Cuantificación de la señal T2 pulmonar por resonancia magnética como factor pronóstico en las hernias diafragmáticas congénitas fetales / Prognostic usefulness of derived T2-weighted fetal magnetic resonance imaging measurements in congenital diaphragmatic hernia

Objetivo: Determinar el pronóstico neonatal de la hernia diafragmática congénita (HDC) partiendo de la señal relativa del pulmón contralateral en secuencias rápidas T2 de resonancia magnética (RM) fetal. Material y métodos: Estudiamos mediante ecografía y RM 28 fetos afectos de HDC y valoramos retrospectivamente en la ecografía fetal la relación entre el cociente observado y el esperado del diámetro axial máximo del pulmón dividido por el perímetro craneal (O/E LHR) y la posición hepática, y en la RM fetal el cociente de señal pulmón/hígado (LLSR) y el cociente de señal pulmón/líquido cefalorraquídeo (L/SF SR). Para determinar su valor pronóstico, los comparamos con los parámetros posnatales: supervivencia, hipertensión pulmonar, necesidad de oxígeno y la necesidad de oxigenación con membrana extracorpórea. Resultados: Encontramos diferencias significativas entre O/E LHR y la necesidad de oxigenación con membrana extracorpórea posnatal (p = 0,033) y la supervivencia posnatal (p = 0,01), y entre el LLSR de los fetos que sobrevivieron más de 45 días y los que no, con unas medianas de 1,91 y 2,56 respectivamente (p = 0,039). Conclusiones: El LLSR se correlaciona con la supervivencia posnatal en fetos con HDC y puede potencialmente utilizarse como parámetro pronóstico de la HDC fetal (AU)

Objective: To determine the usefulness of various parameters based on T2-weighted fetal magnetic resonance (MR) imaging measurements of the uninvolved lung for the neonatal prognosis of congenital diaphragmatic hernia (CDH). Material and methods: We used ultrasonography and MR imaging to study 28 fetuses with CDH. We retrospectively analyzed a) on fetal ultrasonography, the observed-to-expected lung to head ratio (O/E LHR) and the position of the liver, and b) on fetal MR imaging, the lung-liver signal ratio (LLSR) and the lungcerebrospinal fluid ratio (L/CSF SR). To determine the prognostic value of these parameters, we compared them with the following postnatal parameters: survival, pulmonary hypertension, need for oxygen supplementation, and need for extracorporeal membrane oxygenation. Results: We found significant differences between O/E LHR and the need for postnatal extracorporeal membrane oxygenation (P = .033) and postnatal survival (P = .01). We also found significant differences in LLSR between fetuses that survived more than 45 days and those that died within 45 days (1.91 vs. 2.56; P = .039). Conclusions: In fetuses with CDH, the LLSR correlates with postnatal survival and can potentially be used as a prognostic parameter in CDH (AU)

Valoración radiológica de la hernia diafragmática congénita fetal / Fetal magnetic resonance imaging evaluation of congenital diaphragmatic hernia

La hernia diafragmática se define como la entrada de vísceras abdominales en la cavidad torácica a través de un orificio normal o patológico del diafragma. La compresión que las vísceras herniadas ejercen sobre los pulmones produce hipoplasia pulmonar y, secundariamente, hipertensión pulmonar, que son las causas principales de muerte neonatal en los pacientes con hernia diafragmática congénita. En la práctica clínica la entidad se diagnostica mediante ecografía. La resonancia magnética fetal se utiliza básicamente para constatar la localización torácica o abdominal del hígado, aunque también puede aportar datos sobre el resto de estructuras herniadas y la hipoplasia pulmonar. El objetivo de este artículo es revisar los hallazgos por resonancia magnética fetal de la hernia diafragmática congénita y los signos radiológicos que nos permiten establecer el pronóstico neonatal valorando la hipoplasia pulmonar (AU)

A diaphragmatic hernia is defined as the protrusion of abdominal viscera into the thoracic cavity through a normal or pathological orifice. The herniated viscera compress the lungs, resulting in pulmonary hypoplasia and secondary pulmonary hypertension, which are the leading causes of neonatal death in patients with congenital diaphragmatic hernia. Congenital diaphragmatic hernia is diagnosed by sonography in routine prenatal screening. Although magnetic resonance imaging is fundamentally used to determine whether the liver is located within the abdomen or has herniated into the thorax, it also can provide useful information about other herniated structures and the degree of pulmonary hypoplasia. The aim of this article is to review the fetal magnetic resonance findings for congenital diaphragmatic hernia and the signs that enable us to establish the neonatal prognosis when evaluating pulmonary hypoplasia (AU)

