Spinal dysraphism

Spinal dysraphism (SO) is characterized by maldevelopment of neural tube, notochord, mesoderm and cutaneous ectoderm. Incidence of SD is 2–4/1000 live births. One hundred and nineteen patients operated from January 1991-June 1996 at Department of Neurosurgery, All India Institute of Medical Sciences, were studied. Only 21 patients (17.6%) presented when they were less than one year old and 17 patients came in adult age group (> 16 years). Lumbar and lumbosacral region was the commonly involved site in 81 patients (74.7%). Weakness of lower limbs (74%), difficulty in walking (54%),muscle atrophy (41.2%) were the commonest indicators of motor system involvement. Loss of sensation, trophic ulcer, backache were seen in 45, 14, 10 patients respectively. Cutaneous lipoma (26%), hypertrichiosis (20%), dermal sinus (13.4%), midline dimples (7%) were the important cutaneous markers. Foot and limb deformity was seen in 25% cases. Tethering of cord, syringomyelia & split cord malformation were the most common radiological findings. Only 10% of our patients had hydrocephalus that requiredshunt. Out on 119 cases operated, 43 improved. Vwenty had sensory improvement and 18 showed motor improvement. Fifteen patients regained continence. Twelve patients were lost to follow-up. Sixtyseven patients had no change in neurological status, post-operatively. Six cases deteriorated in terms of motor or sensory deficit and one patient lost continence. CSF leak (8%) and wound infection (6%) were the common complications. Six patients required second surgery as T.R Shunt (4), rotation flap (1), reexploration and duraplasty(1).     

小儿颈椎及颈脊髓损伤

目的 总结小儿颈椎及颈脊髓损伤的诊断治疗经验。方法 回顾分析４７例小儿颈椎及颈脊髓损伤。男３１例,女１６例,年龄６－１４岁,平均１１．６岁。新鲜损伤１９例,陈旧性损伤２８例。其中上颈椎损伤３３例,下颈椎损伤１０例,多节段间隔性颈椎损伤１例。无Ｘ线异常的脊椎损伤３例,非手术治疗２７例,手术治疗１９例,未治１例。结果 早期死亡１例。４５例获１－１２年随访,恢复正常或接近正常者３４例,明显改善者６例,５     

Spinal muscular atrophy of the scapulo-peroneal-type

A case report is given on a non-familial muscular atrophy with scapuloperoneal distribution in an 8 year old boy. A muscle biopsy and electromyographic studies indicated a neurogenic lesion. Spinal muscular atrophy appears to be the most probable diagnosis. Similar cases cited in the literature are compared with the present case. The differences to spinal muscular atrophy of the Kugelberg-Welander type are pointed out.

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Zusammenfassung Es wird über einen Fall von spinaler Muskelatrophie mit scapulo-peronealer Verteilung bei einem 8jährigen Jungen berichtet. In der sehr gründlich erhobenen Familienanamnese fand sich kein Hinweis auf eine ähnliche Erkrankung bei den Vorfahren, so daß es sich ähnlich wie eine von Emery beschriebene Beobachtung um einen Einzelfall handelt.Die Diagnose wurde auf Grund der neurologisch-klinischen Befunde sowie den Ergebnissen von Muskelbiopsie und EMG gestellt. Differentialdiagnostisch läßt sich die spinale Muskelatrophie vom scapulo-peronealen Typ gegen die von Kugelberg u. Welander beschriebene Amyotrophie abgrenzen.

     

Spinal tuberculosis in a 14-year-old immigrant in the Netherlands

We present a case of Pott’s disease, where the patient presented with neurological impairment due to vertebral granulomatous necrosis, needing immediate decompression and later stabilizing and reconstructive orthopaedic surgery, in order to create awareness for TB in general, especially this forgotten form of spinal tuberculosis. The authors did not have any financial support or conflict of interest regarding this case report.     

Spinal cord compression by vertebral hemangioma

Asymptomatic vertebral hemangiomas occur frequently in adults but the discovery of a similar lesion in the pediatric age group is uncommon. A case which involves all the neural arch components with resultant spinal cord compression is rare and is reported with a review of pertinent literature.     

