[Insertion of intercostal drain into the liver in a right congenital diaphragmatic hernia]

Mortality of congenital diaphragmatic hernia [CDH] remains high despite the progress in resuscitation assessment. The diagnosis can be difficult especially in the right side form. We report a liver diaphragmatic hernia which been erratically diagnosed as a right pleuropneumopathy. An intercostal drain was inserted into the hepatic parenchyma. The diagnosis was secondarily rectified after thoracic tomodensitometry. The diagnosis of CDH must be systematically evoked in new-borns or infants presenting respiratory or gastrointestinal symptoms with chest X-ray abnormalities

Hernia diafragmática congénita: revisión de casos en el Hospital Guillermo Grant Benavente 2001-2007 / Congenital diaphragmatic hernia

The congenital diaphragmatic hernia is one of the most common severe malformations, with a high mortality that has been maintained for the time. There have been numerous markers that predict postnatal survival, of which the LHR and the presence of intrathoracic liver are the most reliable. Materials and methods: A review of the tabs of patients carrying fetuses with diagnosed CHD in the HGGB’s Department of Echography, as well as records of entry, exit and neonatal mortality in the same hospital. Results: We found 20 cases of CHD with an incidence of 1:1.647 births. Two patients were discarded by incomplete data. The 77,7 percent were diagnosed leftists. A 50 percent presenting deformities partners, among which one was diagnosed as Sd. Pallister Killian. The presence of intrathoracic liver was found in 38,8 percent. The mortality was 66,6 percent. Patients with LHR <1 were 100 percent mortality. Patients with liver Intrathoracic had 71,4 percent of mortality. Conclusion: The HDC is a malformation of high perinatal mortality. The indicators used as predictors of survival were very effectively. Our results are comparable to current publications.

La hernia diafragmática congénita constituye una de las malformaciones severas más comunes, con una alta mortalidad que se ha mantenido durante el tiempo. Se han formulado numerosos marcadores predictores de sobrevida postnatal, de los cuales el LHR y la presencia de hígado intratorácico son los más confiables. Material y método: Se realizó una revisión de las fichas de pacientes portadoras de fetos con hernia diafragmática congénita diagnosticadas en el Departamento de Ecografía del HGGB, además de registros de ingreso, egreso y mortalidad de neonatología del mismo hospital. Resultado: Se encontraron 20 casos de hernia diafragmática congénita con una incidencia de 1:1.647 partos. Se descartaron 2 pacientes por data incompleta. El 77,7 por ciento fueron diagnosticadas izquierdas. Un 50 por ciento presentó malformaciones asociadas, entre las cuales una fue diagnosticada como síndrome Pallister Killian. La presencia de hígado intratorácico se encontró en el 38,8 por ciento. La mortalidad fue de 66,6 por ciento. Las pacientes con LHR<1 tuvieron 100 por ciento mortalidad. Las pacientes con hígado intratorácico tuvieron 71,4 por ciento de mortalidad. Conclusión: La hernia diafragmática congénita constituye una malformación de alta mortalidad perinatal. Los indicadores utilizados como predictores de sobrevida tuvieron una alta efectividad. Nuestros resultados son comparables a las publicaciones actuales.

Prenatal diagnosis and perinatal outcome of 38 cases with congenital diaphragmatic hernia: 8-year experience of a tertiary Brazilian center

