Thursday 29 October 2020

Case Reports in JBGSR

Tuberculosis of the Breast with Erythema Nodosum: A Case Report by Richmond Ronald Gomes in Open Access Journal of Biogeneric Science and Research (OAJBGSR)

Abstract

There have been an increasing number of tuberculosis cases worldwide, but tuberculosis of the breast remains rare. In rare cases, this is seen with a cutaneous manifestation of erythema nodosum. We report the case of a 33-year-old woman with tuberculosis of the left breast accompanied by erythema nodosum on the anterior aspect of both lower legs and dorsum of feet. Due to her poor clinical response to conventional therapy, and the histopathological findings of fine needle aspiration cytology with epitheloid granuloma with caseation, there were strong indications of tuberculosis. She received anti tuberculous therapy for 6 months. During her follow-up examination after 6 months, no evidence of either residual or recurrent disease was present. Histopathological features and a high index of clinical suspicion is necessary to confirm a diagnosis of tuberculosis of the breast. Anti-tuberculous therapy (ATT) with or without simple surgical intervention is the core treatment.

 

Keywords: Tuberculosis, erythema nodosum, caseation, granuloma, anti tuberculous therapy.

Introduction

Tuberculosis (TB) is one of the leading infectious diseases worldwide. Extra pulmonary TB involving the breast is extremely rare. It often mimics breast carcinoma and pyogenic breast abscess clinically and radiologically, may both co-exist. Vulnerability to breast TB is increased in women who are young, married, multiparous and who breast-feed [1]. Histopathological examination using fine needle aspiration cytology (FNAC) may reveal caseating epitheloid cell granulomas and acid-fast bacilli (AFB). Although the presence of an acid-fast stain or culture is essential to confirm diagnosis, it does not give a positive result in most patients [2,3]. Molecular detection of Mycobacterium tuberculosis by polymerase chain reaction can be particularly useful in the validation of a diagnosis of tuberculosis in clinical settings where the diagnosis is uncertain [3,4]. Diagnosis is usually based on high index of suspicion, findings of granulomatous lesion with Langhans’ giant cells, tuberculosis culture and response to antitubercular therapy (ATT). We report a case of TB of the breast associated with a cutaneous manifestation of erythema nodosum.

Case Report

A 33-year-old married, recently detected diabetic( on metformin),HIV negative housewife presented at medicine outpatient, Ad-dinWomens Medical College & Hospital, Dhaka with fever, painful swelling over both shin and dorsum of the feet, bilateral knee and ankle pain for 8 days. She also gave history of gradually growing painful discharging lump over her left breast for last 1 month with on and off undocumented low grade fever. For the last 8 days fever became high grade, intermittent (maximum recorded 1030F) associated with chills and rigor and subsided temporarily after taking anti pyretic. She did not have any personal medical history of TB or diabetes mellitus. She also neither had family history of breast cancer nor had any contact history with a patient with active tuberculosis. She also denied any cough, weight loss, night sweat, anorexia, bloody diarrhea. She was immunized as per EPI schedule. With these complaints she visited a local physician and got 7 days 2nd generation oral cephalosporin without any improvement. She had a three-year-old child.

 

Upon admission she had a body temperature of 102 °F, blood pressure of 126/68mmHg, a pulse rate of 89/minute, and a respiratory rate of 19/minute. On physical examination, there was a firm, erythematous, mass of 5 × 6 cm with brownish discharge over the upper outer quadrant of her left breast accompanied by retraction of adjacent skin. Mass was not fixed to underlying structure or overlying skin. Nipple, areola was normal. There was also a firm, non-tender mobile lymph node over left medial axilla. Dark reddish plaque skin lesions were found over both lower legs and the dorsal aspect of her feet. Both ankle and knee joints were also red, swollen. There was also painful restriction of both active and passive movement in these joints. No other jointsor axial skeleton was involved. Her blood test results showed the following: Hb% 9.3 gm/dl, MCV- 81, MCH 29,white blood cells at 15.20 × 103/μL, neutrophils at 77.3%, lymphocytes at 12.7%, platelets at 418 × 103/μL, C-reactive protein at 86.8 mg/dL (normal range ≤ 5), and an erythrocyte sedimentation rate (ESR) during the first hour of 70mm/hour (normal ≤ 12).Peripheral blood film revealed normocytic normochromic anemia with neutrophilic leukocytosis. Her blood culture revealed no growth, while her chest radiography was unremarkable. MT was positive with induration 14 mm after 72 hours.

