Spinal dysraphism

OBJECTIVE: Spina bifida represents one of the most common birth defects, occurring in approximately 1-2 children per 1000 live births worldwide. The functional level of patients with spina bifida is highly variable and believed to be correlated with the anatomical level of the lesion. The variable clinical picture is well established, but the correlation with anatomical level and intraoperative neuromonitoring (IONM) data has not been investigated. Furthermore, the potential for preserving function beyond the apparent clinical level has also not been investigated...

A caudal cutaneous appendage known as the true human tail is a rare and benign condition. Different classification systems have been established, mostly based on the presence of associated spinal dysraphism. Imaging studies play an important role in detecting the prognosis and developing a management plan. Here, we present a rare case of a true human tail with no underlying spinal dysraphism in a preterm neonate.

OBJECTIVE: This study aimed to ascertain the conus medullaris position by counting the number of ossification centers in the vertebral bodies below the conus medullaris endpoint (N) and assess its utility in screening for closed spinal dysraphism and tethered cord syndrome. METHODS: A total of 900 normal fetuses and 146 fetuses with closed spinal dysraphism or tethered cord syndrome were included in this study. The N values were tallied and compared along the spinal longitudinal plane...

Adolescent idiopathic scoliosis is a commonly encountered condition often diagnosed on screening examination. Underlying, asymptomatic neural axis abnormalities may be present at the time of diagnosis. At certain institutions, total spine MRI is obtained preoperatively to identify these abnormalities. We provide a framework for the radiologist to follow while interpreting these studies. In part 1, we discuss Arnold Chiari malformations, syringomyelia, and the tethered cord. In part 2, we focus on spinal cord tumors, dysraphisms, to include diastematomyelia, and vertebral anomalies...

Adolescent idiopathic scoliosis is a commonly encountered condition often diagnosed on screening examination. Underlying, asymptomatic neural axis abnormalities may be present at the time of diagnosis. At certain institutions, total spine MRI is obtained preoperatively to identify these abnormalities. We provide a framework for the radiologist to follow while interpreting these studies. In part 1, we discuss Arnold Chiari malformations, syringomyelia, and the tethered cord. In part 2, we focus on spinal cord tumors, dysraphisms, to include diastematomyelia, and vertebral anomalies...

Intramedullary spinal capillary hemangioma is a rare occurrence in pediatric patients, and only limited cases have been reported. This study presents the first two cases of spinal capillary hemangioma co-present with retained medullary cord and one case of spinal capillary hemangioma with lumbosacral lipomatous malformation. Previous literature on ten patients with this pathology was reviewed. We speculated pathogenesis, imaging features, and histopathologic findings of the disease.

BACKGROUND: Split cord malformation (SCM) is a rare congenital malformation of the spinal cord in which the cord is split longitudinally. Identification and diagnosis in adulthood is rare, with the majority of cases diagnosed in the paediatric population. Isolated segmental cervical SCM is rarer still. CASE PRESENTATION: Here, the authors present the case of a 26-year-old female who presented with neck pain and longstanding bimanual synkinesis secondary to an isolated type II SCM in the C4-C7 region...

OBJECTIVE: Despite the high prevalence and significant implications of pediatric hydrocephalus, the etiological distribution of pediatric hydrocephalus across the diverse Asian demographic is poorly understood. This study aimed to inform clinical guidelines and public health decisions by identifying the etiological distribution of pediatric hydrocephalus across Asia. METHODS: In this systematic review and meta-analysis, the authors searched EMBASE, MEDLINE, CENTRAL, Global Health, Global Index Medicus, and Scopus, with no language restriction, from inception to January 27, 2023...

OBJECTIVES: To compare differences in bowel-specific quality of life (QOL), overall qQOL, and neurogenic bowel dysfunction (NBD) severity by bowel management program in patients with spina bifida. METHODS: We performed a retrospective cross-sectional study of patients ≥12 years old at our multidisciplinary spina bifida center who completed both a modified Peristeen NBD questionnaire (assessing bowel symptom severity and bowel-specific QOL) and the Patient-Reported Outcomes Measurement Information System Pediatric Global Health questionnaire (assessing overall QOL)...

OBJECTIVES: Our objectives were: (1) to assess the visualization rate of the choroid bar in a consecutive series of 306 first trimester scans; (2) to verify - in this cohort of fetuses - the normalcy of the posterior fossa later in pregnancy; (3) to confirm the non-visualization of the choroid bar in a retrospective series of fetuses with posterior fossa malformations. METHODS: The study include a prospective and a retrospective series. The former includes 306 fetuses undergoing routine obstetric ultrasound at our Unit both in the first and the second trimester over the last 6 months, the latter includes 12 cases of posterior fossa malformations...

