Intramedullary spinal neurenteric cyst with fluid-fluid level

Spinal neurenteric cysts are rare intradural extramedullary lesions of the spine, commonly located in the cervical and thoracic regions. The majority localize ventral to the spinal cord and are associated with other vertebral anomalies. Here, the authors report a rare case of a 3-year-old boy presenting with a 1-week history of meningismus followed by rapid-onset (over a few hours) paraplegia. Magnetic resonance imaging revealed an intramedullary cystic lesion with a fluid-fluid level in the cervicothoracic region of the spinal cord without associated bony or soft tissue abnormalities. To the best of the authors' knowledge, such clinical and radiological presentation of a spinal neurenteric cyst has never been reported. A brief review of the pertinent literature is presented, and the possible pathophysiology of such a presentation is also discussed.     

Cervical spine in patients with diastrophic dysplasia--radiographic findings in 122 patients

BACKGROUND: In previous studies, typical radiological findings in the cervical spine of patients with diastrophic dysplasia (DD) have been kyphosis, displacement of the vertebrae, spina bifida occulta (SBO), anterior hypoplasia of vertebrae C3-5, and hyperplasia and dysmorphism of the odontoid process. OBJECTIVES: To make a radiological analysis of the cervical spine in patients with DD. MATERIALS AND METHODS: The study comprised 122 patients (50 males, 72 females), with an average age of 19 years (range newborn-63 years). Follow-up was available on 62 patients (51%), for an average duration of 11 years. Cervical spine alignment was measured according to Cobb's method. The height (H) and depth (D) of the vertebral body and sagittal diameter (S) of the spinal canal were measured. H/D and S/D ratios were then calculated from the measurements. The shape of the vertebrae was assessed. Displacement and movement of cervical vertebrae in neutral and bending radiographs were measured. RESULTS. The average lordosis in the last radiograph was 17(degrees) (range 4 degrees -55(degrees)). Five (4%) patients had a cervical kyphosis with an average of 92(degrees) (range 10-165(degrees)) on their last radiograph. The H/D ratio increased slowly during growth and showed significant correlation with age. There was no growth spurt at puberty. The S/D ratio was fairly stable until 7-8 years of age, when it started to decline slowly. The percentage of vertebrae with a flat vertebral body and narrow spinal canal value tended to increase with age. Vertebral hypoplasia and displacement between vertebrae were most common in the mid-cervical region and resolved spontaneously with age. Degenerative changes seemed to increase with age and were already visible during the second decade of life. SBO was noted in 79% of patients. CONCLUSIONS: The most common alignment in the cervical spine is lordosis in adulthood. The vertebral bodies are flattened and the spinal canal is narrowed. Vertebral body hypoplasia and displacement usually resolve spontaneously during growth. Degenerative changes in the cervical spine are common, but vertebral anomalies are rare. Prevalence of SBO is high.     

Fetal evaluation of spine dysraphism

Spinal dysraphism or neural tube defects (NTD) encompass a heterogeneous group of congenital spinal anomalies that result from the defective closure of the neural tube early in gestation with anomalous development of the caudal cell mass. Advances in ultrasound and MRI have dramatically improved the diagnosis and therapy of spinal dysraphism and caudal spinal anomalies both prenatally and postnatally. Advances in prenatal US including high frequency linear transducers and three dimensional imaging can provide detailed information concerning spinal anomalies. MR imaging is a complementary tool that can further elucidate spine abnormalities as well as associated central nervous system and non-CNS anomalies. Recent studies have suggested that 3-D CT can help further assess fetal spine anomalies in the third trimester. With the advent of fetal therapy including surgery, accurate prenatal diagnosis of open and closed spinal dysraphism becomes critical in appropriate counselling and perinatal management.     

