Preliminary study on the value of fetal MRI lung volume and mediastinal shift angle for predicting prognosis of left congenital diaphragmatic hernia / 中华放射学杂志

Objective:To explore the value of fetal lung volume and mediastinal shift angle (MSA) based on fetal MRI in predicting the prognosis of congenital diaphragmatic hernia (CDH).Methods:Fetuses with left CDH that did fetal MRI in Xinhua Hospital Affiliated with Shanghai Jiao Tong University School of Medicine from September 2016 to January 2022 were retrospectively collected. There were 65 patients, and their gestational age was 29 (26, 35) weeks when they were diagnosed with left CDH by MRI. Observed fetal lung volume and MSA were measured based on fetal MRI, and observed/expected lung volume (o/eFLV) based on gestational age was calculated. The clinical data were collected from birth to discharge, and patients were divided into survival group and death group in case of prognosis at discharge, with 54 cases in the survival group and 11 cases in the death group. The student′s t test was used to compare the difference of o/eFLV and MSA between the survival group and the death group, and the receiver operating characteristic (ROC) curve was used to evaluate the value of o/eFLV and MSA in predicting the prognosis of left CDH. Results:The o/eFLV of the survival group was 51.5%±18.3%, higher than that of the death group (27.8%±4.4%), and the difference was significant ( t=8.29, P<0.001). The MSA of the survival group was 33.1°±1.2°, lower than that of the death group (41.8°±2.7°), and the difference was significant ( t=-11.15, P<0.001). The area under the ROC curve (AUC) of o/eFLV to predict the fetal survival or death was 0.939 (95%CI 0.851-0.983), the cutoff value was 33.8%, the sensitivity was 100%, the specificity was 88.9%. The AUC of MSA was 0.998 (95%CI 0.941-1.000), the cutoff value was 37.2°, the sensitivity was 100%, the specificity was 98.2%. Conclusions:The o/eFLV and MSA that were measured based on fetal MRI can effectively predict the fetus′s prognosis with left CDH.

Hernia diafragmática derecha postraumática de presentación tardía. Un caso infrecuente / Post-traumatic right diaphragmatic hernia of late presentation. A rare case

RESUMEN La hernia diafragmática traumática se define como el paso del contenido abdominal a la cavidad torácica a través de un defecto en el diafragma producido por un trauma. Su diagnóstico es difícil, a menudo puede pasar inadvertido, debido a la gravedad de las lesiones que le acompañan. Las claves para el diagnóstico son un alto grado de sospecha y el conocimiento de los distintos signos en la tomografía computarizada. Una vez diagnosticada la ruptura diafragmática, se repara quirúrgicamente para evitar complicaciones. Se presenta el caso de un paciente masculino, de 88 años de edad con antecedentes de haber sufrido trauma toracoabdominal cerrado hacía 35 años, que acudió con un cuadro clínico de oclusión intestinal. Se publica este caso porque, además de ser una entidad poco frecuente, resulta importante el conocimiento de esta enfermedad, para valorarla dentro de las posibilidades diagnósticas en aquellos pacientes que aquejen síntomas relacionados con sus complicaciones.

ABSTRACT Traumatic diaphragmatic hernia is defined as the passage of abdominal contents into the thoracic cavity through a defect in the diaphragm caused by trauma. The diagnosis of traumatic diaphragmatic hernia is difficult, it can often go unnoticed, due to the seriousness of the accompanying injuries. The keys to diagnosis are a high degree of suspicion and knowledge of the various signs on computed tomography. Once the diaphragmatic rupture is diagnosed, it is surgically repaired in order to avoid complications. The case of an 88-year-old male patient with a history of having suffered closed thoracoabdominal trauma 35 years earlier, who presented with a clinical picture of intestinal obstruction is presented. This case is published because, in addition to being a rare entity, knowledge of this disease is important, in order to assess it within the diagnostic possibilities, in those patients who suffer from symptoms related to its complications

Value of MRI findings for the evaluation of a hernia sac in fetuses with congenital diaphragmatic hernia / 中华放射学杂志

