Aneurysmal bone cyst of skull and vertebrae in children. Analysis of own material and review of the literature

Aneurysmal bone cyst (ABC) is a benign, expansive, osteolytic lesion, consisting of blood-filled cysts, capable both of rapid enlargement and spontaneous resolution. Asymptomatic cases have been reported too. The aim of this paper was to analyse the outcomes of surgical treatment of ABC in children and a review of pertinent literature. We adopted the method of retrospective analysis of medical documentation of 10 patients with ABC. These were patients at the Department of Neurosurgery of the Children's Memorial Health Institute (Warsaw, Poland) from 1980 to 2002. There were 2 cases of cranial lesions and 8 cases of vertebral lesions. All the patients underwent surgical treatment only. Total lesionectomy was obtained in 7 cases, subtotal--in 3 cases. With the mean follow-up time of 5.1 years, good outcome (no neurological deficits) was noticed in 5 cases, moderate disability in the form of paraparesis--in 4 cases and full paraplegia--in 1 case. The following perioperative complications were noticed: transient paraplegia (1 case) and recurrence of ABC requiring reoperation (1 case). Among the 8 patients with vertebral lesions, progressive scoliosis requiring instrumental stabilization of the spine was noticed in 5 cases (4 vertebral body lesions and 1 laminar lesion). Primaiy infiltration of vertebral body by an ABC may lead to subsequent progressive scoliosis, which requires instrumental stabilization of spine. This demands careful planning and development of a comprehensive treatment program. ABC in children is a predominantly aggressive lesion, but even subtotal excision does not entrain a recurrence. Localization of lesion at the D3-5 levels is associated with an increased risk of postoperative neurological deterioration. Patients should be treated surgically before the development of severe deficits, which later may prove irreversible.     

Validation of Danish case register diagnosis for schizophrenia

The ABC schizophrenia study aims at investigating sex differences in age of onset, symptoms and course of schizophrenic and paranoid disorders. For this purpose, we used case register data from Denmark and Mannheim and a directly examined sample of first admissions (ABC sample). The Danish case register sample included less clinical diagnoses of schizophrenia and more schizophrenia-related disorders (acute paranoid reaction, paranoid states and borderline schizophrenia) than the Mannheim data (case register and ABC sample). The problem therefore was whether the two datasets are comparable and the results are valid. For this reason a randomized, stratified sample of 116 patients was drawn from the Danish case register sample. The case notes of these 116 patients were requested from the hospitals where the patients had been treated and analyzed by means of a scoring sheet based on the Interview for the Retrospective Assessment of the Onset of Schizophrenia (IRAOS). The use of operationalized diagnoses of the CATEGO program, based on PSE items, which are integrated in IRAOS, demonstrated that the samples of the Danish and the Mannheim case registers and the directly investigated ABC sample have comparable diagnostic distributions. Possible explanations for the differences between the clinical and the CATEGO diagnoses in the Danish case register may be the frequent use of diagnoses of borderline schizophrenia and reactive psychoses (previously called psychogenic psychoses), and above all a more narrow concept of schizophrenia; in Denmark, schizophrenia is diagnosed relatively late, i.e., after the presence of enduring negative symptoms, and thus mostly after the appearance of residual state. These diagnostic preferences may help to explain the fall in first admission rates for schizophrenia – above all in women – in Denmark and the low incidence rates of schizophrenia by first contact within the WHO determinants of outcome study. The earlier hospitalization of men could be replicated as well as the course of treatment (readmissions and discharges) of schizophrenic men and women over 10 years after first admission.     

Cystic Giant Sacral Schwannoma Mimicking Aneurysmal Bone Cyst : A Case Report and Review of Literatures

To present a rare case of a cystic giant schwannoma of the sacrum mimicking aneurysmal bone cyst (ABC). A 54-year-old man visited our institute complaining left leg weakness and sensory change for several years. Magnetic resonance imaging revealed a large multilocular cystic mass with canal invasion and bone erosion confined to left S1 body. The lesion showed multiple septal enhancement without definite solid component. Initially the tumor was considered as ABC. The patient underwent grossly-total tumor resection with lumbosacral reconstruction via posterior approach. The tumor was proved to be a cystic schwannoma. The postoperative course was uneventful and the patient was relieved from preoperative symptoms. We present a rare case of pure cystic giant schwannoma confined to sacrum mimicking ABC. The surgical treatment is challenging due to the complex anatomy of the sacrum. Schwannoma should be considered in the differential diagnosis of osteolytic sacral cysts.     

