Aneurysmal bone cyst of the spine in children. MRI imaging at 0.5 tesla

Two patients with aneurysmal bone cysts of the spine demonstrate striking similarities when examined with magnetic resonance imaging (MRI), while the plain radiographs and images on computed tomography (CT) are quite different. Gadolinium DTPA enhanced T1W images were especially helpful in the definition of the lesion by showing enhancement of the septations of the aneurysmal bone cysts. The septations represent the fibrous walls inherent to these lesions.     

Soft-tissue aneurysmal bone cyst: report of a case with t(5;17)(q33;p13).

We describe a primary aneurysmal bone cyst (ABC) of the soft tissue of the distal thigh in a 10-year-old girl. Radiographs showed an oblong density in the soft tissue that was consistent with hemorrhage or calcification; the underlying bone was unremarkable. Pathologic findings were consistent with myositis ossificans on gross examination, but numerous hemorrhagic cysts were present in the lesion. Histopathologic findings showed features of an ABC. Cytogenetic analysis of the tumor revealed 46,XX,t(5;17)(q33;p13); 17p13 breakpoints have been reported in intraosseous ABC. Thirteen months after diagnosis, the patient had excellent function and no radiologic evidence of recurrent disease. Soft-tissue ABC is a rare, benign lesion that can have a similar radiologic appearance to myositis ossificans but has a histologic appearance identical to that of its intraosseus counterpart.     

Aneurysmal bone cyst: a report of three cases.

Three children presenting with aneurysmal bone cysts are described. The first patient was 10 months old with a cyst of the scapula. The second was more typical but his cyst was treated initially as a malignant tumour. In the third child the second cervical vertebra was affected which posed considerable problems of management; it was treated by radiotherapy. Despite the problems all 3 children have made a good recovery.     

Sclerotherapy in aneurysmal bone cysts in children: a review of 17 cases

OBJECTIVE: To determine the efficacy of percutaneous sclerotherapy in the treatment of aneurysmal bone cysts. MATERIALS AND METHODS: Seventeen patients (7 girls, 10 boys) with aneurysmal bone cysts were treated by the percutaneous approach with Ethibloc ( n=14) and histoacryl glue ( n=3) in our institution between January 1994 and June 2000. The cysts were located in the extremities ( n=6), pelvis ( n=2), spine ( n=2), mandible ( n=5), rib ( n=1) and sphenoid bone ( n=1). Percutaneous sclerotherapy was performed with fluoroscopic and/or computed tomographic guidance under general anesthesia. Clinical and imaging follow-up lasted from 24 months to 9 years and 6 months (mean: 57.3 months). The results were quantified as: excellent (residual cyst less than 20% of the initial involvement), satisfactory (residual cyst 30-50%), unsatisfactory (residual cyst more than 50%). RESULTS: The age of the patients ranged from 4 years and 6 months to 15 years and 8 months (mean: 11 years and 2 months). In nine patients, the therapeutic procedure was repeated 2-5 times. Excellent regression was observed in 16 (94%), satisfactory results in 1 (6%). There was no failure (unsatisfactory result or no response to treatment) in this reported series. The complications were minor and included: local inflammatory reaction ( n=2), small blister ( n=1), and leakage ( n=1). Relief of symptoms was achieved in all patients. No recurrence was noted during follow-up. CONCLUSION: Percutaneous sclerotherapy of aneurysmal bone cysts with Ethibloc is safe and effective. It is an important alternative to surgery, especially when surgery is technically impossible or not recommended in high-risk patients.     

Elastic stable intramedullary nailing for the treatment of complicated juvenile bone cysts of the humerus.

Juvenile bone cysts usually are asymptomatic and may manifest as pathological fractures. Since the new method of flexible intramedullary nailing (" Elastic Stable Intramedullary Nailing" - ESIN or " Embrochage Centro- Médullaire Elastique Stable" - ECMES) has demonstrated superb results in the treatment of non-pathologic fractures of the long bones in childhood, this method is rapidly gaining popularity for the treatment of spontaneous or pathological fractures. Given the self-limiting natural history of juvenile bone cysts with eventual spontaneous healing, our goal is to stabilise the pathological fracture and the biomechanically weakened humerus. We treated 15 patients with 16 pathological fractures (one re-fracture) due to juvenile bone cysts of the proximal humerus. All fractures healed completely without pseudarthrosis. Complications were a secondary fracture in otherwise correctly positioned nails. Five of the 15 implants remain in situ, in 6 cases a repeat osteosynthesis was necessary, in one case the nails had to be changed because of the re-fracture. Ten of the 15 juvenile bone cysts healed over a period of 3 years, the nails were removed and so far there have been no further fractures in this group. In the other 5 cases, the juvenile bone cysts have progressively filled with sclerotic bone, and the nails remain in situ.     

