'Pediatric Ehlers-Danlos syndrome VI with retroperitoneal Hemorrhage: case report and management strategy'.

Abstract

Ehlers-Danlos syndrome (EDS) encompasses a spectrum of heritable connective tissue disorders, with the Kyphoscoliotic variant representing a rare manifestation. We present a case of an 11-year-old male diagnosed with Ehlers-Danlos Syndrome VI (Kyphoscoliotic Variant) who presented with an acute onset of symptoms suggestive of vascular hemorrhage. Despite the rarity of such presentations in pediatric populations, a multidisciplinary team swiftly intervened and thoroughly evaluated the emergent situation. Imaging studies revealed a retroperitoneal hemorrhage extending from the T10 to lower sacral vertebra, along with a sizable aneurysm closely juxtaposed to the celiac artery. After evaluation, the team opted for conservative management, emphasizing vigilant monitoring and pain management in the intensive care unit. This case underscores the significance of tailored management and research to optimize outcomes and reduce complications in pediatric patients with kyphoscoliotic variant of Ehlers-Danlos syndrome presenting with vascular hemorrhage.