Síndrome de Herlyn-Werner Wünderlich: reporte de dos casos / Herlyn-Werner-Wünderlich syndrome: Two case report

El síndrome de Herlyn-Werner Wünderlich, también llamado OHVIRA por sus siglas en inglés (obstructed hemivagina and ipsilateral renal anomaly), es una anomalía congénita mülleriana poco frecuente que se caracteriza por la asociación entre útero didelfo, hemivagina obstruida y agenesia renal ipsilateral. La presentación clínica más común es la masa abdominal secundaria a hematocolpos, dolor y dismenorrea. Se asocia a infertilidad, endometriosis, alteraciones menstruales y obstétricas. La ecografía es la técnica de elección para la evaluación inicial, mientras que la resonancia magnética sigue siendo el método más exacto para el diagnóstico. La septotomía vaginal es el tratamiento recomendado. Se describen 2 casos clínicos con el objetivo de destacar la importancia del diagnóstico temprano para evitar las posibles complicaciones futuras.

Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.

Manejo de Linfangioma en la edad pediátrica: presentación de caso / Management of Lymphangioma in pediatric age: case presentation / Manejo do Linfangioma em idade pediátrica: apresentação de caso

Las malformaciones vasculares congénitas afectan con mayor frecuencia al sistema tegumentario y se hacen evidentes al nacer o en las primeras semanas de vida con una prevalencia estimada del 4,5%. Las anomalías linfáticas, suelen tener una presentación variable, y en la mayoría de ocasiones su manejo se convierte en un desafío. Se estima que su incidencia global oscila entre 1 en 2.000 y 1 en 16.0004,5 casos. Objetivo: Determinar la importancia de un adecuado manejo por cirugía vascular en el contexto de un paciente pediátrico con Linfangioma en miembro inferior tomando como metodología la presentación de un caso clínico. Descripción del caso: Paciente de 7 meses de edad sin antecedentes clínicos o quirúrgicos de interés, quien es traído por su madre a consulta de cirugía vascular por presencia de masa en miembro inferior derecho que progresivamente ha mostrado crecimiento, cuadro que se acompaña de tumefacción en partes blandas y roce o dolor a la distensión en la zona de la malformación, de acuerdo a la clasificación de Mulliken y Glowacki se concluye que se trata de un Linfangioma, diagnóstico que se corroboró por ultrasonografía Doppler, Angiotac y flebografía ascendente. Para su manejo se realizó drenaje percutáneo ecoguiado y escleroterapia mediante espuma de polidocanol al 1% para sellar la malformación vascular, obteniendo mejoría clínica con resultados estéticos y funcionales satisfactorios. Conclusión: El Linfangioma es una malformación vascular poco frecuente que no se ha documentado localmente, por lo tanto, la presentación de este caso pretendió proporcionar información científica actualizada sobre el tratamiento de la patología vascular y abogar por un manejo basado en la evidencia médica existente, que contribuya a resultados favorables para los pacientes pediátricos

Congenital vascular malformations most frequently affect the integumentary system and become evident at birth or in the first weeks of life with an estimated prevalence of 4.5%. Lymphatic anomalies usually have a variable presentation, and in most cases their management becomes a challenge. Its global incidence is estimated to range between 1 in 2,000 and 1 in 16,0004.5 cases. Objective: Determine the importance of adequate management by vascular surgery in the context of a pediatric patient with Lymphangioma in the lower limb using the presentation of a clinical case as a methodology. Description of the case: A 7-monthold patient with no clinical or surgical history of interest, who was brought by his mother to a vascular surgery consultation due to the presence of a mass in the right lower limb that has progressively shown growth, a condition that is accompanied by swelling in the soft tissues and friction or pain upon distension in the area of the malformation, according to the classification of Mulliken and Glowacki, it is concluded that it is a Lymphangioma, a diagnosis that was confirmed by Doppler ultrasonography, Angiotac and ascending phlebography. For its management, ultrasound-guided percutaneous drainage and sclerotherapy using 1% polidocanol foam was performed to seal the vascular malformation, obtaining clinical improvement with satisfactory aesthetic and functional results. Conclusion: Lymphangioma is a rare vascular malformation that has not been documented locally, therefore, the presentation of this case aimed to provide updated scientific information on the treatment of vascular pathology and advocate management based on existing medical evidence. that contributes to favorable outcomes for pediatric patients

