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Journal of the association of physicians of india • vol 63 • january, 2015
59
A Rare Cause of Dysphagia Kommerell’s Diverticulum
Pathik Parikh*, Mukesh Nasa**, Sandeep Patil*, Jatin Patel*, Prasad Bhate*,
Prabha Sawant***
Abstract
A right-sided aortic arch with an aneurysm of the aberrant subclavian artery is a rare disease. We
report a case of Kommerell’s diverticulum of an aberrant left subclavian artery in a patient with a rightsided aortic arch with associated ventricular septal defect. Fewer than 50 cases have been reported in
literature so far. Our patient presented with short duration of dysphagia without any syncope or left
subclavian steal syndrome. The major morbidity was caused by Barrett’s oesophagus with reflux and a
mixed paraoesophageal and hiatal hernia. There was associated psoriasis. An attempt at repair was not
undertaken because of the high operative risk and a small aneurysm. Left thoracotomy for direct repair
of Kommerell’s diverticulum is a simple and safe method.
Introduction
A
right-sided aortic arch first described over 200 years ago 1 is an uncommon
congenital defect of the aorta. It is usually associated with cardiac anomalies.
This anomalous aortic arch remains asymptomatic unless an aneurysmal dilatation
of artery develops, leading to compression of surrounding structures and increased
risk of arterial rupture.This usually occurs at the level of the take-off of an aberrant
left subclavian artery and is known as a Kommerell’s aneurysm. In spite of its rarity,
We here report a similar case of right sided aortic arch with Kommerell’s aneurysm.
Case Report
*
Senior Resident,
Superspeciality Medical
Officer, ***Professor and
Head, Department of
Gastroenterology, Lokmanya
Tilak Municipal Medical College
and General Hospital, Sion,
Mumbai
Received: 23.09.2013;
Accepted: 14.10.2013
**
A 56- year-old woman presented to the outpatient department with dysphagia for
two months. She had more difficulty swallowing solids than liquids and there was
no improvement with any specific posture. She also had history of regurgitation of
gastric contents and retrosternal burning for 6 years. Blackish discolouration of dorsal
surface of elbows, knee, nape and even groin was noted. She denied history of syncope,
claudication-like symptoms, anorexia and weight loss. The blood pressure difference
between the two arms was 20 mm of Hg systolic blood pressure. An upper GI endoscopy
showed circumferential ulcerations in the lower oesophagus with a 7 cm hiatus hernia
with mucosal biopsy showing Barrett’s oesophagus without any dysplasia. A chest
X-ray showed of widened superior mediastinum, with a right-sided aortic knuckle and
narrowing of trachea and left major bronchus (Figure 1). 2- D echo showed hypertensive
heart disease with mild diastolic dysfunction with a small membranous ventricular
septal defect without any evidence of valvular defect. A CT scan of the thorax (Figures
2, 3) was suggestive of a right-sided aortic arch and an aberrant subclavian artery with
an aneurysmally-dilated Kommerell’s pouch measuring 2.6 x 2.6 cm. The left common
carotid was seen arising as the first branch followed by the right common carotid and
lastly the two subclavians. The distal aortic arch was located in prevertebral region
and caused anterior displacement of the trachea and the oesophagus. The superior and
middle 1/3 rd of the oesophagus were located anterolateral to the descending aorta and
was compressed at two places, i.e. between the Kommerell’s pouch and the left common
carotid artery at the level of D2-D3 intervertebral disc, and between the descending
aorta and MPA at D4-D5 vertebral levels. A small periumbilical defect with omental
herniation was noted. A high resolution manometry (Figure 4) was done to evaluate
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Journal of the association of physicians of india • vol 63 • january, 2015
Fig. 3 : The figure shows the diverticulum (Star) with
compression of trachea (arrow) and oesophagus
(Arrow Head). The patient however, had no
respiratory symptoms
Fig.1 : Chest Xray showing widened mediastinum with
displaced trachea (Star)
(A)
Fig. 2 : CECT Thorax suggestive of Right sided Aortic arch
(Arrow). The aorta lies on the right side of the spine
the significance of compression which showed a
high pressure area in midoesophagus corresponding
to the arterial compression, and a hiatus hernia. A
dermatology opinion indicated that the skin lesions
were of psoriasis vulgaris. A cardiothoracic surgery
opinion was sought. In view of a small aneurysm
and high surgical risk conservative management was
advised. The dysphagia of the patient has improved
with treatment of GORD.
