Land Sierra D, Gallagher Taryn, Salwi Sanjana R, Reynolds Tom A, Mathew Leny, Oliver Edward R, Weiss Dana A, Flanders Tracy M, Gebb Juliana S, Adzick N Scott, Heuer Gregory G
Richard D. Wood Jr. Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Childs Nerv Syst. 2025 Jul 9;41(1):226. doi: 10.1007/s00381-025-06866-6.
To evaluate the association between lesion volume and postnatal outcomes in patients with closed spina bifida (SB).
Single-center retrospective review of all patients diagnosed with a closed SB evaluated between 2013 and 2023. Prenatal lesion volume < 3 mL was categorized as "no sac," and volume ≥ 3 mL was categorized as "sac."
Of eligible patients, 22 had a sac, and 33 did not. Myelocystocele patients more commonly had an associated saccular lesion than lipomyelomeningoceles (80% vs. 28%, p = 0.02). Lipomyelomeningocele patients demonstrated less prenatal lesion growth compared to myelocystocele patients (6.26 vs. 58.0 mL) over a median of 12.7 and 10.5 weeks, respectively. Patients with a sac had a higher proportion of talipes (40.9% vs. 9.1%; p = 0.007) compared to those without. At 5 years old, a higher proportion of patients with no sac was able to ambulate independently and required less CIC than patients with a sac (80% vs. 42%; 0% vs. 41.7%). VUDS showed a greater frequency of detrusor overactivity (60.0% vs. 45.0%) and abnormal bladder compliance (60.0% vs. 5.0%) in those with a sac compared to those without. Repeat tethered cord release was also more common (30.0% vs. 3.4%) among patients with a sac-associated defect.
Patients with a sac had an increased risk of prenatal talipes, decreased ability to ambulate independently, increased use of CIC, and increased need for repeat tethered cord release compared to patients without a sac. Given numerous associated morbidities reported in this study, longitudinal multidisciplinary follow-up is critical in the care of these patients.
评估隐性脊柱裂（SB）患者的病变体积与产后结局之间的关联。
对2013年至2023年间诊断为隐性SB的所有患者进行单中心回顾性研究。产前病变体积<3 mL分类为“无囊”，体积≥3 mL分类为“有囊”。
符合条件的患者中，22例有囊，33例无囊。脊髓脊膜膨出患者比脂肪瘤型脊髓脊膜膨出患者更常伴有囊状病变（80%对28%，p = 0.02）。脂肪瘤型脊髓脊膜膨出患者与脊髓脊膜膨出患者相比，在分别为12.7周和10.5周的中位时间内，产前病变生长较少（6.26对58.0 mL）。与无囊患者相比，有囊患者马蹄内翻足的比例更高（40.9%对9.1%；p = 0.007）。在5岁时，无囊患者能够独立行走的比例更高，且与有囊患者相比需要的间歇性导尿更少（80%对42%；0%对41.7%）。排尿性膀胱尿道造影显示，有囊患者逼尿肌过度活动（60.0%对45.0%）和膀胱顺应性异常（60.0%对5.0%）的频率高于无囊患者。与囊相关缺陷的患者中，重复脊髓拴系松解术也更常见（30.0%对3.4%）。
与无囊患者相比，有囊患者产前发生马蹄内翻足的风险增加、独立行走能力下降、间歇性导尿的使用增加以及重复脊髓拴系松解术的需求增加。鉴于本研究报告了众多相关的发病情况，纵向多学科随访对这些患者的护理至关重要。