Hydrocephalus occurs when the cerebrospinal fluid (CSF) accumulates in the cerebral ventricles. This is due to either obstruction in the CSF flow, decreasing its absorption by the arachnoid villus to the Dural venous sinuses, or increasing production of the CSF. The most disproportionately and severely affected by the disease consequences are African children. This is because of the high incidence of postinfectious hydrocephalus and spinal dysraphism compared with other world children. The health care system in Africa has access to 488 neurosurgeons which represents less than 1% of the global neurosurgeons, thus pediatric hydrocephalus is considered an emerging public health problem in Africa because of the difficulty of the patient's access to proper care...

BACKGROUND: Preoperative anxiety leads to adverse clinical outcomes and long-term maladaptive behavioural changes. The role of intranasal atomised dexmedetomidine and atomised ketamine as premedication to produce sedation and anxiolysis in paediatric neurosurgical patients has not been extensively studied. OBJECTIVE: To study the efficacy of intranasal atomised dexmedetomidine and intranasal atomised ketamine as premedication in producing sedation and facilitating smooth induction in children undergoing spinal dysraphism surgery...

PURPOSE: This study aimed to analyze organ system-based causes and non-organ system-based mechanisms of death (COD, MOD) in people with myelomeningocele (MMC), comparing urological to other COD. METHODS: A retrospective review was performed of 16 institutions in Canada/United States of non-random convenience sample of people with MMC (born > = 1972) using non-parametric statistics. RESULTS: Of 293 deaths (89% shunted hydrocephalus), 12% occurred in infancy, 35% in childhood, and 53% in adulthood (documented COD: 74%)...

INTRODUCTION: Conventionally the split appendix has been used to address the need for dual conduits such as the Mitrofanoff and the ACE, however limited by its length. We present a video demonstration of an alternate solution. MATERIAL AND METHODS: Size-12 Nelaton catheter introduced via appendicular stump and skirted along the lateral wall of the caecum. Ethicon Endopath 35 mm Linear Stapler was used to create a caecal tube of about 3 cm based on the appendicular stump...

A posterior midline cutaneous lesion known as a faun tail nevus or aberrant lumbar hypertrichosis is significant to doctors because it may serve as a cutaneous signal for an underlying spinal cord and spine abnormalities. We describe a 17-year-old child who, since infancy, has had excessive hair development over his lumbosacral area. The lower spinal cord was affected by a related spinal abnormality. Clinical evidence was used to make the diagnosis. The patient presented with complaints of asymmetric monoparesis which on clinical examination and radiological investigations was found to be cauda equina syndrome secondary to spina bifida occulta...

OBJECTIVES: To assess the effectiveness of subtemporal decompression in the management of slit ventricle syndrome. METHODS: We conducted a retrospective review of all patients with slit ventricle syndrome (SVS) who underwent subtemporal decompression (STD) at our centre between 2010 and 2021. Cases were identified using the hospital database. Medical records for each patient were reviewed, including operative and radiological reports. RESULTS: Fifteen patients underwent STD for the management of SVS...

OBJECTIVE: To evaluate the prenatal diagnosis of closed dysraphism (CD) and its correlation with postnatal findings and neonatal adverse outcomes. METHODS: A retrospective cohort study including pregnancies diagsnosed with fetal CD by prenatal ultrasound (US) and magnetic resonance imaging (MRI) at a single tertiary center between September 2011 and July 2021. RESULTS: CD was diagnosed prenatally and confirmed postnatally in 12 fetuses. The mean gestational age of prenatal imaging was 24...

BACKGROUND AND OBJECTIVES: Low- and middle-income countries (LMICs) face higher incidences and burdens of care for neural tube defects (NTDs) and hydrocephalus compared with high-income countries (HICs), in part due to limited access to neurosurgical intervention. In this scoping review, we aim to integrate studies on prenatal care, counseling, and surgical management for families of children with spinal dysraphism and hydrocephalus in LMICs and HICs. METHODS: PubMed, Embase, Global Index Medicus, and Web of Science electronic databases were searched for English language articles pertaining to prenatal care, counseling, and surgical management for families of children with spinal dysraphism and hydrocephalus in HICs and LMICs...

We present a case of a two-year-old male with a history of congenital scoliosis and anterolateral thoracic meningocele. He was able to walk and run, but his parents reported left leg weakness and a frequent cough. The patient had normal neurological examination findings. Magnetic resonance imaging (MRI) of the spine without contrast showed left convex upper thoracic congenital scoliosis and rightward anterolateral meningocele inferiorly to T3, with the spinal cord tethered at this location. Neurosurgical cord detethering and repair of the meningocele were performed simultaneously with scoliosis repair by orthopedics...

No abstract text is available yet for this article.