Eventos adversos em crianças submetidas a cirurgias orofaciais / Adverse events in children submitted to orofacial surgery / Eventos adversos en niños sometidos a cirugía orofacial

Objetivo: Identificar os eventos adversos no pós-operatório imediato de queiloplastia e/ou palatoplastia em crianças e comparar os eventos identificados aos notificados ao Núcleo de Segurança do Paciente. Métodos: Estudo descritivo, retrospectivo e quantitativo, realizado em um hospital público e terciário brasileiro. Os dados foram coletados por meio da descrição nos registros de enfermagem e comparados aos notificados ao Núcleo de Segurança do Paciente, referente a junho e dezembro de 2019. Os resultados foram submetidos a análise estatística descritiva. Resultados: A amostra constou de 203 crianças, das quais 51% (n=103) apresentaram evento adverso. Foram identificados 176 eventos adversos, de 8 tipos, com prevalência da laringite pós-extubação (n=50; 28%), edema de língua (n=34; 19%) e lesão de comissura labial (n=25; 14%). Destes, apenas 5% (n=9) foram notificados ao Núcleo de Segurança do Paciente. Conclusão: Os eventos adversos prevalentes se relacionaram a cavidade oral e tecidos adjacentes, e a subnotificação foi expressiva. (AU)

Objective: To identify adverse events in the immediate postoperative period of cheiloplasty and/or palatoplasty in children and compare the identified events to those notified to the Patient Safety Center. Methods: Descriptive, retrospective and quantitative study, carried out in a Brazilian public and tertiary hospital. Data were collected through descriptions in nursing records and compared to those notified to the Patient Safety Center, referring to June and December 2019. The results were subjected to descriptive statistical analysis. Results: The sample consisted of 203 children, of which 51% (n=103) had an adverse event. A total of 176 adverse events of 8 types were identified, with prevalence of post-extubation laryngitis (n=50; 28%), tongue edema (n=34; 19%) and labral commissure lesion (n=25; 14%). Of these, only 5% (n=9) were notified to the Patient Safety Center. Conclusion: The prevalent adverse events were related to the oral cavity and adjacent tissues, and underreporting was significant. (AU)

Objetivo: Identificar eventos adversos en el postoperatorio inmediato de queiloplastia y/o palatoplastia en niños y comparar los eventos identificados con los notificados al Centro de Seguridad del Paciente. Métodos: Estudio descriptivo, retrospectivo y cuantitativo, realizado en un hospital público y terciario brasileño. Los datos se recolectaron mediante descripciones en registros de enfermería y se compararon con los notificados al Centro de Seguridad del Paciente, referidos a junio y diciembre de 2019. Los resultados fueron sometidos a análisis estadístico descriptivo. Resultados: La muestra estuvo conformada por 203 niños, de los cuales el 51% (n = 103) tuvo un evento adverso. Se identificaron un total de 176 eventos adversos de 8 tipos, con prevalencia de laringitis posextubación (n=50; 28%), edema de lengua (n=34; 19%) y lesión de la comisura del labrum (n=25; 14%). De estos, solo el 5% (n=9) fueron notificados al Centro de Seguridad del Paciente. Conclusion: Los eventos adversos prevalentes se relacionaron con la cavidad bucal y los tejidos adyacentes y el subregistro fue significativo. (AU)

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.

Perceptions and feelings of pregnant women undergoing outpatient follow-up for toxoplasmosis

Introduction: Toxoplasmosis persists as a neglected disease and poses a challenge to public health, especially due to the risk of vertical transmission, which can lead to countless biological complications for the newborn and to psychological and emotional repercussions for the mother. Objective: To understand the perceptions and feelings of pregnant women affected by toxoplasmosis undergoing outpatient follow-up. Materials and Methods: A qualitative and exploratory study developed with 12 women with gestational toxoplasmosis undergoing specialized outpatient follow-up in a municipality from the state of Paraná, Brazil. The data were collected through semi-structured individual interviews and subjected to content analysis, supported by descending hierarchical classification. Results: The pregnant women experienced situations ranging from diagnosis and treatment to preventing the disease in the child and family. These experiences generated fear, distress and uncertainty about the disease, which were not adequately addressed during prenatal assistance in primary care. However, the pregnant women emphasized the importance of the multiprofessional team at the secondary level in monitoring and health education. Discussion: Although the pregnant women felt confident about the treatment and its implications for the child's health, discovering the diagnosis impacted their everyday lives and those of their families, especially due to lack of reliable information about toxoplasmosis and to the absence of emotional support at the primary level. Conclusions: There was a temporary scenario of disinformation among these women, who were not properly guided and supported. However, the guidelines offered in secondary health care were essential for improving knowledge and practices in health.

