Congenital pulmonary sequestration

Orpha code: 3161OMIM code:

Definition

Congenital pulmonary sequestration is a rare respiratory malformation characterized by a cystic or solid mass of nonfunctioning primitive segmental lung tissue that does not communicate with the tracheobronchial tree and has anomalous systemic blood supply. Intralobar pulmonary sequestration may be asymptomatic or may present with recurrent pulmonary infections, hemoptysis, chest pain, cough and is usually diagnosed in older children and adults. Extralobar pulmonary sequestration present with respiratory distress, cyanosis, difficulty feeding or infection, may be associated with other anomalies and is mostly diagnosed in neonates or infants.

Disease data
Classification

Malformation syndrome

Synonyms
Congenital bronchopulmonary sequestration
Wrodzona sekwestracja oskrzelowo-płucna
ORPHA code
3161
OMIM code
-
ICD10 code
Q33.2
ICD11 code
LA75.6

No additional description.

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