Spinal Arachnoid Cyst in a Newborn Infant

A male infant was born with asphyxia following a prolonged breech delivery at 39 weeks of gestation. He had a chylohemothorax from birth. Soon after birth he exhibited flaccid paraplegia with an absence of deep tendon reflexes in the lower extremities. At 17 days of age, magnetic resonance imaging (MRI) disclosed a hypodense area in the thoracic cord between C₇ and Th₄ in T,-weighted images, and spinal sonography revealed an echo-free space compressing the spinal cord in the same region observed with MRI. A spinal cyst was removed surgically at 24 days of age. The cyst was confirmed by pathology to be a true arachnoid cyst between C₇ and Th₄. The cause of this cyst may have been a spinal cord injury during the perinatal period.     

Radiology, histology and short-term outcome of asymptomatic congenital thoracic malformations

Aim: The aim of this study was to evaluate the safety and short‐term outcome of our management of asymptomatic children with antenatally diagnosed congenital thoracic malformations (CTM), compared with recommendations from a recent review and meta‐analysis. Methods: Twenty‐two asymptomatic children with CTM, born in January 1, 2002 to January 8, 2009 were reviewed. Data on complications and respiratory symptoms were collected. Results: No severe respiratory symptoms were recorded. Seventeen children were referred to surgery. Complications were seen in one child. The final diagnoses were congenital pulmonary airway malformation (CPAM) in 13 children, two had sequestrations, and two had other significant malformations. Five children had minor malformations and did not undergo surgery. No malignancies were reported. Conclusion: Elective surgery at 1 year of age is safe and carries no apparent risk to asymptomatic children with CTM. The rate of complications was equal to that reported in a recent review and meta‐analysis.     

先天性脊柱侧凸合并脊髓异常

调查先天性脊柱侧凸患儿脊髓异常的发生并探讨影像学诊断的地位。方法１０７例先天性脊柱侧凸病例中合并脊髓异常者３６中３３．６％。所有病例均摄Ｘ线平片,脊髓造影或ＣＴ脊髓造影２５例,ＭＲＩ检查２７例。     

Spinal cord hamartoma with pseudopancreatic cyst

A 2-year-old boy presented with weakness of both lower limbs with bladder and bowel involvement and history of frequent falls. Magnetic resonance imaging of the spine revealed a T2 altered signal intensity enhancing mass lesion seen in the spinal epidural space extending from sixth cervical to fourth thoracic vertebrae, which was compressing the adjacent spinal cord. Histopathology of the lesion was suggestive of hamartoma. A brief review of the literature including its embryogenesis is discussed here. The child subsequently developed pseudopancreatic cysts possibly because of repeated falls leading to blunt trauma abdomen.     

Spinal cord injury without radiographic abnormalities

Spinal cord injury without radiographic abnormality (SCIWORA) has a reported rate of incidence varying from 19% to 34% of all spinal cord injuries in children. This acronym refers to the presence of neurological lesion, despite normal plain radiographs, but where magnetic resonance imaging (MRI) shows significant pathology. The clinical evidence of the damage could be delayed in 6–54% of cases, usually within 48 h after the trauma. We report two patients affected by SCIWORA in the pediatric population. The first patient was a child of 22 months who had fallen when attempting to get out of bed. The second patient was an 11-month-old child admitted to the Emergency Service department after a car accident. Spinal cord injury has to be suspected in the presence of neurological signs, despite normal plain radiography. MRI is the appropriate diagnostic examination to identify the presence of SCIWORA.     

先天性马蹄内翻足患儿脊髓MRI表现

目的 先天性马蹄内翻足(CCF)患儿脊髓MRI图像是否有异常.方法 2004年10月至2005年12月我科共收治先天性马蹄内翻足患儿21例,同时收集无脊柱畸形及脊髓神经系统症状患儿的脊髓MRI图像13例.记录脊髓位置.对蛛网膜下腔、椎体和腰膨大按年龄分二组进行测量,SPSS 12.0统计学分析软件进行分析.结果 患病组7例膀胱肿大,2例肾脏发育不良,1例椎管内皮样囊肿.1例椎管内血管瘤,1例终丝脂肪沉积.且蛛网膜下腔前后径与相应椎体的比值从腰1(L1)到腰5(L5),患病组(CCF组)比对照组高.脊髓圆锥CCF组位于L1者占73.3%,而对照组为40%.7～12个月组和13个月到3岁组腰膨大的前后宽较对照组窄.结论 CCF患儿的脊髓位置低,且在6个月后腰膨大变窄,蛛网膜下腔前后径相对较宽.CCF患儿的小腿及足的神经异常可能来源于腰骶段脊髓的发育异常.     

