Congenital Lung Conditions
Surgery for lung conditions present from birth — in teenagers and adults

• Present from birth, often found later. Many congenital lung conditions only cause symptoms, or are discovered, in the teenage or adult years — frequently on a scan done for another reason.
• The main conditions treated in this group. Pulmonary sequestration, congenital pulmonary airway malformation (CPAM), congenital lobar emphysema and bronchial atresia. A bronchogenic cyst and airway narrowing are related but covered separately.
• Treatment is usually keyhole, and often curative. Where surgery is needed, the affected segment or lobe is removed robotically or by VATS, commonly in a single operation.
• Not everything needs surgery. Some small, symptom-free lesions are monitored. The decision is individual, made after the scan is reviewed.
• Adults and adolescents, with joined-up care. Dr Okiror is an adult thoracic and robotic surgeon with paediatric cardiothoracic training, working with Evelina London for younger and more complex patients.

A congenital lung condition is a difference in the way part of the lung, its airways or its blood vessels formed before birth. They are individually uncommon, and they cover a spectrum — from a small area of abnormal tissue that never causes trouble, to a lesion that leads to repeated infection or bleeding and needs to be removed. Increasingly they are picked up before birth or in early childhood, but a significant number are only recognised later, when a young adult has a scan for an unrelated reason, or when the same part of the lung keeps becoming infected.

The conditions most often dealt with in teenagers and adults are pulmonary sequestration, congenital pulmonary airway malformation (CPAM), congenital lobar emphysema and bronchial atresia. Two closely related conditions are covered on their own pages: a bronchogenic cyst, which sits in the central chest (the mediastinum), and a narrowing of the windpipe or airway. The sections below explain each of the main conditions in turn, when treatment is needed, and what an operation involves.

A pulmonary sequestration is a segment of lung tissue that formed separately and is not connected to the normal airways. Its defining feature is that it draws its blood supply from an abnormal artery, usually arising directly from the main artery in the chest (the aorta) rather than from the normal lung circulation. Because the tissue does not drain or ventilate normally, it is prone to becoming infected, and it can also be a source of coughing up blood.

There are two types. An intralobar sequestration sits within a normal lobe and most often presents in older children and adults with recurrent chest infection or coughing of blood. An extralobar sequestration has its own covering and is more often found earlier in life. Where a sequestration is causing symptoms, the usual treatment is to remove it, and the abnormal feeding artery must be carefully identified and controlled during the operation — a routine but important part of planning, which is why a good-quality CT scan is so useful before surgery.

A congenital pulmonary airway malformation (CPAM), once called a congenital cystic adenomatoid malformation (CCAM), is an area of abnormal, often cystic lung tissue that formed before birth. Many are now identified on antenatal scans and followed through childhood. Some never cause any difficulty; others lead to repeated infection in the same area, and a minority are removed because of infection, symptoms, or to confirm the diagnosis with certainty.

In a teenager or adult, a CPAM that is causing recurrent infection, or where the diagnosis needs to be established, is usually treated by removing the affected segment or lobe — most often by keyhole surgery. Where a CPAM has been monitored since childhood, the decision about whether and when to operate is made individually, weighing the pattern of symptoms against the operation itself.

Congenital lobar emphysema (also called congenital lobar overinflation) is an over-expansion of one lobe of the lung, caused by air being trapped within it. In infancy it can cause significant breathlessness and is treated early; milder forms are sometimes recognised later and may need removal of the affected lobe if they cause symptoms or recurrent infection.

Bronchial atresia is a short segment of airway that did not form a normal opening, so the lung beyond it is poorly aired and may trap mucus. It is frequently discovered by chance on a CT scan in an adult and often needs no treatment at all; where it causes recurrent infection, the affected segment can be removed. As with the other conditions on this page, the right course is decided after the scan and the symptoms are reviewed together — not on the diagnosis alone.

When treatment is needed, it almost always means removing the affected part of the lung, and in teenagers and adults this is usually done by keyhole surgery — either robotic or video-assisted (VATS) — through small incisions. Depending on the size and position of the lesion, this may be a segmentectomy (removing one anatomical segment and sparing the rest of the lobe) or removal of a whole lobe. For most people, a single operation deals with the problem definitively, with a short hospital stay and a quicker recovery than open surgery. Where a lesion is large, densely scarred from previous infection, or unusually placed, an open operation is occasionally the safer choice, and that is discussed openly beforehand.

Equally, not every congenital lung lesion needs an operation. A small, symptom-free lesion may simply be monitored, and the purpose of the first consultation is an honest assessment of whether treatment is needed at all. Dr Okiror reviews the CT scan personally and sets out the options — including doing nothing — so that any decision to operate is made for clear reasons.

Many congenital lung conditions first cause problems in the teenage and young-adult years, and where a young person is treated depends on their age. Adults from 18 are seen and treated privately at London Bridge Hospital and The Lister Hospital Chelsea. For 16- and 17-year-olds, NHS care is provided at Guy’s and St Thomas’, while private treatment for under-18s takes place at The Portland Hospital, HCA’s dedicated children’s hospital, arranged individually for each patient. Younger children, and more complex cases, are cared for jointly with the paediatric team at Evelina London. This is set out in full on the dedicated page for this age group.

Referrals are welcome from GPs, paediatric, respiratory and congenital-heart (ACHD) colleagues, including for young people transitioning from paediatric to adult services. A congenital lung lesion discovered incidentally on cross-sectional imaging, a sequestration presenting with recurrent infection or haemoptysis, or a CPAM under long-term surveillance are all appropriate to refer for an opinion on whether and when surgery is indicated. Where the picture overlaps with congenital heart disease, assessment is coordinated with the relevant ACHD team.

Cross-sectional imaging — particularly a contrast CT that defines any abnormal arterial supply — is the single most useful thing to send with a referral, as the diagnosis and the operative plan usually rest on it. A brief covering note and the imaging are enough to begin. Complex paediatric and neonatal cases remain within specialist children’s services; the focus here is the adolescent and adult presentation, with joint working where a patient sits across the two.

Disclosures

This page is general patient and family information, not medical advice for any individual. Dr Lawrence Okiror is a Consultant Thoracic and Robotic Surgeon at Guy’s and St Thomas’ NHS Foundation Trust, with private practising privileges at London Bridge Hospital and The Lister Hospital Chelsea. He trained in paediatric cardiothoracic surgery and works with the paediatric team at Evelina London for younger and more complex patients; complex paediatric and neonatal care remains within specialist children’s services. Private treatment of under-18s takes place at The Portland Hospital and is arranged individually. He has no commercial relationships relevant to this content. Decisions about whether surgery is appropriate, and where it should take place, should be made on a case-by-case basis after appropriate clinical assessment.