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: criterios pronósticos y estado actual del tratamiento prenatal. Guía clínica / Congenital diaphragmatic hernia: prognosis and current status of fetal therapy. A practice guideline

La hernia diafragmática congénita es una malformación severa con mal pronóstico neonatal que frecuentemente se encuentra asociada a anomalías genéticas y malformaciones en otros sistemas. Una vez se ha diagnosticado, se ha de poner en marcha un protocolo de estudio con el objetivo primario de descartar anomalías asociadas y valorar factores pronósticos, esencialmente tamaño pulmonar y herniación hepática. Con ello se puede ofrecer un pronóstico de supervivencia relativamente preciso e individualizado a los padres. Las opciones de manejo disponibles en la actualidad incluyen la interrupción de la gestación, conducta expectante prenatal con tratamiento postnatal y terapia fetal. La terapia fetal se ofrece por parte del consorcio FETO (Barcelona, Londres, Lovaina) y otros centros en el mundo. Los resultados disponibles de varias series publicadas sugieren que la terapia fetal incrementa la supervivencia en un 35-40% respecto a la supervivencia de base. Existen diversos estudios aleatorizados multicéntricos para evaluar diferentes aspectos relevantes sobre la técnica y sus beneficios(AU)

Congenital diaphragmatic hernia (CDH) is a severe congenital birth defect, which is commonly associated with genetic abnormalities and malformations in other systems. Once the diagnosis is made, there should be a thorough evaluation to confirm that the malformation is isolated, as well as an assessment of prognostic factors, essentially lung size and liver herniation. With these parameters an individualized and relatively accurate prognosis can be offered to parents. Management options include termination of pregnancy, conservative prenatal management and post-natal treatment, or fetal therapy. Fetal therapy consists of fetal endoscopic tracheal occlusion (FETO) and is offered in a small number of centers worldwide. Available evidence suggests that prenatal treatment might increase the chances of survival by 35-40% compared with baseline survival. Several randomized trials are now underway to assess the impact of therapy on very severe and moderate cases of CDH(AU)

Hernia diafragmática congénita de presentación tardía / Late-presenting congenital diaphragmatic hernia

No disponible

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.

Utilidad de la ECMO en pacientes con hernia diafragmática congénita / Utility of the ecmo in patients with congenital diaphragmatic hernia

Actualmente, la membrana de oxigenación extracorpórea (ECMO)constituye el último eslabón en la terapéutica del manejo del fallo respiratorio en pacientes con hernia diafragmática congénita (HDC). Presentamos nuestra experiencia. Desde enero de 2001 disponemos en UCI- neonatal de ECMO. De76 HDC, 13 (3 derechas y 10 izquierdas) han precisado ECMO (uno en dos ocasiones; en total 14 procedimientos). Criterios de inclusión: hipoxemia refractaria, índice de oxigenación > 40 y peso > de 2 kg. 5 niñas y 8 niños con edad gestacional entre 35 y 41 semanas (media:38) y peso al nacer entre 2.300 y 3.500 gramos (media 2.817). En 6casos (5 trasladados desde otros centros), el diagnóstico fue posnatal. De los 7 con diagnóstico prenatal, en 4 casos se había realizado terapiafetal mediante oclusión traqueal. Procedimiento veno-venoso en 8 y veno-arterial en 5. Rango de duración: 68-606 horas, media de 228,35. La cirugía se ha realizado antes de la ECMO en 9 casos, 2 durante y 1 después. En una ocasión (..) (AU)

At the moment the extracorporeal membrane oxygenation (ECMO)constitutes the last link in the therapeutic one of the handling of the respiratory failure in patients with Congenital Diaphragmatic Hernia (HDC).We presented our experience. From January 2001 we arrange the ECMO in neonative UCI. 76HDC, 13 (3 rights and 10 lefts) they have needed ECMO (one in two occasions; altogether 14 procedures). Criteria of inclusion: refractory hypoxaemia, oxigenaction index > 40 and weight > 2 kg. 5 girls and 8 boys with gestacional age between 35 and 41 weeks(average: 38) and weight when being born between 2,300 and 3,500grams (average 2,817). In 6 cases (5 transferred from other centers) the diagnosis was posnatal. Of the 7 with prenatal diagnosis, in 4 cases fetal therapy by means of traqueal occlusion had been made. Veno-venous in 8 and veno-arterial procedure in 5. Rank of duration: 68-606 hours, average of 228.35. The surgery has been made before the ECMO in 9 cases, 2 during and 1 later. In an occasion there was no (..) (AU)