Aneurysmal bone cyst in early childhood

A 1-year-old child with an aneurysmal bone cyst in the scapula is presented. The sonographic examination showed a cystic formation with intraosseal fluid levels that shifted after repositioning of the child. Similar findings have been reported on CT scans and it is suggested that shifting fluid levels are an indicative feature of aneurysmal bone cyst.     

Aneurysmal bone cyst secondary to infantile cartilaginous hamartoma of rib

We report the first case, in a 4-month-old girl, of an aneurysmal bone cyst secondary to infantile cartilaginous hamartoma of the rib. Infantile cartilaginous hamartoma of the rib (benign mesenchymoma) is a large, expansile tumour that is characterised by cartilaginous, vascular and primitive-appearing stromal and mesenchymal elements. Most cases are diagnosed at less than 1 year of age and may even be congenital. Aneurysmal bone cyst may be a secondary lesion to pre-existing tumours such as giant cell tumours, osteoblastomas, angiomas and chondroblastomas. This unique case is presented with radiological and pathological findings. Received: 15 November 1996 Accepted: 18 February 1997     

Mesenchymal hamartoma of chest wall in infancy: natural history of two cases

Two cases of mesenchymal hamartoma of chest wall in infancy are reported. These distinctive and rare lesions arise in the antenatal period, present at birth or in early life as chest wall masses with marked rib deformation, and may produce respiratory compromise through encroachment on the chest cavities. Histologically composed of chondroid and primitive mesenchymal elements with giant-cell formation, endochondral ossification, and maturation to trabecular bone, they exhibit some features of aneurysmal bone cyst. Because of their cellularity and proliferative appearance, they are often misinterpreted as sarcomas; however, they pursue a benign course and can be well managed surgically. Our cases possessed multiple chest wall masses that were clinically apparent at birth and sequentially resected over a period of several months, creating an opportunity to document their hitherto unreported histologic evolution. Their pattern of maturation is in keeping with a hamartomous malformation.     

Classic Adamantinoma with Osteofibrous Dysplasia-like Foci and Secondary Aneurysmal Bone Cyst

Adamantinoma, a rare bone lesion of the tibia and fibula, has two distinct variants, classic adamantinoma and osteofibrous dysplasia-like adamantinoma. Composite lesions have not been described. Aneurysmal bone cyst is a benign cystic lesion which may also occur in the tibia and fibula. We report an unusual case of classic adamantinoma with osteofibrous dysplasia-like areas and foci of secondary aneurysmal bone cyst with prominent giant cells. A lesion was diagnosed in a 17-year-old girl with a 14-year history of a slowly enlarging left tibial mass and increasing deformity. Pathologically, the predominant pattern was classic adamantinoma, with minor foci of osteofibrous dysplasia-like adamantinoma and areas of secondary aneurysmal bone cyst with abundant multinucleated giant cells. We report the clinical, radiologic, and pathologic features of this case, and summarize lesions associated with secondary aneurysmal bone cyst. To our knowledge, the association of adamantinoma with secondary aneurysmal bone cyst has not been previously reported.     

Mesenchymal Hamartoma of Chest Wall in Infancy Natural History of Two Cases

Two cases of mesenchymal hamartoma of chest wall in infancy are reported. These distinctive and rare lesions arise in the antenatal period, present at birth OT in early life as chest wall masses with marked rib deformation, and may produce respiratory compromise through encroachment on the chest cavities. Histologically composed of chondroid and primitive mesenchymal elements with giant-cell formation, endochondral ossification, and maturation to trabecular bone, they exhibit some features of aneurysmal bone cyst. Because of their cellularity and proliferative appearance, they are often misinterpreted as sarcomas; however, they pursue a benign course and can be well managed surgically. OUT cases possessed multiple chest wall masses that were clinically apparent at birth and sequentially resected over a period of several months, creating an opportunity to document their hitherto unreported histologic evolution. Their pattern of maturation is in keeping with a hamartomous malformation.     

Simple (unicameral) bone cyst of the calcaneus: a pathologic variant revisited

A 17-year-old girl was admitted to the hospital for surgery of an enlarging, painful mass of the left calcaneus. Preoperative imaging studies suggested either a simple (unicameral) or aneurysmal bone cyst. Intraoperative biopsy of the lesion revealed a simple bone cyst with extensive cholesterol clefts. Such cysts are not uncommon in the calcaneus. However, the pathology of this case is unusual and often overlooked. The typical presentation, treatment, and pathology of these lesions are reviewed.     

