Riñon torácico y hernia diafragmática: diagnóstico prenatal y manejo exitoso. Reporte de caso y revisión de la literatura / Thoracic kidney and diaphragmatic hernia: prenatal diagnosis and successful management. Case report and review of the literature

Objetivos: Reportar un caso de diagnóstico prenatal de riñón ectópico intratorácico (REI) con hernia diafragmática y manejo quirúrgico neonatal, y hacer una revisión de la literatura sobre diagnóstico prenatal de REI y el pronóstico perinatal. Materiales y métodos: Se reporta el caso de un feto de 28 semanas en el que se observó imagen ecográfica sugestiva de masa en hemitórax derecho que desplazaba corazón y pulmón; se confirmó que correspondía a un riñón intratorácico. Por laparoscopia, al recién nacido se le colocó una malla en continuidad con el diafragma dejando el riñón en el abdomen, con buena evolución. Se realizó una búsqueda bibliográfica en PubMed, Embase y Cochrane. Se buscaron cohortes, reportes y series de caso de gestaciones con diagnóstico prenatal de riñón intratorácico fetal. Se extrajo información del diseño, la población, el diagnóstico por imágenes, el tratamiento y el pronóstico. Resultados: En la búsqueda se identificaron 8 estudios que cumplieron con los criterios de inclusión y que informan en total ocho casos. El diagnóstico ecográfico mostró REI asociado a hernia diafragmática en todos los sujetos. Se utilizó también la RM fetal en cinco casos. Seis neonatos sobrevivieron sin complicaciones, en uno hubo interrupción voluntaria del embarazo, y otro presentó sepsis y dificultad respiratoria, finalmente fue dado de alta en buenas condiciones. Conclusiones: El REI es una anomalía congénita susceptible de diagnóstico prenatal. La sobrevida a la cirugía correctora en el periodo neonatal es frecuente. La literatura disponible en torno al diagnóstico prenatal de REI es escasa y se limita a reportes de casos.

Objectives: To report a case of prenatal diagnosis of ectopic intrathoracic kidney with diaphragmatic hernia managed surgically after birth, and to conduct a review of the literature on prenatal diagnosis of ectopic intrathoracic kidney and perinatal prognosis. Material and methods: We report the case of a 28-week fetus in which, on ultrasound imaging, a mass was observed displacing the heart and lung in the right hemithorax, which was was confirmed by magnetic resonance (MR) to be an ectopic intrathoracic kidney. After birth, the neonate was approached by laparoscopy to place a mesh in continuity with the diaphragm, leaving the kidney in the abdomen, with good evolution. A search was conducted in the PubMed, Embase and Cochrane databases for cohorts, case reports and case series of prenatal diagnosis of intrathoracic kidney in the fetus. Information was retrieved regarding design, population, imaging diagnosis, treatment and prognosis. Results: The search identified 8 studies that met the inclusion criteria, reporting a total of 8 cases. Ultrasound diagnosis showed ectopic intrathoracic kidney associated with diaphragmatic hernia in all the subjects. Fetal magnetic resonance imaging (MRI) was also used in 5 cases. Conclusions: Ectopic intrathoracic kidney is a congenital abnormality amenable to prenatal diagnosis. Survival after corrective surgery performed in the neonatal period is common. There is a paucity of publications, limited to case reports, regarding the prenatal diagnosis of this condition.

Hernia diafragmática congénita de presentación tardía Reporte de caso con revisión de literatura / Late Presentation Congenital Diaphragmatic Hernia: Case Report with Literature Review

Resumen Una hernia diafragmática congénita ocurre cuando existe un defecto estructural en el diafragma que permite la migración de los órganos abdominales a la cavidad torácica. Se considera de presentación tardía cuando se diagnostica después de los 30 días de vida extrauterina. Más del 60% de los pacientes con hernia diafragmática congénita cuentan con un diagnóstico erróneo al momento del nacimiento, encontrándose dentro de los diagnósticos más frecuentes al derrame pleural, neumonía, neumotórax, neumatocele y absceso pulmonar. Presentamos el caso de una paciente del sexo femenino de 3 años que acudió a urgencias por dolor abdominal, náuseas, vómito, intolerancia a la vía oral y dificultad respiratoria. La radiografía de tórax evidenció migración de la cámara gástrica hacia el tórax, dessplazamiento de la silueta cardiaca y las estructuras del mediastino hacia la derecha con la punta de la sonda nasogástrica ubicada en el hemitórax izquierdo. Se concluyó el diagnóstico de hernia diafragmática de presentación tardía. La paciente recibió tratamiento quirúrgico, el cual fue exitoso. Este trabajo destaca la importancia de sospechar el diagnóstico de hernia diafragmática congénita de presentación tardía cuando se abordan pacientes pediátricos con dificultad respiratoria sin otra causa aparente, dolor abdominal, náuseas y vómito.

