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Background Congenital limb deficiency is a rare anomaly that impairs limb function. Transverse deficiency accounts for approximately half of congenital limb deficiency cases. In Japan, there have been no detailed data of clinical features, especially treatment approaches, of this disorder. The present study aimed to investigate the status of treatment approaches of congenital transverse limb deficiency in Japan. Methods From the national epidemiological survey of congenital limb deficiency undertaken in Japan in 2016, all the data of 200 patients with congenital transverse limb deficiencies were extracted. These data were analysed to reveal the treatment approaches of congenital transverse limb deficiency and its basic clinical features. Results Surgical treatments and prosthetic or orthotic intervention had been implemented or planned for about one-quarter of patients, respectively. In the upper limb deficiencies, prosthetic or orthotic intervention was likely chosen in cases of deficiency at the metacarpal or proximal to metacarpal level. Surgical treatment was chosen only in cases of deficiency at the carpal or distal to carpal level. Although the number of patients with transverse lower limb deficiencies was small, prosthetic or orthotic intervention was likely chosen in proximal deficiencies, and surgical treatment was likely chosen in distal deficiencies. Conclusions Herein, we revealed the status of treatment approaches for congenital transverse limb deficiency in Japan. Approximately half of the patients had no history of—and no plans for—surgical, prosthetic, or orthotic interventions. Further treatment advances may enable patients with congenital limb deficiencies to increase their participation in daily activities. Study Design Cross-sectional survey.

In this study, we retrospectively reviewed our results of managing the clasped thumb deformity in arthrogryposis multiplex congenita patients with first web space deepening and abductor pollicis brevis (APB) rerouting. Twenty-six thumb deformities in 16 children with a mean age of 6.2 years were treated with a mean follow-up of 89.6 months. Following reconstruction with the Spinner flap, the mean angle of the first and second metacarpal axis (M1M2) showed significant improvement from 42° to 61° indicating a wider first web space, and the postoperative mean angle of the thumb proximal phalanx to its metacarpal axis (M1P1) was reduced from 41° to 18°, indicative of a straighter thumb. Following reconstruction with a double-opposing Z-plasty, the mean M1M2 and M1P1 also improved from 38° to 52° and 18° to 9°, respectively. Rerouting of the APB with first web deepening is an efficient and reproducible procedure in improving thumb position. Level of evidence III

The functional dexterity test (FDT) is a timed pegboard test based on the manipulation of each peg and suitable for young children as it is both simple and quick to perform. We assessed the postoperative FDT values for children with Blauth type 2 hypoplastic thumbs after opponensplasty. We evaluated hand function using FDT for 12 hands of 11 patients with Blauth type 2 hypoplastic thumbs. Opponensplasty was performed in all hands following by Huber's procedure. All patients were evaluated from 6 to 12 months after surgery for hand function using three types of FDT scores: time in seconds to complete the test (FDT time), combined total time with penalty seconds added to the initial time (FDT total), and the number of pegs per second to complete the task as a percentage against normative values (FDT speed). We compared the postoperative FDT scores with those for Blauth type 1 or 2 hypoplastic thumb patients without surgical treatment. FDT time and FDT total for the patients postoperatively were both significantly shorter than those in the type 2 patients without surgical treatment. FDT speed was significantly higher than that for the patients without surgical treatment. There were no significant differences in the three FDT scores between the postoperative patients and the type 1 patients. There were several limitations including small sample size, large ranges of the data, and high number of variables. FDT reflected postopponensplasty improvement in hand dexterity in young children with Blauth type 2 hypoplastic thumb.

An ulnar cleft hand is a rare congenital deformity presenting with a cleft between the ring and little finger. It may be associated with the absence of a finger. The goals of treatment are cleft closure, increasing the length of the hypoplastic little finger and preservation of function. We report two patients with ulnar cleft hand without missing fingers. Both underwent cleft closure. One was treated by lengthening of the fifth metacarpal with an external fixator, and the other by finger translocation. Both patients achieved a near normal length of the little finger and a reasonable functional and aesthetic outcome. Level of Evidence: Level V (Therapeutic)

For the severely hypoplastic thumb, including floating thumb, it is difficult to acquire function while preserving the thumb. Pollicization using the index finger after removing the hypoplastic thumb is usually indicated for such cases. However, most parents of children undergoing surgery for deformed hands desire retaining the digit number, 1 thumb and 4 fingers. A floating thumb has a neurovascular band in its pedicle, and we can identify and divide the band with precision for preservation, although it seems impossible to retain it. For acquiring function while retaining the thumb, we developed carpometacarpal arthroplasty of the floating thumb. For the first stage, the fourth metatarsal head was transferred to the first metacarpal base, followed by third metatarsal half-slip transfer to the space of the excised fourth metatarsal head with the first web plasty using a dorsal sliding flap. Second stage surgery was performed ∼6 months after the first stage. For creating stable opponensplasty, the abductor digiti minimi tendon was anchored to the ulnar side of the metacarpophalangeal joint; for extension, extensor indicis proprius transfer to the hypoplastic extensor pollicis longus tendon was performed; for flexion, ring finger flexor digitorum superficialis transfer to the hypoplastic flexor pollicis longus tendon was performed. In general, the functional results were acceptable with a high degree of family satisfaction, although in some cases function was limited to simple object holding. We explain the surgical procedures to preserve the thumb while gaining function as well as appearance using high-quality illustrations, figures, and videos. This should be helpful for surgeons who want children with hypoplastic thumbs to have both 5 digits and reasonable functional ability.

