2 MONTH OLD WITH VOMITING

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2 MONTH OLD WITH VOMITING

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Authored By: Faculty and residents Children's Hospital, Radiologist, Children's Health System, Birmingham, Alabama., USA.

Patient: 2 month 1 week 2 day old female

History: 2 month old female with persistent vomiting and diarrhea

Images:

[medium]


Fig. 1: AP CXR


Fig. 2: lateral CXR


Fig. 3: Lateral GI


Fig. 4: AP GI


Fig. 5: Axial


Fig. 6: MRA double arch


Fig. 7: Normal


Fig. 8: Right sided arch with aberrant L subclavian art.


Fig. 9: Double arotic arch


Fig. 10: Pulmonary sling

Images:

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Findings: CXR
Tracheal deviation towards the left

Esophagram
Extrinsic impression on the posterior aspect of the esphagus at the level of the aortic arch. Ap view demonstrated impression on both sides of the esphageal margin. Increased soft tissue of the superior mediastinum and the right trachea greater than left. Mass effect on the left lateral margin fo the esphagus.

DDx: �DIFFERENTIAL DIAGNOSIS

Right aortic arch with mirror image branching

Associated with cyanotic congenital heart disease (CHD)
- Tetralogy of Fallot,
- pulmonary atresia with ventricular septal defect: 25% incidence right arch
- Truncus arteriosus: 30-40% incidence right arch

Double aortic arch with dominant right arch

Left arch often not well seen, atretic segment and not left ligamentum arteriosum maintains fibrous continuity of vascular ring
Characterized by tracheal narrowing, is nearly always symptomatic (stridor)

Left aortic arch with aberrant right subclavian artery

Mirror image of right aortic arch with aberrant LSA
Oblique posterior esophageal indentation from left-inferior to right-superior
Isolated abnormality, incidentally found and usually without airway compression
Rarely symptomatic from esophageal compression (dysphagia lusoria)

Diagnosis: Right sided arch with aberrant left subclavian artery

Discussion:

Right Aortic Arch

General considerations

Recognized by leftward displacement of barium-filled esophagus
Aortic knob is absent from left side
Aorta descends on right
Mirror-image type almost always has associated congenital heart disease, usually Tetralogy of Fallot
Aberrant Left Subclavian type rarely has associated CHD

Most common variety of right arch

Type 1�Mirror Image Type

Associated with congenital heart disease 98% of time
Imaging Findings

No posterior impression on trachea or barium-filled esophagus
Heart is usually abnormal in size or shape
Aorta descends on right

Type ll�Aberrant Left Subclavian

�� Associated with cardiac defects 5-10% of the time

Tetralogy of Fallot most often (71%)

ASD or VSD next most often� (21%)

Coarctation of aorta rarely (7%)

�� Anomalous left subclavian artery (retroesophageal and retrotracheal)

�� Aorta descends on right

�� Imaging Findings -- Right Aortic Arch with Aberrant LSCA
:Posterior impression on trachea and barium-filled esophagus
:Heart is usually normal in size and shape

Diverticulum of Kommerell�(enlargement of the proximal portion of an aberrant proximal right subclavian artery at its site of origin from the descending aorta. Frequently, the term is also used to refer to the focal aortic enlargement near or at the site of origin of an aberrant left subclavian aorta just beyond a right aortic arch.��
[Although named after Dr. Burckhard Friedrich Kommerell
David Bayford (1739�1790) first described this condition, which, in his words, �may be called lusoria, from Lusus Naturae that gives rise to it.� The English translation of the Latin term lusus naturae means freak of nature.]

LOOSE vs TIGHT Vascular ring
The left ductus persists as ligamentum arteriosum, which completes the vascular ring

Left ligamentum arteriosum connects to subclavian artery → loose vascular ring
Left ligamentum arteriosum connects to aortic diverticulum of Kommerell → tight vascular ring

Double Aortic Arch��

o�� General considerations

�� Most common vascular ring

�� Rarely associated with congenital heart disease

�� Vascular ring produces tracheal and/or esophageal compression

�� Passes on both sides of trachea

�� Joins posteriorly behind esophagus

�� Right arch is larger and higher

�� Left arch is smaller and lower

�� Barium swallow shows bilateral impressions on frontal view

�� Posterior impression on lateral view

�� Anatomy

�� Right arch supplies RSCA and RCC

�� Left arch supplies LCC and LSCA

�� Imaging Findings -- Double Aortic Arch

�� Right arch is higher and larger

�� Left arch is lower and smaller

�� Produces reverse S on esophagram on AP

�� On lateral, arches are posterior to esophagus and anterior to trachea

�PULMONARY SLING

Pulmonary sling occurs because of failure of formation of Left 6th aortic arch so there is absence of Left pulmonary artery

The blood to the Left lung comes from an aberrant Left pulmonary artery which arises from Right pulmonary artery and crosses between esophagus and trachea

Bronchial cyst may produce same finding on esophagus/trachea

References: www.learningradiology.com

www.hawaii.edu/.../pediatrics

The following references are from STAT DX:

1.	Cina CS et al: Kommerell's diverticulum and right-sided aortic arch: a cohort study and review of the literature. J Vasc Surg. 39(1):131-9, 2004
2.	Backer CL: Vascular rings and pulmonary artery sling. In: Mavroudis C, Backer CL ed. Pediatric cardiac surgery. 3rd ed. Philadelphia, Mosby. 234-50, 2003
3.	Rosa P et al: Aberrant right subclavian artery syndrome: a case of chronic cough. J Vasc Surg. 37(6):1318-21, 2003
4.	Subramanyan R et al: Vascular rings: an important cause of persistent respiratory symptoms in infants and children. Indian Pediatr. 40(10):951-7, 2003
5.	Donnelly LF et al: Aberrant subclavian arteries: cross-sectional imaging findings in infants and children referred for evaluation of extrinsic airway compression. AJR Am J Roentgenol. 178(5):1269-74, 2002
6.	Lund GK et al: Magnetic resonance imaging of congenital anomalies of the thoracic aorta. In: Taveras J and Ferrucci J. Radiology Vol 2, Chap 31A, 2001
7.	McLeary MS et al: Magnetic resonance imaging of a left circumflex aortic arch and aberrant right subclavian artery: the other vascular ring. Pediatr Radiol. 28(4):263-5, 1998
8.	Katz M et al: Spiral CT and 3D image reconstruction of vascular rings and associated tracheobronchial anomalies. J Comput Assist Tomogr. 19(4):564-8, 1995
9.	Caus T et al: Right-sided aortic arch: surgical treatment of an aneurysm arising from a Kommerell's diverticulum and extending to the descending thoracic aorta with an aberrant left subclavian artery. Cardiovasc Surg. 2(1):110-3, 1994
10.	Kleinman PK et al: Left-sided esophageal indentation in right aortic arch with aberrant left subclavian artery. Radiology. 191(2):565-7, 1994
11.	Turkenburg JL et al: Case report: aneurysm of an aberrant right subclavian artery diagnosed with MR imaging. Clin Radiol. 49(11):837-9, 1994
12.	van Son JA et al: Imaging strategies for vascular rings. Ann Thorac Surg. 57(3):604-10, 1994
13.	McNally PR et al: Dysphagia lusoria caused by persistent right aortic arch with aberrant left subclavian artery and diverticulum of Kommerell. Dig Dis Sci. 37(1):144-9, 1992

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