 

ALT 29 U/L, serum calcium 8,4 mg/dl, random blood sugar was 13.8 mmol/L. An ultrasonography of our patient's left breast showed a lump measuring about 5 × 5 cm, which was conglomerated, with an irregular margin with hypoechoic heterogeneous echogenicity, and with a left axillary lymph node. A core needle aspiration biopsy of her left breast was also performed. Under the microscope, this section of her left breast showed chronic mastitis mixed with granulation tissue and numerous foreign body giant cellsbut with no evidence of malignancy (Figure 1).Background consisted of necrotic material and fibrinous exudate. A culture of the wound tissues failed to grow any organisms. Stains for AFB were negative. Discharge for GeneXpert TB was not sent due to come unavoidable logistic support. A dermatologist was consulted regarding the dark reddish plaque skin lesions and treated conservatively with NSAIDs.

Figure 1: Hematoxylin and eosin stain of our patients breast tissue, showing giant cell(arrow) and Inflammatory cells.

Our final diagnosis tubercular mastitis was relied on clinical suspicion, histopathological tissue findings and failure to respond to conventional antibiotic therapy. She was put on a 6-month course of anti-tubercular therapy with a 2 month intensive phase of rifampicin, isoniazid, ethambutol, and pyrazinamide without steroid followed by a consolidation phase of rifampicin and isoniazid for another 4 months. After undergoing four months of anti-tuberculous treatment, her left breast mass and axillary node was gradually reduced. Although acid-fast stain showed no tubercle bacilli, her anti-tuberculous therapy was continued. Her left breast mass gradually became smaller and then regressed. She was treated for6 months without any further complication. She was regularly followed up for another 6 months and no evidence of the recurrence of her disease was noted Figures 2 & 3.

Figure 2:  Healed ulcer with sinuses after 2 months of ATT therapy.

Figure 3: After 6 months of completion of AT therapy.

Discussion

Tuberculosis remains one of the leading causes of death from infectious diseases worldwide. Despite the fact that it can affect any organ or site of the body, the breasts, skeletal muscles and spleen are the most resistant to TB [5,6].Tuberculosis comprises approximately 0.025% to 0.1% of all surgically treated diseases of the breast, but this ratio is higher in underdeveloped countries [7]. The first description of mammary tuberculosis was given by Sir Astley Cooper in 1829 [5]. He described TB mastitis as “scrofulous swelling” in the bosom of young women. Although breast TB is primarily considered a disease of the developing world, a steady increase in the incidence of the disease has also been seen in developed countries. This is probably because of the migration of the infected population from endemic zones, and an increasing number of patients who are immune compromised [8]. Tuberculous mastitis is more commonly seen in females of reproductive age group, however, especially during the lactation period, when they are more susceptible since the lactating breast is more vascular and predisposed to trauma [4,5]. Both breasts are reported to be involved with equal frequency. Bilateral disease is rare, occurring in 3% of patients [5].

