Spinal hemorrhages are associated with early neonatal motor function loss in human spina bifida aperta

• Published in: Early human development Vol. 84; no. 7; pp. 423 - 431
• Main Authors: Sival, D.A, Verbeek, R.J, Brouwer, O.F, Sollie, K.M, Bos, A.F, den Dunnen, W.F.A
• Format: Journal Article
• Language: English
• Published: Lausanne Elsevier Ireland Ltd 01.07.2008; New York,NY Elsevier; Amsterdam
• Subjects: Advanced Basic Science; Biological and medical sciences; Biopsy; Embryology: invertebrates and vertebrates. Teratology; Ependymal denudation; Female; Fetal Diseases - physiopathology; Fetal movement; Fundamental and applied biological sciences. Psychology; Gestational Age; Hemorrhage - complications; Histology; Humans; Infant, Newborn; Motor Activity - physiology; Motor neuron; Motor Neuron Disease - etiology; Motor Neuron Disease - pathology; Motor Neurons - pathology; Motor Neurons - physiology; Muscle, Skeletal - pathology; Neonatal and Perinatal Medicine; Pregnancy; Spina bifida; Spina Bifida Cystica - complications; Spina Bifida Cystica - pathology; Spina Bifida Cystica - physiopathology; Spinal Cord - blood supply; Spinal Cord - pathology; Spinal Diseases - complications; Spinal Diseases - pathology; Spinal hemorrhage; MMC; lower motor neuron; H&E; spina bifida aperta; Histology; Motor neuron; LMN; haematoxylin–eosin; Spinal hemorrhage; meningomyelocele; Spina bifida; SBA; CSF; cerebrospinal fluid; Ependymal denudation; Fetal movement; GA; gestational age; Human; Neonatal; Fetat movement; Malformation; Diseases of the osteoarticular system; Early; Spine disease; Congenital disease; Hemorrhage; Spina bifida; Histology; Motor neuron; Spinal hemorrhage; Ependymal denudation
• ISSN: 0378-3782; 1872-6232
• DOI: 10.1016/j.earlhumdev.2007.11.003

Abstract Background In spina bifida aperta (SBA), leg movements caudal to the meningomyelocele are present in utero , but they disappear shortly after birth. It is unclear whether leg movements disappear by impact of the neuro-developmental malformation or by superimposed traumatic damage. If superimposed traumatic damage is involved, targeted fetal intervention could improve motor outcome. Aim To characterize neuromuscular pathology in association with perinatal motor function loss in SBA. Patients/methods In fetal SBA ( n = 8; 16–40 weeks GA), the median time interval between ultrasound registrations of fetal motor behavior and post-mortem histology was 1 week. Histology was assessed cranial, at and caudal to the meningomyelocele and compared with findings in fetal controls ( n = 4). Results Despite fetal movements caudal to the meningomyelocele (5/6), histology indicated muscle fiber alterations (6/6) that concurred with neuro-developmental and traumatic spinal defects [ Neuro-developmental defects: spinal ependymal denudation (3/8), reduced amount of (caspase3-negative) lower motor neurons (LMNs; 8/8), aberrant spinal vascularization (8/8). Traumatic defects: gliosis (7/8), acute/fresh spinal hemorrhages near LMNs (8/8)]. Conclusion In all delivered SBA patients, recent spinal hemorrhages were superimposed upon pre-existing defects. If early therapeutic strategies can prevent these superimposed secondary spinal hemorrhages, motor outcome may improve.