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.

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.

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)

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.

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.

Effects of prolonged waiting time on visual outcomes of patients with pediatric cataract in a Tertiary Public Hospital

Background@#Pediatric cataract is one of the most common preventable cause of childhood blindness worldwide. Early and timely intervention of pediatric cataract is important to maximize the visual outcomes and start prompt visual rehabilitation.@*Objectives@#This study aimed to determine the average time from the day of initial consult at the outpatient clinic to the day of the cataract surgery and compare the effects of delayed surgery on visual outcomes of patients.@*Methods@#This is a retrospective chart review of medical records from January 2015 to June 2022. The dates of the different steps in the process up to the day of intervention were noted and the average interval duration and the total waiting time were determined. Patients operated on within 2 weeks from initial consult was defined as no delay while those operated >2 weeks had delayed surgery. Pre-operative and post-operative best corrected log MAR visual acuity were compared within each group to determine if delay in surgical intervention has a significant effect on the visual outcomes of patients.@*Results@#Median age at initial consult was 4.9 years while median age at surgery was 5.2 years. Ninety-nine (99) patients had developmental cataract and 123 patients had bilateral cataract. Leukocoria was the most common chief complaint (63.45%). Pre-operatively, 94 patients had strabismus, 49 had eye preference, 48 had nystagmus, and 43 had amblyopia in the diagnosis. There was significantly faster admission to cataract surgery during the pandemic compared to pre-pandemic period but there was no difference in the total waiting time. Patients with congenital cataract had the least total waiting time followed by developmental, and rubella cataract. There is no significant difference in visual outcomes between patients operated without delay and with delay.@*Conclusion@#There is delayed age at diagnosis and surgery of pediatric cataract patients in the Philippine General Hospital. Early surgery did not reflect better visual outcomes compared to delayed surgery probably due to delay in consultation of patients.

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.

Pharmaceutical care for a patient with atrial flutter，atrial fibrillation，placenta praevia hemorrhage and throm-bocytopenia-threatened abortion after operation of congenital heart disease / 中国药房

OBJECTIVE To provide a reference for the safe administration of patients with atrial flutter， atrial fibrillation， placenta praevia hemorrhage and thrombocytopenia-threatened abortion after the operation of congenital heart disease. METHODS Clinical pharmacists participated in the diagnosis and treatment of a patient with atrial flutter， atrial fibrillation， placenta praevia hemorrhage and thrombocytopenia-threatened abortion after the operation of congenital heart disease. Given the thrombocytopenia caused by enoxaparin sodium， pharmacists suggested to stop enoxaparin sodium and change it to fondaparinux sodium after a blood routine review. For the patient with fast heart rate and low blood pressure， pharmacists recommended to choose metoprolol and adjust the dosage according to the heart rate， and change furosemide to hydrochlorothiazide. Pharmacists recommended to continue using metoprolol regarding doctors’ plan to replace metoprolol with sotalol before cesarean section. For possible drug interactions in the patient， pharmacists recommended to closely monitor blood potassium and other indicators， and provided drug education. RESULTS The doctors adopted the advice of clinical pharmacists. The patient’s bleeding was controlled， the indicators were kept stable during hospitalization， the gestational week was extended smoothly， and the cesarean section was successfully performed. CONCLUSIONS By participating in the treatment of the patient with atrial flutter， atrial fibrillation， placenta praevia hemorrhage and thrombocytopenia-threatened abortion after the operation of congenital heart disease， clinical pharmacists formulate individualized medication plans for the patient based on adverse drug reactions， drug interactions and medication education， ensuring the safety and effectiveness of medication.

