Cambios en Mecánica Respiratoria en Usuarios Pediátricos en Ventilación Mecánica Post Cirugía de Cardiopatía Congénita Sometidos a Kinesiología Respiratoria versus Succión Endotraqueal. Revisión Estructurada / Changes in Respiratory Mechanics in Pediatric Users on Mechanical Ventilation Post Congenital Heart Disease Surgery Undergoing Respiratory Kinesiology versus Endotracheal Suction. Structured Review

Introducción. Las cardiopatías congénitas (CC) en Chile corresponden a la segunda causa de muerte en menores de 1 año, requiriendo cirugías paliativas y/o correctivas el 65% de estas. En el post operatorio frecuentemente se utiliza ventilación mecánica invasiva (VM) y succión endotraqueal (SET) para remover secreciones. Sin embargo, la kinesiología respiratoria (KTR) ha mostrado mejoras significativas en la distensibilidad toracopulmonar (Cest) y resistencia de vía aérea (Rva) en otros grupos de usuarios pediátricos y adultos en VM. Objetivo. Comparar los cambios en la Cest y Rva en usuarios pediátricos en VM post cirugía de cardiopatía congénita (CCC) sometidos a KTR versus SET exclusiva. Métodos. Revisión sistemática de estudios publicados en bases de datos PUBMED, PeDro, Scielo y Google Scholar que comparan el uso de KTR ó SET sobre los cambios en mecánica ventilatoria en usuarios pediátricos en VM post cirugía de cardiopatía congénita, limitados a inglés, español y portugués, excluyendo a sujetos con traqueostomía o con oxigenación por membrana extracorpórea. Se utilizó guía PRISMA para la selección de artículos. Se revisaron 397 artículos y se seleccionó 1 artículo extra de los artículos sugeridos. Se eliminó 1 artículo por duplicidad. Por títulos y resúmenes se seleccionaron 2 artículos, los cuales al leer el texto completo fueron retirados debido a que la población no correspondía a cardiópatas. Resultados. El final de artículos seleccionados fue de 0 artículos, debido a lo cual se removió el operador Booleano "NOT", y se removió la población de cardiopatías. De este modo quedaron 2 artículos seleccionados para la revisión cualitativa final donde se compara KTR versus SET, y KTR en kinesiólogos especialistas y no especialistas, mostrando ambos aumento en la Cest y disminución de la Rva a favor de la KTR, hasta los 30 minutos post intervención. Conclusiones. No se encontraron artículos que demuestren cambios en Cest y Rva con el uso de KTR + SET versus SET exclusiva, en usuarios pediátricos ventilados posterior a CCC. Con la remoción de filtros seleccionamos 2 artículos que demuestran aumento de Cest y disminución de Rva en sujetos pediátricos en VM, uno comparando con SET, y por grupos de especialistas y no especialistas en respiratorio. Se sugieren estudios primarios para evaluar los efectos de esta intervención en esta población.

Introduction. Congenital heart diseases (CHD) are the second general cause for children death under 1 year. In Chile, approximately 65% CHD need surgery, could was palliative or corrective. In the postoperative period, invasive mechanical ventilation (MV) is frequently used as a life support method, but it is associated with complications. Tracheal suction (SET) is regularly used to remove secretions; however, respiratory chest physiotherapy (KTR) has shown significant improvements in thoraco-pulmonary compliance and airway resistance in other groups of pediatrics and adult's users in MV. Objetive. to compare changes in thoraco-pulmonary compliance and airway resistance in pediatric subjects under mechanical ventilation after congenital heart disease surgery comparing chest physiotherapy and exclusive tracheal suction. Methods. systematic review of studies published in PUBMED, PeDro, Scielo and Google Scholar databases who compares KTR or SET use on changes in ventilatory mechanics in pediatric users under MV after congenital heart disease surgery, limited to English, Spanish and Portuguese languages, excluding user with tracheostomy or extracorporeal membrane of oxygenation. It was use the PRISMA guide to articles selection. A search was carried out, with a total of 397 articles reviewed (English: PubMed = 3, PeDro = 8, Scholar = 383; Spanish: Scholar = 3, Scielo = 0; and Portuguese: Scielo = 0). One extra article was selected from the suggested articles, and 1 article was eliminated due to duplication. By titles and abstracts, 2 articles were selected, but the population did not correspond to heart disease. Results. the final selected articles were 0 articles. By this reason, it were removed: Boolean operator "NOT", and congenital heart disease population. Thus, 2 articles were selected for the final qualitative review where it was compares KTR versus SET, and KTR by specialist and non-specialist. Both articles shown improvement in compliance and resistance until 30 minutes post intervention. The CC population was in a 40 to 60% range in both studies. Conclusions. it was no found articles that demonstrate changes in compliance and resistance in the airway with the use of KTR + SET versus exclusive SET in pediatric users after CCC connected to MV. After filter remotion, we found 2 studies shown improves in increase compliance and reduce resistance in pediatric user in MV, ones comparing with SET, and the other one comparing between specialists in respiratory pediatric physiotherapy and not specialists. It suggests to made primary clinical studies about this intervention in CC population.

