An Infant with Congenital Diaphragmatic Eventration with Dextrocardia: A Case Report.

Diaphragmatic eventration is a rare condition, and its association with dextrocardia is even a rarer clinical entity. Patients are usually asymptomatic, but the typical features include rapid breathing and recurrent respiratory infections. Here we present a rare case of a seven months old infant, who presented with cough, noisy breathing and chest retraction. The patient was diagnosed to have dextrocardia with diaphragmatic eventration with pneumonia by chest imaging and was treated in coordination with the medical team for underlying pneumonia. Afterwards, plication of the diaphragm was done through the trans-abdominal approach and the symptoms gradually improved postoperatively. For dextrocardia, since there were no structural abnormalities, the patient was kept in regular follow-up in the pediatric cardiology unit. Though most patients are asymptomatic, diaphragmatic eventration increases the risk of recurrent chest infection and hampers the quality of life of the patient, so timely diagnosis and intervention will greatly improve their quality of life. Keywords: dextrocardia; diaphragm; diaphragmatic eventration.

Parvovirus B19 Intrauterine Infection and Eventration of the Diaphragm.

Parvovirus B19 infection in pregnancy may have a poor outcome for the fetus. Ocular anomalies, brain damage with hydrocephalus and central nervous system (CNS) scarring, cleft lip and hypospadias, as well myocarditis and congenital heart disease have been reported. We present a case of a preterm female neonate born with ascites, hydrothorax and congenital diaphragmatic eventration (CDE), with a prenatal diagnosis of congenital diaphragmatic hernia (CDH). The neonate was born prematurely at 32 weeks gestation with caesarean section due to a previous caesarean delivery. She was immediately intubated in the delivery room, transferred in the Neonatal Intensive Care Unit (NICU) and supported with high frequency oscillatory ventilation (HFOV). The diagnosis of CDH was sonographically estimated from the 20th week of gestation and surgical correction was decided. During surgery CDE was diagnosed instead of CDH and despite postoperatively care the neonate developed disseminated intravascular coagulation and finally died in the 40th hour of life. Along with the identification of parvovirus B19 in the pleural fluid by PCR, the biopsy of the diaphragm revealed connective tissue, full of vasculature and absence muscle tissue. Although only cytomegalovirus, rubella, and toxoplasmosis were considered to be associated with CDE, parvovirus B19 might also be related to this congenital diaphragmatic malformation. In CDE, the function of the lungs can be compromised as a consequence of the compression applied by the abdominal organs. The neonatologists should include this condition in their differential diagnosis for a more direct and effective management.

Diaphragmatic eventration as a first sign of Scimitar syndrome.

Diaphragmatic eventration is an anomaly of the diaphragm. In Scimitar syndrome, a curved-shaped anomalous pulmonary venous drainage is seen. Association between these conditions is rare. We present a newborn with diaphragmatic eventration, whose diagnosis of Scimitar syndrome was made after surgical repair. Scimitar syndrome is a congenital disorder often associated with other heart and lungs anomalies. Diagnosis can be fortuitous but with important prognostic features.

Three-dimensional reconstruction model in the diagnosis of Morgagni's hernia.

Morgagni's hernia (MH) can be diagnosed by different utilities, but all these methods are not always 100% accurate. Three-dimensional (3D) reconstruction model could be helpful in better understanding the important anatomical structures. We report a case of MH who was once misdiagnosed as diaphragmatic eventration at the other institution and we offered laparoscopic repair according to the 3D reconstruction model. Our case highlights that 3D reconstruction model could be a useful supplementary tool in the diagnosis and preoperative assessment for patients with MH especially when it is confused in diagnosis in clinical practice.

Congenital diaphragmatic eventration and hernia sac compared to CDH with true defects: a retrospective cohort study.

