Descripción angiográfica de los aspectos anatómicos y clínicos de la arteria interventricular anterior en un grupo de personas colombianas

Introducción. La arteria interventricular anterior se origina en la coronaria izquierda, irriga la cara anterior de los ventrículos, el ápex y el tabique interventricular; es la segunda arteria más relevante del corazón. Objetivo. Describir las características anatómicas y clínicas de la arteria interventricular anterior mediante angiografía. Materiales y métodos. Se realizó un estudio descriptivo con 200 reportes angiográficos de personas colombianas; se valoraron el origen, el trayecto y la permeabilidad de la arteria interventricular anterior, así como la dominancia coronaria. Se incluyeron datos relacionados con dolor precordial, infarto agudo de miocardio, dislipidemia y alteración electrocardiográfica. No fue posible hacer pruebas estadísticas, debido a la escasa prevalencia de variaciones anatómicas de dicha arteria. Resultados. Se encontró una arteria interventricular anterior con su origen en el seno aórtico izquierdo, sin puente miocárdico, sin alteración de la permeabilidad y con dominancia izquierda. La frecuencia de los puentes fue del 2 % y la dominancia más frecuente fue la derecha en el 86 %. Se presentaron alteraciones de permeabilidad en el 43 % de los casos, las cuales afectaron principalmente al S13. El 25 % de los pacientes presentó dolor precordial; el 40 %, alteraciones ecocardiográficas; el 5 %, cardiopatía isquémica, y el 59 %, alguna alteración electrocardiográfica. Conclusiones. Las variaciones en el origen de la arteria interventricular anterior son poco prevalentes, según reportes de Chile, Colombia y España. Los puentes miocárdicos de esta arteria fueron escasos respecto a otros estudios, lo cual sugiere mejor especificidad de los hallazgos de la angiotomografía o de la disección directa. La permeabilidad coronaria se valora con la escala TIMI (Thrombolysis in Myocardial Infarction); puntajes de 0 y 1 indican una lesión oclusiva asociada con cardiopatía isquémica. La dominancia coronaria más frecuente, según diversas técnicas, es la derecha, seguida de la izquierda en hombres y de una circulación balanceada en mujeres.

Introduction. The anterior interventricular artery originates from the left coronary artery and irrigates the anterior surface of the ventricles, apex, and interventricular septum, making it the second most relevant artery of the heart. Objective. To describe the anatomical and clinical aspects of the anterior interventricular artery through angiography. Materials and methods. A descriptive study was conducted using 200 angiographic reports of Colombian individuals. The anterior interventricular artery's origin, course, patency, and coronary dominance were evaluated. Data related to chest pain, acute myocardial infarction, dyslipidemia, and electrocardiographic abnormalities were included. Statistical tests could not be performed due to this artery's low prevalence of anatomical variations. Results. One anterior interventricular artery was found to have originated from the left coronary sinus without a myocardial bridge, with no alteration in permeability, and with left dominance. The frequency of bridges was 2%, and the most frequent dominance was right in 86; permeability alterations occurred in 43% mainly affecting S13. Twenty-five per cent presented chest pain; 40%, echocardiographic alterations; 5%, ischemic heart disease, and 59%, electrocardiographic alterations. Conclusions. Variations of origin of the anterior interventricular artery have a low prevalence according to reports from Chile, Colombia, and Spain. anterior interventricular artery myocardial bridges were scarce compared to other studies, suggesting better specificity of computed tomography angiography or direct dissection for these findings. The assessment of coronary permeability is graded with the thrombolysis in myocardial infarction scale; values 0 and 1 indicate occlusive lesion associated with ischemic heart disease. According to various techniques, the most frequent coronary dominance the right, followed by the left in men and balanced circulation in women.

Manejo de la hemorragia posparto en una paciente con útero bicorne con la sutura B-Lynch. Reporte de caso y revisión de la literatura / Management of postpartum hemorrhage in a patient with bicornuate uterus using the B-Lynch suture. Case report and review of the literature

Objetivos: Describir un caso de manejo con sutura de B-Lynch en una mujer con útero bicorne que presenta hemorragia posparto (HPP), y realizar una revisión de la literatura acerca de las estrategias de control quirúrgico de la HPP en pacientes con malformaciones müllerianas, los resultados maternos en términos de control de la hemorragia, así como las complicaciones tempranas y tardías. Materiales y métodos: Se reporta el caso de una paciente con útero bicorne, atendida en un hopsital de referencia regional, que, posterior a parto por cesárea, presenta hemorragia posparto, la cual es controlada con éxito empleando la sutura de B-Lynch. Se realizó una búsqueda en las siguientes bases de datos: PubMed, Embase, Medline, Google Scholar y LILACS. Se emplearon los términos MeSh: "Uterine Atony", "Postpartum Hemorrhage", "Immediate Postpartum Hemorrhage", "Bicornuate Uterus", "Mullerian Anomalies", "Mullerian Ducts Abnormalities", "Uterine Compression Suture". Se incluyeron reportes y series de casos que hubieran incluido pacientes con malformaciones müllerianas con HPP que no respondieran al manejo farmacológico inicial, con uso de procedimientos quirúrgicos conservadores para el control de la hemorragia. Se hace análisis narrativo de los hallazgos respecto a las características de los estudios, técnicas utilizadas y complicaciones. Resultados: Se seleccionaron 5 estudios, 4 de tipo reporte de caso y 1 serie de casos, que corresponden a 12 mujeres con malformación uterina que desarrollaron HPP, en las cuales se empleó manejo quirúrgico (suturas de compresión uterina), y con dispositivo (tipo balón intrauterino) para el control de la hemorragia, logrando controlar el sangrado en 11 casos, solo una (8,3 %) requirió histerectomía. No se reportan complicaciones a largo plazo. Conclusiones: El manejo de la HPP en mujeres con malformaciones uterinas müllerianas cuenta con una literatura limitada a reportes de casos, con uso tanto de suturas compresivas como dispositivos médicos tipo balones intrauterinos. La sutura compresiva tipo B-Lynch parece ser una buena alternativa en el control de la HPP en estas mujeres con el fin de preservar la fertilidad, con baja tasa de complicaciones. Se requiere continuar documentando este tipo de casos con el fin de consolidar evidencia de la utilidad de esta técnica en el control del sangrado uterino posparto en esta población.

