Macedo Antonio, Ottoni Sérgio Leite, Garrone Gilmar, Campelo Taiane Rocha, Aragon Raul Garcia, Correa Renata, Balladares Rafael Jordan, Macedo Emanuelle Lima, Leal da Cruz Marcela
Department of Pediatrics, Federal University of São Paulo, São Paulo, Brazil; Department of Urology, CACAU-NUPEP, São Paulo, Brazil.
Department of Urology, CACAU-NUPEP, São Paulo, Brazil.
J Pediatr Urol. 2025 Apr;21(2):283-288. doi: 10.1016/j.jpurol.2024.07.003. Epub 2024 Jul 14.
In-utero myelomeningocele repair is the gold standard treatment after the publication of the MOMS trial. We have performed a retrospective analysis from our prospective in-utero myelomeningocele closure database (started in 2011), and selected only patients with the incontinent bladder pattern according to the Leal da Cruz categorization (Leal da Cruz, et al. J Urol 2015) to review mid-term clinical outcomes.
We identified 30 patients with leaking pressure under 40 cmH20 (incontinent pattern) at first urodynamic evaluation (UE) from the whole cohort of 129 patients who underwent in-utero myelomeningocele closure. We selected patients with a minimum active follow-up of 48 weeks (4 years) to provide mid-term data. Patients were followed according to the same protocol with the proposal of yearly sonogram and UE. All clinical and radiological data were reviewed.
We found 11 patients, with a mean age of 10.2 years old, median age at diagnosis of 19 weeks, surgery performed at 25.6 weeks and birth at 33.2 weeks. The mean follow-up was 81.73 months (6.81 years). Mean age at first urological evaluation was 5 months, and UE was 5.6 months. Febrile UTI incidence in the whole observation period was 27.3%. The average initial DLPP was 30 cmH2O. 71.4% of the patients had bladder capacity less than 50% of the expected age. Bladder compliance could not be determined in 63.7% of cases due to leakage. A total of 5.7 urodynamic studies per patient were performed. Surgery was recommended for 8 patients and done in 4 (36.3%). Surgery consisted of Macedo catheterizable reservoir and Macedo-Malone ACE, associated with urethral sling (2 patients) and bladder neck closure (2). It took an average of 5 UE before the final surgical decision was confirmed. Last urodynamic study showed persistent leakage and low DLPP in 3 patients, normal bladder pressure in 2 (under CIC and anticholinergics), and 1 patient changed his bladder pattern into a high risk group. All operated patients are fully continent (urinary >4hs) and fecal.
Despite initially presenting a low risk for the most patients, we found surgery in 36.3% (4/11) and if we considered all cases with surgery indication proposed to treat urinary incontinence it would be even higher (72.7%).
自MOMS试验公布以来，宫内脊髓脊膜膨出修补术一直是金标准治疗方法。我们对前瞻性宫内脊髓脊膜膨出闭合数据库（始于2011年）进行了回顾性分析，仅选择根据Leal da Cruz分类（Leal da Cruz等人，《泌尿外科杂志》2015年）确定为膀胱失禁型的患者，以评估中期临床结果。
我们从129例行宫内脊髓脊膜膨出闭合术的患者队列中，确定了30例在首次尿动力学评估（UE）时漏尿压力低于40 cmH20（失禁型）的患者。我们选择了至少有48周（4年）有效随访的患者以提供中期数据。按照相同方案对患者进行随访，建议每年进行超声检查和尿动力学评估。对所有临床和放射学数据进行了审查。
我们发现11例患者，平均年龄10.2岁，诊断时中位年龄19周，手术在25.6周进行，出生在33.2周。平均随访时间为81.73个月（6.81年）。首次泌尿外科评估的平均年龄为5个月，尿动力学评估为5.6个月。整个观察期内发热性尿路感染的发生率为27.3%。初始平均膀胱漏点压为30 cmH2O。71.4%的患者膀胱容量小于预期年龄的50%。由于漏尿，63.7%的病例无法确定膀胱顺应性。每位患者平均进行了5.7次尿动力学检查。建议8例患者手术，4例（36.3%）进行了手术。手术包括Macedo可导尿贮尿囊和Macedo-Malone可控性膀胱造口术，联合尿道悬吊带术（2例）和膀胱颈闭合术（2例）。在最终确定手术决策之前，平均进行了5次尿动力学评估。最后一次尿动力学研究显示，3例患者持续漏尿且膀胱漏点压低，2例（在间歇性导尿和使用抗胆碱能药物情况下）膀胱压力正常，1例患者的膀胱类型转变为高风险组。所有接受手术的患者均完全控尿（尿失禁时间>4小时）和控便。
尽管最初大多数患者风险较低，但我们发现36.3%（4/11）的患者接受了手术，如果我们考虑所有建议治疗尿失禁的手术适应症病例，这一比例甚至会更高（72.7%）。