Resultados a largo plazo de pacientes con herniadiafragmática congénita / Long-term results of patients with congenital diaphragmatic hernia

Introducción. La introducción de avanzadas modalidades terapéuticas ha permitido alcanzar mejorías considerables en la supervivencia de la hernia diafragmática congénita (HDC). Sin embargo, existen pocos estudios que analicen la evolución clínica de los supervivientes a largo plazo. El objetivo de este trabajo es analizar la evolución a largo plazo de una serie de pacientes diagnosticados de HDC en nuestro hospital. Material y métodos. Entre 1998 y 2005 fueron tratados en nuestro centro 55 neonatos afectos de HDC. La supervivencia al alta hospitalaria fue del 72% (40 pacientes), siendo estos los incluidos en el estudio. En 6de ellos se requirió ECMO durante el tratamiento neonatal. Realizamos un estudio descriptivo transversal mediante revisión exhaustiva del historial clínico y encuesta telefónica a los padres, analizando las alteraciones respiratorias, cardiológicas, digestivas y el estado neurológico actual, utilizando para ello los métodos diagnósticos estándar en cada caso. La edad media de los niños en el momento del estudio es 4,2 años (1-9). Resultados. El 8,3% de los niños requirió oxigenoterapia domiciliaria, retirándose a los 3 meses en todos los casos. Ingresaron por problemas respiratorios el 22%, siendo la bronquiolitis y neumonía los diagnósticos más frecuentes. Solo un paciente padece asma de esfuerzo. El reflujo gastroesofágico es la patología más frecuente a largo plazo (47%), pero solo requiere tratamiento quirúrgico el 8,3%.En el ámbito cardiológico, el 14% padece hipertensión pulmonar, siendo leve-moderada en todos los casos excepto 1 niño que falleció (..) (AU)

Objective. Introduction of advanced therapeutic modalities fordiaphragmatic congenital hernia (CDH) has allowed to reach considerable improvements in survival rate. Nevertheless, there are few studies which analyze the clinical evolution of the long-term survivors. The aim of this work is to analyze the outcomes of the patients with CDH in our hospital. Methods. Fifty-five neonates with CDH were treated in our center between 1998 and 2005. We included in the study those patients that were alive at the moment of first hospital discharge (72%; n = 40patients). ECMO therapy was needed in 6 of them during neonatal treatment. A descriptive transverse review of the clinical record as well as a telephonic interview to the parents was performed for the respiratory, cardiological, digestive and neurological conditions, following standard diagnostic studies in every case. The mean age of the children in the moment of the study was 4.2 years (1-9).Results. The 8.3% of the children needed domiciliary oxygen therapy during a maximum of 3 months in all the cases. 22% of the cases suffered from respiratory problems, being bronchiolitis and pneumonia the most frequent diagnoses. Only a patient developed asthma. The gastroesophageal reflux is the most frequent long-term condition (47%),but only 8.3% needs surgical treatment. Regarding to cardiological problems, 14% developed pulmonary hypertension, being slight - moderate (..) (AU)

Hernia diafragmática paraesternal de Morgagni-Larrey en adulto / Morgagni-Larrey parasternal diaphragmatic hernia in the adult

Con una prevalencia de 0,3-0,5/1.000 nacimientos, la hernia diafragmática congénita (HDC) sigue siendo una anomalía grave, no bien entendida, alta mortalidad y tratamiento no siempre efectivo. En España se ha informado de una frecuencia del 0,69%oo con una tendencia decreciente en el periodo 1980-2006 del 0,10%oo por año. No obstante, hasta un 5% se diagnostican en adultos durante la realización de un reconocimiento por otra causa. Presentamos un cuadro de vómitos de tres meses de evolución en una mujer de 74 años por hernia diafragmática paraesternal de Morgagni-Larrey (retrocondroesternal, retrocostoxifoidea, retroesternal, subcostal, subesternal o subcostoesternal), que nos ha permitido realizar una actualización de esta patología en adultos y de la morfogénesis del diafragma toracoabdominal. Es en la embriología del diafragma donde encontramos explicación de algunas de sus alteraciones morfológicas y características clínicas, si bien persisten aspectos confusos de la misma. También analizamos el grado de controversia que persiste en algunos aspectos de su tratamiento quirúrgico (vías de acceso, uso o no de mallas y reducción o no del saco herniario). Por lo general priman las técnicas mínimamente invasivas. Consideramos el abordaje laparoscópico como de elección en pacientes adultos con hernia paraesternal candidatos a la cirugía(AU)