Duplicación del colédoco asociado a unión biliopancreática anómala: Reporte de caso / Common bile duct duplication associated with anomalous biliopancreatic junction: A case report

Introducción. La duplicación del colédoco es una anomalía congénita poco frecuente. En la mayoría de los casos este defecto se asocia a cálculos en la vía biliar, unión pancreatobiliar anómala, pancreatitis, cáncer gástrico o colangiocarcinoma. Por esta razón, el diagnóstico y el tratamiento temprano son importantes para evitar las complicaciones descritas a futuro. Métodos. Se presenta el caso de una paciente de 30 años, con antecedente de pancreatitis aguda, con cuadro de dolor abdominal crónico, a quien se le realizaron varios estudios imagenológicos sin claro diagnóstico. Fue llevada a manejo quirúrgico en donde se documentó duplicación del colédoco tipo II con unión pancreatobiliar anómala. Resultados. Se hizo reconstrucción de las vías biliares y hepatico-yeyunostomía, con adecuada evolución postoperatoria y reporte final de patología sin evidencia de tumor. Conclusión. El diagnóstico se hace mediante ecografía endoscópica biliopancreática, colangiorresonancia o colangiopancreatografía retrógrada endoscópica. El tratamiento depende de si está asociado o no a la presencia de unión biliopancreática anómala o cáncer. Si el paciente no presenta patología neoplásica, el tratamiento quirúrgico recomendado es la resección del conducto con reconstrucción de las vías biliares.

Introduction. Double common bile duct is an extremely rare congenital anomaly. This anomaly may be associated with bile duct stones, anomalous biliopancreatic junction, pancreatitis, bile duct cancer, or gastric cancers. Thus, early diagnosis and treatment is important to avoid complications. Clinical case. We report a rare case of double common bile duct associated with an anomalous biliopancreatic junction in a 30-year-old female, with prior history of acute pancreatitis, who presented with chronic abdominal pain. She underwent several imaging studies, without clear diagnosis. She was taken to surgical management where duplication of the type II common bile duct was documented with anomalous pancreatobiliary junction. Results. Reconstruction of the bile ducts and hepatico-jejunostomy were performed, with adequate postoperative evolution and final pathology report without evidence of tumor. Conclusion. Diagnosis is usually performed by an endoscopic ultrasound, magnetic resonance cholangiopancrea-tography, or endoscopic retrograde cholangiopancreatography. Treatment depends on the presence of anomalus biliopancreatic junction or concomitant cancer. In cases without associated malignancy, resection of bile duct and biliary reconstruction is the recommended surgical treatment.

Síndrome de Eisenmenger secundario a comunicación interatrial tipo ostium secundum en mujer adulta / Eisenmenger Syndrome Associated with an ostium secundum Atrial Septal Defect in an Adult Woman

El síndrome de Eisenmenger es la forma más severa de presentación de hipertensión arterial pulmonar secundaria a defectos cardíacos congénitos no reparados, aunque su prevalencia es baja, continúa siendo un reto para los sistemas de salud de los países en vías de desarrollo por su complejidad en el manejo. Presentación del caso. Paciente femenina sin antecedentes médicos conocidos quien consulta por disnea relacionada a los esfuerzos y policitemia. Intervención terapéutica. Se realiza ecocardiograma transesofágico que arroja la presencia de defecto interatrial tipo ostium secundum e hipertensión arterial pulmonar severa, con cortocircuito de derecha a izquierda, se inicia oxigenoterapia y terapia farmacológica. Evolución clínica. Paciente permaneció ingresada presentando notable mejora a la disnea, se le dio de alta con referencia a la clínica de cardiopatías congénitas del adulto en Hospital Nacional Rosales.

Eisenmenger syndrome is the most severe form of pulmonary arterial hypertension secondary to an unrepaired congenital heart disease. Despite the low prevalence, it remains a challenge for the public health service of developing countries due to the complexity of the treatment. Case presentation. A female patient without known medical history, who consults with dyspnea on exertion and polycythemia. Treatment. A transesophageal echocardiogram was performed, showing an ostium secundum atrial septal defect and severe pulmonary arterial hypertension with a right-left shunt. Supplemental oxygen was administrated and pharmacological treatment was started. Outcome. The patient presented remarkable clinical improvement to dyspnea, she was discharged with medical reference to the Adult Congenital Heart Disease clinic at Rosales National Hospital.

Prevalence of Critical Congenital Heart Disease Detected in the Pulse Oximetry Test in Asymptomatic Newborns, ≥35 Gestational Weeks, in a Maternity in Southern Brazil

Abstract Background Congenital heart diseases (CHD) are one of the most prevalent malformations, and the screening tests to identify critical congenital heart disease (CCHD) is the pulse oximetry test, with subsequent investigation and treatment. Objective To quantify positive pulse oximetry tests and verify the prevalence of CCHD detected by it in asymptomatic newborns, ≥35 gestational weeks, in a Brazilian maternity hospital. Methodology This is an observational, retrospective, quantitative, analytical and cross-sectional study, conducted from October 2020 to May 2022, in a maternity hospital in southern Brazil, through the collection of records of positive oximetry pulse tests, following the norms of screening CCHD test of the Brazilian Society of Pediatrics (SBP), after they were evaluated with echocardiography for confirmation or exclusion of CHD. Results A total of 5,667 newborns were evaluated in this study, according to the inclusion criteria; 0.17% (n = 10) had a positive pulse oximetry test. Regarding the results of the echocardiography of the neonates with a positive test, two were normal, seven were cases of patent foramen ovale (PFO), and one was a case (0,017%) with interatrial communication (IAC) diagnosis. In the same period, five neonates with CCHD were born, symptomatic, diagnosed by physical examination, and referred to neonatal intensive care units (NICU) before taking the neonatal cardiac screening (< 24 hours of life). Conclusion The prevalence of positive pulse oximetry tests was 0.17% and none CCHD was detected. Five cases of CCHD were born in this period, but they were diagnosed before the recommend time to perform the screening test.