Background: Lateral humeral condylar fractures often heal with some residual elbow deformity. However, details of angulation or tilting angle of the lateral condyle after the fracture have not been evaluated so far. Methods: Between 2008 and 2016, we followed up 80 mild fractures of the lateral humeral condyle for more than a year. Thirty fractures were treated by open reduction and internal fixation (ORIF) with Kirschner wires. Fifty cases were treated with a long arm splint for 3 weeks (Fig. 1). The average age of the patients at the time of the injury was 5.5 years. The humerus-elbow-wrist angle (HEWA), Baumann’s angle (BA), and tilting angle (TA) were measured on the radiographs. The active range of motion (ROM) was clinically assessed at unaffected and affected sides at the final follow-up. Results: No significant differences were detected between the sides about TA or ROM at the final follow-up. However, HEWA/ BA showed more significant loss of correction. There were significant differences in BA at the affected side between the ORIF and splint groups. Conclusions: Cubitus varus deformity after lateral humeral condylar fracture is not accompanied by a change in TA or ROM, unlike the deformity after supracondylar or distal epiphyseal fracture of the humerus (Fig. 2). Operative treatment to precisely correct and fix the lateral condylar fracture still retained some cubitus varus deformity, although it might lessen or prevent the deformity when compared to conservative treatment with a splint.

Exostosis is one of the most common benign bone lesions. It could be a solitary lesion or multiple lesions presented as part of HME. This condition often affects the metaphyseal region of long bones, including femur, tibia, humerus and ribs. Rib exostosis may cause severe complications such as pneumothorax, hemothorax, and diaphragmatic injury. We report two patients with asymptomatic rib exostosis who underwent surgical excision to prevent severe complications. The first patient was a 13-year-old male with HME. At the eight-year follow-up, the size of the exostosis at the left ninth rib was found to have increased to 5 cm in diameter and it compressed the diaphragm. Surgical excision was performed to prevent possible complications. The second patient was a 10-year-old male with HME. At the seven-year follow-up, the size of the exostosis at the right fourth rib was found to have increased to 5 cm in diameter and it protruded into the thoracic cavity. Surgical excision was performed to prevent possible complications. Both patients recovered uneventfully. The periodic evaluation of the size and shape of rib exostosis is important in HME patients, because we must judge the proper timing for surgical excision to prevent severe complications.

Radial club hand is a complex congenital abnormality of the radial or pre-axial border of the upper extremity. It has a wide range of phenotypes from hypoplasia of the thumb to complete absence of the radius and the first ray. Centralization with tendon transfer is a popular method for maintaining the correct position of radial club hand. On the other hand, various corrections were devised,e.g. radialization after distraction to emphasize the fact that the head of the ulna is positioned under the radial carpal bones and is no longer placed in a slot in the center of the carpus, microvascular epiphysis transfer, gradual correction using Ilizarov method, for Bayne Type III or Type IV. We should pay attention to the recurrence of radial deformity or circulatory impairment with the tension. Lunate excision or ulnar shortening can be selected for tension-free correction. Radialization can be indicated for avoiding the recurrence of radial flexion. However, we should pay attention of the radial protrusion of the ulnar head. For avoiding the recurrence of radial deformity or circulatory impairment, gradual correction using Ilizarov external fixation can be indicated, especially in the cases with severe radial deviation or with short forearm. In the mild cases, Bayne Type I or Type II, radius lengthening is accompanied by a soft-tissue distraction or release at the ulnar carpal joint with keeping wrist and forearm motion without producing growth plate damage.

The objective of this paper is to clarify the strength property of cement-treated clay subjected to cyclic loading during curing period. In this experiment, a series of unconfined compression tests was conducted in term of the clay type, the cement content and slag content to enhance the strength recovery, the timing and level of cyclic loading. The following conclusions are obtained. 1) No significant strength reduction is observed for cement-treated clay when cyclic loading is introduced during curing period. 2) The deformation modulus during cyclic loading increase 4 times of the specimen without cyclic loading. 3) When cyclic loading is subjected to cement- treated clay at the early stage of curing period (such as 7 curing day), the strength increases with increasing Ground-granulated blast furnace slag content. 4) When cyclic loading is subjected in the last stage of curing period (such as 28 curing day), the strength is almost constant irrespective of cyclic loading.

