An Interesting Case of a 62-year-old Female Patient’s Reappearing Lung!

The Vanishing Lung Syndrome

Department of Pulmonology & Cardiothoracic Surgery

WHAT WAS THE CASE?

A 62-year-old obese female patient had progressively worsening dyspnea for 5 years and a past history of COPD Treatment along with being on home oxygen therapy. A chest X-ray and CT scan were done in 2017 and they revealed a large bulla in the right lung, compressing the normal lung
and shifting the mediastinum to left, thus also compressing the normal left lung. Surgery was clearly indicated but she was advised against it, as it was considered to be of very high risk.

The bulla continued to enlarge and almost the entire right lung disappeared. She had multiple episodes of LRTI requiring hospitalization and higher antibiotics. Dyspnoea progressed to the point that she needed home oxygen therapy and became kind of a respiratory cripple.

WHAT IS THE VANISHING LUNG SYNDROME?

Vanishing Lung Syndrome (VLS) is a rare radiological syndrome in which the lungs appear to be disappearing on X-ray. It is a chronic, progressive condition characterised by giant emphysematous bullae, commonly developed in the upper lobes which compresses the remaining normal lung. The commonest differential diagnosis is Tension Pneumothorax. The pathogenesis of the disease is due to the destruction of the alveolar walls which results in the formation of subpleural blebs that coalesce to form a giant bulla.

HOW WAS THE CASE DEALT WITH?
With progressively worsening dyspnea for 5 years, the patient was advised routine management of COPD by the usage of various inhalers. Even though she was on home oxygen therapy, her dyspnea continued to worsen. The patient was non-diabetic and non-hypertensive with a normal heart rate and normal routine blood test reports. However, she was overweight and could not walk more than a distance of 100 m.
She had been admitted to the hospital multiple times earlier and diagnosed with chronic obstructive pulmonary disease (COPD) on the basis of her history and chest X-ray findings. Eventually, her CT chest revealed the diagnosis of giant bullous disease or the vanishing lung syndrome. By April 2022, her right lung had almost disappeared.
The department of Cardiothoracic Surgery along with the department of Interventional Pulmonology and the department of Critical Care carefully operated on the patient with the following surgical details.

• Double Lumen Tube (Endo-Bronchial Tube) placed with Bronchoscopic guidance
• Triple Lumen Central Line in right Internal Jugular Vein (IJV)
• Left Radial Arterial Line
• Indwelling Thoracic Epidural
• Urinary Catheter
• Continuous monitoring of ECG (two leads), IBP, CVP, SpO2, EtCO2, Core Temp – Anesthesia Agent Monitoring
• Lab tests during surgery: ABG, CBC, RBS, Electrolytes

After discussing the pros and cons of the surgery with the family, the patient was accepted for the same. Her routine pre-operative workup was normal. Thoracotomy was done from the right fifth intercostal space. A large bulla arising from the superior segment of the right lower lobe almost popped out of the incision. The rest of her entire lung collapsed. Bulla was opened and all air-leaking bronchioles were painstakingly identified and closed. To restore the normal lung anatomy, the bulla cavity was obliterated by suturing margins of the bulla wall with each other (capitonnage).

• The Bulla popping out of the incision
• Inside of Bulla
• Completed Repair
• Final appearance – all three lobes fully expanded

The surgery encompassed the right lung kept in isolation with an open lumen (no ventilation). Since the bulla does not expand under positive pressure ventilation, whatever air escaped from it made it smaller. Only the left lung was allowed ventilation till the bulla was opened surgically. The repair process included the Bulla wall being cut off with just an inch of margin around. The bulla cavity was later collapsed by stitching the residual bulla walls to each other using multiple sutures of 3-0 using polypropylene. All three lobes of the right lung expanded very well and filled upto 80% of the chest cavity. The patient tolerated the given anaesthesia surprisingly well.

WHAT WAS THE POST-OPERATIVE CONDITION?

The patient’s expanded lung filled upto 85-90% of her chest cavity. Along with overnight ventilation and supply of good blood gases in the morning, she resulted in a hemodynamically stable condition with good ventilatory parameters. She made a slow but complete recovery. Lung took time to expand completely. The expansion was aided by continuous low suction on chest drains. Due to the rising in the total counts, she needed an escalation of antibiotics. She had effortless extubation the following morning but was kept under observation in the ICU for the next three days.
She was discharged on day 12 with a fully expanded lung, no air leak, no fever, off oxygen and able to climb one flight of stairs. With twice a day of physiotherapy sessions, the patient had a slow but steady recovery.

LOOKING FOR TREATMENT?

Cardiology or Cardiothoracic diseases and conditions are best treated with the comfort, support, right guidance and the best and advanced technology. With an aim to always put patients first, Dr. Apurv Vaidya, Cardiothoracic Surgeon, Dr. Nirav Panchani, Interventional Cardiologist and Dr. Arpan Desai, Interventional Cardiologist from the department of Cardiology and Cardiothoracic Surgery at Aadicura Superspeciality Hospital treats and provides you with the best possible outcomes that help you recover faster and prevent any further damage from the disease.

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