OBJETIVO: Avaliar os resultados neonatais dos casos de hérnia diafragmática congênita com diagnóstico pré-natal.PACIENTES E MÉTODOS: De janeiro de 1995 a dezembro de 2003 foram revisados os dados de 38 fetos com hérnia diafragmática diagnosticada durante o período pré-natal na Unidade de Medicina Fetal do Departamento de Obstetrícia e Ginecologia da Faculdade de Medicina da Universidade de São Paulo. Os dados pré-natais analisados foram: idade gestacional no diagnóstico, cariótipo fetal, lado da lesão, presença de malformação estrutural associada, herniação hepática e desvio garve de mediastino. Os dados perinatais foram avaliados consultando os prontuários médicos ou por contato telefônico. RESULTADOS: A idade gestacional média no diagnóstico foi de 29 semanas (16-37). Trinta (79%) casos apresentavam lesão à esquerda e 8 (21%) à direita. Malformações estruturais associadas foram observadas em 21 (55%) casos, dos quais 12 fetos apresentaram cariótipo normal, enquanto 9 exibiram anomalias cromossômicas. Hérnia diafragmática isolada foi identificada em 17 (457%) casos. A taxa de mortalidade geral foi de 92%. A taxa de óbito fetal, neomorto precoce, neomorto tardio e sobrevivente após 28 dias de vida foram respectivamente: (i) para o grupo com malformação estrutural associada e cariótipo normal, de 42%, 50%, 0% e 8%; (ii) para os casos com cromossomopatia, de 56%, 44%, 0% e 0%; (iii) para os casos com lesões isoladas de 0%, 76%, 12% e 12%. Na hérnia diafragmática congênita isolada, a mortalidade neonatal foi de 88%. CONCLUSÃO: Em nosso serviço, a mortalidade perinatal nos casos de hérnia diafragmática diagnosticada durante o pré-natal é muito alta. Óbitos neonatais precoces estão associados com presença de outros defeitos estruturais ou anomalias cromossômicas. Nos casos de HDC isolada, a mortalidade está relacionada à presença de fígado herniado, lado direito da lesão e desvio grave de mediastino.

Hernia diafragmática, diagnóstico prenatal y seguimiento / Diaphragmatic hernia, prenatal diagnosis and follow up

La hernia diafragmática congénita es un defecto del diafragma que permite el desplazamiento de los órganos abdominales hacia la cavidad torácica. A pesar de los avances en el manejo prenatal y postnatal, esta malformación se asocia a una elevada mortalidad. Se presenta una revisión de los casos clínicos con diagnóstico prenatal de hernia diafragmática ingresados al CERPO durante el período 2003-2005. Se analiza en cada caso la edad gestacional al diagnóstico, lado afectado, compromiso hepático, asociación con otras malformaciones y cromosomopatías, resultado perinatal, cirugía y evolución.

Hernia diafragmática congénita de Bochdalek en el adulto / Congenital Bochdalek diaphragmatic hernia in the adult

El interes del presente articulo es revisar el tema sobre hernia de Bochdaiek en el adulto, a proposito de la presentacion de un caso. Esta patologia es excepcional en el adulto; en la literatura mundial se han reportado muy pocos casos. Se elabora esta revision haciendo enfasis en la epidemiologia, el diagnostico y el tratamiento de esta entidad.

Congenital diaphragmatic hernia: mortality determinants in a Hispanic population

OBJECTIVES: Determine which factors were associated with mortality in our patients, specifically whether ventilatory parameters and arterial blood gas could be used to predict outcome. The role of delaying surgery and the presence of contra lateral pneumothorax were also assessed. BACKGROUND: Mortality among babies born with congenital diaphragmatic hernia remains high. The associated pulmonary hypoplasia and hypertension account for most of the overall mortality. There is no uniform consensus as to which parameters predict outcome. METHOD: Study population consisted of thirty-two patients with CDH managed during a ten-year period. Retrospective data obtained included: perinatal data, postnatal complications, ventilatory parameter data, arterial blood gas, type and age of surgery. Ventilatory index, oxygenation index and arterial to alveolar oxygen difference (A-aDO2) within the first 24 hours of life and after surgical correction were compared among the 23 patients who underwent surgical correction. Timing of surgery and frequency of pneumothorax were compared between survivors and non-survivors. Epi-Info Software Package was used for statistic analysis. RESULTS: Overall survival was 40 per cent. Survival of surgically corrected infants was 61 per cent. Non-survivors had significantly higher A-aDO2 than survivors (p < 0.05). No significant differences in pCO2, ventilatory index, or oxygenation index were identified between survivors and non survivors. Surgical repair performed after the first twenty-four hours of life, was associated with a higher survival rate (p < 0.05). Fourteen patients (39 per cent) developed contralateral pneumothorax, eleven (79 per cent) of these died. CONCLUSIONS: (1) contralateral pneumothorax was associated with higher mortality, 2) A-aDO2 was a better prognostic indicator than pCO2, ventilatory index, or oxygenation index, 3) delaying surgical repair was associated with better survival rate.