic murmur at the left base, femoral pulse was normokinetic, and patient was emaciated and prostrated at the time of clinical evaluation. Atrial septal defect was suspected and then confirmed by Doppler echocardiography that revealed a discontinuous area in the interatrial septum, and by color doppler images an aliased signal, that extended through the interatrial septum and shunted from the left to the right atria, was visualized, confirming an ostium secundum atrial septal defect. After a right intercostal thoracotomy, a subphrenic pericardiectomy was performed to provide access to the heart. Cranial and caudal vena cava as well as the azygos vein, were dissected, which allowed placement of a Satinsky’s clamp to proceed with TIVO. Before TIVO initiation, a pursestring suture with 3-0 polypropylene was applied to the right atrium.[...]

Use of total venous inflow occlusion to enable correction of an atrial septal defect in a dog

Background: Atrial septal defects are generally classified into three types: ostium secundum, ostium primum, and sinus venosus. Diagnose is normaly confirmed with Doppler echocardiography, which can identify and classify atrial septal defects types. This cardiac anomaly may be corrected by different surgical approaches, such as cardiopulmonary bypass or transvenous approaches. Therefore, we chose to close the atrial septal defect using a total venous inflow occlusion technique (TIVO), which has been successfully used in other procedures without major postoperative complications, and are notably cheaper and requires no specialized equipment, been able to be done in different places.Case: An American Pit Bull Terrier was referred to our surgical service for ostium secundum atrial septal defect correction, by the time of surgery the patient presented dyspneic; normal capillary refill time and rectal temperature; the owner mentioned the patient exhibited exercise intolerance and delayed development compared to other dogs of the same age or from the same litter. Cardiac auscultation revealed a systolic murmur at the left base, femoral pulse was normokinetic, and patient was emaciated and prostrated at the time of clinical evaluation. Atrial septal defect was suspected and then confirmed by Doppler echocardiography that revealed a discontinuous area in the interatrial septum, and by color doppler images an aliased signal, that extended through the interatrial septum and shunted from the left to the right atria, was visualized, confirming an ostium secundum atrial septal defect. After a right intercostal thoracotomy, a subphrenic pericardiectomy was performed to provide access to the heart. Cranial and caudal vena cava as well as the azygos vein, were dissected, which allowed placement of a Satinskys clamp to proceed with TIVO. Before TIVO initiation, a pursestring suture with 3-0 polypropylene was applied to the right atrium.[...](AU)

Pentalogy of fallot in a Crioulo foal

Background: Cardiac anomalies account for 3.5% of congenital defects in horses. Tetralogy of Fallot (TOF) is characterized by ventricular septal defect, pulmonary stenosis, dextroposition the aorta and right ventricular hypertrophy secondary. Pentalogy of Fallot (POF) is a variation and is characterized by a defect in the right atrioventricular valve or persistence of the ductus arteriosus, in addition to the other defects observed in tetralogy. Affected animals usually have stunted growth, exercise intolerance, weakness, lethargy, cyanosis, dyspnea and syncope. The aim of this study was to describe a case of POF, as diagnosed in a Crioulo foal in southern Brazil. Case: Clinical signs were observed at the fifth day of life and were characterized by cyanosis, intense tracheal stertor, tachycardia, marked heart murmur, HR 160 bpm, RR 80 breaths/min, T 39.8C and syncope. These signs worsened with physical activity. Endoscopic evaluation of the upper airways revealed no anatomical conformation changes or tissue dysfunction. A cardiac ultrasound showed a loss of continuity of the interventricular septum, right ventricular wall thickening and a hyperechoic appearance of the area where the right atrioventricular valve (tricuspid) should be. The crises have become more severe and more frequent, the animal began to show signs of ischemia, such as loss of motor coordination and limited [...]

Pentalogy of fallot in a Crioulo foal

Background: Cardiac anomalies account for 3.5% of congenital defects in horses. Tetralogy of Fallot (TOF) is characterized by ventricular septal defect, pulmonary stenosis, dextroposition the aorta and right ventricular hypertrophy secondary. Pentalogy of Fallot (POF) is a variation and is characterized by a defect in the right atrioventricular valve or persistence of the ductus arteriosus, in addition to the other defects observed in tetralogy. Affected animals usually have stunted growth, exercise intolerance, weakness, lethargy, cyanosis, dyspnea and syncope. The aim of this study was to describe a case of POF, as diagnosed in a Crioulo foal in southern Brazil. Case: Clinical signs were observed at the fifth day of life and were characterized by cyanosis, intense tracheal stertor, tachycardia, marked heart murmur, HR 160 bpm, RR 80 breaths/min, T 39.8C and syncope. These signs worsened with physical activity. Endoscopic evaluation of the upper airways revealed no anatomical conformation changes or tissue dysfunction. A cardiac ultrasound showed a loss of continuity of the interventricular septum, right ventricular wall thickening and a hyperechoic appearance of the area where the right atrioventricular valve (tricuspid) should be. The crises have become more severe and more frequent, the animal began to show signs of ischemia, such as loss of motor coordination and limited [...](AU)

