What is Kommerell diverticulum?
Kommerell diverticulum, a remnant of the left dorsal arch, is located where the left aberrant subclavian artery originates, causing external posterior compression of the esophagus. Patients often become symptomatic in later decades as the arteries become tortuous and aneurismal.
How is aberrant right subclavian artery treated?
A muscle-sparing right thoracotomy was used in the pediatric patients, and a supraclavicular approach was used in the adult. Patients were treated successfully by division of the aberrant right subclavian artery and translocation to the right common carotid artery, without graft interposition.
How do you get an aberrant right subclavian artery?
The aberrant origin of the right subclavian artery is caused by the involution of the right fourth vascular arch and proximal right dorsal aorta and the persistence of the seventh intersegmental artery originating from the proximal descending thoracic aorta, forming the abnormal course of the artery lusoria [5, 6].
Can aberrant right subclavian artery causing dysphagia?
The aberrant right subclavian artery (ARSA) is a rare anatomical variation with a reported prevalence of 0.4% in the general population and 0.2–2.5% in autopsy series [1,2]. Due to its course behind esophagus, it may be a rare cause of dysphagia in patients (also called ‘dysphagia lusoria’) [3].
What causes ARSA heart?
An aberrant right subclavian artery (ARSA) is a rare vascular anomaly that is believed to induce feeding and swallowing difficulties in 20% of the patients, caused by dorsal compression of the esophagus by the anomalous artery.
What is ARSA disease?
ARSA (Arylsulfatase A) is a Protein Coding gene. Diseases associated with ARSA include Metachromatic Leukodystrophy and Metachromatic Leukodystrophy, Juvenile Form. Among its related pathways are Metabolism and Glycosphingolipid metabolism.
What is ARSA test?
Genetics Test Information This is the preferred test to rule-out metachromatic leukodystrophy. Metachromatic leukodystrophy is caused by deficient activity of arylsulfatase A (ARSA) enzyme and is characterized by progressive neurologic changes and leukodystrophy with variable age of onset.
What is left ventricular diverticulum?
Left ventricular diverticulum is defined as an outpouching structure that contains endocardium, myocardium, and pericardium and displays normal contraction. Left ventricular diverticula are distinguished from LV aneurysms, which have fibrous walls and exhibit paradoxical motion.
Is ARSA a disease?
ARSA Gene – Arylsulfatase A Defects in this gene lead to metachromatic leucodystrophy (MLD), a progressive demyelination disease which results in a variety of neurological symptoms and ultimately death.
What is a cardiac diverticulum?
Congenital cardiac diverticulum (CCD) is a very rare congenital malformation characterized by a muscular appendix emerging from the left ventricular apex, rarely from the right ventricle or from both chambers, with clinical manifestations ranging from asymptomatic to life-threatening hemodynamic collapse.
What is an apical diverticulum?
Apical diverticulum is usually associated with midline thoracoabdominal defects and other heart malformations, whereas non-apical diverticulum occurs in isolation [11]. Our patient has the apical diverticulum but there were no other congenital abnormalities found by the computed tomography.
How is Kommerell’s diverticulum repaired?
Surgical repair is commonly done through a lateral thoracotomy, requiring single lung ventilation. We present a case of Kommerell’s diverticulum causing unexpected significant tracheal deviation and difficulty in lung separation with a review of a series of similar cases in our practice.
What is the prevalence of Kommerell’s diverticulum?
Kommerell’s diverticulum is a rare condition that occurs in association with a left aortic arch (LAA) with aberrant right subclavian artery (ARSA) (0.5%-2.0% of the population),1 or a right aortic arch (RAA) with aberrant left subclavian artery (ALSA) (0.05%-0.1%).2 Fewer than 50 cases have been reported in the literature.
What is Kommerell’s Diverticulum with a right-sided aortic arch?
Introduction Kommerell’s diverticulum with a right-sided aortic arch is a rare congenital anomaly. It may be asymptomatic and can be detected as an incidental finding or it can present with symptoms related to compression of mediastinal structures. Rarely it can present with rupture of the diverticulum or aortic dissection.
What are the symptoms of dilatation of Kommerell’s diverticulum?
Dilatation of Kommerell’s diverticulum results in compression of the surrounding structures, such as dysphagia, dyspnea, stridor, wheezing, cough, recurrent pneumonia, obstructive emphysema, or chest pain.12 In our study, 2 patients had dysphagia, and 1 patient had chest discomfort.