单开门椎管成形治疗儿童颈胸段椎管内髓外肿瘤

目的 探讨单开门椎管成形、重建椎管后部结构治疗儿童颈胸段椎管内髓外良性肿瘤的可行性及临床疗效.方法 1998年5月至2012年5月,应用单开门掀起椎板、显微切除椎管内髓外肿瘤、椎板棘突复合体原位复位重建椎管后部结构的方法治疗儿童颈胸段椎管内髓外肿瘤28例.男17例,女11例;年龄1岁8个月～13岁,平均8.2岁;病程3个月～2年.影像学显示:肿瘤长度0.8～4 cm,占据1.5～4个脊髓节段.肿物偏向左侧12例,右侧10例,居中6例.结果 随访12个月～5年,平均32个月.手术部位颈段19例、胸段9例,椎板开门数2～5个,平均2.5个.平均手术时间86 min (75～146 min),术中平均失血128ml(85～356ml).所有病例均行X线片检查,未见脊柱侧凸、后凸畸形及失稳征象,23例复查CT显示复位的椎板棘突复合体无内陷、移位,28例复查MRI见椎管内肿瘤无复发,无椎管狭窄、黄韧带和硬脊膜增厚.脊柱曲度的变化:颈椎病例中颈椎曲度由术前的(17.8±8.4)°减少为术后12个月时的(16.1±7.8)°,手术前后比较无统计学差异(P＞0.05);胸椎病例局部Cobb角由术前的(11.6±3.8)°增加至术后12个月时的(12.5±4.6)°,手术前后比较无统计学差异(P＞0.05).结论 单开门稚管成形、重建椎管后部结构治疗儿童颈胸段椎管内髓外肿瘤,对椎管内髓外肿瘤的暴露及切除效果满意的同时,保存了一侧椎板的连续性,椎板棘突韧带复合体复位后恢复了脊柱的原有解剖结构,对脊柱的稳定性影响小,可避免术后儿童发育过程中脊柱畸形的发生.     

Oesophageal atresia and vertebral anomalies

Oesphageal atresia and/or tracheo-oesophageal fistula are relatively common congenital anomalies, occurring once in 3, 000 births [6, 10]. Associated anomalies are seen frequently, and in a series of 538 patients admitted to the Royal Children's Hospital, Melbourne, 256 had additional anomalies (47.5%). Evaluation of the spine was possible in 345 patients; in 57, structural vertebral anomalies were present (16.5%). Of the 57 with a structural abnormality of the spine, 40 had an abnormality of formation, 6 an abnormality of segmentation, and 10 had a combined defect. In the other patient, a bipartite cervical vertebra was present. Eight patients had scoliosis without a recognisable congenital spinal abnormality. In 81 there was a congenital rib abnormality; 56 of this group had a normal spine, 24 had a structural abnormality of the spine, and in 1 patient scoliosis was present without a structural abnormality of the spine. Comparison is made between our findings and the findings in other reported series and comments are made on the significance of spinal and/or rib anomalies in association with oesophageal atresia. Offprint requests to: N. A. Myers     

Ependymoma of the cauda equina starting with communicating hydrocephalus: a case report

The presence of concomitant hydrocephalus in cases with spinal cord tumors is relatively rare. Here, we describe a case of myxopapillary ependymoma of the cauda equina starting with communicating hydrocephalus in a 14-year-old boy. The patient presented to the clinic without underlying causes of hydrocephalus on the neuroimaging data. After ventriculoperitoneal shunt placement, despite the numerous malfunction incidents, the patient was doing well. Eight years later, he developed progressive weakness and coldness of the lower limbs. Multiple cystic lesions in the cervicothoracic area were found on magnetic resonance imaging. Decompressive hemilaminectomy of the cervicothoracic region was performed with temporary improvement of the patient's condition. Because of persisting complaints, the sacral area of the spine was also observed and neuroimaging data for a tumoral lesion in the cauda equina region were found. The lesion was surgically removed and the histological result was myxopapillary ependymoma. Therefore, cases presenting with internal hydrocephalus without clear-cut intracranial etiology should have detailed neuroimaging of the whole central nervous system.     

Relation between oligohydramnios and spinal flexion in the human fetus

BACKGROUND: Oligohydramnios, a severe reduction in the volume of amniotic fluid, is associated with fetal lung hypoplasia but underlying processes are unclear. Studies in sheep suggest that oligohydramnios may lead to lung hypoplasia by causing increased flexion of the fetal spine, but this has not been demonstrated in the human, which has a different uterine anatomy. AIMS: Our aims were to quantify spinal flexion in the human fetus and to determine the relationship between oligohydramnios and the degree of spinal flexion. SUBJECTS AND METHODS: In 35 pregnancies, we used ultrasonography to assess amniotic fluid volume and to image the fetal spine so as to provide an index of mean spinal radius between the upper thoracic and lumbar spine. In 17 pregnancies with evidence of oligohydramnios resulting from premature rupture of membranes (mean +/- SEM gestation 28.8 +/-1.4 weeks), the mean radius of fetal spinal curvature between the upper thorax and sacrum was compared with values from 18 control fetuses with normal amounts of amniotic fluid (at 28.9 +/- 1.6 weeks). RESULTS: In each fetus, the spine could be imaged and the mean radius of curvature calculated. Oligohydramnios was associated with a significantly increased degree of fetal spinal flexion compared to controls. CONCLUSION: In human pregnancy, oligohydramnios is associated with increased flexion of the fetal spine, which is likely due to reduced uterine volume. This could contribute to the development of fetal lung hypoplasia, fetal immobility and other fetal anomalies.     