Abstract A congenital diaphragmatic hernia occurs when the diaphragm has a structural defect that allows the migration of abdominal organs into the chest cavity. It is called late presentation when its diagnosis does after 30 days of life. More than 60% of patients with congenital diaphragmatic hernia are misdiagnosed. The most common misdiagnoses are pleural effusion, pneumonia, pneumothorax, pneuma tocele, and lung abscess. We present a case of a 3-year-old female who attended the emergency room due to abdominal pain, nausea, vomiting, intolerance to the oral route, and respiratory distress. The chest X-ray showed migration of the gastric chamber towards the thorax, displacement of the cardiac silhouette and the mediastinal structures to the right, and the tip of the nasogastric tube located in the left hemithorax. The doctors concluded a late presentation diaphragmatic hernia. The patient received surgical treatment, which was successful. This paper highlights the importance of suspecting the diagnosis of late-onset congenital diaphragmatic hernia when treating pediatric patients with respiratory distress without another apparent cause, abdominal pain, nausea, and vomiting.

The myocardial capillary network is altered in congenital diaphragmatic hernia in the fetal rabbit model

Congenital diaphragmatic hernia (CDH) is associated with thoracic compression of the lungs and heart caused by the herniated abdominal content, leading to cardiac modifications including pressure and vascular changes. Our aim was to investigate the experimental immunoexpression of the capillary proliferation, activation, and density of Ki-67, VEGFR2, and lectin in the myocardium after surgical creation of a diaphragmatic defect. Pregnant New Zealand rabbits were operated on the 25th gestational day in order to create left-sided CDH (LCDH, n=9), right-sided CDH (RCDH, n=9), and Control (n=9), for a total of 27 fetuses in 19 pregnant rabbits. Five days after the procedure, animals were sacrificed, and histology and immunohistochemistry studies of the harvested hearts were performed. Total body weight and heart weight were not significantly different among groups (P=0.702 and 0.165, respectively). VEGFR2 expression was increased in both ventricles in the RCDH group (P<0.0001), and Ki-67 immunoexpression was increased in the left ventricle in the LCDH group compared to Control and RCDH groups (P<0.0001). In contrast, capillary density was reduced in the left ventricle in the LCDH compared to the Control and RCDH groups (P=0.002). Left and right ventricles responded differently to CDH in this model depending on the laterality of the diaphragmatic defect. This surgical model of diaphragmatic hernia was associated with different expression patterns of capillary proliferation, activation, and density in the myocardium of the ventricles of newborn rabbits.

Post-natal prognostic factors in CDH: experience of 11 years in a referral center in Brazil

Abstract Objective To describe post-natal risk factors associated with death in Newborns (NB) with Congenital Diaphragmatic Hernia (CDH) in a Brazilian reference center. Methods In this retrospective cohort study, post-natal clinical factors of all NB diagnosed with CDH were reviewed in an 11-year period (2007‒2018). The primary outcome was death. Secondary outcomes included clinical features, prognostic indexes, type of mechanical ventilation, complications during hospitalization and surgical repair. Results After applying the exclusion criteria, the authors analyzed 137 charts. Overall mortality was 59% (81/137), and the highest rates were observed for low-birth-weight NB (87%), syndromic phenotype (92%), and those with major malformations (100%). Prognostic indexes such as Apgar, SNAPPE-II and 24hOI (best oxygenation index in 24 hours) were all associated with poor evolution. In a multivariate analysis, only birth weight and 24hOI were statistically significant risk factors for mortality, with a reduction in mortality risk of 17.1% (OR = 0.829, 95% IC 0.72‒0.955, p = 0.009) for each additional 100g at birth and an increase by 26.5% (OR = 1.265, 95% IC 1.113‒1.436, p = 0.0003) for each unitary increase at the 24hOI. Conclusion Prognostic indexes are an important tool for predicting outcomes and improving resource allocation. Post-natal risk factors may be more suitable for settings where antenatal diagnosis is not universal. Classical risk factors, such as prematurity, low birth weight, higher need for supportive care, and poorer prognostic indexes were associated with mortality in our CDH population.

