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Orthopaedic Proceedings
Vol. 105-B, Issue SUPP_7 | Pages 34 - 34
4 Apr 2023
Kaneko Y Minehara H Nakamura M Sekiguchi M Matsushita T Konno S
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Recent researches indicate that both M1 and M2 macrophages play vital roles in tissue repair and foreign body reaction processes. In this study, we investigated the dynamics of M1 macrophages in the induced membrane using a mouse femur critical-sized bone defect model.

The Masquelet method (M) and control (C) groups were established using C57BL/6J male mice (n=24). A 3mm-bone defect was created in the right femoral diaphysis followed by a Kirschner wire fixation, and a cement spacer was inserted into the defect in group M. In group C, the bone defect was left uninserted. Tissues around the defect were harvested at 1, 2, 4, and 6 weeks after surgery (n=3 in each group at each time point). Following Hematoxylin and eosin (HE) staining, immunohistochemical staining (IHC) was used to evaluate the CD68 expression as a marker of M1 macrophage. Iron staining was performed additionally to distinguish them from hemosiderin-phagocytosed macrophages.

In group M, HE staining revealed a hematoma-like structure, and CD68-positive cells were observed between the spacer and fibroblast layer at 1 week. The number of CD68-positive cells decreased at 2 weeks, while they were observed around the new bone at 4 and 6 weeks. In group C, fibroblast infiltration and fewer CD68-positive cells were observed in the bone defect without hematoma-like structure until 2 weeks, and no CD68-positive cells were observed at 4 and 6 weeks. Iron staining showed hemosiderin deposition in the surrounding area of the new bone in both groups at 4 and 6 weeks. The location of hemosiderin deposition was different from that of macrophage aggregation.

This study suggests that M1 macrophage aggregation is involved in the formation of induced membranes and osteogenesis and may be facilitated by the presence of spacers.


The Bone & Joint Journal
Vol. 101-B, Issue 11 | Pages 1459 - 1463
1 Nov 2019
Enishi T Yagi H Higuchi T Takeuchi M Sato R Yoshioka S Nakamura M Nakano S

Aims

Rotational acetabular osteotomy (RAO) is an effective joint-preserving surgical treatment for acetabular dysplasia. The purpose of this study was to investigate changes in muscle strength, gait speed, and clinical outcome in the operated hip after RAO over a one-year period using a standard protocol for rehabilitation.

Patients and Methods

A total of 57 patients underwent RAO for acetabular dysplasia. Changes in muscle strength of the operated hip, 10 m gait speed, Japanese Orthopaedic Association (JOA) hip score, and factors correlated with hip muscle strength after RAO were retrospectively analyzed.


Orthopaedic Proceedings
Vol. 99-B, Issue SUPP_22 | Pages 97 - 97
1 Dec 2017
Ikeda S Uchiyama K Kishino S Nakamura M Yoshida K Minegishi Y Sugo K Fukushima K Takahira N Kitasato H Takaso M
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Aim

The preparation of antibiotic-containing polymethyl methacrylate (PMMA), as spacers generates a high polymerization heat, which may affect their antibiotic activity; it is desirable to use bone cement with a low polymerization heat. Calcium phosphate cement (CPC) does not generate heat on polymerization, and comparative elution testings are reported that vancomycin (VCM)-containing CPC (VCM-CPC) exceeded the antibiotic elution volume and period of PMMA (VCM-PMMA). Although CPC alone is a weak of mechanical property spacer, the double-layered, PMMA-covered CPC spacer has been created and clinically used in our hospital. In this study, we prepared the double-layered spacers: CPC covered with PMMA and we evaluated its elution concentration, antimicrobial activity and antibacterial capability.

Method

We prepared spherical, double-layered, PMMA-coated (CPC+PMMA; 24 g CPC coated with 16 g PMMA and 2 g VCM) and PMMA alone (40 g PMMA with 2 g VCM) spacers (5 each). In order to facilitate VCM elution from the central CPC, we drilled multiple holes into the CPC from the spacer surface. Each spacer was immersed in phosphate buffer (1.5 mL/g of the spacer), and the solvent was changed daily. VCM concentrations were measured on days 1, 3, 7, 14, 28, 56, and 84. Antimicrobial activity against MRSA and MSSA was evaluated by the broth microdilution method. After measuring all the concentration, the spacers were compressed at 5 mm/min and the maximum compressive load up to destruction was measured.


