儿童组织细胞增生症X颅骨病变的影象学诊断

分析儿童组织细胞增生症X颅骨病变的影像学表现和特点，并结合病理阐述其在临床诊断中的价值。材料和方法：回顾性分析经病理证实的儿童组织细胞增生症X伴颅骨破坏者21例，其中男性16例，女性5例。年龄从8个月～9岁，平均3．8岁。除X线平片外，13例还进行了CT扫描，其中单纯平扫5例，平扫＋增强8例。结果：21例颅骨破坏中，颅顶部破坏20例，占95％；颅底部9例，占42．9％；眼眶7例，占33．3％；面颅骨3例，占143％。结论：本症颅骨病变在X线影像学上有较特殊的表现，是临床诊断和鉴别诊断的重要手段，有时甚至可起到决定性的作用，并能有助于观察疗效，了解有无复发等。     

骨血管源性肿瘤的影像学诊断(附23例报告)

目的探讨骨血管源性肿瘤的影像学特点,旨在提高其诊断水平。方法对发生在不同部位的23例骨血管源性肿瘤的X线片、CT片和MRI片进行回顾性分析。结果19例骨血管瘤典型影像学表现根据其发生部位分为3种:椎体为栅栏状或网眼状;颅骨为放射状;长管状骨为多囊状或泡沫状。19例中术前误诊为骨纤维异常增殖症和骨巨细胞瘤各1例。4例骨血管肉瘤的影像学主要表现为溶骨性骨质破坏或骨质破坏与成骨增生混合存在。术前误诊为骨纤维异常增殖症及脊索瘤各1例和未定性2例。4例术后虽然进行放、化疗,但仍有2例在短期内发生肺及纵隔转移。结论典型骨血管瘤的影像学具有一定的特征性表现,结合临床资料,一般可做出诊断。而骨血管肉瘤的影像学表现无特征性。     

Normal and abnormal single photon emission computed tomography of the skull: comparison with planar scintigraphy

Using a rotating gamma camera the normal single photon emission computed tomography (SPECT) anatomy of the skull was defined in eight subjects. The value of SPECT as compared with planar scintigraphy was assessed in 34 patients with known or suspected disease of the skull. Seven patients had normal planar scintigraphy and SPECT. In 12 of 27 patients with bone involvement SPECT and planar scintigraphy showed essentially the same findings. In 15 patients SPECT was superior to planar scintigraphy. In three of these patients SPECT detected lesions while planar scan was normal. In the other 12 patients SPECT showed better anatomic localization and defined the full extent of the lesion. This was most obvious in patients with involvement of sphenoid, petrous, clivus, maxilla, and zygomatic bones. Our findings confirm the potential of SPECT to detect lesions in deep bones that are overlapped by superficial bony structures that cannot be visualized clearly with planar scintigraphy.     

家族性颅骨锁骨发育不全的影像学表现(附6例分析)

目的探讨家族性锁骨颅骨发育不全的X线及CT表现特征。方法分析6例(2个家庭)锁骨颅骨发育不全的临床、X线及CT表现,并从遗传学角度进行探讨。结果家族性锁骨颅骨发育不全的X线及CT表现主要为颅顶膨隆或下陷、囟门未闭、颅缝增宽、多发缝间骨、颅底相对较窄,蝶骨短、蝶窦小,颅底骨明显增厚,并呈棉花团状改变,边缘模糊、牙齿发育不良、锁骨发育不全或缺如、全身骨骼发育不全。结论家族性锁骨颅骨发育不全的X线及CT表现具有特征性,能够作出正确的诊断。     

Paget's disease. Radiological changes occurring in untreated patients and those on therapy with salmon calcitonin during two years' observation

Radiological observations over two years were made in 23 patients with Paget's disease. Radiological criteria for progression and regression of disease are defined. Fourteen of the patients were treated with salmon calcitonin. Of these treated patients four showed regression of the disease and only two showed progression, both in the skull vault. Of the remaining nine untreated patients none showed regression but two showed progression. The radiological findings confirm that calcitonin will prevent the extension and sometimes induce healing of Paget's disease in long bones but that the skull appears to respond differently. Radiological changes were slow to occur and yearly or two yearly follow up is sufficient.     

Imaging features of thalassemia

Thalassemia is a kind of chronic, inherited, microcytic anemia characterized by defective hemoglobin synthesis and ineffective erythropoiesis. In all thalassemias clinical features that result from anemia, transfusional, and absorptive iron overload are similar but vary in severity. The radiographic features of β-thalassemia are due in large part to marrow hyperplasia. Markedly expanded marrow space lead to various skeletal manifestations including spine, skull, facial bones, and ribs. Extramedullary hematopoiesis (ExmH), hemosiderosis, and cholelithiasis are among the non-skeletal manifestations of thalassemia. The skeletal X-ray findings show characteristics of chronic overactivity of the marrow. In this article both skeletal and non-skeletal manifestations of thalassemia are discussed with an overview of X-ray findings, including MRI and CT findings. Received: 21 August 1999; Revision received: 22 December 1999; Accepted: 15 February 1999     

