Rare presentation of bronchiectasis with multiple bronchial stones

Introduction

Bronchiectasis with broncholithiasis is a very rare condition and often a diagnostic challenge. Very few cases have been reported so far. Bronchiectasis was first described by Lannec in 1819. ¹ Often, termed as orphan disease due to low clinical suspicion, commercial interest and research activity, ² it is still a major problem in developing countries. Chronic, complicated, unattended bronchiectasis leads to formation of broncholithiasis. Bronchiectasis is today, diagnosed frequently due to advent of high resolution CT (HRCT) scan. Being progressive disease, early resection of localized disease is preferred, to preserve uninvolved lung parenchyma and prevent complications e.g. hemoptysis, obstructive pneumonia or secondary bronchiectasis. ³ Long-term medical treatment reduces quality of life, increases risk for life-threatening complications and has mortality rates of 19–31%. ² Bronchoscopic or thoracoscopic removal of broncholithiasis has also been tried, but with little success.

Case report

A 60-year-old male presented to the department of surgery with a cough since six years and breathlessness since six months. The cough was productive, copious amount (250–500 ml), white, sticky, occasionally purulent and foul smelling. The patient had only one episode of haemoptysis, five years previous. The patient developed breathlessness, insidious onset six months earlier on exertion, which later progressed to breathlessness at rest since one month. The patient had fever, moderate grade, occasionally high grade, every two to three days responding to medications, along with easy fatigability and weight loss.

On admission, the patient was afebrile with pulse rate of 90 min and blood pressure 110/70 mmHg. He had tachypnoea (respiratory rate 24 min). He was pale, anicteric with no lymphadenopathy. Respiratory examination showed decreased air entry in right lower lobe with decreased tactile vocal fremitus and vocal resonance. However, there was no wheeze. Rest of the systemic examination was normal.

Investigations revealed haemoglobin 10 g% with total leucocyte count 20,600 cells/mm³. The kidney function test was normal, however the patient had hypokalemia (S. Potassium = 2.2 meq/L). Chest X-ray revealed right lower lobe cystic lung disease with calcifications and calculi (Figure 1). Sputum culture sensitivity showed 20–25 pus cells/hpf and growth of pseudomonas aeruginosa and Escherichia coli sensitive to ciprofloxacin, ceftazidime and amikacin. The profile for tuberculosis was negative. The electrocardiograph was normal, apart from short T waves. Computed tomography (CT) of thorax revealed cystic dilated structure with air fluid levels in right middle lobe and anterior basal segment of lower lobe with multiple hyperdense nodular opacities within cysts likely to be infected bronchiectasis and broncholithiasis (Figure 2). Pulmonary function test showed decreased forced expiratory volume 1 (FEV1) and vital capacity. OPEN IN VIEWER

OPEN IN VIEWER

Figure 2.

CT thorax of the patient showing cystic dilated structure in right middle and lower lobe with air fluid levels and multiple hyperdense nodular opacities suggestive of infected bronchiectasis and broncholithiasis.

The dyselectrolytemia was corrected and patient was started on specific antibiotic 10 days prior to surgery. He was also given postural drainage in left lateral position along with chest physiotherapy.

A fifth intercoastal space, right posterolateral thoracotomy was performed. Intraoperatively, dense adhesions were present between pleura and median basal lobe. The adhesions were separated. The median basal segment of right lung was fibrosed. A median basal segmentectomy with lobectomy was performed. Excessive bronchial dissection was avoided and peribronchial tissues were preserved. On opening the lobe, multiple broncholiths with thick secretions were present. The bronchial stump was sutured with synthetic monofilament suture and was reinforced with mediastinal fat tissue. The patency of upper lobe was checked. Complete hemostasis was achieved and closure done in layers with an underwater seal drain.

On gross examination, external surface of bronchus was dilated, irregular and black with cut surface showing cystically dilated bronchi and shrunken lung parenchyma. Multiple broncholiths were present, largest measuring 1 cm in diameter (Figure 3). Microscopic examination showed bronchiectasis with interstitial fibrosis and atelectasis. OPEN IN VIEWER

The patient had an uneventful recovery. Chest physiotherapy was started immediately after operation and continued throughout hospital stay. Culture specific antibiotics were given for 10 days. Postoperative chest X-ray showed expansion of lung. At time of discharge, pulmonary function test showed no significant differences in respiratory function preoperatively and postoperatively.

The patient was followed up thrice, at three monthly intervals. The patient had no symptoms postoperatively and repeat chest radiograph was normal.