儿童型脊髓性肌萎缩症的临床与病理研究

目的：总结42例儿童型(I～III型)脊髓性肌萎缩症(SMA)的临床与病理特征,以探讨儿童型SMA临床与病理学特征及其意义。方法：收集42例做过肌活检的SMA I～III型病例,进行临床、肌肉病理学(常规组织学及组织化学方法)分析。结果：不同型SMA临床各有特点,主要是病情轻重和起病年龄有关。起病越早者,病情越重。肌活检显示SMA I型为大组分布的圆形萎缩肌纤维,而非角形纤维,呈不完全同型肌群化,常累及整个肌束;SMA II型少见大组萎缩肌纤维,同型肌群化突出;SMA III型病理变化多样,以同型肌群化为主。结论：临床表现结合肌电图、肌活检可协助诊断儿童型脊肌萎缩症。     

Spinal cord compression: a review of 70 pediatric patients

The authors report their experience with 70 pediatric patients with spinal cord compression (SCC) due to malignancies identified among 898 patients with solid tumors. An extradural tumor was the most frequent cause of SCC (71%); 54% of these were soft tissue sarcomas and neuroblastoma. Most intradural tumors (70%) were outside the spinal cord, 9/12 being metastatic medulloblastomas. The SCC localized mainly to the dorsal and lumbosacral regions (42% each). Pain was the most common symptom (94%). MRI proved diagnostic in all cases in which it was used, while myelography was diagnostic in 85% of 26 patients. CT scan demonstrated the lesion in 83% of the patients. Laminectomy was provided for patients with paraplegia of less than 96 h evolution; isolated recurrence of the main tumor; a primary spinal cord tumor; progression of neurologic symptoms after chemotherapy/radiotherapy; chemotherapy and radiotherapy-resistant tumor (when known); resection of a paraspinal tumor. Surgery was avoided when prognosis of primary disease was poor or risks exceeded possible benefits. Twelve/twenty-one (57%) patients with paraplegia were able to walk after laminectomy only, while 14% (2/14) improved after chemotherapy and radiotherapy. Survival rates were 38% for the former and 36% for the latter. Overall survival was related to the original malignancy. All patients (12) admitted without paraplegia and submitted to laminectomy were able to walk, and of these, 6 presented a primary spinal cord tumor. The remaining had paraspinal tumors that extended to the spinal canal. Almost 87% (20/23) of the patients without paraplegia who submitted to medical treatment were able to walk, while only one progressed to paraplegia. Patients with SCC may entertain radio- and chemotherapy when harboring tumors responding to such therapies and present no evidence of neurologic damage progression. The latter manifestation is a strong indication for laminectomy without delay.     

小儿脊髓拴系综合征的诊断与治疗

为探讨小儿脊髓拴系综合征（ＴＣＳ）的发病机理、临床表现、诊断和治疗，对１９８６年６月～１９９４年６月手术治疗的４６例患儿进行分析。临床凡有排尿、排便障碍，下肢感觉、运动障碍，足畸形等症，体检发现隐性脊柱裂、显性脊柱裂（脊髓脊膜膨出）或曾行脊膜膨出手术的患儿均可疑为该病。磁共振（ＭＲＩ）和Ｍｅｔｒｉｚａｍｌｕｅ脊髓造影ＣＴ检查可确定诊断。本组术后随访１～８年，手术有效率５２％。该病一经确诊即应尽早行脊髓拴系松解术，以缓解症状，减少神经系统病残率。     

Spinal cord dysfunction in neonatal meningococcal meningitis

A 3-week-old infant with meningococcal meningitis is described whose clinical course was complicated by paraparesis and urinary retention. This rare complication of meningococcal meningitis has not previously been reported in the neonatal age group.