Objective:To explore the value of MRI signs in assessing the presence or absence of hernia sacs in fetuses with congenital diaphragm hernia.Methods:MRI images of 57 patients with congenital diaphragm hernia confirmed by postpartum surgery were analyzed from November 2016 to December 2020 in Xinhua Hospital, Shanghai Jiao Tong University School of Medicine, with a gestational age of 20－40 (28±5) weeks. In postpartum surgery, 18 cases were found with hernia sacs (hernia sac group) and 39 cases without hernia sacs (hernia-free group). Seven MRI signs were analyzed, including hernia peripheral enveloping sensation, smooth lung-hernia interface, crescent-shaped lung compression, residual lung tissue on the affected side, heart displacement, effusion above the lung-hernia interface and effusion below the lung-hernia interface. The differences in MRI signs between the hernia sac and hernia-free groups were compared using the χ 2 test or Fisher′s exact probability method. The diagnostic efficacy of each sign was calculated. The MRI signs with statistical differences between the two groups were included in the predictive integration model, and 1 point was scored for each sign, the imaging score of each fetus was calculated, and the efficacy of imaging points in diagnosing the presence or absence of hernia sacs was assessed by the subject manipulation receiver operating characteristics (ROC) curve. Results:There were statistically significant differences in 5 MRI signs between the hernia sac and the hernia-free groups, namely hernia peripheral enveloping sensation (χ2=25.74, P<0.001), smooth lung-hernia interface (χ2=48.20, P<0.001), crescent-shaped lung compression (χ2=57.00, P<0.001), residual lung tissue on the affected side (χ2=12.14, P<0.001) and effusion above the lung-hernia interface (χ2=4.31, P=0.022). Among them, the sign of crescent-shaped lung compression had the highest diagnostic efficacy, and the sensitivity, specificity and accuracy all were 100%. Five statistically significant MRI signs were included in the predictive integration model, and the area under the ROC curve was 0.999, the sensitivity was 100%, the specificity was 94.9%, and the optimal threshold was 2 points. Conclusion:Fetal MRI signs and predictive integration model can effectively identify the presence or absence of hernia sacs in fetuses with congenital diaphragm hernia, which has certain clinical significance.

Natural evolution of perforating wounds of 30% extension of the left diaphragm and the anatomopathological characteristics of its healing. Experimental Study / Evolução natural das feridas perfurocortantes de 30% de extensão do diafragma esquerdo e das características anatomopatológicas de sua cicatrização. Estudo Experimental

ABSTRACT Introduction: diaphragmatic injury is a challenge for surgeons. It is an injury that can be isolated. It is frequent in penetrating thoracoabdominal trauma. It represents a diagnostic challenge and the ideal approach is not yet well established. The occurrence of spontaneous healing of these injuries is still much discussed and even more, if it does, what is the healing mechanism? Objective: to macroscopically and histologically evaluate the natural evolution of perforation and cutting wounds equivalent to 30% of the left diaphragm. Method: 50 specimens of rats underwent a surgical procedure and, after 30 days, were euthanized and those that presented scar tissue in the diaphragm, the samples were submitted to histopathological study, using the hematoxylin and eosin stains, Massons trichrome and Picrosirius to assess the presence of collagen or muscle fibers (hyperplasia) in the scar. Results: it was found that healing occurred in diaphragmatic injuries in 90% of rats. We also observed the presence of fibrosis in all analyzed samples. Conclusion: Spontaneous healing occurred in most diaphragmatic injuries and the inflammatory reaction represented by the presence of fibrosis and collagen deposition was observed in all our samples. Muscle fiber hyperplasia did not occur.

RESUMO Introdução: o ferimento diafragmático é um desafio para os cirurgiões. É uma lesão que pode ser isolada. É frequente nos traumas penetrantes toracoabdominais. Representa um desafio diagnóstico e a conduta ideal ainda não está bem estabelecida. A ocorrência da cicatrização espontânea dessas lesões é ainda muito discutida e mais ainda, se ocorre, qual o mecanismo de cicatrização? Objetivo: avaliar macroscopicamente e histologicamente a evolução natural das feridas perfuro cortantes equivalentes a 30% do diafragma esquerdo. Método: 50 espécimes de ratos, foram submetidos a procedimento cirúrgico e, após 30 dias, foram submetidos à eutanásia e aqueles que apresentaram tecido cicatricial no diafragma, as amostras foram submetidas a estudo histopatológico, usando as colorações de hematoxilina e eosina, tricrômico de Masson e Picrossirius para avaliar a presença de colágeno ou de fibras musculares (hiperplasia) na cicatriz. Resultados: verificou-se que ocorreu nas lesões diafragmáticas, a cicatrização em 90% dos ratos. Observamos também, a presença de fibrose em todas as amostras analisadas. Conclusão: ocorreu cicatrização espontânea na maioria das lesões diafragmáticas e a reação inflamatória representada pela presença de fibrose e deposição de colágeno foi observada em todas as nossas amostras. Não ocorreu hiperplasia de fibras musculares.

