Distinctive appearance of the distal phalanges in children with primary hypothyroidism.

An osseous projection from the midportion of the metaphysis of the distal phalanges of the digits was seen in 79% of untreated children with primary hypothyroidism. There is a significant difference (p less than .0005) in the incidence of this projection between children with primary hypothyroidism and those with idiopathic hypopituitarism or psychosocial dwarfism. The presence of this projection together with delayed skeletal should suggest primary hypothyroidism.     

Radiographic aspects of the hand in diffuse idiopathic skeletal hyperostosis

Radiographs of the hand of 53 patients with diffuse idiopathic skeletal hyperostosis (DISH) were compared with 20 acromegalic subjects and 100 matched controls. Bone qualitative changes, characteristic of DISH, include: (1) arrow-heading of the distal phalangeal tufts; (2) enthesopathy of the proximal phalanges; (3) enlarged sesamoid bones; (4) hook-like exostoses at the metacarpophalangeal heads; (5) cortical thickness of diaphyseal tubular bones. Therefore, the hand in this disease appears as a "robust" form, particularly in males, resembling acromegalic pattern, but in this disease, the hand is much more coarse (so called squared hand), especially because of the soft tissue involvement and articular cartilaginous hypertrophy. Only enthesopathy seems to be the most characteristic and peculiar finding of the disease. Quantitative bone change measurements have demonstrated in DISH that metacarpal and Exton Smith indices differ statistically toward the controls. The hand is wider, but in males bone mass may result the same and in female is shortly reduced. Soft tissue alterations, compared with the controls, are not significant. On the contrary, in acromegaly there is a significant statistical difference in all the quantitative measurements of both the skeletal and soft tissue lesions, compared with the controls and patients affected by DISH.     

MR imaging of brain maturation in normal and developmentally handicapped children.

White matter myelination was assessed in the frontal, temporal, and occipital lobes and in the internal capsule in 91 neurodevelopmentally handicapped infants on T2-weighted images (spin echo 3,000/120 ms) and compared with myelination scores from 53 normal control subjects. Clinical diagnosis was birth asphyxia (34), seizures with delays of various causes (33), congenital infections (15), and intracerebral hemorrhages (9). Myelination in the total group of patients was generally delayed. However, we found distinct differences in myelin deposition between groups. Myelination of handicapped children with seizures or with intrauterine infections was retarded most severely at all ages. Children with intracerebral hemorrhages were almost never significantly different from normals in any part of the brain, whereas children with birth asphyxia had myelination scores in between. We conclude that magnetic resonance staging of developmental processes, such as myelination, in the infants' brain helps to recognize delays at an early age.     

Radionclide imaging in skeletal inflammatory and ischemic disease in children.

Sixty-five children were evaluated for presence of skeletal inflammatory and ischemic disease with bone scans and roentgenograms. Several characteristic scintigraphic patterns were observed. Bone scans were significantly more sensitive than roentgenograms in early diagnosis of osteomyelitis and its differentiation from cellulitis, septic arthritis, and bone infarction. The child presenting with possible inflammatory bone disease now is benefited by this important refinement in diagnosis. Faced with the difficult dilemma of choosing appropriate therapy in these frustratingly similar problems, the physician can integrate the clinical findings with nuclear imaging to arrive at early appropriate diagnosis and management.     

MRI of anterior cruciate ligament injuries and associated findings in the pediatric knee: changes with skeletal maturation

