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Congenital Emphysema

Congenital Lobar Emphysema


Presentation

  • 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]
  • To the best of our knowledge, this type of presentation is not reported before.[jiaps.com]
  • Less severe CLE may not present until later in infancy or childhood.[ejca.eg.net]
  • 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]
  • These findings are consistent with the most typical presentations of CLE.[omicsonline.org]
Foreign Body Aspiration
  • Bronchoscopy in the diagnosis of CLE is controversial, although it should be used to exclude foreign body aspiration in suspected cases. Total lobectomy of the involved lobe is the standard treatment in patients with CLE and severe symptoms.[appliedradiology.com]
  • In older children, foreign-body aspiration with regional emphysema may mimic lobar emphysema. A particularly difficult problem in the differential diagnosis is primary atelectasis with compensatory overinflation of the good side.[healio.com]
Hypothermia
  • Standard precautions to avoid hypothermia were taken and premedication with iv atropine 0.03mg and fentanyl 0.006 mg was given. A surgeon was washed up and ready to perform an emergency thoracotomy if the need arose.[joacp.org]
  • Standard precautions to avoid hypothermia were taken and premedication with intravenous atropine 0.04 mg and fentanyl 7 μg was given. A surgeon was made to scrub in and be ready to perform an emergency thoracotomy if the need arose.[ejca.eg.net]
Falling
  • Plumb, a surgeon source of lasers error is that the incidence may require a bright red Cheap Enthusia 25mg Sioux Falls which imaging with a factual CT scanner cannot be done.[sinizastom.ru]
Disability
  • However, this affection can cause a severe respiratory distress with high level of mortality or result in serious morbidity and disability. The exact etiology of disease is not known.[jcnonweb.com]
Weakness
  • Patient 2 Newborn male aged 20 days, with no personal history of interest, attended the accident and emergency department after 24 h of presenting symptoms characterised by weakness, vomiting, fever, and test results compatible with urinary tract infection[analesdepediatria.org]
Stridor
  • Stridor. On the tenth day of life a deterioration of the general condition was detected, the respiratory failure appeared.[hoajonline.com]
Constipation
  • The clinical features of an 8-year-old boy with congenital emphysema, cryptorchidism, a penoscrotal web, deafness, constipation and mental retardation are described.[ncbi.nlm.nih.gov]
Strabismus
  • In mother: aggravated obstetric anamnesis, convergent strabismus, insolvent scar on the uterus. Ventriculomegaly in fetus. Birth weight 3564 g height -53 cm. Apgar score of 6-8 points.[hoajonline.com]
Cryptorchidism
  • The clinical features of an 8-year-old boy with congenital emphysema, cryptorchidism, a penoscrotal web, deafness, constipation and mental retardation are described.[ncbi.nlm.nih.gov]

Workup

  • 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]

Treatment

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

Prognosis

  • 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

  • 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]
  • Malacia or stenosis of the bronchial cartilage can be seen in patients and are thought to represent possible etiologies. Cardiovascular anomalies can be seen in about 15% of cases.[roentgenrayreader.blogspot.com]
  • 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]

Epidemiology

  • 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]
Sex distribution
Age distribution

Pathophysiology

  • 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

  • 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]
  • 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]
  • 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]
  • 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]

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