Presentation
We describe a patient who presented with a left lower lobe lung lesion suspicious for cancer with possible hilar involvement. Intraoperatively, we found a primary left phrenic nerve tumor, diaphragmatic eventration, and left lower lobe atelectasis. [ncbi.nlm.nih.gov]
Entire Body System
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Recurrent Respiratory Infections
True diaphragmatic eventration is a rare congenital defect that is most commonly asymptomatic, but can result in dyspnea and recurrent respiratory infections. [ncbi.nlm.nih.gov]
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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]
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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]
Gastrointestinal
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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]
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Recurrent Vomiting
We report a case of such an association in a 2-month-old boy who presented with tachypnea aggravated by feeding and recurrent vomiting. [ncbi.nlm.nih.gov]
Liver, Gall & Pancreas
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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
X-Ray
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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]
In adults, mostly it is asymptomatic, usually it involves left hemidiaphragm with right sided mediastinal shift [ 1 ]. [omicsonline.org]
[…] gastric and colonic herniation in left chest cavity with collapsed lung and mediastinal shift to the right. [archintsurg.org]
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]
X-ray abdomen in erect position showed raised left hemidiaphragm with a large air-fluid level in the left hypochondrium, paucity of distal gas and right mediastinal shift [Fig.1]. [casereports.in]
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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]
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Right Pleural Effusion
This is a report of a 10-day-old term female infant with right diaphragmatic eventration in whom the initial diagnosis was right pleural effusion with probable ipsilateral lung hypoplasia. [ncbi.nlm.nih.gov]
Serum
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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]
Prognosis
Prenatal differential diagnosis of eventration is critical because postnatal managing and prognosis of these conditions vary significantly. [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]
Etiology
In this study we discussed the etiology, diagnosis, treatment and postoperative courses of diaphragmatic eventration. [ncbi.nlm.nih.gov]
The etiology of diaphragm paralysis is usually defined based on the history and previous chest X-ray of the patients. [cardiothoracicsurgery.biomedcentral.com]
It was admitted that CDE results from inadequate development of the diaphragm muscle or absence of the phrenic nerves [1].However, the exact etiology of CDE remains unknown, although it may be associated with fetal rubella or cytomegalovirus infection [latunisiemedicale.com]
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]
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]
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]
Attaching the diaphragm patch to the thoracic wall, as was done in this study, may prevent the re-elevation of the diaphragm postoperatively. [academic.oup.com]