Objectives: To describe the use of the B-Lynch suture in a case of postpartum hemorrhage of a woman with bicornuate uterus, and to carry out a review of the literature on PPH control strategies in patients with müllerian anomalies, maternal outcomes in terms of hemorrhage control, as well as early and late complications. Material and methods: Case report of a patient with bicornuate uterus who presented to a regional referral hospital with postpartum hemorrhage following a cesarean section, which was successfully controlled using the B-Lynch suture. A search was conducted in the PubMed, Embase, Medline, Google Scholar and LILACS databases. The MeSh terms used were: "Uterine Atony," "Postpartum Hemorrhage," "Immediate Postpartum Hemorrhage," "Bicornuate Uterus," "Müllerian Anomalies," "Müllerian Duct Abnormalities". Case reports and case series of patients with müllerian malformations and PPH not responding to initial pharmacological management in whom conservative surgical procedures were used to control bleeding were included. A narrative analysis of the findings was carried out based on study characteristics, techniques used and complications. Results: Five studies were selected, 4 case reports and 1 case series which included 12 women with uterine malformations who developed PPH and in whom surgical management (uterine compression sutures) or devices (intrauterine balloon) were used for hemorrhage control. Bleeding was successfully controlled in 11 cases, with hysterectomy required only in one case (8.3 %). No long-term complications were reported. Conclusions: The literature on PPH management in women with müllerian uterine malformations is limited to case reports in which either compression sutures or medical devices such as intrauterine balloons were used. The B-Lynch-type compression suture appears to be a good option for controlling PPH in these women in order to preserve fertility, with a low rate of complications. Further documentation of these types of cases is needed in order to build the evidence regarding the usefulness of this technique for controlling postpartum uterine bleeding in this population.

Riñón pélvico fusionado con uréter único, hidrometrocolpos y ano imperforado: conjunción de malformaciones infrecuentes en una adolescente / Pancake kidney with a single ureter, hydrometrocolpos, and imperforate anus: a set of uncommon malformations in a female adolescent

Una niña de 11 años de edad con antecedentes de ano imperforado, infección urinaria y episodios de constipación intermitentes se presentó a la consulta con cólicos abdominales y náuseas de una semana de evolución. Estudios radiológicos revelaron hidrometrocolpos y fusión renal pélvica con uréter único hidronefrótico. El examen vaginal evidenció un tabique transverso no permeable. Se evacuó temporalmente la colección con resolución de los síntomas. La paciente fue programada para cirugía vaginal reconstructiva definitiva. Se destaca en este caso no solo la asociación de malformaciones infrecuentes, sino una sintomatología muy común en la práctica pediátrica a causa de una patología rara vez considerada en el diagnóstico diferencial, y la importancia de una evaluación precoz y completa de este tipo de malformaciones para un tratamiento oportuno.

An 11-year-old girl with a history of imperforate anus, urinary tract infection, and intermittent episodes of constipation presented with abdominal pain and nausea for 1 week. The x-rays revealed hydrometrocolpos and fused pelvic kidney with a single hydronephrotic ureter. The vaginal examination revealed a non-permeable transverse vaginal septum. The collection was temporarily drained and symptoms resolved. The patient was scheduled for definitive vaginal reconstructive surgery. In this case, it is worth noting the association of infrequent malformations and also the signs and symptoms very common in pediatric practice due to a pathology rarely considered in the differential diagnosis, and the importance of an early and complete assessment of this type of malformations for a timely treatment.

Radial peripapillary capillary network in optic disc anomalies with abnormal tissues on disc surface

Background: Optic disc anomalies with abnormal tissue on the disc surface includes, myelinated nerve fiber, optic disc drusen, and Bergmeister papillae. Imaging the radial peripapillary capillary (RPC) network in optic disc anomalies with optical coherence tomography–angiography (OCTA) can give information on the RPC network in these conditions. Purpose: This video describes the OCTA of optic nerve head and RPC network using the angio disc mode in cases of optic disc anomalies with abnormal tissue on the disc surface. Synopsis: This video presents characteristic features of RPC network in one eye each of myelinated nerve fiber, optic disc drusen, and Bergmeister papillae. Highlights: OCTA in optic disc anomalies with abnormal tissue on the disc surface show a dense RPC microvascular network. OCTA is an effective imaging modality to study vascular plexus/RPC and their alteration in these disc anomalies