Tetralogia de Fallot em cão - relato de caso / Tetralogy of Fallot in a dog - case report / Tetralogía de Fallot en perro - relato de caso

La tetralogía de Fallot es una enfermedad congénita rara, caracterizada por estenosis de la válvula pulmonar, hipertrofia ventricular derecha, comunicación interventricular y dextroposición de la arteria aorta. La fisiopatología y las características clínicas con que se presenta dependen principalmente del grado de obstrucción de la arteria pulmonar. Desde el punto de vista clínico, los animales presentan cianosis, resistencia al ejercicio, disnea y síncopes. El diagnóstico definitivo y el pronóstico se obtienen mediante ecocardiografía (Doppler). Si bien la cirugía es el único método efectivo de tratamiento, en casos menos graves, existen medicamentos que actúan de manera paliativa. Este trabajo relata el caso de un perro mestizo de once meses con diagnóstico de tetralogía de Fallot, haciendo énfasis en la ecocardiografia (Doppler) como método de diagnóstico definitivo.

Tetralogy of Fallot is a rare congenital disease consisting of pulmonary valve stenosis, right ventricular hypertrophy, ventricular septal defect and aortic dextroposition. Pathophysiological consequences and clinical presentation depend mainly on the degree of pulmonary artery obstruction. Symptoms include cyanosis, exercise intolerance, dyspnea, and syncope. Definitive diagnosis and prognosis are obtained by Doppler echocardiography. Surgery is the only effective method of treatment, however, pharmacotherapy may be indicated as palliative treatment in mild cases of the disease. This work reports on the case of an eleven-month-old mongrel, diagnosed with tetralogy of Fallot and highlights the importance of Doppler echocardiography as the definitive diagnostic tool for this disease.

A tetralogia de Fallot é urna rara doença congênita, definida por estenose da valva pulmonar, hipertrofia ventricular direita, defeito do septo interventricular e dextroposição da aorta. Suas consequências fisiopatológicas, assim como a apresentação clínica, dependem principalmente do grau de obstrução da artéria pulmonar. Clinicamente, os animais apresentam cianose, intolerância ao exercício, dispneia e síncopes. O diagnóstico definitivo e o prognóstico são obtidos por meio da ecodopplercardiografia. A cirurgia é o único método efetivo para o tratamento; no entanto, em casos brandos, a terapia medicamentosa pode ser indicada como forma paliativa. O presente trabalho relata o caso de um cão de onze meses de idade, sem raça definida, diagnosticado com tetralogia de Fallot, enfatizando a ecoclopplercardiografia como método diagnóstico definitivo da doença.

Tetralogia de Fallot em cão - relato de caso / Tetralogy of Fallot in a dog - case report / Tetralogía de Fallot en perro - relato de caso

La tetralogía de Fallot es una enfermedad congénita rara, caracterizada por estenosis de la válvula pulmonar, hipertrofia ventricular derecha, comunicación interventricular y dextroposición de la arteria aorta. La fisiopatología y las características clínicas con que se presenta dependen principalmente del grado de obstrucción de la arteria pulmonar. Desde el punto de vista clínico, los animales presentan cianosis, resistencia al ejercicio, disnea y síncopes. El diagnóstico definitivo y el pronóstico se obtienen mediante ecocardiografía (Doppler). Si bien la cirugía es el único método efectivo de tratamiento, en casos menos graves, existen medicamentos que actúan de manera paliativa. Este trabajo relata el caso de un perro mestizo de once meses con diagnóstico de tetralogía de Fallot, haciendo énfasis en la ecocardiografia (Doppler) como método de diagnóstico definitivo.(AU)

Tetralogy of Fallot is a rare congenital disease consisting of pulmonary valve stenosis, right ventricular hypertrophy, ventricular septal defect and aortic dextroposition. Pathophysiological consequences and clinical presentation depend mainly on the degree of pulmonary artery obstruction. Symptoms include cyanosis, exercise intolerance, dyspnea, and syncope. Definitive diagnosis and prognosis are obtained by Doppler echocardiography. Surgery is the only effective method of treatment, however, pharmacotherapy may be indicated as palliative treatment in mild cases of the disease. This work reports on the case of an eleven-month-old mongrel, diagnosed with tetralogy of Fallot and highlights the importance of Doppler echocardiography as the definitive diagnostic tool for this disease.(AU)