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_8 | Pages 90 - 90
1 May 2016
Kawashima H Nakano S Yoshioka S Toki S Kashima M Nakamura M Chikawa T Kanematsu Y Sairyo K
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Flexion contracture sometimes occurs after primary total knee arthroplasty (TKA). In most cases, flexion contracture after TKA gradually improves over time. However, some severe cases require manipulation or revision surgery.

We searched our clinical database for patients who underwent primary TKA at our institution between 2008 and 2015. By reviewing patient records, we identified three patients (one man and two women) with a severe flexion contracture 30° after primary TKA. Although all three patients gained more than 120° in flexion intraoperatively, they developed flexion contracture after discharge from our institution. We performed manipulation under anaesthesia (MUA) for all three cases several months later. The two female patients had improved range of motion (ROM) right after the manipulation. However, one of them regained flexion contracture 1 year after the MUA.

We report the details of the male patient, who had the worst flexion contracture (−60°). An 80-year-old man had right knee osteoarthritis. His history indicated only hypertension. The right knee ROM before the TKA was −20° extension and 135° flexion. His radiographs showed advanced-stage osteoarthritis. We performed cemented TKA (posterior stabiliser design). Three weeks after the operation, his right knee pain improved. The right knee ROM was −10° extension and 100° flexion just before discharge. However, he returned to our institution because of right knee pain and flexion contracture 31 months after the surgery. The flexion contracture gradually worsened without any trauma. When he returned, the right knee ROM was −60° extension and 135° flexion. Manipulation under general anaesthesia was not effective. Therefore, we performed revision TKA immediately. We excised the scar tissue of the posterior knee joint. Then, we shortened the distal femoral end by 1 cm and reduced the size of the femoral component. After the operation, the right knee ROM was improved to −10° flexion and 130° extension.

The reported prevalence of stiffness after TKA was from 1.3% to 13%. Although the deleterious effects of persistent flexion contractures > 15° is well understood, whether they resolve with time or need surgical intervention is controversial. MUA is generally the initial option for patients with flexion contractures, with the possibility of some improvement. If severe flexion contracture remains after manipulation, revision TKA, which may be considered as a useful treatment option, should be considered.


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_8 | Pages 55 - 55
1 May 2016
Iwamoto T Matsumura N Ochi K Nakamura M Matsumoto M Sato K
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Objective

Computed tomography based three-dimensional surgical preoperative planning (3D-planning) has been expanded to achieve more precise placement of knee and hip arthroplasties. However, few reports have addressed the utility of 3D-planning for the total elbow arthroplasty (TEA). The purpose of this study was to assess the reliability and precision of 3D planning in unlinked TEA.

Methods

Between April 2012 and April 2014, 17 joints in 17 patients (male 4, female 13) were included in this study. Sixteen patients were rheumatoid arthritis and one was osteoarthritis and the average age at the time of the procedure was 61 years (range 28–88). Unlinked K-NOW total elbow system (Teijin-Nakashima Medical. Co. Ltd.) was used in all cases and 3D planning was performed by Zed View (Lexi.Co.). After the appropriate size and position of the prosthesis were decided on the 3D images [Figure 1], the position of the bone tunnel made for the insertion of humeral and ulnar stem was recorded on axial, sagittal, and coronal plane (4 point measurements for humerus, and 6 points for ulna, See Figure 2). After the elbow was exposed via a posterior approach, bone resection and reaming was performed according to the 3D planning. The surgeon took an appropriate adjustment to align the prosthesis properly during the surgery. The final position of the stem insertion was recorded immediately prior to set the prostheses. We analyzed the accuracy of stem size prediction, the correlation between preoperative and final measurements, and postoperative complications.


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_9 | Pages 75 - 75
1 May 2016
Nakano S Yoshioka S Toki S Kashima M Nakamura M Chikawa T Kanematsu Y Sairyo K
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Purpose

Proximal femoral osteotomy is an attractive joint preservation procedure for osteonecrosis of the femoral head. The purpose of this study was to investigate the cause of failure of proximal femoral osteotomy in patients with osteonecrosis of the femoral head.

