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A 12-year-old boy with repaired right Bochdalek hernia and hypoplastic lung. a Chest radiograph shows a small, lucent right lung and asymmetric increased pulmonary vascularity to the left lung. b Contrast-enhanced CT scan with mediastinal windows shows
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Congenital diaphragmatic hernias are complex and life-threatening lesions that are not just anatomic defects of the diaphragm, but represent a complex set of physiologic derangements of the lung, the pulmonary vasculature, and related structures. Imaging plays an increasingly important role in the care of these infants. Prenatal sonography and MRI...
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Background
Bochdalek hernia (BH) of congenital diaphragm hernia is infrequently seen in adults. Strangulation of the diaphragm hernia has been recognized as a severe complication. Among several factors, pregnancy is an important cause of diaphragm hernia’s deterioration. However, nausea, vomiting, and upper abdominal pain are often considered non-s...
Survival of patients with congenital diaphragmatic hernia (CDH) depends both on non-modifiable congenital conditions and on modifiable pre and postnatal management. ECMO improves survival up to 80% in neonates with CDH in the best ECMO centers worldwide. The first Neonatal ECMO Program in Chile was started in our University in 2003. Our objective i...
Background: Defining risk factors for long-term comorbidities in patients after neonatal repair of congenital diaphragmatic hernia (CDH) is an important cornerstone of the implementation of targeted longitudinal follow-up programs.
Methods: This study systematically assessed serial chest radiographs of 89 patients with left-sided CDH throughout a...
Anterior diaphragmatic hernias through the foramen of Morgagni are rare accounting for about 3% of surgically treated diaphragmatic hernias. Current management of Morgagni type diaphragmatic hernias involves surgical intervention, which is traditionally done by open laparotomy, laparoscopy or thoracoscopic approach. Here we present the case of a tw...
Resumen Introducción: La Hernia Diafragmática Congénita (HDC) corresponde a una malformación del diafragma por la cual los órganos abdomi-nales protruyen hacia la cavidad torácica durante el desarrollo intrauterino. Los recién nacidos afectados presentan grados variables de insuficiencia respiratoria e hipertensión pulmonar, asociándose a una alta...
Citations
... En RM clasifica según el grado de ascenso y la posición del estómago (21) (ver Figura 6). En algunos estudios la presencia de estómago retrocardiaco (grado 3) se asocia a mal pronóstico (22). Además, el grado de ascenso se relaciona con mayor morbilidad gastrointestinal, respiratoria y neurológica. ...
La Hernia Diafragmática Congénita es una malformación mayor relativamente frecuente con un pronóstico potencialmente mortal. Incluye no sólo la alteración del desarrollo del diafragma, sino también de los pulmones, en especial de su vasculatura y de otras estructuras relacionadas. Las imágenes son cruciales en el diagnóstico, evaluación pre y post quirúrgica y control seriado. En la etapa prenatal la ecografía tiene un rol fundamental en el diagnóstico precoz, la resonancia magnética fetal permiten evaluar el grado de hipoplasia pulmonar y la presencia de anomalías asociadas para predecir la sobrevida y el eventual requerimiento de estrategias de mayor complejidad en el periodo perinatal cómo la oxigenación por membrana extracorpórea (ECMO). En la etapa postnatal la radiografía portátil y la ecografía son fundamentales en la evaluación seriada de los niños, ya sean sometido a terapias de soporte como ventilación mecánica invasiva, ECMO o a cirugía. El conocer la fisiopatología y las manifestaciones radiológicas de cada una de estas fases es fundamental para permitir una adecuada evaluación, optimizar el tratamiento y finalmente mejorar la sobrevida de estos niños.
... Prenatal diagnosis of CDH is based on the visualization of abdominal organs in the fetal thoracic cavity [7]. CDH may be accompanied by polyhydramnios or rarely hydrops. ...
Congenital diaphragmatic hernia (CDH) is one of the most common major congenital anomalies. In utero visceral organ herniation into the thoracic cavity can result in lung hypoplasia and pulmonary hypertension may ensue. Post-natal mortality rates in isolated CDH remain high reaching up to 80% in severe cases. Several prenatal predictors of morbidity and mortality have been proposed. Reliable predictive markers can aid clinicians in providing effective family counselling, prediction of survival, and propose therapeutic options. Decreased total fetal lung volume (TFLV) via magnetic resonance imaging (MRI) has been reported to be significantly associated with mortality. We report on 2 cases of CDH, with fetal MRI performed at third trimester, focusing on the TFLV and observed-to-expected TFLV. We would like to highlight the importance of predictive mortality calculator which provides statistical data for healthcare providers in counselling families and aids in risk-stratification.
