Técnica de Kapetansky-Juri modificada para la corrección de defectos labiales en silbido / Modified Kapetansky-Juri technique to correct whistling lip defects

RESUMEN La deformidad en silbido es uno de los defectos residuales más frecuentes entre pacientes operados de hendiduras congénitas del labio superior. La técnica de Kapetansky-Juri ha permitido corregir los defectos en silbido (moderados y graves). Se presentó una paciente de 16 años de edad operada de una hendidura bilateral labio palatina; a causa de esta hipoplasia regional y tras el proceso de crecimiento y desarrollo craneofacial, persistió la insuficiencia del prolabio como un defecto en silbido moderado. La paciente fue tratada con la técnica de Kapetansky-Juri con una pequeña modificación, lo que permitió, no solo dar volumen al prolabio, sino también un intento en la corrección del arco de Cupido. Los resultados alcanzados fueron satisfactorios y se corresponden con los objetivos finales de toda cirugía reparadora de las hendiduras labio alveolo palatinas.

ABSTRACT Whistling deformity is one of the most frequent residual defects in patients operated on for congenital clefts of the upper lip. Kapetansky-Juri technique has made it possible to correct whistling defects (moderate and severe). We present a 16-year-old female patient operated on a bilateral cleft lip and palate; given this regional hypoplasia and after the process of growth and craniofacial development, insufficiency of the prolabium persisted as a moderate whistling defect. She was treated with the Kapetansky-Juri technique with a small modification, allowing us not only to give volume to the prolabium, but also an attempt to correct the Cupid's bow. The achieved results were satisfactory and correspond to the final objectives of all reconstructive surgery of the cleft lip and palate.

Morbidity and mortality due to surgical congenital malformations from the perspective of surgical neonatal ICU outside a maternity service: a retrospective cohort study

SUMMARY OBJECTIVE: To describe the characteristics of patients treated at a level III surgical Neonatal Intensive Care Unit outside of a maternity service and analyze possible risk factors for mortality in this population. METHODS: A retrospective cohort study evaluating patients admitted to a level III surgical Neonatal Intensive Care Unit from June/2015 to November/2017. Univariate analysis was performed by the Chi-square test and T-student test or Mann-Whitney test. Multivariate analysis by logistic regression was performed including in the model the variables with a P-value <0.2 in univariate analysis. Kaplan-Meier curve and Log-Rank test were performed using the variables that were statistically associated with death in the multivariate analysis. A significance level of a=5% and an error B=80% were adopted. RESULTS: During this period, 246 patients were admitted to this service. 58 (23.8%) patients died, with a mean time until death of 18 days. Half of the patients had a clinical diagnosis of sepsis (50.6%), blood culture was positive in 25.2%, and gram-positive bacteria (48.4%) were the main pathogens isolated. The variables that remained in the final model after multivariate analysis were diagnosis of congenital heart disease (OR = 4.5; p = 0.016), clinical diagnosis of sepsis (OR = 8.1; p = 0.000), and isolation of gram-positive bacteria in blood culture (OR = 3.9; p = 0.006). CONCLUSION: The level III surgical Neonatal Intensive Care Unit outside of a maternity service has a different profile of morbidity and mortality, and death was associated with the diagnosis of congenital heart disease, the clinical diagnosis of sepsis, and the isolation of gram-positive bacteria in the blood culture

RESUMO OBJETIVO: Traçar o perfil de pacientes atendidos em uma Unidade de Terapia Intensiva Neonatal cirúrgica nível III desvinculada de maternidade e analisar fatores de risco para mortalidade nesta população. MÉTODOS: estudo de coorte retrospectivo, avaliando os pacientes internados em um serviço de Unidade de Terapia Intensiva Neonatal cirúrgica nível III de referência no estado no período de junho/2015 a novembro/2017. A análise univariada foi realizada pelo teste de Qui-quadrado e T-student ou Mann-Whitney. Foi realizada a analise multivariada por regressão logística incluindo no modelo as variáveis que apresentaram valor de P <0,2 na analise univariada. Foi realizada curva de Kaplan-Meier e teste Log-Rank com as varáveis que foram estatisticamente associadas ao óbito na análise multivariada. Adotou-se um nível de significância de a=5% e um erro B=80%. RESULTADOS: Neste período, 246 pacientes foram internados neste serviço. Foram a óbito 58 (23,8%) pacientes, com tempo médio de óbito de 18 dias. Metade dos pacientes apresentaram diagnóstico clínico de sepse (50,6%), com hemocultura foi positiva em 25,2% e bactérias gram-positivas (48,4%) foram os principais patógenos isolados. As variáveis que se mantiveram no modelo final após a análise multivariada foram diagnóstico de cardiopatia congênita (OR=4,5; p=0,016), diagnóstico clinico de sepse (OR=8,1; p=0,000), e isolamento de bactéria gram-positiva na hemocultura (OR=3,9; p=0,006). CONCLUSÃO: O serviço de Unidade de Terapia Intensiva cirúrgica nível III não vinculada a maternidade apresenta perfil diferenciado de morbimortalidade, e o óbito esteve associado ao diagnóstico de cardiopatia congênita, ao diagnostico clinico de sepse e ao isolamento de bactérias gram-positivas na hemocultura.

Manejo pré-natal da hérnia diafragmática congênita: presente, passado e futuro / Prenatal management of congenital diaphragmatic hernia: present, past and future

A hérnia diafragmática congênita é um defeito de formação do diafragma que acomete entre 1:2.000 e 1:4.000 nascidos vivos e constitui 8% das principais anomalias congênitas. Avanços médicos nos últimos 30 anos envolvendo diagnóstico pré-natal, intervenção fetal, manejo clinico e cirúrgico neonatal têm mudado a sobrevivência dos seus portadores. A evolução histórica desses avanços ajuda a compreender o esforço na busca de melhores resultados desse defeito muitas vezes fatal. Perspectivas na utilização de bioengenharia e terapia envolvendo células tronco podem trazer novas esperanças para os fetos com hérnia diafragmática congênita.

The congenital diaphragmatic hernia is a defect in the formation of the diaphragm, which affects between 1:2,000 and 1:4,000 live births and represents 8% of major congenital anomalies. Medical advances in the last 30 years involving prenatal diagnosis, fetal intervention, neonatal surgical and clinical management have changed the survival of these patients. The historical evolution of these advances helps us to understand the effort in pursuit of better results of this defect, which is often lethal. Perspectives on the use of bioengineering and therapy involving stem cells may bring new hope for fetuses with congenital diaphragmatic hernia.

Bifid nose - a mild degree of frontonasal dysplasia. A case report.

Frontonasal dysplasia is an unusual congenital condition with a wide phenotypic range. Because of this, only a small number of cases and their management have been reported in the literature. The ideal surgical procedures to correct mild cases of frontonasal dysplasia, and the time to perform them, are still controversial. The case of a 9-month-old girl with a mild form of this condition (a congenital bifid nose and a duplicated frenulum), and its surgical management, is presented. The surgery achieved an early improvement of the patient's appearance and she had no complications. In the future, it is probable that she will need secondary rhinoplasty to aid in the projection of the tip and refine the shape of the nose.