2017, Volume 33, Number 1, Page(s) 074-076
Congenital Lobar Emphysema with Pulmonary Extramedullary Hematopoiesis
Kishor SURYAWANSHI1, Dhiraj NIKUMBH1, Sudhir SINGHAVI2, Rajshri DAMLE1, Nandkumar DRAVID1
1Department of Pathology, A.C.P.M. Medical College, DHULE, INDIA
2Department of Consultant Paediatric Surgeon, DHULE, INDIA
Keywords: Extramedullary hematopoiesis, Emphysema, Pneumonectomy
Congenital lobar emphysema is a one of the rare variety of congenital
abnormality of the lung causing respiratory distress in newborns and
infants. Herein, we report a case of congenital lobar emphysema in
a 3-week-old female admitted to the pediatric intensive care unit
with severe respiratory distress. CT scan revealed a hyperinflated,
hyperlucent left upper lobe, collapse of the ipsilateral lung and
marked mediastinal shift to the right. The patient underwent
emergency left upper lobectomy with improvement of her condition
in the postoperative period. Histopathological findings confirmed
the clinical diagnosis of congenital lobar emphysema with an unusual
finding of pulmonary extramedullary hematopoiesis. Congenital
lobar emphysema often presents a diagnostic and therapeutic
dilemma and therefore requires a high index of suspicion in neonates
presenting with respiratory distress to avoid morbidity and mortality.
Congenital lobar emphysema (CLE) is a relatively
uncommon congenital lung malformation characterized by
severe progressive hyperinflation of one or more pulmonary
lobes, compression and collapse of the remaining lung
parenchyma and mediastinal shift to the opposite side1
is an important cause of respiratory distress in the neonatal
and infantile period with a high level of mortality and can
result in serious morbidity and disability. CLE is a rare
congenital malformation with a prevalence of 1 in 20000 to
. Radiological investigations including chest X-ray
and CT scan of the thorax are diagnostic modalities.
Though rare, clinical awareness of this entity is important
for early diagnosis and effective surgical treatment in
neonates and infants presenting with respiratory distress.
A 3-week-old female child weighing 2.8 kg and from a low
socioeconomic status family was referred to the pediatric
intensive care unit with severe respiratory distress. The
patient's relatives gave a history of repeated episodes of
difficulty breathing, poor feeding and fever on and off since
birth. She was treated with antibiotics, antipyretics and
nebulization therapy for pneumonitis but there was only
partial improvement each time. Her respiratory distress had
increased for the last 10-12 days and she was referred to the
Pediatric Intensive Care Unit for further management. The
baby had been born at term by normal vaginal delivery at
a primary health center and weighed 2.5 kg at birth. There
was a history of delayed crying. She had been vaccinated
with oral polio vaccine and BCG. On admission, the general examination of the baby showed severe respiratory
distress with a respiratory rate of 90/min, heart rate of 140/
min, and temperature of 39.50C. The baby was cyanosed
and intercostal retraction was present. Respiratory system
examination revealed reduced breath sounds on the left
upper chest with hyper-resonant percussion note on the left
hemithorax. The trachea was shifted to the right. X-ray of
the chest showed a hyperlucent left upper lobe with reduced
lung markings, collapse of the left lower lobe and mediastinal
shift to right with patchy consolidation in the right middle
lobe. CT scan showed hyperinflated, hyperlucent left upper
lobe, collapse of ipsilateral lung and marked mediastinal
shift to right (Figure 1
). The patient underwent emergency
left upper lobectomy with improvement of her condition in
the postoperative period.
Click Here to Zoom
|Figure 1: CT scan showed hyperinflated, hyperlucent left upper
lobe, collapse of ipsilateral lung, and marked mediastinal shift to
Grossly, the received lobectomy specimen measuring
8.5x6.5x2.0 cm appeared overinflated. The external surface
of lung showed hazy pleura, brownish grey in color at
places. The cut section was grayish white with occasional
brownish lobules separated by fibrous septae, soft to firm
in consistency (Figure 2). Microscopically most of the
lung parenchyma revealed lobules with overdistension
(dilatation) of alveolar sacs. Alveolar septae were widened
and edematous and showed congested blood vessels. Focal
areas showed intra-alveolar hemorrhages with pigment
laden macrophages. Focal area showed alveolar lumina
with proteinaceous material, fibrin strands and foci of
extramedullary hematopoiesis consisting of myeloid cells
and megakaryocytes (Figure 3). The section studied from
the left segmental bronchus revealed normal bronchial
cartilage and mucus glands.
Click Here to Zoom
|Figure 2: Lobectomy specimen measuring 8.5x6.5x2.0 cm
appears overinflated. External surface of the lung showed hazy
pleura, brownish grey at places. The cut section was grayish white,
with occasional brownish lobules separated by fibrous septae, soft
to firm in consistency.
Click Here to Zoom
|Figure 3: Microscopically most of the lung parenchyma revealed
lobules with overdistension (dilatation) of the alveolar sacs.
