Morfología de las paredes ventriculares en fetos humanos con defectos septales

Introducción: las cardiopatías congénitas constituyen defectos estructurales y/o funcionales del corazón y los grandes vasos, como consecuencia de un error en la embriogénesis de estas estructuras. Los defectos septales suelen ser las cardiopatías congénitas más frecuentes. Objetivo: identificar la asociación entre la morfología de las paredes ventriculares y los defectos septales en fetos humanos. Materiales y métodos: se realizó un estudio observacional, descriptivo y transversal en la Facultad de Ciencias Médicas de Pinar del Río Dr. Ernesto Guevara de la Serna y el Departamento de Anatomía Patológica del Hospital General Docente Abel Santamaría Cuadrado, en el período de enero de 2019 a diciembre de 2020, con 54 fetos producto de interrupciones de embarazo indicadas por genética. Resultados: predominaron los defectos de septación en el sexo masculino. La comunicación interventricular fue el defecto de septación más frecuente. El grosor del ventrículo derecho mostró diferencias lineales y estadísticas significativas en relación con la edad gestacional en los tres defectos estudiados. Similares características presentó el grosor del tabique interventricular en los defectos troncoconales. Conclusiones: ante la presencia de defectos septales se observan modificaciones del grosor de los tabiques cardiacos.

Introduction: congenital heart diseases are structural and/or functional defects of the heart and large vessels, as a consequence of an error in the embryogenesis of these structures. Septal defects are usually the most common congenital heart diseases. Objective: to identify the association between ventricular walls morphology and septal defects in human fetuses. Materials and methods: an observational, descriptive and cross-sectional study was carried out in the Medical Sciences Faculty of Pinar del Rio Dr. Ernesto Guevara de la Serna and the Department of Pathological Anatomy of the General Teaching Hospital Abel Santamaría Cuadrado, in the period from January 2019 to December 2020, with 54 fetuses resulting from pregnancy interruptions indicated by genetics Results: septation defects predominated in males. Interventricular communication was the most frequent septation defect. The thickness of the right ventricle showed significant linear and statistical differences in relation to gestational age in the three studied defects. Similar characteristics showed the thickness of the inter-ventricular septum in truncoconal defects. Conclusions: in the presence of septal defects, modifications of the heart septum thickness are observed.

Doble cuarto arco aórtico persistente en un perro (Canis familiaris): reporte de caso

El doble arco aórtico persistente es una patología caracterizada por anomalías embrionarias en la vascularización, que pueden afectar de manera indirecta a otros sistemas, como el digestivo y el respiratorio. El objetivo de este documento es reportar un caso de doble arco aórtico, persistente en un cachorro Bull terrier, de seis meses de edad. El paciente ingresó a consulta por motivo de regurgitaciones frecuentes y pérdida ponderal. En el estudio radiográfico, se evidenció dilatación esofágica craneal a la base del corazón y en la toracotomía, se confirmó un doble arco aórtico persistente. Se realizó manejo nutricional y posteriormente, corrección quirúrgica de la anomalía vascular. Este es el primer caso de una anomalía de este tipo en Colombia. Se concluye, que un manejo quirúrgico enfocado a liberar el anillo estenosante y a recuperar la función esofágica, son la base terapéutica de este tipo de alteraciones.

Persistent double aortic arch is a pathology characterized by embryonic vascularization anomalies, which can indirectly affect other systems such as the digestive and respiratory systems. The objective of this document is to report a case of persistent double aortic arch in a six-month-old Bull Terrier puppy. The patient was admitted for consultation due to frequent regurgitation and weight loss. The radiographic study revealed cranial esophageal dilation at the base of the heart, and a thoracotomy confirmed a persistent double aortic arch. Nutritional management was performed and subsequently, surgical correction of the vascular anomaly. This is the first case of an anomaly of this type in Colombia. It is concluded that surgical management focused on releasing the stenosing ring and recovering esophageal function are the therapeutic basis for this type of alteration.