Discussion

Bronchiectasis is anomalous, irreversible dilatation of segmental and subsegmental bronchi and bronchioles caused by destruction of elastic and muscular layers of bronchial wall, secondary to chronic recurrent necrotizing infection and inflammation with reduction in clearance of secretions and expiratory air flow. It is a condition in which some bronchi become permanently scarred and enlarged. The damaged cilia are unable to effectively sweep away mucus. Accumulation of mucus results in lung infections. Recurrent infections cause further scarring and bronchial enlargement thereby perpetuating the condition. Sometimes, in long-standing cases, these scarred areas act as site for dystrophic calcification and may lead to formation of calculi as in our case.

Though immune deficiency syndromes (IgA and IgG deficiency, leucocyte dysfunction), metabolic (cystic fibrosis, alpha 1 antitrypsin deficiency) and structural defects (primary ciliary dyskinesia, young syndrome) are predominant etiologic factors in developed countries, bacterial (tuberculosis), viral infections (measles, pertussis) and foreign body obstruction are major causes in developing countries. ⁴ Etiology remains undetermined in 40% (30–74%) of patients, despite advanced immunological and genetic diagnostic techniques. ⁴

Overall, one third of bronchiectasis is unilateral affecting single lobe, one third is unilateral affecting more than one lobe and one third is bilateral. ⁵ Basal posterolateral segments of lower lobe are most frequently affected (56%), followed by middle lobe and lingula. It is more common on left side. The morphological classification of bronchiectasis includes cylindrical and saccular (cystic) variety. ³ Cystic Bronchiectasis is more common and has a worse clinical picture secondary to daily bronchorrhoea, life threatening hemoptysis, clubbing and chronic respiratory failure. ⁶

Broncholithiasis is the presence of calcified material within bronchus or adjacent communicating cavity. ⁷ It occurs when peribronchial calcific nodal disease (secondary to chronic necrotizing lymphadenitis by tuberculosis and histoplasmosis) distorts tracheobronchial tree, due to repeated physical impingement by respiratory motion, with or without erosion into it. ⁸ Other causes of broncholithiasis are aspiration of bone tissue, in situ calcification of aspirated foreign material, erosion by and extrusion of calcified bronchial cartilage plates with subsequent sequestration and migration of calcified material from distant site to bronchus e.g. pleural plaque or kidney (by nephrobronchial fistula).

The chemical composition of broncholith is similar to bone, 85–95% calcium phosphate and 7–10% calcium carbonate. ⁹ Broncholiths are variable sized, irregular shaped and possess spur-like projections or sharp edges. Preferential sites are proximal right middle lobe and anterior segmental upper lobes bronchus. ¹⁰ Histologic examination of broncholiths shows amorphous or laminated necrotic material with extensive dystrophic calcification.

Diseases that mimic broncholithiasis are calcified fungal ball, calcified primary endobronchial actinomycosis (very rare), calcified endobronchial tumor e.g. carcinoid, osteomas, osteosarcomas, chondromas, chondrosarcomas, hamartoma with central cartilaginous core, tracheobronchial diseases with stippled sural calcification and hypertrophied bronchial artery secondary to infections with intraluminal protrusion. ¹⁰

The most common symptoms of broncholithiasis are persistent nonproductive cough, frequent hemootysis (occasionally life threatening) and features of recurrent obstructive pneumonia (pain, fever with chills and dyspnoea). ¹¹ A history of expectoration of calcified material (lithoptysis) prompt the diagnosis, but is infrequent. The physical findings are nonspecific (bilateral basal crackles and wheeze). In addition to this, symptoms of bronchiectasis e.g. recurrent and persistent pulmonary infections leading to productive cough, bronchial suppuration and chronic purulent foul-smelling bronchorrhoea (upto 500 ml/day) may also be present. Dyspnoea is seen in 70% cases of bronchiectasis as reported by Nicotra et al. ¹²

Pulmonary function test (PFT) is normal in localized bronchiectasis. In severe cystic bronchiectasis, mixed obstructive and restrictive pattern with hypoxaemia is seen. ⁵ Preoperative fibre optic bronchoscopy, today, has little role except for preoperative bronchial lavage. Broncholiths are identified in only 28–44% of patients with bronchoscopy. ⁸ HRCT is currently the modality of choice for diagnosis of bronchiectasis with broncholithiasis. ⁴ Although chest X-ray has poor sensitivity (being normal in 20%), it is used as screening tool and initial investigation.