Diagnosis and treatment of adult diaphragmatic hernia / 中华消化外科杂志

The etiology of diaphragmatic hernia in adults is mainly congenital and traumatic, its overall incidence in adults is low, and adult diaphragmatic hernia is easily misdiagnosed and missed diagnosis because of lacking specificity in clinical presentation. There is no clinical guidelines or consensus for the diagnosis and treatment of diaphragmatic hernia in adults. The authors inquire into the diagnosis and treatment of diaphragmatic hernia in adults by summarizing relevant litera-tures and combining with clinical practice, and recommend that multi-slice spiral computed tomo-graphy should be promptly refined for suspected cases, especially focusing on sagittal images. The symptomatic patients should be repaired promptly, with a preference for laparoscopic surgery, and mesh augmentation is recommended in those with larger defects.

Hernia diafragmática congénita posterolateral derecha sin herniación hepática: presentación de caso con tratamiento exitoso / Right posterolateral congenital diaphragmatic hernia without liver herniation: presentation of a case with successful treatment

Resumen La hernia diafragmática congénita es un defecto en la formación del diafragma con una alta mortalidad y morbilidad para el recién nacido. La ubicación en el lado derecho corresponde a solo el 10% de todos los casos y se asocia con una menor supervivencia, especialmente cuando hay una herniación hepática. No se conoce con precisión el porcentaje de casos de hernia diafragmática congénita derecha que no implican una herniación hepática. Aun no hay certeza del tiempo estimado para obtener el mayor beneficio de la corrección quirúrgica, en nuestro caso realizado con éxito a los 2 días de vida, logrando previamente una adecuada estabilidad hemodinámica y ventilatoria. El manejo perioperatorio en unidad de cuidado intensivo neonatal, es fundamental para reducir la morbimortalidad asociada. Se presenta un caso de hernia diafragmática congénita derecha tratado con éxito y se exponen los aspectos más relevantes del manejo médico-quirúrgico de esta patología. MÉD.UIS.2021;34(3): 71-7.

Abstract Congenital diaphragmatic hernia is a congenital defect in the formation of the diaphragm with high mortality and morbidity for the newborn. The location on the right side corresponds to only 10% of all cases and is associated with a lower survival, especially when there is a liver herniation. The percentage of cases of right congenital diaphragmatic hernia that do not involve liver herniation is not known with precision. An estimated time to obtain the greatest benefit from surgical correction is not known with certainty, in our case it was carried out successfully at 2 days of life, previously achieving adequate hemodynamic and ventilatory stability. Perioperative management in the neonatal intensive care unit is essential to reduce the associated morbidity and mortality. A case of congenital right diaphragmatic hernia treated successfully is presented and the most relevant aspects of the medical-surgical management of this pathology are exposed. MÉD. UIS.2021;34(3): 71-7.

Hernia diafragmática: Un desafío diagnóstico / Diaphragmatic Hernia: A Diagnostic Challenge

Introducción: La hernia diafragmática traumática es una complicación grave del traumatismo abdominal o torácico. Se considera una entidad poco frecuente. El diagnóstico es difícil y, a menudo, puede pasar inadvertido. Objetivo: El propósito de este caso clínico es demostrar la necesidad de sospechar la presencia de hernia diafragmática en los pacientes con trauma toracoabdomial. Presentación del Caso: Paciente de 75 años que sufre accidente de tránsito, con evaluación inicial sin alteraciones clínico-radiológicas, que posteriormente presentan evolución desfavorable con hallazgo radiográfico de una hernia diafragmática traumática. Conclusiones: La hernia diafragmática cuando se presenta constituye un verdadero reto para el diagnóstico por parte del personal médico; pues esta requiere de un alto índice de sospecha y el uso adecuado de las imágenes diagnósticas. Se considera una entidad importante para la evaluación del paciente traumatizado(AU)