右侧鼻腔-筛窦脊索瘤CT 和MRI 影像学表现一例

脊索瘤好发于中轴骨，以颅底及骶尾区域最为常见，发生在其他部位较罕见。笔者报道了1例经组织病理学检查结果证实的发生在右侧鼻腔-筛窦的脊索瘤病例，分别从临床症状、术前CT及MRI影像学表现、手术所见及术后组织病理学、免疫组织化学检查结果分析了该病的特点，并通过文献复习鉴别诊断，加深了对该病的认识，为临床诊断及治疗方式提供有价值的参考。     

Single photon emission computerized tomography of the skull

The diagnostic contribution of single photon emission tomography for detection of bone lesions of the skull was explored in 125 cases and compared with planar imaging. Twenty-one localizations (16% of the total group) were only visualized by scintitomography, these were predominantly lesions of the base of the skull and facial bones. Scintitomography gave a false negative result in only one lesion out of 49 visible on skull radiographs. Together with the revelation of unsuspected bone abnormalities, SPECT generally provides a better visualization of the skull lesions and their extent than does planar imaging. In cases where disease of the facial bones and the base of the skull is suspected, scintitomography is an indispensable adjunct to planar scintigraphy.     

Radiographic manifestations of congenital anomalies of the skull

Congenital anomalies of the pediatric skull are caused by a diverse group of disorders. For the purposes of this discussion, these entities can be classified according to the radiographic appearance of the skull, which may be similar in a variety of different diseases. Enlarged parietal foramina, sinus pericranii, aplasia cutis congenita, anterior fontanelle dermoid, cephaloceles, and craniolacunia are all examples of loceles, and craniolacunia are all examples of calvarial defects. Although there are numerous causes for wormian bones (Table 1), OI, cleidocranial dysplasia, congenital hypothyroidism, and hypophosphatasia are disorders that are commonly associated with defective ossification and the appearance of wormian bones. Osteopetrosis is an important example of rare bony dysplasias that cause sclerosis and hyperostosis of the skull. A partial list of other disorders causing similar radiographic findings is found in Table 2. Craniosynostosis results in an abnormality of skull shape. The suture(s) involved may be predicted by the deformed calvarial configuration. Knowledge of the growth and development of the skull and an understanding of the varied causes of congenital skull anomalies can enable the radiologist to provide the diagnosis or an informed differential diagnosis when confronted with a specific radiographic finding.     

Paget disease of the skeleton. Internal medicine aspects

Paget's disease of bone seems to be a slow virus infection of a single or several areas of the skeleton. Pagetic lesions are rather common among elderly people, but the disease does not manifest itself very often. Compared to the incidence of Paget's disease in England it seems to be rarer in this country. The pelvis is most frequently involved, followed by bones of the leg, skull, lumbar spine. The patients suffer from pain and deformities, arthroses of the adhering ankles, increased temperature of the area, nerve irritation and nerve damage due to increased bone growth (e.g. hearing loss due to Paget's disease of the skull). The development of sarcoma is rare and is only seen in severe cases. Diagnosis is made by X-ray, confirmed by bone biopsy, if necessary. Asymptomatic lesions are detected by bone scintigraphy. The activity of the disease is expressed by increased alkaline serum phosphatase and urinary hydroxyproline excretion. For treatment calcitonins and diphosphonates (especially EHDP, Diphos) are used. Both inhibit the overactive osteoclasts, and the increased bone turnover is normalized. The patients feel considerable relief; the elevated biochemical parameters fall to about 50% of initial values after calcitonin or EHDP monotherapy. In severe cases the combination of both substances may be profitable. The cytostatic drug mithramycin which can also be effective is only needed in exceptional cases.     

MRI findings in headbangers

Magnetic resonance imaging (MRI) findings in a mentally retarded adult female who exhibits headbanging behavior are presented. Radiographic changes include enlargment of the diploic space in the parietal and occipital bones, and gray matter loss adjacent to the bony changes. This pattern of injury is compared with skull changes previously reported in headbangers, and neuronal injury seen in boxers (dementia pugilistica) and Minimata disease.     

Radiographic features of craniometadiaphyseal dysplasia,wormian bone type

We describe the radiographic findings in two siblings with a previously unrecognized craniotubular bone dysplasia. We call this condition craniometadiaphyseal dysplasia, wormian bone type. Because the parents of the siblings are consanguineous, this is probably a genetically determined condition with an autosomal recessive type of transmission. The findings in the siblings are compared with those of a woman with the same condition, previously reported as an example of craniometaphyseal dysplasia. The combination of findings in these patients seems diagnostic: characteristic skull changes including multiple wormian bones; wide long tubular bones without normal metaphyseal flaring; wide short tubular bones without normal diaphyseal constriction and sometimes actual diaphyseal expansion; and wide ribs and clavicles.     