Patients' prognosis with congenital heart disease followed by ten years: survival and associated factors / Prognóstico de pacientes com cardiopatia congênita seguidos por dez anos: sobrevida e fatores associados

ABSTRACT Objective: To evaluate the prognosis and influence of associated factors in patients with congenital heart disease admitted for the first time to the Intensive Care Unit of the Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, especially those factors associated with death. Methods: Patients were prospectively and consecutively allocated over a period of one year (August 2005 to July 2006). Now, 15 years after the initial selection, we collected data from these patients in the database of the Cytogenetics Laboratory of the Universidade Federal de Ciências da Saúde de Porto Alegre and in the medical records of the hospital. Results: Of the 96 patients, 11 died and 85 were alive until 20 years old. Four patients died in the Intensive Care Unit. The survival probability up to 365 days of life was 95.8%. The survival assessment identified that the deaths occurred mainly before the patients completed one thousand days of life. We found that complex heart disease was independently associated with an odds ratio of 5.19 (95% confidence interval — CI:1.09-24.71; p=0.038) for death. Conclusions: Knowledge about the factors that interfere with the prognosis can be crucial in care practice planning, especially considering that congenital heart disease is an important cause of mortality in the first year of life.

RESUMO Objetivo: Avaliar o prognóstico e a influência de fatores associados em pacientes com cardiopatia congênita internados pela primeira vez na Unidade de Terapia Intensiva do Hospital da Criança Santo Antônio/Irmandade da Santa Casa de Misericórdia de Porto Alegre, principalmente aqueles fatores associados ao óbito. Métodos: Os pacientes foram alocados prospectiva e consecutivamente por um período de um ano (agosto de 2005 a julho de 2006). Agora, 15 anos após a seleção inicial, coletamos dados desses pacientes no banco de dados do Laboratório de Citogenética da Universidade Federal de Ciências da Saúde de Porto Alegre e nos prontuários do hospital. Resultados: Dos 96 pacientes, 11 faleceram e 85 permaneceram vivos até completar 20 anos. Quatro pacientes morreram na Unidade de Terapia Intensiva. A probabilidade de sobrevida até 365 dias de vida foi de 95,8%. A avaliação da sobrevida identificou que os óbitos ocorreram principalmente antes de os pacientes completarem mil dias de vida. Verificamos que a doença cardíaca complexa foi independentemente associada a um odds ratio de 5,19 (intervalo de confiança — IC95% 1,09-24,71; p=0,038) para morte. Conclusões: O conhecimento dos fatores que interferem no prognóstico pode ser fundamental no planejamento da prática assistencial, principalmente considerando-se que as cardiopatias congênitas são importante causa de mortalidade no primeiro ano de vida.

Role of Computed Tomography Angiography in the Short-Term Follow-up of Aortic Coarctation Repair

ABSTRACT Introduction: Coarctation of the aorta (CoA) is a narrowing of the thoracic aorta that often manifests as discrete stenosis but may be tortuous or in long segment. The study aimed to evaluate pre and post-surgical aspects of pediatric patients submitted to CoA surgical correction and to identify possible predisposing factors for aortic recoarctation. Methods: Twenty-five patients were divided into groups according to presence (N=8) or absence (N=17) of recoarctation after surgical correction of CoA and evaluated according to clinical-demographic profile, vascular characteristics via computed angiotomography (CAT), and other pathological conditions. Results: Majority of males (64%), ≥ 15 days old (76%), ≥ 2.5 kg (80%). There was similarity between groups with and without recoarctation regarding sex (male: 87% vs. 53%; P=0.277), age (≥ 15 days: 62.5 vs. 82%; P=0.505), and weight (≥ 2.5 kg: 87.5 vs. 76.5; P=0,492). Altered values of aortic root/Valsalva diameter, proximal transverse arch, and distal isthmus, and normal values for aorta prevailed in preoperative CAT. Normal values for the aortic root/Valsalva sinus diameter were observed with and without recoarctation, the same for both groups regarding ascending and descending aorta in postoperative CAT. No significant difference for altered values of proximal transverse arch and alteration in distal isthmus was observed. Conclusion: No predictive risk for recoarctation was observed. CTA proved to be important in CoA diagnosis and management, since CoA is mainly related with altered diameter of aortic root/sinus of Valsalva and proximal and distal aortic arch/isthmus, however, it failed to show predictive risk for recoarctation.

Lactate and Lactate Clearance Are Predictive Factors for Mortality in Patients with Extracorporeal Membrane Oxygenation

ABSTRACT Introduction: Findings of inadequate tissue perfusion might be used to predict the risk of mortality. In this study, we evaluated the effects of lactate and lactate clearance on mortality of patients who had undergone extracorporeal membrane oxygenation (ECMO). Methods: Patients younger than 18 years old and who needed venoarterial ECMO support after surgery for congenital heart defects, from July 2010 to January 2019, were retrospectively analyzed. Patients successfully weaned from ECMO constituted Group 1, and patients who could not be weaned from ECMO were in Group 2. Postoperative clinics and follow-ups of the groups including mortality and discharge rates were evaluated. Results: There were 1,844 congenital heart surgeries during the study period, and 55 patients that required ECMO support were included in the study. There was no statistically significant difference between the groups regarding demographics and operative variables. The sixth-, 12th-, and 24th-hour lactate levels in Group 1 were statistically significantly lower than those in Group 2 (P=0.046, P=0.024, and P<0.001, respectively). There were statistically significant differences regarding lactate clearance between the groups at the 24th hour (P=0.009). The cutoff point for lactate level was found as ≥ 2.9, with 74.07% sensitivity and 78.57% specificity (P<0.001). The cutoff point for lactate clearance was determined as 69.44%, with 59.26% sensitivity and 78.57% specificity (P=0.003). Conclusion: Prognostic predictive factors are important to initiate advanced treatment modalities in patients with ECMO support. In this condition, lactate and lactate clearance might be used as a predictive marker.