A fetus with upper limb amelia, “caudal regression” and Dandy-Walker defect with an insulin-depedent diabetic mother

We describe the fetus delivered to an insulin-dependent diabetic woman who had had a previous large, stillborn, non-malformed male infant and a normal female infant. The present fetus had a most unusual combination of malformations which to date had not been described in diabetic embryopathy. The anomalies include: upper limb amelia; caudal regression with bilateral absence of the fibulae, unilateral absence of a femur and ipsilateral oligodactyly; undescended testes; atrial septal defect; multiple vertebral and rib anomalies with cervical scoliosis and right webbed neck; left cleft lip and cleft palate; severe micrognathia; left microtia with atresia of the ear canal; and central nervous system defects including hydrocephalus with the Dandy-Walker malformation, asymmetry of the lateral ventricles, abnormal frontal gyral formation, and ependymal and ganglion cell heterotopias of the spinal cord. The pathogenesis of diabetic embryopathy is discussed.     

Abnormal anatomy of the lumbosacral region imaged by magnetic resonance in children with anorectal malformations

OBJECTIVE: To investigate the frequency of lumbosacral anomalies, the association with urogenital abnormalities, and the correlation with defaecation pattern by magnetic resonance imaging (MRI). METHODS: A prospective analysis was performed of routine MRI in patients with anorectal malformations. Between 1990 and 1994, MRI was performed in 43 such patients: 31 boys and 12 girls. Twenty four had a high anorectal malformation, 16 had a low anorectal malformation, and three had Currarino's triad. MRI was performed before reconstruction in 26, and postoperatively in 17. Urogenital anomalies were found in 21. RESULTS: Abnormalities of the spinal cord and spine were found with MRI in 20 patients (46.5%); caudal regression syndrome in 10, tethered cord in two, a combination of both in three, and other spinal anomalies in five. These anomalies were found in 30% of the patients with low anorectal malformations, and in 50% with high anorectal malformations. In patients with urogenital malformations, MRI more often showed spinal anomalies (13/21, 62%) than in patients without (7/22, 32%). In high anorectal malformations, defaecation was more often a problem in patients with spinal anomalies (12/15, 80%) than in patients without (2/8, 25%). CONCLUSIONS: Spinal anomalies in the lumbosacral region were found with MRI in 46.5% of patients with anorectal malformations. Since presence of these anomalies seems to be related to clinical outcome, MRI should be performed routinely in all such patients.     

Abnormal anatomy of the lumbosacral region imaged by magnetic resonance in children with anorectal malformations.

OBJECTIVE: To investigate the frequency of lumbosacral anomalies, the association with urogenital abnormalities, and the correlation with defaecation pattern by magnetic resonance imaging (MRI). METHODS: A prospective analysis was performed of routine MRI in patients with anorectal malformations. Between 1990 and 1994, MRI was performed in 43 such patients: 31 boys and 12 girls. Twenty four had a high anorectal malformation, 16 had a low anorectal malformation, and three had Currarino''s triad. MRI was performed before reconstruction in 26, and postoperatively in 17. Urogenital anomalies were found in 21. RESULTS: Abnormalities of the spinal cord and spine were found with MRI in 20 patients (46.5%); caudal regression syndrome in 10, tethered cord in two, a combination of both in three, and other spinal anomalies in five. These anomalies were found in 30% of the patients with low anorectal malformations, and in 50% with high anorectal malformations. In patients with urogenital malformations, MRI more often showed spinal anomalies (13/21, 62%) than in patients without (7/22, 32%). In high anorectal malformations, defaecation was more often a problem in patients with spinal anomalies (12/15, 80%) than in patients without (2/8, 25%). CONCLUSIONS: Spinal anomalies in the lumbosacral region were found with MRI in 46.5% of patients with anorectal malformations. Since presence of these anomalies seems to be related to clinical outcome, MRI should be performed routinely in all such patients.     

In utero spontaneous cervical thoracic epidural hematoma imitating spinal cord birth injury

A neonate male born cesarian due to a breech presentation was noted to have no spontaneous movements of the limbs after delivery. Radiographs were not demonstrative of pathology. However, MRI revealed a large intraspinal mass with significant distortion of the cervicothoracic spinal cord. At operation, a brown, fibro-gelatinous, moderately adherent mass was evident extradurally dorsal to the spinal cord. It was noted to extend anterolaterally to the left such that the cord was deviated anteriorly and to the right. There was no indication of the mass being under pressure but the cord was not pulsatile. There was sufficient mass to the anterolateral component of the cord that it appeared rotated to the right within the canal. The right cervical roots exited dorsally, with a markedly lengthened course through the spinal canal before exiting above their respective pedicles. Histology was that of blood clot. The patient clinically demonstrated no neurologic improvement post-operatively. Now, six months after surgery, the patient has still had no significant change in clinical function. To our review, this is the first reported case of a spontaneous spinal epidural hematoma mimicking a birth-related spinal injury.     