We reviewed the outcomes of post-traumatic varus deformity treated with modified step-cut osteotomy in 19 patients (average age, 7.4 years; range, 4.3-16.8 years at time of surgery). The average follow-up period was 29.6 months. The mean range of motion was 15.0°/124.7° (extension/flexion) before surgery and 6.8°/132.6° at final follow-up. The humerus-elbow-wrist angle was -21.1° before surgery and 4.2° at final follow-up, with a loss of 4.4° from the value of the humerus-elbow-wrist angle after the surgery. Osteotomy was fixed with Kirschner wires, and, in five cases, chips of excised bone could be inserted to avoid elbow extension. However, in eight cases, usually concerning younger patients, the elbow was fixed in hyperextension higher than 5°. No patient developed postoperative infections or later complications. Only one patient had transient nerve palsy. The modified step-cut osteotomy can precisely and stably correct the varus deformity in the coronal plane, especially in patients under 10 years of age. To avoid radial nerve palsy, we recommend that the retractors be removed sometimes during the operation.

Summary Background In patients with congenital anomalies of the thumb, the metacarpophalangeal joint often undergoes radial deviation to compensate for a narrow first web space. In this study, we evaluated the relationship between the thumb and index finger in patients with arthrogryposis multiplex congenita (AMC), by measuring the thumb–index angle on radiographs taken while the patient held a styrene foam cone. Methods Investigation was carried out on nine patients (13 cases: four bilateral and five unilateral) with AMC. The average patient age was 4.4 years (range, 2.6–7.2 years). Overhead radiographs were obtained while the patient held a styrene foam cone. The radiographs were used to evaluate the apparent thumb–index web angle, the thumb to index finger metacarpal angle (1–2MCA), and the first metacarpophalangeal angle (1MPA). Results In the five unilateral cases, significant differences were seen on the affected versus unaffected sides in 1–2MCA and 1MPA. All 13 cases were treated surgically, and significant differences were observed between the groups before surgery and 2 years after surgery in the 1–2MCA and 1MPA. In addition, the mean postoperative 1MPA in the bilateral cases was significantly smaller compared with the unilateral cases. Conclusions This radiographic technique enabled us to evaluate the severity of the thumb–index web-space narrowing and the radial instability of the thumb metacarpophalangeal joint in patients with AMC. The bilateral cases tended to show better rehabilitative improvement compared with the unilateral ones, probably because the latter patients could use their unaffected hand.

Background: When examining children with congenital anomalies of the extremities, it is not uncommon for parents to ask about the possibility of similar anomalies occurring in their next child. However, the inheritance of the disease in many congenital anomalies of the extremities has never been elucidated. Methods: In the present study we reviewed cases of their occurrence in siblings that we encountered in our department, and we investigated their characteristics. Results: The results did not reveal any disease specificity, but a tendency for bilateral cases and male cases (cases in brothers) to be more common was observed. Conclusions: In recent years there have been reports of the discovery of causative genes in some congenital anomalies, but because cases of occurrence in siblings with no familial occurrence in the past are seen, there may be a variety of causative genes in many congenital anomalies. In the present study there were many male cases (cases in brothers) and many bilateral cases, and there appears to have been a strong possibility of familial occurrence in such cases, but there were also quite a few exceptions. It is necessary to bear the possibility of heredity in mind in every case and provide the parents with an explanation.

Purpose The purpose of this study was to identify the variety of anatomical abnormalities of extrinsic tendons in type IIIA hypoplastic thumbs. Methods We reviewed 79 thumbs in 67 patients. Opponensplasty, stabilizing of the thumb metacarpophalangeal joint, and widening of the first web space were performed in all patients. At the time of surgery, we made detailed observations of the anatomical abnormalities of the extrinsic tendons of the thumb. Results Fifty thumbs (50 of 79; 63%) had an interconnection between the flexor pollicis longus (FPL) and the extensor pollicis longus (EPL) tendons. Twenty-six thumbs (26 of 79; 33%) had bifurcations (25 [32%] bifurcated from the FPL; 1 [1%] bifurcated from the EPL). There were 25 FPL abnormalities (4 [5%] complete absence; 8 [10%] proximal absence; 2 [3%] distal absence; 11 [14%] tendon hypoplasia) and 7 EPL abnormalities (2 [3%] proximal absence; 5 [6%] tendon hypoplasia). Conclusions Interconnections between the FPL and the EPL tendons and a duplicated FPL were observed frequently. Clinical relevance The present study investigates the detailed anatomy of the type IIIA hypoplastic thumbs. The data might help improve the design of surgical procedures.