As malformações vasculares congênitas afetam mais frequentemente o sistema tegumentar e tornam-se evidentes ao nascimento ou nas primeiras semanas de vida, com prevalência estimada em 4,5%. As anomalias linfáticas costumam ter apresentação variável e na maioria dos casos seu manejo torna-se um desafio. Estima-se que sua incidência global varie entre 1 em 2.000 e 1 em 16.0004,5 casos. Objetivo: Determinar a importância do manejo adequado por cirurgia vascular no contexto de um paciente pediátrico com Linfangioma em membro inferior utilizando como metodologia a apresentação de um caso clínico. Descrição do caso: Paciente de 7 meses, sem antecedentes clínicos ou cirúrgicos de interesse, que foi trazido pela mãe à consulta de cirurgia vascular devido à presença de uma massa no membro inferior direito que apresentava crescimento progressivo, quadro que vem acompanhado de inchaço nos tecidos moles e fricção ou dor à distensão na área da malformação, segundo a classificação de Mulliken e Glowacki, conclui-se que se trata de um Linfangioma, diagnóstico que foi confirmado por Ultrassonografia Doppler, Angiotac e flebografia ascendente. Para seu manejo foi realizada drenagem percutânea guiada por ultrassom e escleroterapia com espuma de polidocanol a 1% para selar a malformação vascular, obtendo melhora clínica com resultados estéticos e funcionais satisfatórios. Conclusão: O linfangioma é uma malformação vascular rara e não documentada localmente, portanto, a apresentação deste caso teve como objetivo fornecer informação científica atualizada sobre o tratamento da patologia vascular e defender uma gestão baseada na evidência médica existente que contribua para resultados favoráveis para a pediatria. pacientes

Manejo de Linfangioma en la edad pediátrica: presentación de caso

Las malformaciones vasculares congénitas afectan con mayor frecuencia al sistema tegumentario y se hacen evidentes al nacer o en las primeras semanas de vida con una prevalencia estimada del 4,5%. Las anomalías linfáticas, suelen tener una presentación variable, y en la mayoría de ocasiones su manejo se convierte en un desafío. Se estima que su incidencia global oscila entre 1 en 2.000 y 1 en 16.0004,5 casos. Objetivo: Determinar la importancia de un adecuado manejo por cirugía vascular en el contexto de un paciente pediátrico con Linfangioma en miembro inferior tomando como metodología la presentación de un caso clínico. Descripción del caso: Paciente de 7 meses de edad sin antecedentes clínicos o quirúrgicos de interés, quien es traído por su madre a consulta de cirugía vascular por presencia de masa en miembro inferior derecho que progresivamente ha mostrado crecimiento, cuadro que se acompaña de tumefacción en partes blandas y roce o dolor a la distensión en la zona de la malformación, de acuerdo a la clasificación de Mulliken y Glowacki se concluye que se trata de un Linfangioma, diagnóstico que se corroboró por ultrasonografía Doppler, Angiotac y flebografía ascendente. Para su manejo se realizó drenaje percutáneo ecoguiado y escleroterapia mediante espuma de polidocanol al 1% para sellar la malformación vascular, obteniendo mejoría clínica con resultados estéticos y funcionales satisfactorios. Conclusión: El Linfangioma es una malformación vascular poco frecuente que no se ha documentado localmente, por lo tanto, la presentación de este caso pretendió proporcionar información científica actualizada sobre el tratamiento de la patología vascular y abogar por un manejo basado en la evidencia médica existente, que contribuya a resultados favorables para los pacientes pediátricos.

