Hemoptysis in a 11-Year Old
Radiology Cases in Pediatric Emergency Medicine
Volume 5, Case 13
Linda M. Rosen, MD
Kapiolani Medical Center For Women And Children
University of Hawaii John A. Burns School of Medicine
This is an 11-year old female who presents to the
emergency department with a chief complaint of
coughing up blood. She was spending the night at her
grandmother's house when she was awakened at 5:00
am by a need to cough up mucous she felt was in her
right chest. After repeated coughing she was able to
bring up some mucous plugs and tasted blood. She
then got up, ran to the bathroom and spit out
approximately two tablespoons of bloody mucous.
After this she felt fine but due to concern over the
blood, she was brought to the emergency department.
She had not felt ill upon going to bed that night and
specifically denies fever, headache, rhinnorhea, cough.
She did have some intermittent crampy abdominal pain
for three days previous but had an increased appetite,
attended school and had no vomiting or diarrhea.
There is no history of ill contacts, unusual ingestions,
trauma, bleeding or bruising.
Previous medical history reveals that she had a
history of frequent bronchitis and has often been tired
with poor appetite. Her mother wishes she would put
on weight. She has never been hospitalized. No
surgeries, no medications, no allergies.
Exam: VS: HR 110, RR 24, BP 110/70, Temp
37.0 (TM). She is a cooperative thin girl in no distress.
HEENT exam is unremarkable. There are no nasal or
oral lesions. Chest without increased work of breathing,
nontender, clear to auscultation. Heart regular, grade
II/VI soft systolic murmur along the left sternal border
and a fixed split S2. Abdomen flat, soft, nontender
without hepato/splenomegaly or masses. Skin well
perfused without bruising or petechiae.
A CBC, PT/PTT and chest radiograph are ordered.
The blood work is normal.
View chest radiograph [PA view]
This chest radiograph demonstrates slight
cardiomegaly. There is prominent central pulmonary
vascularity. The volume of the right lung is somewhat
smaller than the left lung. There is a curvilinear density
next to the right heart border. This can be seen on the
PA view, but it is most prominent on the lateral view.
See the curvilinear density pointed out on the lateral
After seeing this chest radiograph you interview the
patient and mother again. Mother is unaware that
patient has had any abnormal heart murmur, she is not
very athletic but has normal exercise tolerance without
dyspnea, orthopnea, chest pain or cyanosis. She had a
normal neonatal course. Family history is
A echocardiogram and CT scan of the chest are
View chest CT.
CT1: This cut of the upper chest shows the right
lung to be smaller than the left. Also note the different
vascular pattern on each side.
CT2: This cut shows that the right lung is bi-lobar as
demonstrated by the bifurcation of the right mainstem
bronchus (arrow), a pattern usually seen only in the
Review of this CT of the chest demonstrates that
this patient has a generally hypoplastic right lung with
abnormal systemic blood supply to the right lower lung
directly from the aorta. There is also an anomalous
pulmonary vein from below the diaphragm draining into
the inferior vena cava. These findings are consistent
with the "Scimitar Syndrome". The echocardiogram
demonstrates an atrial septal defect. The patient
undergoes surgery for correction of her atrial septal
defect and redirection of the abnormal pulmonary vein.
She does well clinically.
Two years after surgery, she presents with a history
of frequent coughing and vague right sided chest pain.
A chest radiograph is obtained.
View chest radiograph.
Her chest radiograph now shows a normal heart
size. The differences in size and vascularity of the right
and left lung are more pronounced.
This patient's findings are consistent with several of
the features of a syndrome variously described as
Scimitar Syndrome, Halasz's Syndrome and Venolobar
Syndrome, the primary feature of which is an abnormal
drainage of a right pulmonary vein into the vena cava.
This abnormal drainage was first described by a British
surgeon, Cooper, in 1836. The abnormal vessel he
described can often be seen on plain chest radiograph
as a curvilinear density along the right heart border.
Because of this appearance, the anomaly was
subsequently named the "scimitar syndrome" by Neill,
et al, in 1960. Although the abnormal venous drainage
is the most recognized anomaly, which has given rise to
the popular name, there is most often a group of
additional associated anomalies of the right lung which
1. Partial agenesis or hypoplasia of the right lung
with bronchial isomerism, and diverticulum or
hypoplasia of the right bronchial system.
2. Hypoplasia or agenesis of the right pulmonary
4. Congenital cardiac malformations.
5. Abnormal systemic blood supply to at least part
of the right lung, most frequently the posterior basal
segment of the lower lobe.
6. Abnormal venous drainage. A vessel emptying
from below into the inferior vena cava is most common
and forms the "scimitar" seen on chest radiographs but
other forms of anomalous pulmonary venous drainage,
sometimes with obstruction, are found.
The etiology of this association of malformations is
unknown but is felt to be due to abnormal development
of the entire right lung bud early in embryogenesis. The
reason for vulnerability of the right lung is not
understood, but the association of malformations has
only been reported to occur in the left lung with extreme
rarity. The scimitar syndrome is known to be more
common in females. It is occassionally familial. The
clinical presentation assumes three basic forms.
