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Diaphragmatic Eventration


Presentation

  • Here, we present a 12-month-old female infant presenting with a mediastinal and cervical mass, showing left diaphragmatic eventration.[ncbi.nlm.nih.gov]
  • Abstract We describe a patient who presented with a left lower lobe lung lesion suspicious for cancer with possible hilar involvement.[ncbi.nlm.nih.gov]
  • Our goal is to present imaging features and management of this entity together with a literature review.[ncbi.nlm.nih.gov]
  • METHOD/RESULT: A report of a case of diaphragmatic eventration in a four-month-old child is presented with a review of the relevant literature.[ncbi.nlm.nih.gov]
  • Abstract A case of right diaphragmatic eventration, associated with a lung sequestration, in a 7-month-old infant is presented. Failure to thrive was the initial symptom.[ncbi.nlm.nih.gov]
Arachnodactyly
  • Neonatal Marfan syndrome, the most severe presentation of Marfan syndrome phenotypes (MIM 154700), is characterised mainly by joint contractures, arachnodactyly, loose skin, crumpled ears, severe atrioventricular valve dysfunction and pulmonary emphysema[ncbi.nlm.nih.gov]
  • الصفحة 896 - Genetic linkage of the Marfan syndrome, ectopia lentis, and congenital contractural arachnodactyly to the fibrillin genes on chromosomes 15 and 5. N Engl J Med 1992;326:905-909. ‏[books.google.com]
Disability
  • Author information 1 Department of Pediatrics, Metropolitan Fuchu Medical Center for Severe Motor and Intellectual Disabilities, 2-9-2 Musashi-dai,Fuchu-shi, Tokyo 183-0042, Japan.[ncbi.nlm.nih.gov]
Streptococcal Infection
  • We report a child with antenatally documented right diaphragmatic eventration 4 weeks before birth who subsequently developed a beta-haemolytic streptococcal infection in the neonatal period.[ncbi.nlm.nih.gov]
Hiccup
  • Hiccups: A subtle sign in the clinical diagnosis of gastric volvulus and a review of the literature. Dig Dis Sci Nov 2008;53:3033-6. 6. Dogan NO, Aksel G, Demircan A, Keles A, Bildik F.[archintsurg.org]
Liver Dysfunction
  • Extravascular liquid accumulation was probably further aggravated by hypoproteinaemia secondary to liver dysfunction resulting from the venous congestion and cardiac failure.[ncbi.nlm.nih.gov]

Workup

Mediastinal Shift
  • A mediastinal shift to the contralateral side may cause significant compression of the affected chest contents, resulting in compromised pulmonary function, especially when both sides are involved.[ncbi.nlm.nih.gov]
  • […] gastric and colonic herniation in left chest cavity with collapsed lung and mediastinal shift to the right.[archintsurg.org]
  • A chest X-ray revealed pneumoperitoneum, raised left diaphragm and right-sided mediastinal shift (Fig. 1).[sajs.redbricklibrary.com]
  • Chest radiography was revealed total elevation of the right dome in 3 cases and of the left dome in 2 cases above the fourth intercostal space (figure1) and a mediastinal shift in 2 cases Figure 1 : Preoperative chest radiography shows elevation of the[latunisiemedicale.com]
  • The moment the pressure had been taken off by mediastinal shifting back to its normal position, the small right diaphragmatic defect became vulnerable to increased abdominal pressure generated as a result of relocation of herniated viscera into peritoneal[jpma.org.pk]
Unilateral Diaphragmatic Elevation
  • SUBJECTS AND METHODS: Between October 2007 to December 2013, 3498 children with congenital heart disease underwent cardiac surgery in our hospital, and 40 (1.14%) of them had unilateral diaphragmatic elevation on postoperative chest X-ray (mean elevation[ncbi.nlm.nih.gov]
Hypercapnia
  • It is characterized by a lack of response to habitual respiratory stimulants, especially hypercapnia. In this article, the management of this syndrome by diaphragmatic pacing is discussed.[ncbi.nlm.nih.gov]
  • The development of respiratory acidosis with hypercapnia and hypoxia must be avoided as this would trigger off the reactive pulmonary hypertension so one should err on the side of over ventilation rather than underventilation.[jpma.org.pk]