Congenital diaphragmatic eventration (CDE) and congenital diaphragmatic hernia (CDH) with or without hernia sac are three different types of congenital diaphragmatic malformations, which this study evaluates. All surgically treated patients with CDE or Bochdalek type CDH between 2000 and 2016 were included in this retrospective analysis. Demographics, CDH-characteristics, treatment, and clinical outcome were evaluated. In total, 200 patients were included. Patients with an eventration or hernia sac had no significant differences and were compared as patients without a true defect to patients with a true defect. The 1-year survival of patients with a true defect was significantly lower than patients with no true defect (76% versus 97%, p = 0.001). CDH with no true defect had significantly better short-term outcomes than CDH with true defect requiring patch repair. However, at 30 days, they more often required oxygen supplementation (46% versus 26%, p = 0.03) and had a higher recurrence rate (8% versus 0%, p = 0.006) (three eventration and two hernia sac patients). Conclusion: Patients without a true defect seem to have a more similar clinical outcome than CDH patients with a true defect, with a better survival. However, the recurrence rate and duration of oxygen supplementation at 30 days are higher than CDH patients with a true defect.What is Known:• Congenital diaphragmatic hernia with or without hernia sac and congenital diaphragmatic eventration (incomplete muscularization) are often treated similarly.• Patients with hernia sac and eventration are thought to have a relatively good outcome, but exact numbers are not described.What is New:• Congenital diaphragmatic eventration and patients with hernia sac seem to have a more similar clinical outcome than Bochdalek type CDH with a true defect.• Patients without a true defect (eventration or hernia sac) have a high recurrence rate.

Congenital Cystic Diaphragm with Diaphragmatic Eventration in a Fetus: A Case Presentation.

Introduction: Congenital diaphragmatic eventration (CDE) is defined as the abnormal elevation of the diaphragm, due to incomplete muscularization of the diaphragm with a thin membranous sheet replacing normal diaphragmatic muscle. Case report: We report a prenatal case with a diaphragmatic mesothelial cyst combined with CDE. Conclusion: A large cystic mass between the thoracic wall and the liver in early pregnancy is highly suggestive of cystic diaphragm.

Bilateral congenital eventration of diaphragm: keep in mind, the other side.

Eventration of diaphragm is an uncommon disorder in which diaphragmatic muscle is replaced by fibroelastic tissue, either partially or completely. Bilateral eventration is even rarer. We present a case of bilateral eventration of diaphragm in newborn with a fibroelastic sac on left side and diaphragmatic eventration with good muscular lips on right side. The right-sided diaphragmatic eventration was not evident initially, but manifested after surgical repair of the left-sided eventration.

Four-Dimensional CT of the Diaphragm in Children: Initial Experience.

Objective: To evaluate the technical feasibility of four-dimensional (4D) CT for the functional evaluation of the pediatric diaphragm. Materials and Methods: In 22 consecutive children (median age 3.5 months, age range 3 days-3 years), 4D CT was performed to assess diaphragm motion. Diaphragm abnormalities were qualitatively evaluated and diaphragm motion was quantitatively measured on 4D CT. Lung density changes between peak inspiration and expiration were measured in the basal lung parenchyma. The diaphragm motions and lung density changes measured on 4D CT were compared between various diaphragm conditions. In 11 of the 22 children, chest sonography was available for comparison. Results: Four-dimensional CT demonstrated normal diaphragm (n = 8), paralysis (n = 10), eventration (n = 3), and diffusely decreased motion (n = 1). Chest sonography demonstrated normal diaphragm (n = 2), paralysis (n = 6), eventration (n = 2), and right pleural effusion (n = 1). The sonographic findings were concordant with the 4D CT findings in 90.9% (10/11) of the patients. In diaphragm paralysis, the affected diaphragm motion was significantly decreased compared with the contralateral normal diaphragm motion (-1.1 ± 2.2 mm vs. 7.6 ± 3.8 mm, p = 0.005). The normal diaphragms showed significantly greater motion than the paralyzed diaphragms (4.5 ± 2.1 mm vs. -1.1 ± 2.2 mm, p < 0.0001), while the normal diaphragm motion was significantly smaller than the motion of the contralateral normal diaphragm in paralysis (4.5 ± 2.1 mm vs. 7.6 ± 3.8 mm, p = 0.01). Basal lung density change of the affected side was significantly smaller than that of the contralateral side in diaphragm paralysis (89 ± 73 Hounsfield units [HU] vs. 180 ± 71 HU, p = 0.03), while no significant differences were found between the normal diaphragms and the paralyzed diaphragms (136 ± 66 HU vs. 89 ± 73 HU, p = 0.1) or between the normal diaphragms and the contralateral normal diaphragms in paralysis (136 ± 66 HU vs. 180 ± 71 HU, p = 0.1). Conclusion: The functional evaluation of the pediatric diaphragm is feasible with 4D CT in select children.

Diaphragmatic eventration presenting as a recurrent diaphragmatic hernia.