Some patients, approximately one fourth, are
symptomatic in the newborn period presenting with
respiratory insufficiency and/or cardiac failure. The
most common underlying physiologic disturbance has
been demonstrated to be pulmonary hypertension
produced by the right lung anomalies. The mechanism
for pulmonary hypertension is most often a large left to
right shunt via the systemic arterial supply to the right
lung. Partially obstructed pulmonary venous return may
contribute to pulmonary hypertension. Additional
symptomatology in the newborn period may be caused
by associated congential heart defects.
Approximately 25% of patients with the scimitar
syndrome have associated cardiac anomalies, most
commonly atrial septal defect, but also intraventricular
defects, coarctation of the aorta, tetralogy of Fallot,
pulmonic stenosis and persistent left superior vena
Surgery is indicated for patients symptomatic during
the newborn period. Its success is dependant on the
nature and severity of the underlying abnormalities.
The general aim of surgery is to reduce any systemic
shunting, and if necessary, to redirect pulmonary
venous return. Complete studies delineating the
vascular and ventilation patterns must be completed in
order to plan appropriate surgery.
The second type of presentation is associated with
recurrent pulmonary infections during young adulthood.
These infections are usually of the right lower lobe
which is the one most likely to have an abnormal
arterial supply and venous drainage. These patients
may require partial lobectomy or occasionally right
pneumonectomy to prevent recurrent infections.
Post-operative prognosis is generally good.
The third presentation, which is farily common, is
that the syndrome is found incidentally in a patient who
has a chest radiograph as in the case presented.
These patients have minimal if any symptoms and may
be managed conservatively. Although our patient was
felt to be somewhat thin and not terribly athletic, she
had only one episode of hemoptysis which may be
attributable to her anomalies. If our patient did not have
an atrial septal defect, surgery would probably not have
There is some overlap between reported cases of
scimitar syndrome and those classified as pulmonary
sequestration (refer to the next case, Case 14 of
Volume 5). As mentioned, the term "Venolobar"
syndrome has also been used to encompass the
vascular and general lung anomlies grouped together.
In a recent review by Woodring et al, in the journal,
Radiographics, the authors use the term "congenital
pulmonary venolobar syndrome" (CPVS). They state
"Major components of CPVS include hypogenetic (ie.,
hypoplastic) lung, partial anomalous pulmonary venous
return (this and the former are two of the most
constantly occurring components), absence of a
pulmonary artery, pulmonary sequestration, systemic
arterialization of the lung, absence of the inferior vena
cava, and accessory diaphragm. Minor components of
CPVS include tracheal trifurcation, eventration and
partial absence of the diaphragm, phrenic cyst,
horseshoe lung, esophageal and gastric lung,
anomalous superior vena cava, and absence of the
Thus the term "scimitar syndrome" may be
misleading. Although the anomalous vein of the
"scimitar" is one of the most consistent findings in this
group of anomalies, it may be absent when the
anomalous drainage takes another anatomic form. The
term "Congenital Pulmonary Venolobar Syndrome
(CPVS)" may be a more rational umbrella for this
grouping of anomalies of the right hemithorax, but not
nearly as catchy as "Scimitar Syndrome". Findings of
any of the aforementioned anomalies should prompt a
complete evaluation for the others. There have been
rare reports of abnormalities of the left lung which
mimic the scimitar syndrome.
1. Cooper G. Case of malformation of the thoracic
viscera consisting of imperfect dvelopment of the right
lung and transposition of the heart. London Med Gas
2. Neill CA, Ferenca C, Sabiston DC. The familial
ccurence of hypoplastic right lung with systemic arterial
supply and venous return, "Scimitar Syndrome". Bull
Johns Hopkins Hosp 1960;107:1-21.
3. Canter CE, Martin TC, Spray TL, Weldon CS,
Stuass AW. Scimitar syndrome in childhood. Am J
4. Haworth SG, Sauer K, Buhlmeyer K. Pulmonary
hypertension in scimitar syndrome in infancy. Br Heart
5. Farnsworth SG Sauerk, Buhlmery K. The
spectrum of scimitar syndrome. J Thorac Cardiovasc
6. Dupuis C, Charaf L, Breviere GM, Abou P.
"Infantile" form of the scimitar syndrome with pulmonary
hypertension. Am J Cardiol 1993;71:1326-1330.
7. Thibault C, Perrault L, Delisle G, Cartier P,
Cloutier A, Houde C, Deslauriers J. Lobectomy in the
treatment of the scimitar syndrome. Ann Thorac Surg
8. Dupuis C, Charaf LAC, Breviere GM, Abou P,.
Remy-Jardin M, Helmius G. The "adult" form of the
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9. Schramel FM, Westermann CJ, Knaepen PJ, van
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Congenital pulmonary venolobar syundrome revisited.
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findings in scimitar syndrome. Am J Roentgenol
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