dysphagia lusoria

1. A CASE OF
LUSUS NATURAE
HOD AND PROFESSOR DR.R.SRIDHAR
DR.MATHIYALAGAN 2ND YEAR PG
STANLEY MEDICAL COLLEGE.

2. A 46 year old female, a homemaker
came to our hospital with
C/O
 Cough with expectoration for 1 week duration
 White, mucoid sputum
 H/O difficulty in swallowing for the past 6 months
Intermittent in nature, more to solids than liquids

3.  No H/O haemoptysis
 No H/O loss of appetite present
 No H/O loss of weight present
 No H/O fever
 No H/O voice changes
 No H/O breathlessness
 No H/O trauma
 Normal bowel and bladder habits

4. known case of sht for past 4 years on irregular treatment
Not a known case of DM/BA/PTB/CAD
No family h/o bronchial asthma
no prior h/o of anti tuberculosis drugs
no h/o trauma
menopause attained 2 years back
PAST HISTORY

5. GENERAL PHYSICAL EXAMINATION
Conscious, oriented
Afebrile
No pallor/icteric/cyanosis/clubbing /significant pheripheral
lymphadenopathy /pedal edema
Bp:140/100 mm hg both upper limb
Sp02 : 99% RA
Breast examination :normal
SYSTEMIC EXAMINATION
CVS :S1 S2 heard no murmur
RS :NVBS
PA :soft ,no organomegaly
CLINICAL EXAMINATION

6.  The baseline blood investigations were
 CBC,RFT & LFT – Normal
 BT ,CT - Normal
 ICTC – Non Reactive
 SPUTUM –Negative for AFB
 ECHO –cardiac stable
 Usg abdomen :no significant abnormalities
INVESTIGATIONS

7. DIGITAL CXR

8. RAIDERs
TRIANGLE

9. BARIUM SWALLOW
Barium swallow indicating a
esophageal indentation

10.  Endoscopy: no significant abnormalities

11. ct chest axial view :aberrant right subclavian artery is noted
extending across the midline posterior to the esophagus and trachea
causing mass effect on oesophagus

12. Ct coronal view
RIGHT ABERRANT SUBCLAVIAN
ARTERY FROM AORTIC ARCH
tortuous descending aorta is
noted extending to the right
side of the midline in the
middle third
Ct ANGIOGRAM

13. CT ANGIOGRAM RIGHT ABERRANT SUBCLAVIAN ARTERY
WHICH IS COMPRESSING THE ESOPHAGUS

16.  We report a case of an uncommon type of
dysphagia, due to esophagus compression by an abnormal
right subclavian retro esophageal artery
This condition was first described by Bayford in 1794 as lusus
naturae, meaning a freak or jest of nature, the so called
“Dysphagia Lusoria”.
The Aberrant Right Subclavian Artery (ARSA), also known as
“Arterial Lusoria” (AL), is the most common intra-thoracic
embryologic anomalies involving main arteries, with an
incidence of 0.4% to 2%

17.  Since the patient’s symptoms were mild and intermittent,
without significant effect on her nutritional status, she was
managed conservatively with dietary modifications and
medical therapy with proton pump inhibitor and prokinetic
agent.
 Surgical repair and reconstruction of the aberrant vessel
should be considered if the patient develops severe
dysphagia.

18.  By 5 weeks of fetal development,
the primordial heart tubes have
fused and six aortic (bronchial)
arches have formed between the
ventral roots and dorsal aortae
 During normal development,
persistence of the left fourth aortic
arch forms the arch of the aorta
and proximal left subclavian artery.
 The right fourth aortic arch forms
the innominate and right
subclavian arteries.
 Involution of the distal right aorta
results in an unpaired single aortic
arch
EMBRYOLOGY

19.  The right subclavian
artery formed from
the:
Right 4th aortic
arch
Right dorsal aorta
&
Right 7th
intersegment
artery

The left subclavian
artery formed from
the left 7th
intersegment artery
SUBCLAVIAN ARTERY

20.  CLINICAL CLASSIFICATION:
Vascular rings.
 Non-ring vascular compression.
 Non-compressive arch malformations.
 Ductal dependent arch anomalies-Interrupted
aortic arch , Isolated subclavian,carotid or
innominate arteries.
AORTIC ARCH ANOMALIES
CLASSIFICATION