Introduction: Traumatic diaphragmatic hernia is a serious complication of abdominal or thoracic trauma. It is considered a rare pathology. The diagnosis is difficult and can often go unnoticed. Objective: The purpose of this clinical case is to demonstrate the need to suspect the presence of diaphragmatic hernia in patients with thoracoabdomial trauma. Case Presentation: Seventy-five-year-old patient who suffers a traffic accident. At initial evaluation no clinical-radiological alterations were observed, but later the patient presented an unfavorable evolution with radiographic finding of a traumatic diaphragmatic hernia. Conclusions: Diaphragmatic hernia, when present, is a real challenge for the diagnosis by the medical staff since it requires a high index of suspicion and an adequate use of diagnostic images. It is considered an important entity in the evaluation of the trauma patient(AU)

Benefit of video-assisted thoracic surgery in the emergency of a complicated postpartum diaphragmatic hernia / Beneficio de la cirugía torácica video-asistida en la emergencia de una hernia diafragmática posparto complicada

Abstract Diaphragmatic hernia during pregnancy or puerperium are rare entities that, in the event of a complication, represent a life-threatening emergency for both mother and the unborn child. Resolution in many cases requires a combined access through the thoracic and abdominal cavity to manage the complica tions of necrosis and/or perforation of an abdominal viscera. To treat the thoracic cavity, thoracotomy is the most described approach. Even more, the minimally invasive access by video-assisted thoracic surgery (VATS) is not widely referenced in the literature despite its clear benefits of this technique in the recovery of patients, even in emergencies. We present the case of a postpartum right diaphragmatic hernia, complicated by necrosis of the colonic wall, treated by combined VATS approach and laparotomy in the emergency.

Resumen Las hernias diafragmáticas durante el embarazo o puerperio son entidades raras que, en caso de complicación, representan una emergencia potencialmente mortal tanto para la madre como para el feto. La resolución en muchos casos requiere un acceso combinado a través de la cavidad torácica y abdominal para manejar las complicaciones de la necrosis o perforación de las vísceras abdominales. Para tratar la cavidad torácica, la toracotomía es la vía más descrita. Más aún, el acceso mínimamente invasivo mediante cirugía torácica video-asistida (VATS) no está ampliamente referenciado en la literatura a pesar de los claros beneficios de esta técnica en la recuperación de los pacientes, incluso en emergencias. Presentamos el caso de una hernia diafragmática derecha posparto, complicada por necrosis de la pared colónica, tratada combinado VATS y laparotomía en la urgencia.

Abdomen agudo obstructivo por hernia diafragmática crónica postraumática / Obstructive acute abdomen due to postraumatic chronic diaphragmatic hernia

Se describe el caso clínico de un paciente de 37 años de edad, que acudió al Servicio de Emergencia del Hospital General Docente de Riobamba, en Ecuador, con manifestaciones clínicas e imagenológicas que permitieron diagnosticarle un abdomen agudo obstructivo por hernia diafragmática crónica postraumática, por lo que el tratamiento fue quirúrgico. Teniendo en cuenta la evolución satisfactoria del paciente se le dio el alta hospitalaria 10 días después de la operación. Se mantuvo con seguimiento durante 3 meses sin presentar complicaciones.

The case report of a 37 years patient that went to the Emergency Service of the Teaching General Hospital of Riobamba, in Ecuador, is described. He presented clinical and imaging signs that led to the diagnosis of an obstructive acute abdomen due to postraumatic chronic diaphragmatic hernia, reason why the treatment was surgical. Taking into account the patient's favorable clinical course he was discharged from the hospital 10 days after the surgery and received follow-up care during 3 months without presenting complications.

A rare case of congenital diaphragmatic hernia in adult: an incidental finding during an elective procedure

Congenital diaphragmatic hernia consists of a defect of the diaphragm that allows the passage of abdominal viscera into the thorax. Congenital diaphragmatic hernia most commonly present in infancy and childhood due to respiratory distress. However, a few of them may escape into adulthood. A very few of these adult cases may be identified during a routine medical checkup for various other reasons without any cardio-respiratory problems. In our case report, we have diagnosed incidentally an asymptomatic left diaphragmatic hernia with cardio-mediastinal shifting in a 38 years old female admitted for left breast fibroadenoma excision during a routine pre-anesthetic check-up and underwent breast surgery without any cardio-respiratory problems.

