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Rare lung & airway condition

Congenital lung malformations in adults

Congenital lung malformations - sequestrations, bronchogenic cysts, CPAMs and bronchial atresia - sometimes surface first in adulthood through infection, bleeding or an incidental scan, and each has its own management logic.

Key points

The essentials at a glance.

What to look out for, how the diagnosis is confirmed, who looks after it, and the options that exist.

  • 01

    Pulmonary sequestration is lung tissue with its own systemic artery; recurrent infection or haemoptysis are typical presentations, and surgery is usually curative.

  • 02

    Bronchogenic cysts can compress airways or become infected; symptomatic cysts are excised.

  • 03

    CPAMs (congenital pulmonary airway malformations) found in adults carry infection risk and a small malignancy association - resection is often advised.

  • 04

    Bronchial atresia is frequently just observed once confirmed.

  • 05

    CT angiography defines anatomy - especially aberrant vessels - before any intervention.

Understanding it

What it is, and what happens next.

In plain English - what is going on, how specialists confirm it, and the routes to treatment in the UK.

Not every developmental variation of the lung announces itself in childhood: a sequestration can sit quietly until its first serious infection at 30, a bronchogenic cyst until it presses on an airway. In adults the questions are always the same - what exactly is it, has it caused trouble, could it, and does its removal cost less than its retention?

A thoracic MDT weighs these case by case: cross-sectional imaging maps the lesion and any anomalous blood supply, symptomatic lesions are generally resected (increasingly by keyhole surgery, with embolisation an option for some sequestrations), and truly incidental, low-risk findings such as bronchial atresia can be documented and left in peace. In many parts of the UK, routine NHS outpatient referrals for problems like this can take several months, whereas an initial appointment with a private consultant is typically available within around two weeks.

A quiet reminder

This guide is for information, not medical advice.

Your GP or specialist knows your history and can tell you which parts apply to you. If in doubt, speak to them.

Published 2026-09-27 · Next review 2027-09-27

Frequently asked

Common questions about Congenital lung malformations in adults.

Quick, plain-English answers to what people ask us most.

  • What are congenital lung malformations?

    Congenital lung malformations are structural abnormalities of the lung present from birth, resulting from abnormal lung development during pregnancy. Examples include congenital pulmonary airway malformation (CPAM), bronchopulmonary sequestration, and congenital lobar emphysema. Many are identified and treated in childhood, but some, particularly milder forms, may not be diagnosed until adulthood, sometimes found incidentally on imaging done for another reason.

  • What causes congenital lung malformations?

    These result from abnormal development of the lung tissue or its blood supply during pregnancy, though the exact underlying cause in most individual cases is not fully understood. They are not generally thought to be caused by anything the mother did during pregnancy, and most cases occur without any specific identifiable genetic or environmental cause. They are typically isolated findings rather than part of a broader genetic syndrome.

  • What symptoms can these malformations cause in adults?

    Symptoms, if present, can include recurrent chest infections (since the abnormal lung tissue often doesn't function normally and can be more prone to infection), breathlessness, or chest pain, though some malformations cause no symptoms at all and are found incidentally on imaging. The specific symptoms depend on the type, size, and location of the malformation. Some adults with a previously undiagnosed malformation are only identified after investigation for recurrent chest infections or other respiratory symptoms.

  • Are congenital lung malformations dangerous in adults?

    This depends on the specific type and size — some cause no significant problems and can simply be monitored, while others carry a risk of recurrent infection or, rarely, other complications, and some specific types have a small but recognised association with certain lung cancers over the long term, particularly if left untreated. A specialist respiratory or thoracic surgery assessment can clarify the specific risks relevant to your particular malformation. Many adults with these malformations, once appropriately assessed, are managed successfully with either surgery or careful monitoring.

  • How are congenital lung malformations in adults treated?

    Management depends on the specific type, size, symptoms, and any associated risks, ranging from monitoring alone for asymptomatic, low-risk malformations, to surgical removal for those causing recurrent infections, significant symptoms, or carrying a more significant long-term risk. A specialist thoracic surgery and respiratory medicine team will discuss the most appropriate approach based on your individual findings. Both monitoring and surgical approaches are well-established, appropriate options depending on the specific circumstances.

  • When should I see a specialist?

    See a doctor if you have recurrent chest infections localised to the same area, or a lung abnormality found incidentally on imaging that hasn't been specifically assessed. NHS respiratory or thoracic surgery referral can take several weeks; a private consultation is typically available within about 2 weeks for earlier specialist assessment.