Consideraciones actuales sobre los teratógenos y sus efectos durante el embarazo / Current considerations on the teratogens and their effects during pregnancy

Introducción: El desarrollo alcanzado por la teratología como ciencia y las observaciones clínicas efectuadas por los científicos, permitieron establecer sus principios, representados por una serie de factores que determinan la capacidad de un agente de provocar trastornos congénitos, los cuales resultan actualmente de gran utilidad e importancia en la formación del profesional de la salud y repercuten en el Programa de Atención Materno Infantil. Objetivo: Describir los agentes teratógenos asociados a la aparición de defectos congénitos. Métodos: Se realizó una revisión bibliográfica acerca de los agentes teratógenos y sus posibles efectos teratogénicos en las bases de datos LILACS, SciELO, Clinicalkey y Google Académico, así como en la plataforma Biblioteca Virtual en Salud. Resultados: Las consecuencias de la exposición al teratógeno dependen, entre otras cosas, del momento en que se encuentre el proceso de gestación. Existen múltiples agentes teratógenos y algunos factores de riesgo que frecuentemente concomitan con ellos, por lo que se deben considerar en la prevención primaria para disminuir alteraciones y defectos congénitos por estas noxas ambientales. Consideraciones finales: El conocimiento de los efectos de los agentes teratógenos permite valorar el posible daño al feto.

Introduction: The development reached by teratology as science and the clinical observations made by the scientists, allowed to establish their principles, represented by a series of factors that determine the capacity of an agent to cause congenital dysfunctions, which are at the moment of great utility and importance in the training of health professional and rebound in the Infantile Maternal Care Program. Objective: To describe the teratogen agents associated with the appearance of congenital defects. Methods: A literatura review was carried out about the teratogen agents and their possible teratogenic effects in the LILACS, SciELO, Clinicalkey databases and Academic Google, as well as in the platform Virtual Library in Health. Results: The consequences of the exhibition to the teratogen depend, among other things, of the moment in which is the gestation process. There are multiple teratogen agents and some risk factors that frequently concomitan with them, for what should be considered in the primary prevention to diminish alterations and congenital defects for these environmental noxas. Final considerations: The knowledge of the effects of the agents teratogen allows to value the possible damage to the fetus.

Polisindactilia preaxil. Presentación de caso / Preaxile polysyndactyly. Case presentation / Polissindactilia do preáxil. Apresentação do caso

RESUMEN La polisindactilia es un defecto congénito que tiene lugar en las manos o en los pies, o en ambos al mismo tiempo y que consiste en la existencia de múltiples dedos supernumerarios además de la fusión de tejido blando y/o óseo entre dedos adyacentes, generando la ausencia total o parcial del espacio entre dos dedos. Se presenta el caso de una paciente femenina de 35 años de edad. No presenta ningún antecedente patológico personal de interés, ni alergias medicamentosas conocidas. Presenta una sindactilia compleja en el dedo pulgar de la mano derecha, con polidactilia preaxil, acompañado de disminución de la movilidad en las articulaciones de este dedo. Esta paciente voluntariamente y con consentimiento informado forma parte del proyecto de investigación "Defectos congénitos del esqueleto apendicular de la institución." Este defecto afecta el 1.2% de los recién nacidos y el 10 % de esta aparición ocurre en las extremidades superiores, siendo la duplicación del pulgar la polidactilia más común de la mano. La polisindactilia es una entidad con muy poca frecuencia, es más común en la población blanca y asiática, en esta entidad es importante el tratamiento temprano para evitar deformidades en el dedo afectado, Idealmente se debe realizar la cirugía entre los 10 y 12 meses de edad.

ABSTRACT Polysyndactyly is a congenital defect that occurs in the hands or feet, or both at the same time and that consists of the existence of multiple supernumerary fingers in addition to the fusion of soft tissue and / or bone between adjacent fingers, generating the total or partial absence of the space between two fingers. The case of a 35-year-old female patient is presented. He does not present any personal pathological history of interest, or known drug allergies. It presents a complex syndactyly in the thumb of the right hand, with preaxial polydactyly, accompanied by decreased mobility in the joints of this finger. This patient voluntarily and with informed consent is part of the research project "Congenital defects of the appendicular skeleton of the institution." This defect affects 1.2% of newborns and 10% of this appearance occurs in the upper extremities, being the duplication of the thumb the most common polydactyly of the hand. Polysyndactyly is an entity with very little frequency, it is more common in the white and Asian population, in this entity early treatment is important to avoid deformities in the affected finger, Ideally, surgery should be performed between 10 and 12 months of age.

