Pulmonary sequestration 1619219 221099058 2008-06-23T01:27:52Z DOI bot 6652755 Citation maintenance. Initiated by [[User:Fconaway|Fconaway]]. You can [[WP:DOI|use this bot]] yourself! Please [[User:DOI_bot/bugs|report any bugs]]. {{Infobox_Disease | Name = {{PAGENAME}} | Image = Pulmonary-sequestration-001.jpg| Caption = | DiseasesDB = 32120 | ICD10 = {{ICD10|Q|33|2|q|30}} | ICD9 = {{ICD9|748.5}} | ICDO = | OMIM = | MedlinePlus = | eMedicineSubj = ped | eMedicineTopic = 2628 | eMedicine_mult = {{eMedicine2|radio|585}} | MeshID = D001998 | }} A '''pulmonary sequestration''', also known as a '''bronchopulmonary sequestration''' or a '''cystic lung lesion''', is a medical condition where a piece of tissue that develops into [[lung]] tissue is not attached to the pulmonary blood supply and does not communicate with the other lung tissue. Often it gets its blood supply from the [[thoracic]] [[aorta]]. ''Communication'' is a medical phrase indicating that it is not connected to the standard [[bronchial]] airways and that it performs no function in [[respiration (physiology)|respiration]]. This condition is normally detected in children and is generally held to be [[congenital]] in nature. The treatment for this is a [[segmentectomy]] via a [[thoracotomy]]. More and more, these lesions are diagnosed by [[prenatal]] [[ultrasound]]. ==Variations== There are two different kinds of pulmonary sequestrations, [[intralobar]] and [[extralobar]]. The generally accepted difference between these seems to whether or not the sequestration has its own [[pleura]], although some [[thoracic surgery|thoracic surgeons]] seem to prefer a definition that relates to the degree of [[Blood vessel|vascular]] connection for the sequestration. ==Symptoms== Symptoms can vary greatly, but they include a persistent dry cough. ==Diagnosis== Sequestrations can be identified in-utero via an abnormal artery on ultrasound. The [[gold standard (test)|gold standard]] for diagnosis is pulmonary angiography. But since it is a very invasive procedure, it is getting replaced by [[ct scan|CT Scan]] with a contrasting fluid, as the investigation of choice. Further studies are required for comparing sensitivity and specificity of angiograms versus ct scans in diagnosing pulmonary sequestration. ==Complications== Failure to have a pulmonary sequestration removed can lead to a number of complications. These include: * It can be fatal if you have [[hemorrhage]] of the blood vessels * It can cause cardiovascular problems due to the creation of a [[shunt]] where blood flows in a shortcut through the feed off the aorta. * It is necessary to prevent long-term infections. Things like [[tuberculosis]], [[aspergillosis]], bronchial carcinoid, brunchogenic squamous cell carcinoma. ==Treatments== Usually the sequestration is removed after birth via surgery. In most cases this surgery is safe and effective; the child will grow up to have normal lung function. In a few instances, fetuses with sequestrations develop problematic fluid collections in the chest cavity. In these situations a Harrison catheter shunt can be used to drain the chest fluid into the amniotic fluid. In rare instances where the fetus has a very large lesion, resuscitation after delivery can be dangerous. In these situations a specialized delivery for management of the airway compression can be planned called the [[EXIT procedure]]. ==Sources== *{{ cite journal | author=Truitt AK, Carr SR, Cassese J, Kurkchubasche AG, Tracy TF Jr, Luks FI. | title=Perinatal management of congenital cystic lung lesions in the age of minimally invasive surgery | journal=J Pediatr Surg | year=2006 | pages=41:893–896 }} *{{ cite journal | author=Savic B, Birtel FJ, Tholen W, Funke HD, Knoche R. | title=Lung seqestration: report of seven cases and review of 540 published cases | journal=Thorax | year=1979 | pages=34:96–101 }} *{{ cite journal | author=Fabre O, Porte H, Godart F, Rey C, Wurtz A. | title=Long-Term Cardiovascular Consequences of Undiagnosed Intralobar Pulmonary Sequestration | journal=Annals of Thoracic Surgery | year=1998 | pages=65;1144–6 }} *{{cite book | author=Ferguson | title=Gibbons surgery of the Chest | chapter=Congenital lesion of the lungs and emphysema | publisher=WB Saunders | year=1983 | id=ISBN Unknown |pages=668-709 |edition=4th ?}} *{{ cite journal | author=Rubin E, Garcia H, Horowitz M, Guerra J. | title=Fatal Massive Hemoptysia Secondary to Intralobar Sequestration | journal=Chest | year=1994 | pages=954–955 | doi=10.1378/chest.106.3.954 | volume=106 | pmid=8082388 }} *{{cite book | author=Sabiston D, Spencer F | title=Surgery of the Chest |pages=853-862 |edition=6th}} ==External links== * [http://www.emedicine.com/ped/topic2628.htm Emedicine on pulmonary sequestrations] * [http://fetus.ucsfmedicalcenter.org/pulmonary_sequestration/ UCSF Fetal Treatment Center: Pulmonary Sequestration] {{Congenital malformations and deformations of respiratory system}} [[Category:congenital disorders]] [[Category:Obstetrics]] [[Category:Pulmonology]] [[es:Secuestro broncopulmonar]] [[pl:Sekwestracja płuca]]