Diagnostic performance of multislice computed tomography to detect diaphragmatic injuries in hemodynamically stable patients. Preliminary results / Rendimiento diagnóstico de la tomografía computarizada multidetector para la identificación de heridas diafragmáticas en pacientes hemodinámicamente estables. Resultados preliminares

Abstract Introduction: Diaphragmatic injuries are a common finding in patients with penetrating thoracoabdominal trauma. Their diagnosis requires exploration through open or laparoscopic surgery. However, multislice computed tomography (MSCT) emerges as a useful noninvasive tool for this purpose. Objective: To determine the diagnostic performance of MSCT for detecting diaphragmatic injuries in hemodynamically stable patients with penetrating thoracoabdominal trauma. Materials and methods: Prospective study conducted on 119 patients treated at the Hospital Universitario del Valle, Cali, Colombia, between March 2012 and June 2015. In order to evaluate the diagnostic performance of MSCT, the results obtained through this test were compared with those reported in the reference test (open surgery). Two readings of the imaging studies were performed by 2 radiologists. Intra- and interobserver agreement on the MSCT readings were analyzed using the Fleiss' Kappa coefficient. Results: MSCT had sensitivity and specificity of 94.4% and 46.8%, respectively. Its positive (PPV) and negative (NPV) predictive values were 44.7% and 94.8%, respectively. The positive likelihood ratio (LR+) was 1.7765, and the negative likelihood ratio (LR-) was 0.1186. Regarding interobserver agreement, Fleiss' Kappa coefficient between the first reading of both radiologists was 0.4425. Conclusions: MSCT specificity for diagnosing diaphragmatic injuries found in this study differs considerably from that reported in the literature. Considering the sensitivity, PPV and LR- values obtained in the present study, MSCT could be an important tool for the management of these patients since conservative treatment may be used in patients whose diaphragmatic injuries are not detected with this type of scan.

Resumen Introducción. Las heridas diafragmáticas son un hallazgo común en pacientes con trauma toracoabdominal penetrante. Su diagnóstico requiere exploración mediante cirugía abierta o laparoscópica. Sin embargo, la tomografía computarizada multidetector (TCMD) surge como una herramienta no invasiva útil para este propósito. Objetivo. Determinar el rendimiento diagnóstico de la TCMD en la identificación de heridas diafragmáticas en pacientes con trauma toracoabdominal penetrante hemodinámicamente estables. Materiales y métodos. Estudio prospectivo realizado en 119 pacientes atendidos en el Hospital Universitario del Valle, Cali, Colombia, entre marzo de 2012 y junio de 2015. Para evaluar el rendimiento diagnóstico de la TCMD, los resultados obtenidos mediante esta prueba fueron comparados con los reportados por el test de referencia (cirugía abierta). Se realizaron 2 lecturas de los estudios de imagen por 2 radiólogos. La concordancia intra e interobservador respecto a la lectura de las TCMD se analizó mediante el coeficiente Kappa de Fleiss. Resultados. La TCDM tuvo una sensibilidad y especificidad de 94.4% y 46.8%, respectivamente. Sus valores predictivos positivo (VPP) y negativo (VPN) fueron 44.7% y 94.8%, respectivamente. La razón de verosimilitud positiva (LR+) fue 1.7765, y la razón de verisimilitud negativa (LR-), 0.1186. Respecto a la concordancia interobservador, el coeficiente Kappa de Fleiss entre la primera lectura de ambos radiólogos fue de 0.4425. Conclusiones. La especificidad de la TCMD para el diagnóstico de heridas diafragmáticas encontrada en el presente estudio difiere considerablemente de lo reportado en la literatura. Teniendo en cuenta la sensibilidad, el VPN y la LR- obtenidos en el presente estudio, la TCMD podría constituir una herramienta importante en el manejo de estos pacientes, ya que en aquellos en los que no se detecte herida diafragmática mediante este tipo de tomografía podría considerarse un manejo conservador.

Research advances in diaphragmatic hernia after thermal ablation for primary liver cancer / 临床肝胆病杂志

Thermal ablation, including radiofrequency ablation and microwave ablation, is a frequently used method for local treatment of primary liver cancer with a marked clinical effect, and it has been widely used in clinical practice in recent years. Diaphragmatic hernia is a rare complication of thermal ablation, but its incidence rate tends to gradually increase in recent years. This article reviews the cases of diaphragmatic hernia after thermal ablation for primary liver cancer in China and globally and elaborates on possible risk factors, diagnosis and treatment, and prevention strategies, so as to improve the understanding of diaphragmatic hernia after thermal ablation for primary liver cancer among clinicians and achieve the goal of preoperative prevention, rapid diagnosis and intervention, and reduced mortality rate.

