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2003-05-21-09 Kommerell"s diverticulum © Sosa

Kommerell’s diverticulum

Alberto Sosa Olavarría, L. Díaz Guerrero, G. Giugni Chalbaud

Centro de Estudios Ultrasonográficos Perinatales. Maternidad Las Acacias. Valencia. Venezuela.

Introduction: Anomalies of the aortic arch may be associated with congenital heart defects (50%), or present as incidental findings in asymptomatic infants. Coarctation, pseudocoarctation, interruption of the arch aortic, aberrant ramification with or without vascular rings, are some of the anomalies of the aortic arch whose diagnosis is possible by means of the radiological studies, magnetic resonance imaging (MRI) and spiral computed tomography (CT).  Several of these congenital anomalies of the aortic arch have been described in pediatrics and adult patient, while there are few prenatal diagnoses by ultrasound.  

Achiron et al., indicate that the anomalies of the aortic arch require a clear definition of the position of the fetal trachea in relation to the pulmonary artery, the ductus arteriosus and the aortic arch.  In this sense Yoo et al., suggested that the view of cross sectional of the upper fetal mediastinum, that demonstrates the three major vessels: the superior vena cava, aorta and pulmonary artery owed complementary the exhaustive study of the heart fetal. While Yagel et al., in their study defined in an understandable way to undertake this “three vessel and trachea view”, established nomograms of the major vessels and they showed the benefit of their methodology in the precise diagnosis of some great-vessels anomalies.

Aortic diverticulum or Kommerell’s diverticulum is a rare aortic congenital anomaly that is produced as a result of the aberrant origin of the left subclavian artery, that arises of right arch aortic as a fourth branches and passes behind of the esophagus to the left shoulder instead of arising from the right brachiocephalic artery.

This anomaly is a result of regression in the 4th left aortic arch between the left carotid and left subclavian arteries. The left subclavian artery arises from an aortic pouch or diverticulum at the junction of the right aortic arch and the right descending aorta and passes obliquely upward, behind the esophagus, toward the left arm. This anomaly can occur in a number of anomalies of the aortic arch system that can, but do not always, cause symptoms of tracheal or esophageal compression, which they can be declared at the moment of the birth, during the lactation or in the adulthood (dysphagia lusoria).

The vast majority of patients with a vascular ring present with symptoms in infancy or very early in childhood. However, a small number of patients do not manifest symptoms until later in life, and others remain entirely asymptomatic
Common symptoms include laryngeal stridor, cyanosis, respiratory distress, apnea, and/or a characteristic high-pitched, brassy cough. Additional findings include a history of asthma, recurrent pneumonia, or evidence of dysphagia or difficulty with feedings. In some cases, airway symptoms are worsened or aggravated by feedings. Intercostal retractions during respiration are observed in some infants with severe obstruction.
When is associated with a right aortic arch, there is a 5 – 10% incidence of accompanying congenital heart diseases. These include tetralogy of Fallot. Atrial septal defect, ventricular septal defect and coarctation of the aorta.

Case report: The patient (G1) was referred at 34 weeks of gestation for a routine prenatal ultrasonographic study. The study includes the seven images standard utilized for the fetal echocardiographic views, calling the attention the presence of a diverticulum located toward the top end of the thoracic aorta and in the three vessel and trachea view we could observe an anomaly in the position of the major vessels with regard to the trachea.

In the figures 1 and 2 we can observe the normal anatomy of upper mediastinum and the normal ultrasound of the three vessel and trachea view, while in the figures 3 and 4 the ones that show the Kommerell’s aortic diverticulum. The left subclavian artery arises from a diverticulum at the junction of the right aortic arch and the right descending aorta. The figure 5 show the three vessel and trachea view in this case, with loss of relations of the trachea with de major vessels (U shape image).

Figure 1

Figure 2

Figure 3

Figure 4

Figure 5

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