A rare clinical entity of asymptomatic adult bochdalek hernia

Bochdalek hernia (BH) is the commonest congenital diaphragmatic hernia, caused by the failure of the posterolateral diaphragmatic foramina to fuse properly. It is extremely rare in adults and accounts for 5-10%. Presenting a case of 48 years female with complaints of dry cough and left chest pain for 1 week. Diminished breath sounds and abnormal gurgling sounds heard on auscultation of left chest wall. X-ray chest showed elevated left hemi diaphragm and gastric bubble. Computed tomography (CT) chest revealed left diaphragmatic hernia with splenic flexure, transverse colon, mesocolon, spleen and upper pole of left kidney as content and atelectasis of left lung lower lobe. Patient underwent laparoscopic repair of hernia with mesh plasty. Intraoperatively, the contents were reduced into the abdominal cavity and left lung expansion noted. The defect of size 6×10 cm in the left diaphragm was sutured and composite mesh placed. Post-operative chest x-ray showed expanded left lung. On follow up of patient after 2 weeks and 1 month, patient was asymptomatic. BH in adults is an uncommon. The contents can be reduced via thoracic or abdominal approach, with abdominal approach having easier access. With the advent of minimal access techniques, delineating clear anatomy, more working space, early recovery, and early return to home and work is possible. Thus, laparoscopic repair of adult diaphragmatic hernia is a safe and effective modality of surgical treatment.

Hernia de Bochdalek en el embarazo. Reporte de un caso y revisión de la literatura / Bochdalek hernia in pregnancy. Case report and literature review / Hérnia de Bochdalek na gestação. Relato de caso e revisão da literatura

Resumen: Se expone el caso de una paciente de 28 años de edad quien presenta caso de hernia de Bochdalek, mostró cuadro de choque séptico por oclusión intestinal y perforación, la cual ameritó manejo integral por cirugía general, ginecología y terapia intensiva, siendo exitoso su manejo, por lo que se reporta el caso.

Abstract: We present a case of a 28-year-old female patient, who presents a case of Bochdalek hernia, which presented a septic shock due to intestinal occlusion and perforation, which merits comprehensive management due to general surgery, gynecology and intensive care, being your therapy was successful, so the case is reported.

Resumo: Apresentamos o caso de uma paciente de 28 anos, do sexo feminino, que apresentou um caso de hérnia de Bochdalek, com um quadro de choque séptico por oclusão e perfuração intestinal, que mereceu tratamento abrangente por cirurgia geral, ginecologia e terapia intensiva, sendo tratamento bem-sucedido, para que o caso seja relatado.

Comparación de recidiva posterior a hernioplastía diafragmática con cierre directo versus colocación de parche / Comparison of recurrence after diaphragmatic hernioplasties with direct closure versus patch placement

Resumen Objetivo: Investigar con cuál técnica quirúrgica, cierre directo o con parche de Goretex, las hernias diafragmáticas congénitas operadas tienen más recidiva. Metodología: Los datos de los pacientes sometidos a reparación por hernia diafragmática congénita en el Hospital Nacional de Niños, entre enero 2008 y el 31 diciembre de 2017, fueron revisados retrospectivamente. De los 94 pacientes, solo 59 cumplen con los criterios de inclusión. Para la comparación de las variables cuantitativas se empleó pruebas de T de Student e intervalos de confianza al 95 %; las variables cualitativas se analizaron por la prueba de Fisher con un nivel de significancia de 0,05. Resultados: La mayoría de las hernias diafragmáticas operadas fueron izquierdas (78 %) y posterolaterales (91 %). La técnica de reparación más frecuente utilizada fue el cierre directo (68%). Hubo más recidivas posteriores a cierre con parche de Goretex. Se presentaron entre 1 y 12 meses postoperatorio. No hubo diferencia estadísticamente significativa entre recidivas con técnica de cierre directo versus cierre con parche de Goretex. Conclusión: Realizar un cierre directo del diafragma es una buena opción quirúrgica para reparación de hernia diafragmática congénita. No hubo en este estudio diferencias estadísticamente significativas en cuanto a recidiva entre utilizar técnica con cierre directo o con parche.

