1. La sindrome del lobo medio fernando maria de benedictis Azienda Ospedaliero-Universitaria “Ospedali Riuniti” - Ancona Ospedale Pediatrico di Alta Specializzazione “G. Salesi” Dipartimento di Pediatria

2. “I have not given the middle lobe syndrome a great deal of respect ….” Editorial Respecting the Middle Lobe Syndrome Rubin, Pediatr Pulmonol 2006;41:803

3. The middle lobe syndrome in children is characterized by a spectrum of clinical and radiographic presentations, from persistent to recurrent atelectasis to pneumonitis and bronchiectasis of the right middle lobe and/or lingula Middle Lobe Syndrome: what’s in the name ?

4. Middle Lobe Syndrome: a 70-year-old story Brock, 1937First description of MLS (secondary to TB adenopathy) Graham Graham, 1948First non-TB cases Paulson, 1949Description of anatomy of RML bronchus Harper, 1950Description of involvement of lingula Bradam, 1966Role of chronic infection of RML Culiner, 1966Role of poor collateral ventilation of RML Danielson, 1967Description of familiar cases

5. Characteristics of RML Compressed between RUL and RLL Relative anatomic isolation Poor collateral ventilation (incomplete development pores of Kohn and channels of Lambert in in early childhood) Middle Lobe Syndrome Predisposing factors (1)

6. Characteristics of RML bronchus Acute take-off angle Narrow diameter Soft bronchial wall Surrounded by many lymphnodes Middle Lobe Syndrome Predisposing factors (2)

7. Middle Lobe Syndrome Causes Obstructive - intrabronchial (foreign body, mucosal edema, mucus plugs, bronchial stenosis, bronchiectasis, tumor) - extrabronchial (lymphnodes, tumor, cardiomegaly) Non-obstructive Inflammation, infection The mechanisms may be interactive

8. Intraluminal/extraluminal obstruction Obstructive type of MLS Pathophysiology Atelectasis Blood absorption of trapped gas Isolated lobe / segment

9. Infection / inflammation Non-obstructive type of MLS Pathophysiology Usually partial obstruction due to edema and/or mucous plugging Atelectasis Difficulty of the lobe to re-expand Recurrent pneumonia,bronchiectasis, fibrosis

10. Middle Lobe Syndrome Underlying conditions Always consider associated conditions Asthma Primary ciliary dyskinesia Cystic fibrosis Immunological disorders

11. Middle Lobe Syndrome Clinical findings 65% 29% 7% 100% 71% 7% Priftis, Chest 2005;128:2504 In half of the population, MLS were unnoticed, although symptoms persisted for many months 55 children with MLS, mean age 5.5 yrs - Asthma, CF, PCD, Immunodeficit excluded Mean duration of symptoms 14.5 months – Mean follow-up for 24 months

12. Middle lobe syndrome in children with asthma Sekerel, J Asthma 2004;41:411 22 days % 8 days Persistent asthma symptoms and/or sputum production should alert the physician to complicating MLS !!! 56/3528 (1,6%) asthmatic children with MLS, mean age 6.2 yrs, mean duration of symptoms 22 days, mean follow-up 3.6 yrs 8% 49%

13. The role of timely chest radiograph in diagnosing middle lobe syndrome Chest X-ray, please ! ? “Any postponement in obtaining a chest radiograph in a patient with non-specific, often mild, persistent respiratory symptoms may result in failure to diagnose longstanding MLS”

14. Chest radiograph Blurred right heart border and loss of volume of the RML on P-A view Wedge-shaped density extending from the hilum on L-L view RML collapse secondary to hyperinflation of adjacent lobes on P-A view Middle Lobe Syndrome Diagnostic tools (1)

15. Diagnosing middle lobe syndrome in the real life 5/63 (8%) 43/63 (68%) 28/28 (100%) Only a minority of previously undiagnosed cases had been evaluated with lateral radiograph ! 63 episodes of MLS in asthmatic children with MLS, mean duration of symptoms 22 days Sekerel, J Asthma 2004;41:411