Alveolar septae were widened and edematous and showed
congested blood vessels. Focal areas showed alveolar lumina with
fibrin strands and foci of extramedullary hematopoiesis consisting
of myeloid cells and megakaryocytes (H&E; x400).
Congenital lobar emphysema (CLE) was first described
in 1932 by Nelson. It is a rare disease with an incidence of
1 in 20000 to 30000 births2,3
. CLE is a one of the rare
cystic malformations of lung development characterized
by overinflation of the pulmonary lobe. Hyperinflation of
alveoli within the pulmonary lobe results in mediastinal shift
to the opposite side with compression of the contralateral
lung and collapse of the remaining lung<4>. 90% of cases present before 6 months of age but can occur as late as 5
years. Male children are affected more commonly than
Respiratory distress and dyspnea are the major complaints
followed by recurrent respiratory tract infections and
cyanosis. A review of the literature showed that the left
upper lobe is usually the most frequently affected lobe
followed by the middle and right upper lobe, the lower
lobes being rarely affected. Okazaki and Kumart reported
bilateral involvement of the lung in CLE in their studies6,7. Definite etiological factors could not be identified
in half of the cases. Again in half of the cases, there is
partial bronchial obstruction due to absent, hypoplastic or
immature bronchial cartilage producing ball valve effect
with consequent overinflation, endobronchial obstruction
by exuberant mucosal fold, extrinsic compression by
aberrant bronchi or vascular structures bronchogenic cyst
and rarely congenital CMV infection8. Recently theory
of polyalveosis has been described as a cause of CLE9. In
20% cases, CLE is known to occur with other malformations
and especially cardiac malformations5. The bronchial
cartilage was normal in our case. A definite etiological
factor could not be identified in our case as reported in
various studies and other malformations including cardiac
Extramedullary hematopoiesis (EMH) is a compensatory
phenomenon in hematological diseases with ineffective
hematopoiesis, either due to bone marrow replacement or
hemolytic anemia with ineffective erythropoiesis. EMH
occurs in the liver, spleen and less frequently in the lymph
nodes, lungs, serosal surfaces and the urogenital system10.
Pulmonary EMH has been reported in the literature due to
myeloproliferative disorders, hemolytic anemia, hereditary
spherocytosis and Gaucher's disease11. Ineffective
erythropoiesis forces expansion of the hematopoietic
tissue outside the marrow and leads to hematopoietic
compensatory involvement, mostly in the form of masses
in other regions of the body. A review of the literature
revealed congenital cystic adenomatoid malformations
(CCAM) with EMH in lung but association of CLE with
EMH has not been reported12. Our case had CLE with
EMH without association of any secondary pathology, the
cause of which could not be find out. The presumptive
pathogenetic mechanism and possible cause of EMH may
be chronic hypoxia due to nonfunctional hyperinflated lung
compressing surrounding lung parenchyma and release of
cytokines that recruit progenitor cells to the lung causing
The differential diagnosis includes pneumothorax, cysts, and
diaphragmatic hernia that can be ruled out by the presence
of linear bronchovascular and alveolar markings on chest
x-ray. Other causes include pulmonary sequestration,
CCAM and upper airway obstruction that can be ruled out by ultrasonography, Color Doppler imaging and magnetic
resonance imaging. Prenatal diagnosis of CLE is rare,
possibly because of either its low prevalence in utero or the
increased echogenicity of the lungs that could be too subtle
to be appreciated in utero13.
Acute severe respiratory distress in association with
upper respiratory tract infection occurs in 12% of patients
requiring emergency surgical intervention1. Surgical
excision of the emphysematous lobe is the treatment of
choice in severe respiratory distress with a mortality rate
less than 5%. Mild cases may be managed conservatively
with follow-up of the patient. Surgically treated patients may
have abnormal pulmonary function test results but remain
asymptomatic with normal growth and development.
Review of the literature showed two recent studies of case
series of CLE. In one study, 18 CLE patients were followedup
over a period of 10 years. Male preponderance (55.5%)
with varying degrees of respiratory distress (44.4%) and left
upper lobe involvement were the commonest findings in
this study. No associated congenital anomaly was detected14. In another study, 20 patients were followed-up over
a period of 30 years at two university hospitals. Equal sex
distribution was present while respiratory distress and left
upper lobe involvement were the commonest findings in
this study. Eighteen patients were treated by lobectomy
while in two patients bilobectomy was performed, in
conjunction with bronchogenic cyst resection in one case.
There were no cartilaginous changes or other etiological
factors except for a bronchogenic cyst compressing the
upper lobe in one case5.
Our patient was a female child who presented in a neonatal
period with intermittent and progressive respiratory distress.
Clinical features were confused with pneumonitis in the
early period and pneumothorax later on but characteristic
chest x-ray and CT scan findings ruled out these conditions.
Emergency left upper lobectomy was performed in view of
the severe respiratory distress. Histopathological findings
confirmed CLE with an unusual finding of extramedullary
To conclude, CLE often presents a diagnostic and
therapeutic dilemma and therefore requires a high index of
suspicion in neonates presenting with respiratory distress
to avoid morbidity and mortality.
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