Stress urinary incontinence as the presenting complaint of benign joint hypermobility syndrome
A 20-year-old nulliparous woman was referred to the Department of Uro-neurology for unexplained urinary incontinence. She experienced incontinence most often with effort or exertion; however, this was particularly worse during coughing from exacerbations of chronic sinusitis. Symptoms were intrusive and she was requiring to use as many as 10 incontinence pads a day. In addition to this complaint, she also used to experience urinary urgency associated occasionally with incontinence, as well as urinary frequency, visiting the toilet once every 20 minutes in the daytime and waking up four times at night. There was no history of haematuria or dysuria, and she did not experience urinary tract infections.\nShe also reported constipation, and would open her bowels only once every three days and with rare episodes of faecal incontinence. She had symptoms akin to irritable bowel syndrome, with frequent abdominal bloating relieved by passing stools.\nThere was no evident urogenital prolapse. To evaluate her mixed incontinence, she had videourodynamics carried out (Figure ). Screening showed a normal bladder outline which was normally sited and a closed bladder neck at rest. There was evidence for detrusor overactivity during the filling phase. When lying supine, coughing did not lead to any leakage of urine. When standing, asking the patient to cough produced descent of the bladder base with opening of the bladder neck, which led to incontinence. When she was asked to cough during the filling phase, there was evidence of beaking of the bladder neck, demonstrating urethral hypermobility. This was responsible for her stress incontinence. The patient voided a volume of 400 mL with a maximum flow rate (Qmax) of 61 mL/s and no postvoid residual.\nIn view of the finding of urethral hypermobility, she was further asked about a possible disorder of collagen and, indeed, she gave a history of recurrent shoulder joint dislocation. She was seen by a rheumatology consultant and the patient was confirmed to have a BJHS. Diagnosis of this condition is made on clinical grounds using the Brighton criteria which assesses joint extensibility as well as associated symptoms that contribute to the syndrome (Table ). A Beighton score of 4 (incorporated into the Brighton criteria) or more and longstanding generalized arthralgia, contribute major factors on the overall Brighton criteria. Associated symptoms contribute minor factors. A score of two major criteria, one major criteria and two minor criteria, or two minor criteria and an affected first-degree relative determines a diagnosis of BJHS.\nThe patient was found to be hypermobile bilaterally in thumbs, elbows, knees and lumbar spine, giving her a Beighton score of 7 out of 9. Her history was also positive for the major criteria of arthalgia for at least three months in four or more joints, with reported generalized aching and lower back pain. She also fulfilled a minor criterion with her history of recurrent dislocations of the shoulder. Of note, the patient's skin was not considered to be overly stretchable.\nTo understand the cause for her detrusor overactivity, a magnetic resonance imaging of her spine was performed to look for any evidence for spinal cord compression from vertebral subluxation or atlantoaxial dislocation, which may accompany disorders of collagen, and this was normal.\nThe patient is on regular follow-up with the Uro-neurology and Rheumatology Departments. Pelvic floor exercises have been started to strengthen her pelvic floor and prevent stress incontinence, and an antimuscarinic, solifenacin, for her symptoms of urgency. She has been started on laxatives for her constipation. For the management of her joint condition, a home exercise programme was developed by a physiotherapist to improve core stability, balance and muscle strength. She was also given advice on general fitness training to counter the effects of deconditioning. These measures should prevent future injury through dislocation and help to manage arthralgia.