Patients and Methods

Between 2008 and 2014, proximal femoral osteotomy was performed by one surgeon in 13 symptomatic hips. Ten trans-trochanteric rotational osteotomies (anterior: 7, posterior: 3) and 3 intertrochanteric curved varus osteotomy were performed. Of the patients, 9 were male and 1 was female, with a mean age at surgery of 36.9 years (range, 25–55 years). The mean postoperative follow-up period was 38 months (range, 12–72 months). Three patients (4 hips) had steroid-induced osteonecrosis, and 7 (9 hips) had alcohol-associated osteonecrosis. At 6 postoperative weeks, partial weight bearing was permitted with the assistance of 2 crutches. At more than 6 postoperative months, full weight bearing was permitted. Patients who had the potential to achieve acetabular coverage of more than one-third of the intact articular surface on preoperative hip radiography, computed tomography, and magnetic resonance imaging were considered suitable for this operation. A clinical evaluation using the Japanese Orthopaedic Association (JOA) scoring system and a radiologic evaluation were performed. Clinical failure was defined as conversion to total hip arthroplasty (THA) or progression to head collapse and osteoarthritis. The 13 hips were divided into two groups, namely the failure and success groups.


Orthopaedic Proceedings
Vol. 98-B, Issue SUPP_10 | Pages 145 - 145
1 May 2016
Yoshioka S Nakano S Toki S Kashima M Nakamura M Chikawa T Kanematsu Y Sairyo K
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Introduction

Pelvic osteotomy such as Chiari osteotomy and rotational acetabular osteotomy (RAO) have been used successfully in patients with developmental dysplasia of the hip (DDH). However, some patients are forced to undergo total hip arthroplasty (THA) because of the progression of osteoarthritis. THA after pelvic osteotomy is thought to be more difficult because of altered anatomy of the pelvis. We compared six THAs done in dysplastic hips after previous pelvic osteotomy between 2008 and 2015 with a well-matched control group of 20 primary procedures done during the same period.

Materials and methods

Six THAs for DDH after previous Pelvic osteotomy (three Chiari osteotomies and three RAOs) were compared with 20 THAs for DDH without previous surgery. The patients were matched for age, sex, and BMI. Minimum follow-up for both groups of patients was one year (range, 12–79 months and 12–77 months, respectively). The average interval from pelvic osteotomy to total hip arthroplasty was 19.8 years (range 12–26 years). Clinical and Radiological evaluations were performed.


The Bone & Joint Journal
Vol. 97-B, Issue 11 | Pages 1582 - 1587
1 Nov 2015
Suzuki T Seki A Nakamura T Ikegami H Takayama S Nakamura M Matsumoto M Sato K

This retrospective study was designed to evaluate the outcomes of re-dislocation of the radial head after corrective osteotomy for chronic dislocation. A total of 12 children with a mean age of 11 years (5 to 16), with further dislocation of the radial head after corrective osteotomy of the forearm, were followed for a mean of five years (2 to 10). Re-operations were performed for radial head re-dislocation in six children, while the other six did not undergo re-operation (‘non-re-operation group’). The active range of movement (ROM) of their elbows was evaluated before and after the first operation, and at the most recent follow-up.

In the re-operation group, there were significant decreases in extension, pronation, and supination when comparing the ROM following the corrective osteotomy and following re-operation (p < 0.05).

The children who had not undergone re-operation achieved a better ROM than those who had undergone re-operation.

There was a significant difference in mean pronation (76° vs 0°) between the non- re-operation and the re-operation group (p = 0.002), and a trend towards increases in mean flexion (133° vs 111°), extension (0° vs 23°), and supination (62° vs 29°). We did not find a clear benefit for re-operation in children with a re-dislocation following corrective osteotomy for chronic dislocation of the radial head.

Cite this article: Bone Joint J 2015;97-B:1582–7.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXV | Pages 252 - 252
1 Jun 2012
Utsunomiya R Nakano S Nakamura M Chikawa T Shimakawa T Minato A
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Permanent patellar subluxation is treated with surgeries such as proximal realignment and distal realignment, however, it is difficult to cure this condition by using any methods. We performed mobile-bearing total knee arthroplasty (TKA) in a case of severe knee osteoarthritis complicated with permanent patellar subluxation since childhood, and obtained good results without performing any additional procedures.