Comparison of Congenital Rubella Syndrome Cases at a Philippine Tertiary Hospital from 2009-2012 to 2019-2022

Background and Objective@#The Philippines does not have a national congenital rubella syndrome (CRS) surveillance or registry. Regular monitoring of CRS cases in hospitals, including in a Philippine tertiary hospital, helped in the past to provide clinico-epidemiologic data on CRS. This study aimed to continue providing clinico-epidemiologic data on CRS cases seen in the Philippine tertiary hospital from 2009-2012 and 2019-2022 and compare the cases seen from said timelines.@*Methods@#A cross-sectional study was used, employing chart review of patients newly diagnosed with CRS from 2009-2012 and 2019-2022 in the Department of Ophthalmology and Visual Sciences at the Philippine tertiary hospital.@*Results@#Forty-two patients newly diagnosed with CRS from 2009-2012 and 2019-2022 were included. Only 14 (33%) were serologically-confirmed cases (albeit qualitatively). Median age (first and third interquartile ranges) at consult was 1 year (0.4, 2.5). Twenty-four (57%) patients had maternal history of rashes and/or fever. Trimester of pregnancy when mother became symptomatic was not significantly correlated with chief complaint (p=0.20) and numbers of ophthalmic (p=0.68) and systemic manifestations (p=0.32). Cataract was the most common ophthalmic manifestation present in 40 (95%) patients. Twenty-six (62%) patients had other associated systemic findings of which hearing loss was the most common. Only 29 of 40 patients with cataract underwent lensectomy, with 23 patients having poor visual prognosis prior to surgery (5 with nystagmus alone, 10 with nystagmus and strabismus, and 8 with strabismus alone). @*Discussion@#Using ophthalmic manifestations as primary indicator, this study provided an update on the CRS cases in the country. Laboratory confirmation remains a challenge in diagnosing CRS as the tests are costly and not widely available. There was increase from 2009-2012 compared to 2019-2022 in number of patients who underwent surgical treatment for cataract but visual outcomes were suboptimal due to delay in consultation. Although there was a decrease in number of CRS cases seen in the Philippine tertiary hospital, this cannot be attributed to increased rubella-containing vaccine (RCV) coverage alone. @*Conclusion@#Provision of data from individual hospital-based studies similar to this highlights the need for a national CRS surveillance system or registry. This can better gauge the burden of CRS and identify the gap in RCV coverage.

Gestational surrogacy / Philippine Journal of Obstetrics and Gynecology

@#Some women may not be able to carry their own children even when capable of conceiving biological offspring. In-vitro fertilization and embryo-transfer (IVF-ET) through surrogacy can now make this possible for these women. Surrogacy however, is still considered unacceptable in the Philippines due to moral and legal issues. This article will explore the need and acceptability of surrogacy in this age of IVF-ET in a country where the prevailing social norms and religious values still disapprove of third-party assisted reproductive technology (ART). Medical indications that would benefit from gestational surrogacy were enumerated and briefly discussed. The differentiation between traditional and gestation surrogacy, as well as commercial and altruistic surrogacy were defined. IVF with gestational surrogacy is a feasible solution to a number of medical difficulties in the carrying of a gestation. Strictly regulating the practice and restricting its use only to cases with legitimate medical indications will prevent its misuse and exploitation. Moral issues, admittedly will still remain an issue particularly for commercial surrogacy. However, limiting these only to altruistic and gestational surrogacy in some cases may be an acceptable compromise.

Restoration of a dental defect in a patient with ectodermal dysplasia: a case report and literature review / 口腔疾病防治

Objective@#To explore the treatment options for congenitally missing teeth in patients with ectodermal dysplasia and provide a clinical reference.@*Methods@#A patient with ectodermal dysplasia with a concave midface, anterior protrusion of the chin, and underdevelopment of the lower third of the face presented with congenital loss of multiple maxillary teeth, malocclusion of the remaining teeth, congenital loss of mandibular dentition, small dental arches, and upper and lower alveolar bone hypoplasia. The patient was treated by means of a removable partial maxillary prosthesis, implants in the anterior region of the lower mandible designed with the assistance of digital guides, and bar-clamped implant-overlay prostheses. A literature review of the protocol for the treatment of this condition was also conducted.@*Results@#In addition to good retention and stability after denture wear, an excellent occlusal relationship, improvement of the patient's facial appearance, including upper and lower lip fullness, more equal balancing of the lower and middle 1/3 of the face, and improved masticatory function were achieved. The results of the literature review showed that patients with ectodermal dysplasia who are congenitally edentulous usually have a complex intraoral situation that makes restoration difficult, and common restorative modalities for these patients include fixed bridges, removable partial dentures, complete dentures, overdentures, and implant prostheses, which need to be selected according to the actual intraoral situation of each patient. Currently, there is no consensus on the treatment of congenitally missing teeth in patients with ectodermal dysplasia, and some scholars have suggested that fixed restorations be recommended for patients with fewer missing teeth, while the option of removable or implant-covered denture restorations should be given to patients with more missing teeth, with removeable prostheses for underage patients that are replaced with permanent fixed prostheses when the jaws have stabilized.@*Conclusion@#In patients with ectodermal dysplasia with congenital tooth loss, all factors should be taken into account, and an individualized restorative plan should be developed.

