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]

• Turkish

  […] bronchial lumen obstruction Onufry Torbus, Józef Dzielicki, Magdalena Jachimowicz, et al Case Rep Clin Pract Rev. 2002; 3(4): 260-263 [Pubmed] | [ PDF ] 3 Konjenital Lobar Amfizem Şevval Eren, Akın Eraslan Balcı, Refik Ülkü, Mehmet Nesimi Eren, Nihal Kılınç Turkish [jpgmonline.com]

  Turkish Journal of Thoracic and Cardiovascular Surgery. 2012; 20(3): 563-566 [Pubmed] 9 Radiology in paediatric non-traumatic thoracic emergencies Charlotte Lange Insights into Imaging. 2011; 2(5): 585 [Pubmed] | [DOI] 10 Anaesthetic management of a case [annals.in]

• Hyperresonance

  Left upper lobe - 41% Right middle lobe - 34% Right upper lobe - 21% Congenital lobar emphysema has 2 forms: Hypoalveolar (fewer than expected number of alveoli) Polyalveolar (greater than expected number of alveoli) The thorax on the involved side is hyperresonant [emedicine.medscape.com]

  Preoperative examination revealed a heart rate of 130 bpm, a respiratory rate of 76 breaths/min, presence of intercostal and sternal recession, and hyperresonant percusion on the right side. [journals.lww.com]

  Physical examination may reveal asymmetric chest expansion with focal hyperresonance and diminished breath sounds over the affected lobe. [joacp.org]

  There is hyperresonance to percussion over the affected lobe. Breath sounds are paradoxically decreased over the radiologically hyperaerated lobe, and this can be a clue to diagnosis. [healio.com]

• Hypertelorism

  The neonatologist who was consulted, noted other anomalies namely hypertelorism, short columella, hooknose, large philtrum, large upper lip with loose skin on dorsum of hands and back of neck. [jpgmonline.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]

Because of this disorder, the respiration was getting obstructed and did not respond to any line of treatment. [jpgmonline.com]

TREATMENT Lobectomy is the current method of treatment in most cases of congenital lobar emphysema.4,15"17 For many years it was believed that nonsurgical therapy was contraindicated on the grounds that surgery would improve pulmonary function by releasing [healio.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 of congenital lobar pneumonia is excellent as reported in literature. 10 Conclusion Early diagnosis and treatment improves the prognosis of CLE. [jpma.org.pk]

[…] evaluate the mediastinal vascular structures, and to rule out other abnormalities. shows above features in greater detail attenuation of vascular structures in affected lobe 4 may also show compressive atelectasis of neighboring lobes Treatment and prognosis [radiopaedia.org]

Prognosis Surgery for congenital lobar emphysema has excellent results. Prevention Congenital lobar emphysema cannot be prevented. [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]

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]

congenital,epidemiology,radiography,surgery,Treatment Outcome, How to cite this article: Karapurkar S A, Borkar J D, Birmole B J. [jpgmonline.com]

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. http://www.lungusa.org. 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]

[…] best way to prevent emphysema or stop it from getting worse. [medicalnewstoday.com]