Hérnia diafragmática congênita simulando derrame pleural: relato de caso / Congenital diaphragmatic hernia simulating pleural effusion: case report

JUSTIFICATIVA E OBJETIVOS: A hérnia diafragmática congênita de Bochdalek se constitui um defeito na posição póstero-lateral do diafragma, ocorrendo normalmente na infância, sendo causa importante de hipoplasia pulmonar. Em adultos, é uma ocorrência rara, com pouco mais de 100 casos descritos. Os adultos comumente são assintomáticos, podendo apresentar sintomas gastrointestinais e raramente, sintomas respiratórios inespecíficos. O objetivo deste estudo foi relatar uma apresentação tardia de hérnia de Bochdalek em um adulto com sintomatologia respiratória. RELATO DO CASO: Paciente do sexo masculino, 30 anos, com queixa de dispneia de longa data, que teve o seu diagnóstico realizado após apresentar um quadro similar a um derrame pleural. CONCLUSÃO: Devido à raridade das hérnias diafragmáticas congênitas em adultos, é necessário raciocínio clínico, técnico e tático diferenciado por parte do médico clínico, o que pode evitar graves complicações como pneumotórax e necrose intestinal.

BACKGROUND AND OBJECTIVES: Bochdalek congenital diaphragmatic hernia constitutes a defect in the posterolateral position of the diaphragm, usually occurring in childhood and being an important cause of pulmonary hypoplasia. In adults,it is a rare occurrence, with little more than 100 cases reported. Adults are usually asymptomatic but gastrointestinal symptoms and, rarely, nonspecific respiratory symptoms, can occur. This study aimed at reporting a late presentation of Bochdalek herniain adult patient with respiratory symptoms. CASE REPORT: Male patient, 30-year-old with a complaint of long-standing dyspnea, who had the diagnosis made after presenting a clinical picture similar to a pleural effusion. CONCLUSION: Since the occurrence of congenital diaphragmatic hernias in adults is very rare, the clinician needs clinical,technical and tactical reasoning, in order to prevent serious complications such as pneumothorax and intestinal necrosis.

Late-Presenting Congenital Diaphragmatic Hernia in Children: The Experience of Single Institution in Korea

PURPOSE: Late-presenting congenital diaphragmatic hernia (CDH) beyond the neonatal period is rare and often misdiagnosed, with delayed treatment. MATERIALS AND METHODS: We retrospectively reviewed our experience with late-presenting CDH over 30 years at a single institution to determine the characteristics of late-presenting CDH for early diagnosis. RESULTS: Seven patients had operations due to late-presenting CHD in our institution over 30 years. The patients' ages ranged from 2.5 months to 16 years. There were six boys and one girl. Five hernias were left-sided, one was right-sided and one was a retrosternal hernia. All patients had normal intestinal rotation. Non-specific gastrointestinal or respiratory symptoms and signs were usually presented. Intestinal malrotations were absent; therefore, only organs adjacent to the defect or relatively movable organs such as the small bowel and transverse colon were herniated. Two cases were accompanied by stomach herniation with the volvulus and liver, respectively. The duration from presentation to diagnosis varied from 5 days to 1 year. Diagnoses were made by chest X-ray, upper gastrointestinal series and chest computed tomography. All patients underwent primary repair with interrupted non-absorbable sutures by a transabdominal approach. None had postoperative complications. The follow-up period in six patients ranged from 4 months to 20 years (median 3.8 years). There was no recurrence in any of the patients on follow-up. CONCLUSION: A high index of suspicion is important for the diagnosis of late-presenting CDH because it can be a life-threatening condition such as CDH with a gastric volvulus. Early diagnosis and appropriate treatment can lead to a good prognosis.