Manejo laparoscópico de hernia diafragmática traumática: reporte de un caso / Laparoscopic management of traumatic diaphragmatic hernia: a case report

La hernia diafragmática traumática representa un reto al momento del diagnóstico. Una detección oportuna permite establecer un correcto tratamiento quirúrgico. Se presenta el caso de un paciente masculino de 37 años de edad con antecedente de trauma torácico por arma blanca 4 años antes; quien acude por cuadro de dolor abdominal intenso localizado en epigastrio que se irradia a hipocondrio izquierdo de aparición súbita 72 horas antes de su ingreso. Al examen físico murmullo vesicular disminuido en campo pulmonar izquierdo, y abdomen doloroso a la palpación a nivel de epigastrio e hipocondrio izquierdo. En exámenes de laboratorio no se evidencian alteraciones, mientras que la tomografía reporta hernia diafragmática izquierda; la misma que fue resuelta quirúrgicamente mediante técnica laparoscópica.

Traumatic diaphragmatic hernia represents a challenge at diagnosis. A timely detection allows to establish a correct surgical treatment. It is presented the case of a 37-year-old male patient with a history of stabbing chest trauma 4 years earlier; who comes for symptoms of intense abdominal pain located in the epigastrium that radiates to the left hypochondrium of sudden onset 72 hours before admission. On physical xamination, vesicular murmur decreased in the left lung field, and a painful abdomen on palpation at the level of the epigastrium and left hypochondrium were showed. Laboratory tests did not show any alterations, but tomography reports a left diaphragmatic hernia; this one was solved surgically by laparoscopic technique.

Thoracotomy compared to laparotomy in the traumatic diaphragmatic hernia. Systematic review and proportional methanalysis

Abstract Purpose: To evaluate the most used approach to treat traumatic diaphragmatic ruptures, and in which one the requirement to assess the second cavity is more frequent. Methods: Systematic review, observational studies. Outcomes: moment of approach, most commonly via addressed and the requirement to open the other cavity. Bases searched: Lilacs, Pubmed, Embase, Clinicaltrials.gov and Web of Science. Statistical analysis: StatsDirect 3.0.121 software. Results: Sixty eight studies (2023 participants) were included. Approach in acute phase was performed four times more than in chronic phase. Approach: abdominal 65% (IC 95% 63-67%), thoracic 23% (IC 95% 21-24%), abdominal in the acute phase 75% (IC 95% 71-78%), and chronic 24% (IC 95% 19-29%), thoracic in the acute phase 12% (IC 95% 10-14%) and chronic 69% (IC 95% 63-74%). Thorax opening in the abdominal approach: 10% (95% CI 8-14%). Abdomen opening in the thoracic approach: 15% (95% CI 7-24%). Conclusions: The most common approach was the abdominal. The approach in the acute phase was more common. In the acute phase the abdominal approach is more frequent than the thoracic approach. In the chronic phase the thoracic approach is more frequent than the abdominal one. The requirement to open the second cavity was similar in both approaches.

Acquired diaphragmatic hernia in pediatrics after living donor liver transplantation / 中华普通外科杂志

Objective To explore the diagnosis and treatment of diaphragmatic hernia (DH)secondary to living donor liver transplantation (LDLT) in pediatrics.Methods The primary disease was biliary atresia and all of the 4 patients underwent LDLT using a donor's left lateral graft.The ages of recipients were 5-7 months at LDLT and the onset of DH were 1.5-16 months after LDLT.There were 3 right DH and 1 left DH,and 3 were emergency cases.Results The graft weight and graft to recipient body weight ratio (GRWR) were respectively between 170-290 g and between 2.7％-5.0％.Clinical symptoms included urgent respiratory distress,dyspepsia or gastrointestinal obstruction.DH was diagnosed by computed tomography scan or X-ray of the chest.Laparotomy were performed successfully to repair the DH including emergency laparotomy in three patients.Herniated organs were partial intestines or colon and partial stomach.All cases recovered without major complications.Conclusions DH post-LDLT is an unusual complication,often calls for emergent management.

Effect and significance of tetrandrine on expression of Rho/Rho kinase in fetal lung of congenital diaphragmatic hernia rats / 重庆医学

