Open Journal of Pediatrics, 2011, 1, 75-78
doi:10.4236/ojped.2011.14018 Published Online December 2011 ( OJPed
Published Online December 2011 in SciRes.
A rare clinical presentation of pulmonary hypoplasia
Figen Gülen1, Sakine Kar1, Levent Midyat1*, Esen Demir1, Süphan Özyurt2, Arif Ruhi Özyürek3,
Remziye Tanaç1, Naim Ceylan4
1Department of Pediatric Pulmonology-Allerg y, Faculty of Medicine, Ege University, Izmir, Turkey;
2Department of Pediatric Pulmonology-Allergy, Social Security Tepecik Children’s Hospital, Izmir, Turkey;
3Department of Pediatric Cardiology, Faculty of Medicine, Ege Universi ty, Izmir, Turkey;
4Department of Radiology, Faculty of Medicine, Ege University, Izmir, Turkey.
Email: *
Received 7 October 2011; revised 18 November 2011; accepted 24 November 2011.
Pulmonary hypoplasia is a rare congenital anomaly,
which is frequently associated with other congenital
anomalies. Clinical symptoms vary depending on the
other system anomalies and severity of pulmonary
hypoplasia. Although it is usually diagnosed in in-
fancy and childhood, some cases do not show any
symptoms until the adolescent ages. In adolescents
and adults with unilateral hypolucent lung, although
it is seen rarely, pulmonary hypoplasia should always
be kept in mind. In this article, we present a case
with pulmonary hypoplasia who remained asympto-
matic until puberty.
Keywords: Lung; Unilateral; Hypoplasia; Adolescent
Unilateral pulmonary hypoplasia is a rare congenital
anomaly. Although its exact prevalence is not well
known, it is estimated to be seen in about 1 - 2 out of
every 12.000 births. There is a decrease in number or
size of the airways, vessels and alveoli; however, gross
morphology of the lung is essentially unremarkable [1].
Hypoplastic region of the lung becomes small, fibrotic
and unfunctional as a consequence of developmental
delay in the alveolar tissue. Anomalies may be seen in
several systems, especially in the cardiovascular, gastro-
intestinal, urinary and musculoskeletal systems [2-6].
Depending on the severity of hypoplasia, the patients
may either be asymptomatic or may present with severe
respiratory distress, and the disease usually shows its
symptoms in the neonatal, infancy or childhood period
[1]. In this article, a case report of a patient with this
anomaly is presented who remained asymptomatic until
puberty, which is a rare clinical presentation of pulmo-
nary hypoplasia.
14-years-old male patient was admitted to our clinic with
complaints of acute respiratory distress, cough, and chest
pain for one day and with presumptive diagnoses of for-
eign body aspiratio n or endobronchial mass, which were
considered upon finding of total volume loss in the left
lung on posteroanterior chest radiograph. It was noted
that medical and family history of the patient was unre-
markable and he was completely asymptomatic for res-
piratory system and other systems until his admission to
the hospital. On physical examination, reduced respira-
tory sounds on the left hemi-thorax, matity on percus-
sion, systolic murmur of 2/6 intensity on the pulmonary
focus was found in the patient as well as scoliosis and
right medial strabismus that was noted to be congenital.
Volume loss in the left lung, compensatory increase in
aeration of the right lung, mediastin al sh ift to the left and
elevation on the left diaphragm was found on pos-
teroanterior chest radiograph of the patient whose rou-
tine hemogram, biochemical results and PPD (purified
protein derivative) test were normal (Figure 1).
On bronchoscopic examination of the patient, no en-
dobronchial lesion was found, and concentric narrowing
of the left main bronchus and the left lower bronchus
was observed. His respiratory function test showed re-
strictive and reversible obstructive p attern. The left main
pulmonary artery and the left lung was hypoplastic on
thoracic high-resolution computed tomography (CT)
examination. Hypoplasia was found on the left main
pulmonary artery also on thoracic CT and magnetic
resonance (MR) angiographic examinations of the pa-
tient whose GI (gastrointestinal) barium X-ray which
was taken to investigate congen ital diaphragmatic hernia
was normal (Figure 2). Cardiac catheterization of the
patient revealed that the left main pulmonary artery was
hypoplastic and he had no additional cardiac anomaly. It
was decided to give the patient symptomatic treatment
F. Gülen et al. / Open Journal of Pediatrics 1 (2011) 75-78
Figure 1. Hypoplasia of the left lung, compensatory increase
in aeration of the right, mediastinal shift to the left, and eleva-
tion on the left diaphragm on posteroanterior chest radiograph.
and reevaluate him for operation in the case of worsen-
ing symptoms. The patient was discharged with long-
acting beta-2 agon ist treatmen t.