The patient was an 82-year-old woman with severe pain in the left knee. During the initial examination, the range of motion of the left knee joint was -10°of extension to 140°of flexion, and the Japanese Orthopaedic Association (JOA) score for knee osteoarthritis was 40 points (maximum score: 100). Preoperative radiographs showed a varus deformity in the left lower extremity with a femorotibial angle (FTA) of 188°, the axial view showed luxation of the patella.

We performed TKA using a mobile-bearing implant. Intraoperative findings revealed that the central articular surface of the distal femur had disappeared, and that the patellar articular surface was concave and dome-shaped. The lateral patellofemoral ligament was released; this procedure was identical to that performed in conventional TKA.

Postoperative radiographs showed good alignment, with an FTA of 173°. In the axial view, the patella was located in a reduced position at any angle of knee joint flexion.

The postoperative range of motion of the left knee joint was 0°of extension to 130°of flexion. The patient was able to walk without the support of a T-shaped cane.

There are many surgical treatments for permanent patellar subluxation. The appropriate treatment is selected according to the type and seriousness of the dislocation and the age of the patient.

From the findings of the present case, we believe that in a case of knee osteoarthritis complicated with permanent patellar subluxation, surgery performed using a mobile-bearing implant would eliminate the necessity of performing additional proximal realignment and distal realignment.


Orthopaedic Proceedings
Vol. 94-B, Issue SUPP_XXV | Pages 239 - 239
1 Jun 2012
Tamaki Y Nakano S Utsunomiya R Nakamura M Chikawa T Shimakawa T Minato A
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Background

The bearing surface is one of the important factors that affect the longevity of total hip replacement (THR). The ceramic on ceramic bearing decreases the rate of dislocation event and the amount of wear debris. We encountered cases of incomplete seating of the liner with the TriAD acetabular system.

Patients and Methods

We examined 25 hips in 24 patients who had undergone total hip replacement by using the TriAD shell with a metal-backed alumina liner. We used the Hardinge approach for performing surgery in all patients. Incomplete seating was judged on the basis of plain anteroposterior and/or oblique radiographs obtained immediately and 3 months after the operation.


Orthopaedic Proceedings
Vol. 93-B, Issue SUPP_IV | Pages 422 - 422
1 Nov 2011
Nakano S Kosaka H Nakamura M Chikawa T Taoka Y Shimakawa T Minato A Ikata T Sogame M
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Objective: The number of hip prostheses replacement surgeries particularly in elder people with osteoporosis, has been increasing every year; given this scenario, treatment of postoperative periprosthetic femoral fracture has become a critical problem. Osteosynthesis is generally selected as the procedure of choice for the surgical treatment of fractures, provided the stem prostheses do not show loosening. Stable fixation of periprosthetic femoral fracture is difficult in the elderly because they have osteoporotic bone and most of the intramedullary space is occupied by the metal stem implanted in the proximal femoral shaft. With a view to solving this problem, we developed a new surgical treatment for postoperative periprosthetic femoral fracture; this procedure use a trimming intramedullary nail, which we have termed “docking nail.” [Materials] The subjects were 3 patients (81, 75 and 76 years old) who had suffered a femoral shaft fracture around the femoral prosthesis after total hip replacement; in all 3 patients, there was no apparent sign of loosening of the stem prosthesis. The implanted stem was cemented in one patient and uncemented in the other two.

Method: Using information on the size and shape of the stem prosthesis as well as information from the pre-operative radiographs, we cut the docking nail till the proper length was achieved and trimmed it to suit the cutting site in order to ensure that it was compatible with tip of the stem. We then performed osteosynthesis using instruments of an ordinary supracondylar type intramedullary nail. In cases where it was difficult to reposition or where it was necessary to remove excessive cement and bone from around the tip of stem and graft a free bone in the bony defect, we exposed the fracture site as minimally as possible. The major difference between our procedure and the conventional procedure is that in our procedure, the docking nail is connected to the tip of the implanted stem to ensure proper alignment. Postoperative immobilization was not used in any of the patients except for the 81-year-old patient, for whom partial weight-bearing was allowed at 4 weeks, and full weight bearing at 12 weeks. The mean follow up period was 22 months (range, 6–48 months).