Congenital vascular malformations most frequently affect the integumentary system and become evident at birth or in the first weeks of life with an estimated prevalence of 4.5%. Lymphatic anomalies usually have a variable presentation, and in most cases their management becomes a challenge. Its global incidence is estimated to range between 1 in 2,000 and 1 in 16,0004.5 cases. Objective: Determine the importance of adequate management by vascular surgery in the context of a pediatric patient with Lymphangioma in the lower limb using the presentation of a clinical case as a methodology. Description of the case: A 7-month-old patient with no clinical or surgical history of interest, who was brought by his mother to a vascular surgery consultation due to the presence of a mass in the right lower limb that has progressively shown growth, a condition that is accompanied by swelling in the soft tissues and friction or pain upon distension in the area of the malformation, according to the classification of Mulliken and Glowacki, it is concluded that it is a Lymphangioma, a diagnosis that was confirmed by Doppler ultrasonography, Angiotac and ascending phlebography. For its management, ultrasound-guided percutaneous drainage and sclerotherapy using 1% polidocanol foam was performed to seal the vascular malformation, obtaining clinical improvement with satisfactory aesthetic and functional results. Conclusion: Lymphangioma is a rare vascular malformation that has not been documented locally, therefore, the presentation of this case aimed to provide updated scientific information on the treatment of vascular pathology and advocate management based on existing medical evidence. that contributes to favorable outcomes for pediatric patients.

As malformações vasculares congênitas afetam mais frequentemente o sistema tegumentar e tornam-se evidentes ao nascimento ou nas primeiras semanas de vida, com prevalência estimada em 4,5%. As anomalias linfáticas costumam ter apresentação variável e na maioria dos casos seu manejo torna-se um desafio. Estima-se que sua incidência global varie entre 1 em 2.000 e 1 em 16.0004,5 casos. Objetivo: Determinar a importância do manejo adequado por cirurgia vascular no contexto de um paciente pediátrico com Linfangioma em membro inferior utilizando como metodologia a apresentação de um caso clínico. Descrição do caso: Paciente de 7 meses, sem antecedentes clínicos ou cirúrgicos de interesse, que foi trazido pela mãe à consulta de cirurgia vascular devido à presença de uma massa no membro inferior direito que apresentava crescimento progressivo, quadro que vem acompanhado de inchaço nos tecidos moles e fricção ou dor à distensão na área da malformação, segundo a classificação de Mulliken e Glowacki, conclui-se que se trata de um Linfangioma, diagnóstico que foi confirmado por Ultrassonografia Doppler, Angiotac e flebografia ascendente. Para seu manejo foi realizada drenagem percutânea guiada por ultrassom e escleroterapia com espuma de polidocanol a 1% para selar a malformação vascular, obtendo melhora clínica com resultados estéticos e funcionais satisfatórios. Conclusão: O linfangioma é uma malformação vascular rara e não documentada localmente, portanto, a apresentação deste caso teve como objetivo fornecer informação científica atualizada sobre o tratamento da patologia vascular e defender uma gestão baseada na evidência médica existente que contribua para resultados favoráveis para a pediatria. pacientes.

Malformaciones congénitas diagnosticadas en el servicio de medicina materno fetal de la Maternidad Concepción Palacios / Congenital malformations diagnosed in the maternal fetal medicine service of the Maternidad Concepción Palacios

Objetivo: Identificar y clasificar las diferentes anomalías del desarrollo diagnosticadas en la unidad de ecografía del servicio de medicina materno fetal de la Maternidad Concepción Palacios entre enero y diciembre de 2023. Métodos: Estudio retrospectivo, descriptivo, transversal que incluyó la evaluación de los 4225 reportes de ultrasonido obstétrico realizados en 2023. Se excluyeron los estudios sin diagnóstico morfológico. Las variables evaluadas fueron características clínicas de las gestantes, prevalencia según tipo de anomalía del desarrollo y según el aparato o sistema afectado. Resultados: Se diagnosticaron anomalías del desarrollo en 282 pacientes, para una frecuencia de 6,7 %. Las anomalías fueron únicas en 187 casos (66,3 %) y múltiples en 95 pacientes (33,7 %). El total de malformaciones fue 360 (8,5 %). El mínimo de lesiones detectadas fue una y el máximo fue tres. El sistema afectado con mayor frecuencia fue el sistema nervioso central, con 104 casos (28,9 %); le siguen, en orden de frecuencia, los marcadores aislados, vistos en 92 pacientes (25,6 %) y las anomalías cardiovasculares, en 49 fetos (13,6 %). Conclusión: La frecuencia de malformaciones congénitas diagnosticadas en el año 2023 fue de 6,7 % de las ecografías realizadas en la unidad de ecografía del servicio de medicina materno fetal de la Maternidad Concepción Palacios; en las dos terceras partes de los casos fueron únicas y el tercio restante fueron múltiples. En orden de frecuencia, los sistemas afectados fueron sistema nervioso central, marcadores aislados de aneuploidías y anomalías cardiovasculares(AU)