A Simplified Formula Using Early Blood Gas Analysis Can Predict Survival Outcomes and the Requirements for Extracorporeal Membrane Oxygenation in Congenital Diaphragmatic Hernia

The aims of this study were to investigate whether early arterial blood gas analysis (ABGA) could define the severity of disease in infants with congenital diaphragmatic hernia (CDH). We conducted a retrospective study over a 21-yr period of infants diagnosed with CDH. Outcomes were defined as death before discharge, and extracorporeal membrane oxygenation requirements (ECMO) or death. A total 114 infants were included in this study. We investigated whether simplified prediction formula [PO2-PCO2] values at 0, 4, 8, and 12 hr after birth were associated with mortality, and ECMO or death. The area under curve (AUC) of receiver operating characteristic curve was used to determine the optimum ABGA values for predicting outcomes. The value of [PO2-PCO2] at birth was the best predictor of mortality (AUC 0.803, P < 0.001) and at 4 hr after birth was the most reliable predictor of ECMO or death (AUC 0.777, P < 0.001). The value of [PO2-PCO2] from ABGA early period after birth can reliably predict outcomes in infants with CDH.

Effect of nitrofen in the final stages of development of the diaphragm muscle in rats / Efeito do nitrofen na fase final do desenvolvimento da musculatura do diafragma em ratos

PURPOSE: To evaluate the expression of myosin in muscle fibers of the diaphragm in experimental congenital diaphragmatic hernia (CDH). METHODS: Fetuses of pregnant rats were divided into four groups: External Control (EC), composed of non-manipulated rats; Nitrofen, composed of pregnant rats that received 100 mg of nitrofen (2,4-dichloro-4'nitrodiphenyl ether) diluted in olive oil on gestational day (GD) 9.5, whose fetuses developed CDH (N+) or not (N-), and Olive Oil Placebo (OO), composed of pregnant rats that received the oil on the same GD. The fetuses were collected on GD 18.5, 19.5, 20.5 and 21.5 (term = 22 days). We obtained body weight (BW) and photographed the diaphragm area (DA), hernia area (HA) and subsequent calculated the HA/DA ratio in N+ group. Samples of Diaphragm muscle were processed for histological staining with H/E and immunohistochemistry (IHQ) for myosin.} RESULTS: The fetuses of N- and N+ groups had decreased BW and DA compared to EC and OO groups (p <0.001). HA was decreased on GD 18.5 compared to 21.5 (p <0.001) and the HA/DA ratio showed no difference. IHQ showed decreased expression of myosin in nitrofen groups. CONCLUSION: CDH induced by nitrofen model contributes to the understanding of muscularization in the formation of the diaphragm where the myosin expression is decreased.

OBJETIVO: Avaliar a expressão da miosina na muscularização do diafragma na hérnia diafragmática congênita (CDH) experimental. MÉTODOS: Fetos de ratas foram divididos em quatro grupos: Controle Externo (EC), composto de ratas não manipuladas; Nitrofen, composto de ratas que receberam 100 mg de nitrofen (2,4-dicloro-4'nitrodifenil éter) diluído no azeite no dia de gestação (GD) 9.5, cujos fetos desenvolveram CDH (N+) ou não (N-) e Placebo óleo de oliva (OO), composto de ratas que ingeriram apenas óleo no mesmo GD. Os fetos foram coletados com 18,5, 19,5, 20,5 e 21,5 GD (termo = 22 dias). Foi obtido o peso corporal (BW) e tiradas fotografias da área do diafragma (DA), da hérnia (HA) e calculada a relação HA/DA no grupo N+. Amostras de diafragmas foram processadas histologicamente para coloração com H/E e imunohistoquímica. RESULTADOS: Os fetos dos grupos N- e N+ tiveram BW e DA diminuídos em relação aos grupos EC e OO (p<0.001). Só houve diferença na HA entre os GD 18.5 e 21.5 (p<0.001) e a relação HA/DA não mostrou diferença entre os grupos. A imunohistoquímica mostrou menor expressão de miosina nos grupos que receberam nitrofen. CONCLUSÃO: O modelo de CDH induzida por nitrofen contribui para entender a muscularização na formação do diafragma onde a expressão da miosina está diminuída.

Hernia diafragmática congénita: caso clínico y la importancia del diagnóstico prenatal / Congenital diaphragmatic hernia: a case report

Congenital diaphragmatic hernia (CDH) is a disease with a high postnatal morbidity and mortality due to the lung hypoplasia associated, which is why it is so important an early diagnosis, being recommended in average in the second trimester of gestation. This finding allows a proper immediate management of the newborn in a specialized health center. Through a case report we will present a review of CDH types and diagnosis, making emphasis in theultrasonographic diagnosis during pregnancy.