Abstract Objective: To investigate with which surgical technique, direct closure or use of a Goretex patch, congenital diaphragmatic hernias recurre more. Methodology: The data of the patients who underwent repair of congenital diaphragmatic hernia at the National Children's Hospital from January 2008 to December 31, 2017, were reviewed retrospectively. Of the 94 patients, only 59 met the inclusion criteria. For the comparison of the quantitative variables, Student's t-test and 95% confidence intervals were used, the qualitative variables were analyzed by the Fisher's test with a significance level of 0.05. Results: Most of the operated diaphragmatic hernias were left 78% and posterolateral 91%. The most frequent repair technique used was direct closure, 68%. There were more recurrences after closing when a Goretex patch was used. They occurred between 1 and 12 months post-operatively. There was no statistically significant difference between recurrences with the direct closure technique versus closure with the Goretexpatch. Conclusion: Performing a direct closure of the diaphragm is a good surgical option for surgical repair of congenital diaphragmatic hernias. There were no statistically significant differences in relapse in this study between using the direct closure or patch technique.

Morgagni-Larrey parasternal diaphragmatic hernia masquerading as pleural effusion: an interesting presentation with review of literature

Congenital diaphragmatic hernia (CDH) is a major malformation found in newborns and babies but rarely in adults. CDH is defined by the presence of a defect in the diaphragm, which permits the herniation of abdominal contents into the thorax. The diagnosis of CDH is based on clinical history, examinations and is confirmed by plain X-ray films and computed tomography scans. Here we report a case which was misdiagnosed at another centre as pleural effusion and managed by placement of intercostal tube drainage and later on diagnosed to be case of Morgnani-Larrey parasternal diaphragmatic hernia. Congenital diaphragmatic hernia (CDH) is a major malformation found in newborns and babies but rarely in adults. CDH is defined by the presence of a defect in the diaphragm, which permits the herniation of abdominal contents into the thorax. The diagnosis of CDH is based on clinical history, examinations and is confirmed by plain X-ray films and computed tomography scans. The purpose of reporting this case is to provide information on late-presenting CDH and to emphasize that a high index of suspicion is required for diagnosis and to avoid unwanted complications.

Hernia diafragmática congénita: predictores posnatales de mortalidad / Congenital diaphragmatic hernia: postnatal predictors of mortality

La hernia diafragmática congénita (HDC) es una enfermedad de baja prevalencia, con elevada morbimortalidad. Los factores pronósticos posnatales, durante el primer día de vida, son útiles para la toma de decisiones.Objetivos. Determinar la capacidad predictiva de los factores posnatales ecocardiográficos, clínicos y bioquímicos de mortalidad en los recién nacidos con HDC durante el primer día de vida.Método. Estudio observacional analítico de cohorte retrospectiva. Se incluyeron los pacientes con HDC, en forma consecutiva, desde marzo de 2012 a noviembre de 2018. Se analizaron como predictores el índice de oxigenación (IO), valor más alto de presión parcial de dióxido de carbono en sangre (pCO2), puntaje de gravedad SNAPPE II, ecocardiograma, dosaje de NT-pro péptido natriurético B (NT-proPNB), todos medidos en el primer día de vida.Resultados. La población fue de 178 pacientes con HDC. La sobrevida, del 75 %. El 24 % recibió oxigenación por membrana extracorpórea. La presencia precoz de hipertensión pulmonar sistémica o suprasistémica no mostró capacidad predictiva (OR 2,2; IC 95 %: 0,8-8), p = 0,1. NT-proPNB tampoco mostró buena discriminación (área bajo la curva (ABC) 0,46, p = 0,67). El IO, SNAPPE II y el valor más alto de pCO2 mostraron buena discriminación, ABC IO 0,82, ABC SNAPPE II 0,86 y ABC pCO2 0,75, p < 0,001.Conclusión.SNAPPE II, IO y valor más alto de pCO2, medidos el primer día de vida, mostraron buena capacidad predictiva con respecto a la evolución; SNAPPE II fue superior al IO y al valor más alto de CO2.