Objective: To identify and classify the different developmental anomalies diagnosed in the ultrasound unit of the maternal-fetal medicine service of the Concepción Palacios Maternity Hospital between January and December 2023. Methods: Retrospective, descriptive, cross-sectional study that included the evaluation of the 4225 obstetric ultrasound reports performed in 2023. Studies without morphological diagnosis were excluded. The variables evaluated were clinical characteristics of the pregnant women, prevalence according to type of developmental anomaly and according to the affected apparatus or system. Results: Developmental abnormalities were diagnosed in 282 patients, with a frequency of 6.7%. The anomalies were single in 187 cases (66.3%) and multiple in 95 patients (33.7%). The total number of malformations was 360 (8.5%). The minimum number of injuries detected was one and the maximum was three. The most frequently affected system was the central nervous system, with 104 cases (28.9%); This is followed by isolated markers, seen in 92 patients (25.6%), and cardiovascular anomalies, in 49 fetuses (13.6%). Conclusion: The frequency of congenital malformations diagnosed in 2023 was 6.7% of the ultrasound scans performed in the ultrasound unit of the maternal-fetal medicine service of the Concepción Palacios Maternity Hospital; Two-thirds of the cases were singles and the remaining third were multiples. In order of frequency, the affected systems were central nervous system, isolated markers of aneuploidies, and cardiac anomalies(AU)

Expansión Rápida del Maxilar: Revisión actualizada de las técnicas disponibles / Rapid Maxillary Expansion: Updated Review of Available Techniques

La deficiencia transversal del maxilar es una anomalía dentomaxilar capaz de producir problemas funcionales en la oclusión, respiración y estéticos. Su presentación clínica tradicional es la mordida cruzada posterior y una alteración en la relación transversal intermaxilar, la cual impide la correcta erupción de las piezas dentarias. Su manejo corresponde a la expansión rápida del maxilar, la cual puede ser asistida mediante mini-implantes (MARPE), cirugía (SARPE) o una combinación de ambas técnicas (MISMARPE). El objetivo del presente artículo es presentar un contraste entre las técnicas empleadas en la expansión rápida del maxilar, para simplificar la toma de decisiones clínicas. Se elaboró una revisión narrativa en las bases de datos PubMed, Scopus y Epistemonikos contemplando revisiones sistemáticas, metaanálisis, ensayos clínicos aleatorizados y estudios observacionales publicados entre el año 2013 a 2023. Un total de 23 artículos fueron incluidos, los cuales cumplían con los criterios de inclusión y exclusión establecidos. El manejo clínico de la deficiencia transversal del maxilar frecuentemente requiere un abordaje interdisciplinario combinando un enfoque ortopédico y quirúrgico. Según lo encontrado en la actual revisión, tanto el MARPE, SARPE y MISMARPE reportan indicaciones y limitaciones, así como complicaciones asociadas, sin embargo, serían efectivas en la resolución de deficiencias transversales. Se recomienda al clínico considerar dicha información de acuerdo a las necesidades particulares de cada caso clínico, así como profundizar y prolongar el estudio de nuevas técnicas para analizar su estabilidad a largo plazo en comparación a las otras corrientes terapéuticas.