Purpose Radial longitudinal deficiency (RLD) presents on a spectrum of severity and associated diagnoses. The literature is limited in describing patient presentation without comparative data between countries. In a study comparing 2 cohorts of patients, 1 in the United States and 1 in Japan, we hypothesized that there would be a similar presentation of forearm deficiency severity, thumb hypoplasia severity, and associated syndromes between the 2 cohorts. Methods Patients with RLD were identified via a comprehensive chart review at 2 pediatric hospital cohorts, 1 in the United States and 1 in Japan, capturing patients presenting over 15 years. We assessed RLD and thumb hypoplasia severity via a modified Bayne and Klug and modified Blauth classifications. The relationship between these 2 diagnoses and the presence of common medical conditions were evaluated and correlated. Results A total of 194 Japanese patients with 290 involved extremities were compared with 107 U.S. patients with 174 involved extremities. The U.S. cohort had a significantly more severe RLD, and a higher rate of bilaterality (63% vs 50%, respectively). A total of 131 Japanese patients (68%) and 41 U.S. patients (38%) had associated medical syndromes/associations, most frequently vertebral abnormalities, anal atresia, cardiac abnormalities, tracheoesophageal fistula and/or esophageal atresia, renal agenesis and dysplasia, and limb defects (VACTERL; 46 Japanese, 14 U.S.), Holt-Oram (44 Japanese, 5 U.S.), and thrombocytopenia absent radius syndrome (0 Japanese, 12 U.S.). Correlation analysis showed that increased RLD severity was associated with increased thumb hypoplasia severity in both groups, with 95% of modified Bayne and Klug III, IV, or V patients having severely affected thumbs (type IIIb, IV, or V). Conclusions The U.S. patients had a more severe RLD and a higher rate of bilaterality. Japanese patients had a higher incidence of associated syndromes and radial polydactyly. Both cohorts showed that increased forearm severity was associated with more severe thumb hypoplasia. Type of study/level of evidence Differential diagnosis/symptom prevalence study III.

Background Congenital limb deficiency is a rare and intractable disease, which impairs both function and appearance of the limbs. To establish adequate medical care, it is necessary to reveal the actual conditions and problems associated with this disease. However, there have been no extensive epidemiological surveys in Japan addressing this disease. This is the first nationwide epidemiological survey of congenital limb deficiency in this country. Methods With the cooperation of epidemiology experts, we performed a two-stage nationwide survey to estimate the number of patients with congenital limb deficiency and reveal basic patient features. We targeted orthopaedic surgery, paediatric, and plastic surgery departments. Hospitals were categorized according to the institution type and the number of hospital beds; hospitals were randomly selected from these categories. We selected 2283 departments from a total 7825 departments throughout Japan. In this study, we defined congenital limb deficiency as partial or total absence of the limbs, proximal to the proximal interphalangeal joint of the fingers/lesser toes or interphalangeal joint of the thumb/great toe. We distributed the first survey querying the number of initial patient visits from January 2014 to December 2015. Targets of the second survey were departments that reported one or more initial patient visits in the first survey. Results In the first survey, 1767 departments responded (response rate: 77.4%). Among them, 161 departments reported one or more initial patient visits. We conducted the second survey among these 161 departments, of which 96 departments responded (response rate: 59.6%). The estimated number of initial visits by patients with congenital limb deficiency was 417 (95% confidence interval: 339–495) per year in 2014 and 2015. The estimated prevalence of congenital limb deficiency in Japan was 4.15 (95% confidence interval: 3.37–4.93) per 10,000 live births. The sex ratio was 1.40. Upper limbs were more affected than lower limbs. Conclusions We revealed the estimated number of initial patient visits per year and birth prevalence of congenital limb deficiency in Japan. Our results will contribute to establishing the disease concept and grades of severity of congenital limb deficiency. Electronic supplementary material The online version of this article (10.1186/s12891-018-2195-3) contains supplementary material, which is available to authorized users.

Background Lipoblastomas are rare benign tumors that arise from embryonic white fat and almost always occur in babies and children. Here, we report a case of a giant popliteal lipoblastoma in a 23-month-old Japanese girl that was successfully treated via complete resection. Case presentation Our patient was a 23-month-old Japanese girl. At 6 months of age, she presented at a nearby hospital with a mass on the popliteal side of her lower right leg. She had no symptoms and was diagnosed as having a benign adipose tumor via magnetic resonance imaging. The mass gradually increased in size, and she was referred to our hospital at 1 year and 11 months of age. A physical examination and radiology revealed a localized mass 13 × 10 × 7 cm in size in the aforementioned area that restricted knee movement and caused proximal tibia deformity. Magnetic resonance imaging showed a giant circumscribed subcutaneous mass with multiple partitions that was hyperintense on T1-weighted and T2-weighted images but not fat-saturated on T2-weighted images. Based on these findings, she was diagnosed as having a lipoblastoma. Because the mass surrounded her popliteal artery and vein and part of the popliteal nerve, surgical resection was considered risky, and we opted to simply observe her. However, owing to rapid growth of the mass and the worsening of symptoms, she underwent complete resection at 2 years and 6 months of age. A histological examination confirmed the diagnosis of a lipoblastoma. She was discharged from our hospital 3 days after surgery with no symptoms. She could walk without pain at the 6-month follow-up, and no local recurrence was observed. Conclusions We successfully treated a giant popliteal lipoblastoma without complications by performing a total resection. Our report provides evidence that lipoblastomas should be considered for surgical resection when they progress or symptoms appear.