Result: Within 3 months, bony union with good alignment was achieved in all 3 patients without malunion or infection. The clinical and radiographic examinations conducted during the follow-up period showed good results.

Conclusion: The advantages of this method are that it is less invasive and simple compared to the conventional methods. Its only disadvantage is that it requires considerable, preoperative planning and minor trimming of the nail. Although this series is small, we think that this new treatment can be recommended and will be beneficial for treating periprosthetic femoral fractures without a loose stem. However, these preliminary findings need to be confirmed by further investigations.


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_I | Pages 76 - 76
1 Jan 2003
Minamikawa Y Nakamura M Iida H Nakatani K Nieda T
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Objective

Destruction and deformity in both PIP and MP joints are not uncommon and cause sever disabilities. Arthroolasty of the MP joint combined with arthrodesis of the PIP joint are the usual choice for this condition. Some motion in ulnar digits provides great benefits for rheumatoid patients. We report implant arthroplasty of the PIP joint and also simultaneous replacement of PIP and MP joint in same finger.

Material & Methods

Twenty-five joints in 15 patients underwent PIP arthroplasty either with silicone or surface replacement implant. Twelve PIP joints in 6 patients used Silicone implant (6 Swanson and 6 Avanta) alone and mean follow up was 42(6–84) months. New cementless surface implant (Self Locking Finger Joint, SLFJ) were developed and clinically used for about 3 years. Because of the stem design, SLFJ are able to use both PIP and MP joint simultaneously or combined with silicone implant. Four PIP joints in 3 patients used SLFJ alone and 6 patients underwent both PIP and MP joint with implant simultaneously. Combination of SLFJ PIP and silicone MP was 4 finger in 2 hand, SLFJ PIP and SLFJ MP was 3 finger in 2 hand, silicone PIP and SLFJ MP was 3 finger in 2 patients and silicone PIP and silicone MP was 1 finger. Mean follow up for SLFJ in either PIP or MP was 18(4–37) months.

Results

Average arc of the PIP with silicone implant alone was 38(10–50 ° and SLFJ alone was 55(45–60) °. One PIP SLFJ dislocates immediate after surgery and was converted silicone later on. Of 7 SLFJ with combined PIP and MP arthroplasties, 2 PIP lost motion completely, one PIP move only 15°, 4 PIP move 75 °in average. Four silicone PIP combined with MP arthroplasties move 45°in average.

One PIP SLFJ had breakage in stem legs, which believed to occur during interaction of stem insertion from both side of the basal phalanx, and was seen at immediate post op X-ray. There was one instability in index replaced with SLFJ for sever Swan neck deformity and no infection. Patient satisfaction for simultaneous replacement in PIP and MP joint were excellent except one whose age was 72.

Discussion

Stability of the PIP joint in index finger is important for pinch and PIP motion of ring and little fingers are required for grip motion. Although arthrodesis of the PIP joint were performed more often and functional improvement usually obtained compared to pre-operative condition, ulnar 2 digits better to preserve some motion in the PIP joint as long as there is a possibility, and especially for the young patients. The results of the simultaneous replacement in PIP and MP joint seems discourage, however, when considering the severity of the deformities of this series, there is a good chance in the future. We will improve implant design and surgical technique as well as post-operative therapy, and continue to challenge the simultaneous replacement of PIP and MP joint.


Orthopaedic Proceedings
Vol. 85-B, Issue SUPP_I | Pages 74 - 74
1 Jan 2003
Minamikawa Y Nakamura M Iida K Nakatani K
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Objective

Although silicone finger implant has several disadvantages including loss of motion, implant breakage and osteolysis, Swanson or other silicone prosthesis are still most widely accepted in the world. Osseointegrated implant showed great improvement as for as stem fixation, however, still used silicone joint with high incidence of fracture. Cement-less surface type finger joint prosthesis has been developed and clinical trial has carried out. Ninety three joint replacement in 30 hands with mean follow up about 2 years are reported.