A tetralogia de Fallot é urna rara doença congênita, definida por estenose da valva pulmonar, hipertrofia ventricular direita, defeito do septo interventricular e dextroposição da aorta. Suas consequências fisiopatológicas, assim como a apresentação clínica, dependem principalmente do grau de obstrução da artéria pulmonar. Clinicamente, os animais apresentam cianose, intolerância ao exercício, dispneia e síncopes. O diagnóstico definitivo e o prognóstico são obtidos por meio da ecodopplercardiografia. A cirurgia é o único método efetivo para o tratamento; no entanto, em casos brandos, a terapia medicamentosa pode ser indicada como forma paliativa. O presente trabalho relata o caso de um cão de onze meses de idade, sem raça definida, diagnosticado com tetralogia de Fallot, enfatizando a ecoclopplercardiografia como método diagnóstico definitivo da doença.(AU)

Malformações congênitas multissistêmicas em um feto bovino abortado / Congenital multisystemic malformations in an aborted bovine fetus

Background: Congenital anomalies are structural and functional abnormalities that are present at birth, and they are frequently the result of environmental or genetic factors or both. Their etiology may also be related to infectious agents, such as viruses, hereditary factors, or the ingestion of toxic plants or chemical agents. Congenital anomalies usually occur sporadically, but they also can occur as outbreaks. The objective of this paper is to describe the multiple congenital malformations observed in an aborted bovine fetus. Case: A bovine fetus was sent to Laboratory Veterinary Pathology UFRGS for necropsy, histological analysis and complementary exams such as bacteriology, direct immunofluorescence for Leptospira sp. and immunohistochemistry for bovine viral diarrhea virus (BVDV). The fetus presented multiple congenital alterations, such as dextraposition of the aorta and absence of the pulmonary artery, tracheal stenosis, a hypoplastic lung, multiple heart defects, arthrogryposis, osteopetrosis, and the absence of the middle phalanx and accessory digits in the forelimbs. Furthermore, dystocia and enlargement of the liver were observed, the organ showed irregular surface, firm consistency and nodular formation. A histological exam verified the accentuated hepatic periportal fibrosis. All complementary exams were negative. Discussion: Congenital anomalies can affect only one organ or can be multisystemic due to a malformation of one part of the body leading to the loss of another. Arthrogryposis can delay delivery, and signs of dystocia may be present in the resulting fetus. The etiology of arthrogryposis is difficult to determine, and even studies that were previously performed in ruminants have failed to identify a specific agent. Osteopetrosis, which was also observed, is a congenital skeletal anomaly caused by the failed resorption of cartilage and endochondral bone, and it has been associated with intrauterine BVDV infections, can also be associated with autosomal recessive genes in the Angus bovine breed. Congenital heart defects are relatively rare in cattle. Ventricular sept defects and transposition of the great vessels are two of the most common cardiovascular anomalies in bovines, and they can occur individually or in association with other heart defects. Generally, these malformations are linked to genetic factors. In this study, the animal presented with several conformational heart defects that resulted in embryonic development alterations in its structure or that resulted in progressive heart failure. Blood flow changes caused by an anatomic defect can significantly influence the structural and functional development of the circulation, resulting in secondary alterations. In this case, the primary defect is hard to determine, but the observed bilateral pulmonary hypoplasia may be the consequence of a blood flow decrease to the lungs because the pulmonary artery was absent. Pulmonary hypoplasia is defined as the incomplete development of the lungs, reducing the number of divisions of the bronchial tree and the associated alveoli. Many causes of pulmonary hypoplasia have been described in human beings and animals, such as chest abnormalities, congenital diaphragmatic hernia, chromosome anomalies, musculoskeletal disorders and heart failure. The accentuated hepatic fibrosis observed in this study, which was predominantly periportal, correlated with congestive heart failure due to the cardiac and pulmonary malformation observed in the fetus, which would have altered the systemic blood flow. It is suggested that the studied case is a congenital malformation of sporadic origin, characterized by multiple deformities associated with unknown genetic factors.