La Hernia Diafragmática Congénita (HDC) es una enfermedad que presenta una alta morbimortalidad postnatal por la hipoplasia pulmonar asociada, poresto es importante un diagnóstico precoz, siendo recomendado en promedio dentro del segundo trimestre de gestación. Este hallazgo permite unadecuado manejo inmediato del recién nacido en un centro de salud especializado. En base al caso clínico que se presentará a continuación realizamos una revisión sobre los tiposy el diagnóstico de la HDC haciendo énfasis en el diagnóstico ultrasonográfico durante el embarazo.

Oclusão traqueal para fetos com hérnia diafragmática esquerda grave isolada: um estudo experimental controlado não randomizado / Tracheal occlusion for fetuses with severe isolated left-sided diaphragmatic hernia: a nonrandomized controlled experimental study

OBJETIVO: Comparar a sobrevida pós-natal de fetos com hérnia diafragmática congênita esquerda grave isolada, os quais foram submetidos à oclusão traqueal, com a de controles não randomizados contemporâneos. MÉTODOS: Estudo experimental não randomizado e controlado, conduzido de abril de 2007 a setembro de 2011. Fetos portadores de hérnia diafragmática congênita esquerda isolada com herniação hepática e relação pulmão/cabeça <1,0, que foram submetidos à oclusão traqueal (grupo de estudo) ou conduta expectante (controles não randomizados contemporâneos), foram comparados quanto à relação pulmão/cabeça e ao observado/esperado da relação pulmão/cabeça no diagnóstico, à idade gestacional por ocasião do parto e sobrevida neonatal com alta do berçário. A evolução da relação pulmão/cabeça e do observado/esperado da relação pulmão/cabeça depois da oclusão traqueal foi descrita. Testes de Fisher, Mann-Whitney e Wilcoxon foram usados na análise estatística. RESULTADOS: Não houve diferenças significativas entre o grupo de estudo (n=28) e o Controle (n=13) quanto à relação pulmão/cabeça (p=0,7) e ao observado/esperado da relação pulmão/cabeça (p=0,5), no momento do diagnóstico, nem à idade gestacional no parto (p=0,146). A sobrevida com alta do berçário foi maior (p=0,012) no grupo da oclusão traqueal (10/28=35,7 por cento) do que no Grupo Controle (0/13=0,0 por cento). Houve aumento da relação pulmão/cabeça (p<0,001) e do observado/esperado da relação pulmão/cabeça (p<0,001) entre o momento do diagnóstico da hérnia diafragmática congênita [relação pulmão/cabeça: 0,8 (0,4-0,9); observado/esperado da relação pulmão/cabeça: 27,0 (15,3-45,0)] e um dia antes da retirada do balão traqueal [relação pulmão/cabeça: 1,2 (0,5-1,8); observado/esperado da relação pulmão/cabeça: 40,0 (17,5-60,0)]. CONCLUSÕES: Houve melhora significativa na sobrevida pós-natal com alta do berçário de fetos com hérnia diafragmática congênita esquerda isolada grave, que foram submetidos à oclusão traqueal em relação a controles não randomizados contemporâneos.

PURPOSE: To compare postnatal survival to hospital discharge of fetuses with severe isolated left-sided congenital diaphragmatic hernia, who underwent tracheal occlusion, with that of nonrandomized contemporaneous controls. METHODS: Experimental nonrandomized controlled study, performed from April 2007 to September 2011. Fetuses with severe isolated left-sided congenital diaphragmatic hernia with liver herniation into the chest and lung area-to-head circumference ratio <1.0, who underwent tracheal occlusion (study group) or expectant management (non-randomized contemporaneous controls), were compared in terms of lung area-to-head circumference ratio and observed/expected lung area-to-head circumference ratio (observed/expected lung area-to-head circumference ratio) at the time of diagnosis, gestational age at birth, and survival to hospital discharge. Modifications in lung area-to-head circumference ratio and o/e lung area-to-head circumference ratio after tracheal occlusion were also analyzed. Fisher's exact test, Mann-Whitney's or Wilcoxon's tests were used for the comparisons. RESULTS: There were no significant differences between the Study Group (TO=28) and Controls (n=13) in terms of the lung area-to-head circumference ratio (p=0.709) and the observed/expected lung area-to-head circumference ratio (p=0.5) at the time of diagnosis and gestational age at birth (p=0.146). The survival to hospital discharge was higher (p=0.012) in the tracheal occlusion group (10/28=35.7 percent) than in controls (0/13=0.0 percent). There was a significant increase in lung area-to-head circumference ratio (p<0.001) and observed/expected lung area-to-head circumference ratio (p<0.001) between the diagnosis of the congenital diaphragmatic hernia [lung area-to-head circumference ratio: 0.80 (0.40-0.94); observed/expected lung area-to-head circumference ratio: 27.0 (15.3-45.0)], and the day before retrieval of the balloon [lung area-to-head circumference ratio: 1.2 (0.50-1.80); observed/expected lung area-to-head circumference ratio: 40.0 (17.5-60.0)]. CONCLUSIONS: There was a significant improvement in the survival rate to hospital discharge of fetuses with severe isolated left-sided congenital diaphragmatic hernia, who underwent tracheal occlusion in comparison to nonrandomized contemporaneous controls.