Treatment

  • OBJECTIVE: This study sought to summarize the diagnostic and treatment aspects of congenital diaphragmatic eventration (CDE) in children by retrospectively analyzing their medical records to identify and understand the complications of CDE, its treatment[ncbi.nlm.nih.gov]
  • The treatment initially should be supportive, including assisted ventilation if needed.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Thoracoscopic plication is feasible and safe, and we consider this approach as the gold standard for the treatment of CDE.[ncbi.nlm.nih.gov]
  • CONCLUSIONS: Treatment using VATS is a safe and effective alternative to conventional surgery. Functional improvement persists at long-term follow-up.[ncbi.nlm.nih.gov]
  • It is seldom symptomatic and often requires no treatment, but may be confused with a traumatic rupture of the diaphragm. We present a 51-year-old man with previously-undiagnosed congenital eventration, mimicking traumatic rupture of the diaphragm.[ncbi.nlm.nih.gov]

Prognosis

  • Prenatal differential diagnosis of eventration is critical because postnatal managing and prognosis of these conditions vary significantly.[ncbi.nlm.nih.gov]
  • Nakada PA index results, however, were statistically significant (93.07 /- 32.02 vs. 121.07 /- 27.08, P 0.05) In conclusion, Apgar scores, preductal PaCO2, oxygen index, and pH level can predict prognosis in infants with CDH and SDE.[ncbi.nlm.nih.gov]
  • This information is clinically important for management and counseling because the prognosis and treatment for CDH and congenital diaphragmatic eventration are different. Published by John Wiley & Sons, Ltd. Copyright (c) 2007 ISUOG.[ncbi.nlm.nih.gov]
  • After surgical treatment, the long-term prognosis is good. For severely symptomatic patients who were on the ventilator before surgery, diaphragmatic plication can help come off mechanical ventilator support. Updated: 11/2016 Author: Romeo C.[eapsa.org]
  • MRI is related to prognosis and can be used to support ultrasound in making decisions. MRI occasionally provides complementary morphological information.[ncbi.nlm.nih.gov]

Etiology

  • In this study we discussed the etiology, diagnosis, treatment and postoperative courses of diaphragmatic eventration.[ncbi.nlm.nih.gov]
  • Etiology of DE was phrenic nerve injury (LP, n 11; TP, n 1) and muscular deficiency (LP, n 2; TP, n 6). Mean age (LP, 18 months; TP, 25 months) and weight (LP, 8.0 kg; TP, 9.7 kg) at surgery were not significantly different.[ncbi.nlm.nih.gov]
  • The etiologic mechanism could be identified in 11 out of 12 patients (trauma, n 9; Charcot-Marie disease, n 1; calcified para-aortic nodes, n 1).[ncbi.nlm.nih.gov]
  • The exact etiology is unknown but a high proportion of cases have been found to have chroinosomal disorders such as Trisomy 18.13, or 15 3 . No chromosomal analysis could be done on ourcases.[jpma.org.pk]
  • The etiology, diagnosis, and management of this condition remains a controversial subject.[jdeonline.in]

Epidemiology

  • Clinical and epidemiological characteristics of infants with body wall complex with and without limb deficiency. Am J Med Genet. 1997;73:170–5. Google Scholar [5] Van Allen MI, Curry C, Gallagher L. Limb body wall complex: I. Pathogenesis.[degruyter.com]
Sex distribution
Age distribution

Pathophysiology

  • Pathophysiology: Eventration of the diaphragm causes problems in 4 ways: Inability to ventilate the ipsilateral lung Direct Compression of the ipsilateral lung Pneumonia due to chronic atelectasis Paradoxical motion of the diaphragm making ventilation[surgery4children.com]

Prevention

  • Surgical correction while on extracorporeal life support was unsuccessful due to attachments of the liver which prevented reduction into the abdominal cavity and our inability to distinguish the true defect from complete agencies of the right hemidiaphragm[ncbi.nlm.nih.gov]
  • Essential steps to prevent any rise in intrathoracic and intra-abdominal pressures along with care to minimize intragastric volume were taken.[ncbi.nlm.nih.gov]
  • Early diagnosis and prompt intervention prevents development of necrosis and perforation of stomach.[archintsurg.org]
  • This prevented his right lung from developing. The baby was later diagnosed with bowel, kidney and heart disease.[chop.edu]
  • A normal diaphragm is also responsible for prevention of abnormal displacement of abdominal viscera and gastric volvulus.[casereports.in]

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