RESUMO A polissindactilia é um defeito congênito que ocorre nas mãos ou nos pés, ou ambos ao mesmo tempo e que consiste na existência de múltiplos dedos supranumerários além da fusão de tecidos moles e / ou osso entre os dedos adjacentes, gerando o total ou parcial ausência de espaço entre dois dedos. É apresentado o caso de uma paciente de 35 anos de idade. Ele não apresenta nenhuma história patológica pessoal de interesse ou alergia a medicamentos conhecida. Apresenta sindactilia complexa no polegar da mão direita, com polidactilia pré-axial, acompanhada de diminuição da mobilidade nas articulações deste dedo. Este paciente voluntariamente e com consentimento informado faz parte do projeto de pesquisa "Defeitos congênitos do esqueleto apendicular da instituição". Esse defeito atinge 1,2% dos recém-nascidos e 10% desse aparecimento ocorre nas extremidades superiores, sendo a duplicação do polegar a polidactilia da mão mais comum. A polissindactilia é uma entidade com muito pouca frequência, é mais comum na população branca e asiática, nesta entidade o tratamento precoce é importante para evitar deformidades no dedo afetado. Idealmente, a cirurgia deve ser realizada entre 10 e 12 meses de idade.

Caracterización clínica epidemiológica de los defectos congénitos del tracto genitourinario / Clinical-epidemiological Characterization of Congenital Defects of the Urinary Tract

Introducción: La malformación congénita es una alteración estructural de un órgano o parte de este, que sucede como consecuencia de una alteración durante la morfogénesis y que puede corresponder a defectos menores o mayores, únicos, múltiples o asilados. Objetivo: Caracterizar clínica y epidemiológicamente los defectos congénitos del tracto genitourinario. Métodos: Se realizó un estudio observacional retrospectivo en 453 fetos con diagnóstico por ecografía bidimensional de defecto congénito del tracto genitourinario. Para ello se tomó en cuenta la edad materna y gestacional al diagnóstico, antecedentes personales y familiares de interés clínico genético y la conducta terapéutica según criterio médico. Los datos fueron procesados mediante el Programa Microsoft Excel 2010, aplicándoles el método porcentual y los resultados expuestos en forma de tablas. Resultados: El 75,27 por ciento de las anomalías se presentaron en gestantes con edad materna entre 20 y 34 años. El 62,6 por ciento de los defectos fueron diagnosticados en el segundo trimestre del embarazo, con predominio del sexo masculino en los fetos estudiados. La pielocaliectacia (27,3 por ciento) resultó la principal causa de evaluación inicial seguida de la hidronefrosis (26,2 por ciento). Hubo correlación entre el diagnóstico definitivo por ultrasonido y el resultado de la necropsia. Los casos en seguimiento no presentaron ninguna complicación y solo en nueve gestantes se registró interrupción anterior por defectos genitourinarios. Conclusiones: Se constató aumento progresivo del diagnóstico de anomalías congénitas del tracto genitourinario por años de estudio, las pielocaliectacias bilaterales resultaron la principal causa de evaluación inicial(AU)

Introduction: A congenital malformation is a structural alteration of an organ or part of it, which happens as a consequence of an alteration during morphogenesis and may correspond to minor or major, unique, multiple or isolated defects. Objective: To characterize, clinically and epidemiologically, the congenital defects of the genitourinary tract. Methods: A retrospective observational study was carried out in 453 fetuses diagnosed with a congenital defect of the genitourinary tract by using two-dimensional ultrasound. For this, we considered the maternal and gestational ages at diagnosis, personal and family history of clinical genetic interest, and therapeutic behavior according to medical criteria. The data was processed using the program Microsoft Excel 2010, applying the percentage method and the results presented in tables. Results: 75.27 percent of the anomalies occurred in pregnant women with maternal ages between 20 and 34 years. 62.6 percent of the defects were diagnosed at second trimester of pregnancy, with a predominance of the male sex in the studied fetuses. Pyelocaliectasis (27.3 percent) was the main cause of initial evaluation, followed by hydronephrosis (26.2 percent). There was a correlation between the definitive ultrasound diagnosis and the outcome of the autopsy. The follow-up cases did not present any complications and only nine pregnant women presented a previous interruption due to genitourinary defects. Conclusions: A progressive increase in the diagnosis of congenital anomalies of the genitourinary tract was verified for years of study. Bilateral pyelocaliectasis was the main cause of initial evaluation(AU)