Mucopolissacaridose canina: relato de oito casos / Mucopolysaccharidosis in dogs: report of eight cases / Mucopolisacaridosis canina: informe de ocho casos

Mucopolissacaridoses são doenças hereditárias caracterizadas pela deficiência de enzimas que degradam as glicosaminoglicanas (GAGs): ácido hialuronico, sulfato de condroitina, sulfato de dermatina, queratan sulfato e heparina, sendo que estas se acumulam nos lisossomos de células ósseas, cartilagens e sinóvias. São descritos sete tipos de mucopolissacaridoses. As manifestações clínicas incluem hepatoesplenomegalia, opacificação de córnea, alterações cardiovasculares, edema de articulações, alterações neurológicas e má formações na face. A detecção de grânulos de Alder Reilynos leucócitos, além de deformidades ósseas bem como o comprometimento do fechamento de discos epifisários, observados ao exame radiográfico, e o aumento de GAGs na urina confirmam o diagnóstico. O objetivo deste trabalho foi relatar oito casos de cães com mucopolissacaridose e alertar sobre este diagnóstico diferencial.(AU)

Mucopolysaccharidoses are hereditary disorders characterized by a deficiency of enzymeswhich degrade glycosaminoglycans (GAGs): hyaluronic acid, chondroitin sulphate, dermatinsulphate, keratansulphate and heparin, and they accumulate in lysosomes of bone cells, cartilage and synovium. There are seven types of mucopolysaccharidosis. Clinical signs include hepatosplenomegaly, corneal opacification, cardiovascular abnormalities, edema of joints, neurological disorders and malformations of the face. The detection of Alder Reily granules in leukocytes, and bone deformities as well as radiographic findings of defect on closing epiphyseal discs, and increase of GAGs in urine confirm the diagnosis. The aim of this study was to report eight cases of dogs with mucopolysaccharidosis, and warn of this differential diagnosis.(AU)

Mucopolisacaridosiss on transtornos hereditarios caracterizados por una deficiencia de enzimas que degradan glicosaminoglicanos (GAGs): ácido hialurónico, sulfato de condroitina, sulfato de dermatina, sulfato de queratano y heparina, y se acumulan en los lisosomas de las células de hueso, cartílago y la membrana sinovial. Se describen siete tipos de mucopolisacaridosis. Las manifestaciones clínicas incluyen hepatoesplenomegalia, opacificación corneal, cambios cardiovasculares, edema de las articulaciones, trastornos neurológicos y malformaciones en la cara. La detección de gránulos Alder Reily en leucocitos, y deformidad de los huesos, así como los discos epifisarias de cierre y signos radiológicos y la acumulación de GAGs en la orina a confirmar el diagnóstico. El objetivo de estudio es reportar ocho casos de perros con mucopolisacaridosis, advertir de este diagnóstico diferencial.(AU)

Mucopolissacaridose canina: relato de oito casos / Mucopolysaccharidosis in dogs: report of eight cases / Mucopolisacaridosis canina: informe de ocho casos

Mucopolissacaridoses são doenças hereditárias caracterizadas pela deficiência de enzimas que degradam as glicosaminoglicanas (GAGs): ácido hialuronico, sulfato de condroitina, sulfato de dermatina, queratan sulfato e heparina, sendo que estas se acumulam nos lisossomos de células ósseas, cartilagens e sinóvias. São descritos sete tipos de mucopolissacaridoses. As manifestações clínicas incluem hepatoesplenomegalia, opacificação de córnea, alterações cardiovasculares, edema de articulações, alterações neurológicas e má formações na face. A detecção de grânulos de Alder Reilynos leucócitos, além de deformidades ósseas bem como o comprometimento do fechamento de discos epifisários, observados ao exame radiográfico, e o aumento de GAGs na urina confirmam o diagnóstico. O objetivo deste trabalho foi relatar oito casos de cães com mucopolissacaridose e alertar sobre este diagnóstico diferencial.

Mucopolysaccharidoses are hereditary disorders characterized by a deficiency of enzymeswhich degrade glycosaminoglycans (GAGs): hyaluronic acid, chondroitin sulphate, dermatinsulphate, keratansulphate and heparin, and they accumulate in lysosomes of bone cells, cartilage and synovium. There are seven types of mucopolysaccharidosis. Clinical signs include hepatosplenomegaly, corneal opacification, cardiovascular abnormalities, edema of joints, neurological disorders and malformations of the face. The detection of Alder Reily granules in leukocytes, and bone deformities as well as radiographic findings of defect on closing epiphyseal discs, and increase of GAGs in urine confirm the diagnosis. The aim of this study was to report eight cases of dogs with mucopolysaccharidosis, and warn of this differential diagnosis.

Mucopolisacaridosiss on transtornos hereditarios caracterizados por una deficiencia de enzimas que degradan glicosaminoglicanos (GAGs): ácido hialurónico, sulfato de condroitina, sulfato de dermatina, sulfato de queratano y heparina, y se acumulan en los lisosomas de las células de hueso, cartílago y la membrana sinovial. Se describen siete tipos de mucopolisacaridosis. Las manifestaciones clínicas incluyen hepatoesplenomegalia, opacificación corneal, cambios cardiovasculares, edema de las articulaciones, trastornos neurológicos y malformaciones en la cara. La detección de gránulos Alder Reily en leucocitos, y deformidad de los huesos, así como los discos epifisarias de cierre y signos radiológicos y la acumulación de GAGs en la orina a confirmar el diagnóstico. El objetivo de estudio es reportar ocho casos de perros con mucopolisacaridosis, advertir de este diagnóstico diferencial.