Recent advances in the management of congenital diaphragmatic hernia.

The management of congenital diaphragmatic hernia (CDH) is undergoing continual change and refinement, fuelled by recent advances in this field. Although many studies have documented the benefits of these recent advances but definite recommendations are lacking. Also, injudicious use of some of these strategies may be counterproductive, underscoring the importance of evidence based treatment strategy. This article discusses the utility of the recent advances in the management of CDH.

Thoraco-abdominal mature teratoma in a left diaphragmatic hernia.

This case describes a contiguous mediastinal and retroperitoneal mature teratoma in a congenital diaphragmatic defect, a combination that is hitherto unreported in literature. It substantiates embryological chronology of events during the cephalad migration of the primordial germ cells through the developing diaphragm, prior to its closure.

Diferentes índices volumétricos dos pulmões de feto com hernia diafragmática congênita avaliados pela ultrassonografia tridimensional / Different volumetric indices of lung in fetuses with congenital diaphragmatic hernia by three-dimensional ultrasonography

Objetivo: avaliar o potencial dos diferentes índices volumétricos de pulmões de fetos com hérnia diafragmática congênita isolada (HDC) medidos pela ultrassonografia tridimensional (US-3D) para predizer o resultado perinatal. Forma de estudo: Cinquenta e cinco fetos apresentando HDC foram avaliados pela US-3D entre janeiro de 2002 e maio de 2007. Os volumes pulmonares foram calculados pelo método rotacional na ultrassonografia tridimensional nos fetos com HDC (volumes observados) e comparados com aqueles descritos previamente em fetos normais (volumes esperados para determinada idade gestacional).

Objective: To evaluate the potencial of different lung mesurements using three-dimensional ultrasonography (3DUS) to predict perinatal outcome in cases of isolated congenital diaphragmatic hernia (CDH). Study design: Fifty-five fetuses presenting isolated CDH were prospectively evaluated by 3DUS between January 2002 and May 2007. Observed/expected total, contralateral and ipsilateral fetal lung volume ratios TFLV, CFLV and IFLV, respectively were calculated using the VOCAL™ technique and ultrasonographic fetal total lung volume to body weight ratio (US-FLW)...

Abordagem ventilatória protetora no tratamento da hérnia diafragmática congênita / Gentle ventilatory approach for the treatment of congenital diaphragmatic hernia

Objetivo: Descrever a evolução de recém-nascidos com diagnóstico de hérnia diafragmática congênita admitidos na Unidade de Terapia Intensiva Neonatal de um hospital privado de nível terciário, no qual aplicou-se uma estratégia ventilatória protetora. Métodos: Coorte histórica com análise de prontuários de pacientes portadores de hérnia diafragmática congênita, admitidos de junho de 2001 a julho de 2006. Avaliaram-se dados referentes ao recém-nascido (índices prognósticos antenatais, peso ao nascimento, idade gestacional, sexo), dados da reanimação e estabililização pré-operatória, cuidados pós-operatórios e taxa de sobrevida. Resultados: Oito neonatos tiveram diagnóstico de hérnia diafragmática congênita. O peso variou entre 2,38 e 3,45kg e a idade gestacional, entre 36 e 39 semanas; cinco deles eram do sexo masculino. Todos foram intubados em sala de parto até o final do primeiro minuto de vida. A correção cirúrgica ocorreu entre o segundo e o sexto dias de vida e, em quatro pacientes, houve necessidade do uso de patch. Uma estratégia ventilatória protetora foi utilizada em seis neonatos, com dados gasométricos visando PaO2 pré-ductal normal e tolerando-se hipercapnia (PaCO2 50 a 60mmHg). A extubação ocorreu entre o primeiro e o 12o dias do pós-operatório, com exceção de um paciente. Seis recém-nascidos receberam alta, em média, com 30 dias de vida (19 a 55 dias). A sobrevida foi de 75%. Conclusões: A sistematização do cuidado de pacientes com hérnia diafragmática congênita pode garantir, em nosso meio, uma sobrevida comparável aos principais centros mundiais que lidam com a doença.