Midline cervical cleft: a case report

A 14-year-old boy presented with complaints of a sinus in the middle of neck, with mucoid discharge present since birth. MRI sinogram along with MRI neck was taken which revealed a sinus tract opening in midline, extending posteriorly and traversing inferiorly to end blindly above sternum. Patient was planned for an elective excision of sinus tract, with z-plasty for decreased scarring. Congenital midline cervical cleft is a rare entity that was first described in 1924. Subsequently there have been fewer than 100 cases reported in medical literature. It typically manifests at birth as a cleft at level of suprasternal notch with a pseudonipple above it. Pathogenesis of congenital midline cervical clefts remains speculative. Differential diagnosis includes thyroglossal duct cysts or branchial cleft anomalies.

Efficacy of MAGPI procedure in the management of hypospadias

Background: Hypospadias is a relatively common congenital defect of male external genitalia. It is present in approximately 1 in 300 males new born. The meatus may be located anywhere along the shaft of the penis from glans to scrotum or even perineum. The objective is to study the efficacy of MAGPI procedure in the management of hypospadias.Methods: Detailed case study was done as per the proforma, in majority of cases patient's mother were informants, thorough clinical examination was done in all cases and looked for any congenital anomalies and family history was also taken, and any drugs intake was also taken. All the cases routine investigation was done like (Hb, BT, Ct, Wt). USG was done in required cases. Routine pre-operative preparation was done like keeping nil orally, preparing parts was done. The type of surgery for each patient was assessed after clinical examination of location of meatus: Anterior, Middle, and Posterior. On discharge, the patients and mothers were advised to bring their children for regular check up to hospital.Results: Most common position of hypospadias was glanular and coronal. The most common surgery performed was Snodgrass technique and for distal and mid penile hypospadias and MAGPI for glanular type of hypospadias. MAGPI procedure was most commonly performed for glanular and coronal type of hypospadias. Other minor Complication was wound infection and penile torsion of mild degree and was managed conservatively.Conclusions: There is significant difference in outcome of hypospadias surgery done by pediatric urologist and other surgeons.

Evaluación del crecimiento y desarrollo de pacientes con síndrome Down en Santiago de Cuba / Evaluation of growth and development of patients with Down syndrome in Santiago de Cuba

Se realizó un estudio descriptivo, transversal y cuantitativo de 54 pacientes con síndrome de Down, atendidos en la consulta de Genética Clínica del Hospital Pediátrico Sur Docente Dr Antonio María Béguez César de Santiago de Cuba, desde el 2000 hasta el 2015, con el objetivo de determinar el patrón de crecimiento de los afectados. Entre las variables analizadas figuraron la edad, el sexo y los índices antropométricos: peso para la edad, talla para la edad, índice peso/talla e índice de masa corporal; asimismo, se evaluaron los defectos congénitos más frecuentes en la muestra. Se concluye que el patrón de crecimiento de dichos pacientes es similar al descrito en la bibliografía médica relacionada con el tema, lo cual puede ser útil para la atención clínica que se les debe brindar, así como también para orientar a los padres sobre los hábitos alimentarios y estilos de vida como parte del tratamiento de los niños y adolescentes con este síndrome

A descriptive, cross-sectional and quantitative study of 54 patients with Down syndrome, assisted in the Clinical Genetics Service of Dr Antonio María Béguez César Teaching Southern Children Hospital was carried out in Santiago de Cuba, from 2000 to 2015, aimed at determining the growth pattern of those affected patients. Among the analyzed variables there were age, sex and anthropometric indexes: weight according to age, height according to age, weight/height index and body mass index; also, the most frequent congenital defects were evaluated in the sample. It was concluded that the growth pattern of these patients is similar to the one described in the medical literature related to the topic, which can be useful for the clinical care that should be offered, as well as to guide the parents on the eating habits and lifestyles as part of the treatment of children and adolescents with this syndrome