... Most of the defects created by hernia are incidental, asymptomatic posterolateral diaphragmatic defects. [30,31] Mullins et al. [32] conducted the most comprehensive study on Bochdalek hernias. These researchers retrospectively reviewed reports of 13,138 abdominal CT scans and found that this finding was reported in 22 patients, representing a prevalence of 0.17%. ...
INTRODUCTION: This study aimed to describe the radiological features of omental hernias originating from the diaphragm and their localization on the diaphragm, examine their relationship with the thoracic and abdominal organs, and present guiding data to clinicians in operational planning.
MATERIALS AND METHODS: This study was obtained as a result of retrospective scanning of the images of 824 patients aged 18–65 who applied for thorax and/or upper abdomen computerized tomography (CT). The patients’ thorax and upper abdomen regions were examined in detail and divided into two groups of individuals with and without hernias. Hernia types, content, localization, and effect types of patients with hernia were recorded and analyzed separately.
RESULTS: Diaphragmatic hernia was detected in 197 (23.9%) of 824 patients. While 50.8% of these patients were female, 49.2% were male. Of the patients diagnosed with diaphragmatic hernia, 49.2% (n = 97) had Morgagni hernia, 30.5% (n = 60) had Bochdalek hernia, and 17.8% had hiatal hernia. While Morgagni hernia had anterior localization in 82.5%, Bochdalek hernia was generally localized on the left side (75.8%), and hiatal hernias were sliding type with a rate of 84.2%. The highest effect
was observed in Bochdalek hernias (71.1%). Omental tissue (59.4%) was observed most frequently in Morgagni hernias, while stomach content (91.9%) was found to be the highest in hiatal hernias (P < 0.05).
DISCUSSION–CONCLUSION: Diaphragmatic omental hernias are rare. The rarity, as well as the uncertain and nonspecific presentations, contributes to the retard in diagnosis. Commonly, the presentation in the adult age group is that of recurrent chest infection and rarely with gastroesophageal reflux and esophagitis. Physicians caring for these patients should be aware of this, and a high index of suspicion is recommended to obviate delay in diagnosis with its associated morbidity. We think the radiological features of diaphragmatic hernias should be detailed in determining and applying the optimal treatment approach. In addition, contrary to what was thought, we found that the prevalence of diaphragmatic hernia in our population is higher than that reported in the literature.
... La confirmación diagnóstica debe realizarse con un estudio radiológico esófago-gastro-duodenal con contraste. La realización de tomografía computada (TC) habitualmente debe reservarse para casos donde existan dudas diagnósticas o para precisar la posición anatómica 3 . ...
... The physical examination can reveal displaced heart sounds, an excavated abdomen, and bowel sounds in the chest. On imaging, we saw a hemithorax filled with multiple air locules with well-defined walls, which reflects bowel loops and abdomen with a paucity of gas, which are well-characterized findings of the disease [14]. In our case, the disease was quickly identified by accurate interpretation of radiographs, and management was appropriately started. ...
Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly that leads to herniation of abdominal viscera to the chest, which presents with respiratory distress shortly after birth. Spleen herniation is a rare finding, and kidney herniation is even more exceedingly rare. We hereby report a case of a neonate that developed severe respiratory distress secondary to CDH. After confirming the diagnosis with chest and abdominal X-ray and initial stabilization, the patient underwent laparotomy, which revealed a large diaphragmatic defect with herniation of the ileum, colon, spleen, and left kidney. Contents were reduced to the abdomen, and the defect was repaired. The patient had a complete recovery with no complications. After reviewing the literature, we noticed the paucity of data in the Middle East region regarding the disease burden and the increased rate of complications with delayed diagnosis. Therefore, we believe that this case, which was presented in the United Arab Emirates with kidney and spleen herniation and received prompt management, is a valuable addition to the literature.
... Pri novorojenčkih pomislimo na CDH ob akutni dihalni stiski, ciano zi in pridruženi sodčasti obliki prsnega koša, skafoidni (tj. ploski, ugreznjeni) obliki trebuha, oslabljenih dihalnih zvokih na strani kile, spremenjenih avskultatornih mestih srčnih tonov ali avskultaciji peristaltike v prsnem košu (1,28,29). CDH potrdimo z rentgenskim slikanjem, pri katerem se CDH kaže z opacificiranim prsnim košem na stra ni kile in kontralateralnim premikom medpljučja. če je v prsnem košu priso tno črevo, se bo kmalu po rojstvu napol nilo z zrakom in bo vidno na rentgenski sliki kot številne, z zrakom napolnjene vijuge (Slika 5) (29,30). ...