Fibrous dysplasia in combination with aneurysmal bone cyst presenting as a subarachnoid haemorrhage

Abstract Fibrous dysplasia (FD) is a rare tumour, representing 2.5% of all bone tumours and 7% of benign tumours. Aneurysmal bone cyst (ABC) is also an uncommon pathology, usually associated with a secondary vascular lesion consisting of an arteriovenous malformation. In this article, we relate a case report of a young female with a rare combination of FD with aneurysmal bone cyst presenting as a subarachnoid haemorrhage (SAH). Despite the possibility of clinical treatment of these lesions, this report demonstrates that symptomatic lesions may be successfully managed by surgical resection. The authors’ opinion is that this treatment should be individualised depending on particularities of each case, such as localisation of the lesion, biopsy results and image exams features. We also present a critical literature review of diagnostic methods and therapeutical options for both ABC and FD, with emphasis on controversial topics surrounding these issues.     

Cranial aneurysmal bone cyst: a diagnostic problem With a review of the literature

Aneurysmal bone cysts (ABC) are benign osteolytic lesions that are more common in young people than in adults and involve the skull only exceptionally. The origin of ABC is still debated; indeed, some authors consider ABC to be an anomalous bony reaction that is secondary to traumas or tumours. Conversely, others consider ABC to be a distinct entity. A case of a healthy young female affected by a left frontal ABC is reported here. The clinical onset was characterised by the sudden appearance of a tender and mildly painful frontal mass. Neuroradiological assessment showed a well-circumscribed lytic lesion of the frontal bone with predominantly outward extension. En bloc surgical removal of the lesion was successfully achieved; a reconstructive cranioplasty was also performed to repair the cranial defect. The rarity of the condition described, together with the absence of clear guidelines, prompted us to review the more recent literature with the twin goals of identifying radiological features and becoming able to address the diagnosis and rules for treatment of such a rare entity. Received: 28 July 2000 Revised: 25 September 2000     

Spinal aneurysmal bone cyst presenting as acute paraparesis during pregnancy

Aneurysmal bone cysts (ABC) are benign but locally aggressive osseous lesions characterized by blood-filled cystic cavities that account for 1–2% of all bone tumors. While pain remains the most common presenting symptom of spinal ABC, extensive anterior column involvement can result in vertebral fractures, spinal instability, and neurological deficits from compression of neural elements. An exceedingly rare case of thoracic ABC presenting as acute paraparesis in a 21-year-old pregnant woman, that was successfully treated with urgent surgical decompression and stabilization, is reported, with discussion of management strategies and review of the pertinent literature.     

Metástasis sacra simulando quiste óseo aneurismático

Cystic spinal lesions with characteristic patterns, such as the presence of haematic fluid-fluid levels (H-FFL), have been associated with many tumoral lineages, more frequently with aneurysmal bone cyst (ABC) and exceptionally with metastasis. We present the case of a 60-year-old man with the finding of a sacral cystic bone lesion with H-FFL, with initial suspicion of ABC and confirmed diagnosis of metastasis. The case presented is, to our knowledge, the second case published of spinal cystic bone metastasis with H-FFL pattern with unknown primary tumour at the time of diagnosis and the only one that received resective surgical treatment, achieving pulmonary and metastatic disease control with good quality of life after 1 year of follow up.     

Questionnaire screening for comorbid pervasive developmental disorders in congenitally blind children: A pilot study

Teachers and parents completed the Autism Behavior Checklist (ABC) on a clinical sample of 17 congenitally blind children. Of the 17 children, 4 had a definite or likely pervasive developmental disorder (PDD) as judged by independent case note review. The ABC was administered both in its original format and in a slightly modified format. Only teacher-completed ABCs detected group differences and had satisfactory testretest reliability. The modified-format ABC completed by teachers detected 3 of the 4 children with PDDs without any false positives. Screening questionnaires may have a limited but useful role in locating subjects with blindness plus putative PDDs for further study.The authors thank Patricia Sonksen and Helen McConachie for help in locating subjects, Andrew Pickles for statistical advice, Suzanne Pemberton for administrative assistance, the anonymous reviewers for helpful comments on earlier drafts, and the parents and teachers for their willing cooperation.     