先天性无肛畸形儿骶髓的异常改变

采用连续切片的组织学方法，对１０例先天性无肛畸形儿和５例正常新生儿的骶髓标本进行观察，发现１０例畸形儿的末段能髓均存在不同类型的异常改变：６例中央管呈菱形扩大，实质变薄，１例中央管未发育，１例中央管内有一纵行隔膜，另外２例中央管横向扩大。除此之外，骶髓前用内侧群的运动神经元的数目减少，高中位畸形儿和低位畸形儿分别为正常的３４．４％和７０．５％。本研究结果提示，肛门闭锁畸形可能是胚胎早期尾端神经管发育异常所致综合征的病变之一。     

儿童椎管内硬脊膜外蛛网膜囊肿的外科治疗

目的 探讨儿童椎管内硬脊膜外蛛网膜囊肿的临床特点及其诊治.方法 回顾性分析2001年1月至2009年8月我科收治的4例儿童椎管内硬脊膜外蛛网膜囊肿.其中颈胸段1例,胸腰段3例.临床表现3例患儿出现运动障碍,1例有感觉障碍,1例有小便失禁,1例有根痛表现.4例患儿均采用外科手术治疗,其中2例囊肿节段小于3个椎体的采用椎板切除+囊肿切除术,2例囊肿节段大于3个椎体的采用椎板成形+囊肿切除术.结果 4例患儿术后均恢复良好,除1例患儿术后随访1年下肢仍有轻度乏力外,其余临床症状均消失,恢复学习.随访1～9年均未见囊肿复发,复查脊柱三维CT未见脊柱畸形的发生.结论 儿童椎管内硬脊膜外蛛网膜囊肿是一种少见疾病,外科手术治疗能获得满意的结果,椎板成形术能够减少囊肿节段过长的患儿术后脊柱畸形的发生.

Abstract：

Objective To review the clinical features and surgical treatment of spinal extradural arachnoid cysts (SEACs) in children. Methods Four children with SEAC from were admitted to our department between January,2001 to August,2009. One SEAC was located at the cervicothoracic spine whereas the other three were found in the thoracolumbar spines. All cases were treated by surgery.Two children underwent the laminectomy for resection of the SEAC, which occupied less than 3 segments. SEAC, spreading more than 3 segments in the remaining cases, were treated by laminoplasty and en bloc resection. Results Postoperatively, the children had good recovery and resumed school. Followup of 1 to 9 years revealed no recurrences. The three-dimensional CT spine did not demonstrate any spine deformity in follow-up study. Conclusions SEACs in children are rarely reported in the literature. Excision of the SEAC result in a favorable outcome. The larninoplasty can reduce spinal deformity in patients with the long segment SEAC.     

先天性无肛畸形儿MR成像及其与排便功能关系研究

目的评估先天性肛门直肠畸形儿（anorectal malformations，ARM）尾端发育情况及横纹肌复合体（sphincter muscle complex，SMC）发育状态与排便功能的关系。方法39例ARM，男28例，女11例，应用不同序列体部相控阵列线圈或头部线圈MRI显示ARM直肠闭锁水平、骶尾椎、骶髓、泌尿生殖系的发育情况，并对SMC从多个层面进行定量分析，评估其发育状态与临床评分和肛门功能客观检查的关系。结果39例患儿中，MRI T1WI、T2WI扫描判断畸形水平的正确诊断率为100％，T2WI扫描瘘管检出率为96．9％。骶尾椎和骶髓的异常占41．0％，泌尿生殖系统畸形占20．5％。当耻骨直肠肌宽度的相对值PRWR〈0．18，外括约肌宽度的相对值EASWR〈0．15时，71．4％的ARM术后出现肛门失禁。当PRWR〉0．18，EASWR〉0．15时，91．3％的ARM术后排便功能好。结论MR检查能清楚地显示ARM类型、闭锁水平、SMC发育状态、脊柱脊髓及泌尿生殖系统存在的伴发畸形，从MR影像中找到了评价SMC的定量指标。     