 

The duration of symptoms varies from a few months to several years, but in most instances, it is less than a year. Constitutional symptoms such as fever, weight loss, night sweats, or a failing of general health are infrequently encountered [2] and is present is less than 20% of cases. Our patient presented only a month history of breast lump without much constitutional symptoms. Its clinical manifestations are variable. Patients usually have a positive tuberculin skin test [9] so as our patient had. The common presentation of breast TB is a lump in the breast with or without ulceration, may associate with the sinus. Other presentations are diffuse nodularity and multiple sinuses. Multiple lumps are less common. Pain in the lump is present more frequently in breast TB cases than in breast carcinomas but our patient had painful breast lump. The involvement of the nipple and the areola is rare in TB. Fixation of the skin, which resembles a neoplastic lesion, may also be present but nipple discharge is uncommon. Associated axillary lymphadenopathy is found in some patients [1,3,10] as our patient had. Other uncommon presentations include; a typical undermined tuberculosis ulcer, purulent discharge from the nipple or with a fluctuant swelling which, if inadvertently incised, produces a discharging ulcer [5,11]. Both breasts can be affected equally but bilateral involvement is very uncommon. Although the upper outer quadrant seems to be the most frequently involved site due to its proximity to the axillary nodes, any area of the breast can be affected9. Lung lesions (active or healed) on radiographic examination are rare now. Mammography is of limited use since the findings are often indistinguishable from a malignancy [11] and young patients have highly dense breast tissue. Co-existing tuberculosis and carcinoma of the breast was reported by Alzaraa et al. [12].

 

Tuberculosis of the breast is mainly classified according to its primary and secondary forms. Although it was initially believed that as much as 60% of breast tuberculosis was primary, it is now accepted that mammary tuberculosis is almost invariably secondary to a lesion elsewhere in the body. Primary infection of the breast however, through abrasions in the skin or through the duct openings on the nipple is a possibility. The most common mode of infection is thought to be retrograde lymphatic spread from the pulmonary focus through the para- tracheal and internal mammary lymph nodes. Hematogenous spread and direct extension from contiguous structures are other modes of infection [5].

 

Breast tuberculosis was originally classified by McKeon et al [13] into the following categories:

        a)       Acute miliary type – rare, due to blood borne infection in miliary tuberculosis;

        b)      Nodular type – the most common type, which presents as a localized lump with or without sinuses in one quadrant of the breast;

        c)       Disseminated type – involving the entire breast with multiple sinuses;

       d)      Sclerosing type – minimal caseation and extensive hyalinization of the stroma, shrinkage of the breast tissue with early skin retraction and late sinus formation; clinically this type is indistinguishable from carcinoma; and (e) tuberculous mastitis obliterans – a rare form due to intra ductal infection with fibrosis and obliteration of the ductal system; sinus formation is infrequent [3,5,13]. Mantoux testing does not offer definitive diagnosis, but confirms exposure of the patient to tubercle bacilli.

 

Radiological imaging modalities like mammography and ultrasonography are unreliable in distinguishing breast TB from breast carcinoma. Similarly, computed tomography (CT) scan and MRI do not give a conclusive diagnosis without histopathological confirmation. CT scan is useful in differentiating between the primary and secondary forms. It is also helpful in evaluating the relationship between deeply located lesions with the chest wall and pleura and in detecting parenchymal lesions of the lung. As such it provides valuable guides to surgery and in defining the extent of the disease, including the involvement of the chest wall [10,14]. The demonstration of acid fast bacilli (AFB) from the lesions is usually difficult [15]. In tuberculosis mastitis, AFB are identified only in 12% of the patients. Our patient also did not show any AFB in the lesion. Therefore, clinical suspicion and demonstration of caseating granulomas with Langhans’ giant cells from the breast tissue and involved lymph nodes may be sufficient for the diagnosis. In tuberculosis-endemic countries, the finding of granuloma in FNAC warrants empirical treatment for tuberculosis even in the absence of positive AFB and without culture results [15,16].