The aim of this study is to establish a recapitulation system of human endochondral ossification as a paradigm of developmental engineering. Chondrocytes were isolated from the epiphyseal cartilage of the supernumerary digits of infants with polydactyly. In vivo studies showed that implanted chondrocytes exhibited cartilaginous regeneration over a short period of time and subsequent endochondral ossification with a marrow cavity. Tracing studies revealed that cells of donor origin at the periphery of the cartilage migrated into the center of the cartilage and transformed into osteoblasts, adipocytes, and endothelial cells. Bone marrow was formed through anastomosis with the recipient endothelial system at 13 weeks, and from the migration of recipient hematopoietic cells at 50 weeks. This study provides a human endochondral ossification model system with transdifferentiation of the donor cells at the periphery of the cartilage. J. Cell. Physiol. 230: 1376-1388, 2015. © 2015 Wiley Periodicals, Inc., A Wiley Company.

This paper proposes a method for designing a socket and motorized hand as part of a myoelectric prosthetic hand (EMG prosthetic hand) for partial hand amputees with fingers remaining on the amputation stump. The proposed design method can adapt to individual amputation stumps and remaining finger functions. This method incorporates the affected angular and distance parameters into the hand kinematic model, and designs sockets by shelling the 3D solid model of amputation stumps from the expanded 3D solid model of amputation. The socket and motorized hand were integrated using 3D CAD and the output was provided to a 3D printer. We designed customized EMG prosthetic hands for two subjects with partial hand amputation and remaining fingers, and prototyped them using a 3D printer. The manipulation ability was evaluated using a pick and place experiment, and the results confirmed the validity of the proposed EMG prosthetic hand.

Background Although a simple bone cyst carries the risk of pathological fractures, it rarely causes severe deformity. Here we report a case of severe femoral deformity after multiple pathological fractures due to simple bone cysts, and consider the reason for the progression of malunion despite multiple previous treatments. Finally, we propose a treatment option for malunion correction. Case presentation A 9-year, 7-month-old Japanese girl was referred to our facility with obvious deformity of her right femur, caused by multiple simple bone cyst-related pathological fractures. The deformity included bowing of approximately 90° and an internal rotation of 60° in the middle third of the femoral shaft. To correct this deformity, we excised the lesion, thus shortening the femur, then corrected the alignment and applied an Ilizarov fixator to extend the bone. At present, 3 years after surgery, the deformity has not recurred and our patient is living without any limitations in daily activities or regular exercise. Conclusions When a long bone is in a prolonged state of deformation, the deformity not only progresses as the bone grows, but the soft tissues remain unbalanced and treatment becomes increasingly difficult. To prevent increasing bone deformity and fragility, the deformity should be corrected as quickly as possible using intramedullary nailing or other fixation techniques. We believe that our shortening-distraction method is effective for the treatment of severe deformity with unbalanced soft tissues.

Restoring the hand functionality of partial amputees requires a myoelectric prosthetic hand, which is a robotic hand controlled by myoelectric signals from the skin surface and has the potential to restore human hand functionality. Most myoelectric hands have been developed for forearm amputees, while those for partial-hand amputees are few in number despite the higher numbers of the latter. Partial-hand amputees have limited hand functionality and cannot grasp and manipulate various objects in a manner comparable to individuals with a healthy hand; thus, they require a myoelectric hand. In this study, design issues were identified, and three-dimensional computer-aided design was used to propose an integrated skeleton and housing with a supporting socket. A passive thumb mechanism with motion in the remaining part of the hand was developed where the motion control system is based on the amputee’s muscles. An amputation system is proposed comprising mixed metacarpal and center-part cuts. A prototype was constructed, and its gripping functionality was evaluated. The results demonstrated an enhanced gripping performance compared to the non-use of prosthetics, which attests to the viability and effectiveness of the system.

SHOX haploinsufficiency due to mutations in the coding exons or microdeletions involving the coding exons and/or the enhancer regions accounts for approximately 80% and 2-16% of genetic causes of Leri-Weill dyschondrosteosis and idiopathic short stature, respectively. The most characteristic feature in patients with SHOX deficiency is Madelung deformity, a cluster of anatomical changes in the wrist that can be attributed to premature epiphyseal fusion of the distal radius. Computed tomography of SHOX-deficient patients revealed a thin bone cortex and an enlarged total bone area at the diaphysis of the radius, while histopathological analyses showed a disrupted columnar arrangement of chondrocytes and an expanded hypertrophic layer of the growth plate. Recent studies have suggested that perturbed programmed cell death of hypertrophic chondrocytes may underlie the skeletal changes related to SHOX deficiency. Furthermore, the formation of an aberrant ligament tethering the lunate and radius has been implicated in the development of Madelung deformity. Blood estrogen levels and mutation types have been proposed as phenotypic determinants of SHOX deficiency, although other unknown factors may also affect clinical severity of this entity.

