Noriaki Kikuchi

Long-Term Follow-up of Radial Shortening Osteotomy for Kienböck Disease

BACKGROUND Three previous studies have investigated the long-term outcome of radial osteotomy in the treatment of Kienböck disease. However, none used patient-based assessment. The purpose of this study was to investigate the long-term clinical and radiographic outcomes of this osteotomy, including the subjective evaluation of the patient with use of the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire. METHODS A DASH questionnaire was sent to nineteen patients with Kienböck disease who had undergone a radial shortening osteotomy, and thirteen replied. The mean age at the time of surgery was thirty-nine years. On the basis of the Lichtman classification, six patients had stage-II, four had stage-IIIA, and three had stage-IIIB disease. Prior to surgery, ulnar variance was positive in six patients, neutral in four, and negative in three. The mean duration of follow-up was twenty-one years. Clinical evaluation, including calculation of the modified Mayo wrist score, and radiographic evaluation were also performed on twelve of the thirteen patients. RESULTS The mean DASH score was 8 points (range, 0 to 23 points), and patient satisfaction was high. Compared with the findings in the contralateral wrist, the mean range of motion was 81% in flexion and 82% in extension and mean grip strength was 88%. The mean modified Mayo wrist score was 83 points, and the clinical results were excellent in six patients, good in five, and moderate in one. The DASH scores tended to be worse in patients with Lichtman stage-IIIB disease. Follow-up radiographs revealed that the Lichtman stage had progressed in six of the twelve patients. CONCLUSIONS Although most patients had mild wrist pain, patient satisfaction and the clinical results were satisfactory following a radial shortening osteotomy. This procedure is a reliable long-term treatment for Lichtman stage-II and IIIA disease and may be a reasonable option for patients with stage-IIIB disease.

Snapping elbow with congenital radial head dislocation: case report.

An 11-year-old boy with congenital radial head dislocation experienced painful snapping of his left elbow upon movement. He had no previous history of trauma. A plain radiograph of his left elbow showed anterior dislocation of the radial head and flexion deformity of the hypoplastic radial neck. Arthroscopy showed that the snapping of the elbow occurred between the annular ligament and the dislocated radial head during elbow flexion and extension. After the annular ligament was released, the snapping immediately disappeared. Five years after the surgery, the patient has no pain or snapping upon elbow movement.

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.

Sec6/8 regulates Bcl-2 and Mcl-1, but not Bcl-xl, in malignant peripheral nerve sheath tumor cells

Sec6 and Sec8, which are components of the exocyst complex, has been concerned with various roles independent of its role in secretion, such as cell migration, invadopodia formation, cytokinesis, glucose uptake, and neural development. Given the vital roles of the exocyst complex in cellular and developmental processes, the disruption of its function may be closely related to various diseases such as cancer, diabetes, and neuronal disorders. Malignant peripheral nerve sheath tumors (MPNSTs) have high malignant potential and poor prognosis because of aggressive progression and metastasis. To date, no chemotherapeutic agents have been validated for MPNSTs treatment because how MPNSTs are resistant to chemotherapeutic agents remains unknown. This study demonstrates that combination of doxorubicin and sorafenib induces apoptosis in MPNST cells through downregulation of B cell lymphoma protein 2 (Bcl-2), Bcl-2-related protein long form of Bcl-x (Bcl-xl), and myeloid cell leukemia 1 (Mcl-1). Moreover, both Sec6 and Sec8 levels decreased after treatment with doxorubicin and sorafenib and were found to be associated with Bcl-2 and Mcl-1 expressions, but not Bcl-xl. Although Sec8 was found to be involved in the regulation of both Bcl-2 and Mcl-1 at the mRNA level, Sec6 regulated Bcl-2 at the mRNA level and the binding affinity of F-box and WD repeat domain containing 7 and Mcl-1, thereby controlling Mcl-1 at the protein level. Bcl-2 or Mcl-1 mRNA suppression by Sec6 or Sec8 depletion resulted in significant changes in nuclear factor-kappa B, cAMP response element, and p53 transcriptional activity. These results suggest that Sec6 and Sec8 are therapeutic target molecules in MPNST.