Defeitos congênitos em bovinos da Região Central do Rio Grande do Sul / Congenital defects in cattle from central Rio Grande do Sul, Brazil

Foram revisados casos de defeitos congênitos (DCs) diagnosticados em bovinos no Laboratório de Patologia da Universidade Federal de Santa Maria em 1964-2010. Durante o período estudado, foram examinados materiais provenientes da necropsia de 7.132 bovinos e foram encontrados 31 bezerros (0,4 por cento) com DCs, os quais foram classificados em 34 tipos e alocados nos sistemas orgânicos primariamente afetados. Os DCs ocorriam isoladamente (19 [61,3 por cento]) ou afetavam múltiplos sítios anatômicos (15 [28,7 por cento]) com frequência semelhante em ambos os sexos. Como vários terneiros mostraram múltiplos DCs, um total de 53 DCs foi computado. Dos 53 DCs diagnosticados, 15 (28,3 por cento) afetavam o sistema nervoso central (craniósquise [4], abiotrofia cerebelar [2], degeneração esponjosa [2], hidrocefalia [2], meningocele [2], espinha bífida [1], hipoplasia cerebelar [1] e hipomielinogênese [1]); nove (17,0 por cento) afetavam o sistema urogenital (agenesia testicular [1], agenesia vaginal [1], hipoplasia peniana [1], formação de cloaca [1], freemartinismo [1], hamartoma vascular de ovário [1], hipoplasia renal [1], cistos renais [1] e úraco persistente [1]); oito DCs (15,1 por cento) eram primários do sistema musculoesquelético (artrogripose [4], escoliose [1], plagiocefalia, [1] schistosomus reflexus [1] e diprosopia [1]); e outros oito (15,1 por cento) foram alocados no sistema digestivo (palatosquise [3], atresia anal [1], atresia anorretal [1], atresia - anocolônica [1], fístula reto-vaginal [1] e fístula reto-uretral [1]); em cinco ocasiões (9,4 por cento) o DC afetava o sistema cardiovascular (persistência do ducto arterioso [2], persistência do forame oval [2] e defeito do septo ventricular [1]); quatro (7,5 por cento) afetavam o sistema linfático e consistiam de hipoplasia ou aplasia de vasos linfáticos e linfonodos associadas a linfedema. Dois casos (3,4 por cento), de hipotricose foram observados afetando o integumento; um caso (1,9 por cento) de estenose traqueal foi encontrado no sistema respiratório e um caso (1,9 por cento) de bócio envolvia o sistema endócrino. Os resultados indicam que a maioria dos DCs em bovinos na Região Central do Rio Grande do sul é esporádica. No entanto, seu estudo continuado é importante para o estabelecimento de sua etiologia e controle.(AU)

Cases of congenital defects (CDs) in cattle diagnosed at the Laboratory of Veterinary Pathology of the Federal University of Santa Maria from 1964 to 2010 were reviewed. During the studied period tissues collected from the necropsy of 7,132 cattle were examined and 31 calves (0.4 percent) with CDs were found. These CDs were classified into 34 different types and ascribed to the body system primarily affected. CDs were solitary (19 [61.3 percent]) or multiple (12 [38.7 percent]) and found with similar frequency in calves of both sexes. As several calves displayed multiple defects, a total of 53 CDs were computed. Out of the 53types of CDs diagnosed 15 (28.3 percent) affected the central nervous system (cranioschisis [4], cerebellar abiotrophy [2], spongy degeneration [2], hydrocephalus [2], meningocele [2], spina bifida [1], cerebellar hypoplasia [1], and hypomyelinogenesis [1]); nine (17.0 percent) affected the urogenital system (testicular agenesis [1], vaginal agenesia [1], penile hypoplasia [1], cloacal formation [1], freemartinism [1], ovarian vascular hamartoma [1], renal hypoplasia [1], renal cysts [1], and persistent urachus [1]); eight CDs (15,1 percent) were primary to the musculoskeletal system (arthrogryposis [4], scoliosis [1], plagiocephaly [1] schistosomus reflexus [1], and diprosopus [1]) and another eight (15,1 percent) were in the digestive system (palatoschisis [3], anal atresia [1], anorectal atresia [1], ano-colonic atresia [1], recto-vaginal fistula [1], and recto-urethral fistula [1]); in five instances (9.4 percent) the CD affected the cardiovascular system (persistent ductus arteriosus [2], persistence of foramen ovale [2], and ventricular septal defect [1]); four (7.5 percent) affected the lymphatic system and consisted of inherited hypoplasia or aplasia of lymphatic vessels and lymph nodes associated with lymphedema. Two cases (3.0 percent), of hypotrichosis were observed affecting the integument; one case (1.9 percent) of tracheal stenosis was found in the respiratory system and one case of goiter (1.9 percent) involved the endocrine system. The results of this survey inidicate that the majority o CDs in cattle in the central Rio Grande do Sul are sporadic; nevertheless their continued study is important for determining the etiology and control.(AU)