16. How can we differentiate atelectasis from lobar consolidation of RML ? AtelectasisConsolidation Lung volume loss++-Not significant Compensatory emphysema++--- Mediastinum shiftToward lesion-- Diaphragm positionUnilateral elevatedNot significant

17. HRCT scan Extension and characteristics of parenchymal damage Bronchiectasis Patency of RML bronchus Mediastinal lymph nodes Middle lobe syndrome Diagnostic tools (2)

18. 40/75 (73%) 55 pts 15/55 (27%) 55 children with MLS - Duration of symptoms before presentation from 3 to 48 months HRCT scan performed after an aggressive medical treatment There was a positive correlation between the duration of symptoms and the development of bronchiectasis The role of timely intervention in middle lobe syndrome in children Priftis, Chest 2005;128:2504

19. 55 children with MLS, median age 5,5 yrs Aggressive timely intervention – Follow-up for 24 months Response to management CureBetter No change There was a clear association between the presence of bronchiectasis and an unfavorable clinical and radiographic outcome The role of timely intervention in middle lobe syndrome in children Priftis, Chest 2005;128:2504

20. Middle lobe syndrome Diagnostic tools (3) “FOB has been recognized as a useful and safe tool in the investigation of infants and children with airway diseases, including persistent atelectasis” Midulla, ERS Task Force ERJ 2003;22:698 Fibroptic bronchoscopy Patency of the RML bronchus BAL: cells profile and microbiology Biopsy

21. The role of timely intervention in middle lobe syndrome in children 55 children with MLS, median age 5,5 yrs Bronchoscopy and BAL after radiographyc diagnosis BAL fluid cellular components Microbiology 36 9 9 3 3 33 % H Influenzae S Pneumoniae S aureus M catarrhalis P aeruginosa Mucobacteria Fungi 58 51 10 12 3 % Eosinophil Neutrophil Lynphocyte Macrophages Normal MLS is strengthly associated with asthma, and chronic inflammation of the lung is present in more than half of population Over half of the patients have an underlying bacterial infection, although none had clinically diagnosed pneumonia Priftis, Chest 2005;128:2504

22. Conservative treatment Antibiotics Chest physiotherapy and postural drainage Inhaled bronchodilators Inhaled corticosteroids Systemic corticosteroids Mucolytics Middle lobe syndrome Management

23. 1st step: Bronchoscopy Removal of foreign bodies, retained secretions, tumor 2nd step: Surgical resection recurrent atelectasis or failure of RML to re-expand after conservative therapy and bronchoscopy presence of severe bronchiectasis extensive infection / destruction of a lobe or segment refractory to medical therapy Middle Lobe Syndrome Invasive - Surgical management

24. Middle lobe syndrome Outcome Usually favourable with conservative treatment Bronchoscopy may be resolutive Surgery is rarely required

25. 17 children, mean age at diagnosis 3.3 yrs, mean interval follow-up 6.2 yrs Outcome after right middle lobe syndrome in children De Boeck, Chest 1995;108:150 14/17 had repeated episodes of RML collapse 5/17 had further respiratory symptoms 71% 29% 40% 41% 60% 12% PFT and PD20 MCH were significantly lower in patients with ongoing respiratory symptoms

26. Middle Lobe Syndrome: what should we remember ? 1- It is a well defined clinical/radiographic entity 2- Do not trust on physical examination alone 3- In case of suspect, consider timely and “complete” chest radiograph 4- In case of long duration of clinical history, HRCT is mandatory 5- An aggressive, rational, multidisciplinary intervention is often resolutive

27. “….middle lobe syndrome deserves our respect ” Editorial Respecting the Middle Lobe Syndrome Rubin, Pediatr Pulmonol 2006;41:803