Spontaneous bladder rupture in a patient with adult Ehlers-Danlos syndrome without bladder diverticulae

Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders due to mutations of the connective tissue genes. It is characterized by the triad of skin hyperelasticity, joint hypermobility, and connective tissue fragility. A 50-year-old man presented with acute clot hematuria and dysuria with no preceding trauma. He had a background of EDS and chronic lower urinary tract symptoms secondary to benign prostatic hyperplasia. The diagnosis of extraperitoneal bladder rupture was made on imaging. This is the first known reported case of spontaneous bladder rupture in an adult with EDS, although there have been 2 reports of children who spontaneously ruptured a bladder diverticulum. We suggest that patients with EDS and evidence of bladder outlet obstruction should be managed closely. Early surgical intervention may be needed. Care should also be taken so that the bladder is not overdistended during cystoscopic procedures.