Celiac Artery Compression Syndrome in Children, Adolescents, and Young Adults

Abstract

Abdominal pain is one of the most common conditions in childhood and adolescence. Celiac artery compression syndrome (CACS) is rarely suspected in this population. We present clinical and sonographic features from a series of 59 patients with this disorder. A total of 3449 patients from 0 to 18 years were investigated with 7639 abdominal color duplex sonographic examinations. Celiac artery flow velocity was measured at the branching off from the aorta in inspiration, expiration, and in between. Diagnosis of CACS was made if a greater than 2-fold acceleration of peak systolic flow in the celiac artery compared to the abdominal aorta or a peak systolic velocity greater than 200 cm/s was measured in the mid position and if a variation of flow velocity occurred during respiration. In 22 cases, angiography (21 magnetic resonance angiography and 1 digital subtraction angiography) was used to prove sonographic diagnosis. Fifty-nine patients (among them 81% female) from 22 months to 19 years (clustering between 14 and 17 years) fulfilling color Doppler sonographic criteria of CACS were found, for a prevalence of 1.7%. Their symptoms included, among others, abdominal pain (71%), nausea (29%), thoracic pain (22%), heartburn (17%), weight loss (15%), vomiting (15%), systolic murmur (15%), postprandial accentuation of symptoms (15%), diarrhea (14%), respiratory discomfort (14%), and syncope (12%). Celiac artery compression syndrome is more prevalent than expected, and conditions not usually connected to the disease were found. Alertness to CACS is recommended, especially in patients with a combination of characteristic symptoms. Color Doppler sonography is the modality of choice for diagnosing CACS.