A 43-year-old male presented to the Makerere University Lung Institute in Kampala as a referral from a rural tertiary hospital in Uganda. He presented with a 30-year history of a productive cough, whose initial onset followed an episode of cough during a nationwide outbreak of whooping cough at 10 years of age. The cough had worsened over the past one year, had no diurnal variation, was wet and associated with copious amounts of purulent sputum, difficulty in breathing, chest pain and weight loss for 3 months. The chest pain was predominantly left-sided and non-radiating. There was no history of hemoptysis, nasal blockage, loss of sense of smell, fever, or night sweats. Patient reported no history of loud snoring, he had no episodes of gasping for air during sleep and no history of somnolence. He however reported palpitations, dyspnea, orthopnea, and body swelling involving the lower limbs and abdomen. On presentation, he had had several admissions because of worsening cough, and had unscheduled outpatient visits almost monthly for the cough in the past 30 years. He had been treated with intravenous and oral medications with minimal to no improvement. At 19 years of age, he was treated for clinically diagnosed pulmonary TB (no bacteriological confirmation), but with no improvement. He had no known allergies. He denied any history of cigarette smoking and alcohol use. He had no family history of similar illness. He was a farmer until 3 years prior to presentation when the symptoms limited his physical activity.
On physical examination, he was sick-looking, severely wasted (37kgs body weight, height -160.5cm, BMI-14.4 kilogram per meters2; Figure 1A). He had proptosis grade 3 digital clubbing (Figure 1B), central and peripheral cyanosis and pretibial oedema (Figure 1C). He was afebrile (temperature 36.9 Celsius). He had no pallor, jaundice, lymphadenopathy or signs of dehydration. 
On respiratory system examination, he had mild respiratory distress with a respiratory rate of 32 breaths per minute (tachypneic). His SPO2 was 99% on room air. He had no chest deformities or asymmetry. The trachea was centrally placed. There was equal air entry bilaterally with bilateral widespread loud harsh breath sounds, bilateral crepitations and rhonchi. In cardiovascular examination, he had a thin volume pulse with a tachycardia of 108 beats/minute. The blood pressure was normal (92/70mmHg). The apex beat was in the 5th intercostal space, mid-clavicular line. The heart sounds I and II were heard, with a 3rd heart sound (a gallop rhythm) and a loud second heart sound in the pulmonary area (loud P2). On abdominal examination, he had mild abdominal distension, shifting dullness (indicative of moderate ascites), and a tender hepatomegaly of 8 cm below the coastal margin with a positive hepatojugular reflux.
The diagnostic work up is summarized in Table 1. Serum creatinine, urea, electrolytes, liver enzymes, bilirubin were all normal. Spirometry evaluation was attempted but he had very poor respiratory effort to complement the examination.
The chest X-ray showed dilated trachea and principal bronchi with diffuse reticulolinear and ring-like opacities involving mainly the mid and lower lung zones bilaterally. Figure 2 shows the chest X-ray. A sequential high resolution computed tomography (HRCT) scan was performed using a 128-slice multidetector CT scanner. The study was performed in full inspiration with a scan time of five seconds. The scan time was short enough to enable the patient hold his breath throughout the scanning period and this avoided motion artifacts. An expiratory scan would have complemented the findings; however, this was not performed. Figures 3-5show the High-resolution chest computed tomography (HRCT).    
In this patient, a diagnosis was made of tracheobronchomegaly (Mounier Kuhn syndrome), complicated with pulmonary hypertension, and right-sided heart failure. Differential diagnoses of cystic fibrosis and bronchiectasis were considered. The patient was initiated on oral prednisolone 20 mg (short-course), oral furosemide 40 mg, rhinathiol syrup, oral azithromycin (empirically for suspected lung infection), megestrol (for cachexia), and low-dose oral morphine (for the dyspnea), inhaled corticosteroid/long-acting beta agonist, inhaled tiotropium, and chest physiotherapy. Two months later, the patient reported improvement. The sputum volume was much less, had less exercise intolerance and had gained 3 kgs. He was also linked to the palliative care team to offer home-based palliative care in addition to the long-term therapies above. He was also scheduled for monthly reviews at the Makerere University Lung Institute. However, he passed away from home in the fourth month of follow-up from a lung infection that was managed at a peripheral rural health facility.