抗磷脂抗体和狼疮性肾炎

抗磷脂抗体是一族针对磷脂或磷脂一蛋白复合物的异质性自身抗体,可导致抗磷脂抗体综合征,近年来证实抗磷脂抗体和狼疮性肾炎关系密切。为了进一步了解抗磷脂抗体和狼疮性肾炎的关系,以便更好地指导临床,文章对近年来有关文献进行总结分析。系统介绍抗磷脂抗体、抗磷脂抗体综合征及其与狼疮性肾炎的关系。文献中抗磷脂抗体综合征已知病因的大部分病例中以系统性红斑狼疮占首位,而系统性红斑狼疮患者中常可检测到抗磷脂抗体。抗磷脂抗体综合征可导致肾脏的血管性损害,即肾脏的非炎症性血栓性血管病,在狼疮性肾炎患者中抗磷脂综合征肾病可以是另一个损害肾脏的重要因素,严重者可危及生命。抗磷脂抗体和狼疮性肾炎关系密切,对于狼疮性肾炎患者,应注意检测抗磷脂抗体及抗磷脂综合征肾病,给予合理的治疗。     

儿童重度闭合性肾挫裂伤的处理

目的 探讨儿童重度闭合性肾挫裂伤的处理.方法 回顾性总结分析2000至2008年临床诊治小儿闭合性肾损伤33例,重点是其中的15例重度肾挫裂伤(3例系肾积水合并肾挫裂伤的特殊病例)的资料.结果 全组无死亡,无早期肾切除病例,18例轻度肾损伤经保守治疗愈合.15例重度闭合性肾挫裂伤患儿中,其中8例采用保守治疗成功,1例肾蒂血管损伤早期急诊行肾静脉修补术,3例行选择性肾动脉栓塞治疗,3例肾积水外伤后肾破裂.3例肾积水肾破裂中,2例行早期肾盂成形肾周引流术,1例因合并肾功能不全,对侧肾萎缩,早期作肾造瘘,延期肾盂成形术,重度肾损伤病例随访5～28个月(平均18个月).4例继发尿性囊肿,1例早期行肾盂输尿管吻合,3例延期做尿性囊肿剥离切除;1例6个月后出现高血压、受损肾萎缩行肾切除.结论 轻度的肾损伤采用保守治疗易于治愈,重度的肾损伤因应病情选用相宜的方法 处理,绝大多数不需早期手术探查,以减少肾切除,选择性肾动脉栓塞微创止血效果好,重度肾挫裂伤需要密切随访,以处理相续可能发生的尿性囊肿和高血压等并发症.     

Completely isolated, noncontiguous duplication cyst.

Enteric duplication cysts are hollow, epithelium-lined, cystic, or tubular structures that are intimately attached to a portion of the gastrointestinal tract. Completely isolated duplication cysts are an extremely rare variety of gastrointestinal duplications with their own exclusive blood supply. Only two such previous cases have been reported in the literature so far. We report a third case of completely isolated, giant duplication cyst with a vascular pedicle in a four-day-old neonate with a review of the literature.     

Lymphatic malformations

Lymphatic malformations are low-flow vascular malformations that arise due to errors in vascular development. Lymphatic malformations are benign and usually noted at birth or in the first few years of life. Lymphatic mass lesions are composed of varying size of cysts; this article focuses on discussion of cystic lymphatic malformations. Lymphatic malformations can occur throughout the body especially in lymphatic rich areas such as the cervical and axillary locations as well as the groin, trunk, retroperitoneum, extremities, abdominal or thoracic cavities. Treatment options vary based upon size of cysts and location. A multimodal and interdisciplinary approach is essential to care for patients with lymphatic malformations. Management options include observation, pharmacotherapy, sclerotherapy, and surgical procedures.     