AbstractObjective: To describe the clinical evolution of newborns with congenital diaphragmatic hernia admitted to neoretal Intensive Care Unit of a tertiary private hospital and treated with a gentle ventilatory approach. Methods: Analysis of charts of patients born between June 2001 and July 2006. The following data were collected: birth weight, gestational age, sex, delivery room procedures, pre and post-surgery parameters and survival rate. Results: Eight newborns with diagnosis of congenital diaphragmatic hernia were included. They presented birth weight from 2.38 to 3.45kg, gestational age between 36 and 39 weeks; five of them were males. All infants were intubated at delivery within the first minute of life. The surgery was performed between the 2nd and the 6th days of life, and a patch was needed in four patients. A ôgentleõ ventilation strategy was used in six infants, targeting normal pre-ductal PaO2 and allowing hypercapnia (PaCO2 between 50 and 60mmHg). The extubation occurred between the 1st and 12th day after surgery, except for one infant who died. Six newborns were discharged with an average post-natal age of 30 days (19 to 55 days). The survival rate was 75%. Conclusions: The systematic care of infants with diagnosis of congenital diaphragmatic hernia can assure a survival rate comparable to reference centers.

Perinatal management of common neonatal thoracic lesions.

Esophageal atresia, congenital diaphragmatic hernia, bronchopulmonary malformations and cystic lung diseases are the common neonatal thoracic surgical lesions encountered in practice. The availability of antenatal ultrasonography has lead to these lesions being detected before birth. Antenatal diagnosis can be made with a fair degree of accuracy in tertiary fetal medicine centres. Antenatal intervention is limited in a very few centres in the western world and not being done in India at present. The outcome of these babies with antenatal diagnosis of thoracic lesions has changed in the last decade. Earlier intervention is now possible in cystic lung disease before infectious complication has set in. All these lesions are managed exclusively in well developed neonatal surgery units with excellent outcome in the western world. The present study reviews the antenatal detection, clinical presentation, interventional/surgical procedures [antenatally and postnatally] and outcome of these common neonatal thoracic surgical lesions.

Hernia de Bochdalek: caso clínico / Bochdalek hernia: clinical case

La Hernia Diafragmática es la penetración de una o más visceras abdominales en el tórax, a trvés de un orificio normal o anormal del diafragma. Se han descrito 4 formas diferentes de hernias diafragmáticas congénitas: Hernia de Hiato, Hernia paraesofágica, Hernia de Morgagni-Larrey y Hernia de Bochdalek, así como también se han descrito Hernias diafragmáticas post-traumáticas. La Hernia de Bochdalek (posterolateral) es el resultado de un defecto diafragmático congénito en la parte costal posterior del diafragma, en la región de las costillas X y XI. Es una patología frecuente en el recién nacido y rara en el adulto. En la revisión de la literatura mundial encontramos 100 casos reportados en el adulto. Suele haber una comunicación libre entre las cavidades torácica y abdominal. Este defecto es más frecuente (90 por ciento) en el lado izquierdo, aunque puede producirse en el lado derecho, donde el hígado a menudo impide su diagnóstico. Es dos veces más frecuente en el varón. Presentamos el caso de una paciente femenina de 76 años, quién ingresó con dificultad para respirar y dolor torácico, desde hace (04) meses, se constata en el hemitórax izquierdo ruidos respiratorios abolidos con ruidos hidroaéreos presentes. Se realizan métodos diagnósticos a través de imágenes y se confirma evidente desplazamiento apical de estructuras retroperitoneales, riñón y asas intestinales hacia el hemitórax izquierdo. Se le realizó toracotomía izquierda, posterolateral, nefrectomía izquierda y afrontamiento de defecto diafragmático. Se realiza la revisión de dicha patología.