Need of optical aids for schools for blind students in Pune and Nasik districts and compliance of their use

Purpose: To assess the need for spectacles and low?vision aids (LVA) in students attending schools for the blind and study their compliance with use. Methods: Comprehensive ocular evaluation was performed using a hand?held slit lamp and ophthalmoscope. Vision acuity was measured using a logarithm of the minimum angle of resolution (logMAR) chart for distance, as well as for near. Spectacles and LVAs were dispensed after refraction and LVA trial. Follow?up was performed to assess the vision using the LV Prasad Functional Vision Questionnaire (LVP?FVQ) along with compliance with use after 6 months. Results: Of the 456 students examined from six schools, 188 (41.2%) were female; 147 (32.2%) were <10 years of age. In all, 362 (79.4%) were blind since birth. The students dispensed only LVAs were 25 (5.5%), only spectacles were 55 (12.1%), and both spectacle and LVAs were 10 (2.2%). The vision improved using LVAs in 26 (5.7%) and using spectacles in 64 (9.6%). There was a significant improvement in LVP?FVQ scores (P < 0.001). Also, 68/90 students were available for follow?up, of whom 43 (63.2%) were compliant to use. Causes of not wearing spectacles or LVA in 25 were left somewhere or lost 13 (52%), broken 3 (12%), uncomfortable to use 6 (24%), not interested to use 2 (8%), and got operated 1 (4%). Conclusion: Although the dispensing of LVA and spectacles improved the visual acuity and vision function of 90/456 (19.7%) students, nearly a third were not using them after 6 months. Efforts need to be taken to improve the compliance of use

Actualización en anomalías vasculares de la órbita en pediatría: diagnóstico por imágenes y tratamiento / Update on orbital vascular anomalies in pediatrics: imaging studies and management

Las anomalías vasculares de la órbita (AVO) son un grupo heterogéneo de patologías que pueden presentarse con frecuencia en el cono orbitario, la región periorbitaria o dentro de la órbita misma. Las AVO se dividen en tumores y malformaciones. Su presentación clínica más frecuente es el exoftalmos, asociado o no a alteración del eje visual. Además, pueden presentar complicaciones agudas, como hemorragia intralesional o celulitis entre las más frecuentes, y complicaciones crónicas, como ambliopía y afectación de la agudeza visual a largo plazo. La evolución de las técnicas de imágenes, el uso de nuevos fármacos y la utilización de innovadores procedimientos en radiología intervencionista han posibilitado obtener una mejora significativa en los procesos diagnósticos y terapéuticos de estos pacientes, permitiendo un diagnóstico y tratamiento preciso.

Orbital vascular anomalies (OVAs) are a heterogeneous group of disorders frequently found in the orbital cone, the periorbital region, or within the orbit itself. OVAs are divided into tumors and malformations. The most frequent clinical presentation is exophthalmos, associated or not with an alteration of the visual axis. They may also cause acute complications, being intralesional bleeding or cellulitis the most frequent, and chronic complications, such as amblyopia and long-term visual acuity impairment. The development of imaging techniques, the use of new drugs, and the implementation of innovative procedures in interventional radiology have resulted in a significant improvement in the diagnostic and therapeutic approaches to these patients, essential to an accurate diagnosis and management.

Tendência temporal da prevalência e mortalidade infantil das anomalias congênitas no Brasil, de 2001 a 2018 / Temporal trends in prevalence and infant mortality of birth defects in Brazil, from 2001 to 2018

Resumo As anomalias congênitas (AC) configuram um relevante problema para a saúde pública global, afetando em média de 3% a 6% dos recém-nascidos em todo o mundo. No Brasil, ocupam a segunda posição entre os principais grupos de causas de óbito infantil. Assim, estudos amplos são necessários para mostrar o impacto das AC na saúde infantil. O presente estudo descreve a tendência temporal da prevalência e da mortalidade infantil por AC entre nascidos vivos (NV) no Brasil e em suas cinco regiões de 2001 a 2018, utilizando dados vinculados entre as bases de dados do Sistema de Informações sobre Nascidos Vivos (SINASC) e do Sistema de Informações sobre Mortalidade (SIM). A prevalência e mortalidade infantil por AC mostrou-se crescente no Brasil na maioria das regiões, principalmente no Norte e no Nordeste. Aquelas do aparelho osteomuscular foram as mais prevalentes ao nascimento (29,8/10.000 NV); as do aparelho circulatório passaram para a segunda posição (12,7/10.000 NV) após a vinculação das bases e representam a primeira causa de morte desse grupo. A técnica de vinculação de dados aplicada corrigiu a prevalência nacional das AC em 17,9% no período analisado, após serem recuperadas as AC notificadas no SIM, mostrando ser uma boa ferramenta para melhorar a qualidade das informações das AC.

Abstract Congenital anomalies (CA) are a relevant problem for global public health, affecting about 3% to 6% of newborns worldwide. In Brazil, these are the second main cause of infant mortality. Thus, extensive studies are needed to demonstrate the impact of these anomalies on births and deaths. The present study describes the temporal trends of prevalence and infant mortality due to CA among live births in Brazil and regions, from 2001 to 2018, using the related data between the Live Birth Information System (SINASC, acronym in Portuguese) and the Mortality Information System (SIM, acronym in Portuguese). The prevalence and infant mortality due to CA has increased in Brazil and in most regions, especially in the Northeast and North. CAs in the musculoskeletal system were the most frequent at birth (29.8/10,000 live births), followed by those in the circulatory system (12.7/10,000 live births), which represented the primary cause of death in this group. The applied linkage technique made it possible to correct the national prevalence of CA by 17.9% during the analyzed period, after retrieving the anomalies reported in SIM, thereby proving to be a good tool to improve the quality of information on anomalies in Brazil.