Comportamiento clínico epidemiológico de los defectos congénitos en La Habana / Clinical and epidemiological situation of congenital defects in La Habana province

INTRODUCCIÓN: los defectos congénitos son causa importante de morbilidad y mortalidad infantil porque representan un problema de salud global. En Cuba estos defectos constituyen la segunda causa de muerte en niños menores de un año, lo que hace necesario la existencia de sistemas de vigilancia que permitan analizar el comportamiento de estas alteraciones. OBJETIVO: caracterizar clínica y epidemiológicamente a los recién nacidos con defectos congénitos, así como describir el comportamiento de los defectos congénitos diagnosticados prenatalmente por los diferentes programas. MÉTODOS: se realizó un estudio observacional y descriptivo basado en la información del Registro Cubano de Malformaciones Congénitas correspondiente a un periodo de 5 años. Se estudiaron un total de 1 816 recién nacidos con defectos congénitos y 1 238 interrupciones terapéuticas voluntarias, para un total de 3 054 productos de la gestación con defectos congénitos. RESULTADOs: el 81,2 % de estos defectos fueron aislados, con respecto a las malformaciones múltiples los más frecuentes fueron los síndromes. Según el área de residencia, la mayor frecuencia de los defectos en nacidos vivos se presentó en Boyeros, con 272, y predominaron las interrupciones terapéuticas en Arroyo Naranjo con un total de 116. El 14,2 % de la mortalidad en niños menores de un año estuvo asociada a defectos congénitos. CONCLUSIONES: los defectos congénitos más frecuentes son los aislados, dentro de ellos, predominan las cardiopatías congénitas. Con respecto a los defectos congénitos múltiples, los más frecuentes son los síndromes. El municipio de Boyeros presenta el mayor número de nacimientos con defectos congénitos, mientras que el municipio de Arroyo Naranjo presenta el mayor número de interrupciones terapéuticas.

INTRODUCTION: congenital defects are significant causes of infant mortality and morbidity because they represent a global health problem. These defects in Cuba are the second cause of death in children aged less than one year, which makes it necessary to have surveillance systems that allow analyzing the behavior of these disorders. OBJECTIVE: to clinically and epidemiologically characterize the newborns with congenital defects as well as to describe the behavior of such defects diagnosed before birth by the different programs. METHODS: observational and descriptive study based on the information provided by the Cuban Register of Congenital Malformations in a 5 year period. One thousand and eight hundred sixteen newborn with congenital defects and 1 238 voluntary therapeutic abortions were studied for a total number of 3 054 gestational products with congenital defects. RESULTS: in the study, 81.2 % of these defects were single; in the multiple malformations the most common were syndromes. According to the area of residence, the highest frequency of defects in newborns was found in Boyeros municipality with 272 and the voluntary therapeutic abortions predominated in Arroyo Naranjo municipality with 116. The mortality rate of 14.2% in children under one year of age was associated to congenital defects. CONCLUSIONS: the most frequent congenital defects were single, being congenital cardiopathies predominant. Regarding the multiple congenital defects, syndromes were the most common. Boyeros municipality presented the highest number of births with congenital defects whereas Arroyo Naranjo municipality has the highest number of therapeutic abortions.

Laparoscopic repair of congenital pleuroperitoneal hernia using a polypropylene mesh in a dog / Correção laparoscópica de hérnia pleuroperitoneal utilizando malha de polipropileno em cão

Pleuroperitoneal hernias are the most uncommon type of diaphragmatic hernias in dogs and cats. The treatment of choice is surgery and may involve the use of prosthetic implant through celiotomy. In the current report, laparoscopic repair of a congenital pleuroperitoneal hernia using polypropylene mesh in a dog is described. The surgery was feasible. Appropriate reduction of the hernia was carried out and no complications were noted.

Hérnias pleuroperitoneais são o tipo mais incomum de hérnias diafragmáticas em cães e gatos. O tratamento de escolha é cirúrgico e pode envolver o uso de implantes protéticos na abordagem via laparotomia. No presente relato, é descrito o reparo de uma hérnia pleuroperitoneal congênita através de laparoscopia com utilização de malha de polipropileno. A cirurgia foi viável. Houve redução apropriada da hérnia sem observação de complicações.