Objective To investigate the effect and significance of the traditional Chinese medicine tetrandrine(TET) prenatal intervention on the expression of RhoA protein and Rho kinase ROCK1 in the fetal lung of congenital diaphragmatic hernia (CDH) rat model.Methods SD female rats with 9.5 d of gestation were randomly divided into the control group,CDH group and TET group.The CDH group and TET intervention group were administered with nitrofen by gavage for establishing CDH model.The TET intervention was given on 16.5 d of gestation.The fetal rat lungs were taken by cesarean section on 21.5 d of gestation and the lung weight/body ratio(Lw/Bw) was measured.The lung development and small pulmonary arterial morphologic changes in HE staining in all groups were observed with microscopy.The protein expression of RhoA and Rho kinase ROCK1were respectively examined by immunohistochemistry and Western blot.Results In the CDH group,the lungs had obvious maldevelopment and the fetal lung development in the TET group was close to that in the control group.The lung development indicators of Lw/Bw,PAA％,and lung vascular remodeling indicators of lumen area and vascular total area ratio(LA％) in the CDH group were significantly lower than those in the control group(2.11±0.36 vs.4.24±0.31;33.60±3.12 vs.58.81 ±2.92;38.58±2.15 vs.61.20±3.23,P＜0.05),the indicators of Lw/Bw.PAA％ and LA％ after TET intervention were significantly improved compared with the CDH group(3.61±0.24 vs.2.11±0.36;42.46±3.68 vs.33.60±3.12;56.07±3.32 vs.38.58±2.15,all P ＜0.05);the ratio of small pulmonary artery wall thickness to vascular external diameter (WT％) and the medium thickness percentage (MT％) in CDH group were significantly higher than those in the control group(26.64±2.41 vs.13.50±1.45 and 25.98±2.79 vs.16.47±2.07,P＜0.05),WT％ and MT％ in the TET group were obviously lower than those in the CDH group (16.02±2.35 vs.26.64± 2.41 and 17.96 ± 1.95 vs.25.98 ± 2.79,P＜0.05).The immunohistochemistry and Western blot detection indicated that the expressions of RhoA and ROCK1 from low to high were the control group ＜TET group ＜ CDH group.Conclusion Pulmonary hypoplasia and lung vascular remodeling exist in fetal rats with CDH and Rho/Rho kinase signaling pathway may be involved in the process.Prenatally giving TET may play the lung protective effect by regulating the Rho/Rho signal pathway.

An Unexpected Adverse Event during Colonoscopy Screening: Bochdalek Hernia / 대한소화기학회지

Bochdalek hernia (BH) is defined as herniated abdominal contents appearing throughout the posterolateral segment of the diaphragm. It is usually observed during the prenatal or newborn period. Here, we report a case of an adult patient with herniated omentum and colon due to BH that was discovered during a colonoscopy. A 41-year-old woman was referred to our hospital with severe left chest and abdominal pain that began during a colonoscopy. Her chest radiography showed colonic shadow filling in the lower half of the left thoracic cavity. A computed tomography scan revealed an approximately 6-cm-sized left posterolateral diaphragmatic defect and a herniated omentum in the colon. The patient underwent thoracoscopic surgery, during which, the diaphragmatic defect was closed and herniated omentum was repaired. The patient was discharged without further complications. To the best of our knowledge, this case is the first report of BH in an adult found during a routine colonoscopy screening.

An Incidental Discovery of Morgagni Hernia in an Elderly Patient Presented with Chronic Dyspepsia / 대한소화기학회지

A Morgagni hernia was first described in 1761 by Giovanni Morgagni. In adults, it is accompanied by gastrointestinal- or respiratory-type symptoms. Herein, we report an 84-year-old woman presented to our hospital with nausea and vomiting. After hospitalization, an X-ray revealed a right diaphragmatic hernia. Based on the results of abdominal computed tomography, duodenoscopy, and upper gastrointestinography (gastrografin), we concluded that her symptoms were caused by Morgagni hernia. Our patient underwent laparoscopic surgery, and shortly thereafter, her symptoms resolved.

Hernia diafragmática traumática. Presentación de un caso / Traumatic Diaphragmatic Hernia. A Case Report

El trauma diafragmático se produce de forma secundaria tras lesiones penetrantes y trauma cerrado del abdomen y tórax. La hernia diafragmática traumática representa una entidad poco frecuente en la clínica. Cuando se presenta constituye un verdadero reto diagnóstico para el internista, cirujano y el radiólogo. Requiere de un alto índice de sospecha y el uso adecuado de las imágenes diagnósticas. El tratamiento siempre es quirúrgico. Por tales razones se presenta el caso de un paciente que fue operado debido a una herida por arma blanca en el tórax y que con posterioridad se le diagnosticó una hernia diafragmática traumática en el lado izquierdo del torax. Volvió a ser operado y la evolución posquirúrgica resultó satisfactoria.

Diaphragmatic trauma is secondary to penetrating injuries and blunt abdominal and thoracic trauma. Traumatic diaphragmatic hernia is an uncommon entity. Its diagnosis poses a real challenge to internists, surgeons and radiologists since it requires a high level of suspicion and appropriate use of diagnostic imaging. Surgical treatment is always needed. For these reasons, we present the case of a patient operated on due to a stab wound to the chest who was subsequently diagnosed with a traumatic diaphragmatic hernia on the left side of the chest. He underwent surgery again and had a successful postoperative recovery.

