Difficulties in diagnosing intrinsic spinal cord tumours.

Thirteen children with intrinsic spinal cord tumours were seen between 1984 and 1995. In only one was this the presumptive diagnosis at referral, despite a high incidence of characteristic features. Eight had presented to their local paediatrician, four to local orthopaedic teams, and one to a general surgeon. Eleven had back pain. Eleven had either spinal curvature or change in gait. The interval between onset of symptoms and diagnosis ranged from one week to six years, with a mean of 17.5 months. In nine children symptoms had been present for four or more months. In nine, unrewarding investigations had been carried out. This paper highlights typical presenting features of these tumours and how earlier diagnosis can be achieved.     

Congenital intramedullary spinal cord tumours: a report of two cases

We present two different cases of congenital intramedullary tumours, one of a patient in whom treatment was started without pathological confirmation of a malignant tumour and the other of a primitive neuroectodermal tumour. Magnetic resonance imaging is the most useful tool in the diagnosis of malignant intramedullary tumours and differentiation from other types of spinal cord lesions.     

Difficulties in diagnosing Marfan's syndrome in childhood and adolescence

BACKGROUND: The diagnosis of Marfan's syndrome in childhood and adolescence is made by the criteria of the Gent nosology, which evaluates genetic data, family history and a spectrum of clinical criteria. Due to the age dependent manifestations of the clinical symptoms, combined with the extreme heterogeneity of Marfan's syndrome diagnosis in early childhood remains sometimes difficult. PATIENTS: Prospectively, we analyzed the clinical symptoms of all patients where Marfan's syndrome was suspected. We evaluated those patients between January 1997 and April 2002 by an interdisciplinary approach. METHODS: We compared the clinical datas of the patients by using the Gent nosology and the Berlin nosolgy. RESULTS: 34 patients underwent full follow-up. The median age was 10,32 years with a range of 0,01 to 37,31 years, 19 patients were male, 15 patients were female. In eight patients Marfan's syndrome could be rouled out, 9 of 26 patients (34,6 %) fullified the criteria of the Gent nosology, in 17 of 26 patients (65,4 %) Marfan's syndrome remained just suspected, but was not fullified by the criteria of the Gent nosology. Concerning the Berlin nosology 14 of 26 patients (53,8 %) fullified the criteria, 12 of 26 patients (46,2 %) failed. Due to the criteria of the Gent nosology 14 patients (53,8 %) fullified the criteria of skeletal involvement, 21 patients (80,8 %) fullified cardiovascular major manifestation, 6 patients (23,1 %) had an ophthalmic major manifestation, and 9 patients (34,6 %) had an affected first degree relative or were genetically determined. CONCLUSIONS: On the basis of the data of our patients the diagnosis of Marfan's syndrome in childhood and adolescence can be made more sensitive by the criteria of the Berlin nosology compared to the Gent nosology. This seems to be caused by the age dependent manifestations of the symptoms. Until diagnostic algorhythms of Marfan's syndrome in childhood remain suboptimal, continuous clinical follow-up for all cases even those only in the case of suspected Marfan's syndrome are necessary to exclude complicated course and to improve outcome.     

Congenital inclusion tumours in spinal dysraphism

Objective  

To review the presentation, diagnosis and management of children with spinal dysraphism and CIT.     

Neonatal spinal cord injury

Neonatal spinal cord injury (SCI) is well described in the literature, though its diagnosis is often delayed or missed in the neonatal period. We present a neonate who was referred with upper gastrointestinal bleed and a diagnosis of spinal cord injury was subsequently made clinically and confirmed radiologically.     

Intramedullary spinal cord abscess

Intramedullary spinal cord abscess is a treatable cause of paraparesis. Magnetic resonance imaging is an important diagnostic modality. Combination of antimicrobial therapy and surgical intervention can provide good results in patients with subacute presentation.     

Neonatal spinal cord injury

Neonatal Spinal Cord Injury (SCI) is well described in the literature, though its diagnosis is often delayed or missed in the neonatal period. We present a neonate who was referred with upper gastrointestinal bleed and a diagnosis of spinal cord injury was subsequently made clinically and confirmed radiologically.     

Intramedullary spinal cord astrocytomas in children

BACKGROUND: Intramedullary spinal cord astrocytomas are uncommon tumors in childhood. There is little information on therapy and outcome of astrocytomas in this location. PROCEDURE: A retrospective review was performed for the 10 children who were treated between 1996 and 2003 for spinal cord astrocytomas in our institution. Only one had metastatic disease. All ten patients underwent surgical resection, nine partial and one total. Eight had low-grade tumors, and two high-grade tumors. Two had surgery only, four had chemotherapy only, two had radiation only, and two had both radiation and chemotherapy. RESULTS: Progression free survival was 58% and survival was 68% at 4 years. Four patients had disease progression, of which three died. Both children with high-grade astrocytomas died. Two of eight of the children with low-grade astrocytomas of the cord recurred, one having received radiation as initial therapy and the other chemotherapy. The child, who relapsed after radiation, had a spastic quadriplegia from radiation myelitis and no salvage therapy was attempted. The four patients, all with low-grade astrocytomas, who treated with chemotherapy alone, received carboplatin and vincristine. Of these four, three are in continuous remission and one relapsed, but was salvaged with radiation. CONCLUSIONS: Chemotherapy and radiation did not benefit those with high-grade astrocytomas of the spinal cord. Good outcomes can be achieved by conservative surgery for low-grade astrocytomas of the cord when adjuvant therapy is given. Carboplatin and vincristine appeared to be effective, safe therapy for those with low-grade astrocytomas of the cord.     