OBJECTIVE: The objective of our study was to evaluate the MRI characteristics of anterior cruciate ligament (ACL) injuries and associated findings relative to skeletal maturity. We also contrast the frequency of findings in this younger population to adult data. MATERIALS AND METHODS: Eighty-two consecutive knees with an MRI report diagnosis of ACL injury (partial tear, sprain, or complete tear) or tibial spine avulsion fracture imaged over 4 years were retrospectively reviewed. Patients were grouped by degree of skeletal maturity as determined from the MR images. The examinations were reviewed for the type of ACL injury, secondary imaging findings, and associated knee injuries. Findings were correlated to skeletal maturity, and frequencies were compared with adult data. RESULTS: ACL injuries were more common in boys in the skeletally immature group, but more common in girls in the skeletally mature group (p = 0.03). Tibial spine avulsion fractures were most common in skeletally immature patients (p < 0.01), whereas complete tears of the ACL were most common in skeletally mature patients. Associated injuries were less common in the skeletally immature group, but this trend did not reach statistical significance. Most secondary signs of ACL injuries occurred at similar rates in all groups with frequencies similar to those reported in adults. CONCLUSION: ACL injuries in skeletally immature patients are seen more often in boys. Tibial avulsion fractures and partial tears are more common in younger, less rigid skeletons that may absorb the forces of trauma. As children mature, complete ACL tears and associated injuries occur in frequencies approaching those patterns seen in adults. Similarly, skeletally mature girls are affected more often than mature boys.     

Soft-tissue tumors of the hand and wrist of children

We report 23 children with soft-tissue tumors of the hand and wrist. Tumors of blood and lymph vessel origin accounted for nine patients; giant-cell tumors of tendon sheaths and tumoral calcinosis occurred in four patients each and soft-tissue chondroma in three. Synovial osteochondromatosis and aggressive fibromatosis were each represented by one patient. Finally, there was one child with a malignant soft-tissue tumor, an embryonal rhabdomyosarcoma of the hand. Hand and wrist ganglia were excluded. Imaging methods used in investigating hand and wrist lesions are discussed but plain radiography remains the first and often only examination necessary.     

Imaging features of pseudoaneurysms of the hand in children and adults

OBJECTIVE: Our objective was to review the imaging features of pseudoaneurysms of the hand in 25 patients. The patients presented with a mass, peripheral paresthesia, or ischemia. Pseudoaneurysm of the hand is a rare and often clinically unsuspected diagnosis. Correct diagnosis is important because there are risks for distal embolic disease with ischemia or gangrene of the fingers, ulnar or digital nerve dysfunction, rupture, or bone erosion and joint destruction. Scant reports appear in the world literature, and this report is the first review, to our knowledge, of the imaging features. The cause may be a history of a single direct trauma or chronic trauma, as seen in patients with hypothenar or thenar hammer syndrome. CONCLUSION: Awareness of the specific imaging appearances of pseudoaneurysms of the hand and their complications may improve the accuracy of radiologic diagnosis, advance the preoperative workup, and prevent possible clinical complications such as digital gangrene, nerve dysfunction, and aneurysm rupture.     

Bone maturation in children with chronic renal failure. Effect of 1 alpha-hydroxy vitamin D3 and renal transplantation.

In 8 of 11 children with terminal chronic renal failure, bone age was retarded 6 months to 3 years. During hemodialysis the bone maturation rate decreased. 1 alpha-OH vitamin D3 treatment and renal transplantation normalized the bone maturation rate.     

儿童戈谢病骨骼影像特征分析

目的 探讨儿童戈谢病骨骼病变的X线平片及MRI表现特点.方法 109例戈谢病患儿均行常规脊柱正、侧位及双股骨正位X线检查,同时行骨盆止位检查18例,左腕正位检查14例;14例患儿同时行股骨MR平扫.MR扫描序列为标准T1WI、T2WI、T2压脂像冠状面及矢状而扫描.综合分析其影像特点.结果 骨骼异常X线征象包括:长骨干骺端烧瓶样畸形89例(81.7%)、骨质稀疏91例(83.5%)、长骨十骺端密度不同程度的减低86例(78.9%)、骨皮质变薄69例(63.3%)、溶骨性破坏31例(28.4%)、骨硬化12例(11.0%)、骨囊性病变16例(14.7%),病理性骨折26例(23.9%),其中椎体压缩性骨折24处、股骨颈陈旧性骨折5处、脊柱弧形后凸3例、股骨头碎裂变形4例、髋关节脱位4例.,14例股骨MRI显示,T1WI及T2WI骨髓内均可见异常信号,分别表现为T1WI、T2WI信号减低4例;在T2WI及压脂像信号广泛减低的基础上出现限局性高信号或混杂信号10例.结论 儿童戈谢病骨骼病变的影像表现有一定特征性,对临床诊治及观察疗效具有较大的帮助.