Background In the patient of the cloacal exstrophy, cloaca with local abdominal wall is disrupted and exposed inner surface of the bladder needs early closure. Pelvic osteotomies are required for severe cases whose bladder cannot be closed by the suture of soft tissue only. We developed a technique involving the gradual positioning of bone fragments using a light, Ilizarov external fixator. The usefulness of the technique was assessed. Methods We enrolled 3 patients with cloacal exstrophy and 1 with bladder exstrophy as a gradual transfer group and 6 patients who were treated by other osteotomies as a control group. The patients aged 6.7–8.4 months at the time of surgery were followed up for 4.0–8.6 years. An external fixator with carbon fiber half-rings was placed to internally rotate and anteriorly move the distal bone fragment over 2 weeks. Then, the bladder was closed. Computed tomography (CT) images were used to assess the pelvis form. Wound dehiscence and number of the surgeries after the osteotomies are also compared between the two groups. Results CT analysis of correction of the pelvic deformity achieved more and less decreasing its volumetric capacity in the gradual transfer group. No patients had wound dehiscence after the primary closure with pelvic osteotomy in the gradual transfer group but all had them in the control group. The mean number of the surgeries after the osteotomies were 2.25 in the gradual transfer group whereas 5.5 in the control group. Conclusions Sufficient closure of the abdominal wall and bladder was achieved in all cases in the gradual transfer group. The correction of pelvic bones were more with less decreasing of their pelvic capacities, no patients had wound dehiscence after the closure and there was an effect to decrease the number of the surgeries after the treatment by this method.

Recent reports have suggested that fascicular constriction located proximal to the elbow may be the primary aetiology of spontaneous anterior interosseous nerve palsy (sAINP), and recommended interfascicular neurolysis ranging from the proximal forearm to distal upper arm (wide incision surgery: WIS) for its treatment. On the basis of these reports, it was hypothesised that, if the aetiology of sAINP was fascicular constriction, neurolysis limited proximal to the elbow (minimal incision surgery: MIS) should have similar results to those of WIS. Twenty-five surgically treated sAINP cases were retrospectively evaluated. The mean age of onset was 42.8 years, the mean preoperative period was 5.3 months, and mean follow-up period after operation was 31 months. Eleven patients underwent MIS, while 14 underwent WIS according to the patient's intention (intention-to-treat). Twenty-one patients had fascicular constriction, and no entrapment neuropathy was seen in this series. At the latest follow-up, 82% of the patients had British Medical Research Council grade of ≥4 in flexor-pollicis-longus, while 80% had grade of ≥4 in flexor-digitorum-profundus-of-the-index-finger. There were no significant differences between the results of either group. These result suggested that the aetiology of sAINP may not be external compression, but fascicular constriction, and MIS may be its favourable surgical treatment.

The cell-based therapy for cartilage or bone requires a large number of cells serial passages of chondrocytes are, therefore, needed. However, fates of expanded chondrocytes from extra fingers remains unclarified. The chondrocytes from human epiphyses morphologically changed from small polygonal cells, to bipolar elongated spindle cells and to large polygonal cells with degeneration at early passages. Gene of type II collagen was expressed in the cells only at a primary culture (Passage 0) and Passage 1 (P1) cells. The nodules by implantation of P0 to P8 cells were composed of cartilage and perichondrium. The cartilage consisted of chondrocytes with round nuclei and type II collagen-positive matrix, and the perichondrium consisted of spindle cells with type I collage-positive matrix. The cartilage and perichondrium developed to bone with marrow cavity through enchondral ossification. Chondrogenesis and osteogenesis by epiphyseal chondrocytes depended on replication number in culture. It is noteworthy to take population doubling level in correlation with pharmaceutical efficacy into consideration when we use chondrocytes for cell-based therapies.

Background: Arthrogryposis is the general term given to conditions characterized by multiple joint contractures resulting in substantial disability most frequently involving a child. Early muscle transfer reconstructive surgery reliably restores elbow flexion. In the present study, we aimed to determine which preoperative condition is best able to restore elbow flexion in patients with arthrogryposis.Methods: We retrospectively reviewed five children (seven cases) who underwent elbow flexor reconstruction (Steindler flexorplasty, four cases; latissimus dorsi transfer, two cases; pectoralis major transfer, one case). Upper-extremity function was assessed on the basis of range of elbow extension and flexion and elbow flexor muscle power pre- and postoperatively.Results: The mean postoperative active flexion and extension range of motion was 82.1° (60° to 100°) and -15.0° (-40° to 0°) respectively. Mean postoperative elbow flexor muscle power was graded as a 3.4 (2 to 4) as measured with the Medical Research Council (MRC) scale. The preoperative passive elbow flexion angle was found to correlate with postoperative muscle power (MRC) (r = 0.830, p = 0.042) and postoperative active elbow flexion angle (r = 0.902, p = 0.027). Age at operation was not found to correlate with postoperative muscle power (MRC) (r = -0.063, p = 0.878) or active elbow flexion angle (r = -0.134, p = 0.743).Conclusions: We found a positive correlation between preoperative passive elbow flexion/range of elbow motion and postoperative results including active range of motion and transferred muscle power. Diminished preoperative elbow flexion appeared to correlate with having a poor outcome. The present results suggest that choosing another muscle, such as the gracilis, may be beneficial for muscle transfer in more severe cases of arthrogryposis.