A case of occult fronto temporo parietal meningoencephalocele in an infant.

We describe a case of an occult fronto temporo parietal meningoencephalocele discovered in a 45 days old infant baby. The most common cause for meningoenphalocele is trauma by any means during birth or during development. But here in our case there is lack of such significant history of trauma and so the possible cause of the lesion may be congenital defect only. Preoperative Clinical, CT and MR images are presented. Lesion was removed surgically with satisfactory post operative recovery.

Anatomical Variant of Atlas : Arcuate Foramen, Occpitalization of Atlas, and Defect of Posterior Arch of Atlas

OBJECTIVE: We sought to examine anatomic variations of the atlas and the clinical significance of these variations. METHODS: We retrospectively reviewed 1029 cervical 3-dimensional (3D) CT images. Cervical 3D CT was performed between November 2011 and August 2014. Arcuate foramina were classified as partial or complete and left and/or right. Occipitalization of the atlas was classified in accordance with criteria specified by Mudaliar et al. Posterior arch defects of the atlas were classified in accordance with criteria specified by Currarino et al. RESULTS: One hundred and eight vertebrae (108/1029, 10.5%) showed an arcuate foramen. Bilateral arcuate foramina were present in 41 of these vertebrae and the remaining 67 arcuate foramina were unilateral (right 31, left 36). Right-side arcuate foramina were partial on 18 sides and complete on 54 sides. Left-side arcuate foramina were partial on 24 sides and complete on 53 sides. One case of atlas assimilation was found. Twelve patients (12/1029, 1.17%) had a defect of the atlantal posterior arch. Nine of these patients (9/1029, 0.87%) had a type A posterior arch defect. We also identified one type B, one type D, and one type E defect. CONCLUSION: Preoperative diagnosis of occipitalization of the atlas and arcuate foramina using 3D CT is of paramount importance in avoiding neurovascular injury during surgery. It is important to be aware of posterior arch defects of the atlas because they may be misdiagnosed as a fracture.

Differentiating between Traumatic Pathology and Congenital Variant: A Case Report of Butterfly Vertebra

Butterfly vertebra is a rare congenital malformation of the spine, which is usually reported in the literature as an isolated finding. We describe a 40-year-old woman that presented to our emergency department with back pain and sciatica. Initial radiological evaluation revealed an incidental finding of a L4 butterfly vertebra in the anteroposterior and lateral view radiographs. The patient presented with no neurological deficit. This rare congenital anomaly is usually asymptomatic, and awareness of its non-traumatic nature is critical in order to establish a correct diagnosis. Further evaluation of the patient is necessary to exclude pathologic fracture, infection, or associated vertebral anomalies and syndromes, such as Alagille, Jarcho-Levin, Crouzon, and Pfeiffer syndromes. Furthermore, in the emergency setting, awareness of this entity is needed so that a correct diagnosis can be established.

Fractura - hundimiento craneal congénita: una realidad obstétrica / Congenital depressed skull fracture: an obstetric reality

Las fracturas craneales congénitas tienen baja incidencia, de una etiología desconocida, generando una gran alarma respecto a la actuación obstétrica en el momento del parto. Se presenta el diagnóstico, exploración radiológica y evolución clínica de dos recién nacidos con fractura-hundimiento craneal intrauterina, uno de ellos con manejo expectante y el otro con manejo quirúrgico. Ambos con buena evolución posterior y sin secuelas neurológicas ni estéticas.

The congenital skull fractures presented a low incidence, unknown aetiology, and it causes great alarm as far as the obstetric actions to be taken at birth are concerned. This work presents the diagnosis, radiology examinations and clinical evolution of two live-born infants with an intrauterine depressed skull fracture, one with expectant management and the other with surgical management. Both neonates showed good subsequent evolution with no neurological and no aesthetic sequelae.