Abstract：

Objective To discuss the imaging features of skeletal changes in children with Gaucher disease on X-ray and MRI images.Methods One hundred and nine children with Gaucher disease were enrolled in this study.They all received routine X-ray for spine with anterior-posterior(A-P)and lateral view and bilateral femurs with A-P view.Among them.18 patients received X-ray for pelvic with A-P view.14 patients received X-ray for left wrist with A-P view.and 14 patients received MRI scan for femur.The MRI scan included T1-weighted imaging,T2-weighted imaging and fat-suppressed T2-weighted imaging with short tau inversion recovery(STIR)sequence.The imaging features of the X-ray and MRI images were analyzed retrospectively.Results The most common feature is osteoporosis,which presented in 91 cases (83.5%).Besides this,decreased density of metaphysis occurred in 86 cases(78.9%).erlenmeyer flask deformity of metaphysis occurred in 89 patients(81.7%),thinner cortex occurred in 69 cases(63.3%),osteolytic destruction occurred in 31 cases(28.4%).pathological fractures occurred in 26 cases (23.9%),osteosclerosis occurred in 12 cases(11.0%).cystic degeneration of bone occurred in 16 cases (14.7%),and dislocation of the hip occurred in 4 cases.All 14 patients received MRI presented abnormal signals.Among them,4 patients presented low signal intensity both on T1-weighted and T2-weighted images in bone marrow;the other ten presented high signal intensity mixed in low signal intensity areas on T2-weighted and fat-suppressed T2-weighted images.Conclusions The imaging features of skeletal changes in children with Gaucher disease are of some characteristics,which could provide useful information for the clinical treatment.     

儿童严重手爆炸伤的治疗

探讨儿童严重手爆炸伤治疗方法的选择。根据损伤特点,采取I期修复或延期修复,可有效恢复手的功能,降低感染率。     

MR findings in growth hormone deficiency: correlation with severity of hypopituitarism.

BACKGROUND AND PURPOSEGrowth hormone deficiency may present as an isolated deficit (IGHD) or in association with multiple deficiencies (MPHD). Previous studies have not compared the MR imaging findings with the severity of hypopituitarism. Our purpose was to determine whether MR imaging can distinguish between IGHD and MPHD.METHODSForty-four patients with growth hormone deficiency who were examined by MR imaging were included in this retrospective study. On the basis of the endocrinologic findings, 21 were determined to have IGHD and 23 to have MPHD. The presence, size, location, and morphologic characteristics of the stalk, the neurohypophysis, and the adenohypophysis were recorded in each case. Findings in the two groups were compared. Statistical significance was determined by t-test.RESULTSThe stalk was normal in one patient with IGHD and in none of those with MPHD; it was truncated or thin in 19 patients with IGHD (90%) and in only one with MPHD (4%); it was absent in 22 patients with MPHD (96%) and in only one patient with IGHD (5%). These differences between the two groups were highly significant. In 81% of the IGHD patients and in 91% of the MPHD patients the location of the neurohypophysis was ectopic. This difference between the two groups was not significant. Among IGHD patients, the adenohypophysis was of normal size in 13 patients (62%), small in six (29%), and absent in two (9%); the corresponding findings in MPHD patients were seven (30%), six (26%), and 10 (44%).CONCLUSIONThe majority of IGHD patients had a truncated or thin stalk and a normal or small adenohypophysis. An absent stalk and adenohypophysis are characteristic of MPHD. MR imaging can contribute to the prediction of the pattern and severity of hypopituitarism in patients with growth hormone deficiency.