Long-term outcomes of intraoperative triamcinolone injection versus postoperative oral prednisolone in congenital cataract surgery / Resultados de longo prazo do uso intraoperatório de triancinolona versus oral pós-operatório de prednisolona na cirurgia de catarata congênita

ABSTRACT Purpose: To compare the long-term ocular findings of children that were operated of congenital cataract before the age of two and that received an intraoperative intracameral triamcinolone injection or used postoperative oral prednisolone to modulate ocular inflammation. Methods: All patients who had previously participated in a clinical trial that analyzed the 1-year surgical outcomes of congenital cataract surgery utilizing intracameral triamcinolone (study group) or oral prednisolone (control group) were eligible to participate in this prospective cohort research. Patients' medical records were reviewed, and the children underwent a complete ophthalmologic exam on final follow-up. Biomicroscopic findings, intraocular pressure, central corneal thickness, the need for additional surgical interventions, and findings compatible with glaucoma were the primary end measures. Results: Twenty-six eyes (26 patients) were included (study group = 11 eyes; control group = 15 eyes). The mean follow--up was 8.2 ± 1.2 years and 8.1 ± 1.7 years in the study and control groups, respectively (p=0.82). All eyes presented a centered intraocular lens. There was no statistically significant difference between the groups with regards to the presence of posterior synechia (p=0.56), intraocular pressure (p=0.49), or central corneal thickness (p=0.21). None of the eyes fulfilled the glaucoma diagnostic criteria, presented secondary visual axis obscuration, or were reoperated. Conclusion: The long--term ocular findings of children that underwent congenital cataract surgery and received an intraoperative intracameral triamcinolone injection were similar to those that used postoperative oral prednisolone to modulate ocular inflammation. This suggests that intracameral triamcinolone may substitute oral prednisolone in congenital cataract surgery, facilitating the postoperative treatment regimen and compliance.

RESUMO Objetivo: Comparar os achados oculares em longo prazo de crianças que se submeteram à cirurgia de catarata congênita antes dos dois anos de idade e receberam uma injeção intracameral de triancinolona no intraoperatório ou usaram prednisolona oral no pós-operatório para modular a inflamação ocular. Métodos: Neste estudo prospectivo de coorte, todos os pacientes que participaram de um ensaio clínico anterior, que analisou os resultados cirúrgicos de 1 ano da cirurgia de catarata congênita usando triancinolona intracameral (Grupo de Estudo) ou prednisolona oral (Grupo Controle), eram elegíveis para participar. Os prontuários médicos dos pacientes foram revisados e as crianças foram submetidas a um exame oftalmológico completo no acompanhamento final. As principais medidas de desfecho foram: achados biomicroscópicos, pressão intraocular, espessura central da córnea, a necessidade de intervenções cirúrgicas adicionais e achados compatíveis com glaucoma. Resultados: Vinte e seis olhos (26 pacientes) foram incluídos (Grupo de Estudo = 11 olhos; Grupo de Controle = 15 olhos). O seguimento médio foi de 8,2 ± 1,2 anos e 8,1 ± 1,7 anos nos Grupos de Estudo e Controle, respectivamente (p=0,82). Todos os olhos apresentavam lente intraocular centrada. Não houve diferença estatisticamente significativa entre os grupos com relação à presença de sinéquia posterior (p=0,56), pressão intraocular (p=0,49) ou espessura central da córnea (p=0,21). Nenhum dos olhos preencheu os critérios diagnósticos para glaucoma, apresentou opacificação secundária do eixo visual ou foi reoperado. Conclusão: Os achados oculares em longo prazo de crianças que se submeteram à cirurgia de catarata congênita e receberam uma injeção intracameral de triancinolona no intraoperatório foram semelhantes aos que usaram prednisolona oral no pós-operatório para modular a inflamação ocular, sugerindo que a triancinolona intracameral pode substituir a prednisolona oral na cirurgia de catarata congênita, facilitando o tratamento pós-operatório e a adesão ao mesmo.

Performance Evaluation of Geometrically Different Pediatric Arterial Cannulae in a Pediatric Cardiopulmonary Bypass Model

ABSTRACT Objective: To define a reference chart comparing pressure drop vs. flow generated by a set of arterial cannulae currently utilized in cardiopulmonary bypass conditions in pediatric surgery. Methods: Cannulae from two manufacturers were selected considering their design and outer and inner diameters. Cannula performance was evaluated in terms of pressure drop vs. flow during simulated cardiopulmonary bypass conditions. The experimental circuits consisted of a Jostra HL-20 roller pump, a Quadrox-i pediatric oxygenator (Maquet Cardiopulmonary AG, Rastatt, Germany), and a custom pediatric tubing set. The circuit was primed with lactated Ringer's solution only (first condition) and with human packed red blood cells added (second condition) to achieve a hematocrit of 30%. Cannula sizes 8 to 16 Fr were inserted into the cardiopulmonary bypass circuit with a "Y" connector. The flow was adjusted in 100 ml/min increments within typical flow ranges for each cannula. Pre-cannula and post-cannula pressures were measured to calculate the pressure drop. Results: Utilizing a pressure drop limit of 100 mmHg, our results suggest a recommended flow limit of 500, 900, 1400, 2600, and 3100 mL/min for Braile arterial cannulae sizes 8, 10, 12, 14, and 16 Fr, respectively. For Medtronic DLP arterial cannulae sizes 8, 10, 12, 14, and 16 Fr, the recommended flow limit is 600, 1100, 1700, 2700, and 3300 mL/min, respectively. Conclusion: This study reinforces discrepancies in pressure drop between cannulae of the same diameter supplied by different manufacturers and the importance of independent translational research to evaluate components' performance.