Helical CT with thin collimation (<3 mm) shows peribronchial lymph nodes, calcification and changes of bronchial obstruction e.g. atelectasis, infiltrates, secondary bronchiectasis, air trapping, mucoid impaction, obstructive pneumonitis and absence of associated soft tissue mass. ⁸ Internal diameter of bronchus >1.5 times that of accompanying artery and lack of tapering of bronchi confirms bronchiectasis. The false positive and negative rates are 2% and 1% ¹³ with sensitivity and specificity of 66% and 92%, respectively. ¹⁴ CT scans viewed at bone setting (300 Hounsfield units (HU)) with window of 1800–2000 HU are valuable to confirm broncholith. ¹⁴

Goal of surgical treatment is complete resection of affected segment while preserving maximum function, to improve quality of life and to resolve complications e.g. empyaema, hemoptysis and lung abscess. A minimum of two lobes or six pulmonary segments must be spared to ensure adequate pulmonary function.

Initial treatment for all patients with bronchiectasis is infection control (antimicrobial treatment), reduction of airway obstruction by bronchodilatation and corticosteroids, chest physiotherapy (postural drainage) and vibratory massage. However, with each recurrent infection, surrounding normal lung is affected and bronchiectatic areas become larger and further destroys lung. Medical management has mortality rate of 19 ± 31% with reduced morbidity. ¹⁵

Hodder et al. ¹⁶ stated that surgery is indicated in failure of medical treatment (resistance to antibiotic, postural drainage and chest physiotherapy for at least two years), specific symptoms (hemoptysis, recurrent pneumonia), ¹⁷ bronchiectatic pulmonary tuberculosis for residual disease, unexpandable lobe, persistent hemorrhage despite interventional radiology or irreversible destructed parenchyma causing respiratory distress and fatique. ¹⁸ The productive cough with abundant fetid sputum is a psychological indication of surgery. ⁵

Resectional surgery is indicated in localized unilateral cystic bronchiectasis and bilateral localized bronchiectasis. Selected bilateral and multiple bronchiectasis is not a contraindication to surgery, even incomplete resection often results in considerable benefit to patient. Transplantation remains indicated with diffuse cystic disease with seriously compromised function and chronic respiratory failure i.e. end-stage bronchiectasis. ¹⁹ Bierne et al.reported one year survival rate of 68%. ²⁰ Most common resection types are lobectomy and segmentectomy – single or multiple (on same or different lobe). Incomplete resection and pneumonectomy are also done. Results of surgery in diffuse and multisegmental disease, pseudomonas infection, permanent obstructive pathology and resected perfused segment are poor. ²¹ Success rate is higher if surgical intervention is performed before complications, early in age, is complete and in dry period. Surgery did not improve forced vital capacity (FVC) or FEV1 despite parenchymal resection as excised lung segment contributed little to ventilation. ¹ Video assisted thoracoscopic surgery (VATS) is used for middle lobe or lingual resection, with minimum adhesions and no calcified nodes . Surgical treatment has mortality and morbidity rates of 1–8.6% and 14–53% respectively. ¹⁹

Treatment guidelines are controversial for broncholithiasis due to few case reports. In asymptomatic forms or mild forms, patient may be observed only or broncholithiasis can be removed bronchoscopically, if broncholith is completely free within the bronchus.^22,23 Trastek et al. stated indications for surgery in bronchoithiasis as severe or recurrent hemoptysis, symptoms of distal obstruction or oesophagobronchial fistula. ²⁴

The surgical technique used is simple broncholithotomy to pulmonary resection with maximum preservance of healthy lung parenchyma. Pulmonary resection is preferred to broncholithotomy. Cole et al. mentioned that during severe adhesions, opening of mass capsule and cauterizing the contents can prevent lethal complications of pulmonary resection. Simple broncholithotomy without pulmonary resection is insufficient and causes late complications and reoperation. In very ill patients unfit for surgery, where lesion cannot be removed bronchoscopically, yttrium aluminium laser has some benefitt. ²³

Complications usually range between 9.4% and 24.6%. ⁶ Main complications are bleeding and prolonged air leak, secondary to dense adhesions. Bronchopleural fistula is observed in 9.1% cases. Postoperative empyaema is higher in bronchiectasis.

Rare complications of broncholithiasis requiring surgery include bronchoesophageal or bronchoaortic fistula, obstructive pneumonia, secondary bronchiectasis and hemoptysis (due to erosion into adjacent bronchial vessels, pulmonary vessels or aorta). Hemoptysis is most dangerous complication of broncholithiasis. Massive hemoptysis is controlled by bronchial artery embolisation and surgery. ⁹ Fujimato and colleagues showed three variables as significant prognostic factors i.e. cylindrical type of bronchiectasis, absence of sinusitis and complete resection of diseased parenchyma (Most important).

Conclusion

Bronchiectasis with broncholithiasis is a very rare condition.