... CDH potrdimo z rentgenskim slikanjem, pri katerem se CDH kaže z opacificiranim prsnim košem na stra ni kile in kontralateralnim premikom medpljučja. če je v prsnem košu priso tno črevo, se bo kmalu po rojstvu napol nilo z zrakom in bo vidno na rentgenski sliki kot številne, z zrakom napolnjene vijuge (Slika 5) (29,30). Dodatno lahko pri diagnosticiranju prirojene diafra gmalne kile pomagata umeščenost in potek nazogastrične sonde (NGS) in žilnih katetrov. ...
... NGS je v delu požiral nika premaknjena kontralateralno od diafragmalne kile, prisotnost končne ga dela NGS v prsnem košu pa kaže, da skozi odprtino trebušne prepone pre haja želodec. Prisotnost jeter v prsnem košu lahko spremeni tudi položaj umbi likalnega venskega katetra, medtem ko položaj umbilikalnega arterijskega katetra običajno ostane nespreme njen (29). Spremembe na rentgenskem posnetku, vključno s prezračenostjo prizadetega pljučnega krila in kontra lateralnega pljučnega krila, stopnjo pomika medpljučja ter vsebino diafra gmalne kile, nimajo napovedne vred nosti za nadaljnji klinični potek bolezni (31). ...
Prirojena diafragmalna kila je razmeroma pogosta nepravilnost v razvoju trebušne prepone, ki jo v več kot polovici primerov prepoznamo že pred rojstvom. Diagnozo v večini primerov postavimo ob rutinskem ultrazvočnem pregledu v drugem trimesečju nosečnosti, ko ugotovimo prisotnost trebušnih organov v prsnem košu. Za postavitev diagnoze zadošča ultrazvočna (UZ) preiskava, za natančnejšo oceno obsega kile, vsebine kilne vreče in prostornine posameznih pljučnih kril, ki so napovedni kazalniki poteka bolezni, pa je v pomoč magnetnoresonančno slikanje (MRI). Preostali delež neodkritih diafragmalnih kil običajno odkrijemo zgodaj po otrokovem rojstvu z rentgenskim slikanjem zaradi dihalne stiske. Majhen delež kil, ki ne povzročajo kliničnih simptomov ali znakov, odkrijemo kasneje v življenju, pogosto naključno. Pogoste ponovitve diafragmalnih kil po operaciji in številne spremljajoče bolezni zahtevajo skrbno in natančno dolgoročno spremljanje bolnikov.
... Pri novorojenčkih pomislimo na CDH ob akutni dihalni stiski, ciano zi in pridruženi sodčasti obliki prsnega koša, skafoidni (tj. ploski, ugreznjeni) obliki trebuha, oslabljenih dihalnih zvokih na strani kile, spremenjenih avskultatornih mestih srčnih tonov ali avskultaciji peristaltike v prsnem košu (1,28,29). CDH potrdimo z rentgenskim slikanjem, pri katerem se CDH kaže z opacificiranim prsnim košem na stra ni kile in kontralateralnim premikom medpljučja. če je v prsnem košu priso tno črevo, se bo kmalu po rojstvu napol nilo z zrakom in bo vidno na rentgenski sliki kot številne, z zrakom napolnjene vijuge (Slika 5) (29,30). ...
... CDH potrdimo z rentgenskim slikanjem, pri katerem se CDH kaže z opacificiranim prsnim košem na stra ni kile in kontralateralnim premikom medpljučja. če je v prsnem košu priso tno črevo, se bo kmalu po rojstvu napol nilo z zrakom in bo vidno na rentgenski sliki kot številne, z zrakom napolnjene vijuge (Slika 5) (29,30). Dodatno lahko pri diagnosticiranju prirojene diafra gmalne kile pomagata umeščenost in potek nazogastrične sonde (NGS) in žilnih katetrov. ...
... NGS je v delu požiral nika premaknjena kontralateralno od diafragmalne kile, prisotnost končne ga dela NGS v prsnem košu pa kaže, da skozi odprtino trebušne prepone pre haja želodec. Prisotnost jeter v prsnem košu lahko spremeni tudi položaj umbi likalnega venskega katetra, medtem ko položaj umbilikalnega arterijskega katetra običajno ostane nespreme njen (29). Spremembe na rentgenskem posnetku, vključno s prezračenostjo prizadetega pljučnega krila in kontra lateralnega pljučnega krila, stopnjo pomika medpljučja ter vsebino diafra gmalne kile, nimajo napovedne vred nosti za nadaljnji klinični potek bolezni (31). ...
