Lung agenesis

Jump to: navigation, search

WikiDoc Resources for Lung agenesis

Articles

Most recent articles on Lung agenesis

Most cited articles on Lung agenesis

Review articles on Lung agenesis

Articles on Lung agenesis in N Eng J Med, Lancet, BMJ

Media

Powerpoint slides on Lung agenesis

Images of Lung agenesis

Photos of Lung agenesis

Podcasts & MP3s on Lung agenesis

Videos on Lung agenesis

Evidence Based Medicine

Cochrane Collaboration on Lung agenesis

Bandolier on Lung agenesis

TRIP on Lung agenesis

Clinical Trials

Ongoing Trials on Lung agenesis at Clinical Trials.gov

Trial results on Lung agenesis

Clinical Trials on Lung agenesis at Google

Guidelines / Policies / Govt

US National Guidelines Clearinghouse on Lung agenesis

NICE Guidance on Lung agenesis

NHS PRODIGY Guidance

FDA on Lung agenesis

CDC on Lung agenesis

Books

Books on Lung agenesis

News

Lung agenesis in the news

Be alerted to news on Lung agenesis

News trends on Lung agenesis

Commentary

Blogs on Lung agenesis

Definitions

Definitions of Lung agenesis

Patient Resources / Community

Patient resources on Lung agenesis

Discussion groups on Lung agenesis

Patient Handouts on Lung agenesis

Directions to Hospitals Treating Lung agenesis

Risk calculators and risk factors for Lung agenesis

Healthcare Provider Resources

Symptoms of Lung agenesis

Causes & Risk Factors for Lung agenesis

Diagnostic studies for Lung agenesis

Treatment of Lung agenesis

Continuing Medical Education (CME)

CME Programs on Lung agenesis

International

Lung agenesis en Espanol

Lung agenesis en Francais

Business

Lung agenesis in the Marketplace

Patents on Lung agenesis

Experimental / Informatics

List of terms related to Lung agenesis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Overview

Lung agenesis is defined as partial or complete failure of the lung to form with absence of the lung parenchyma, the bronchus and the pulmonary vessels.

Classification

The condition can be unilateral (compatible with life) or bilateral (fatal).

Pathophysiology

Embryogenesis

The trachea begins to develop at about 4 weeks of gestation as an outpouching from the foregut. Lung agenesis develops as a result of the bronchial analogue to develop into tow lung buds.

On the affected side, the pleural cavity is absent, and the healthy lung occupies the entire thorax. If a left lung develops, it is often trilobed.

Symptoms are due to due to tracheal compression and kinking of the trachea, shift of the mediastinal contents, posterior dislocation of the trachea as a result of a dislocated aortic arch and somtimes an associated truncus arteriosus.

Associated Conditions

Epidemiology and Demographics

Lung agenesis is extremely rare and is observed in 1:15,000 autopsies.

Gender

There is a slight female preponderance.

Natural History, Complications, Prognosis

Natural History

The natural history is variable.

Complications

There can be kinking of the trachea and compression of the trachea by the aorta.

Prognosis

Although 50% of patients may die before the age of 5, the remainder of the individuals may be asymptomatic throughout their lives. Bilateral lung agenesis is a lethal anomaly.

Diagnosis

Symptoms

Treatment

Symptomatic Patients

Symptoms are due to due to tracheal compression and kinking of the trachea, shift of the mediastinal contents, posterior dislocation of the trachea as a result of a dislocated aortic arch and somtimes an associated truncus arteriosus.

Indications for surgery include:

The surgical procedure varies and includes aortopexy and diaphragmatic translocation which improve mediastinal shift and reduce heart rotation. Relief of tracheal compression is critical as is reduction in hyperinflation of the remaining lung.

Asymptomatic Patients

Prophylactic surgery is not necessary.

References

  1. Krivchenya DU, Dubrovin AG, Krivchenya TD, Khursin VN, Lysak CV (2000). "Aplasia of the right lung in a 4-year-old child: surgical stabilization of the mediastinum by diaphragm translocation leading to complete recovery from respiratory distress syndrome". Journal of Pediatric Surgery. 35 (10): 1499–502. doi:10.1053/jpsu.2000.16424. PMID 11051161. Retrieved 2012-09-16. Unknown parameter |month= ignored (help)
  2. Skandalakis JE, Gray SW, Symbas P: The trachea and the lungs. In: Skandalakis JE, Gray SW, editors. Embryology for Surgeons. 2nd ed. Baltimore, MD: Williams and Wilkins; 1994. p 429-32.
  3. Dohlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: Airway obstruction and its release by aortopexy. J Pediatr Surg 1990;25:290-3.
  4. Dohlemann C, Mantel K, Schneider K, Guntner M, Kreuzer E, Hecker WC. Deviated trachea in hypoplasia and aplasia of the right lung: Airway obstruction and its release by aortopexy. J Pediatr Surg 1990;25:290-3.

Linked-in.jpg