Anomalous aortic origin of the left coronary artery / Origen aórtico anómalo de la arteria coronaria izquierda

Abstract We present an unusual case of an anomalous left coronary artery arising from the contralateral sinus of Valsalva: a 63-year-old male patient who consulted to our emergency department with 1-week history of progressive dyspnea on exertion with clinical signs of heart failure, associated with lung congestion on the chest X ray and elevated NT-proBNP levels (2000 pg/ml; normal value <150). Doppler echocardiography showed severe dilation of both left atrium and left ventricle, with severe deterioration of LV systolic function (Ejection fraction of 26%), global hypokinesia and a moderate mitral regurgitation with central jet. A cardiac cath eterization was performed, which evidenced an anomalous origin of the left main coronary artery from the right coronary sinus with a proximal lesion of nearly 50%. A coronary computed tomographic angiography confirmed the diagnosis of an anomalous origin with an intramyocardial path at the level of the interventricular septum, as sociated with moderate extrinsic compression. To determine the degree of functional ischemia presented by the left main coronary artery lesion we performed a fractional flow reserve evaluation, resulting in 0.75, which was ranked as significant. An angioplasty with implantation of a drug-eluting stent (with Everolimus) was performed successfully to the target lesion. The patient evolved favorably during hospitalization and was discharged from the medical center to continue outpatient follow-up. Patient remained asymptomatic at 1-month and 6 months, during clinical evaluation, without evidence of ischemia on noninvasive functional assessment.

Resumen Presentamos un raro caso de nacimiento anómalo de arteria coronaria izquierda en el seno de Valsalva contralateral. Se trata de un hombre de 63 años que consultó al servicio de emergencias de nuestro centro por disnea progresiva de una semana de evolución, con signos clínicos de insuficiencia cardíaca, aso ciado a signos de congestión en la radiografía de tórax, y valores de NT-proBNP elevados (2000 pg/ml; valor normal <150). El ecocardiograma Doppler evidenció dilatación grave de la aurícula y del ventrículo izquierdo, con deterioro grave de la función sistólica (fracción de eyección de 26%), hipoquinesia global e insuficiencia mitral moderada con jet central. Se realizó una cinecoronariografía que evidenció el nacimiento anómalo del tronco de arteria coronaria izquierda desde el seno coronario derecho, con una lesión cercana al 50%. Una angiotomografía coronaria confirmó el origen anómalo del vaso coronario, con trayecto intramiocárdico a nivel del septum interventricular asociado a compresión extrínseca moderada. Para determinar el grado de isquemia funcional que presentaba la lesión del tronco coronario izquierdo se evaluó la reserva de flujo fraccional, que arrojó un resultado de 0.75 el cual se consideró significativo, prosiguiendo a angioplastia con implante de stent liberador de droga (con Everolimus) a dicha lesión. El paciente evolucionó favorablemente durante la internación en el hospital, egresando de la institución para continuar seguimiento ambulatorio. Persistió asintomático en los controles realizados al mes y a los 6 meses, sin evidencia de isquemia en la evaluación funcional no invasiva.

Etiology of Hemoptysis in Children and Adolescents – Multicentric Retrospective Data (2012-2021) From Chennai

Objective: To analyze the etiology of hemoptysis in children and to correlate the severity of bleed with the etiology. Methods: This retrospective multicentre study reviewed data from inpatient units of four tertiary care public and private sector pediatric hospitals in Tamil Nadu. Methods: Inpatient case records of children (aged 2 month-15 years) treated for hemoptysis at the four institutions between April, 2012 and March, 2021 were identified, after ethical clearance from respective institutions. Data of children with underlying known bleeding disorders like hemophilia or platelet abnormality were excluded from the study. Hemoptysis was categorized as mild, moderate and severe Results: Of the 73 children who had presented with hemoptysis during the study period, 60 (82.2%) children had mild, 9 (12.3%) had moderate and rest had severe hemoptysis. Idiopathic pulmonary hemorrhage was the most common cause of hemoptysis. The common causes of mild hemoptysis in children were idiopathic pulmonary hemorrhage (n=15, 25%), pulmonary tuberculosis (n=12, 20%) and pneumonia (n=8, 13.3%). Congenital airway anomalies and vascular anomalies were more likely to present with moderate to severe bleed. Conclusion: Etiology of hemoptysis is broad and categorizing them into mild, moderate and severe may give a clue about the possible etiology, there by restricting to the required investigations.