... Unfortunately, chromosomal study was not performed. There are indirect sonographic findings that should provide clues to search for CDH: polyhydramnios, cardio-mediastinal shift, cardiac axis abnormality and absence of the normal stomach bubble [10]. Generally, the diagnosis of CDH can be certain from the true transverse plane study with following findings: absent bowel loops in the abdomen, intrathoracic herniation of the liver (presents in up to 85% of cases and with a worse prognosis but it is difficult to be diagnosed due to the same echogenicity of liver with its neighbouring lung tissue), peristaltic bowel movements in the chest and decreased abdominal circumference due to upshifting of organs to the thorax [11]. ...
... Moreover, the gallbladder is displaced to the left. In the right-sided CDH, the sonographic clues are described as following: the umbilical Furthermore, the left hepatic lobe appears as an echogenic space between the left heart border and stomach [10]. In addition to the mentioned anatomical displacements, the degree of pulmonary hypoplasia can be calculated and correlates with the observed-to-expected lung-to-head ratio (O/E LHR) with sonography or more accurately with MRI [12,13]. ...
Bilateral Congenital Diaphragmatic Hernia (CDH) is a birth defect with a rare occurrence. The neonates who are born with this defect are not able to survive to undergo the treatment. In this case report, we provide the details of anatomical abnormalities and changes in organ development observed in a male neonate with a huge diaphragmatic defect with three liver lobes which was undiagnosed till after birth.
... The used sequences include Fast Imaging Employing Steady State Acquisition (FIESTA, FOW de 450/500 mm, TR of 5,2 ms, TE of 2,4 ms), Single Shot Fast Spin Echo (SSFSE, FOW de 450/500 mm, TR of 534.4 ms, TE of 160.2 ms), Diffusion Weighted Image (DWI, FOW de 450/500 mm, TR of 6.2 ms, TE of 3.1ms) and Liver Acquisition with Volume Acceleration (LAVA, FOW de 450/500 mm, TR of 6.2 ms, TE of 3.1ms). Slice thickness was between 4 and 6 mm (14). ...
Aims: We searched for correlations between ultrasound findings in pregnancies with congenital diaphragmatic hernia (CDH) and magnetic resonance imaging (MRI) follow-up examinations; MRI was used to confirm and complete the investigation in these difficult cases. In some of them, new elements that ultrasound was not able to fully describe have been also brought. We were especially interested when MRI was superior to ultrasound. Material and methods: This is a retrospective study of 12 pregnancies with congenital diaphragmatic hernia that were diagnosed in two major university clinics of Bucharest, Romania. Ultrasounds and MRI examinations were performed to evaluate pulmonary hypoplasia and correctly asses the herniated organs. We used standard international protocols and guidelines for calculating different parameters. All patients signed an informed consent before being enrolled in the study. Results: We described the herniated organs, dimensions of the hernia and the remaining lung capacity, so that we could correctly evaluate the prognosis. We have also used the lung to head ratio (LHR) in an attempt to better determine the degree of lung hypoplasia. Conclusion: High quality ultrasound followed by an MRI examination helped correctly assess the prognostic, treatment possibilities and total affected lung volume. It not only confirmed the diagnosis, but also offered new information that ultrasound was not able to provide.
... This comprehensive review explains the genetic contributions to CDH are highly heterogeneous and suggests CDH genes are often transcription factors, genes involved in cell migration or the components of extracellular matrix. In cases of multiple abnormalities, malformations may occur in all major organ systems Pober, 2007;Taylor et al., 2009). Since the etiology of CDH varies and is difficult to establish, with this review, we would like to draw attention to the prenatal presentation of multiple congenital anomalies syndromes, resulting from defects in the synthesis of glycosylphosphatidylinositol (GPI) anchors, as a possible cause, and to be considered in a prenatal assessment of fetuses with DH and Fryns-like phenotype. ...
Fryns syndrome is an autosomal recessive multiple congenital anomaly syndrome, with diaphragmatic defects and secondary lung hypoplasia as cardinal features. Despite it was reported first in 1979, its exact etiology has not been established to date. With this review, we would like to draw attention to the prenatal presentation of multiple congenital anomalies syndromes, resulting from defects in the synthesis of glycosylphosphatidylinositol anchors, to be considered in a prenatal assessment of fetuses with DH and Fryns-like phenotype.