Discussion
Kommerell 2 in 1936 reported an aberrant right
subclavian artery originating from the descending
thoracic aorta of a left sided arch and associated with
persistence of a remnant of the right dorsal aorta.
The latter appeared as a diverticulum from which
(B)
Fig. 4 : The figure shows the High Resolution Manometry
(A) Normal, and (B) our patient with area of vascular
compression. The black area shows localised
rise in pressure at the level of mid oesophagus
corresponding to site of extrinsic compression. The
peristaltic wave appears to be normal. There is also
Hiatal Hernia (Arrow)
Journal of the association of physicians of india • vol 63 • january, 2015
61
the aberrant right subclavian artery originated. The
condition as the diverticulum of Kommerell 2 can occur
in in association with a left aortic arch with aberrant
right subclavian artery (0.5–2.0% of the population),
or a right aortic arch with abnormal left subclavian
artery (0.05–0.1%). Fifty percent of right-sided aortic
arches are associated with an aberrant left subclavian
artery. 3 An aberrant right subclavian artery with a left
sided aortic arch is the most common of the anomalies
involving the subclavian artery.
with relief of symptoms. Simple ligation, however,
can lead to ischaemic symptoms, 11 which can be
prevented by re-establishment of the blood flow by
reimplanting the aberrant vessel to the ascending
aorta. 12 Reconstruction of the left subclavian artery
should be done in all patients as in young individuals
this may prevent arm claudication, and in older
patients may avoid the possibility of a subclavian steal
syndrome. 5 Subclavian-to-carotid artery transposition
or a Dacron graft may be used to revascularise the
upper extremity. 13,14 Endoaneurysmorrhaphy is ideal
for small Kommerell’s aneurysms with a normal
descending thoracic aorta.
The adult aortic arch is formed from the fourth
primitive left aortic arch while the right fourth arch
generally disappears. If the left fourth arch disappears
and the right persists, a right aortic arch develops. If
both arches persist, they form a double aortic arch or
a vascular ring encircling the trachea and oesophagus.
There are three main types of right-sided aortic
arch: 4 type I, with mirror-image branching of the
major arteries; type II, with an aberrant subclavian
artery; and type III, with isolation of the subclavian
artery where the subclavian artery is connected to
the pulmonary artery through the ductus arteriosus.
Type I is the most common representing 59% of all
right aortic arches, type II 39.5%, and type III 0.8%. 4
Congenital heart anomalies are seen with these
anomalies namely, tetralogy of Fallot, pulmonary
stenosis with ventricular septal defects, tricuspid
atresia, and truncus arteriosus. They are present
in 75% to 85% of type I and type III 5.A right aortic
arch with tetralogy of Fallot is known as Corvisart’s
disease. Tetralogy of Fallot is the most common heart
anomaly in type I (93%). 5
A deletion in chromosome 22q11 is known to be
associated with a 24% incidence of isolated anomalies
of laterality of branching of the aortic arch. 6
A right-sided aortic arch may be asymptomatic.
In infancy, symptoms are related to congenital heart
anomalies or to compression of mediastinal structures.
In adulthood, symptoms are more often the result
in early atherosclerotic changes of the anomalous
vessels, arterial dissection, or aneurysmal dilatation
of arteries with compression of surrounding structures
causing dysphagia (dysphagia lusoria), dyspnoea,
stridor, wheezing, cough, choking spells, recurrent
pneumonia, obstructive emphysema, or chest pain. 7-9
An aberrant subclavian artery may be located behind
the oesophagus (80%), between the oesophagus and
the trachea (15%), or in front of the trachea (5%). 10
The condition is reported to be associated with 19%
risk of rupture of aneurysm, with 100% mortality.
Dissection of aorta was a more common feature in
another study. Elective surgery for an operative
mortality for Kommerell’s aneurysm carries mortality
of 16.6%. 15 This aggressive treatment is indicated
for aneurysms of 3 cm or greater in diameter.
Division of the artery alone is often associated
Conclusion
Kommerell’s diverticulum is a rare abnormality
associated with abnormal origin of subclavian artery.
The condition may be asymptomatic and mainly
presents with compression of surrounding structures.
The mortality with the complicated ruptured aneurysm
being high, surgical option should be considered.
Acknowledgements
We are thankful to the Dean, Staff Research Society
and our colleagues for the whole-hearted support.
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