Progress in surgical treatment of congenital entropion / 国际眼科杂志(Guoji Yanke Zazhi)

Congenital entropion, an abnormal condition in which the eyelids roll inward, with the eyelashes losing their normal angulation and tilting toward the eye, especially in the lower eyelids and inner canthus, often occurs in infants and young children. Congenital entropion may lead to corneal epithelial abrasion, inflammation and ulcer, which may affect the function of the eye if not treated in time. Early surgical intervention is helpful to the health of children's eyes. The purpose of surgery is to change the structure of eyelid and weaken the force of entropion, thus improving the symptoms and corneal astigmatism. At present, there are many surgical treatments for congenital entropion. In this paper, the advantages, disadvantages and indications of these treatments are analyzed and summarized, providing a reference for clinical practice.

A case report of a Filipino boy with childhood cataract and clinically diagnosed Roberts Syndrome

@#Childhood cataract is a common cause of visual impairment. Familial types are uncommon among Filipinos. Furthermore, it is not common to have one that follows an autosomal dominant pattern of inheritance but with associated syndromic presentation like Roberts syndrome which is an autosomal recessive disorder. This is a case of a 9-year-old Filipino boy with cataract in the left eye associated with low-set ears, facial asymmetry, underdeveloped nasal ala, cleft lip and palate, macroglossia, micrognathia, short right shin, and absent feet. Patient was clinically diagnosed with Roberts syndrome. We present a clinically diagnosed Roberts syndrome (RS), the first reported RS in a Filipino in local and international literature to our knowledge with an autosomal dominant childhood cataract. Genetic testing can assist in the confirmation of this case.

Research progress in liver transplantation in patients with situs inversus totalis / 器官移植

Situs inversus totalis (SIT) is a rare congenital condition, with an extremely low incidence. There is no difference between SIT individuals without onset of diseases and healthy counterparts. However, when SIT individuals suffer from diseases, the diagnosis and treatment are highly challenging due to insufficient understanding of SIT populations, especially for those complicated with end-stage liver disease and requiring liver transplantation. It is a huge challenge for surgeons whether SIT individuals serve as donors or recipients of liver transplantation. In this article, recent case reports related to liver transplantation in SIT patients were summarized, and the development, key procedures, clinical prognosis and postoperative complications of liver transplantation in SIT patients were reviewed.

Study on the management of granulation during surgery for congenital preauricular fistula infection stage / 临床耳鼻咽喉头颈外科杂志

Objective:To investigate the management of granulation tissue during surgery for infected congenital preauricular fistula and to assess the surgical outcomes. Methods:To summarize the surgical methods and the treatment of granulation methods in 140 cases of congenital preauricular fistula during the period of infection treated in our department from January 2018 to September 2022. The study divided patients into an observation group （79 patients） undergoing fistulectomy without granulation treatment, and a control group （61 patients） where fistulectomy and granulation resection were performed concurrently.. After six months of follow-up, the wound healing, recurrence rates, and the aesthetic assessment of granulation healing were evaluated using the Stony Brook Scar Evaluation Scale（SBSES）. Results:The two surgical approaches were applied to a total of 140 patients with infected congenital preauricular fistula. There was no statistical difference in wound healing and recurrence rates between the observation group and the control group. However, the observation group exhibited smaller scars. Conclusion:In cases of infected congenital preauricular fistula, surgical removal without excising granulation tissue is feasible, leading to effective healing and lesser scar formation.