How surgeons saved a teenage girl’s life from a disease that destroys blood vessels from within- The Week

Some diseases announce themselves loudly like a sudden fracture, a visible tumour, a fever that refuses to break. And then some diseases work quietly, insidiously attacking the body from the inside with no obvious warning until the damage is done. Behçet’s Disease belongs to the second category. Rare, poorly understood, and capable of causing catastrophic harm to blood vessels, it remains one of medicine’s most challenging adversaries.

For a 15-year-old girl from Bengaluru, this silent enemy had been part of her life since 2018. Diagnosed at a young age, she had grown accustomed to the discipline of long-term immunosuppressive therapy, which included her daily medications, clinic visits, and watchful waiting. She and her family had learned to live alongside the disease. What they had not anticipated was how suddenly and violently it could escalate.

Behçet’s Disease: The disease that attacks its own

Behçet’s Disease (BD) is a rare, chronic, systemic vasculitis and an autoimmune condition in which the body’s immune system mistakenly attacks its own blood vessels. The disease does not discriminate by vessel size. It can inflame veins, arteries, capillaries, and major vessels alike, and affect both the arterial and venous systems simultaneously. The inflammation damages the vessel walls, weakening them structurally and predisposing them to thrombosis (clot formation), occlusion, or aneurysm, which is an abnormal ballooning of the vessel wall due to structural weakness.

There is no definitive blood test or imaging that diagnoses Behçet’s disease. The process often takes years, during which patients may be misdiagnosed with Crohn’s disease, multiple sclerosis, recurrent aphthous stomatitis, or lupus.

A Life in the Balance: The Patient’s Story

Our patient was first diagnosed with Behçet’s Disease in 2018, when she was approximately eight years old. She began long-term immunosuppressive therapy with a combination of corticosteroids, colchicine, and azathioprine, along with other disease-modifying agents, which were designed to suppress the overactive immune response and protect her blood vessels from ongoing inflammation.

For several years, the treatment appeared to be working. Her disease was managed and monitored. But Behçet’s Disease is not an enemy that one can simply overcome. In 2025, routine imaging tests revealed what clinicians had feared: the development of an abdominal aortic aneurysm, a bulge in the aorta, the great vessel that carries oxygenated blood from the heart to the body’s lower half through the abdomen.

When the first fix fails: The endoleak crisis

Following her stenting procedure, the young girl initially appeared to be on the road to recovery. But soon, the warning signs returned: recurring abdominal and back pain, persistent fever, and declining appetite. Advanced imaging, like CT angiography, delivered findings of a grave nature: a Type 1A endoleak.

An endoleak occurs when blood continues to flow outside the stent graft but within the original aneurysm sac. And it had expanded too. The aneurysm had grown to nearly five centimetres and was now pressing against the spine – a testament to the enormous force of the blood-filled sac. In this context, a rupture was not a theoretical risk, but an imminent reality. A ruptured aortic aneurysm carries mortality rates exceeding 80 per cent even with immediate surgical intervention. One of the defining principles of modern complex surgery is that no single medical speciality can manage everything. The weeks and days before this operation exemplified this principle.

Behçet’s disease, affecting large blood vessels, is rare and can lead to rapidly progressing aneurysms. Managing such cases requires a delicate balance between controlling inflammation and performing timely surgery.

The road to recovery: Intensive care and gradual progress

The surgery was a success, but the work was not over. The postoperative period in paediatric aortic surgery, particularly in a patient with inflammatory vascular disease, is a critical and unpredictable phase. The young girl was transferred to the intensive care unit, where she was kept sedated and ventilated while her body began the slow process of recovery.

The early postoperative days brought expected challenges. Blood pressure proved difficult to regulate as hypertensive swings are common after aortic surgery when the cardiovascular system recalibrates to its new anatomy. The surgical team navigated these fluctuations carefully, balancing the need for adequate organ perfusion against the risk of placing excessive stress on fresh surgical anastomoses.

She was discharged from the hospital in stable condition, maintaining normal oxygen saturations, tolerating oral intake, and beginning the long but far more hopeful journey of outpatient recovery and ongoing Behçet’s management. Her family, who had stood vigil through the long hours of surgery and the tense ICU days, finally had some relief.

Vascular Behçet’s disease in children is not common. The failure of initial endovascular stenting and the subsequent need for open repair reflect a well-recognised challenge in Behçet’s disease. Endovascular techniques are appealing precisely because of their minimally invasive nature.

Why is early diagnosis important?

Perhaps the most important lesson from this case is the critical need for early diagnosis and sustained follow-up in Behçet’s Disease. Had this young girl not been diagnosed and managed in 2018, her aneurysm might have formed and ruptured without warning — an outcome from which there would have been no recovery. Regular surveillance imaging in patients with known vascular involvement or high-risk features is essential to detect complications before they become unsurvivable.

In India, where Behçet’s Disease is underrecognised, raising clinical awareness among general practitioners, paediatricians, and internal medicine specialists is an urgent priority. The diagnosis should be considered in any young patient presenting with recurrent oral ulcers, unexplained inflammatory episodes, or vascular pathology that does not conform to the expected pattern of atherosclerosis.

Dr Varun Shetty, Senior Consultant, Cardiac Surgeon, Narayana Health City, Bengaluru.

The opinions expressed in this article are those of the author and do not purport to reflect the opinions or views of Buzz.