The cell-based therapy for cartilage or bone requires a large number of cells; serial passages of chondrocytes are, therefore, needed. However, fates of expanded chondrocytes from extra fingers remain unclarified. The chondrocytes from human epiphyses morphologically changed from small polygonal cells to bipolar elongated spindle cells and to large polygonal cells with degeneration at early passages. Gene of type II collagen was expressed in the cells only at a primary culture (Passage 0) and Passage 1 (P1) cells. The nodules by implantation of P0 to P8 cells were composed of cartilage and perichondrium. The cartilage consisted of chondrocytes with round nuclei and type II collagen-positive matrix, and the perichondrium consisted of spindle cells with type I collage-positive matrix. The cartilage and perichondrium developed to bone with marrow cavity through enchondral ossification. Chondrogenesis and osteogenesis by epiphyseal chondrocytes depended on replication number in culture. It is noteworthy to take population doubling level in correlation with pharmaceutical efficacy into consideration when we use chondrocytes for cell-based therapies.

Background: A variety of osteotomies has been proposed to correct posttraumatic cubitus varus deformity as well as any associated hyperextension and/or rotational deformities. However, lateral closing-wedge osteotomy and step-cut osteotomy, both of which have been used extensively with satisfactory outcomes, correct only in the coronal plane. To date, no direct comparison has been made between three-dimensional and simple coronal plane osteotomies. Methods: Between 1983 and 2007, we treated eighty-six elbows with a posttraumatic varus deformity. We classified patients who underwent three-dimensional osteotomies as Group I and those who underwent simple coronal plane osteotomies as Group II, and we compared the outcomes between the groups. Clinical evaluation included an assessment of the carrying angle and measurement of the passive range of motion before surgery and at the time of the final follow-up. To evaluate the remodeling capacity of the bone to recover elbow flexion in Group II, we assessed the range of motion before surgery and at the time of the final follow-up in patients who were less than ten years old and those who were more than ten years old. Results: There was no significant difference between the groups with regard to the carrying angle or the elbow range of motion, either before surgery or at the time of the final follow-up. However, Group I had more significant loss of correction (p = 0.018). In Group II, elbow motion reached the physiological range by the time of the final follow-up in patients who were less than ten years old. Conclusions: For osteotomies to correct cubitus varus deformity, correction of internal rotation is not needed. With a three-dimensional osteotomy, it is difficult to maintain correction and to acquire the planned carrying angle because of the small area of bone contact. It is necessary to correct hyperextension in patients older than ten years of age, as after that age bone remodeling is not expected to increase elbow flexion. Level of Evidence: Therapeutic Level III. See Instructions to Authors for a complete description of levels of evidence.

Purpose: We investigated the kinematics of nerve growth factor (NGF)mRNA and brain-derived neurotrophic factor (BDNF)mRNA in a skeletal muscle following spinal cord and peripheral nerve injuries by utilizing the reverse-transcription polymerase chain reaction/high-performance liquid chromatography (RT-PCR/HPLC) method. Methods: We made mice models of spinal cord transection and sciatic nerve transection, plus sham and control groups. After RNA extraction from gastrocnemius muscle of mice, RT-PCR was done. We measured the levels of NGFmRNA and BDNFmRNA in a skeletal muscle following spinal cord and peripheral nerve injuries by utilizing the RT-PCR/HPLC method. All values are analyzed and graphed as means ±SED. Bonferroni test (Post-hoc test) was used when two values were compared. Differences were considered statistically significant when p < 0.05. Results: According to the wet muscle weight, at day 7, a significant difference was found between the peripheral lesion and control group, and at days 14 and 28, significant differences were found between the spinal and peripheral lesion and control group. According to the NGFmRNA, in the peripheral nerve injury group, the levels increased up to day 28 and when compared with the control group, the levels were significantly higher at days 14 and 28. According to the BDNFmRNA, levels at days 2, 7, 14, and 28 were significantly lower in the spinal cord injury group than in the control group. However, in peripheral nerve injury group, when compared with the control group, the levels significantly increased at days 7, 14, and 28. Conclusion: We clarified marked differences in chronological changes in neurotrophin kinetics in a skeletal muscle between spinal cord injury and peripheral nerve injury.

