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

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