Nexus of ocular motility and dry eye

Purpose: To evaluate the effectiveness of vision therapy (VT) in patients with chronic presumed refractory dry eye disease (DED) and concurrent nonstrabismic binocular vision anomalies (NSBVAs). To propose an algorithmic approach to manage patients with refractory DED. Methods: Thirty?two patients with chronic (>1 year) presumed refractory DED and NSBVA were prospectively evaluated. The baseline dry eye evaluation and comprehensive orthoptic evaluation were done. VT was administered by a trained orthoptist for 2 weeks. The binocular vision (BV) parameters and percentage subjective improvement were assessed after the VT. Results: On evaluation, 12 patients (37.5%) had both DED and NSBVA, and 20 patients (62.5%) had only NSBVA. Twenty?nine patients (90.62%) showed significant improvement in BV parameters following VT. Binocular near point of accommodation (median, range) improved from 17 (8–40) to 12 (5–26) mm (P value < 0.0001), and near point of convergence (median, range) improved from 6 (3–33) to 6 (5– 14) (P value 0.004) with VT. Thirty?one patients (96.87%) reported symptomatic improvement after VT, and 62.5% of these showed more than 50% improvement in symptoms. Conclusion: The present study confirms the beneficial role of VT in the treatment of patients with DED with concurrent NSBVA. It is essential to diagnose and treat NSBVA in patients with DED to ensure complete relief of symptoms and patient satisfaction. As there is a significant overlap between symptoms of dry eye disease and that of NSBVA, a complete orthoptic evaluation is recommended in all patients presenting with refractory dry eye disease related symptoms

Does Postpartum Hypopituitarism Influence the Craniofacial Pattern? A Case-Control Study with Sheehan's Syndrome Patients

This study aimed to assess the craniofacial morphologic aspects of Sheehan's syndrome (SHS) patients.An observational study was performed with 19 women diagnosed with SHS and 19 controls matched by age and sex. Lateral cephalometric radiographs were obtained, and 30 linear and angular measurements were analyzed using the Radiocef Studio 2 software. The mean age of patients was 65.47 ± 10.19 years. The main findings were propositioned maxilla (52.63 %) and mandible (52.63 %) relative to the cranial base, mandibular prognathism in 73.68 %, deep growth pattern in 42.1 %, increased mandibular plane in 36.84 %, and reduction in anterior facial height. The SHS group showed statistically significant differences in SNB (p=0.026), N-Me (p=0.006), soft palate length (p=0.011), and Ena-Me (p<0.001) in comparison with controls. The standard deviation score analysis revealed altered values in relation to total maxillary and mandibular lengths. SHS showed altered craniofacial morphology, characterized by maxillo- mandibular prognathism, brachyfacial type, increased mandibular plane, and reduction in soft palate length. This study reports novel findings in SHS.

Este estudio tuvo como objetivo evaluar los aspectos morfológicos craneofaciales de los pacientes con síndrome de Sheehan (SHS). Se realizó un estudio observacional con 19 mujeres diagnosticadas con SHS y 19 controles asociados por edad y sexo. Se obtuvieron radiografías cefalométricas laterales y se analizaron 30 medidas lineales y angulares mediante el software Radiocef Studio 2. La edad media de los pacientes fue de 65,47 ± 10,19 años. Los principales hallazgos fueron proposición maxilar (52,63 %) y mandíbula (52,63 %) con respecto a la base del cráneo, prognatismo mandibular en 73,68 %, patrón de crecimiento profundo en 42,1 %, aumento del plano mandibular en 36,84 % y reducción de la altura facial anterior. El grupo SHS mostró diferencias estadísticamente significativas en SNB (p=0,026), N-Me (p=0,006), longitud del paladar blando (p=0,011) y Ena-Me (p<0,001) en comparación con los controles. El análisis de la puntuación de la desviación estándar reveló valores alterados en relación con las longitudes maxilares y mandibulares totales. El SHS mostró una morfología craneofacial alterada, caracterizada por prognatismo maxilomandibular, tipo braquifacial, aumento del plano mandibular y reducción de la longitud del velo del paladar. Este estudio informa hallazgos novedosos en SHS.

Radial peripapillary capillary network in congenital cavitary optic disk anomalies

Background and Purpose: "Congenital cavitary optic disk anomalies" is a term used to include optic disk pit (ODP), optic disk coloboma, and morning glory disk anomaly (MGDA). Imaging the radial peripapillary capillary (RPC) network in congenital optic disk anomalies with optical coherence tomography?angiography (OCTA) can shed light on its pathogenesis. This video describes the OCTA findings of optic nerve head and RPC network using the angio?disk mode in five cases of congenital cavitary optic disk anomalies. Synopsis: The video presents characteristic RPC network alterations in two eyes of ODP, one eye of optic disk coloboma, and two eyes of noncontractile MGDA. Highlights: OCTA in ODP and coloboma shows absence of RPC microvascular network and a region of capillary dropout. This finding is in contrast to MGDA, where the microvascular network is dense. OCTA is an effective imaging modality to study vascular plexus and RPC and their alteration in congenital disk anomalies, which could provide information about the structural differences among them.