 

Detailed histological evaluation is, however, mandatory to rule out a co-existing carcinoma. Core needle aspiration biopsy from our patients left breast revealed caseation, epitheloid giant cells without any evidence of malignancy. FNAC is very useful and it is a promising technique in expert hands16. A biopsy is mandatory for confirmation of diagnosis. Anti-tuberculous chemotherapy is still the main treatment for breast TB, and no specific guidelines are available for this kind of treatment. The disease should be treated as any other form of extrapulmonary TB. Anti-tuberculosis therapy comprises rifampicin, isoniazid, pyrazinamide and ethambutol for the initial two months, which is then followed by rifampicin and isoniazid for another four months. The extension of anti-tuberculosis therapy from 12 to 18 months is required in patients with slow clinical response, and complete resolution is obtained in most patients. Our patient showed response in 6 months so further extension of therapy was not given. FNAC should be repeated to confirm that the residual mass is fibrotic. In refractory cases that lead to breast destruction, a simple mastectomy may be performed [1,3,9,10]. Radical mastectomy is best avoided unless there is a co-existing malignancy [5,11]. The duration of follow-up after therapy is variable. In a study by Shinde all patients were followed up for a minimum of two years to determine that they were free of the disease after therapy1.After complete ATT, residual lumps localized to a quadrant should be excised via segmental or sector mastectomy. Aspiration or surgical drainage may be required in some cases.

 

The cutaneous involvement of TB is rare. Underlying systemic involvement of TB is often seen in cutaneous TB, especially in children. Cutaneous TB is classified as true TB or tuberculids. True cutaneous TB is composed of tuberculous chancre, miliary TB, lupus vulgaris, scrofuloderma, TB verrucosa cutis, tuberculous metastatic abscess and orificial TB. Tuberculids are delayed sensitivity reactions to M. tuberculosis in patients with a strong immune response. Tuberculids include lichen scrofulosorum and papulonecrotictuberculid. Facultative tuberculids consist of erythema induratum and erythema nodosum. Erythema induratum is a recurrent, painful subcutaneous nodule usually on the posterior aspect of the leg. Biopsy shows lobular panniculitis with vasculitis and granulomatous inflammation. Eythemanodosum is a painful subcutaneous nodule, mostly found on the anterior aspect of the leg. Biopsy shows septalpanniculitis with an absence of vasculitis and usually without granuloma. Erythema nodosum often occurs in association with a granulomatous disease, including sarcoidosis, TB and granulomatous colitis. TB remains an important cause of erythema nodosum in endemic countries [17-19].

Conclusion

In endemic TB regions, a painful breast mass with cutaneous manifestation of erythema nodosum is clinically relevant to determine a diagnosis of breast TB. To conclude, diagnosis of tuberculous mastitis is usually based on high index of suspicion, finding of granulomatous lesion with Langhans’ giant cells and response to ATT. FNAC and biopsy may also be inconclusive. AFB is not seen in most cases. Prompt diagnosis and adequate treatment can avoid unnecessary operation in these patients.


Wednesday 28 October 2020

Journals on Medical Research | JBGSR

Efficacy of the Treatment of the Distal Lung in a Patient with Bronchiectasis using only Volume and Expiratory Airflow Strategies: A Case Report by Marina Garofano in Open Access Journal of Biogeneric Science and Research (OAJBGSR)

Abstract

Introduction: Respiratory physiotherapy and the use of Airway Clearance Techniques (ACTs) is fundamental in treating obstructive diseases, like bronchiectasis. Lots of recent studies show the importance of the expiratory airflow in the airway clearance as the principle of the movement of secretions, replacing the concept of gravity. This case report aims to verify the efficacy of a treatment carried out using the combination of volume and airflow strategies, without any aids or machines.

    Case presentation: C.L., an Italian 54-year-old female, after two years of several investigations because of frequent bronchial infections, was diagnosed with bronchiectasis in 2014. The patient is followed from February 2017 to August 2018: she complained about constant, productive, and purulent cough and fatigue in the pursuit of activities of daily life. The first intervention included the use of a Positive Expiratory Pressure (PEP) aid, Expiration Lente Totale Glotte Ouverte en infra Latéral (ELTGOL), and IET (Increased Expiratory Technique): to the first follow up, following patient's preferences, this treatment has been modified, replacing the flutter with the RIM (Resistive Inspiratory Maneuver). The main outcome considered was: BORG scale for dyspnoea and fatigue, Medical Research Council (MRC) score, COPD Assessment Test (CAT) score to assess the impact of the disease on the quality of life, the sputum quantity, the respiratory rate, and the number of exacerbations per year.