A comparison of the Aberrant Behavior Checklist and the GAF among adults with mental retardation and mental illness

Psychiatric assessment among individuals with a diagnosis of both mental retardation and mental illness presents a clinical challenge. This retrospective study compared two rating scales--the Aberrant Behavior Checklist (ABC) and the Global Assessment of Functioning (GAF)--to determine the scales' utility in a partial hospital setting. Although ABC and GAF ratings were weakly correlated, the ABC revealed symptom patterns consistent with recognizable features of psychiatric syndromes and differential improvement in symptoms within and between diagnostic subgroups. The ABC provided a more useful measure of treatment response than the GAF in this patient population.     

Effects of a behaviour management technique for nursing staff on behavioural problems after acquired brain injury

The ABC method is a behaviour management technique for use by nurses. ABC refers to the identification of Antecedent events, target Behaviours, and Consequent events. In this longitudinal intervention study with double baseline measurements we evaluated the effectiveness of the ABC method in patients with behavioural problems after acquired brain injury. Fifty-six patients participated in this study. Outcome was measured in terms of overall neuropsychiatric problem behaviour, aggression, apathy and emotional burden experienced by nurses. A process evaluation was performed to investigate usability and acceptability of the method and identify factors that influenced effectiveness. Friedman’s ANOVA showed a small significant reduction over time in overall neuropsychiatric problem behaviour and aggression. The reduction was most prominent between the first baseline measurement and the post-implementation and follow-up measurements, not between the second baseline measurement and the post-implementation or follow-up measurements. This first group study on the effects of the ABC method could not prove this technique is effective for patients with behavioural problems after acquired brain injury. Nurses indicated that the ABC method was not fully implemented in their daily routines. This may have influenced results and makes it yet premature to draw firm conclusions on the effects of the ABC method.     

Factor validity and norms for the Aberrant Behavior Checklist in a community sample of children with mental retardation

The Aberrant Behavior Checklist (ABC) is a 58-item rating scale that was developed primarily to measure the effects of pharmacological intervention in individuals living in residential facilities. This study investigated the use of the ABC in a sample of community children with mental retardation. Teacher ratings on the ABC were collected on 666 students attending special classes. The data were factor analyzed and compared with other studies using the ABC. In addition, subscales were analyzed as a function of age, sex, and classroom placement, and preliminary norms were derived. A four-factor solution of the ABC was obtained. Congruence between the four derived factors and corresponding factors from the original ABC was high (congruence coefficients ranged between .87 and .96). Classroom placement and age had significant effects on subscale scores, whereas sex failed to affect ratings. The current results are sufficiently close to the original factor solution that the original scoring method can be used with community samples, although further studies are needed to look at this in more detail.Work on this paper was supported by a research project grant from the National Institute of Mental Health (Grant MH 44122) to M. G. Aman. The authors thank the departments of special education within public school districts, the county boards of MR/DD, and the numerous teachers in central and northern Ohio who participated in this study.     

Expanded endonasal endoscopic approach for resection of a large skull base aneurysmal bone cyst in a pediatric patient with extensive cranial fibrous dysplasia

Aneurysmal bone cysts (ABCs) are uncommon non-neoplastic, hemorrhagic, and expansile osseous lesions. These lesions most commonly occur in the first two decades of life and affect the long bones and spinal column. Skull base involvement is rare. The authors report the case of a 16-year-old boy who presented with acute visual decline and was found to have a large skull base ABC centered in the sphenoid sinus. In addition, the patient had extensive cranial fibrous dysplasia. The patient underwent a staged expanded endonasal endoscopic approach for complete resection of this lesion with excellent return of his vision. This case adds to the growing body of evidence supporting a role for expanded endonasal endoscopic procedures in pediatric patients with skull base pathologies.     