Persistência da veia cava cranial esquerda em cão / Persistent Left Cranial Vena Cava in a Dog

Background: Persistent left cranial vena cava with absent right cranial vena cava is a rare anomaly. Congenitally persistent left cranial vena cava is the most common variant of the systemic venous return to the heart, embryologically resulting from failure of the anterior cardinal vein in becoming obliterated. Persistent left cranial vena cava is the most common form of anomalous venous drainage involving the cranial vena cava, and represents persistence of the left horn of the embryonic sinus venosus, which normally involutes during normal development, becoming the coronary sinus. Usually, persistent left cranial vena cava enters the right atrium through the orifice of an enlarged coronary sinus. Persistent left cranial vena cava is an uncommon congenital cardiovascular anomaly in dogs, generally coexisting with other more serious cardiac defects. The condition is rare as an isolated single defect. The left cranial vena cava is observed only among domestic mammals, but it is a relatively rare anomaly that can be found in other species, including man, without causing clinical problems. The objective of this study was to report the occurence of a rare vascular formation of the cranial vena cava, which caused the persistence of the left cranial vena cava instead of the right one. Case: At the anatomy laboratory of the Faculty of Veterinary of the UFGRS, a male dog was identified with a persistent left cranial vena cava and absent right cranial vena cava.[...]

Persistência da veia cava cranial esquerda em cão / Persistent Left Cranial Vena Cava in a Dog

Background: Persistent left cranial vena cava with absent right cranial vena cava is a rare anomaly. Congenitally persistent left cranial vena cava is the most common variant of the systemic venous return to the heart, embryologically resulting from failure of the anterior cardinal vein in becoming obliterated. Persistent left cranial vena cava is the most common form of anomalous venous drainage involving the cranial vena cava, and represents persistence of the left horn of the embryonic sinus venosus, which normally involutes during normal development, becoming the coronary sinus. Usually, persistent left cranial vena cava enters the right atrium through the orifice of an enlarged coronary sinus. Persistent left cranial vena cava is an uncommon congenital cardiovascular anomaly in dogs, generally coexisting with other more serious cardiac defects. The condition is rare as an isolated single defect. The left cranial vena cava is observed only among domestic mammals, but it is a relatively rare anomaly that can be found in other species, including man, without causing clinical problems. The objective of this study was to report the occurence of a rare vascular formation of the cranial vena cava, which caused the persistence of the left cranial vena cava instead of the right one. Case: At the anatomy laboratory of the Faculty of Veterinary of the UFGRS, a male dog was identified with a persistent left cranial vena cava and absent right cranial vena cava.[...](AU)

Defeitos congênitos diagnosticados em ruminantes na Região Sul do Rio Grande do Sul / Congenital defects in ruminants in southern Brazil

Congenital defects in cattle, sheep and buffalo were studied through a review of necropsy files of the Regional Diagnostic Laboratory of the Veterinary Faculty at the Federal University of Pelotas between 1978 and 2009. The occurrence of congenital defects in cattle, sheep and buffalo were 0.88 percent, 0.36 percent, and 7.54 percent, respectively, from all specimens received. Cattle congenital defects of undetermined etiology represented 45.83 percent of the congenital defects, known hereditary and probably hereditary diseases represented 6.25 percent, and 29.16 percent, respectively, and defects associated to environmental factors represented 16.66 percent. In cattle, of the 48 congenital defects observed 21 (43.75 percent) affected the skeletal system (chondrodysplasia, scoliosis, lateral deviation of the mandible, palatoschisis and unclassified defect), nine (18.75 percent) affected the central nervous system (hypoplasia of olfatory and frontal lobes, cerebellar cortical degeneration, spina bifida, congenital hypomielinogenesis, hereditary hypermetria, cerebellar hypoplasia, and pachygiria), nine (18.75 percent) the muscular system (arthrogryposis), three (6.25 percent) the cardiovascular system (patent ductus arteriosus and unclassified malformation), one (2.08 percent) the lymphatic system (hereditary lymphatic hypoplasia), one (2.08 percent) the alimentary system (atresia ani), and one (2.08 percent) the eye (congenital blindness). In five cases (10.41 percent) different systems were affected (diprosopus). Different hereditary diseases (hereditary hypermetry, arthrogryposis, and lymphatic hypoplasia) or diseases suspected of being hereditary (chondrodysplasia) were diagnosed in cattle. Also occurred, with less frequency, congenital defects associated with environmental factors (hypomyelinogenesis due to cooper deficiency) or probably environmental factors (cleft palate, cerebellar hypoplasia, and cerebellar cortical degeneration). ...(AU)

