成人脊柱裂：以純下泌尿道症狀表現之案例報告

1廖家亨、1,3程威銘、2,3范玉華、4陳信宏

1臺北市立聯合醫院忠孝院區外科部泌尿科

2臺北榮民總醫院泌尿部婦幼泌尿科

3國立陽明交通大學醫學院醫學系泌尿學科

4臺北榮民總醫院神經醫學中心神經外科

Adult Spinal Bifida Presenting with Lower Urinary Tract Dysfunction Only: A Case Report

1Chia-Heng Liao, 1,3Weiming Cheng, 2,3Yu-Hwa Fang, 4Shin-Hung Chen

1Division of Urology, Department of Surgery, Taipei City Hospital, Zhongxiao Branch, Taipei, Taiwan

2Division of Female and Pediatric Urology, Department of Urology, Taipei Veterans General Hospital, Taipei, Taiwan

3Department of Urology, Faculty of Medicine, National Yang Ming Chiao Tung University, Taipei, Taiwan

4Division of Pediatric Neurosurgery, Neurological Institute, Taipei Veterans General Hospital, Taipei, Taiwan

Introduction:

Spinal bifida is a congenital disorder of the spine, which is usually discovered during newborn screening in Taiwan. It usually presents with multiple neurological symptoms including backache, lower extremity muscle weakness, and bowel/bladder dysfunction. The symptoms would progress without surgical intervention because of tethered spinal cord. Here we presented a 25-year-old man with spinal bifida who was diagnosed from lower urinary tract symptoms without other neurological symptoms.

Case report:

This 25-year-old male with no known disease history suffered from hesitancy and incomplete emptying for five years. He also reported to have constipation developed at the same time. No urinary frequency, urgency, nocturia, incontinence, erectile dysfunction, lower extremity muscle weakness or numbness accompanied. Physical examination revealed hair growth at sacral region, and neurological examination showed no motor or sensory defect of the lower extremities. Pressure flow study showed detrusor underactivity (BCI=59) and equivocal bladder outlet obstruction (BOOI=31). Voiding cystography showed non-relaxing sphincter during voiding. Renal sonography excluded hydronephrosis while pelvic MRI revealed spinal cord terminating in a lipoma in sacrum and posterior dysraphism of sacrum, compatible with spinal bifida occulta with tethered cord syndrome. The patient was referred to a pediatric neurosurgeon for surgical release. The operation was done uneventfully, and the lower urinary tract symptoms subsided three months after the surgery. Video urodynamic study would be arranged in April for follow-up.

Conclusion:

Spinal bifida with tethered cord syndrome is usually presenting with multiple neurological symptoms. Patients with bowel and bladder dysfunction only is challenging in diagnosis. Urodynamic study is essential to evaluate the involvement of the lower urinary tract and early referral to neurosurgeon is mandatory to prevent disease progression.