de Jong Tom P V M, Chrzan Rafal, Klijn Aart J, Dik Pieter
Pediatric Renal Center, Department of Pediatric Urology, University Children's Hospital, UMCU, P.O. Box 85090, 3508 AB Utrecht, The Netherlands.
Pediatr Nephrol. 2008 Jun;23(6):889-96. doi: 10.1007/s00467-008-0780-7.
Renal damage and renal failure are among the most severe complications of spina bifida. Over the past decades, a comprehensive treatment strategy has been applied that results in minimal renal scaring. In addition, the majority of patients can be dry for urine by the time they go to primary school. To obtain such results, it is mandatory to treat detrusor overactivity from birth onward, as upper urinary tract changes predominantly start in the first months of life. This means that new patients with spina bifida should be treated from birth by clean intermittent catheterization and pharmacological suppression of detrusor overactivity. Urinary tract infections, when present, need aggressive treatment, and in many patients, permanent prophylaxis is indicated. Later in life, therapy can be tailored to urodynamic findings. Children with paralyzed pelvic floor and hence urinary incontinence are routinely offered surgery around the age of 5 years to become dry. Rectus abdominis sling suspension of the bladder neck is the first-choice procedure, with good to excellent results in both male and female patients. In children with detrusor hyperactivity, detrusorectomy can be performed as an alternative for ileocystoplasty provided there is adequate bladder capacity. Wheelchair-bound patients can manage their bladder more easily with a continent catheterizable stoma on top of the bladder. This stoma provides them extra privacy and diminishes parental burden. Bowel management is done by retrograde or antegrade enema therapy. Concerning sexuality, special attention is needed to address expectations of adolescent patients. Sensibility of the glans penis can be restored by surgery in the majority of patients.
肾损害和肾衰竭是脊柱裂最严重的并发症之一。在过去几十年中,已应用了一种综合治疗策略,使肾脏瘢痕形成降至最低。此外,大多数患者在上小学时就能实现尿液干爽。为取得这样的效果,从出生起就必须治疗逼尿肌过度活动,因为上尿路变化主要始于生命的最初几个月。这意味着新诊断的脊柱裂患者应从出生起就通过清洁间歇性导尿和药物抑制逼尿肌过度活动进行治疗。如果存在尿路感染,则需要积极治疗,而且在许多患者中,需要进行长期预防。在患者后期生活中,治疗可根据尿动力学检查结果进行调整。盆底瘫痪因而存在尿失禁的儿童通常在5岁左右接受手术以实现尿液干爽。膀胱颈腹直肌吊带悬吊术是首选手术,对男性和女性患者均有良好至极佳的效果。对于逼尿肌活动亢进的儿童,如果膀胱容量足够,可选择行逼尿肌切除术替代回肠膀胱扩大术。需要使用轮椅的患者在膀胱上方造一个可控贮尿囊后能更轻松地管理膀胱。这个造口为他们提供了更多隐私,并减轻了家长的负担。肠道管理通过逆行或顺行灌肠疗法进行。关于性方面,需要特别关注青少年患者的期望。大多数患者可通过手术恢复阴茎头的感觉。