Epidemiologic profile and prevalence of live births with orofacial cleft in Brazil: a descriptive study / Perfil epidemiológico e prevalência de nascidos vivos com fissura orofacial no Brasil: um estudo descritivo

ABSTRACT Objective: To describe the epidemiological profile and prevalence of live births with orofacial clefts in Brazil between 1999 and 2020. Methods: Descriptive study. The population corresponded to live births with isolated orofacial clefts in Brazil registered in the Live Birth Information System between 1999 and 2020. Descriptive variables were selected according to their availability and grouped into socioeconomic and demographic, maternal and child health care, and biological variables. Data were submitted to a descriptive analysis using the Software for Statistics and Data Science (STATA). Results: During the period, 33,699 children were born with orofacial clefts, and 82.1% (27,677) of them were isolated clefts. Regarding these cases, the majority were cleft lip and palate (9,619 or 34.7%), followed by cleft palate (9,442 or 34.1%), and by cleft lip (8,616 or 31.3%). Conclusions: Live births with orofacial clefts in Brazil were male, white, with birthweight ≥2,500 g and gestational age ≥37 weeks, born by cesarean section, and with Apgar scores ≥7. The cases were more frequent among mothers who were in their first and single pregnancy and had seven or more prenatal appointments. The mothers were 20 and 29 years old, had eight to ten years of study, and were single. The national prevalence of clefts was 4.24/10,000. The South and Southeast regions of Brazil had the highest prevalence, while the lowest prevalence was recorded in the Northeast and North regions. For the Federative Units, the highest and lowest prevalences were found, respectively, in Paraná and Acre.

RESUMO Objetivo: Descrever o perfil epidemiológico e a prevalência dos nascidos vivos com fissuras orofaciais no Brasil entre 1999 e 2020. Métodos: Estudo descritivo. A população correspondeu aos nascidos vivos com fissuras orofaciais isoladas no Brasil registrados no Sistema de Informação de Nascidos Vivos entre 1999 e 2020. As variáveis descritivas foram selecionadas de acordo com a sua disponibilidade e agrupadas em variáveis socioeconômicas e demográficas, de atenção à saúde materno-infantil e biológicas. Os dados foram submetidos a análise descritiva utilizando o Software for Statistics and Data Science (STATA). Resultados: No período, 33.699 indivíduos nasceram com fissura orofacial no Brasil, e 82,1% (27.677) deles foram fissuras isoladas. Com relação a esses casos, a maioria foi de fissuras de lábio e palato (9.619 ou 34,7%), seguidas por fissura de palato (9.442 ou 34,1%) e por fissura de lábio (8.616 ou 31,1%). Conclusões: O perfil epidemiológico dos nascidos vivos com fissuras orofaciais no Brasil foi de nascidos do sexo masculino, da raça/cor branca, por parto cesáreo, com peso ao nascer ≥2,500 g, idade gestacional ≥37 semanas e com índices de Apgar ≥7. Os casos foram mais frequentes entre mães que estavam na primeira gestação, única e que haviam realizado sete ou mais consultas de pré-natal. As mães, com maior frequência, tinham entre 20 e 29 anos, apresentavam oito ou mais anos de estudo, eram solteiras e residiam em cidades do interior. A prevalência nacional de fissuras foi de 4,24/10.000. As Regiões Sul e Sudeste apresentaram as maiores prevalências, enquanto as menores foram registradas nas Regiões Nordeste e Norte. Para as Unidades Federativas, as maiores e menores prevalências foram encontradas, respectivamente, no Paraná e no Acre.

Dacriocistocele bilateral congénito: reporte de un caso / Congenital bilateral dacryocystocele: case report

Introducción: el dacriocistocele es una malformación congénita rara, secundaria a la obstrucción del conducto nasolagrimal; tiene una incidencia de 0,1 % de pacientes con obstrucción congénita del conducto nasolagrimal y se encuentra bilateralmente hasta en 25 % de casos. Caso clínico: lactante femenina de dos meses con celulitis periorbitaria derecha preseptal no asociada con lesión de entrada y diagnóstico inicial de dacriocistitis derecha. Al examen físico de ingreso, en el ojo derecho se evidencia gran masa abscedada en saco lagrimal; en el ojo izquierdo, un área indurada y leve reflujo a la presión del saco lagrimal. Se realiza tomografía computarizada de órbitas con hallazgos compatibles con dacriocistocele bilateral. Discusión y conclusiones: conocer la presentación y posibles complicaciones asociadas con esta patología previene una morbilidad importante al paciente. La mayoría de los casos de dacriocistocele se pueden manejar médicamente, sin embargo, aquellos asociados con complicaciones requieren de manejo quirúrgico oportuno.

Introduction: Dacryocystocele is a rare congenital malformation secondary to na-solacrimal duct obstruction. It has an incidence of 0.1% of patients with congenital nasolacrimal duct obstruction, being found bilaterally in up to 25% of cases. Case Report: Two-month-old female infant with preseptal right periorbital cellulitis not associated with an entrance lesion, with an initial diagnosis of right dacryocystitis. On physical examination, a large abscessed mass in the lacrimal sac was eviden-ced in the right eye; in the left eye, there was an indurated area and slight reflux to the lacrimal sac pressure. Computed tomography of the orbits was performed with findings compatible with bilateral dacryocystocele. Discussion and conclusions: Knowing the presentation and possible complications associated with this pathology prevents significant patient morbidity. Most cases of dacryocystocele can be mana-ged medically, however, cases associated with complications require timely surgical management.

