Long-term results after simple rotational osteotomy of the radius shaft for congenital radioulnar synostosis.

BACKGROUND: The present study was conducted to clarify the long-term (≥10 years) results of simple rotational osteotomy for congenital radioulnar synostosis (CRUS). METHODS: Twelve forearms in 9 Asian patients with CRUS who underwent simple rotational osteotomy of the radius shaft were monitored for an average of 13.6 years (range, 10-19 years) postoperatively. Before surgery, the forearm fixation averaged 51.3° of pronation (range, 30°-90°). The true position of the forearm in ankylosis was measured by a line through the styloid processes of the radius and the ulna. Palm pronation and supination angles were also measured. The osteotomy was performed at the insertion of the pronator teres to the shaft of the radius. The pronation position was then corrected manually to allow 90° of palm supination with compensatory rotation around the wrist, and a cast was applied. We evaluated activities of daily living items at a mean of 5.2 years after surgery. At the final follow-up, the 11-item version of the Disability of the Arm, Shoulder and Hand score was recorded. RESULTS: After surgery, the forearm was fixed at an average of 4.2° of supination. At the final follow-up, the palm was able to achieve an average motion arc ranging from 26° of pronation to 62° of supination. There were no neurologic or circulatory complications after surgery. Ability to perform daily activities was markedly improved, and all patients were satisfied with the results of surgery. The average score on the 11-item version of the Disability of the Arm, Shoulder, and Hand was 3.79 points at the final follow-up. CONCLUSION: Our procedure for forearm rotation in patients with CRUS is simple, reliable, satisfactory, and safe.

The Nuss technique for Jeune asphyxiating thoracic dystrophy repair in siblings.

Jeune syndrome, or asphyxiating thoracic dysplasia, is an autosomal recessive osteochondrodysplasia. Four forms of Jeune syndrome have been proposed: lethal, severe, mild, and latent. In the severe form, respiratory failure leads to death in early infancy. We present 2 cases of a mild variant of Jeune syndrome in a 14-year-old girl and her 9-year-old brother, who were referred to us because of characteristic concave deformities of bilateral middle-lower chest walls without cardiopulmonary distress or renal failure. In addition, both showed short statue (-2.1 and -2.5 SD), progressive retinal dystrophy, and metaphyseal dysplasia (cone-shaped metaphysis and metacarpal brachydactyly). The chest wall deformity was treated at the age of 9 years in the sister and at the age of 7 years in the brother. According to the Nuss procedure, 2 bent bars were inserted into the thoracic cavity from each lateral intercostal space at the midaxillary line and pulled out over the lower end of the sternum. The ends of inserted bars were fixed to soft tissue over the sternum with nonabsorbable sutures. Conjoined costal cartilage around the sternum restricts the number of bars that can be positioned. A single bar for deformity of each side could not achieve complete reconstruction, but the patients and their parents were satisfied with the results cosmetically.

Trichorhinophalangeal syndrome type II without the chromosome 8 deletion that resembled metachondromatosis.

We describe a 5-year-old girl with features resembling Trichorhinophalangeal syndrome, type I (sparse scalp hair, bushy eyebrows, bulbous nose, long philtrum, cone-shaped epiphyses, clinobrachydactyly, epiphyseal changes in the femoral head and short stature), and appendicular exostoses similar to trichorhinophalangeal syndrome, type II. However, despite physical resemblance to the trichorhinophalangeal syndrome variants, cytological analysis showed a structurally normal chromosome 8 and no mental deficiency was apparent. In addition, morphological congruities between multiple exostoses and metachondromatosis was indicated from radiographic findings.

Immunohistochemical diagnosis of a rare case of epithelioid malignant peripheral nerve sheath tumor with multiple metastases.

BACKGROUND: The epithelioid variant of malignant peripheral nerve sheath tumor (MPNST) is a rare tumor with poor prognosis that sometimes involves the head and neck. The diagnosis is based principally on the histological examination, and it is generally very difficult to reach the correct diagnosis. CASE: An 84-year-old Japanese woman presented with a tumor mass of 2 week's duration in the right medial canthal region. OBSERVATIONS: Although the tumor was excised surgically, metastases occurred three times on her face and head, and the patient died of distant systemic multiple metastases. In the histopathological analysis, the tumor showed a composite pattern comprising spindle or polygonal cells arranged in irregular bands, and a population of larger epithelioid cells in solid sheets and nests. In the immunohistochemical analysis, the tumor cells were positive for S-100 protein, vimentin, and nerve growth factor receptor (NGFR), and negative for cytokeratin and HMB 45 (melanoma-associated antigen). These findings confirmed the diagnosis of MPNST. CONCLUSIONS: Epithelioid MPNST has complex histopathological findings and histopathological features similar to other epithelioid tumors, especially malignant melanoma. Immunohistochemical examination using NGFR and HMB-45 is important in the differential diagnosis.