Analysis of natural history of the diaphragmatic injury on the right in mice / Análise da evolução natural dos ferimentos diafragmáticos à direita em ratos

Objective: to evaluate natural evolution of right diaphragmatic injury after the surgical removal of a portion from hemi diaphragm. Methods: the animals were submitted to a surgical removal of portion from right hemi diaphragm by median laparotomy. The sample consists of 42 animals being 2 animals from pilot project and 40 operated animals. And the variables of the study were herniation, liver protection, healing, persistent diaphragm injury, evaluation of 16 channels tomography and the variables "heart rate" and "weight". Results: we analyzed 40 mice, we had two post-operative deaths; we had 17 animals in this group suffered from herniation (42.5%) and 23 animals didn't suffer from herniation (57.5%). Analyzing the tomography as image method in the evaluation of diaphragmatic hernia, we had as a method with good sensitivity (78.6%), good specificity (90.9%), and good accuracy (86.1%) when compared to necropsy. Conclusion: there was a predominance of healing of right hemi diaphragm, the size of initial injury didn't have influence on occurrence of the liver protection or hernia in mice.

Objetivo: avaliar a evolução natural do ferimento diafragmático à direita após a retirada cirúrgica de uma porção do hemidiafragma. Métodos: os animais foram submetidos à ressecção de uma porção do hemidiafragma à direita através da laparotomia mediana. Foram operados 40 ratos. As variáveis analisadas foram: herniação, proteção hepática, cicatrização, lesão persistente do diafragma, avaliação da tomografia computadorizada, frequência cardíaca e peso. Resultados: foram analisados 40 ratos. Houve duas mortes no pós-operatório. Dezessete animais tiveram herniação (42,5%) e 23 (57,5%), não. Analisando emprego da tomografia computadorizada na avaliação da hérnia diafragmática, tivemos um método com boa sensibilidade (78,6%), boa especificidade (90,9%) e boa acurácia (86,1%) quando comparados com a necrópsia. Conclusão: houve predomínio de cicatrização do hemidiafragma à direita e o tamanho da lesão inicial não influenciou na ocorrência de proteção hepática ou hérnia em ratos.

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

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

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

Integrated prenatal diagnosis and postnatal treatment for moderate and severe congenital diaphragmatic hernia / 中华围产医学杂志

Objective To share our experiences on integrated services in providing fetal diagnosis and postnatal treatment for congenital diaphragmatic hernia(CDH).Methods A retrospective analysis was conducted on 25 pregnancies diagnosed as CDH by both prenatal ultrasound and MRI in Maternal and Children Hospital of Guangdong Province from January 2012 to January 2014.All of the subjects received integral medical management including prenatal management (prenatal diagnosis and consultation), perinatal management (prenatal care and delivery) and neonatal treatment.Results Among the 25 CDH fetuses, 11 were mild, nine were moderate, and five were severe.One severe case, who was diagnosed at 26 gestational weeks, was aborted on demand of the mother.The other 24 cases continued their pregnancy and all delivered after 35 weeks including 13 cesarean sections (one due to twin pregnancy and 12 due to maternal demand) and 11 vaginal birth.The mean gestational age when CDH was diagnosed was (24.5 ± 3.5) weeks, and the 24 women delivered at an average of (37.5 ± 1.4) gestational weeks.The eleven mild cases accepted mask oxygenation.For those 13 moderate or severe CDH cases, all received dexamethasone to promote fetal lung maturity at 32 gestational weeks, seven were intubated before clamp the cord, and the other six did after.These 13 babies accepted high-frequency oscillation ventilation, with a median duration of 58 hours, and some of them treated with inhaled nitric oxide on requirement with a median duration of 52 hours.Except two cases died before operation, the rest 22 cases underwent neonatal surgery.One moderate case died at 48 hours after surgery due to pulmonary hypertension and respiratory failure.Another one severe case withdrew treatment at two months old.The other 20 infants recovered fully.Conclusions Integrated management including prenatal diagnosis and postnatal treatment, provides an effective and streamlined mode for diagnosis and treatment of CDH.Therefore,it might minimize potential medical risks.