Técnica de Puckett en el tratamiento de mamas tuberosas con lipotransferencia complementaria / The Puckett Technique for Treating Tuberous Breasts with Complementary Lipotransfer

Introducción: La mama tuberosa es una anomalía congénita de la mama que se presenta en la pubertad y es relativamente frecuente. Objetivo: El objetivo de este artículo es dar a conocer el resultado estético del tratamiento quirúrgico de esta afección con la técnica de Pukett sin incluir el implante mamario, con lipotransferencia complementaria. Presentación del caso: Se presenta una paciente femenina, de 25 años de edad, con mama tuberosa grado 4. Se le realiza corrección con técnica de Pukett sin implantes mamarios y en su lugar se usa grasa autóloga. Luego de corregir las anomalías que conforman esta malformación se obtuvo una mama armoniosa sin complicaciones. Conclusión: La técnica de Pukett combinada con lipotransferencia ofrece resultados estéticos y estables en el tratamiento de la mama tuberosa(AU)

Introduction: Tuberous breast is a congenital breast anomaly that presents at puberty and is relatively frequent. Objective: The aim of this article is to report the aesthetic outcome after treating this condition surgically using the Puckett technique without breast implant, with complementary lipotransfer instead. Case presentation: The is presented of a 25-year-old female patient with grade-4 tuberous breast. She is performed a correction using the Puckett technique without breast implants; autologous fat is used instead. After correcting the anomalies that make up this malformation, a harmonious breast was obtained without complications. Conclusion: The Puckett technique combined with lipotransfer offers aesthetic and stable outcomes in the treatment of tuberous breast(AU)

Access to emergency surgical treatment for congenital heart diseases in the state of São Paulo

Abstract Objective To analyze access to surgical care for congenital heart diseases in public specialized centers in the state of São Paulo before and during the COVID-19 pandemic and availability of surgical care in specialized hospitals, to identify bottlenecks in the care path for neonatal cardiac surgery. Methods This study included 1,437 children, under one year old, with congenital heart disease and formal referral to heart surgery between February 1, 2019, and February 28, 2021. Quantitative data analysis was performed using t-tests or the Mann-Whitney test. Results Approximately 30 % of children with urgent congenital heart disease could not access recommended care, mainly those needing complex surgeries (categories RACHS 4 to 6). The main diagnoses of neonates accessing care were patency of the ductus arteriosus (10.5 %) and coarctation of the aorta (10.1 %). Referral time for children in RACHS 1 to 3 was 4 days (median), while for those in categories 4 to 6, it was 7 days (p< 0.001). Longer referral time (20 days) was associated with court orders. During the pandemic, referral time decreased to 3 days, compared to 5 days (median) in the pre-pandemic period (p< 0.001). Conclusion The emergency surgical treatment supply for congenital heart diseases is insufficient compared to the current demand. Future research should evaluate if access to care in publicly funded hospitals could be improved by better access to prenatal care for pregnant women, increased diagnostic and therapeutic capacity in pediatric cardiology, and financial incentives for complex cardiac surgeries.

Patau and Edwards Syndromes in a University Hospital: beyond palliative care / Síndrome de Edwards e Patau em um hospital universitário: além do paliativo

ABSTRACT Objective: To describe the newborn population with Patau (T13) and Edwards Syndrome (T18) with congenital heart diseases that stayed in the Intensive Care Unit (ICU) of a quaternary care hospital complex, regarding surgical and non-surgical medical procedures, palliative care, and outcomes. Methods: Descriptive case series conducted from January/2014 to December/2018 through analysis of records of patients with positive karyotype for T13 or T18 who stayed in the ICU of a quaternary hospital. Descriptive statistics analysis was applied. Results: 33 records of eligible patients were identified: 27 with T18 (82%), and 6 T13 (18%); 64% female and 36% male. Eight were preterm infants with gestational age between 30-36 weeks (24%), and only 4 among the 33 infants had a birth weight >2500 g (12%). Four patients underwent heart surgery and one of them died. Intrahospital mortality was 83% for T13, and 59% for T18. The majority had other malformations and underwent other surgical procedures. Palliative care was offered to 54% of the patients. The median hospitalization time for T18 and T13 was 29 days (range: 2-304) and 25 days (13-58), respectively. Conclusions: Patients with T13 and T18 have high morbidity and mortality, and long hospital and ICU stays. Multicentric studies are needed to allow the analysis of important aspects for creating protocols that, seeking therapeutic proportionality, may bring better quality of life for patients and their families.

RESUMO Objetivo: Descrever a população de recém-nascidos com síndrome de Patau (T13) e Edwards (T18) portadores de cardiopatias congênitas, que permaneceram em Unidades de Terapia Intensiva (UTI) de um complexo hospitalar quaternário, com relação a conduta cirúrgica ou não, cuidados paliativos e seus desfechos. Métodos: Série de casos de pacientes internados entre janeiro de 2014 a dezembro de 2018, com análise dos prontuários de portadores de T13 ou T18 que permaneceram internados em UTI que recebem neonatos nesse hospital quaternário. Utilizou-se análise estatística descritiva. Resultados: Foram identificados 33 prontuários para análise — 27 T18 (81,8%) e seis T13 (18,2%); 64% do sexo feminino e 36% do sexo masculino. Oito foram prematuros, nascidos com 30 a 36 semanas (24,2%), e apenas quatro nasceram com mais de 2500 g (12,1%). Quatro pacientes foram submetidos a cirurgia cardíaca e um deles foi a óbito. A mortalidade intra-hospitalar foi de 83% para T13 e 59% para T18. A maioria apresentava outras malformações e foi submetida a outras cirurgias. Cuidados paliativos foram oferecidos a 54% dos pacientes. A mediana do tempo de hospitalização para T18 e T13 foi respectivamente de 29 dias (variação: 2-304) e 25 dias (13-58). Conclusões: Pacientes com T13 e T18 cursam com alta morbimortalidade e longa permanência hospitalar em UTI. São necessários estudos multicêntricos para melhor análise de aspectos importantes para a criação de protocolos que, buscando proporcionalidade terapêutica, tragam melhor qualidade de vida para os pacientes e suas famílias.