Sphingosine kinase inhibition exerts both pro- and anti-atherogenic effects in low-density lipoprotein receptor-deficient (LDL-R(-/-)) mice

Sphingosine 1-phosphate (S1P), a lysosphingolipid associated with high-density lipoprotein (HDL), contributes to the anti-atherogenic potential attributed to this lipoprotein. This study examined whether a reduction of S1P plasma levels affects atherosclerosis in a murine model of disease. LDL-R(-/-)mice on Western diet were given ABC294640, an inhibitor of sphingosine kinase (SphK) for 16 weeks. ABC294640 decreased plasma S1P by approximately 30%. However, ABC294640 failed to affect atherosclerotic lesion formation. Plasma triglycerides were reduced whereas total and HDL-cholesterol remained unchanged in course of ABC294640 treatment. ABC294640 increased plasma interleukin (IL)-12p70 and RANTES concentration as well as IL-12p70, RANTES and interferon (IFN)-γ production by peritoneal cells and this was paralleled by enhanced activity of peritoneal and spleen dendritic cells as evidenced by up-regulation of CD86 and MHC-II on CD11c(+) cells. As a consequence, increased T-cell activation was noted in ABC294640-treated mice as indicated by enhanced CD4(+) splenocyte proliferation, IFN-γ and IL-2 production, and CD69 expression. Concomitantly, however, ABC294640 treatment redistributed CD4(+) and CD8(+) cells from blood to lymphatic organs and reduced T-cell number within atherosclerotic lesions. In addition, plasma sVCAM-1, sICAM-1, and MCP-1 levels as well as in vivo leukocyte adhesion and CCL19-induced T-cell penetration into peritoneum were lower in ABC294640-treated animals. In vitro experiments demonstrated reduced VCAM-1 and ICAM-1 expression and lymphocyte adhesion to endothelial cells exposed to ABC294640. In conclusion, treatment with SphK inhibitor leads to both pro- and anti-atherogenic effects in LDL-R(-/-) mice. As a consequence, SphK inhibition fails to affect atherosclerosis despite significant S1P reduction in plasma.     

Using ABC narrative recording to identify the function of problem behavior: A pilot study

Many professionals report using ABC narrative recording to identify the function of problem behavior in children with developmental disabilities, but research has not established whether their analyses yield valid results. Thus, the purpose of this study was to examine whether the function identified by expert reviewers using ABC narrative recordings would match the one identified by a functional analysis (FA) and the Questions About Behavioral Function (QABF) scale in four children with developmental disabilities. The functions identified by all twelve experts using the ABC narrative recordings matched at least one of the functions identified by the FA for three of four participants. The experts’ analyses also agreed with the informant-based assessment at a statistically significant level for two of three participants with a conclusive QABF. Altogether, the results suggest that ABC narrative recording may be useful to generate hypotheses to identify the function of problem behavior, but that more research is needed before recommending its use as a standalone functional behavior assessment.     

Aneurysmal bone cyst of the orbit : a case report with literature review

Aneurysmal bone cyst (ABC) is benign vascular lesion destructing the cortical bone by the expansion of the vascular channel in the diploic space that usually involve long bone and spine. Orbital ABC is rare and the clinical symptoms deteriorate rapidly after initial slow-progression period for a few months. A 12-year-old female patient visited ophthalmologist due to proptosis and upward gaze limitation of the right eye, and orbital mass was noted in the upper part of right eye on orbital MRI. Five months later, exophthalmos was worsened rapidly with other features of ophthalmoplegia. Orbital mass was enlarged on MRI with intracranial extension. Surgery was done through frontal craniotomy and intracranial portion of the tumor was removed. Destructed orbital roof and mass in the orbit was also removed, and surrounding bone which was suspected to have lesion was resected as much as possible. Histopathological diagnosis was aneurysmal bone cyst. Postoperative course was satisfactory and the patient's eye symptoms improved. Authors report a rare case of orbital ABC with review of the literature. Exact diagnosis by imaging studies is important and it is recommended to perform surgical resection before rapid-progressing period and to resect the mass completely to prevent recurrence.     

GDNF 缓释微球及NogoA,ChABC 缓释微球联合促大鼠损伤脊髓再生修复的实验研究

The purpose of this study was to evaluate the effect of poly(lactide-co-glycolic acid) delayed-release microspheres,which were prepared using glial cell line-derived neurotrophic factor(GDNF),on the delayed-release,controllability,and protection of GDNF activity.The present study is the first to combine chondroitinase ABC,GDNF,and Nogo A antibody delayed-release microspheres for the treatment of spinal cord injury.Results show that the combined therapy of chondroitinase ABC,GDNF,and Nogo A antibody microspheres can increase the immunoreaction of neurofilament 200 in the injured spinal cord,and this therapeutic effect was better than chondroitinase ABC,GDNF,or Nogo A antibody microspheres administered singularly.     

