Hemi-tibia allograft and free microvascularized fibula transplant reconstitute the tibia shaft with side to side healing: 7 year follow up of a 14-year-old boy with adamantinoma.

Adamantinoma is a malignant tumor that usually presents in adult men between 20 and 50 years. Due to its metastatic potential, differentiating Adamantinoma from Osteofibrous dysplasia is essential as treatment varies greatly. We present a case of limb salvage using a free microvascularized fibula transplant and hemi-tibia allograft.

Sonographic evaluation of pediatric localized scleroderma: preliminary disease assessment measures.

BACKGROUND: Our earlier work in the ultrasonograpy of localized scleroderma (LS) suggests that altered levels of echogenicity and vascularity can be associated with disease activity. Utrasound is clinically benign and readily available, but can be limited by operator dependence. We present our efforts to standardize image acquisition and interpretation of pediatric LS to better evaluate the correlation between specific sonographic findings and disease activity. METHODS: Several meetings have been held among our multi-center group (LOCUS) to work towards standardizing sonographic technique and image interpretation. Demonstration and experience in image acquisition were conducted at workshop meetings. Following meetings in 2007, an ultrasound measure was developed to standardize evaluation of differences in echogenicity and vascularity. Based upon our initial observations, we have labeled this an ultrasound disease activity measure. This preliminary measure was subsequently evaluated on over 180 scans of pediatric LS lesions. This review suggested that scoring levels should be expanded to better capture the range of observed differences. The revised levels and their definitions were formulated at a February 2009 workshop meeting. We have also developed assessments for scoring changes in tissue thickness and lesion size to better determine if these parameters aid evaluation of disease state. RESULTS: We have standardized our protocol for acquiring ultrasound images of pediatric LS lesions. A wide range of sonographic differences has been seen in the dermis, hypodermis, and deep tissue layers of active lesions. Preliminary ultrasound assessments have been generated. The disease activity measure scores for altered levels of echogenicity and vascularity in the lesion, and other assessments score for differences in lesion tissue layer thickness and changes in lesion size. CONCLUSIONS: We describe the range of sonographic differences found in pediatric LS, and present our efforts to standardize ultrasound acquisition and image interpretation for this disease. We present ultrasound measures that may aid evaluation of disease state. These assessments should be considered a work in progress, whose purpose is to facilitate further study in this area. More studies are needed to assess their validity and reliability.

The use of Doppler ultrasound to evaluate lesions of localized scleroderma.

Doppler ultrasound shows great promise for the evaluation of localized scleroderma (LS). Disease-related structural changes, such as tissue thickening, atrophy, and architectural alterations, can be readily detected using ultrasound. High spatial resolution enables monitoring of changes in tissue thickness over the course of disease and treatment. Doppler ultrasound may also aid assessment of disease activity. Both abnormal tissue echogenicity and vascularity levels can represent disease activity in some patients. Because tissue thickness, echogenicity, and vascularity vary with body site and age, accurate ultrasound evaluation of the LS lesion requires comparison to a control site, ideally the contralateral side. Evaluation of deeper tissues is important, as disease involvement may reside primarily below the dermis. Further study is needed to determine the validity, sensitivity, and reliability of ultrasound for LS.

Osteochondromas and growth retardation secondary to externally or internally administered radiation in childhood.

For over five decades, osteochondromas (exostoses) and associated growth retardation have been known to be caused by radiation damage to the growing skeleton. Patients can be divided into three exposure groups. Group I received external beam radiation therapy primarily for the treatment of childhood cancers (typical dose 3,500 cGy), and 6-20% developed osteochondromas and growth retardation within the radiation portal. Group II consists of recently described patients who received total body irradiation in preparation for bone marrow transplant (typical dose: 800-1,200 cGy), and about 20% developed osteochondromas and growth retardation. Group III consists of 206 German children who in the 1940s and early 1950s received intravenous radioactive Peteosthor (Ra-224) to treat bone tuberculosis (estimated typical dose: 1,000-2,000 cGy), and 14% developed osteochondromas and growth retardation, among other benign and malignant sequelae. Combining these three exposure groups, osteochondromas and growth retardation develop in at least 6-20% of children who receive therapeutic radiation to their growing skeletons.

Role of computed tomography in guiding the management of peripheral bronchopleural fistula.

The present study was designed to elucidate whether demonstration of a peripheral bronchopleural fistula on CT correlated with the need for surgical management. We retrospectively identified 33 patients, 24 males and nine females, mean age 38 years, with clinical diagnosis of peripheral bronchopleural fistula and whose chest CT scans and medical charts were reviewed. Each chart was reviewed to identify the cause of the peripheral bronchopleural fistula and its treatment. Treatment decisions were categorized as surgical or conservative. Each chest CT was evaluated for the cause of peripheral bronchopleural fistula as follows: bulla(e), lung abscess/necrotizing pneumonia, neoplasms, peripheral bronchiectasis, and trauma. The peripheral bronchopleural fistula was classified as visible on CT if a distinct channel between the lung or a peripheral bronchus and the pleura was seen on the lung windows. We found that CT was useful in guiding surgery by identifying and localizing the cause of the peripheral bronchopleural fistula in the 55% (18/33) of patients who required surgery. The peripheral bronchopleural fistula or its probable cause was identified in 91% (30/33) as follows: bulla(e) (n = 12), lung abscess/necrotizing pneumonia (n = 11), peripheral bronchiectasis (n = 5), malignancy (n = 1), and posttraumatic pneumatocele (n = 1). The peripheral bronchopleural fistula was right-sided in 24, left-sided in nine, and was visible on CT in 36% (12/33). Among the patients with bullae, 58% (7/12) required surgery; however, the peripheral bronchopleural fistula was visible on CT in only 8% (1/12). Among the 21 patients without bulla(e), the peripheral bronchopleural fistula was visible on CT in 52% (11/21). When the fistula was visible in this subgroup, 73% (8/11) required surgery compared with 30% (3/10) in whom the fistula was not visible (p = NS; Fisher exact). In conclusion, CT was useful in guiding surgery by identifying and localizing the peripheral bronchopleural fistula or its probable cause. Peripheral bronchopleural fistulas caused by bulla(e) were less likely to be visible on CT (p < 0.05). Excluding patients with bulla(e), our data suggest a trend toward the need for surgical management for patients in whom the peripheral bronchopleural fistula was visible on CT.