Introduction: Congenital diaphragmatic hernia (CDH) prevalence is low while its associated morbidity and mortality rates are high. Postnatal prognostic factors on the first day of life are useful for predicting the outcome. Objectives: To determine the mortality predictive ability of postnatal echocardiographic, clinical, and biochemical factors among newborn infants with CDH in their first day of life. Method: Observational analytical study of a retrospective cohort. Patients with CDH were consecutively included between March 2012 and November 2018. On the first day of life, analyzed predictors were the oxygenation index (OI), the highest partial pressure of carbon dioxide (pCO2) level in blood, the SNAPPE II severity score, the echocardiography, and the N-terminal pro-B-type natriuretic peptide (NTproBNP) value. Results: The population consisted of 178 patients with CDH. Survival was 75 %. Extracorporeal membrane oxygenation was used in 24 %. The early onset of systemic or suprasystemic pulmonary hypertension showed no predictive ability (OR: 2.2, 95 % CI: 0.8-8), p = 0.1. NT-proBNP did not show good discrimination either (area under the curve [AUC]: 0.46, p = 0.67). The OI, SNAPPE II score, and the highest pCO2 level showed adequate discrimination power, AUC for OI: 0.82, AUC for SNAPPE II: 0.86, and AUC for pCO2: 0.75, p < 0.001. Conclusion: The SNAPPE II score, the OI, and the highest pCO2 level measured on the first day of life, showed a good predictive ability in terms of the course of the disease; the SNAPPE II score was better than the OI and the highest pCO2 level.

Factores de riesgo asociados a mortalidad en recién nacidos con hernia diafragmática congénita / Risk factors associated with mortality in newborn infants with congenital diaphragmatic hernia

Introducción. La hernia diafragmática congénita presenta alta morbimortalidad. Existen herramientas para predecir sobrevida, tanto prenatal (índice pulmón-cabeza observado/esperado ­observed/expected lung-to-head ratio; OE-LHR, por sus siglas en inglés­, presencia de hígado en tórax) como posnatal (puntaje del Grupo de Estudio sobre Hernia Diafragmática Congénita, Congenital Diaphragmatic Hernia Study Group, CDHSG). El objetivo fue identificar factores de riesgo asociados a mortalidad y estimar la mortalidad ajustada por riesgo prenatal en el subgrupo de pacientes con hernia izquierda aislada.Población y métodos. Estudio retrospectivo y analítico de pacientes nacidos en el Hospital Italiano de Buenos Aires durante 2011-2018. Se realizó un análisis multivariable para evaluar factores de riesgo asociados a mortalidad. Para la mortalidad ajustada por riesgo prenatal, se realizó una razón entre la mortalidad observada y la media "esperada" según el OE-LHR.Resultados. Se incluyeron 53 pacientes. La mediana de edad gestacional fue 38 semanas, y la media de peso al nacer, 3054 gramos. El 73 % de los pacientes tuvo hernia aislada. La mortalidad global fue del 45 %, mayor en pacientes con malformaciones asociadas. En el análisis multivariable, la presencia de hipertensión pulmonar grave estimada por ecocardiografía postnatal se asoció en forma independiente a mortalidad: (odds ratio ajustado 6,4; IC 95 %: 1,02-40). La mortalidad global observada en pacientes con hernia izquierda aislada fue similar a la esperada (razón 1,05).Conclusión. La mortalidad global es similar a la esperada según el OE-LHR. En nuestra población, la hipertensión pulmonar grave luego del nacimiento resultó determinante de la mortalidad.

Introduction. Morbidity and mortality are high in congenital diaphragmatic hernia. Some tools help to predict survival, both prenatally (observed/expected lung-to-head ratio [OE-LHR], presence of the liver in the chest) and postnatally (Congenital Diaphragmatic Hernia Study Group [CDHSG] score). Our objective was to identify the risk factors associated with mortality and estimate the risk-adjusted mortality in the prenatal period in the subgroup of patients with isolated left-sided hernia.Population and methods. Retrospective and analytical study of patients born at Hospital Italiano de Buenos Aires between 2011 and 2018. A multivariate analysis was done to assess mortality-associated risk factors. For risk-adjusted mortality in the prenatal period, the ratio between the observed mortality and the mean "expected" mortality based on the OE-LHR was estimated.Results. A total of 53 patients were included. Their median gestational age was 38 weeks, and their mean birth weight was 3054 g. Isolated hernia was observed in 73 % of patients. Overall mortality was 45 %, and higher in patients with associated malformations. In the multivariate analysis, the presence of severe pulmonary hypertension estimated by postnatal echocardiogram was independently associated with mortality (adjusted odds ratio: 6.4, 95 % confidence interval: 1.02-40). The observed overall mortality in patients with isolated left-sided hernia was similar to that expected (ratio: 1.05).Conclusion. Overall mortality was similar to that expected based on the OE-LHR. In our population, severe pulmonary hypertension after birth was a determining factor of mortality