The transverse deficiency of the maxilla is a dentomaxillary anomaly capable of producing functional problems in occlusion, respiration and esthetics. Its traditional clinical presentation is dental crowding, which obstructs the correct eruption of the teeth. Its management corresponds to rapid maxillary expansion, which can be assisted by means of mini-implants (MARPE), surgery (SARPE) or a combination of both techniques (MISMARPE). The objective of this article is to present a contrast between the techniques used in rapid maxillary expansion to simplify clinical decision making. A narrative review was performed in PubMed, Scopus and Epistemonikos databases, including systematic reviews, meta-analyses, randomized clinical trials and observational studies published between 2013 and 2023. A total of 23 articles were included, which met the established inclusion and exclusion criteria. The clinical management of transverse deficiency of the maxilla frequently requires an interdisciplinary management, combining an orthopedic and surgical approach. As found in the current review, all techniques; MARPE, SARPE and MISMARPE, report indications and limitations, as well as associated complications. It is recommended to deepen and prolong the study of new techniques in order to analyze their long-term stability in comparison to other therapeutic currents.

Correlation between development of terminal rectal ganglion and spinal cord/sacral abnormalities in boys with complex anorectal malformations / 陆军军医大学学报

Objective To investigate the relationship between the development of terminal rectal ganglion and spinal cord/sacral abnormalities in boys with complex anorectal malformations(ARMs)in order to improve the understanding of rectal ganglion development abnormalities in ARMs patients.Methods A retrospective trial was conducted on the male patients with complex ARMs admitted to our hospital from 2015 to 2021.The terminal rectal specimens were taken from them during anoplasty.According to the findings on development of terminal rectal ganglion after HE staining,the patients were classified into G1 group(ganglion cells observed)and G2 group(no ganglion cells observed).Imaging techniques were used to evaluate whether there were abnormalities in the spinal cord and sacrum,and their correlation with the terminal rectal ganglion development was analyzed.Results A total of 139 patients were enrolled,and their median age at anoplasty was 5.77(4.57,6.97)months.There were no significant differences between the G1(n=80,57.6％)and G2(n=59,42.4％)groups in ARMs pathological type(P=0.706)and age at surgery(P=0.140).Radiological findings showed there were 48 cases(34.5％)of spinal cord anomalies(SCA),25 cases(18.0％)of sacral abnormalities and 18 cases(12.9％)of coccyx abnormalities.No significant differences were observed in the incidences of SCA and sacral abnormalities between the G1 and G2 groups(P＜0.05).Moreover,the differences of fatty filum terminale and syrinx were statistically significant(P＜0.05).In addition,the ratio of sacrum to coccyx between the G1 and G2 groups were 0.72±0.10 vs 0.67±0.12(P＜0.05)of the anteroposterior position and 0.77±0.09 vs 0.72±0.09(P＜0.05)of the lateral position.Multivariate logistic regression analysis showed that sacral abnormalities,fatty filum terminale and syrinx were independent predictors of rectal terminal ganglion absence in male patients with complex ARMs.Conclusion The development of terminal rectal ganglia in male patients with ARMs is closely associated with the abnormalities of spinal cord and sacrum.Sacral abnormalities,fatty filum terminale and syrinx are independent predictors of rectal terminal ganglion absence in male patients with complex ARMs.

Non-communicating rudimentary horn mimicking a degenerated subserous leiomyoma: A case report

@#Unicornuate uterus with a rudimentary horn is a rare Müllerian anomaly, with an incidence of 0.06%. Due to this relative rarity, an accurate preoperative diagnosis of unicornuate uterus with rudimentary horn still remains to be a diagnostic challenge. Reported here is a case of a 27-year-old nulligravida who complained of severe debilitating dysmenorrhea 5 months prior to admission, necessitating frequent visits to the emergency department for administration of intravenous pain medications and antiemetics. She was misdiagnosed preoperatively with subserous leiomyoma, mainly based on clinical presentation, and findings on ultrasound and pelvic MRI. On exploratory laparotomy, a definitive diagnosis of unicornuate uterus with an enlarged rudimentary horn was established, and patient underwent resection of rudimentary horn, with enterolysis, due to dense adhesions in the left adnexa and cul de sac. We emphasize the need for early diagnosis and prompt management of cases like this to prevent complications, and optimize fertility and quality of life of affected patients.