Dendritic cell and effector cell infiltration in soft tissue sarcomas with reactive lymphoid hyperplasia.

The lymph node is the site of antigen presentation, and dendritic cells are sentinels for anti-tumor immunity. However, little is known about the histological features of lymph nodes and dendritic cells in soft tissue sarcomas. The reactive lymph node and infiltration of dendritic cells or effector cells were studied histologically in 10 soft tissue sarcomas with reactive lymphoid hyperplasia. The cases included four malignant fibrous histiocytomas, two malignant peripheral nerve sheath tumors, one synovial sarcoma, one epithelioid sarcoma, one malignant granular cell tumor, and one liposarcoma. The proportions of the T zone, lymphoid follicle, and lymphoid sinus (which was occupied by cells immunopositive for antibodies against CD3, CD20, or CD68) were 33.4% +/- 11.0%, 6.1% +/- 4.9%, and 13.5% +/- 6.5%, respectively. T zone hyperplasia was observed in all cases, and sinus histiocytosis was found in four. The proportion of the T zone in regional lymph nodes of soft tissue sarcoma patients was significantly higher than that in adult autopsy cases without a cancer history. CD8-, TIA-1-, or granzyme B-positive effector cells were found in each sarcoma tissue. Whereas CD1a-positive dendritic cells were not detected, S-100 protein-positive or CD83-positive dendritic cells were observed in five sarcoma tissues. The coefficient correlation between the numbers of effector cells and dendritic cells positive for CD83 or S-100 protein were demonstrated. Although this is a preliminary report, the present study demonstrated that some soft tissue sarcoma patients showed reactive lymphoid hyperplasia. Furthermore, the association between the infiltration of dendritic cells and that of effector cells was observed in patients with soft tissue sarcomas.

Intercalary non-vascularised toe phalanx transplantation for short finger-type symbrachydactyly.

A two-year-old boy with short finger-type symbrachydactyly involving the index, middle, and ring fingers was treated with intercalary nonvascularised toe phalanx transplantation into the middle finger to obtain stability of the middle finger before syndactyly release. He underwent syndactyly release one year after the transplantation. Two years after the transplantation, the clinical result was satisfactory, although X-ray showed fibrous union between the transplanted phalanx and the host phalanx. Intercalary nonvascularised toe phalanx transplantation is one of the way of stabilising a finger after syndactyly release.

Fragment fixation with a bone graft and dynamic staples for osteochondritis dissecans of the humeral capitellum.

To attain bony union of the fragment in osteochondritis dissecans of the humeral capitellum, fragment fixation was performed with a bone graft and dynamic staples in 4 patients. The staples were inserted not from the articular surface but from the lateral aspect of the capitellum. All patients achieved bony union without complication, and 3 of them returned to playing competitive baseball. At final follow-up after surgery (mean, 7.5 years [range, 2.1-11 years]), 3 patients were able to throw a ball without pain and the remaining patient felt elbow dullness after he played recreational-level baseball as a pitcher. These results suggest that the procedure of fragment fixation with a bone graft and dynamic staples can provide satisfactory results for osteochondritis dissecans of the humeral capitellum with a large osteochondral fragment.

Ultrasonographic assessment of the ulnar collateral ligament and medial elbow laxity in college baseball players.

BACKGROUND: The prevalence of medial elbow instability is high in athletes who throw, such as baseball players. The aim of this study was to assess the medial aspect of the elbow with ultrasonography to detect changes in baseball players. METHODS: Ultrasonography of the medial aspect of the elbow was performed, while gravity stress was applied with the elbow in 90 degrees of flexion, on thirty college baseball players (twelve pitchers and eighteen fielders) to assess medial joint laxity and deformity of the ulnar collateral ligament. The dominant (throwing) extremity was compared with the contralateral extremity. Clinical data were correlated with ultrasonographic findings. RESULTS: The medial joint space was significantly wider on the throwing side than it was on the contralateral side (2.7 mm and 1.6 mm, respectively; p < 0.01), and the proximal part of the ulna was shifted significantly laterally on the throwing side (p < 0.01). Angular deformity of the ulnar collateral ligament was found in five subjects, and it was significantly associated with lateral shift of the proximal part of the ulna (p < 0.01). Medial elbow pain was associated with widening of the medial joint space (p < 0.05) and with the presence of angulation of the ulnar collateral ligament (p < 0.01). CONCLUSIONS: Medial elbow laxity and elbow valgus on the throwing side of baseball players were increased compared with those in nonplayers. Angular deformity of the ulnar collateral ligament suggests that the ligament bends over the distal-medial edge of the trochlea. Ultrasonography can provide useful information about the condition of the ulnar collateral ligament and about medial elbow laxity in athletes who throw.