Hernia diafragmática congénita recurrente asociada a malrotación intestinal / Recurrent congenital diaphragmatic hernia associated with intestinal malrotation

RESUMEN La recurrencia de las hernias diafragmáticas congénitas, luego de reparadas quirúrgicamente, es de baja frecuencia. Se presenta el caso de un paciente masculino de 22 años con hernia diafragmática congénita reparada inicialmente al segundo día de vida, el cual presenta disnea grado 1 y enfermedad por reflujo gastroesofágico. Se evidencia por medio de imagen una hernia diafragmática izquierda, el saco herniario con contenido de bazo, colon, cola de páncreas y glándula suprarrenal izquierda. Se aborda de manera abdominal mediante incisión subcostal izquierda, se reduce el saco herniario, se coloca malla tipo en el defecto diafragmático y se realiza procedimiento de Ladd por la presencia de malrotación intestinal.

ABSTRACT Recurrence of congenital diaphragmatic hernia, after surgical repair, is infrequent. Here, we report the case of a 22-year-old male patient with a history of congenital diaphragmatic disease initally treated with surgery on the second day of life who presented class I dyspnea and gastroesophageal reflux. A left diaphragmatic hernia with a hernia sac containing the spleen, colon, tail of pancreas and left adrenal gland. The patient underwent surgical repair via a left subcostal incision; the hernia sac was reduced, the diaphragmatic defect was repaired with a mesh and the Ladd procedure was performed due to the diagnosis of intestinal malrotation.

Evaluation of long term outcome of congenital diaphragmatic hernia survivors: a single centre 8 years’ experience in a developing nation with limited facilities

Background: The objective of the study was to find out incidence of long term complications in congenital diaphragmatic hernia (CDH) survivors in a developing nation with limited facilities.Methods: A retrospective study was done on patients who underwent CDH repair at our institution from 2012 to 2019. 71 patients were identified of these 55 patients operated in neonatal age were considered. After applying exclusion criteria 42 patients included in the study were then divided in 2 groups. Group 1 (26 patients): neonates requiring ventilation within 6 hrs of birth and group 2 (16 patients): not requiring ventilation or intubated after 6hrs of birth. Data from medical records were supplemented by a questionnaire regarding perceived physical function and medical follow up till date. Respiratory, central nervous, musculoskeletal and gastrointestinal systems were concentrated upon and questionnaire set. These were then compared with respect to their long term outcomes. Also, overall incidence of these in the two groups combined was noted compared with other studies.Results: On comparing these 2 groups incidence of long term complications was found more in group 1; however on statistical analysis difference was not significant. As also incidence of individual long term complications in all patients together were identical or lower than in other published series. Mortality in our study was 8 of 49 neonates which was 17% of all patients presenting with CDH or born at our centre.Conclusions: Despite the growing population of CDH survivors the morbidity is not very significant and most patients lead a normal average active life.

Thoraco-laparoscopic management of diaphragmatic hernia of adults: a case series

Diaphragmatic hernia (DH) usually occurs in childhood; however, in adult it is uncommon. Most common aetiology of DH in adult is trauma while spontaneous DH is a rare entity accounting for <1%. The management is primarily surgical repair in the form of open or laparoscopic approach; the later has been associated with low morbidity, mortality and excellent long term outcomes with low rate of recurrence. In this case series, confirmation of the diagnosis was done by CT scan and by laparoscopy in all the patients; laparoscopic repair was performed in all the patients along with thoracoscopic aid in two cases. We found that minimal access techniques can be safely done in DH; in conditions when the herniated content are difficult to get reduced laparoscopically alone, a simultaneous thoracoscopy has helped in great deal in reducing the content and completing the procedure entirely by minimal access techniques. This gives all the advantages of minimally invasive approach like less postoperative pain, shorter hospital stay, quicker recovery and low recurrence rate; provided tissue repair is done in tension free manner by an experienced laparoscopic surgeon.