We report a case of bilateral CLE in a 2-month-old male infant presented with acute respiratory distress. In this report, the presentation, diagnosis, and surgical management of this rare condition are discussed. [thoracicmedicine.org]

Respiratory distress is the commonest mode of presentation; most present with moderate dyspnea in the first days of life, which may associated with wheezing, grunting respiration and tachypnea. This may progress on to cyanosis within days. [prognosisapp.com]

Less severe CLE may not present until later in infancy or childhood. [ejca.eg.net]

These findings are consistent with the most typical presentations of CLE. [omicsonline.org]

CASE 39 Clinical Presentation A 6-month-old presents with focal wheezing and mild respiratory distress. [radiologykey.com]

• Grunting

  Respiratory distress is the commonest mode of presentation; most present with moderate dyspnea in the first days of life, which may associated with wheezing, grunting respiration and tachypnea. This may progress on to cyanosis within days. [prognosisapp.com]

  Other symptoms and signs include dyspnea, wheezing, grunting respiration, tachypnea and sometimes progressive cyanosis. Upper lobe of left lung is most commonly affected (41%), followed by right middle lobe (34%) and right upper lobe (21%). [ijccm.org]

  If hypoxemia ensues, the infant may become increasingly agitated, anxious and cyanosed with grunting and coughing. [joacp.org]

PIE is a radiographic and pathologic diagnosis (see image below and Workup ). The introduction of surfactant and newer ventilator modalities has decreased the incidence of PIE. [emedicine.medscape.com]

• Base Deficit

  They concluded that the number of lobes affected and base deficit at presentation were associated with a high mortality rate in their study. [11] A strong clinical suspicion is required to diagnose this rare anomaly which may mimic other causes of respiratory [lungindia.com]

[…] some cases are real emergencies and urgent surgical treatment would therefore be justified. [analesdepediatria.org]

Congenital lesion/malformation Treatments Bronchoscopy Copyright the authors 2016 [erj.ersjournals.com]

The pathogenesis is still controversial, and the usual treatment recommended is a lobectomy. [ncbi.nlm.nih.gov]

Alternative treatment Alternative treatments that may be helpful for congenital lobar emphysema are aimed at supporting and strengthening the patient's respiratory function. [medical-dictionary.thefreedictionary.com]

Prognosis - Emphysema- congenital lobar Surgery for congenital lobar emphysema has excellent results. Treatment - Emphysema- congenital lobar For infants with no, mild, or intermittent symptoms, no treatment is necessary. [checkorphan.org]

Prognosis Surgery for congenital lobar emphysema has excellent results. Prevention Congenital lobar emphysema cannot be prevented. Resources Organizations American Lung Association. 1740 Broadway, New York, NY 10019. (800) 586-4872. . [medical-dictionary.thefreedictionary.com]

Prognosis and survival with surgery is good (8-10). The infants in the three antenatal cases reported all survived after lobectomy (1 ,16 ). [fetalultrasound.com]

The prognosis depending of the precocity of management means that the clinicians must have a high index of suspicion for CLE in the context of respiratory distress in neonate period, and ask for imaging techniques. [jcnonweb.com]

Prognosis of congenital lobar pneumonia is excellent as reported in literature. 10 Conclusion Early diagnosis and treatment improves the prognosis of CLE. [jpma.org.pk]

Etiology CLE may occur secondary to a variety of causes. In approximately half of cases, no demonstrable cause can be found. Dec 21, 2015 Posted by in PEDIATRIC IMAGING Comments Off on 39 Congenital Lobar Emphysema [radiologykey.com]

Etiology Variable, the most common factors are: external compression of the bronchus by anomal blood vessels bronchogenic cysts mucosal valves insufficiently formed bronchial cartilage Clinical signs quickly developing respiratory distress syndrome after [atlases.muni.cz]

Lobar emphysema of infancy: diagnosis, treatment and etiology aspects. Ann Those Surg 1967; 4:374-375. 10. Brown TCK, Fisk GC. Anesthesia for thoracic surgery. In: Anesthesia for children, 2nd ed. [ijaweb.org]

The etiology is not known but speculation includes an intrinsic defect with lung tissue like abnormal or decreased bronchial cartilage or an external defect like compression from blood vessels or cysts. [openanesthesia.org]

The exact etiology of disease is not known. [jcnonweb.com]

Respir-Med. 2000 Aug; 94 Suppl C: S18-21 Miravitlles-M Alpha1-antitrypsin deficiency: epidemiology and prevalence. [malattierare.regione.veneto.it]

Epidemiology of Congenital Lobar Emphysema CLE is an uncommon congenital anomaly, occurring in 1/20,000-30,000 deliveries. Despite the prevalence of current ultrasonographic prenatal imaging, the majority of cases are diagnosed postnatally. [clinicaladvisor.com]

Epidemiology 2 145-148. 1388. Kramer SA, Bredael J, Croker BP, Paulson DF, Glen JF (1979). Pleasing non- urachal adenocarcinoma of the majority. J Urol 121 278-281. 1389. Krasna IH, Lee M, Sciorra L, Sponsors M, Smilow P (1985). [sinizastom.ru]

More investigation is needed Independent risk factors for PIE in preterm infants include higher oxygen requirements during resuscitation as well as greater need for surfactant and higher ventilatory pressures before confirmation of the diagnosis. [4] Epidemiology [emedicine.medscape.com]

Grasp the nuances of the pathophysiology of today’s major respiratory conditions—including pulmonary hypertension, COPD, asthma, environmental lung disease, sleep disorders, infections of the immunocompromised, neonatal breathing disorders, and drug-resistant [books.google.com]

Successful anesthetic management of CLE can be achieved by proper understanding of pathophysiology, good perioperative monitoring, and adopting novel management strategies. [ijccm.org]

In many cases, the underlying pathophysiology is unclear. [prognosisapp.com]

The pathophysiology is that of a “ball-valve” type of bronchial obstruction due to weakened or absent bronchial cartilage. [ispub.com]

Pathophysiology Pulmonary interstitial emphysema (PIE) is initiated when air ruptures from the alveolar air space and small airways into the perivascular tissue of the lung. [emedicine.medscape.com]

Prevention - Emphysema- congenital lobar Congenital lobar emphysema cannot be prevented. Diagnosis - Emphysema- congenital lobar Congenital lobar emphysema is usually identified within the first two weeks of the infant\'s life. [checkorphan.org]

The case was challenging, as it involved careful and planned anaesthetic management of lung separation as well as prevention of hyperventilation of the un-involved lung. Submitted February 24, 2017 at 11:29 am [ijbms.com]

Prevention Congenital lobar emphysema cannot be prevented. Resources Organizations American Lung Association. 1740 Broadway, New York, NY 10019. (800) 586-4872. . National Heart, Lung and Blood Institute. P.O. [medical-dictionary.thefreedictionary.com]

Strategies to prevent hyperinflation and anaesthetic considerations of various techniques adopted for lung separation in infants have been reviewed. [ijaweb.org]

Ideally, maintenance of spontaneous ventilation is desirable prior to chest wall incision to prevent additional expansion of the emphysematous lobe. [cancertherapyadvisor.com]