Danzer Enrico, Gerdes Marsha, Bebbington Michael W, Sutton Leslie N, Melchionni Jeanne, Adzick N Scott, Wilson R Douglas, Johnson Mark P
Center for Fetal Diagnosis and Treatment, Children's Hospital of Philadelphia and University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA.
Fetal Diagn Ther. 2009;25(1):47-53. doi: 10.1159/000197359. Epub 2009 Jan 28.
To evaluate lower extremity neuromotor function (LENF) and short-term ambulatory potential following fetal myelomeningocele (fMMC) closure.
Retrospective chart review of 54 children that underwent fMMC closure at our institution prior to the NIHCD-MOMS trial. Neonatal LENF was compared to predicted function based on spinal lesion level assigned by prenatal ultrasound. Ambulatory status was classified as independent walkers (walks without assistive appliances), assisted walker (requires walking aid), and non-ambulatory (wheelchair bound).
Thoracic, lumbar, and sacral level lesions were present in 4, 44 and 6 fMMC infants, respectively. 31/54 of fMMC children (57.4%; median: 2 levels, range: 1-5) had better than predicted, 13/54 (24.1%) same as predicted and 10/54 (18.5%; median: 1 level, range: 1-2) worse than predicted LENF at birth. At a median follow-up age of 66 months (36-113), 37/54 (69%) walk independently, 13/54 (24%) are assisted walkers, and 4/54 (7%) are wheelchair dependent. The strongest factors predicting a lower likelihood to walk independently were higher-level lesion (>L4, p = 0.001) and the development of clubfoot deformity after fetal intervention (p = 0.026). Despite the observed improved ambulatory status, structured evaluation of coordinative skills revealed that the majority of independent ambulators and all children that require assistive devices to walk experience significant deficits in lower extremity coordination.
We observed that fMMC surgery in this highly selective population results in better than predicted LENF at birth and short-term ambulatory status. However, fMMC toddlers continue to demonstrate deficits in movement coordination that are characteristic for children with spina bifida.
评估胎儿脊髓脊膜膨出(fMMC)闭合术后的下肢神经运动功能(LENF)及短期行走潜力。
对在国立儿童健康与人类发展研究所-胎儿脊髓脊膜膨出研究(NIHCD-MOMS)试验之前于本机构接受fMMC闭合术的54名儿童进行回顾性病历审查。将新生儿LENF与基于产前超声确定的脊柱病变水平所预测的功能进行比较。行走状态分为独立行走者(无需辅助器具行走)、辅助行走者(需要行走辅助)和非行走者(依赖轮椅)。
4例、44例和6例fMMC婴儿分别存在胸段、腰段和骶段水平病变。54例fMMC儿童中,31例(57.4%;中位数:2个节段,范围:1 - 5)出生时的LENF优于预期,13例(24.1%)与预期相同,10例(18.5%;中位数:1个节段,范围:1 - 2)比预期差。在中位随访年龄66个月(36 - 113个月)时,54例中有37例(69%)独立行走,13例(24%)为辅助行走者,4例(7%)依赖轮椅。预测独立行走可能性较低的最强因素是较高节段病变(>L4,p = 0.001)以及胎儿干预后发生马蹄内翻足畸形(p = 0.026)。尽管观察到行走状态有所改善,但对协调技能的结构化评估显示,大多数独立行走者以及所有需要辅助装置行走的儿童在下肢协调方面存在明显缺陷。
我们观察到,在这一高度选择性人群中进行的fMMC手术导致出生时的LENF优于预期且短期行走状态良好。然而,fMMC幼儿在运动协调方面仍存在缺陷,这是脊柱裂儿童的典型特征。