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]


[Lateral 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 
     view.

     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 
unremarkable.
     A echocardiogram and CT scan of the chest are 
ordered.

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 
left lung.

     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.  

Discussion
     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 
may include:
     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 
artery. 
     3.  Dextrocardia.
     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 
cava. 
     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 
been done.
     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 
pericardium." 
     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.

References
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viscera consisting of  imperfect dvelopment of the right 
lung and transposition of the heart.  London Med Gas 
1836;18:600-601.
     2.  Neill CA, Ferenca C, Sabiston DC.  The familial 
ccurence of hypoplastic right lung with systemic arterial 
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