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

Abstract

Objective. 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. Methods. 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. Results. 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%). Conclusions. 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.

Clinical anatomy of celiac artery compression syndrome: A review

“…Recently, Alehan and Dogan (2004), Schweizer et al (2005) and Scholbach (2006) have reported pediatric cases of CACS. This may have implications for an embryologic origin of this disease.…”

Clinical and Radiologic Review of Uncommon Cause of Profound Iron Deficiency Anemia: Median Arcuate Ligament Syndrome

“…Diagnosis of MALS 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 (12). However, in our patient; Doppler US of the abdominal vessels showed stenosis of the celiac trunk with an increase in flow velocity on expiration (490 cm/s) and improvement on inspiration.…”

Imaging features of vascular compression in abdomen: Fantasy, phenomenon, or true syndrome

“…A greater than 3:1 ratio of PSV in the celiac artery in expiration compared with the PSV in the abdominal aorta just below the diaphragm is another useful criterion to diagnose MALS. [49] CT angiography plays a major role in establishing diagnosis. Owing to the superior spatial resolution of CT, the median arcuate ligament may be detectable, a thickness of more than 4 mm is considered abnormal [Figure 3].…”