Cord Compression In Children Research Articles

Two pediatric observations of spinal extradural arteriovenous fistulas presenting with epidural hemorrhages and cord compression.

We report two cases of acute spinal cord compression in children with low-flow spinal epidural arteriovenous fistulas (SEAVFs) and discuss the clinical presentation and management of these vascular anomalies. While most low-flow SEAVFs without radiculomedullary drainage are benign lesions typically diagnosed incidentally, we suggest that asymptomatic lesions may warrant aggressive management in specific circumstances, including lesions diagnosed at an early age or in patients under anticoagulation therapy. Our observations also emphasize that patients with a "spontaneous" epidural hemorrhage should undergo dedicated preoperative or postoperative vascular imaging to identify a possible underlying vascular anomaly.

Symptomatic malignant spinal cord compression in children: a single-center experience

BackgroundMalignant spinal cord compression (MSCC) is associated withpoor prognosis and may lead to permanent paralysis, sensory loss, and sphincter dysfunction. Very limited data are available on incidence and etiology of MSCC in pediatric population. We aimed to examine etiology, clinical presentation and treatment of pediatric patient with MSCC admitted to the Santobono-Pausilipon Children’s Hospital, Naples, Italy.MethodsForty-four children under 18 yearsadmitedsince 2007 and assessed for MSCC clinical presentations, evaluation, and treatment.were retrospectively collected from our institutional pediatric oncology and neurosurgery database.ResultsThe median age at time of MSCC diagnosis was 52 months, with a peak in young (≤3 years) patients. The leading cause of MSCC was extramedullary tumors (63.6%), in particular neuroblastoma (27.2%) followed by Ewing sarcomas (15.9%). Cord compression was the presenting feature of a new malignancy in 33 (75%) patients, and a consequence of metastatic disease progression or relapse in the remaining 11 (25%) patients. Motor deficit was the initial symptoms of spinal compression in all patients, while pain was present in about 60% of patients, followed by sphincteric deficit (43.2%). The primary tumor site was located in the neck in 3 (6.8%) patients, thorax in 16 (36.4%), cervico-thoracic region in 3 (6.8%), thoraco-lumbar region in 8 (18.2%), abdomen in 5 (11.4%), lumbar-sacral region in 7 (15.9%) and thoracic-lumbar-sacral region in 1 (2.3%). The median length of the interval between symptom onset and tumor diagnosis varied widely from 0 to 360 days in the entire population, however this interval was significantly shorter in patients with known neoplasia in comparisonto patients with new diagnosis (at relapse 7 days [interquartile range 3–10] vs at diagnosis 23 days [7–60]). Pre and post-operative spine magnetic resonance imagingwas performed in all cases, and most(95%) patients underwent neurosurgical treatment as first treatment. Severe motor deficit was associated with younger age and severe motor deficit at diagnosis was associated withworst motor outcomes at discharge from neurosurgery. Patients with progression or relapsed disease showed a worst prognosis, while the majority of patients (70.5%) were alive at 5 years after diagnosis.ConclusionsThe natural history of MSCC in children is associated to permanent paralysis, sensory loss, and sphincter dysfunction, thus prompt diagnosis and correct management are needed to minimize morbidity. Treatment strategies differed widely among cancer types and study groups in the absence of optimal evidence-based treatment guidelines. When the diagnosis is uncertain, surgery provides an opportunity to biopsy the lesion in addition to treating the mass.

Recurrent spontaneous spinal epidural hematoma in children: case report

Spontaneous spinal epidural hematomas are a rare cause of spinal cord compression in children. These are typically isolated events and could be associated to hemophilia. Symptoms typically include a sudden onset of back pain followed by neurologic deterioration including weakness, numbness, and incontinence. Recurrent spontaneous spinal epidural hematomas have been reported only in two instances in adults. There has been one report of recurrent spontaneous spinal epidural hematomas involving a child. This case report details the case of a 9-year-old female with a recurrent spontaneous spinal epidural hematomas who presented with one episode of lower extremity paresis and 2 years after first episode developed a recurrence hematoma causing cervical pain without motor deficit. The patient was operated and had a good outcome. The authors described this rare case.

Chronic spontaneous cervicothoracic epidural hematoma in an 8-month-old infant

Spontaneous spinal epidural hematoma is an uncommon cause of cord compression in children, especially in infants. An 8-month-old infant was admitted to our hospital for a 40-day history of paraparesis in the lower extremities. This rapidly progressed to paraparesis with an inability to move the lower extremities. MRI of the cervicothoracic spine revealed an epidural mass with compression of the spinal cord. The infant underwent C7-T3 total laminectomies. The pathology and postoperative MRI confirmed spinal epidural hematoma from a vascular malformation. We present the case to highlight the significance of recognizing this chronic spontaneous spinal epidural hematoma and discuss the diagnosis, treatment options and prognosis.

