la sindrome del lobo medio fernando maria de benedictis azienda ospedaliero-universitaria...
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La sindrome del lobo medioLa sindrome del lobo medio
fernando maria de benedictisfernando maria de benedictis
Azienda Ospedaliero-Universitaria “Ospedali Riuniti” - AnconaOspedale Pediatrico di Alta Specializzazione “G. Salesi”
Dipartimento di Pediatria
“I have not given the middle lobe syndrome agreat deal of respect ….”
EditorialRespecting the Middle Lobe Syndrome
Rubin, Pediatr Pulmonol 2006;41:803
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 ?
Middle Lobe Syndrome: a 70-year-old story
Brock, 1937 First description of MLS (secondary to TB adenopathy)
GrahamGraham, 1948 First non-TB cases
Paulson, 1949 Description of anatomy of RML bronchus Harper, 1950 Description of involvement of lingula
Bradam, 1966 Role of chronic infection of RML
Culiner, 1966 Role of poor collateral ventilation of RML
Danielson, 1967 Description of familiar cases
Characteristics of RML
Compressed between RUL and RLL
Relative anatomic isolationRelative anatomic isolation
Poor collateral ventilation (incomplete development pores of Kohn and channels of Lambert in in early childhood)
Middle Lobe SyndromePredisposing factors (1)
Characteristics of RML bronchus Acute take-off angle
Narrow diameter
Soft bronchial wall
Surrounded by many lymphnodes
Middle Lobe SyndromePredisposing factors (2)
Middle Lobe SyndromeCauses
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
Intraluminal/extraluminalobstruction
Obstructive type of MLSObstructive type of MLSPathophysiologyPathophysiology
Atelectasis
Blood absorptionof trapped gas
Isolated lobe / segment
Infection / inflammation
Non-obstructive type of MLSNon-obstructive type of MLSPathophysiologyPathophysiology
Usually partialobstruction due to edemaand/or mucous plugging
Atelectasis
Difficulty of the lobe to re-expand
Recurrent pneumonia,bronchiectasis, fibrosisRecurrent pneumonia,bronchiectasis, fibrosis
Middle Lobe SyndromeUnderlying conditions
Always consider associated conditions
Asthma
Primary ciliary dyskinesia
Cystic fibrosis
Immunological disorders
Middle Lobe SyndromeMiddle Lobe SyndromeClinical findingsClinical findings
%
Cough Sputumproduction
Wheezing Recurrentfever
Chest pain Crackles
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 55 children with MLS, mean age 5.5 yrs - Asthma, CF, PCD, Immunodeficit excludedexcluded
Mean duration of symptoms 14.5 months – Mean follow-up for 24 monthsMean duration of symptoms 14.5 months – Mean follow-up for 24 months
0
20
40
60
80
100
Boys Atopy Frequentdyspnea
Sputumproduction
Duration ofsymptoms
MLS Control
Middle lobe syndrome in children with asthmaMiddle lobe syndrome in children with asthma
Sekerel, J Asthma 2004;41:411Sekerel, 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%
The role of timely chest radiograph in diagnosing middle lobe syndrome
Chest X-ray, Chest X-ray, please ! ?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”
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 SyndromeDiagnostic tools (1)
Diagnosing middle lobe syndrome in the real lifeDiagnosing middle lobe syndrome in the real life
0
20
40
60
80
100
%
Physicalexamination
Chest X-Rays HRCT
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;41:411Sekerel, J Asthma 2004;41:411
HRCT scan
Extension and characteristics of parenchymal damage
Bronchiectasis
Patency of RML bronchus
Mediastinal lymph nodes
Middle lobe syndromeDiagnostic tools (2)
%
Total patients No bronchiectasis Bronchiectasis
40/75 (73%)
55 pts
15/55 (27%)
55 children with MLS - Duration of symptoms before presentation from 3 to 48 months 55 children with MLS - Duration of symptoms before presentation from 3 to 48 months HRCT scanHRCT scan performed after an aggressive medical treatment 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 The role of timely intervention in middle lobe syndrome in childrenin children
Priftis, Chest 2005;128:2504Priftis, Chest 2005;128:2504
55 children with MLS, median age 5,5 yrs55 children with MLS, median age 5,5 yrsAggressive timely interventionAggressive timely intervention – Follow-up for 24 months – Follow-up for 24 months
%
No bronchiectasis (n. 40) Bronchiectasis (n. 15)
Response to management
CureCureBetterBetterNo changeNo 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 The role of timely intervention in middle lobe syndrome in childrenin children
Priftis, Chest 2005;128:2504Priftis, Chest 2005;128:2504
Middle lobe syndromeDiagnostic 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
The role of timely intervention in middle lobe syndrome The role of timely intervention in middle lobe syndrome in childrenin children
55 children with MLS, median age 5,5 yrs55 children with MLS, median age 5,5 yrsBronchoscopyBronchoscopy and BAL after radiographyc diagnosis and BAL after radiographyc diagnosis
BAL fluid cellular components Microbiology
36
9 9
3 3 3 3
%
H Influenzae
S Pneumoniae
S aureus
M catarrhalis
P aeruginosa
Mucobacteria
Fungi
5851
1012
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
Conservative treatment
Antibiotics
Chest physiotherapy and postural drainage
Inhaled bronchodilators
Inhaled corticosteroids
Systemic corticosteroids
Mucolytics
Middle lobe syndromeManagement
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 SyndromeInvasive - Surgical management
Middle lobe syndromeOutcome
Usually favourable with conservative treatment
Bronchoscopy may be resolutive
Surgery is rarely required
17 children, mean age at diagnosis 3.3 yrs, mean interval follow-up 6.2 yrs17 children, mean age at diagnosis 3.3 yrs, mean interval follow-up 6.2 yrs
Outcome after right middle lobe syndrome in childrenOutcome after right middle lobe syndrome in children
De Boeck, Chest 1995;108:150De Boeck, Chest 1995;108:150
14/17 had repeated episodes of RML collapse
5/17 had further respiratory symptoms
0
20
40
60
80
100
Total pts Atopy x-Rayabnormalities
Symptomatic Asymptomatic
71%
29%
40% 41%
60%
12%
PFT and PD20 MCH were significantly lower in patients
with ongoing respiratory symptoms
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
“….middle lobe syndrome deserves our respect ”
EditorialRespecting the Middle Lobe Syndrome
Rubin, Pediatr Pulmonol 2006;41:803