Diaphragmatic eventration is an uncommon condition, usually discovered incidentally in asymptomatic patients. Even in symptomatic patients, the diagnosis can be challenging and should be considered among the differential diagnoses of diaphragmatic hernia. The correct diagnosis can often only be made in surgery. We describe the case of a 31-year-old patient with diaphragmatic eventration that was misdiagnosed as a recurrent congenital diaphragmatic hernia and review the corresponding literature.

Congenital diaphragmatic eventration complicated with neonatal gastric perforation.

Congenital diaphragm eventration is a rare and usually asymptomatic developmental defect. Neonatal gastric perforation is also a rare but lifethreatening condition. In our knowledge, the association of these two pathologies has been, exceptionally reported. We report a case who illustrates clinical and radiological features of this possible co-morbidity. A full-term male neonate was born from uneventful pregnancy and delivery. The antenatal scan was reported as normal. At birth, clinical exam was normal, no special resuscitation was necessary. The newborn was examined and admitted the 4th day of life for fever, tachypnea, cyanosis, hemodynamic shock and refusing feeds. Clinical examination suggested peritonitis. Chest radiography and ultrasonography suggested congenital hernia. A laparotomy was performed after a brief resuscitation and confirmed the presence of diaphragm eventration with gastric perforation. Suturing of gastric perforation with a diaphragmatic plication was performed with favorable evolution.

Is polysomnographic examination necessary for subjects with diaphragm pathologies?

OBJECTIVES: While respiratory distress is accepted as the only indication for diaphragmatic plication surgery, sleep disorders have been underestimated. In this study, we aimed to detect the sleep disorders that accompany diaphragm pathologies. Specifically, the association of obstructive sleep apnea syndrome with diaphragm eventration and diaphragm paralysis was evaluated. METHODS: This study was performed in Süreyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital between 2014-2016. All patients had symptoms of obstructive sleep apnea (snoring and/or cessation of breath during sleep and/or daytime sleepiness) and underwent diaphragmatic plication via video-assisted mini-thoracotomy. Additionally, all patients underwent pre- and postoperative full-night polysomnography. Pre- and postoperative clinical findings, polysomnography results, Epworth sleepiness scale scores and pulmonary function test results were compared. RESULTS: Twelve patients (7 males) with a mean age of 48 (range, 27-60) years and a mean body mass index of 25 (range, 20-30) kg/m2 were included in the study. Preoperative polysomnography showed obstructive sleep apnea syndrome in 9 of the 12 patients (75%), while 3 of the patients (25%) were regarded as normal. Postoperatively, patient complaints, apnea hypopnea indices, Epworth sleepiness scale scores and pulmonary function test results all demonstrated remarkable improvement. CONCLUSION: All patients suffering from diaphragm pathologies with symptoms should undergo polysomnography, and patients diagnosed with obstructive sleep apnea syndrome should be operated on. In this way, long-term comorbidities of sleep disorders may be prevented.

Is polysomnographic examination necessary for subjects with diaphragm pathologies?

OBJECTIVES: While respiratory distress is accepted as the only indication for diaphragmatic plication surgery, sleep disorders have been underestimated. In this study, we aimed to detect the sleep disorders that accompany diaphragm pathologies. Specifically, the association of obstructive sleep apnea syndrome with diaphragm eventration and diaphragm paralysis was evaluated. METHODS: This study was performed in Süreyyapasa Chest Diseases and Thoracic Surgery Training and Research Hospital between 2014-2016. All patients had symptoms of obstructive sleep apnea (snoring and/or cessation of breath during sleep and/or daytime sleepiness) and underwent diaphragmatic plication via video-assisted mini-thoracotomy. Additionally, all patients underwent pre- and postoperative full-night polysomnography. Pre- and postoperative clinical findings, polysomnography results, Epworth sleepiness scale scores and pulmonary function test results were compared. RESULTS: Twelve patients (7 males) with a mean age of 48 (range, 27-60) years and a mean body mass index of 25 (range, 20-30) kg/m2 were included in the study. Preoperative polysomnography showed obstructive sleep apnea syndrome in 9 of the 12 patients (75%), while 3 of the patients (25%) were regarded as normal. Postoperatively, patient complaints, apnea hypopnea indices, Epworth sleepiness scale scores and pulmonary function test results all demonstrated remarkable improvement. CONCLUSION: All patients suffering from diaphragm pathologies with symptoms should undergo polysomnography, and patients diagnosed with obstructive sleep apnea syndrome should be operated on. In this way, long-term comorbidities of sleep disorders may be prevented.