Occurrence of Central Polydactyly, Syndactyly, and Cleft Hand in a Single Family: Report of Five Hands in Three Cases

Central polydactyly, syndactyly, and cleft hand are categorized separately in the International Federation of Societies for Surgery of the Hand classification. However, some investigators have proposed that these malformations should be classified into a single category: abnormal induction of finger rays. In this article, we report 5 hands with central polydactyly, syndactyly, and cleft hand in 3 patients from the same family and discuss the phenotypes in each hand.

Aberrant muscle syndrome: Hypertrophy of the hand and arm due to aberrant muscles with or without hypertrophy of the muscles

Five patients were reported in our congenital anomaly registry who had six hands in total with muscular hyperplasia, aberrant muscles, ulnar drift of the fingers in the metacarpophalangeal (MP) joints, flexion contractures of the MP joints, and enlargement of the metacarpal spaces. Thirty patients with unilateral involvement of this condition have been reported previously. We reviewed these cases and found that the condition varied in severity and that it was reported using different names. However, this condition seems different from true macrodactyly and multiple camptodactyly, including windblown hand, and seems to be an isolated entity of congenital upper limb anomaly. The authors recommend ‘aberrant muscle syndrome’ or ‘accessory muscle syndrome’ as a diagnostic name, because this seems to be the most common pathological finding in this condition.

Significant improvement in temporary dental rehabilitation by notable miniplate application in the hard palate for a patient with a large anterior maxillary defect: a case report/technique article.

The present report describes the application of a miniplate in the hard palate of a 36-year-old patient with a large anterior maxillary defect. The combination of orthodontic elastics with a titanium miniplate improved the stability of the prefabricated prosthesis. This structure retained the ointment gauze covering the wounds and maintained the facial contour. In addition, contracture deformity was prevented by insertion of the prefabricated prosthesis intraoperatively or immediately postoperatively. Furthermore, a soft diet could be ingested immediately postoperatively. The miniplate also supported the anterior part and the definitive prosthesis. This prosthesis restored adequate masticatory, deglutitive, and speech functions and maintained the facial contour with minimum overloading of the remaining teeth.

DE QUERVAIN DISEASE CAUSED BY ABDUCTOR POLLICIS LONGUS TENOSYNOVITIS: A REPORT OF THREE CASES

De Quervain disease is caused by a stenosing tenosynovitis in the first dorsal compartment, and the main aetiology is extensor pollicis brevis (EPB) tenosynovitis. We encountered three cases in which EPB tenosynovitis was absent and abductor pollicis longus (APL) tenosynovitis was confirmed during operation. In the treatment of de Quervain disease, APL tenosynovitis should be paid as much attention as EPB tenosynovitis.

Myoepithelioma of the upper lip

Myoepithelioma is a rare form of salivary gland tumor composed entirely of myoepithelial cells. This tumor was formerly considered to be a subtype of pleomorphic adenoma; however, in the 1991 World Health Organization classification, it is listed as an independent entity. The most favorable site of occurrence of myoepithelioma is the parotid gland. Here, we report an extremely rare case of myoepithelioma of the upper lip. A 56-year-old woman presented with a painless mass on her upper lip. Magnetic resonance imaging revealed a 23 mm × 18 mm well-defined ovoid tumor. A benign minor salivary gland tumor was clinically suspected, and the patient underwent complete resection of the tumor under general anesthesia. The tumor was histopathologically diagnosed as a benign myoepithelioma of the minor salivary gland. Immunohistochemically, the tumor cells were positive for S-100 protein, AE1/AE3, CAM5.2, CK7, vimentin, and calponin, confirming the morphologic diagnosis of myoepithelioma. The patients postoperative clinical course was uneventful, and satisfactory results were obtained both functionally and esthetically. To the best of our knowledge, this is the sixth case of myoepithelioma of the upper lip reported in English-language research.

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

ABSTRACT  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.