Materials & methods

The endoprosthesis consists of 3 different parts; two titanium joint anchor (fixture) in proximal and distal, titanium joint head in proximal, and HMWDP joint socket in distal. The joint anchor has tapered self tapping screw in joint side surface and has two flat long legs attached toward canal side which spread and compressed to intramedural canal firmly by turning a locking screw within the joint anchor. Each joint component is fixed to the anchor within a square hole. The MP joint component has anatomical head but semiconstrained toward volar dislocation by deep dorsal roof shape of the joint socket. Each socket has 2 different thickness. Because of cement less joint fixture mechanism and design, this endoprosthesis system is able to preserve collateral ligament and adjust the tension of collateral ligament. Twenty-six patients (24 women and 3 men) with average age of 59. 8years(range 35–80 years) were operated. Four patients were operated in both hands. Total of 93 joints were operated in 30 hands, average 3. 1 joints in one hand (4 in 16, 3 in 5, 2 in 5 and 1 in 4).

Results

Follow up averaged 23. 9 (range 14–37) months. The average arc of motion was 47(20–85) ° and extension lag was significantly improved in most cases. Ulnar deviation recurred moderately about 25%, and 2 cases complained pain with flexion contracture. There were 3 dislocations and 2 loosening with fracture of the leg.

Discussion

Surface type endoprosthesis preserving surrounding soft tissue is the optimal design in large implant arthroplasties. This new finger joints prosthesis provided cement-less joint fixture mechanism and surface type design and preliminary results were satisfactory. Although further follow up and more cases are required, this finger joint prosthesis has proven to be a very good alternative to silicone implant replacement especially for the young patients.


Orthopaedic Proceedings
Vol. 84-B, Issue SUPP_III | Pages 256 - 256
1 Nov 2002
Fukuta S Kuge A Nakamura M
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Objective: To investigate the clinical outcome of debridement arthroplasty using medial approach for the osteoarthritis of the elbows.

Methods: 40 elbows in 36 patients with osteoarthritis of the elbow were reviewed retrospectively. All of the patients were men and the mean age at the time of surgery was 60.0 years. The mean duration of follow-up was 35 months. All elbows were managed operatively with debridement arthroplasty. This procedure consisted of removal of free bodies, resection of inflammatory synovia, and resection of osteophytes. Medial approach with medial epicondylectomy was applied for all cases. In 10 elbows, posterior approach was used additionally for the debridement of posterior compartment.

Results: 23 elbows (57.5 %) had an excellent result, 11 elbows (27.5 %) had a good result, and six (15 %) had a fair result. 10 elbows (25%) had complete relief of pain while the other 30 elbows (75 %) had partial relief. Remaining pain was mostly located at the lateral aspect in patients with severe radiohumral arthritis. Flexion was improved from 106.5 degrees to 121.5 degrees.

Conclusion: This procedure is effective for patients with osteoarthritis of the elbow which is localized in ulnohumeral joint. Total elbow arthroplasty must be considered for the severe radiohumeral arthritis. Careful selection of patients is essential for successful outcome.


The Journal of Bone & Joint Surgery British Volume
Vol. 80-B, Issue 1 | Pages 19 - 24
1 Jan 1998
Matsumoto M Fujimura Y Suzuki N Nishi Y Nakamura M Yabe Y Shiga H

We studied degenerative changes in the cervical intervertebral discs of 497 asymptomatic subjects by MRI and evaluated disc degeneration by loss of signal intensity, posterior and anterior disc protrusion, narrowing of the disc space and foraminal stenosis. In each subject, five disc levels from C2–C3 to C6–C7 were evaluated.

The frequency of all degenerative findings increased linearly with age. Disc degeneration was the most common observation, being present in 17% of discs of men and 12% of those of women in their twenties, and 86% and 89% of discs of both men and women over 60 years of age. We found significant differences in frequency between genders for posterior disc protrusion and foraminal stenosis. The former, with demonstrable compression of the spinal cord, was observed in 7.6% of subjects, mostly over 50 years of age.

Our results should be taken into account when interpreting the MRI findings in patients with symptomatic disorders of the cervical spine.


The Journal of Bone & Joint Surgery British Volume
Vol. 78-B, Issue 1 | Pages 145 - 147
1 Jan 1996
Nakamura M Masumi S Nakamura M

We describe three sisters in a family who presented with a clinical form of osteomesopycnosis which is a rare, benign osteosclerotic bone disorder limited to the axial skeleton. It must be distinguished from other osteosclerotic conditions which carry a worse prognosis. This is the first report of the condition in a Japanese family, and we believe it to be the first to be identified in Asian races.