Surgery of benign bone lesions in H2 region of femoral neck in children北大核心CSCD

Objective To explore surgical procedures for benign bone lesions in H2 region of femoral neck in children. Methods From January 2010 to October 2022, the relevant clinical data were reviewed for 14 children with benign bone lesions of femoral neck in H2 region.There were 9 boys and 5 girls with an average age of 122(47-192) years.Six cases were diagnosed as fibrous dysplasia (monostotic FD, n=5; polyostotic FD, n=1). There were bone cysts (n=2), aneurysmal bone cysts (n=4), nonossifying fibroma (n=1) and osteoid osteoma (n=1). All lesions were located in H2 zone according to the classification scheme of International Society of Limb Salvage: Femoral Neck.Focal curettage and bone graft were performed via an anteriolateral Smith-Peterson approach.Lateral percutaneous femoral neck internal fixation was applied with cannulated screw and/or Kirschner wire. Results Surgical incision healed in one stage.Bone healing time was averaged at 4(3-6) months.No immune rejection occurred.During an average follow-up period of 25.5(6-74) months, there was no onset of recurrence, distant metastasis, postoperative pathological fracture, femoral head necrosis or secondary arthritis.Only 1 patient with pathological fracture had early closure of femoral head epiphysis.All of them obtained satisfactory functional rehabilitation of hip joint.The mean score of Musculoskeletal Tumor Society (MSTS) was 29(28-30) points. Conclusions For benign bone lesions in H2 region of femoral neck in children, anterolateral approach curettage and bone grafting plus lateral percutaneous internal fixation of femoral neck with cannulated screw and/or Kirschner wire have satisfactory outcomes. © The Author(s) 2023.     

Absent Pulmonary Valve with Intact Ventricular Septum Presenting as Cardiorespiratory Failure at Birth

Absent pulmonary valve with intact ventricular septum is a rare congenital anomaly. Most cases are not diagnosed until years after birth, although several cases have been reported in the literature in which cardiorespiratory distress developed during the neonatal period. We present a patient who showed improvement after fairly conservative therapy to alleviate the pulmonary vascular resistance, with ultimate palliative surgery. The aneurysmal dilatation of the pulmonary artery was for the most part confined to the proximal portion of the trunk, possibly because the ductus arteriosus was patent. The literature is reviewed.     

Computerised tomography in the evaluation of expansile lesions arising from the skull vault in childhood — a report of 5 cases

Expansile lesions of the skull vault are rare in childhood, and often present as relatively asymptomatic calvarial swellings. The cases of 5 children with expansile lesions of the skull vault due to both benign and malignant primary bone lesions are described. The value of computerised tomography in demonstrating that the “tumour” arises primarily from the skull vault as opposed to the underlying brain, and in demonstrating clinically unsuspected endocranial extension of the mass is described. The CT findings in 2 cases of aneurysmal bone cyst, including the significance of the presence of “fluid levels” in reaching a definitive diagnosis are discussed. The successful pre-operative embolisation in one case of aneurysmal bone cyst is reported.     

Immunohistochemical localization of an HIV epitope in cerebral aneurysmal arteriopathy in pediatric acquired immunodeficiency syndrome (AIDS)

A 6-year-old boy with acquired immunodeficiency syndrome (AIDS) developed aphasia and quadriplegia 3 months before his death. Cerebral vascular ectasia and multiple cerebral infarcts were noted on premortem radiological studies. Postmortem evaluation revealed diffuse aneurysmal dilatation of the circle of Willis associated with fresh and organizing thrombi, destruction of the elastic lamina, and marked intimal fibroplasia. Multiple cerebral infarcts and subacute AIDS encephalitis with basal ganglia calcification were also present. Immunohistochemistry with a monoclonal antibody (anti-gp41) to human immunodeficiency virus (HIV) demonstrated positively stained cells in the arterial wall of the circle of Willis and in the cerebral parenchyma. Double immunostaining demonstrated that gp41-positive cells in the circle of Willis were also positive for a macrophage marker or leukocyte-common antigen, but not with an endothelial marker. Some macrophages or microglia in the cerebrum were also colabeled with anti-gp41. These results suggest that HIV may be directly involved in vascular pathology associated with pediatric AIDS.     

Immunohistochemical Localization of an Hiv Epitope in Cerebral Aneurysmal Arteriopathy in Pediatric Acquired Immunodeficiency Syndrome (AIDS)

A 6-year-old boy with acquired immunodeficiency syndrome (AIDS) developed aphasia and quadriplegia 3 months before his death. Cerebral vascular ectasia and multiple cerebral infarcts were noted on premortem radiological studies. Postmortem evaluation revealed diffuse aneurysmal dilatation of the circle of Willis associated with fresh and organizing thrombi, destruction of the elastic lamina, and marked intimal fibroplasia. Multiple cerebral infarcts and subacute AIDS encephalitis with basal ganglia calcification were also present. Immunohistochemistry with a monoclonal antibody (anti-gp41) to human immunodeficiency virus (HIV) demonstrated positively stained cells in the arterial wall of the circle of Willis and in the cerebral parenchyma. Double immunostaining demonstrated that gp41-positive cells in the circle of Willis were also positive for a macrophage marker or leukocyte-common antigen, but not with an endothelial marker. Some macrophages or microglia in the cerebrum were also colabeled with anti-gp41. These results suggest that HIV may be directly involved in vascular pathology associated with pediatric AIDS.