Conclusion: A year after the beginning of the physiotherapy treatment, there was an improvement in the patient's quality of life (reduction of CAT score), in sputum amount and number of exacerbations. These results show that the use of aids or machines may not be necessary for compliant patients if expiratory flows and lung volumes-based approaches are used.


KeywordsBronchiectasis; Airway clearance; Distal lung; Expiratory airflow

Abbreviations: CAT: COPD Assessment Test; MRC: Medical Research Council; HRCT: High-Resolution Computed Tomography; COPD: Chronic Obstructive Pulmonary Disease; ACTs: Airway Clearance Techniques; FET: Forced Expiration Technique; OPEP: Oscillating Positive Expiratory Pressure; ACBT; Active Cycle of Breathing Techniques; CT: Computed Tomography; ADL: Activities of Daily Living; HRCT: High-Resolution Computed Tomography

Background

Bronchiectasis is a chronic lung condition, characterized by a pathological and permanent dilatation of one or more bronchi. This harm affects the usual mucociliar clearance, resulting in persistent bronchial infection and a chronic inflammation of the airway: the relationship between these processes forms a vicious cycle, defined by Cole in 1986 [1,2]. Pseudomonas aeruginosa, Haemophilus influenzae, Streptococcus pneumoniae, Staphylococcus aureus are common pathogenic agents that cause bronchiectasis infection. The Gold Standard to diagnose bronchiectasis in the High-Resolution Computed Tomography (HRCT) [3]. Two types of factors may favor the occurrence of bronchiectasis: congenital factors, including Cystic Fibrosis, Immune Deficiency conditions, Kartageners Syndrome, or acquired factors such as severe pneumonia, Chronic Obstructive Pulmonary Disease (COPD) and asthma, gastroesophageal reflux or inhalations from cigarette smoke. The most common symptoms are chronic cough, sputum of various quantities, recurrent chest infections, dyspnea, hemoptysis.

Treatment of bronchiectasis may be pharmacological (antibiotics, corticosteroids, mucolytics, bronchodilators), physiotherapy and in some special cases, surgical. The most recent guidelines [4] suggest the importance of Airway Clearance Techniques (ACTs) in patients with stable bronchiectasis: regular twice daily respiratory physiotherapy increases sputum expectoration, dyspnea, and cough symptoms and, in general, quality of life. ACTs have developed in the last thirty years, challenging the idea that secretions move by gravity [5] and showing the value of an expiratory airflow, directed in the cephalic direction and behind the obstruction, to help collect and remove secretions [6].

The most used techniques in patients with bronchiectasis are Active Cycle of Breathing Techniques (ACBT) and oscillating positive expiratory pressure (OPEP), like Flutter and Acapella, which are both effective [4]. In addition, it is recommended to consider gravity assisted positioning to improve the effectiveness of ACTs and the inclusion of the Forced Expiration Technique (FET) or huff for all these techniques. British Thoracic Society guidelines suggest considering patient preference and adherence [7]. It is important to customize the duration and frequency of treatment according to the needs of the patient: however, treatment longer than thirty minutes is not recommended. It is customary to consider the session over until two clear huffs or coughs are completed. This case report adopts an approach based on studies in which the importance of a personalized rehabilitative intervention has been shown [4,8,9]. The combination of several strategies is necessary to allow the displacement of secretions from the distal part of the bronchial tree to the mouth: volume strategies work on distal portions, exploiting the elasticity of the lung parenchyma, while flow strategies are more effective at the proximal level. Therefore, the rehabilitation intervention is built after a careful assessment, which allows you to identify the areas to be treated: the correct modulation of expiratory flows and volumes enables work on the portions most obstructed at that time. The situation may change, so the treatment will change too.