Foi realizado um estudo dos defeitos congênitas diagnosticados em bovinos, ovinos e bubalinos mediante revisão dos protocolos de necropsia do Laboratório Regional de Diagnóstico da Faculdade de Veterinária da Universidade Federal de Pelotas entre 1978 e 2009. A ocorrência de defeitos congênitos em bovinos, ovinos e bubalinos representou 0,88 por cento, 0,36 por cento e 7,54 por cento respectivamente, de todos os materiais dessas espécies recebidos. Em bovinos os defeitos esporádicos representaram 45,83 por cento dos diagnósticos, os hereditários 6,25 por cento, os provavelmente hereditários 29,16 por cento e os ambientais ou provavelmente ambientais 16,66 por cento. Dos 48 casos de defeitos congênitos diagnosticados em bovinos 21 (43,75 por cento) afetaram o sistema esquelético (condrodisplasia, escoliose, desvio lateral da mandíbula, fenda palatina e malformação não classificada), nove (18,75 por cento) o sistema nervoso central (hipoplasia dos lobos frontais e olfatórios, degeneração cerebelar cortical, espinha bífida, hipomielinogênese congênita, hipermetria hereditária, hipoplasia cerebelar e paquigiria), nove (18,75 por cento) o sistema muscular (artrogripose), três (6,25 por cento) o sistema cardiovascular (persistência do ducto arterioso e malformação não classificada), um (2,08 por cento) o sistema linfático (hipoplasia linfática), um (2,08 por cento) o sistema gastrintestinal (atresia anal), e, um (2,08 por cento) o olho (catarata congênita). Em cinco casos (10,41 por cento) vários sistemas estavam afetados (diprosopo). ...(AU)

Anomalia congênita em vasos da base do coração de um potro / Congenit anomaly in vessels of base in a foal heart

Anomalias congênitas do coração e de grandes vasos estão entre as mais comuns encontradas em animais, porém são raras em eqüinos. Este relato descreve uma anomalia cardíaca que apresenta confluência entre a parede da aorta e do tronco pulmonar, ramificação da veia cava caudal no átrio esquerdo com presença de válvula e conseqüentemente posição errônea do coração na cavidade torácica de um potro da raça Quarto-de-Milha encontrado morto após o nascimento. O diagnóstico foi realizado pela associação dos achados macroscópicos durante a necrópsia e o exame histopatológico realizados no Curso de Medicina Veterinária- UNESP-Araçatuba.(AU)

Congenital anomalies of heart and large vessels are among more common found in animals, however rare in horses. This survey describes a cardiac anomaly that presents confluence between aorta wall and lung trunk, ramification of caudal cava vein in left atrium with presence of valve and consequently erroneous position of heart in thoracic cavity of one foal found dead after birth. The diagnostic was carried out for macroscopic finds association during the necropsy and histopathologic exams carried out at School of Veterinary MedicineUnesp Araçatuba.(AU)

Persistência do arco aórtico direito com ducto arterioso patente em um cão: relato de caso / Persistence of the right aortic arch with concurrent patent ductus arteriosus in a dog: case report

A persistência do arco aórtico direito é uma anomalia cardíaca congênita que leva à constrição do esôfago com regurgitação pós prandial em filhotes no período de desmame. A patência do dueto ar¬terioso trata-se de anomalia vascular congênita na qual o dueto persiste após o nascimento; a afecção causa grave sobrecarga de volume sanguíneo podendo levar à insuficiência cardíaca congestiva no átrio e ventrículo esquerdos, além de edema pulmonar no primeiro ano de vida. Este relato descreve associação das duas anomalias em um cão Pastor Belga que apresentava regurgitação, desenvolvi¬mento físico deficiente e sopro cardíaco contínuo. O histórico clínico associado à auscultação cardíaca e exames radiográficos subsidiaram o diagnóstico, porem, não se explorou a possibilidade de shunt reverso através do exame ecodopplercardiográfico. O paciente foi encaminhado à cirurgia e foi a óbito por descompensação cardiopulmonar(AU)

