Surgical treatment of abdominal compression syndromes: The significance of hypermobility‐related disorders

• Published in: American journal of medical genetics. Part C, Seminars in medical genetics Seminars in medical genetics, 2021-12, Vol.187 (4), p.570-578
• Main Authors: Sandmann, Wilhelm, Scholbach, Thomas, Verginis, Konstantinos
• Format: Article
• Language: English
• Subjects: Abdomen; Abdomen - diagnostic imaging; Abdomen - surgery; Case reports; Compression; Disorders; Ehlers-Danlos syndrome; Ehlers-Danlos Syndrome - complications; Gastrointestinal system; Gastrointestinal tract; Genetics; Humans; hypermobile Ehlers–Danlos syndrome; Joint Instability - etiology; Joint Instability - surgery; median arcuate ligament syndrome (Dunbar); Pain; Pelvis; Quality of Life; Retroperitoneum; Signs and symptoms; superior mesenteric artery syndrome (Wilkie); Surgery; Syndrome
• ISSN: 1552-4868; 1552-4876
• DOI: 10.1002/ajmg.c.31949

Case reports and systematic studies of the most common hypermobility‐related disorders, hypermobile Ehlers–Danlos syndrome (hEDS), and hypermobility spectrum disorder (HSD) typically describe gastroenterological symptoms and complaints attributed to structural malfunction, autonomic dysfunction, or inflammation of the gastrointestinal tract. However, abdominal compression syndromes (CS) may also contribute to pain and dysfunction in these individuals and be the leading pathology given symptoms significantly reduce or cease after decompressive surgery. Arising not only in the abdomen and causing pain (median arcuate ligament syndrome [MALS] and superior mesenteric artery syndrome [SMAS]), CS also occur in the retroperitoneum and the pelvis (nutcracker syndrome and May–Thurner syndrome), these latter conditions causing chronic pelvic congestion syndrome (PCS). Here, we report primarily on our experience of the assessment and management of MALS and SMAS in a cohort of cases with a surprising prevalence of HSD and hEDS. To our knowledge, this is the first cohort report of its kind in hEDS, HSD, and CS. We recommend that CS are considered in hEDS and HSD individuals with gastrointestinal and other painful complaints within the “belt” area. These CS can be identified using functional ultrasound duplex examination in experienced hands, and in appropriate cases stabilizing surgery can substantially improve quality of life.