Morbilidad por defectos bucomaxilofaciales en las provincias orientales durante el período 2000-2013 / Morbidity due to oral maxillofacial defects in the eastern provinces during the period 2000-2013

Se realizó un estudio descriptivo y retrospectivo de 2441 pacientes, atendidos en el Servicio de Prótesis Bucomaxilofacial de Santiago de Cuba, desde el 2000 hasta el 2013, con vistas a caracterizar la morbilidad por defectos bucomaxilofaciales en los afectados. Hubo un promedio de 200 ingresos y 159 altas anuales de pacientes atendidos. La mayoría de los casos (63,8 %) pertenecieron a la provincia antes citada. Prevalecieron el sexo masculino (63,0 %), el grupo etario de 60 años y más (33,7 %), el nivel escolar primario (32,6 %), los defectos oculares (80,3 %) y el trauma (53,8 %). En los afectados de 0-19 años predominaron los defectos congénitos, en los de 40-59 los traumas, en personas de más de 60 años los oncológicos y por otras causas. Los defectos oculares se produjeron por traumas en 59,0 %, los nasales por causas oncológicas en 100,0 %, los craneales y auriculares por traumas en 78,6 y 76,5 %, respectivamente.

A descriptive and retrospective study of 2441 patients, assisted in the Service of Oral and Maxillofacial Prosthesis in Santiago de Cuba was carried out from the 2000 to 2013, aimed at characterizing the morbidity due to oral and maxillofacial defects in those affected. There was an average of 200 admissions and 159 annual discharges of assisted patients. Most of the cases (63.8%) belonged to the above mentioned province. Male sex (63.0%), the age group 60 years and over (33.7%), the primary school level (32.6%), the ocular defects (80.3%) and trauma (53.8%) prevailed. In the affected age group 0-19 years, the congenital defects prevailed, in the 40-59 years traumas prevailed, in patients over 60 years the oncological causes and others prevailed. Ocular defects were caused by traumas in 59.0%, the nasal defects were caused by oncological causes in 100.0%, the cranial and ears defects were due to traumas in 78.6 and 76.5%, respectively.

Infarto agudo del miocardio en un recién nacido / Acute myocardial infarction in a newborn

Se describe el caso clínico de un recién nacido que presentó infarto agudo de miocardio con evolución desfavorable y fallecimiento a las 31 horas del nacimiento en el Servicio de Neonatología del Hospital Ginecoobstétrico Docente "Tamara Bunke Bider" de Santiago de Cuba. La autopsia clínica reveló un defecto congénito del trayecto intramiocárdico de la arteria coronaria descendente anterior izquierda en las primeras 24 horas de iniciados los síntomas y choque cardiogénico, como causas básica y directa de muerte, respectivamente.

The case report of a newborn is described, who presented with an acute myocardial infarction with bad progress and death 31 hours after his birth in the Neonatology Department of "Tamara Bunke Bider" Gyneco-obstetrical Teaching Hospital of Santiago de Cuba. The clinical autopsy revealed a congenital defect of intramyocardial bridging of the left anterior descending coronary artery within 24 hours of onset of symptoms and cardiogenic shock as basic and direct causes of death, respectively.

Coloboma palpebral: relato de caso de tratamento cirúrgico / Congenital upper eyelid colobomas: case report of surgical treatment

Coloboma palpebral congênito é um raro defeito embriológico que ocasiona falhas no desenvolvimento palpebral. Pode afetar tanto a pálpebra superior quanto a inferior, uni ou bilateralmente. Sua importância clínica reside no fato de que, em sua localização mais comum, a pálpebra superior, tais defeitos provocam exposição da córnea, o que pode levar a ulcerações e, posteriormente, a cegueira. Existem várias técnicas cirúrgicas reparadoras dos colobomas de pálpebra superior, a escolha da técnica dependerá da extensão do defeito. O diagnóstico imediato e sua posterior reparação poderá evitar futuras complicações. O presente trabalho tem como objetivo a descrição de uma técnica cirúrgica simples e eficaz e uma revisão sobre coloboma palpebral.

Congenital eyelid coloboma is a rare embryonic defect that causes failure in eyelid development. It can affect both the upper and the lower eyelid, uni– or bilaterally. Its clinical significance lies in the fact that, in their usual location – the upper lid –, such defects cause exposure of the cornea, which can lead to ulceration and eventually blindness. There are several surgical techniques to repair upper eyelid colobomas, the choice of technique will depend on the extent of the defect. The immediate diagnosis and subsequent repair may prevent further complications. This paper aims at describing a simple and effective surgical technique and a review of eyelid coloboma.