Case Presentation

       Patient History
An Italian 54-year-old female, working as a dental hygienist and leads an active lifestyle; she follows a "Mediterranean" diet. She's not a smoker.
The patient complains about:
a.                   Chronic and productive cough for the whole day, especially in the late morning;
b.                   Hemoptysis during cough in recent months;
c.                    Fatigue in carrying out activities of daily living (ADL), especially during the uphill and/or faster gait.
d.                   Anxious state regarding her condition of chronic cough and the difficulty of having to cough/expectorate in public.

The patient reports an episode of bronchopneumonia in youth, and recurrent bronchitis exacerbations since 2012. From December 2013 she was repeatedly treated, for accentuated bronchitis symptoms, with Levofloxacin and steroids. In March 2014, she was admitted to the Morgagni-Pierantoni Hospital in Forlì for further investigation: she was diagnosed with bronchiectasis using Computed Tomography (CT). On this occasion, the Mycobacterium tuberculosis complex DNA positive has been discovered using the bronchoscopy after a Broncho-Alveolar Lavage (BAL): from April 7th she was treated by anti-tuberculosis therapy, but she was diagnosed with a right pneumothorax two days later and treated by pleural drainage (removed on 21st of April). After discharge, she had repeated episodes of bronchitis exacerbations, with purulent sputum and fever. In June 2015, she had a CT scan which further highlighted the presence of cylindrical bronchiectasis in the left middle lobe, lingula, and lower lobe and signs of atelectasis in both posterior basal regions. Until the end of 2015, the patient had frequent bronchitis with alternation between periods of good health and others of productive cough, fever, and asthenia. In January 2016 a High-Resolution Computed Tomography (HRCT) confirmed the presence of bronchiectasis in the left lingula and lower lobe, and in the right medium and lower lobes too. In addition, it was possible to observe the presence of signs of flogosis and fibrotic rearrangement of the peri-bronchiectasic parenchyma. The patient was first initiated into respiratory physiotherapy, randomly, in January 2017: she had never had any other treatment (Figure 1).

Figure 1: Timeline. Description of relevant dates and timing of the clinical case.

First Physiotherapy evaluation

The patient was first evaluated in February 2017. She had previously carried out a cultural examination showing the presence of Pseudomonas spp. The patient comes walking: she appears alert and attentive. She does not use fixed therapeutic or monitoring devices. This evaluation shows:

a.       No cyanosis
b.       Normal muscle tone and no postural compensation.
c.       Good chest mobility
d.       Nasal breath
e.       No presence of Hoover's sign
f.        No asynchronous thoracoabdominal movements Vital signs:
g.       Respiratory Rate in orthostatic position: 16; in supine position: 20
h.       Heart Rate: 75
i.        O2 Saturation
i.        sitting position 97%
ii.       supine position 96%
iii.      right side bed: 95%
iv.       left side bed: 94% 

All the Pulmonary Function Tests (PFTs) result normal.

Objective information:
A. Chronic and productive cough
B. Dyspnoea exertional (Borg scale = 2; MRC scale = 1)
C. Brown purulent sputum (likely linked to the presence of Pseudomonas spp). Estimated amount: 40 ml/24h
D. Fatigue (Borg scale = 3)
E. No chest pain is found. 

Auscultation: normal breath sounds with a slight left basal decrease, in-expiratory rales prevailing in left lateral decubitus and some expiratory wheezes after coughing are found; It was decided to assess the impact of the disease on the quality of life of the patient through the CAT score, reaching a score of 25. The results of this first evaluation are resumed in the table below (Table 1).

 

Table 1: First evaluation results. Abbreviations: COPD Assessment Test (CAT), Medical Research Council (MRC).