颅骨生长性骨折的X线诊断（附9例报告）

儿童时期颅骨骨折，易发展成颅骨缺损──颅骨生长性骨折。本文报告颅骨生长性骨折９例，对其形成机理，Ｘ线表现及其诊断依据进行了重点讨论，并强调了对儿童颅骨骨折定期拍片复查、及早诊断本病的重要性。     

骨纤维囊性骨炎影像学表现与病理分析

目的:探讨骨纤维囊性骨炎的影像学表现及病理特点。方法:12例患者中男5例,女7例;年龄18～47岁;X线检查12例,CT检查6例,MRI检查2例,骨扫描1例。结果:12例均为多骨多病灶骨炎。以骨盆骨、颅骨多见,上下肢骨、肋骨、脊柱骨次之。X线片及CT图像上以囊样骨质破坏为主,呈单囊或多囊,病灶可有膨胀;边界有硬化边;皮质变薄,可有缺失;无软组织肿块生成;无骨膜增生;部分发生病理骨折。颅骨病灶可呈类圆形团絮状密度增高影。MRI表现为T1WI呈低信号,T2WI呈稍高信号,内可有小类圆形液体高信号。病灶直径大小不一,以0.5～3 cm居多,形成棕色瘤。病理以纤维组织为主,周围有骨样组织,棕色瘤者有多核巨细胞和吞噬细胞。结论:本病影像学和病理表现具有相对特征性,临床、影像、病理三者结合可减少误诊;切除甲状旁腺病变后,骨骼病变大多可逐渐恢复功能。     

Intraosseous lipoma of the frontal bone. A case report

Intraosseous lipomas are among the most uncommon bone tumors. They arise most often in the appendicular skeleton. There are very few reported cases of intraosseous lipomas within the skull bones. We report a case of intraosseous lipoma of the frontal bone with conventional radiography and CT findings.     

Craniofacial melorheostosis

Melorheostosis is a rare benign disease of cortical bone most frequently presenting as peripheral hyperostosis with a characteristic "melting wax" appearance on conventional radiographs. The disease most frequently affects the appendicular skeleton and is seen only rarely in the craniofacial bones. We discuss a case of melorheostosis in the nasal cavity and skull base with an atypical radiographic appearance and suggest findings that may differentiate craniofacial melorheostosis from more common entities in this region.     

Clinical aspects and radiology of histiocytosis X in the skeleton--a retrospective study of 18 patients

The present paper presents the clinical and radiological findings from a retrospective study of 18 children suffering from histiocytosis with skeletal involvement (20 lesions) treated between 1971 and 1983. The different forms of manifestation on the skull, vertebral column, and long bones are described and problems of differential diagnosis discussed. Possible forms of therapy are considered.     

40例儿童郎罕细胞组织细胞病骨骼病变的影像学诊断

目的 :探讨儿童郎罕细胞组织细胞病 (LCH)骨骼病变的影像学表现及其诊断价值。材料和方法 :回顾性分析经病理证实的儿童LCH 40例 ,均行X线平片检查 ,其中 8例CT扫描。 14例分别于 1个月～ 2年内随访。结果 :本组中颅骨破坏 32例 ,占 80 %;脊柱破坏 9例 ,占 2 2 .5 %;长骨破坏 13例 ,占 32 .5 %;扁骨及不规则骨破坏 4例 ,占 10 %。嗜酸性肉芽肿以颅骨、长骨和脊柱多发 ,其他类型以颅骨多发。结论 :LCH骨骼病变具有多发性、多样性和多变性的特点 ;X线平片是本症诊断的基本手段 ,CT对隐蔽部位病变及病变细微结构的显示优于平片。     

Wormian bones in osteogenesis imperfecta and other disorders

When are Wormian bones significant is not an easy question to answer, but its relevance is important in relation to bone dysplasias such as osteogenesis imperfecta. Recognition will differ with age of patient, radiographic objectivity, and personal subjectivity. In order to attempt an answer, the skull radiographs of 81 cases of osteogenesis imperfecta of varying ages were examined for the presence of wormian bones. These were compared against the incidence of Wormian bones in 500 skull radiographs of normal children. Significant Wormian bones as against normal developmental variants were considered to be those more than 10 in number, measuring greater than 6 mm by 4 mm, and arranged in a general mosaic pattern. They were found in all the cases of osteogenesis imperfecta but not in the normal skulls. The occurrence of significant Wormian bones in other bone dysplasias from our material and that of the literature was recorded. Other incidental findings in the skulls of the cases of osteogenesis imperfecta were also appraised.     

Craniodiaphyseal dysplasia: Evolution over a five-year period

A Negro girl has been followed from age 1 1/2 to 6 years for a severe musculoskeletal disorder with many of the clinical and roentgenological characteristics of Camurati-Engelmann disease. Atypical features, however, have included a geographic pattern of sclerosis of the long bones, a markedly increased density of the neural arches of the vertebral column, and a sharply demarcated sclerosis in the calvaria. The findings in the long bones and spine have become somewhat less pronounced over a five-year period, but the sclerosis of the skull has increased in both extent and thickness. Initially thought to have Camurati-Engelmann disease, the patient now is considered, on the basis of skeletal changes during this time, to have a diagnosis of craniodiaphyseal dysplasia.