Retalho miomucoso de bucinador: Relato de caso e revisão de literatura / Buccinator myomucosal flap: Case report and literature review

A reconstrução de pequenos defeitos da região maxilofacial é realizada normalmente com retalhos locais, enquanto os grandes defeitos podem ser reconstruídos com retalhos regionais vascularizados ou com retalhos livres, entretanto defeitos de tamanho moderado, muitas vezes, representam um desafio aos cirurgiões. Uma alternativa é o uso de retalhos compostos por tecidos muscular e mucoso, como o retalho miomucoso do músculo bucinador. Esse retalho apresenta vantagens, como sua anatomia constante, o fornecimento de um volume muscular adequado, uma rápida cicatrização e uma menor morbidade pós-operatória, além de sua boa capacidade de rotação, que permite recobrir a maioria dos defeitos ipsilaterais do palato e da maxila, devendo possuir um local de destaque na escolha da técnica cirúrgica adequada para a correção de defeitos orais de tamanho moderado. O objetivo deste trabalho é relatar um caso no qual se utilizou o retalho miomucoso de bucinador para a reconstrução de um defeito na região de palato duro assim como discutir os aspectos mais importantes dessa técnica cirúrgica.

The reconstruction of small defects in the maxillofacial region is usually done with local flaps, whereas large defects are reconstructed with a variety of vascularized regional flaps or free flaps, however, defects of moderate size many times represent a challenge to the surgeon. An alternative is the use of flaps that contain both muscle and mucosal tissue, such as the buccinator myomucosal flap. This flap presents advantages like its constant anatomy, the possibility of providing sufficient muscle bulk, a good healing and a minimal post-operatory morbidity, besides its reliable arc of rotation, which can cover most ipsilateral defects of the palate and maxilla, leading to this technique a preferential place for the reconstruction of moderate size defects of the oral cavity. The aim of this paper is to report a case in what the buccinator myomucosal flap was used to reconstruct a moderate defect in the hard palate region, as well as to discuss the most important aspects of this surgical technique.

Superdominant right coronary artery with absent left circumflex artery

Noninvasive imaging of coronary artery disease is rapidly replacing angiography as the first line of investigation. Multislice CT is the non-invasive modality of choice for imaging coronary artery disease and provides high speed with good spatial resolution. CT coronary angiography in addition to detecting and characterising atherosclerotic coronary artery disease is also a good imaging tool for evaluating anomalies of coronary arteries. Superdominant right coronary artery with absent left circumflex artery is one such rare coronary artery anomaly which is well evaluated with multislice CT angiography. The authors report one such case of superdominant right coronary artery with absent left circumflex artery imaged with 64-slice MDCT.

The Incidence and Clinical Implications of Congenital Defects of Atlantal Arch

OBJECTIVE: Atlantal arch defects are rare. Few cadaveric and imaging studies have been reported on the variations of such anomalies. Our goal in this study was to examine the incidence and review the clinical implications of this anomaly. METHODS: A retrospective review of 1,153 neck or cervical spine computed tomography (CT) scans was performed to identify patients with atlantal arch defects. Neck CT scans were performed in 650 patients and cervical spine CT scans were performed in 503 patients. Posterior arch defects of the atlas were grouped in accordance with the classification of Currarino et al. In patients exhibiting this anomaly, special attention was given to defining associated anomalies and neurological findings. RESULTS: Atlantal arch defects were found in 11 (11/1153, 0.95%) of the 1,153 patients. The type A posterior arch defect was found in nine patients and the type B posterior arch defect was found in two patients. No type C, D, or E defects were observed. One patient with a type A posterior arch defect had an anterior atlantal-arch midline cleft (1/1153, 0.087%). Associated cervical spine anomalies observed included one C6-7 fusion and two atlantal assimilations. None of the reviewed patients had neurological deficits because of atlantal arch anomalies. CONCLUSION: Most congenital anomalies of the atlantal arch are found incidentally during investigation of neck mass, neck pain, radiculopathy, and after trauma.