An 8-year-old boy with a history of the inability to extend the middle finger at the proximal interphalangeal and distal interphalangeal joints with the wrist and metacarpophalangeal joints in the neutral position since the age of 5 months had surgery. The flexor digitorum profundus muscle of the middle finger had a short muscle belly and was replaced in part with mature fat tissue. Histologic examination of the resected tissue showed replacement of a part of the muscle with mature fat tissue. It was speculated that some myosatellite cells, which typically differentiate into skeletal muscle cells, may have erroneously differentiated into adipocytes in the first postnatal year, during which complete development of skeletal muscles occurs.

Congruity of the ulnohumeral joint, especially its anterior portion, is an important stabilizer of the elbow joint. We report on 3 patients in whom the insufficiency of the coronoid process, such as nonunion or a flattened trochlear notch, was associated with posterolateral rotatory instability of the elbow. In our opinion, addressing the anterior bony integrity of the ulnohumeral joint, in addition to the ligamentous stabilizer and mechanical axis, is essential to achieve stability in these circumstances. This issue is discussed with a review of the literature.

A 13-year-old girl sustained epiphyseal fractures of the proximal phalanges of the left index, middle, and ring fingers. Though manual reduction of the 3 fingers was possible, it was difficult to maintain the reduction due to severe instability of the middle and ring fingers, and closed reduction with external fixation was performed. At 4 years post-injury, the patient had no impairment of daily activities. The use of external fixation (1) causes no injury to the epiphyseal cartilage, (2) enables accurate reduction and maintenance of reduction, (3) is technically easier than pinning, (4) enables earlier range of motion (ROM) exercises of the proximal interphalangeal (PIP) and distal interphalangeal (DIP) joints of the externally fixated and other fingers, and (5) allows repeated fine adjustments after reduction. External fixation is an option for the treatment of children with highly unstable epiphyseal fractures of the proximal phalanges.

A case of rheumatoid thumb carpometacarpal (CMC) arthritis with advanced osteolysis is reported. Although CMC joint arthrodesis was performed, bone union was not achieved, and pain and instability persisted. Therefore, first and second metacarpal bone fusion was performed with satisfactory results. While CMC joint arthrodesis or arthroplasty is typically performed, first and second metacarpal bone fusion appears to be a useful treatment option when there is marked osteolysis.

Peripheral nerve injury changes the kinetics of neurotrophins. The production of several neurotrophins increases at the site of injury. Although numerous reports have described changes in neurotrophins over time in areas of nerve injury, neurotrophin mRNA is present at very low levels in target tissues, making accurate quantitation difficult. We developed a reverse transcription–polymerase chain reaction/high-performance liquid chromatography (RT-PCR/HPLC) method that enables accurate quantitation of neurotrophin mRNA. We then attempted to quantitate mRNA levels for nerve growth factor (NGF) and brain-derived neurotrophic factor (BDNF) produced by skeletal muscle innervated by the sciatic nerve following transection and reattachment of the nerve in mice. In addition, wet weights of the muscle were measured and changes in weight over time were determined. The results indicated that neurotrophin production in muscle increases as a result of peripheral nerve denervation due to transection, and decreases with nerve regeneration and reinnervation resulting from reattachment.

研究開発活動を発展させていく上において，その意義，内容，基本的な考え方などをわかりやすく説明し，研究内容に親しんでもらう，いわゆる教育啓蒙活動は欠くことのできない重要な課題である．NICTではマイクロラブサット１号機での実験により撮影された地球画像を直接メールで配信する「マイクロオリーブ実験写真のメール配信」実験を実施してきた．本論文では，我々が実施したメール配信実験の概要を紹介する．また，配信状況と実験開始からの登録数の遷移について検討する中で，本実験の参加者がインターネットにおいてどのように広がっていったかについて考察する．さらに，アンケートなどで得られた一般の方々からの生の声から，一般の人々が本実験に対してどのような関心を持ったのかについて検討する．これらの情報には，インターネットを用いた情報提供について何が効果的か，また一般の人々が宇宙開発についてどのような関心を持っているのかといったことについての，重要なヒントが含まれていると思われる．

Millions of physical disabilities, who have lost a hand or both hands, are in need of prosthetic hands not only for decoration but also for the functions to help them with basic daily activities. Although EMG prosthetic hands are being extensively studied to satisfy this need, most of them are too expensive to be economically available, difficult to operate and maintain by a user him/herself, or over heavy for longtime wearing. The aim of this study is therefore to develop a simplified EMG prosthetic hand (sim-EMGPH) to solve these problems. The sim-EMGPH consists of five parts: a lightweight robotic hand with two motors to realize the most frequent hand activities, a highly stretchable cosmetic glove with little load on the motors, an EMG measurement system including sensors with high wearability made of soft conductive materials, a controller implemented by a 32-bit microprocessor which performs EMG signal processing, pattern recognition, and motor control, and a human-friendly tablet interface for the user to operate the sim-EMGPH by him/herself. We manufactured three sim-EMGPHs for three subjects: two with congenital upper limb deficiency and one with upper limb amputation. Free task experiments showed that the subjects could operate the sim-EMGPHs by themselves to perform basic activities of daily living. Limitations revealed and improvement plans are also discussed in this paper.