Pulmonary hypoplasia is characterized by reduced
and/or less developed lung tissue to varying degrees [7-
10]. When pathologic specimens of the patients with
pulmonary hypoplasia were examined, it was found that
ratio of lung weight/body weight of the patients was less
than 0.012 [11]. Development of bronchial tree occurs in
26th to 31st days of intrauterine life. Monaldi catego-
rizes developmental disorders of the lung to four catego-
ries [12]. Group I: No bifurcation of trachea; Group II:
Only rudimentary main bronchus; Group III: Uncom-
pleted development after bifurcation of the main bron-
chus; Group IV: Incomplete development of small seg-
ment and subsegmental bronchi of the corresponding
lobe. According to Boyden, developmental disorders are
seen in three different extents [13]: 1) agenesis (com-
plete absence of the lung tissue), 2) aplasia (no lung tis-
sue, but there is a rudimentary bronchus), 3) hypoplasia
(all lung tissues exist but under-developed). The patient
presented in this article is in g roup IV according to Mon-
igure 2. Hypoplasia of the left pulmonary artery and left lung on thorac ic comp uted tomographic angiography.
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F. Gülen et al. / Open Journal of Pediatrics 1 (2011) 75-78 77
aldi classification and is of third degree according to
There are primary (idiopathic) and secondary causes
in the etiology of the pulmonary hypoplasia. It usually
occurs secondary to the intrauterine diseases impairing
normal lung development. Deformities of the chest wall,
pleural effusion associated with hydrops fetalis, cystic
adenomatoid malformation, and diaphragmatic hernia
physically limits the development of the lung. Further-
more, conditions leading to oligohydramnios such as
early rupture of the membranes or fetal renal insuffi-
ciency also impair development of the lungs [1]. Hy-
poplasia may be bilateral in thoracic dystrophies and
oligohydramnios. In such cases, development of the air-
ways and arterial truncation is inhibited and th u s, surface
area required for alveolo-capillary gas exchange be-
comes limited [1,7].
Isolated pulmonary hypoplasia is rare; it is generally
accompanied by anomalies in other systems, especially
in the cardiovascular, gastrointestinal, musculoskeletal
and urogenital systems. Severity of the clinical symp-
toms and timing of their occurrence is related to the de-
gree of hypoplasia [14]. It usually presents with respira-
tory distress or pulmonary hypertension in the newborn
period. Respiratory distress in the first five minutes fol-
lowing birth is typical for these patients, and they usu-
ally require mechanical ventilation. Apgar scores are
usually found to be lower, although babies with com-
pletely normal Apgar scores may also be seen [11,
15-19]. However, as in our patient, patients who remain
asymptomatic until pub erty have also be en reported [7,9 ,
10]. Total atelectasia, pleural effusion, pneumonia, dia-
phragmatic elevation, diaphragmatic hernia, pulmonary
aplasia, and pneumonectomy should be considered in
differential diagnosis of these patients. Postero-anterior
chest radiograph, thoracic CT, CT angiography, MR an-
giography and bronchoscopy may be used for diagnosis
[14]. Some pathologists suggested that radial alveolar
counting (RAC) might be used for confirming the diag-
nosis of hypoplasia [20]. In this method, number of the
alveoli taken from a region between the respiratory
bronchiole and the closest connective tissue is compared
to a normal control sample in the same laboratory. This
rate was found to be reduced in two published studies
[11,15,21-24], although another study did not support
this assumption [18].
Life-span of the patients depends on severity of
anomalies of other systems and degree of the pulmonary
hypoplasia [1]. Mortality has been reported to be high
(71% - 95%) especially in those cases manifesting in the
newborn period [11,25]. Membrane rupture earlier than
25th week of the gestation, presence of severe oligohy-
dramnios (amniotic fluid index < 4) for more than 2
weeks, and preterm labor have been reported to be con-
ditions increasing mortality [25]. Some prenatal inter-
ventions may be attempted to increase fetal lung capac-
ity or to provide lung development, depending on the
underlying etiologic reason. Tocolitics for early mem-
brane rupture, antibiotics, steroids, use of fibrin glue to
correct the lesion transcervically, and consecutive use of
amnio-infusions are methods used currently in several
centers and of which benefits have been reported in
some references [25]. Additionally, other methods in the
postnatal period include mechanical ventilation, provid-
ing ECMO (extracorporeal membrane oxygenation) and
surfactant support, and early initiation of dialysis to
support renal function. No method with proven efficacy
has been found although some surgical techniques are
being tried [25].
Unilateral hypoplasia is a congenital ano maly causing
a wide spectrum of clinical manifestations from mild,
non-specific findings to severe respiratory symptoms.
Our case was presented with nonspecific findings, who
remained asymptomatic until puberty, which is a rare
clinical presentation of pulmonary hypoplasia. In pa-
tients with unilateral hypolucen t lung, although it is seen
rarely, pulmonary hypoplasia should always be kept in
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