Spinal cord compression in children with Wilms’ tumour

To document the clinical, imaging and histopathological features of five children with paraplegia due to Wilms' tumour (WT), highlighting therapeutic options and patient outcome in a developing country. Patients with WT and paraplegia seen at the Department of Paediatric Surgery since 1984 form the study cohort. Patient demographics, duration of neurological symptoms, stage of primary tumour, therapeutic intervention and outcome were recorded. Histology of the primary tumours and paraspinal or epidural biopsies were reviewed. Five patients with WT and paraplegia were identified. Imaging showed epidural masses with paraspinal disease, cord displacement and compression. Four patients have died. Of the two patients with neurological recovery, one relapsed 4 months later. Histology revealed triphasic WT with one case showing anaplasia. Paraspinal or epidural biopsies confirmed WT with post-treatment changes. Three biopsies showed lymphovascular, perineurial and intraneural tumour invasion and one showed epidural venous invasion. Although rare, WT-associated spinal disease may cause permanent neurological deficit, adding considerably to the burden of disease. In developing countries where patients present late, the prognosis is poor, however surgery may provide immediate relief of compression symptoms and biopsy material. The treatment of choice will depend on the facilities available and the clinical circumstances.

Diagnostics of an Acute Vertebromedullar Insufficiency Syndrome in Children Considering a Stage of Pathological Process

Objective. The analysis of diagnostics efficiency of the spine and spinal cord lesions in children. Material and Methods. A retrospective analysis of 622 cases of stable spine lesions without spinal cord compression in children was performed. Sensitivity and specificity of clinical, radiological and neurophysiologic methods of diagnostics were estimated at each stage of pathological process. The importance of each diagnostic method at various stages was evaluated. Results. A tendency to specificity enhancement of clinical and radiological examinations, growth of prognostic value of positive results and decline in importance of negative results with time was revaled. Sensitivity and specificity of primary clinical examination results are rather high, but prognostic value of positive and negative results is low. Time dependence factor is well-defined at diagnosis of spinal cord lesions. Conclusion. To determine a degree of lesion the data of clinical and radiological examinations are more valuable at clinical subcompensated and decompensated stages, additional methods (MRI, ENMG) are more important at the stage of clinical compensation.

An unusual presentation of thoracic cord compression by a dorsal arachnoid cyst in a 14-month-old boy. A discussion of the case and review of the literature

Spinal arachnoid cysts are a rare cause of spinal cord compression in children and presenting symptoms may be subtle. We present a neurologically intact 14-month-old boy who presented with pain and postural irritability from a thoracic arachnoid cyst.

Is anatomical distribution helpful for differentiating TB spondylitis from neoplastic causes of extradural spinal cord compression in children? A pilot study

Background. Extradural spinal cord compression in children may be the result of infection/inflammation or neoplasia. It is vital to differentiate between the two as there is considerable difference in the management of these entities. Objective. The aim of this paper is to determine whether there are significant differences between TB spondylitis and neoplasia causing extradural compression of the cord with regard to the anatomical distribution and compartmentalisation. Materials and methods. A group of 16 children between the ages of 4 months and 14.2 years who had magnetic resonance (MR) evaluation of the spine consecutively were analysed retrospectively. Results. A wide variety of neoplasms demonstrated an anatomical site and compartment on MRI that s ‘characteristic\' of TB spondylitis. Conclusion. Anatomical distribution alone does not assist in narrowing the differential diagnosis. There is still need for biopsy in children with extradural compression by a mass. South African Journal of Radiology Vol. 11 (4) 2007: pp. 80-84

Noncommunicating spinal extradural arachnoid cyst causing spinal cord compression in a child

Extradural arachnoid cysts in the spine are relatively uncommon causes of spinal cord compression in the pediatric population that are thought to arise from congenital defects in the dura mater. Most reports describe such cysts communicating with the intrathecal subarachnoid space through a small defect in the dura. The authors describe the case of a child who presented with spinal cord compression caused by a large spinal extradural arachnoid cyst that did not communicate with the intradural subarachnoid space. An 11-year-old girl presented with urinary urgency, progressive lower-extremity weakness, myelopathy, and severe gait ataxia. Magnetic resonance imaging of the spine demonstrated a large extradural arachnoid cyst extending from T-8 to T-12. The patient underwent a thoracic laminoplasty for en bloc resection of the spinal extradural arachnoid cyst. Intraoperatively, the dura was intact and there was no evidence of communication into the intradural subarachnoid space. Postoperatively, the patient's motor strength and ambulation improved immediately, and no subsequent cerebrospinal fluid leak occurred. Noncommunicating spinal extradural arachnoid cysts are extremely rare lesions that can cause spinal cord compression in children. Because the dura remains intact, they can be removed entirely without subsequent dural repair. The authors review the literature and discuss the proposed underlying mechanisms of formation of these arachnoid cysts.

Spinal Cord Compression by Epidural Tumors in Childhood

Epidural malignancy is the most common cause of nontraumatic spinal cord compression in children. Many different tumors may cause compression, either at presentation or sometime during treatment. Treatment options include surgery, irradiation, and chemotherapy. The decision as to the correct option depends on the tumor type and the degree of compression present.

Atlanto-axial instability and spinal cord compression in children--diagnosis by computerized tomography.

Five children who were at risk for atlanto-axial instability underwent computerized tomography scanning of the cervical spine in flexion and extension to document the degree of bone instability and the presence or absence of spinal cord compression. Two patients had Morquio's syndrome, two had os odontoideum , and one had Klippel-Feil syndrome, and in all five the lateral radiographs of the cervical spine in flexion and extension were equivocal with regard to instability or were difficult to interpret because of the bone anomalies. The computerized tomography scan then provided a diagnosis by a non-invasion technique and quantitated the amount of compromise of the spinal cord by delineating flattening of the cord. The scan therefore helped the surgeon to decide if the spinal cord was at significant risk and if atlanto-axial fusion was advisable.