Dents natales et néonatales, quelle prise en charge: revue systématique de littérature / Natal and neonatal teeth, which management: systematic literature review

Les dents « natales et néo natales ¼ sont des phénomènes rares mais assez bien documentées dans la littérature. Ces dents apparaissent avant l'âge de l'éruption dentaire et sont à l'origine de nombreuses complications. Leur prise en charge est donc une priorité absolue afin de maintenir un état buccal compatible avec les besoins physiologiques du nouveau-né. A cet égard, de nombreux traitements ont été décrits. Objectif : faire le point sur la prise en charge des dents natale et néo natale afin de mieux comprendre et exploiter les modalités thérapeutiques de ce phénomène décrit comme « rare ¼. Les dents natales et néo natales ont longtemps suscité de nombreuses interrogations quant à leurs étiologies, leurs diagnostics et leurs prises en charge. Que ce soit le médecin pédiatre ou le médecin dentiste, l'implication diagnostique et thérapeutique relève de l'unanimité et la collaboration actives entre les équipes médicales. Le traitement allant d'un simple polissage à l'extraction de la dent natale ou néo natale, il est judicieux d'instaurer une thérapeutique adaptée à la situation clinique tout en sensibilisant les parents de l'importance d'un suivi clinique et radiologique de l'état bucco-dentaire de l'enfant présentant cette anomalie particulièrement rare et pouvant être associée à un état pathologique d'ordre général.

"Natal and neonatal" teeth are rare phenomena but fairly well documented in the literature. These teeth appear before the age of dental eruption and are the cause of many complications. Their management is therefore an absolute priority in order to maintain an oral condition compatible with the physiological needs of the newborn. In this regard, many treatments have been described. Aim: to review the management of natal and neonatal teeth in order to better understand and achieve the best therapeutic modalities of this phenomenon described as "rare". Natal and neonatal teeth have long raised many questions about their etiologies, their diagnosis and their treatment. Whether it is the pediatrician or the dentist, the diagnosis and the treatment involvement is based on unanimity and active collaboration between the medical teams. The treatment, ranging from a simple polishing to the extraction of the natal or neonatal tooth, it is wise to establish a therapy adapted to the clinical situation while sensitizing the parents of the importance of a clinical and radiological follow-up of the oral condition of the child with this particularly rare anomaly and which may be associated with a general pathological condition

Experience with 808-nm diode laser in the treatment of 47 cases of oral vascular anomalies

Abstract Treatment of oral vascular anomalies (OVA) has focused on minimally invasive techniques rather than radical surgery. We investigated the efficacy and safety of diode laser using the photocoagulation technique in the management of OVA. Forty-seven subjects with OVA were treated with forced dehydration with induced photocoagulation (FDIP) using diode laser (808 nm/4.5 W). This series consisted mostly of male (63.8%) and non-white (63.8%) patients with a mean age of 57.4 years. Varices (91.5%), venous malformations (6.4%), and hemangiomas (2.1%) with a mean size of 7.1 (±4.9) mm were the conditions treated. OVA presented as a nodular lesion (63.8%) involving mainly the lower lip (46.8%). Pulsed laser mode was used as standard and the number of applications varied from one to four sessions, with the majority requiring only one (83%) FDIP session. Kaplan-Meier analysis revealed that complete clinical healing can occur on the 15th day (n=9/29.5%), followed by the 20th (n=6/45.5%), and 30th (n=7/70.5%) days. Postoperative edema was observed in 31 (66%) patients, and recurrence of the lesion occurred in two (4.2%). Based on the data on complete clinical healing, minimal patient discomfort, and satisfactory esthetic results, we can confirm that FDIP by diode laser is a promising candidate for the safe and efficacious treatment of OVA.

Aquatic therapy in congenital malformation during the use of external fixator for bone lengthening: It is possible?

