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Arcuate ligament vascular compression syndrome in infants and children
Paul Schweizer1, Stephan Berger, Michael Schweizer
1Department of Pediatric Surgery, University of Tübingen, 72076 Tübingen, Germany. profpschweizer@web.de
Insights
Arcuate ligament vascular compression syndrome, a rare cause of recurrent abdominal pain in children, is diagnosed based on clinical symptoms and physical examination. Diagnostic imaging may not always confirm celiac artery compression.
Area of Science:
- Pediatric Surgery
- Vascular Surgery
- Radiology
Background:
- Arcuate ligament vascular compression syndrome is not well-documented in pediatric surgical literature.
- The condition is recognized in vascular surgery, radiology, and orthopedic literature.
Purpose of the Study:
- To present the intraoperative pathological anatomy and treatment principles for 8 pediatric patients with arcuate ligament vascular compression syndrome.
- To highlight the clinical presentation and diagnostic considerations for this rare condition in children.
Main Methods:
- Retrospective analysis of chart records and intraoperative anatomical sketches from 8 pediatric patients.
- Evaluation of preoperative courses and long-term follow-up (3-18 years) using a defined program.
Main Results:
- Diagnosis was suspected in children with chronic recurrent abdominal pain and an epigastric arterial bruit.
- Clinical symptoms were the primary indicators, as imaging techniques like duplex ultrasound and angiography did not consistently confirm celiac axis compression or reduced perfusion.
Conclusions:
- Exclude other causes of recurrent abdominal pain and arterial bruit before surgical intervention.
- While imaging can be helpful, it is not mandatory for diagnosis given the distinct clinical presentation.
- Clinical suspicion is paramount for diagnosing arcuate ligament vascular compression syndrome in pediatric patients.
Background:
Arcuate ligament vascular compression syndrome has not been described previously in the pediatric or pediatric surgical literature. However, it is mentioned in the literature of vascular and general surgery and in journals of radiology and orthopedics. In this review, the intraoperative pathological anatomy and the principles of treatment for 8 children will be presented.
Methods:
The chart records and the anatomical sketches that were documented by the surgeon immediately after each procedure were analyzed retrospectively. In addition, preoperative courses and long-term follow-up (range, 3-18 years) were evaluated by a defined program.
Results:
The diagnosis of celiac artery compression by an arcuate ligament was suspected in children presenting with a history of several years of recurrent acute abdominal pain associated with a typical arterial bruit in the midline of the epigastric region.
Conclusions:
Other diseases with recurrent abdominal pain and an arterial bruit must be excluded before making the decision for an operative intervention. Duplex ultrasound and angiography are possibly helpful tools to establish the respective diagnosis, but in the patients of the present series, these techniques neither confirmed compression of the celiac axis nor demonstrated decreased perfusion of the superior mesenteric artery. However, as the clinical symptoms clearly announce the disease, these diagnostic measures are not mandatory.
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