Multislice CT and Clinical Manifestations of Diaphragmatic Hiatus Hernia with Hernial Sac Effusion / 中国医学影像学杂志

PurposeTo explore the multislice CT (MSCT) and clinical manifestations of diaphragmatic hiatus hernia with hernial sac effusion, in order to improve its diagnostic rate.Materials and MethodsMSCT data were retrospectively analyzed for morphologies and clinical manifestations in 32 patients with diaphragmatic hiatus hernia and hernial sac effusion.Results32 patients included 21 males and 11 females (χ2=0.64,P>0.05). 25 patients were older than 60 years and 7 cases younger than 60 years (χ2=13.58,P<0.01). There were 27 cases with non-viscera hiatus hernia including 23 cases of combined fat-water hernia and 4 cases of simple effusion. 5 cases were of viscera type hiatus hernia (χ2=5.47,P<0.05). 28 cases showed ascites including 23 cases with non-viscera hiatus hernia and 1 case with viscera type hiatus hernia (χ2=9.56,P<0.01). The axial images from different levels of non-viscera hiatus hernia with liquid hernial sac demonstrated quasi-circular, meniscus, ringlike and teardrop shapes.Viscera type hiatus hernia and liquid hernial sac were found to be irregular shape . All patient sufferered from dysphagia, chest distress and epigastric discomfort.ConclusionThe increased pressure gradient between thorax and abdomen driving ascites into supradiaphragmatic hernial sac and clamping by hiatus may be the main mechanism. Quasi-circular, meniscus, ringlike andteardrop were the characteristic signs of diaphragmatic hiatus hernia with hernial sac effusion.

Comorbidity between Klinefelter syndrome and diaphragmatic hernia. A case report / Comorbidade entre síndrome de Klinefelter e hérnia diafragmática.Um relato de caso

CONTEXT: Intrathoracic cystic lesions have been diagnosed in a wide variety of age groups, and the increasing use of prenatal imaging studies has allowed detection of these defects even in utero. CASE REPORT: A 17-year-old pregnant woman in her second gestation, at 23 weeks of pregnancy, presented an ultrasound with evidence of a cystic anechoic image in the fet al left hemithorax. A morphological ultrasound examination performed at the hospital found that this cystic image measured 3.7 cm x 2.1 cm x 1.6 cm. Polyhydramnios was also present. At this time, the hypothesis of cystic adenomatoid malformation was raised. Fet al echocardiography showed only a dextroposed heart. Fet al magnetic resonance imaging produced an image compatible with a left diaphragmatic hernia containing the stomach and at least the first and second portions of the duodenum, left lobe of the liver, spleen, small intestine segments and portions of the colon. The stomach was greatly distended and the heart was shifted to the right. There was severe volume reduction of the left lung. Fet al karyotyping showed the chromosomal constitution of 47,XXY, compatible with Klinefelter syndrome. In our review of the literature, we found only one case of association between Klinefelter syndrome and diaphragmatic hernia. CONCLUSIONS: We believe that the association observed in this case was merely coincidental, since both conditions are relatively common. The chance of both events occurring simultaneously is estimated to be 1 in 1.5 million births. .

CONTEXTO: Lesões císticas intratorácicas são diagnosticadas em ampla variedade de faixas etárias, e o uso aumentado dos estudos de imagem pré-natal tem permitido a detecção desses defeitos ainda intraútero. RELATO DO CASO: Uma gestante de 17 anos que estava em sua segunda gravidez, com 23 semanas de gestação, apresentava ultrassom com evidência de imagem cística anecoica no hemitórax esquerdo fet al. O ultrassom morfológico realizado no hospital verificou que esta media 3,7 cm x 2,1 cm x 1,6 cm. Evidenciou-se também a presença de polidrâmnio. Neste momento, levantou-se a hipótese de malformação adenomatoide cística. A ecocardiografia fet al mostrou apenas coração desviado para a direita. A ressonância magnética fet al revelou imagem compatível com hérnia diafragmática à esquerda, contendo estômago e, pelo menos, primeira e segunda partes do duodeno, lobo esquerdo do fígado, baço, segmentos de intestino delgado e porções do cólon. O estômago mostrava-se muito distendido e o coração, deslocado para a direita. Havia redução importante do volume do pulmão esquerdo. O cariótipo fet al mostrou constituição cromossômica 47,XXY, compatível com a síndrome de Klinefelter. Em nossa revisão da literatura, encontramos apenas um caso de associação entre síndrome de Klinefelter e hérnia diafragmática. CONCLUSÃO: Acreditamos que a associação observada neste caso foi puramente uma coincidência, uma vez que ambas as condições são relativamente comuns. A chance de os dois eventos ocorrerem simultaneamente é estimada em 1 em 1,5 milhões de nascimentos. .