Catarata congênita nas infecções intrauterinas mais comuns (STORCH): revisão de literatura / Congenital cataract in the most common intrauterine infections (STORCH): a review of the literature

RESUMO Objetivo: O objetivo deste estudo foi revisar os aspectos clínicos e patológicos da catarata congênita secundária às infecções por sífilis, toxoplasmose, rubéola, citomegalovírus e herpes simples. Métodos: Trata-se de uma revisão de literatura, na qual foram incluídos artigos de periódicos indexados às bases de dados PubMed®, Cochrane, Lilacs, Embase e SciELO de 2010 a 2023. Resultados: Foram encontrados 45 artigos, e, após seleção, restaram 9 artigos. Além disso, foram adicionados artigos para enriquecer a discussão. A infecção por sífilis está relacionada a alterações corneanas. O citomegalovírus e a toxoplasmose estão relacionados com a coriorretinite e/ou microftalmia. A rubéola é responsável por causar catarata, glaucoma, microftalmia e retinite em sal e pimenta. Conclusão: Foram abordadas as principais etiologias infecciosas e seu quadro clínico na CC. O melhor tratamento para CC é cirúrgico associado a acompanhamento clínico, mas a prevenção é a maneira mais eficaz de combater a CC de etiologia infecciosa. O diagnóstico precoce e o tratamento efetivo previnem alterações e sequelas visuais irreversíveis. Nesse contexto, mostram-se importantes as ações de políticas públicas para o melhor desfecho clínico e melhor qualidade de vida.

ABSTRACT Objective: To review the clinical and pathological aspects of CC secondary to infections by syphilis, toxoplasmosis, rubella, cytomegalovirus, herpes simplex. Methods: This is a literature review. Articles from journals indexed to PubMed, COCHRANE, LILACS, EMBASE and SCIELO from 2010 to 2023 were included. Results: A total of 45 articles were found, which, after selection, remained in 9 articles. Some articles were included to enrich the discussion in this topic. The infection caused by syphilis is related to corneal changes. Cytomegalovirus and Toxoplasmosis due to chorioretinitis and/or microphthalmia. Rubella is responsible for causing cataracts, glaucoma, microphthalmia, and salt and pepper retinitis. Conclusion: The main infectious etiologies and their clinical status in CC were addressed. The best treatment for CC is surgery associated with clinical follow-up, but prevention is the most effective way to combat CC of infectious etiology. Early diagnosis and effective treatment prevent irreversible visual changes and sequelae. In this context, public policy actions are important for the best clinical outcome and better quality of life.

Infección congénita por citomegalovirus en neonatos muy bajo peso

Introducción: La infección congénita por el citomegalovirus en neonatos menores de 1500 gramos puede ser causa de morbilidad, mortalidad y discapacidad. Objetivo: Describir el comportamiento de la infección congénita por citomegalovirus en un servicio de neonatología. Métodos: Se realizó un estudio descriptivo y transversal con 61 neonatos. Se les realizó detección de citomegalovirus en la primera semana de vida en suero y orina, mediante reacción en cadena de la polimerasa, para determinar infección congénita. Se evaluaron variables perinatales en todos los neonatos, así como elementos clínicos y resultados de exámenes complementarios en los infectados. Resultados: La incidencia de infección congénita fue de un 10 % (6/61). El 5 % de los estudios fueron positivos (6/122). Ninguna muestra de orina resultó positiva (0/61) y en el 10 % de las muestras de suero (6/61) se detectó el genoma del virus. Se encontró asociación entre valoración nutricional al nacer e infección por citomegalovirus (p< 0,05). El 83 % de los neonatos infectados presentaron algún signo clínico y el síndrome de dificultad respiratoria fue el más frecuente (67 %). En todos los neonatos con infección congénita el ultrasonido cerebral fue normal y en el 33 % se detectó retinopatía de la prematuridad en el fondo de ojo. Conclusiones: La incidencia de infección congénita por citomegalovirus es alta en este grupo de riesgo. Los signos clínicos encontrados y los resultados del fondo de ojo en neonatos con infección congénita se relacionaron con la prematuridad y la valoración nutricional de hipotrófico se asoció con esta infección.

Introduction: Congenital cytomegalovirus infection in neonates weighing less than 1500 grams can be a cause of morbidity, mortality, and disability. Objective: To describe the behavior of congenital cytomegalovirus infection in a neonatal service. Methods: A descriptive and cross-sectional study was conducted with 61 neonates. Cytomegalovirus was detected in the first week of life in serum and urine, by polymerase chain reaction, to determine congenital infection. Perinatal variables were evaluated in all neonates, as well as clinical elements and results of complementary examinations in infected infants. Results: The incidence of congenital infection was 10% (6/61). 5% of the studies were positive (6/122). No urine samples were positive (0/61) and the virus genome was detected in 10% of serum samples (6/61). An association was found between nutritional assessment at birth and cytomegalovirus infection (p < 0.05). A total of 83% of infected neonates had some clinical sign, with respiratory distress syndrome being the most common (67%). In all neonates with congenital infection, brain ultrasound was normal, and retinopathy of prematurity was detected in 33% of patients with fundus retinopathy. Conclusions: The incidence of congenital cytomegalovirus infection is high in this risk group. The clinical signs found and the results of the fundus in neonates with congenital infection were related to prematurity and the nutritional assessment of hypotrophic was associated with this infection.