Abstract Objectives: The objective of this study is to describe the rehabilitation of individuals with Congenital Malformations (CMF) during the use of an External Fixator (EF) in Aquatic Therapy (AT) and to analyze the association between diagnosis, EF type and location with rehabilitation process outcomes, surgical intervention, and adverse effects. Methods: This retrospective study included 29 medical records from which the personal and rehabilitation data of the patient were collected. The AT used was described and the outcome variables were associated. The medical records were selected by screening the database of the CMF clinic at the AACD. The inclusion criteria were participants with CMF who used EF treated between 2011 and 2019 of both genders and without age restriction. The exclusion criteria were incomplete medical record data or not undergoing AT while using EF. The extracted data included diagnosis, gender, age, EF type and location, objective of the surgery, adverse events, surgical interventions, time of rehabilitation in AT, physiotherapeutic objectives, and rehabilitation process outcomes in AT. Results: The mean age of the participants was 12.1 ± 3.99 years, with male predominance (55 %) and hemimelia cases (37 %). The most used EF was circular (51 %), located in the femur (37 %), and the main objective of surgery was bone lengthening (52 %). The most recurrent adverse effect was infection (62 %) and 76 % completed AT. There was no association between the variables analyzed. Conclusions: It was possible to describe CMF rehabilitation with EF in AT. There was no association between the variables analyzed.

Ponytail Left Anterior Descending Artery: A Case Report

ABSTRACT Division of the anterior descending branch into many small arteries is a rare coronary anomaly. We report the case of a 64-year-old female with severe stenosis (>75%) in the proximal region of the anterior descending branch as indicated by coronary computed tomography angiography (CCTA). In addition, coronary angiography showed that the anterior descending branch of the coronary artery split into numerous small arteries, an anomaly that can confound clinical examination.

Analysis of Down syndrome newborn outcomes in three neonatal intensive care units in Rio de Janeiro, Brazil

SUMMARY OBJECTIVE: The aim of this study was to analyze the outcomes of newborns with Down syndrome admitted to three neonatal intensive care units in the city of Rio de Janeiro, Brazil. METHODS: A retrospective cohort study was conducted by analyzing the medical records between 2014 and 2018 of newborns with Down syndrome admitted to three neonatal intensive care units. The following variables were analyzed: maternal and perinatal data, neonatal malformations, neonatal intensive care unit intercurrences, and outcomes. RESULTS: A total of 119 newborns with Down syndrome were recruited, and 112 were selected for analysis. The most common maternal age group was >35 years (72.07%), the most common type of delivery was cesarean section (83.93%), and the majority of cases were male (53.57%). The most common reasons for neonatal intensive care unit hospitalization were congenital heart disease (57.66%) and prematurity (23.21%). The most common form of feeding was a combination of human milk and formula (83.93%). The second most common malformation was duodenal atresia (9.82%). The most common complications during neonatal intensive care unit hospitalization were transient tachypnea of the newborn (63.39%), hypoglycemia (18.75%), pulmonary hypertension (7.14%), and sepsis (7.14%). The mean length of stay in the neonatal intensive care unit was 27 days. The most common outcome was discharge (82.14%). Furthermore, 12.50% of newborns were transferred to an external neonatal intensive care unit, and 6% died. CONCLUSION: Newborns with Down syndrome are more likely to be admitted to the neonatal intensive care unit, and the length of hospital stay is longer due to complications related to congenital malformations common to this syndrome and prematurity.

Evaluating pregnancy termination decisions for fetal anomalies: a retrospective study in a tertiary referral center

SUMMARY OBJECTIVE: The aim of this study was to examine the factors that influence pregnancy termination due to fetal anomalies, regardless of gestational age, within the legal framework of Turkey. METHODS: This retrospective study was conducted between January 2021 and July 2023 at a tertiary perinatology center to analyze patients undergoing pregnancy termination. The process involved multidisciplinary evaluations and informed consent, resulting in 326 pregnancy terminations, categorized by gestational timing. RESULTS: Of the 326 patients studied, 219 opted for terminations. Gestational week at diagnosis significantly influenced the decision to terminate, with fetal anomalies being the primary indication. Chromosomal abnormalities accounted for 15.9% of the cases, while structural anomalies and maternal disorders accounted for 84.1% and structural malformations accounted for 84.1% of the cases. Late terminations (≥23 weeks) accounted for 30% of cases and required complex procedures. CONCLUSION: The findings of this study indicate that maternal demographic factors have a limited impact on termination decisions. Early diagnosis of fetal anomalies is crucial for informed decision-making and emotional support, and the psychological consequences of late termination highlight the need for maternal support. Obstetricians play a vital role in facilitating early intervention. This study underscores the complex medical, ethical, and psychological aspects of pregnancy termination due to fetal anomalies. It emphasizes the importance of a holistic approach, considering medical, ethical, and psychological factors and the crucial role of healthcare professionals in supporting families during this challenging process.