Cirugía fetal en la hernia diafragmática congénita / Fetal surgery in congenital diaphragmatic hernia

La hernia diafragmática congénita, se cree es resultado de la fusión incompleta de la membrana pleuroperitoneal y el paso del contenido abdominal al interior del tórax. Realizamos una revisión de la literatura sobre la etiología y del tratamiento de esta patología

Congenital diaphragmatic hernia is believed to result from incomplete pleuroperitoneal membrane fusion and passage of abdominal contents into the chest. We carry out a literature review regarding the etiology and treatment of this pathology

O valor da ultra-sonografia e da ressonância magnética fetal na avaliação das hérnias diafragmáticas / The value of fetal ultrasonography and magnetic resonance imaging in the assessment of diaphragmatic hernias

OBJETIVO: Demonstrar a significância dos achados da ressonância magnética e da ultra-sonografia na caracterização pré-natal e avaliação do prognóstico de pacientes com hérnia diafragmática congênita. MATERIAIS E MÉTODOS: Catorze gestantes (idade gestacional média de 28,7 semanas) examinadas com ultra-sonografia e apresentando fetos com suspeita de hérnia diafragmática congênita foram avaliadas por meio da ressonância magnética. Os exames foram realizados em aparelho de 1,5 tesla usando seqüências-padrão. Dois radiologistas avaliaram as imagens e estabeleceram os achados por consenso. RESULTADOS: Doze fetos tinham hérnia diafragmática à esquerda e dois, à direita. O fígado fetal foi localizado no interior do tórax de cinco fetos pela ultra-sonografia (três com hérnia diafragmática esquerda e dois com hérnia diafragmática direita) e de oito pela ressonância magnética (seis com hérnia diafragmática esquerda e dois com hérnia diafragmática direita). Herniação do estômago e alças de intestino delgado foi observada em todos os fetos com hérnia diafragmática esquerda (n = 12), tanto pela ultra-sonografia quanto pela ressonância magnética. Oito fetos sobreviveram após cirurgia (sete com hérnia diafragmática esquerda e um com hérnia diafragmática direita). CONCLUSÃO: A ultra-sonografia e a ressonância magnética são métodos de imagens complementares na avaliação das hérnias diafragmáticas congênitas. A ressonância magnética pode auxiliar a ultra-sonografia na avaliação da posição do fígado, o qual representa importante fator prognóstico.

OBJECTIVE: To demonstrate the relevance of ultrasonography and magnetic resonance imaging findings in the prenatal characterization and prognostic evaluation in cases of congenital diaphragmatic hernia. MATERIALS AND METHODS: Fourteen pregnant women (mean gestational age = 28.7 weeks) who had undergone ultrasonography for suspicion of fetuses with congenital diaphragmatic hernia were assessed by means of magnetic resonance imaging on a 1.5 tesla equipment, following the standard protocol. Two radiologists evaluated the images and the findings were defined by consensus. RESULTS: Twelve fetuses had left diaphragmatic hernia and two, right diaphragmatic hernia. Ultrasonography showed the fetal liver inside the thorax of five fetuses (three with left diaphragmatic hernia, and two with right diaphragmatic hernia) and magnetic resonance imaging in eight fetuses (six with left diaphragmatic hernia, and two with right diaphragmatic hernia). Stomach and small bowel loop herniation was observed in all of the fetuses with left diaphragmatic hernia (n = 12) at both magnetic resonance imaging and ultrasonography. Eight fetuses (seven with left diaphragmatic hernia and one with right diaphragmatic hernia) survived after surgical treatment. CONCLUSION: Ultrasonography and magnetic resonance imaging are complementary imaging methods in the evaluation of congenital diaphragmatic hernia. Magnetic resonance imaging is a helpful diagnostic method complementary to ultrasonography for evaluation of the fetal liver positioning, considering its relevance as a prognostic factor in cases of congenital diaphragmatic hernia.