Testicular Volume of Boys Aged 5-17 Years in Relation to Sexual Maturity Rating and Clinical Onset of Puberty in an Urban Setting in Gujarat, India

Background : Assessment of Sexual Maturity Rating and Testicular Volume are indispensable in the routine assessment of puberty in boys. There is paucity of data in Indian population for Testicular Volume particularly in early adolescence. Aims : The aims of the study were to collect data for testicular volume,correlate testicular volume with Sexual Maturity Rating (SMR) and the clinical onset of puberty; and to identify Testicular abnormalities in boys aged 5 to 17 years in an Urban setting in Gujarat, India. Materials and Methods : A prospective observational study was undertaken in boys aged5 to 17years of age from Gujarat from April, 2019 to August, 2019. Mean Testicular Volume was measured with a Prader抯 orchidometer. Parameters like Age, Weight and Height were also measured and Body Mass Index (BMI) was calculated. Pubertal stage was categorized using Tanner staging. Data was statistically analyzed using Microsoft Excel and SPSS software. Results : 977 boys were included in the study. Mean age at SMR stage 2 was 11.22 years. SMR stage 2 was earliest seen at 6 years and latest at 15 years of age. 15% of boys in pre-adolescence, 60% in early adolescence and 94% in middle adolescence showed changes of Puberty. Precocious puberty was detected in 33 boys (3.38%). Delayed Puberty was detected in 4 boys (0.4%) and Undescended Testes in 4 boys (0.4%). Testicular Volume showed positive correlation with Weight, Height and BMI.

Diagnóstico antenatal de cardiopatías congénitas por telemedicina: experiencia en CERPO, 2017-2022 / Antenatal diagnosis of congenital heart disease by telemedicine: experience in CERPO, 2017-2022

Introducción: Las cardiopatías congénitas son las anomalías más frecuentes y la principal causa de muerte infantil y neonatal. El diagnóstico prenatal mejora el resultado perinatal determinando el lugar de nacimiento y el nivel de cuidado neonata. La telemedicina mediante videoconferencia en tiempo real permite mejorar la precisión diagnóstica y planificar el nacimiento. Objetivo: Determinar el diagnóstico y manejo perinatal de fetos con sospecha de cardiopatía congénitas, evaluadas a través de telemedicina en tiempo real atendidas en CERPO en el periodo 2017-2022. Material y métodos: Estudio retrospectivo de las evaluaciones mediante telemedicina en tiempo real realizadas en CERPO entre los años 2017 a 2022. Se revisó el resultado perinatal y se compararon los diagnósticos pre y postnatales, extraídos de la base de datos CERPO y Unidad de Neonatología del Hospital Luis Tisné Brousse. Resultados: La correlación del diagnóstico de cardiopatía congénita mediante telemedicina es de un 81,8% y de 89,8% con el diagnostico posnatal. Conclusiones: La evaluación por medio de telemedicina permite mejorar la precisión diagnostica de la cardiopatía congénita en áreas con escaso acceso a operadores experimentados en evaluación cardiaca fetal. Esto minimiza el impacto económico y social asociado al manejo perinatal de un feto con cardiopatía congénita en nuestro país.

Introduction: Congenital heart disease is the most common anomaly and the leading cause of infant and neonatal death. Prenatal diagnosis improves perinatal outcomes by choosing the right place of birth and level of neonatal care. Telemedicine by videoconferencing in real-time allows for improved diagnostic accuracy and birth planning. Objective: To determine the diagnosis and perinatal management of fetuses with suspected congenital heart disease, evaluated by telemedicine at CERPO in the period 2017-2022. Material and Methods: Retrospective study of evaluations via real-time videoconferencing performed at CERPO between 2017-2022. The perinatal outcome was reviewed, and pre and postnatal diagnoses were compared. The data was extracted from the CERPO database and the Neonatology Unit of the Luis Tisné Brousse Hospital. Results: The correlation of congenital heart disease diagnosis by telemedicine was 81.8% and 89.8% with postnatal diagnosis. Conclusions: Telemedicine assessment improves the diagnostic accuracy of congenital heart disease in areas with poor access to an experienced fetal cardiac specialist. This minimizes the economic and social impact associated with our countrys perinatal management of a fetus with congenital heart disease.

Seguimiento de dilatación del tracto urinario neonatal diagnosticada en la evaluación de infección del tracto urinario

Objetivo: Mostrar las características y seguimiento de la dilatación del tracto urinario en recién nacidos con infección del tracto urinario. Métodos: Estudio observacional, descriptivo, realizado desde 1992 hasta 2019, en neonatos ingresados con infección del tracto urinario. A todos se les realizó ultrasonido renal y uretrocistografía miccional. Se analizaron las características clínicas y de radioimagen de presentación en el seguimiento de la dilatación del tracto urinario. Resultados: Se estudiaron 403 pacientes. La mediana de seguimiento fue de 13 meses. El ultrasonido renal resultó positivo para dilatación del tracto urinario en 148 (36,7 %). Predominaron los grados de dilatación ligera (5-10 mm.) sobre grados moderados y severos. En 38 casos (9,4 %) la dilatación del tracto urinario se asoció a reflujo vésico-ureteral. La condición más común fue la dilatación transitoria con una mediana de desaparición de la dilatación 5 meses. Hubo asociación estadística significativa de la desaparición de la dilatación del tracto urinario con el grado de esta y entre aquellos casos calificados de transitoria con los otros portadores de alguna anomalía del tracto urinario que también tuvieron desaparición de la dilatación en algún momento de su evolución. Conclusiones: Cerca de una tercera parte de los neonatos con infección del tracto urinario tuvieron dilatación del tracto urinario en ultrasonido renal, con predominio de grado ligero, como probable expresión de una anomalía del tracto urinario subyacente. Con frecuencia ocurre resolución de la dilatación del tracto urinario en aquellos casos no asociados con anomalía del tracto urinario, habitualmente dentro del primer año de vida.