Standardization of pulmonary ventilation technique using volume-controlled ventilators in rats with congenital diaphragmatic hernia / Padronização da técnica de ventilação pulmonar utilizando ventiladores com volume controlado em ratos com hérnia diafragmática congênita

OBJECTIVE: To standardize a technique for ventilating rat fetuses with Congenital Diaphragmatic Hernia (CDH) using a volume-controlled ventilator. METHODS: Pregnant rats were divided into the following groups: a) control (C); b) exposed to nitrofen with CDH (CDH); and c) exposed to nitrofen without CDH (N-). Fetuses of the three groups were randomly divided into the subgroups ventilated (V) and non-ventilated (N-V). Fetuses were collected on day 21.5 of gestation, weighed and ventilated for 30 minutes using a volume-controlled ventilator. Then the lungs were collected for histological study. We evaluated: body weight (BW), total lung weight (TLW), left lung weight (LLW), ratios TLW / BW and LLW / BW, morphological histology of the airways and causes of failures of ventilation. RESULTS: BW, TLW, LLW, TLW / BW and LLW / BW were higher in C compared with N- (p <0.05) and CDH (p <0.05), but no differences were found between the subgroups V and N-V (p> 0.05). The morphology of the pulmonary airways showed hypoplasia in groups N- and CDH, with no difference between V and N-V (p <0.05). The C and N- groups could be successfully ventilated using a tidal volume of 75 ìl, but the failure of ventilation in the CDH group decreased only when ventilated with 50 ìl. CONCLUSION: Volume ventilation is possible in rats with CDH for a short period and does not alter fetal or lung morphology. .

OBJETIVO: padronizar uma técnica para ventilar fetos de rato com HDC usando um ventilador volume-controlado. MÉTODOS: ratas grávidas foram distribuídas em: a) Controle (C); e b) Expostos a Nitrofen com HDC e sem HDC (N-). Fetos dos três grupos foram divididos aleatoriamente em subgrupos ventilados (V) ou não ventilados (NV). Os fetos foram coletados no dia 21,5 da gestação, pesados e ventilados por 30 minutos usando um ventilador volume-controlado. A seguir os pulmões foram coletados para estudo histológico. Nós avaliamos: peso corporal (PC), peso pulmonar total (PPT), peso do pulmão esquerdo (PPE), razão PPT/PC e PPE/PC, histologia morfológica das vias aéreas e as causas das falhas da ventilação. RESULTADOS: PC, PPT, PPE, LLW, PPT/PC e PPE/PC foram maiores em C em relação a N- (p<0,05) e a HDC (p<0,05), mas não houve diferenças entre os subgrupos V e NV (p>0,05). A morfologia das vias aéreas pulmonares mostrou hipoplasia nos grupos N- e HDC, não havendo diferença entre V e NV (p<0,05). Os grupos C e N- puderam ser ventilados com sucesso usando o volume corrente de 75ìl, mas a falha de ventilação no grupo HDC só diminuiu quando ventilados com 50ìl. . CONCLUSÃO: a ventilação a volume de ratos com HDC por um curto período é possível e não altera a morfologia fetal ou pulmonar. .

Prenatal diagnosis and fetal outcome of 57 cases of congenital diaphragmatic hernia / 中华围产医学杂志

Objective To explore the prenatal diagnosis and factors influencing prognosis of congenital diaphragmatic hernia (CDH).Methods Fifty seven cases of CDH prenatally diagnosed by ultrasound in the Department of Obstetrics,Xinhua Hospital from January 1,2006 to March 31,2013 were retrospectively reviewed.Prognosis and risk factors were analyzed by ultrasound characteristics and newborn status.Statistical analysis was performed using t test,x2 test and Logistic regression.Results Forty-five (79％)of all cases were left-side CDH and the rest 12 (21％)were right-side.Fourteen fetuses were aborted.Thirty three were delivered and treated surgically,twenty-three infants survived after surgery and ten died,with an overall survival rate of 40％ (23/57).The other ten cases were delivered,but died of no treated surgically.The birth weight of the surviving neonates was heavier than that of those who died [(3 173 ±348) vs (2 846±568)g,t=2.238,P=0.033].The gcstational age at diagnosis of the aborted fetuses was younger than that of the surviving neonates [(24.0 ± 4.1) vs (30.0± 6.0) weeks,t=3.181,P=0.003].Twenty-one (91％,21/23) of the surviving neonates had a lung-to-head ratio (LHR) ≥ 1.4.A linear trend test showed a negative correlation between LHR and adverse outcome (x2=9.223,P=0.002) with a correlation coefficient-0.730 (P=0.000).LHR＜1.4 (OR=35.867,95％CI:1.861-691.341,P=0.018) and polyhydramnios (OR=9.285,95％CI:1.102-78.212,P=0.040) were risk factors for neonatal death.Right-side CDH (OR=0.158,95％CI:0.009-2.824,P=0.210),liver herniation into the thorax (OR=1.730,95％CI:0.144 20.707,P=0.665) and birth weight (OR=l.002,95％CI:0.999-1.005,P=0.135) were not risk factors.Conclusions The survival rate of CDH is still low.Gestational age on diagnosis,presence of polyhydramnios and LHR＜ 1.4 predict an adverse outcome.