For months, what should have been a joyful journey into parenthood was clouded by uncertainty for the parents of a baby diagnosed before birth with a rare congenital lung disorder affecting both lungs. The diagnosis meant their child would require surgery within months of birth, and there was a real possibility that a large portion of the infant's lungs might have to be removed, potentially affecting breathing capacity for life.
The family's anxiety has now given way to hope after doctors at the All India Institute of Medical Sciences (AIIMS), Delhi, successfully performed a rare lung-preserving keyhole surgery on the four-month-old infant, avoiding the removal of an entire lung lobe and preserving the child's future lung function.
The baby was diagnosed during pregnancy with Congenital Pulmonary Airway Malformation (CPAM), a rare developmental disorder in which part of the lung forms abnormally, creating cyst-like tissue that cannot function like healthy lung. While CPAM typically affects one lung, this infant had lesions in both lungs, making the surgery considerably more challenging.
The child was discharged just 48 hours after the operation and will undergo a second surgery on the left lung after a few months, once adequate recovery and growth have taken place.
According to doctors, the involvement of both lungs meant that conventional surgery involving removal of an entire lobe would have significantly compromised the child's future lung capacity. Instead, the surgical team opted for a technically demanding segmentectomy, removing only the diseased portions of the right lung while preserving as much healthy tissue as possible.
The surgery was led by Dr. (Prof.) Vishesh Jain of the Department of Paediatric Surgery using minimally invasive thoracoscopic, or keyhole, techniques. The operation involved removing segments 9 and 10 of the lower lobe of the right lung—among the most difficult portions of the lung to access because their blood vessels and airways lie deep within the tissue.
"Segments 9 and 10 are among the most difficult to remove in the entire lung, since their vessels and airway are buried deep within the tissue, far from the surface. Performing this safely through keyhole surgery in an infant just four months old is a significant technical achievement," said Dr. Sandeep Agarwala, Head of the Department of Paediatric Surgery at AIIMS, Delhi.
He added, "Outcomes like this are only possible because of teamwork and because of the dedicated healthcare workers at AIIMS, whose infrastructure and facilities are well suited to conducting this kind of complex, lung-preserving paediatric surgery."
Speaking to The Health Outlook, Dr. Jain said the operation is believed to be the first of its kind in the country.
"To the best of our knowledge, this is the first time in India that such a highly selective, lung-preserving segmentectomy has been performed in an infant as young as four months. Instead of removing an entire lobe of the lung, we removed only the diseased segments while preserving as much healthy lung tissue as possible," he said.
Dr. Jain explained that each lung is divided into several functional segments, each supplied by its own airway and blood vessels. While removing an entire lobe is technically less demanding, a segmentectomy requires surgeons to meticulously separate these delicate structures and remove only the diseased tissue without damaging the surrounding healthy lung.
The operation was further complicated by the infant's age. Thoracoscopic surgery in a four-month-old demands exceptional precision because of the tiny size of the lungs, blood vessels and airways.
Another critical component of the procedure was selective lung ventilation, an advanced anaesthesia technique that allows one lung to be ventilated while temporarily collapsing the other to provide surgeons with adequate operating space. The technique is particularly challenging in infants because of their extremely small airways.
The anaesthesia was managed by Dr. Abhishek of the Department of Anaesthesia, enabling the surgery to be completed safely without complications.
Doctors said the infant recovered well and was discharged in stable condition within two days.
Dr. Jain said the case also underscores the importance of prenatal diagnosis and early referral to specialised paediatric surgical centres.
"The important message for parents is that these congenital lung malformations are now routinely detected during antenatal ultrasound examinations. If such an abnormality is identified before birth, parents should consult a paediatric surgeon early. Many of these conditions can now be treated safely through minimally invasive keyhole surgery, with techniques that preserve healthy lung tissue and improve long-term outcomes. Early diagnosis and timely intervention are crucial," he said.
Although the exact cause of CPAM remains unknown, advances in prenatal imaging have enabled doctors to diagnose the condition well before birth, allowing families to plan treatment immediately after delivery.
"The exact cause of this condition is still unknown. It is a congenital abnormality that develops before birth, but medical science does not yet know why it occurs in some babies while others are born with completely normal lungs," Dr. Jain said.
He noted that the disorder was once believed to affect approximately one in every 30,000 to 40,000 births. However, improvements in antenatal ultrasound have substantially increased detection rates.
"Based on current estimates, congenital pulmonary airway malformation is now believed to affect about one in every 20,000 live births," he said.
Doctors emphasised that the condition itself is not invariably fatal. However, untreated lesions can enlarge over time, compress healthy lung tissue and lead to serious breathing difficulties. Children may also experience recurrent respiratory infections, including repeated episodes of pneumonia.
"Another reason for timely surgical treatment is the long-term risk associated with retaining the abnormal tissue. Although uncommon, the malformed lung tissue has the potential to undergo malignant transformation and develop into a tumour later in life. Removing only the diseased segments while preserving the remaining healthy lung offers children the best opportunity for normal lung growth and long-term respiratory function," Dr. Jain said.
The surgery addressed only the diseased portion of the right lung. Since the left lung is also affected, the infant will undergo another lung-preserving procedure after a few months.
Doctors say the case demonstrates how advances in foetal diagnosis, minimally invasive surgery and paediatric anaesthesia are transforming the treatment of rare congenital disorders in India. For families confronting a prenatal diagnosis of CPAM, the successful outcome offers reassurance that even complex bilateral lung malformations can now be managed while preserving the healthy lung tissue that children need for normal growth and development.