Abordaje fisioterapéutico en paciente con eventración compleja / Physiotherapeutic approach in patient with complex eventration

Los pacientes con hernias complejas, se caracterizan por presentar cambios sistémicos y locales. Las complicaciones respiratorias pueden ser mínimas o podrían ser evitadas con la utilización de un protocolo de atención de fisioterapia respiratoria. Se presenta el caso clínico de una paciente de 53 años con diagnóstico de eventración compleja, Obesidad Mórbida y Diabetes Mellitus tipo II. Recibe el tratamiento de fisioterapia respiratoria en las etapas pre y post operatorias, mediante la evaluación clínica kinésica se pudo comprobar la pronta mejoría, pudiendo lograr su máximo grado de dependencia funcional. Resulta interesante medir dicho avance, dado que existen escasas publicaciones sobre este tema.

Patients with complex hernias, are characterized by systemic and local changes. Respiratory complications can be minimal or could be avoided with the use of a protocol of physiotherapy care. Presented the clinical case of a 53 year old patient with diagnosis of complex eventration, morbid obesity and Diabetes Mellitus type II. Receiving physiotherapy treatment in stages pre and post operative, through evaluation clinical kinaesthetic failed to check the prompt improvement, and can achieve its maximum degree of functional dependence. It is interesting to measure such progress, given that there are little publishing on this topic.

Eventración diafragmática en pediatría: revisión de un caso y revisión de la literatura / Diaphagmatic eventration in children: a case report and review of the literature

Diaphragmatic eventration is an abnormal elevation of one of the hemidiaphragms, with deviation of the mediastinum to the contralateral side. It is usually asymptomatic. It can be either congenital or acquired. If it is asymptomatic, the patient should be kept under observation. The resolution must be surgical if the ascent of the diaphragm is pointed, if there is any symptom or recurrent pneumonia. This article describes the case of a patient with congenital diaphragmatic eventration. The difficulties the patient presented in the diagnosis are reviewed.

La eventración diafragmática es la elevación anormal de uno de los hemidiafragmas, con desviación del mediastino hacia el lado contralateral, generalmente asintomática. Se clasifica en congénita y adquirida, si es asintomática el paciente se debe mantener en observación, la resolución debe ser quirúrgica si el ascenso diafragmático es acentuado, presenta síntomas o neumonía recurrente. En este artículo se revisa el caso de un paciente con eventración diafragmática congénita, que presentó dificultades en el diagnóstico.

To Sink the Lifted: Selection, Technique, and Result of Laparoscopic Diaphragmatic Plication for Paralysis or Eventration.

Introduction Diaphragmatic eventration is a congenital defect of the muscular portion of a hemidiaphragm that eventually leads to hemidiaphragmatic elevation and dysfunction. The clinical diagnosis of diaphragmatic eventration or diaphragmatic paralysis may be indistinguishable and diaphragmatic plication is the treatment of choice for both conditions. Discussion We review the indications, patient selection, and surgical techniques for diaphragmatic plication. We explain our preferred technique and guide the reader step by step on our approach. Conclusion Minimally invasive diaphragm plication techniques are effective alternatives to open transthoracic plication and result in significant improvement in dyspnea and quality of life in adequately selected patients.

Diaphragmatic eventration in children.

BACKGROUND: Diaphragmatic eventration (ED) is a rare anomaly defined by a permanent elevation of a hemidiaphragm without defects. Clinical manifestations are diverse, ranging from asymptomatic to life-threatening respiratory distress. The aim of this study is to report our experience of management of eight children with ED over the past 15 years. METHODS: A retrospective study was conducted involving 8 infants and children with ED managed at the department of pediatric surgery of Tunis Children's Hospital. RESULTS: Five patients (2 males, 3 females) whose ages ranged from 5 month to 7 years (mean, 13 months) were operated on using diaphragmatic plication (3 right-sided and 2 left-sided plications) for symptomatic ED with a dramatic improvement in their respiratory status. Another infant (a 3-month-old boy) was admitted for respiratory distress that required mechanical ventilatory support. He died before operation because of sepsis. Two other asymptomatic patients with incidentally diagnosed ED were followed up for 5 years on average. Chest x-ray suggested eventration in all cases. Ultrasound was found to be a useful modality for diagnosis in 6 cases. There were no cases of associated malformation. CONCLUSION: Diaphragmatic eventration in children is usually congenital but may be acquired. Chest ultrasound is an important imaging modality for diagnosis. Diaphragmatic plication appears to be safe and effective. Surgical intervention is not recommended in asymptomatic patients.