Pharmacological Therapy

Until January 2017 and after the physiotherapy taking-over too, the patient followed a pharmacological treatment, with an average of one cycle every two months, only in cases of relapse. It includes:
                     Antibiotic therapy;
                     Aerosol therapy with Salbutamol sulfate, Beclomethasone, and sodium chloride solution, used to hydrate the secretion.
The patient is used to drink more than two gallons of water a day. For about a year, the woman has been using a device (Relvar) based on fluticasone furoate/vilanterol. This treatment has remained unchanged since the duration of taking charge.

Objectives and First Treatment Program

After this first evaluation, the following objectives have been defined:
a) Short-term objectives: clear the airway easier;
b) Medium-term objectives: ACTs learning;
c) Long-term objectives: proper peripheral ventilation, effective self-treatment. To achieve these goals, the first treatment program is set up. It includes:

       Flutter

It is a very small pipe-shaped aid, consisting of a cone trunk containing a stainless-steel marble, which represents the strength of the system. A positive expiratory pressure (PEP) is produced by the patient, exhaling slowly and completely through the system; Flutter has also been demonstrated to be able to alter the rheological properties of secretions [10]. It is done in sitting position: the patient places the elbows on the table and, with one hand, holds the tool, keeping the mouthpiece between the lips, while with the other holds the cheeks remain firm while allowing the vibrations to be transmitted all at the level of the bronchial tree.

Eltgol

This technique uses slow Functional Residual Capacity (FRC) to Residual Volume (RV) exhalation, then total slow expiration, with open glottis. ELTGOL is performed in lateral decubitus: the lung that reaches the best clearance is the inferolateral lung [11,12]. It's an active-assisted or active technique: active support can be applied with the therapist placed behind the patient, having one hand on the patient's abdomen and the other one on the patient's thorax. The therapist follows the slow expiration, using slow thoracic and abdominal or abdominal only compression to improve the lung deflation. The technique can also be carried out by the patient in full autonomy.

       FET and huff

A maneuver used to move secretions, downstream towards the mouth; this technique should be included in any airway clearance routine [4].

       Cough

A reflex defense mechanism activated by irritating stimuli. In this specific order, these techniques were all performed during a single physiotherapy cycle. In our case, the patient is first supervised twice a week by the physiotherapist to learn and become independent of the implementing rules of treatment. The procedure will be done every day because the effects occur not immediately: secretions collected from the distal part of the bronchial tree during the intervention may also be removed a few hours after treatment.

 

        Follow up and outcome

Follow up have been established at six-monthly intervals. Outcomes evaluated are: type of coughs, dyspnea (assessed by the Borg and Medical Research Council-MRC scales), fatigue (assessed by Borg scale), the respiratory rate in orthostasis and supine position, sputum color and quantity (collected in 24 hours), O2 saturation, CAT questionnaire and number of exacerbations (whereas the patient, in the four months preceding the beginning of physiotherapy, had only had a single exacerbation). The second evaluation, in August 2017, showed the results reported in the table below (Table 2). The patient reports an improvement in exercise tolerance and in expectorating and cough less frequent but more intense: short-term objective has been reached.

 

Table 2: Second evaluation results. Abbreviations: COPD Assessment Test (CAT), Medical Research Council (MRC).

        Readjustment of physiotherapy treatment

At the same time, attention is given to the difficulties faced by the patient in the use of Flutter and her tendency to use ELTGOL and FET. She thinks those are more effective. Depending on the patient's preference, the Flutter is replaced by the Resistive Inspiratory Maneuver (RIM) used to treat the distal lung. This maneuver is usually carried out using a specific device that, placed at the mouth, creates a breath resistance: in our case it was unavailable, so the procedure was conducted slightly parting lips, making resistance as well. This technique is performed in lateral decubitus, to expand the superolateral lung, already expanded because of pleural pressure. After the RIM, a four-seconds apnea allows greater recruitment of alveoli; finally, the patient breaths out up to Functional Residual Capacity (FRC). Immediately after RIM, the patient conducts ELTGOL, beginning from the FRC reached before: the procedure is conducted daily, alternating the two decubits in 10/15 minutes, to make use of pleural pressure differences in terms of volume and flow. In fact, while the RIM is useful for the expansion of the superolateral lung, the ELTGOL allows the maximum inferolateral deflation, so that, by alternating the two decubits, the lung is first inflated and then deflated in order to achieve the best clearance of the airway. The Flutter is still used by the patient but only once or twice a week. The patient started this new program in August 2017. The results of the next two follow up are shown in Table 3.