Intramedullary spinal cord abscess masquerading as spinal tumor

We report a 5-year-old girl who presented with acute onset paraparesis with differential loss of sensation. Magnetic resonance imaging of spine revealed exophytic intramedullary mass lesion from T12 to L1. Peroperatively, the diagnosis was confirmed as abscess. The patient recovered following decompression and antibiotic treatment.     

Surgery for spinal cord lipomas

Objectives To analyze the results after surgery for spinal cord lipomas. Methods The authors report their results of management of 63 non consecutive random children with spinal cord lipomas treated over a period from 2001 to 2005, at the All India Institute of Medical Sciences, New Delhi, India, a tertiary care neurosurgical centre. Results There were 63 cases consisting of 32 (52%) conus lipomas, 14 (22%) filum lipomas, 14 (22%) lipomeningomyelocele (lipoMMC), 2 (2.5%) cases of lipomyelocystocele and one (1.5%) case of mixed lipoma. None of the patients who were asymptomatic before surgery deteriorated neurologically, irrespective of the type of lipoma till the last follow up. In those patients with preexisting neurological deficits, the improvement in motor, sensory and bladder abnormalities was only to an extent of 15%, 16% and 21% respectively. None of the children with preexisting neurological deficit regained overall normal function. Nevertheless, the improvement in symptoms after surgery made the patients lead a better social life. Only 6% of patients developed deterioration in neurological function after surgery, all of them occurring in patients already having pre-operative deficits. Conclusions The authors recommend prophylactic surgery which is safe and effective in preventing neurological deficits, irrespective of the type of lipoma. Most of the patients benefit only to some extent even after surgery, once they develop neurological dysfunction. A close long term follow up is recommended in order to detect neurological deterioration even in children operated prophylactically.     

����Ѫ�ܼ���

小儿脊髓血管疾病易于漏诊和误诊,如不能早期诊断和治疗,常可带来不良预后。脊髓的血液供应,主要来自根动脉、脊髓前动脉、脊髓后动脉。根动脉在不同的脊髓节段分别来源于颈动脉、肋间动脉、腰动脉、骶中动脉,它们穿过硬脊膜后,再分支为前根动脉和后根动脉,分别与脊髓前动脉和脊髓后动脉吻合。脊髓前动脉主要供应于前角、侧角、中央灰质和侧柱,脊髓后动脉则供应本侧的后角、后柱。任何原因造成这些动脉的血供障碍或出血均为脊髓血管疾病。在小儿常见的原因为外伤、产伤、血管畸形、感染等。1脊髓缺血(spinal cord ischemia)任何原因造成供应…     

High-grade pediatric spinal cord tumors

Our institutional experience with high-grade pediatric spinal cord tumors includes 11 children treated during the period of 1981-1997. All patients underwent a biopsy or an attempt at resection and received postoperative radiation therapy. Three patients had a gross-total resection of their primary tumor, 6 patients had a subtotal resection and the remaining 2 were biopsied. Histologically, these tumors were characterized as anaplastic astrocytoma (n = 6), glioblastoma multiforme (n = 3) or anaplastic oligodendroglioma (n = 2). Three patients were treated with craniospinal irradiation (38-48 Gy) in addition to a boost to the residual tumor. The median dose to the primary site for all patients was 48.6 Gy (range 38-55 Gy). The median overall survival was 13 months (range 8-149 months). Only 2 patients were alive at 138 and 149 months following radiation therapy. The median progression-free survival following radiation therapy was 10 months (range 2-80 months). There was no difference in progression-free or overall survival for those diagnosed with glioblastoma multiforme when compared to patients diagnosed with anaplastic astrocytoma or anaplastic oligodendroglioma. The pattern of failure was either diffuse or local. For the patients who failed diffusely (n = 6), the median progression-free survival was 2 months compared to 23 months for those whose failure was entirely local (p < 0.01). The median overall survival was significantly shorter for those who failed diffusely compared to those who failed locally (10 vs. 37 months, p < 0.01). High-grade spinal cord tumors in children have a poor prognosis based on this report. It is important to document the extent of disease accurately prior to the initiation of radiation therapy, since a subset of these patients progress rapidly outside of the field of irradiation.     

Pseudopseudohypoparathyroidism with spinal cord compression

We describe a patient with pseudopseudohypoparathyroidism who had an osseous tubercle on the anterolateral margin of the foramen magnum causing compression of the spinal cord. This patient had no evidence for any endocrinopathies and had no other spinal canal anomalies. We suggest that the morphologic phenotype found in patients with pseudopseudohypoparathyroidism, also known as Albright's hereditary osteodystrophy, has an associated risk for spinal cord compression due to congenital vertebral anomalies. The poor recovery of neurologic function following spinal decompression mandates prompt recognition and therapy of this condition in patients with Albright's hereditary osteodystrophy.     

����ѹ��֢

脊髓压迫症是指一组具有占位效应的椎管内病变。脊髓由于受到外界的压迫而产生一系列的病理生理变化。脊髓受压后的变化与受压迫的部位、外界压迫的性质及发生的速度有关。一旦外界压迫超过脊髓的代偿能力,脊髓受压症状可进行性加重,最终可造成脊髓水肿、变性、坏死等病理变化,从而导致脊髓功能的丧失,出现受压平面以下的肢体运动、感觉、反射及括约肌功能障碍。脊髓压迫症常见的原因有:椎管内肿瘤、外伤、感染、脊髓血管畸形、椎间盘突出以及先天性脊柱病变等。在儿童则以椎管内肿瘤、外伤、感染和先天性脊柱畸形较为多见。根据临床表现的…