The persistence of the right aortic arch is a congenital cardiac anomaly that leads to constriction of the esophagus causing regurgitation in puppies after they are fed, during weaning time. The patency of the arterial duet is a congenital vascular anomaly in which the duet persists after birth; the disease causes a severe overload of blood volume, possibly leading to congestive cardiac insufficiency on the left atrium and ventricle, as well as pulmonary edema in the first year of life. This case report descri¬bes the association of the two anomalies in a Belgian Sheppard that was presented with regurgitation, deficient physical development and continuous heart murmur. History associated with auscultation and radiographic examination supported the diagnosis, but the possibility of a reverse shunt wasn't evaluated through echodopplercardiography. The patient underwent surgery and died of cardiopul¬monary complications(AU)

Persistência do arco aórtico direito com ducto arterioso patente em um cão: relato de caso / Persistence of the right aortic arch with concurrent patent ductus arteriosus in a dog: case report

A persistência do arco aórtico direito é uma anomalia cardíaca congênita que leva à constrição do esôfago com regurgitação pós prandial em filhotes no período de desmame. A patência do dueto ar¬terioso trata-se de anomalia vascular congênita na qual o dueto persiste após o nascimento; a afecção causa grave sobrecarga de volume sanguíneo podendo levar à insuficiência cardíaca congestiva no átrio e ventrículo esquerdos, além de edema pulmonar no primeiro ano de vida. Este relato descreve associação das duas anomalias em um cão Pastor Belga que apresentava regurgitação, desenvolvi¬mento físico deficiente e sopro cardíaco contínuo. O histórico clínico associado à auscultação cardíaca e exames radiográficos subsidiaram o diagnóstico, porem, não se explorou a possibilidade de shunt reverso através do exame ecodopplercardiográfico. O paciente foi encaminhado à cirurgia e foi a óbito por descompensação cardiopulmonar

The persistence of the right aortic arch is a congenital cardiac anomaly that leads to constriction of the esophagus causing regurgitation in puppies after they are fed, during weaning time. The patency of the arterial duet is a congenital vascular anomaly in which the duet persists after birth; the disease causes a severe overload of blood volume, possibly leading to congestive cardiac insufficiency on the left atrium and ventricle, as well as pulmonary edema in the first year of life. This case report descri¬bes the association of the two anomalies in a Belgian Sheppard that was presented with regurgitation, deficient physical development and continuous heart murmur. History associated with auscultation and radiographic examination supported the diagnosis, but the possibility of a reverse shunt wasn't evaluated through echodopplercardiography. The patient underwent surgery and died of cardiopul¬monary complications

Megaesôfago devido à persistência do quarto arco aórtico direito: aspectos clínicos e terapêuticos: relato de caso / Megaesophagus due to fourth right aortic persistence: clinical and therapeutic features: case report

O megaesôfago tem sido descrito como uma dilatação esofágica difusa, com diminuição ou ausência da função motora, podendo ocorrer devido a uma desordem congênita, adquirida ou, em adultos, como uma doença de princípio idiopático. Este relato de caso é de uma cadela da raça Fila Brasileiro de três meses de idade que regurgitava sempre após a ingestão de alimento, tinha apetite voraz e apresentava quadro de emagrecimento progressivo. Ao exame clínico, apresentou temperatura retal de 39,9°C, estado geral regular devido à marcante desnutrição e retardo no crescimento, mucosas normocoradas, estertores pulmonares e estridor traqueal. Foram realizados exames radiográficos simples e contrastados, das regiões cervical e torácica, na projeção látero-lateral obtendo o diagnóstico de megaesôfago por persistência do quarto arco aórtico direito. O animal foi submetido à cirurgia e manejo alimentar diferenciado no pós-operatório, sendo o tratamento eficaz para correção do problema e recuperação do paciente(AU)

Megaesophagus has been discribed as an esophagal diffuse dilatation, with the decrease or even absence of the motor function. It may occur owing to a congenital or acquired disorder or, in adults, as an idiopathic one. This report is about a three-month Fila Brasileiro that was always used to regurgitating after food ingestion. The animal presented a voracious appetite, nevertheless progressive loss of weight. Clinical examination documented rectal temperature of 39,9°C, regular general status due to the accentuated underfeeding and growth retardation, normal mucous membranes color, pulmonary crackles and tracheal murmur. Simple and contrasted radiographic examinations from cervical and thoracic regions, in latero-lateral projection, were realized outpointing the diagnosis of Megaesophagus, due to fourth right aortic arch persistence. The animal underwent surgery and a differentiated food handling in post-operatory period was indicated. The therapy was effective to the problem correction and animal recovery(AU)