Complete congenital heart block in the offspring of an asymptomatic woman with isolated high titer anti-ro antibody

@#Among pregnant women, 1-2% are anti-Ro positive and while half of them have symptoms of connective tissue disease, the rest are asymptomatic. The presence of anti-Ro is of concern because of the risk of congenital heart block in the child. We report the case of an asymptomatic 27-year-old G2P1(1001) woman, who presented with persistent fetal bradycardia in her 21st week of gestation (AOG) and was found to have elevated titers for anti-Ro (>320 U/ml). Hydroxychloroquine 200 mg/day and prednisone 10 mg/day were given from the 33rd week of gestation up until the delivery. At 37 weeks AOG, she delivered a live male neonate with a complete heart block. On the 6th day of life, the infant remained bradycardic, hence a pacemaker was inserted and heart rate maintained at 100-120 bpm. On subsequent follow-ups, the mother and child did not develop any systemic manifestations and the infant was thriving well. While a diseased condition may not be apparent in a pregnant anti-Ro positive woman, the risk of neonatal lupus (NL) is demonstrated in this patient’s case. This report illustrates how prenatal care of an asymptomatic woman led to the discovery of a fetal abnormality and served to prepare the family and the medical team to ably handle the birth and subsequent care of a neonate with NL.

Evaluation of the quality of prenatal care in Colombia during the 2016 Zika outbreak in endemic and non-endemic areas

Objetive: To evaluate the quality of prenatal care during the outbreak of Zika virus infection in endemic and non-endemic vector-borne regions in Colombia. Materials and methods: A descriptive study of prenatal care supplemented by interviews to explore personal experiences during the epidemic. A total of 40 pregnant women in endemic areas and 44 in nonendemic areas participated. Information collected included previous pregnancies, reasons for starting prenatal care, information about Zika, prenatal care (activities of doctors, nurses, laboratories, and images), and perceptions of quality. Then, 8 interviews were conducted with pregnant women diagnosed with Zika. Questioned about knowledge of Zika and the quality of medical care services. Results: Problems with laboratories and diagnostic images were found in both regions and dehumanizing treatment in the endemic region. Women from the endemic region received news and communications about the effects of Zika during pregnancy, causing anxiety and fear among some women. The quality of health care was not what the women expected and they thought they would receive more care from doctors and nurses. Discussion: Our findings show deficiencies in education provided in health institutions. The experience during prenatal control in the endemic regions was imprecise and the information came from other sources, different from the health sector. Adittionally, support and follow-up was deficient as well. It's possible that health professionals have few knowledge about information management, which generated confusion, fear and uncertainty among the pregnant women about the adverse effects on the newborns. Conclusions: Findings suggest deficiencies in the technical quality of the prenatal care provided, particularly in the region that was endemic for vector-borne diseases. Reproductive health services and the technical quality of prenatal care need to be strengthened, especially during a sanitary crisis.

Objetivo: Evaluar la calidad de la atención prenatal durante la epidemia de Zika en regiones endémicas y no endémicas de infecciones trasmitidas por vectores en Colombia. Materiales y Métodos: Estudio descriptivo de la atención prenatal complementado con entrevistas, con el fin de explorar experiencias personales durante la epidemia. Participaron 40 gestantes en zona endémica y 44 en no endémicas. La información recolectada incluyó embarazos previos, razones para iniciar control prenatal, información sobre Zika, control prenatal (actividades de médicos, enfermeras, laboratorios e imágenes) y percepción de calidad. Luego se realizaron 8 entrevistas a gestantes con diagnóstico de Zika. Se interrogó sobre conocimiento del Zika y calidad de los servicios de atención médica. Resultados: Se encontraron problemas con laboratorios e imágenes diagnósticas en ambas regiones y trato deshumanizado en la región endémica. Las gestantes en la región endémica recibieron noticias y comunicados sobre los efectos de Zika durante el embarazo, lo que causó ansiedad y miedo en algunas mujeres. La calidad de la atención medica no era la deseada y pensaron que recibirían mejor atención de médicos y enfermeras. Discusión: Nuestros hallazgos demuestran educación deficiente en las instituciones de salud. La experiencia durante el control prenatal en las regiones endémicas fue imprecisa y venía de fuentes diferentes al sector salud. Además, el soporte y seguimiento fue deficiente. Es probable que el personal de salud tenga poco conocimiento sobre cómo manejar la información, lo cual generó confusión, miedo e incertidumbre entre las gestantes sobre los efectos adversos en los bebes. Conclusiones: Los hallazgos sugieren deficiencia en la calidad técnica de la atención prenatal, particularmente en la región endémica. Es necesario fortalecer los servicios de salud reproductiva y la calidad técnica de la atención prenatal, especialmente durante crisis sanitaria.

Intestinal Nonrotation, Imaging Findings in the Malrotation Spectrum: Case Report

Intestinal nonrotation is a rare embryonic developmental anomaly with a reported incidence of up to 0.5% in autopsies. Given the asymptomatic course, the diagnosis may be late, so it becomes an incidental finding. This study presents the most common imaging characteristics to familiarize readers with this pathology. We describe the case of a 73-year-old patient who consulted for pain in the right inguinal region associated with the sensation of a mass. There were no significant findings on physical examination. An abdominal tomography with contrast was requested as a study method, identifying a reversal of the superior mesenteric artery/superior mesenteric vein relationship with the cecum, appendix, and ileocecal valve in the left flank and mesogastrium. A displacement of thin intestinal loops towards the right hemiabdomen was also noted.

La no rotación intestinal es una anomalía del desarrollo embrionario poco frecuente con una incidencia reportada de hasta el 0,5 % en autopsias. El diagnóstico puede ser tardío dado el curso asintomático, por lo que se convierte en un hallazgo incidental. El objetivo de este estudio es dar a conocer las características por imagen más comunes para familiarizar a los lectores con esta patología. Se presenta el caso de un paciente de 73 años que consultó por dolor en la región inguinal derecha asociado a sensación de masa. En el examen físico no hubo hallazgos significativos. Se solicitó una tomografía abdominal con contraste como método de estudio y se identificó una inversión de la relación entre la arteria y vena mesentérica superior con la presencia de ciego, apéndice y válvula ileocecal en el flanco izquierdo y mesogastrio. Adicionalmente, se observó un desplazamiento de asas intestinales delgadas hacia el hemiabdomen derecho.