21.  a)Left arch with retroesophageal Right Subclavian
artery

b) Left aortic arch and retroesophageal
diverticulum of Kommerell

c) Left aortic arch,right. descending
aorta, right .ductus(circumflex aortic arch)

d) Left aortic arch & isolated Right Subclavian artery

e) Left aortic arch with cervical origin of Right
subclavian
LEFT AORTIC ARCH ANOMALIES

22.  Also known as anomalous or aberrant
right
subclavian artery .
 Bayford linked such case with history
of difficulty in swallowing and coined
term
“Dysphagia Lusoria”
 Most common aortic arch anomaly
 Disappearance of Rt 4th arch-distal Rt
dorsal aorta becomes proximal RSCA
forming its retro esophageal portion.
LEFT ARCH WITH RETRO ESOPHAGEAL RIGHT
SUBCLAVIAN ARTERY

23.  clinically most of those individuals with an
aberrant right subclavian artery are asymptomatic.
 who become symptomatic present in bimodal
distribution
 The first presentation is during childhood when
starting solid food, manifesting as recurrent
aspiration pneumonia.
 The second presentation in adulthood might
present as unspecific thoracic pain, dysphagia
(progressive), dyspnea (progressive),
arterioesophageal or arteriotracheal fistulae with
hematemesis or hemoptysis,
and aneurysmal formation with relevant risk of
rupture.

24.  The diagnosis of dysphagia lusoria is difficult as
the symptoms in adult presentation are
nonspecific.
 diagnostic tests such as endoscopy may miss the
diagnosis in 50% of reported cases.
 manometry is not specific in diagnosis or
predicting surgical outcome.
 a barium study of the oesophagus will reveal the
abnormality, but it may miss the diagnosis if
lateral or oblique projections were not used.

25.  The management of the Dysphagia lusoria
depends on the severity of the obstruction.
 Mild severity is treated by reassurance and dietary
modification and education and regular follow up.
.
 those with severe progressive symptoms of
dysphagia or weight loss or with the size of the
diverticulum larger than 2 cm , the risk of rupture
was reported to be high .

this type of severity would require a surgical
therapeutic approach for a cure, such as ligation
and resection of the aberrant vessel with or
without endovascular (hybrid operation)
procedure.

26.  Associations
• as can be expected from embryology, the
right recurrent laryngeal nerve is usually non-
recurrent (that is, enters the larynx directly)
• aneurysmal dilatation aberrant subclavian arterial
aneurysms) of the proximal portion of an aberrant
right subclavian artery can occur, a pouch-like
aneurysmal dilatation is called a diverticulum of
Kommerell
• if there is a retro-oesophageal course
• it can get compressed between the oesophagus
and the vertebra
• the incidence of stenosis/occlusion in this segment
is higher
• it can be associated with trisomy 21 5, trisomy
18 and other chromosomal defects

27.  Dysphagia aggravated by :
 Artery is taut and stretches across the oesophagus
like a bow string
 Vessel become sclerotic and less elastic in the
latter part of life
 Aneurysmal dilatation

28. Asymptomatic patients with type I and II
lusorian artery do not require any treatment.

29.  Type III and IV are primary indications for operative
treatment, whether endovascular, open surgery or combined.
 Supraclavicular approach, left posterolateral thoracotomy,
right posterolateral thoracotomy.
 Acta Chir Belg, 2012, 112, 237-239

30.  A high index of suspicion for the diagnosis of
Dysphagia lusoria should be considered in
asymptomatic or symptomatic patients with right
aortic arch regardless of the severity of the
symptoms.
 The barium esophagram is often suggestive of the
diagnosis, non-invasive angiography in the form of
contrast computed tomography (CT scan) or
magnetic resonance is essential for confirmation
of the diagnosis, follow up and surgical planning.
 Surgical vascular reconstruction is reserved for
those patients with severe progressive symptoms
or those who failed to respond to dietary
modification; however, the type of surgical
procedures will depend on presence or absence of
aortic aneurysm.

31. REFERENCES
 Bayford D. An account of a singular case of obstructed
deglutition. Memoirs Med Soc London 1794;2:275–86.
 Syed et al. Journal of Emergency Medicine, Trauma & Acute
Care 2013:3
 international Scholarly Research Network
ISRN Surgery
Volume 2011, Article ID 819295, 6 pages
doi:10.5402/2011/819295
 Gross RE. Surgical treatment for dysphagia lusoria. Ann Surg
1946;124:532–4
 European Journal of Cardio-Thoracic Surgery, Volume 43,
Issue 2, 1 February 2013, Pages 434–436,
https://doi.org/10.1093/ejcts/ezs498

32. THANK YOU