Pulmonary sequestration
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2008-06-23T01:27:52Z
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{{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]]