OBjective: To show the characteristics and follow-up of urinary tract dilatation in neonates with urinary tract infection. Methods: Observational, descriptive study performed from 1992 to 2019, in neonates admitted with urinary tract infection. All underwent renal ultrasound and voiding urethrocystography. The clinical and radioimaging features of presentation were analyzed in the follow-up of urinary tract dilatation, Results: 403 patients were studied. The median follow-up was 13 months. Renal ultrasound was positive for urinary tract dilatation in 148 (36.7%). Mild degrees of dilatation (5-10 mm) predominated over moderate and severe degrees. In 38 cases (9.4%) urinary tract dilatation was associated with vesico-ureteral reflux. The most common condition was transient dilatation with a median disappearance of dilatation 5 months. There was significant statistical association of the disappearance of urinary tract dilatation with the degree of dilatation and between those cases qualified as transient with the other carriers of some urinary tract anomaly who also had disappearance of dilatation at some point in their evolution. Conclusions: About one third of neonates with urinary tract infection had dilatation of the urinary tract on renal ultrasound, predominantly of mild degree, probably expression of an underlying anomaly. Resolution of dilatation frequently occurs in those cases not associated with urinary tract anomaly, usually within the first year of life.

Dental anomalies in individuals with osteogenesis imperfecta: a systematic review and meta-analysis of prevalence and comparative studies

Abstract Background Osteogenesis imperfecta (OI) is a rare genetic disorder primarily caused by mutations in the genes involved in the production of type 1 collagen. OI is also known as brittle bone disease. Objective This study aims to describe the prevalence of dental anomalies (except dentinogenesis imperfecta) in individuals with OI, and compare the prevalence of dental anomalies between individuals with and without OI and between individuals with different types of OI. Search methods Searches in PubMed, Web of Science, Scopus, Ovid, and gray literature were performed in October 2022. Selection criteria Observational studies (with or without a comparison group) that evaluated the prevalence of dental anomalies in individuals with OI. Data collection and analysis: Data items were extracted by two authors. Quality assessment employing the Joanna Briggs Institute checklists and meta-analyses was conducted. Results were provided in prevalence values and odds ratio (OR) / 95% confidence interval (CI). Strength of evidence was determined. Results Eighteen studies were included. Most prevalent dental anomalies in individuals with OI included pulp obliteration (46.4%), dental impaction (33.5%), dental impaction of second molars (27%), and tooth agenesis (23.9%). Individuals with OI type III/IV had 20.16-fold greater chance of exhibiting tooth discoloration in comparison with individuals with OI type I (CI: 1.10-370.98). In comparison with the group without OI, the individuals with OI had 6.90-fold greater chance of exhibiting dental impaction (CI: 1.54-31.00). High methodological quality was found in 47% of the studies. Strength of evidence was low or very low. Conclusions Pulp obliteration, dental impaction, and tooth agenesis were the most prevalent dental anomalies in the OI group. Individuals with OI were more likely to have dental impaction than individuals without OI. Individuals with OI type III/IV (severe-moderate) are more likely to have tooth discoloration than individuals with OI type I (mild).

Embarazo gemelar espontáneo en útero bicorpóreo: una contribución a la experiencia / Spontaneous twin pregnancy in a bicorporeal uterus, a contribution to the experience

Resumen ANTECEDENTES: Las anomalías uterinas congénitas son consecuencia de la formación anormal, fusión o reabsorción de los conductos de Müller durante el desarrollo embriológico. El útero bicorne, unicorne y didelfo (U1, U2, U3 ESHRE-ESGE) son defectos en la unificación con una baja prevalencia en la población general y en la que tiene antecedente de aborto e infertilidad; se estima en alrededor de 0.4 a 1.1%, respectivamente. La presentación del caso puede aportar información útil relacionada con la evolución del embarazo y los desenlaces perinatales cuando aparece esta anomalía. CASO CLÍNICO: Paciente de 35 años, con amenorrea secundaria y prueba inmunológica de embarazo positiva, con antecedentes de dismenorrea primaria severa y anomalía uterina congénita compatible con útero bicorpóreo (U3 completo ESHRE-ESGE), bicorne completo (4a-AFS) y didelfo. El embarazo trascurrió sin complicaciones hasta la semana 37, cuando finalizó mediante cesárea, con hipotonía uterina que no ameritó transfusión de hemoderivados. CONCLUSIÓN: Los embarazos gemelares con útero bicorne son excepcionales y casi todos se logran espontáneamente; los que llegan a término finalizan mediante cesárea electiva.

Abstract BACKGROUND: Congenital uterine anomalies are the result of abnormal formation, fusion, or resorption of the Müllerian ducts during embryological development. The bicornuate, unicornuate and didelphic uterus (U1, U2, U3 ESHRE/ESGE), are unification defects with a low prevalence both in the general population and in the population with a history of abortion and infertility, is estimated at around 0.4 to 1.1%, respectively. The presentation of the case can provide useful information related to with the evolution of the pregnancy and the perinatal outcomes when this anomaly appears. CLINICAL CASE: A 35-year-old patient with secondary amenorrhea and a positive immunological pregnancy test with a history of severe primary dysmenorrhea and previous magnetic resonance imaging with a congenital uterine anomaly compatible with a bicorporeal uterus (U3 complete ESHRE-ESGE), complete bicornuate (4a /AFS) , uterus didelphys (ASRM). The pregnancy was uncomplicated until 37 weeks, when it was terminated by cesarean section, with uterine hypotonia that did not require transfusion of blood products. CONCLUSION: Twin pregnancy with bicornuate uterus is a rare entity, most of them have been achieved spontaneously and those that reach term have been resolved by elective cesarean section.

