stiinte med 1 2012.indd - Academia de Ştiinţe a Moldovei

Ştiinţe Medicale
compression of the unaffected pulmonary structures,
of the mediastinum, which develop the syndrome of
severe respiratory distress [21, 16, 8 , 9, 2, 6, 11].
History
Congenital lobar emphysema was described for
the first time in 1897 by Whithead, it being a discovery
at necropsy [7], and the first researches of clinical
anatomy belong to Peeles and Philp (1932), R. Gross
and E. Lewis (1945), L. Leahz and W. Butsch (1949)
[24]. E. Lewis and W. Potts (1951) have performed
succesful surgical interventions in congenital lobar
emphysema [24, 6, 41, 42]. In 1951, R. Robertson
and E. Jazmes have thoroughly described the
clinical picture of this pathology, defined as „lobar
emphysema” [22].
Congenital lobar emphysema has an incidence
of 1:20,000-30,000 living newborns [6, 24, 17]
and with a proportion of boys⁄girls of 2-3⁄1 [18, 20,
9, 11,21, 16, 17]. It represents ~14% of congenital
bronchopulmonary malformations [20, 21] and 1.4-
2.2% of all the congenital malformations in children
[6, 18, 23].
Scientific literature reports that: the upper left
lobe is affected in 42% of the cases, the medium left
lobe – in 28-35%, the right upper lobe – in 18-20%
and the lower left lobe – in 1% [ 24, 2, 23, 21, 18, 6,
20, 16, 9, 14, 10].
This malformation is associated with anomalies
of other organs and systems in ~11-40% – persistent
arterial duct, interventricular septal defect,
diaphragmatic hernia and renal malformations etc.
[1, 7, 6, 21, 20].
Embriologically, this bronchopulmonary anomaly
is treated as:
• bronchial collapse secondary to a hypoplasia
of bronchial cartilage, dysplastic bronchopulmonary
tissue [6, 1, 8, 3, 20, 21, 9, 24].
• focal obstruction in ~24% of the cases [17] of the
bronchus by a membraneous fold [1, 6, 20, 9, 24].
• neuromuscular dysplasia in ~10% of the cases
[10] of the polialveolar lobe by an overextension of
alveoli [2, 8, 9, 24].
• as extrinsical causes were also described in ~
2% of the cases [16], the compression of the bronchus
by a mediastinal adenopathy, a vascular anomaly
(newborn) or a large blood vessel anomaly of the
azygos vein in elder children or adults [2, 15, 5, 6, 1,
4, 20, 9, 24].
In the same time, in ~50% of the cases, it is
hard to determine the cause of the congenital lobar
emphysema [9].
The valve mechanism, which is the main
pathogenetic mechanism in the development of CLE
is installed as a result of the intrinsic or extrinsic
causes mentioned above [7, 6, 1, 8, 3, 16, 9].
249
According to the researches made by Ю. Исаков
(1978), there are 3 types of structural malformations
of the bronchopulmonary system that lead to the
development of congenital lobar emphysema [22]:
1. Smooth muscle aplasia of the terminal
and respiratory bronchioli, which contributes to
emphysematous modifications of the pulmonary
parenchyma with the thinning of the interalveolar
septi and to the underdevelopment of the vascular
system.
2. Absence of some intermediate structural
elements of the bronchi.
3. Agenesy of the lobar respiratory terminations
– absence of intralobar bronchi, terminal and alveolar
respiratory bronchioli, called micropolicystic lung or
bronchiolar emphysema.
Types 1 and 2 of the bronchopulmonary structural
malformations are considered as genuine congenital
emphysema, while type 3 is considered as false
congenital emphysema [22].
N. Myers (1969) indicates 3 clinical forms of
local congenital emphysema: [20, 21, 23, 24, 13]
Type 1 – in newborns and suckers with an acute
symptomatology.
Type 2 – in small children with an obvious
symptomatology.
Type 3 – in elder children, asymptomatic.
Other clinical studies (Исаков Ю.Ф., Степанов
Э.А., Гераськин В.И.) describe the clinical picture
as [22]:
- With compensatory evolution.
- With undercompensatory evolution.
- With decompensatory evolution.
In ~10-20% of the cases the symptomatology is
present during the first hours⁄days of life, in ~50%
of the cases the diagnosis is established during the
first month of life, in ~20% – until the age of 1 year
old [21, 20, 9, 16], types 2 and 3 are ocasionally
established beginning with the age of 3 years [20, 21].
According to literary sources, the latest cases of CLE
were diagnosed at the age of 15 and 19 years old [20,
11].
The authors report that the 3-11 years later
results of ~90% of the newborns who benefitted from
immediate lobectomy in the newborn period proved
a compensatory growth of the remaining pulmonary
tissue with an excelent prognosis for the life of
these children [20] . Macroscopically, the affected
lobe is extended, smooth, pale-pink coloured.
The hyperinflated lobe doesn’t regain its normal
dimensions at the sectioning of the lobar bronchus or
at its compression.
In ~50% of the cases the histopathological results