 

Table 3: Follow up evaluation. Abbreviations: COPD Assessment Test (CAT), Medical Research Council (MRC).

Results

In order to show the achievements of respiratory physiotherapy, all data collected have been shown in some graphs, starting from the first assessment in February 2017 to August 2018. The patient has achieved a good quality of life indicated by a decrease in the CAT score (Figure 2a) and an increase in fatigue and dyspnea according to the BORG and MRC scales. This influences the respiratory rate, which is decreased both in orthostatic and supine positions. The amount of sputum decreased significantly: from 40 ml/24hrs in February 2017 to 15 ml/24hrs in August 2018 (Figure 2b). This impacts the number of exacerbations per year, from the average of a relapse every two months before recovery to just one relapse in 2018 (until August) (Figure 2c). The most significant of all graphs is the last one, which represents the number of exacerbations per annum. The reduction in the number of relapses shows the importance of day-to-day practice, especially about the learning of methods by subject, to encourage the break-up of the vicious circle, which is an integral part of the pathogenesis of bronchiectasis. Despite the remodeling of the first treatment set for February 2017, those results were achieved. However, the use of flow and volume strategies alone has allowed the patient to achieve good mucus ciliary clearance, without causing any worsening of symptoms.

Figure 2a: The decline in CAT score (from 25 in February 2017 to 8 in August 2018) indicates a significant change in the patient's quality of life.

Figure 2b: The reduction in sputum volume is important as it represents a substantial increase in airway clearance.

Figure 2c: The number of exacerbations per year is a long-term outcome of the effectiveness of the physiotherapy treatment.

Discussion

This case report makes us reflect on the importance of defining, through a preliminary assessment, the intervention that is most appropriate for the individual patient, combining the different strategies based on the problem. The patient has been treated basing on a study published by Postiaux [13], describing a "new paradigm" for the treatment of the distal lung. The bronchial tree is ideally divided into four different levels and the ACTs, considered to be equally valid [8], can be used each in a different bronchial compartment.

ACTs must be combined to carry secretions in cephalic direction, from distal airways towards the mouth. Therefore, we talk about airway clearance intervention and not just technique: Flutter and RIM are used for the treatment of the distal airway, ELTGOL acts on the central ones and FET allows to move the secretions which are already more proximally. Finally, the cough removes the secretions through the mouth. In that way, there is a therapeutic continuum without aids, but only using inspiratory and expiratory flows, as described by Postiaux in the abovementioned study.

This type of approach could be useful in compliant patients, as in our case, who are able to carry out the techniques autonomously and then at home, ensuring the necessary therapeutic continuity to achieve the goals. The treatment varies according to the patient's conditions, which are valued daily: auscultation is seen as the critical tool for the first evaluation, during which the presence and the position of secretions are highlighted in order to choose the first technique to begin with. This tool is also useful for a reassessment to show the short and long-term efficacy of the approach used [14].

The results obtained by treating the clinical case show that respiratory physiotherapy is capable to improve patient's quality of life. The use of aids or machines, although indispensable in some diseases, especially in the neuromuscular ones, may not be necessary for compliant patients, if expiratory flows and lung volumes-based approaches are used. This is shown by the results obtained treating the clinical case examined, as an essential reduction of relapses and CAT score. Consideration should be given to the pharmacological treatment remained unchanged: it shows that the ACTs, added to the conventional therapy, are very useful.