Descripción angiográfica de los aspectos anatómicos y clínicos de la arteria interventricular anterior en un grupo de personas colombianas / Angiographic description of the anatomy and clinical aspects of the anterior interventricular artery in a group of Colombians individuals

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.

Resorción intracoronal preeruptiva: una revisión de la literatura / Preeruptive intracoronal resorption: a review of the literature / Reabsorção intracoronária pré-eruptiva: uma revisão da literatura

RESUMEN La resorción intracoronal preeruptiva (RIPE) es un hallazgo radiográfico que consiste en la presencia de un área radiolúcida ubicada en la dentina coronal próxima a la unión amelodentinaria en el germen de una pieza dentaria; es usualmente singular y poco profunda (menor de 1/3 de la dentina cameral). La frecuencia reportada en la literatura varía entre 0,85 % y 27,3 %; no se ha encontrado asociación con el sexo; y afecta usualmente a las piezas posteriores. Su etiología no ha sido determinada, pero evidencias histológicas apuntan a que se trataría de secuelas de resorción dentinaria. Los defectos de la RIPE deben ser considerados como lesiones de caries una vez que la pieza erupcione en boca; y aunque no se han desarrollado esquemas de tratamiento o guías de práctica clínica, los tratamientos reportados dependen de la profundidad de los defectos y son frecuentemente conservadores. El objetivo de este trabajo fue revisar los estudios descriptivos y los reportes de casos publicados acerca de la RIPE para que estos defectos sean también considerados en la práctica clínica diaria.

ABSTRACT Preeruptive intracoronal resorption (PIRR) is a radiographic phenomenon characterized by the presence of a radiolucent area situated in the coronal dentin near the amelodentine junction within the tooth's germ. Typically, this radiolucent area is singular and shallow, encompassing less than one-third of the chambered dentin. The reported frequency in the literature varies between 0.85% and 27.3%. No association with gender has been identified, and it predominantly affects posterior teeth. While its precise etiology remains undetermined, histological evidence suggests that it results from a sequelae of dentin resorption. RIPE Defects as Caries Lesions: Once the tooth erupts into the oral cavity, RIPE defects should be regarded in a manner similar to caries lesions. Despite the absence of established treatment schemes or clinical practice guidelines, reported treatments are contingent upon the depth of the defects and frequently lean towards conservative approaches. The primary aim of this study was to comprehensively review descriptive studies and published case reports focusing on RIPE defects. This effort is directed towards integrating these defects into routine clinical practice considerations.

RESUMO A reabsorção intracoronária pré-eruptiva (RIPE) é um achado radiográfico que consiste na presença de uma área radiolúcida localizada na dentina coronária próxima à junção amelodentinária no germe de um dente. Geralmente, é singular e rasa, envolvendo menos de 1/3 da dentina da câmara. A frequência relatada na literatura varia entre 0,85% e 27,3%. Não foi encontrada associação significativa com o sexo, e a RIPE tende a afetar mais comumente os dentes posteriores. Sua etiologia ainda não foi totalmente determinada, mas evidências histológicas sugerem que pode ser consequência de reabsorção da dentina. Os defeitos de RIPE devem ser considerados como lesões de cárie quando o dente irrompe na cavidade bucal. Embora não tenham sido desenvolvidos esquemas de tratamento ou diretrizes de prática clínica específicas, os tratamentos relatados para RIPE são geralmente conservadores e baseiam-se na profundidade dos defeitos. Concluindo, a revisão dos estudos descritivos e dos relatos de casos de RIPE destaca a importância de considerar esses defeitos durante a prática clínica rotineira.

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

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.