Multiple surgical interventions for refractory subcostal pain in hypermobile Ehlers-Danlos syndrome: a case report spanning vascular, skeletal, and nerve entrapment diagnoses
https://orcid.org/0009-0004-2979-7946
Talal Ali
https://orcid.org/0009-0007-3841-4806
Diana Deáková
Ilkin Bakirli
https://orcid.org/0000-0003-4902-2668
Marta Ščipáková
Jozef Živčák
https://orcid.org/0000-0003-4316-5107
Jaroslav Majernik
https://orcid.org/0000-0003-1942-0497
Abstract
Refractory subcostal and abdominopelvic pain in undiagnosed hypermobile Ehlers-Danlos syndrome (hEDS) may lead to prolonged diagnostic odysseys and repeated surgery. We report a 48-year-old woman who met the 2017 clinical criteria for hEDS and developed persistent subcostal pain despite multiple operations for gynecologic disease, posterior Nutcracker syndrome, median arcuate ligament syndrome, xiphoid deformity, and slipping rib syndrome; anterior cutaneous nerve entrapment syndrome was later diagnosed. Imaging demonstrated a retro-aortic left renal vein, celiac trunk compression, and hypermobile false ribs. Pain recurred or persisted after each intervention, with only transient improvement after xiphoid resection and initial improvement after rib fixation followed by relapse. This case illustrates a multi-compression/multi-entrapment phenotype in hEDS, in which connective tissue laxity may reduce the durability of anatomically targeted procedures. Early recognition of hEDS should prompt hypermobility-aware assessment and favor conservative, multidisciplinary management before further invasive treatment. Dynamic evaluation may help identify overlapping pain generators more accurately.
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hypermobile Ehlers-Danlos syndrome, Nutcracker syndrome, Dunbar syndrome, Slipping rib syndrome, ACNES, surgical failure, abdominal pain
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