A huge occipital osteoblastoma accompanied with aneurysmal bone cyst in the posterior cranial fossa

Osteoblastoma is an infrequent benign tumor and skull involvement is extremely rare. The occipital bone is much less frequently involved. We report an unusual case of a huge occipital osteoblastoma with aneurysmal bone cyst in the posterior cranial fossa of a 20-year-old young man. MRI scan and CT three-dimensional reconstruction revealed that the tumor (approximately 8.5 cm x 6 cm x 5 cm) occupied nearly half volume of the posterior cranial fossa. The cerebellum, the forth ventricle and brain stem were compressed remarkably and hydrocephalus was noted. The tumor mass was multiloculated cystic and highly vascular. Excessive bleeding occurred during operation and total removal was achieved. The patient was symptom-free and MRI scan revealed no evidence of recrudescence after a follow-up period of 18 months. Pathological examination revealed that the lesion was a benign osteoblastoma with secondary aneurysmal bone cyst (ABC). ABC constitutes a highly vascular lesion while complete surgical resection is often impeded by excessive intraoperative bleeding. Preoperative embolization may reduce the risk. Despite the benign nature of osteoblastoma, local recurrence after incomplete resection is not rare and malignant transformation during recurrence may occur even after adequate surgery. Our case confirms the findings of previous investigators that if osteoblastoma is removed completely, no recurrence is expected even without adjuvant irradiation and chemotherapy.     

Immunohistochemical localization of chondroitin sulfate in normal and pathological human muscle

The immunohistological localization of chondroitin sulfate (CS) has been studied in normal and pathological human muscle. The bovine nasal cartilage proteoglycan digested with chondroitinase ABC (BNC-PG-Ch ABC) has been utilized for the production of a rabbit polyclonal antiserum. In vitro studies showed that the antiserum binds to the unsaturated disaccharide that remains attached to the core protein after digestion of the CS chains with chondroitinase ABC (Ch ABC). As the disaccharide is created specifically by Ch ABC digestion of the CS chains, the antiserum allows the immunolocalization of CS on tissue sections digested with Ch ABC. The immunohistochemical study on normal and pathological muscle demonstrated a localization of CS in all the extracellular structures: endomysium, perimysium, muscle spindle capsule and intrafusal space. In pathological conditions, the CS was raised in all the cases with increased connective tissue, showing a pattern comparable to that obtained with fibronectin and collagen III. None of the pathological conditions displayed any peculiar character of CS distribution. This finding does not support a primary role for CS in the pathogenesis of muscular dystrophy.     

Factor Analysis of the Aberrant Behavior Checklist in Individuals with Autism Spectrum Disorders

Exploratory factor analysis (varimax and promax rotations) of the aberrant behavior checklist-community version (ABC) in 275 individuals with Autism spectrum disorder (ASD) identified four- and five-factor solutions which accounted for >70% of the variance. Confirmatory factor analysis (Lisrel 8.7) revealed indices of moderate fit for the five-factor solution. Our results suggest that the factor structure of the ABC is robust within an ASD sample. Both solutions yielded a three items self-injury factor. Stratifying on this factor, we identified significant differences between the high- and low-self injury groups on ABC subscales. The emergence of a self-injury factor, while not suggestive of a new subscale, warrants further exploration as a tool that could help dissect relevant neurobiobehavioral groups in ASD.     

汉语失语症计算机辅助流利性检测的研究

目的研究基于计算机模糊智能识别运算的语言障碍诊治仪ZM2．1（简称：语言障碍ZM2．1）对失语流利性检测的效度与信度。方法采用ABC法和语言障碍ZM2．1两种方法分别对58例失语症患者进行流利性检测，两种方法检测间隔时间在3d之内。结果ABC法流利性内部一致性检验，整体克朗巴赫α系数（Cronbach α）=0．8732；语言障碍ZM2．1平均语速和ABC法流利性总分有正相关性趋势，Spearman系数为0．721（P〈0．01）；语言障碍ZM2．1检测流利性的准确度为81％（以XABC法为标准）；测得两者流利性结果一致性良好（P〈0．01）。结论语言障碍ZM2．1在评价失语症流利性方面具有良好的信度和效度，可用于临床进行失语症的流利性检测。