Prevalencia de patologías fetales que pueden beneficiarse de intervenciones intrauterinas en un hospital de tercer nivel del Occidente de México / Prevalence of fetal pathologies that can benefit from intrauterine interventions in a third level hospital in western Mexico

Resumen OBJETIVO: Determinar la prevalencia de afecciones susceptibles de ser tratadas mediante un procedimiento intrauterino en una unidad de Medicina Materno Fetal de un hospital de tercer nivel del Occidente de México, en un periodo de nueve años. MATERIALES Y MÉTODOS: Estudio transversal y descriptivo, con revisión de los reportes de ultrasonidos obstétricos practicados en la Unidad de Medicina Materno Fetal del Hospital Civil de Guadalajara Dr. Juan I. Menchaca del 2013 al 2021, con selección de los casos de pacientes con diagnóstico de alguna afectación susceptible de ser intervenida de manera intrauterina. RESULTADOS: Durante el periodo de estudio se practicaron y registraron 103,721 ultrasonidos obstétricos, de éstos se integraron al estudio aquí publicado 257 pacientes con diagnóstico de alguna afectación susceptible de ser intervenida in útero. La prevalencia de afectaciones con posibilidad de ser intervenidas por vía intrauterina fue del 0.47% de la totalidad de pacientes valoradas. La media de edad de las pacientes fue de 24.6 años; 162 (63%) multigestas y 95 (37%) primigestas. Embarazos únicos 193 (75%) y 64 (25%) múltiples. Las semanas promedio de gestación al diagnóstico de la afectación fueron 25.6. CONCLUSIONES: En este ensayo se estimó una prevalencia de 0.47 padecimientos que pueden ser intervenidos in útero, dejando de lado muchas otros en los que los estudios no han demostrado beneficio de una cirugía fetal, ni los beneficios superan los riesgos, si se practica la cirugía de manera prenatal o posnatal.

Abstract OBJECTIVE: To determine the prevalence of conditions amenable to treatment by an intrauterine procedure in a Maternal-Fetal Medicine unit of a tertiary hospital in western Mexico over a nine-year period. MATERIALS AND METHODS: Cross-sectional and descriptive study, with review of obstetric ultrasound reports performed in the Maternal-Fetal Medicine Unit of the Civil Hospital of Guadalajara Dr. Juan I. Menchaca from 2013 to 2021, with selection of cases of patients diagnosed with any condition susceptible to intrauterine intervention. RESULTS: During the study period 103,721 obstetric ultrasounds were performed and recorded, of which 257 patients were included in the study published here with a diagnosis of a condition that could be treated in utero. The prevalence of conditions that could be treated in utero was 0.47% of all patients assessed. The mean age of the patients was 24.6 years; 162 (63%) were multigestational and 95 (37%) primigravid. Singleton pregnancies 193 (75%) and 64 (25%) multiple pregnancies. Mean weeks of gestation at diagnosis of involvement was 25.6. CONCLUSIONS: This trial estimated a prevalence of 0.47% of conditions that can be intervened in utero, leaving aside many others where studies have not demonstrated benefit of fetal surgery, nor do the benefits outweigh the risks, whether surgery is performed prenatally or postnatally.

A rare cause of deep vein thrombosis: inferior vena cava agenesis / Agenesia da veia cava inferior: uma causa rara de trombose venosa profunda

Abstract Inferior vena cava agenesis is a rare condition and is often misdiagnosed. This anomaly is asymptomatic in the majority of cases and is usually diagnosed during imaging tests carried out for other purposes. The most frequent manifestation is deep vein thrombosis (DVT) in lower limbs and anticoagulation therapy is the most frequent treatment option. Other techniques such as thrombolysis and venous bypass are also described. We report two cases diagnosed at our institution during the last year, both of which presented with an episode of DVT. We opted for indefinite anticoagulation therapy and both patients remain asymptomatic, after 1 year of surveillance in the first case and 6 months in the second, with no new episodes of DVT. Although it is not a life-threatening anomaly, it is important to make an appropriate diagnosis and provide treatment to improve the symptoms and quality of life of these patients.

Resumo A agenesia da veia cava inferior é uma condição rara e, muitas vezes, mal diagnosticada. Essa anomalia é, na maioria dos casos, assintomática, sendo geralmente diagnosticada durante exames de imagem realizados com outras finalidades. A manifestação mais comum é trombose venosa profunda (TVP) em membros inferiores, e a terapia anticoagulante é a opção de tratamento mais frequente, embora outras técnicas, como trombólise e derivações venosas, também tenham sido descritas. Relatamos dois casos diagnosticados na nossa Instituição no último ano, ambos com episódio de TVP. O tratamento consistiu em anticoagulação por tempo indeterminado e, após 1 ano de acompanhamento no primeiro caso e 6 meses no segundo, ambos os pacientes permanecem assintomáticos, sem novos episódios de TVP. Embora não seja uma anomalia com risco de vida, é importante realizar diagnóstico e tratamento adequados para melhorar os sintomas e a qualidade de vida desses pacientes.