Ischio-spinal dysostosis: a previously unrecognised combination of malformations

BACKGROUND: Ischial hypoplasia is an extremely rare malformation, both as an isolated anomaly and as a syndromic constituent. OBJECTIVE: To elucidate the clinical and radiological characteristics in five patients with the combination of ischial hypoplasia and spinal malformations. MATERIALS AND METHODS: The clinical records and radiographs of two females and three males, ranging in age from 3 months to 38 years, were evaluated. RESULTS: Ossification defects of the ischial rami were symmetrical and total in four patients, whereas the right ischial ramus was partly ossified in the other patient. All patients possessed multiple segmental defects of the spine, with rib anomalies of varying severity. One patient characteristically showed multiple rib gaps, resulting in respiratory distress. Severe anomalies of the cervical spine were evident in two patients. Four patients exhibited lumbosacral hypoplasia, which ultimately led to cauda equina syndrome in three older patients. One patient had mild facial dysmorphism and another had a diversity of anomalies, including ichthyosiform skin changes. Four patients were sporadic cases, whereas the other patient was born to consanguineous parents. CONCLUSIONS: The combination of anomalies in these patients constitutes a recognisable pattern of malformations but may represent a heterogeneous group of disorders.     

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.     

Spinal dysraphism in achondroplasia

The neurological complications in achondroplasia are commonly due to spinal canal stenosis. A case with rare coexistence of achondroplasia and dysraphic condition of the spine is presented wherein the neurological problem was presumably due to the latter.     

Reference values for radiological evaluation of cervical vertebral body shape and spinal canal

Background. Defining normal values is essential for reliable evaluation of growth disturbances. Previous studies of the cervical spine have mainly focused on the sagittal canal diameter and interpedicular distances. Values for vertebral body height and depth have been published only in adult men and cadavers. Objectives. To define normal values for vertebral body height (H)/vertebral body depth (D) ratio (H/D ratio) and sagittal canal diameter (S)/vertebral body depth ratio (S/D ratio) in C2–7. Materials and methods. Lateral cervical spine radiographs were available from 441 children and 192 adults. Subjects' ages varied from newborn to 39 years. Vertebral body height and depth and sagittal canal diameter were measured and ratios were calculated. This was a cross-sectional and retrospective study. Results. Vertebral bodies grow relatively more in height than in depth, most actively at puberty. At all levels, the H/D ratio remains below 1, indicating that vertebral body depth is greater than height. The SD ratio is quite stable until 7–8 years of age and then it starts to decline slowly. Conclusions. When estimating platyspondyly, the age of the patient must be taken into consideration because vertebral body height is lower in children. Growth of the spinal canal declines after 7–8 years of age. Received: 1 July 1999/Accepted: 12 August 1999     

Developmental stenosis of the cervical spine in children

Developmental stenosis of the cervical spine results in a reduction of the caliber of the cervical spinal canal that is greatest in the anteroposterior dimensions. This usually becomes symptomatic in adults when a myelopathy may result from compression of the cervical cord by small osteophytes or by hyperextension injury without fracture or dislocation. Although incidence and prevalence data are not available, this condition does occur in the pediatric population since we have encountered three adolescents with this condition. The diagnosis of cervical spinal stenosis can be suggested when narrowing of the sagittal diameter of the spinal canal on a lateral cervical radiograph is identified. Subsequent corroboration can be obtained with myelography, computed tomography, or magnetic resonance imaging.     

Clinical and Experimental Studies on Teratogenic Mechanisms of Congenital Absence of Digits in Longitudinal Deficiencies*

ABSTRACT: In order to clarify teratogenic mechanisms of congenital absence of the digits in longitudinal deficiencies, ulnar deficiency, radial deficiency, central polydactyly, osseous syndactyly and cleft hand (central deficiency) were analyzed. Then, the same anomalies induced in rat fetuses with myleran were analyzed. In ulnar and radial deficiencies, the more severe was the degree of finger deficit, the more severe was the arrest of the forearm bone. The critical periods of these anomalies were earlier than those of other anomalies. Ulnar deficiencies were induced only in Gun: Wistar rats and radial deficiencies only in WKAH/Hkm rats. A genetic factor may influence the teratogenesis of ulnar and radial deficiencies. From histological analysis of tibial deficiency, it may be suggested that the cause of absence of digits in longitudinal deficiency is closely related to a deficit of mesenchymal cells. The analysis of skeletal changes of central polydactylies, osseous syndactylies and cleft hands suggested that there seemed to be the cleft hand formation process from osseous syndactylies and central polydactylies. These anomalies were often observed in both hands in various combinations. They could be induced by the same treatment at the same developmental stage in rats. The teratogenic mechanism of cleft hand seemed to be failure of induction of digital rays in the hand plate.