With a prevalence of 0.3-0.5/1000 births, congenital diaphragmatic hernia (CDH) remains a serious, poorly understood abnormality with a high mortality rate that cannot always be effectively managed. Its reported frequency in Spain is 0.69%oo with a yearly decreasing trend of 0.10%oo during the period 1980-2006. Up to 5% of cases are incidentally identified in adults undergoing studies for other reasons. We report the case of a 74-year-old woman with vomiting for three months due to parasternal diaphragmatic hernia of Morgagni-Larrey (retrochondrosternal, retrocostoxyphoid, retrosternal, subcostal, substernal or subcostosternal hernia), which allowed us to report an update on this condition in the adult, and on thoracoabdominal diaphragm morphogenesis. It is in the embryology of the diaphragm where an explanation may be found for some morphological changes and clinical manifestations, even though a number of uncertainties remain. We also analyze the extent of controversy persisting on some aspects of surgical treatment (access routes, mesh use, hernial sac reduction). Overall, minimally invasive techniques predominate. We consider laparoscopy the approach of choice for adult patients with parasternal hernia eligible for surgery(AU)

Evolución del diagnóstico prenatal de los defectos congénitos estructurales en Asturias. Período 1990-2004 / Prenatal diagnosis trend for structural birth defects in Asturias (Spain). Period 1990-2004

Fundamento. Evaluar el impacto del diagnóstico prenatal(DP) en los defectos congénitos estructurales (DCE).Métodos. Durante 15 años (1990-2004) el Registro deDefectos Congénitos de Asturias (RDCA) recogió informaciónsobre defectos congénitos (DC). El RDCA es un registrode base poblacional que incluye todos los diagnósticos yasean abortos, mortinatos, o nacidos vivos hasta los cincoaños de edad. Se analizaron datos obtenidos de los defectosde cierre del tubo neural (DTN), hernia diafragmática (HD),onfalocele, gastrosquisis, y cardiopatías congénitas, y la evolucióndel DP de estos defectos.Resultados. Se diagnosticaron un total de 3.035 DC(2,9 % sobre el total de nacimientos), siendo 560 los diagnósticosprenatales (18,5 %). Se registraron 126 casos deDTN con un porcentaje de DP igual o superior al 90 % en losperíodos estudiados, principalmente a expensas del diagnósticoecográfico. Hubo 45 casos de HD, con un incrementosustancial en el DP (68 % en los últimos años). En elcaso del onfalocele y la gastrosquisis, también se produjouna mejoría en la frecuencia del DP, especialmente en elcaso de las gastrosquisis, siendo diagnosticadas el 90%prenatalmente. El 10,6 % de las cardiopatías congénitas sediagnosticaron antes de nacer, y analizando las ocho másseveras y estructuralmente más complejas el DP apenas llegaal 20 % (AU)

Objective. To asses the impact of prenatal diagnosis(PD) on structural birth defects.Methods. Over 15 years (1990-2004) the Registro deDefectos Congénitos de Asturias (RDCA) collected informationon birth defects (BD). The RDCA is a populationbasedregistry that includes all diagnosed cases whetherabortions, stillbirths, or live birth until the age of five.Data obtained were analyzed for the defects of the neuraltube closure (DNT), diaphragmatic hernia (DH), omphalocele,gastroschisis, and congenital heart disease, and theevolution of PD in these defects.Results. 3,035 BD were diagnosed (2.9% of totalbirths), of which 560 were prenatally (18.5%). They wererecorded 126 cases of DNT with an average of PD equalto or exceeds 90% in the periods studied, mainly due toultrasound diagnosis. There were 45 cases of DH, with asubstantial increase in the PD (68% the last years). Omphaloceleand gastroschisis, also show an increase in thefrequency of PD, especially in gastroschisis, being diagnosedthe 90% of them prenatally. 10.6% of congenitalheart diseases were diagnosed prenatally, and the eightvalues harsher and more structurally complex PD barelyreaches 20% (AU)

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)...