MR Imaging Detection of CNS Lesions in Tuberous Sclerosis Complex: The Usefulness of T1WI with Chemical Shift Selective Images.

BACKGROUND AND PURPOSE: CNS lesions of tuberous sclerosis complex are diagnosed mainly by T2WI, FLAIR, and sometimes T1WI with magnetization transfer contrast. The usefulness of T1WI with chemical shift selective images was recently reported in focal cortical dysplasia type IIb, which has histopathologic and imaging features similar to those of tuberous sclerosis complex. We investigated the usefulness of the T1WI with chemical shift selective images in detecting CNS lesions of tuberous sclerosis complex. MATERIALS AND METHODS: We retrospectively reviewed 25 consecutive patients with tuberous sclerosis complex (mean age, 11.9 [SD, 8.9] years; 14 males) who underwent MR imaging including T1WI, T1WI with magnetization transfer contrast, T1WI with chemical shift selective, T2WI, and FLAIR images. Two neuroradiologists assessed the number of CNS lesions in each sequence and compared them in 2 steps: among T1WI, T1WI with magnetization transfer contrast and T1WI with chemical shift selective images, and among T2WI, FLAIR, and T1WI with chemical shift selective images. We calculated the contrast ratio of the cortical tubers and of adjacent normal-appearing gray matter and the contrast ratio of radial migration lines and adjacent normal-appearing white matter in each sequence and compared them. RESULTS: T1WI with chemical shift selective images was significantly superior to T1WI with magnetization transfer contrast for the detection of radial migration lines and contrast ratio of radial migration lines. There was no significant difference between T1WI with chemical shift selective images and T1WI with magnetization transfer contrast for the detection of cortical tubers and the contrast ratio of the cortical tubers. Both T2WI and FLAIR were statistically superior to T1WI with chemical shift selective images for the detection of cortical tubers. T1WI with chemical shift selective images was significantly superior to T2WI and FLAIR for the detection of radial migration lines. CONCLUSIONS: The usefulness of T1WI with chemical shift selective images in detecting radial migration lines was demonstrated. Our findings suggest that the combination of T1WI with chemical shift selective images, T2WI, and FLAIR would be useful to evaluate the CNS lesions of patients with tuberous sclerosis complex in daily clinical practice.

Femoral morphology asymmetry in hip dysplasia makes radiological leg length measurement inaccurate.

AIMS: The aim of this study was to examine whether hips with unilateral osteoarthritis (OA) secondary to developmental dysplasia of the hip (DDH) have significant asymmetry in femoral length, and to determine potential related factors. PATIENTS AND METHODS: We enrolled 90 patients (82 female, eight male) with DDH showing unilateral OA changes, and 43 healthy volunteers (26 female, 17 male) as controls. The mean age was 61.8 years (39 to 93) for the DDH groups, and 71.2 years (57 to 84) for the control group. Using a CT-based coordinate measurement system, we evaluated the following vertical distances: top of the greater trochanter to the knee centre (femoral length GT), most medial prominence of the lesser trochanter to the knee centre (femoral length LT), and top of the greater trochanter to the medial prominence of the lesser trochanter (intertrochanteric distance), along with assessments of femoral neck anteversion and neck shaft angle. RESULTS: The percentages of hips with an absolute difference of > 5 mm in femoral GT and LT lengths were significantly larger in the DDH group (24% for both) compared with those of the control group (2% and 7%, respectively). The femoral length GT of the affected femur was significantly shorter in Crowe I and longer in Crowe IV than that of the unaffected side. The affected-to-unaffected difference of the intertrochanteric distance showed positive correlation with that of the femoral length GT in Crowe I and Crowe II/III, and negative correlation with that of the femoral length LT in the Crowe I and Crowe IV groups. CONCLUSION: Hips with unilateral end-stage OA secondary to DDH show significant asymmetry in femoral length between both the greater and lesser trochanter and the knee compared with controls. The intertrochanteric distance was a morphological factor related to femoral-length asymmetry. When undertaking total hip arthroplasty (THA) in the presence of DDH, long leg radiographs or CT measurements should be used to assess true leg-length discrepancy. Cite this article: Bone Joint J 2019;101-B:297-302.

Arthroscopic anatomical reconstruction of the lateral ankle ligaments.

UNLABELLED: Chronic ankle instability secondary to lateral ligament insufficiency is common after sports injury. Many surgical techniques have been described for the treatment of the lateral ankle ligament complex. They can be classified into repair or reconstruction, and non-anatomical or anatomical. A few authors have recently published innovative techniques for arthroscopic ankle ligament management. This paper describes the arthroscopic techniques enabling anatomical lateral ligament reconstruction using gracilis autograft or allograft for chronic ankle instability. This technique and the steps have been developed by the Ankle Instability Group to make this a reproducible procedure. The purpose of this presentation is to document the technique in the future with a view to a clinical study investigating the results of such surgery in a cohort of suitable patients with chronic ankle instability. LEVEL OF EVIDENCE: V.

Three-dimensional morphology and bony range of movement in hip joints in patients with hip dysplasia.

To confirm whether developmental dysplasia of the hip has a risk of hip impingement, we analysed maximum ranges of movement to the point of bony impingement, and impingement location using three-dimensional (3D) surface models of the pelvis and femur in combination with 3D morphology of the hip joint using computer-assisted methods. Results of computed tomography were examined for 52 hip joints with DDH and 73 normal healthy hip joints. DDH shows larger maximum extension (p = 0.001) and internal rotation at 90° flexion (p < 0.001). Similar maximum flexion (p = 0.835) and external rotation (p = 0.713) were observed between groups, while high rates of extra-articular impingement were noticed in these directions in DDH (p < 0.001). Smaller cranial acetabular anteversion (p = 0.048), centre-edge angles (p < 0.001), a circumferentially shallower acetabulum, larger femoral neck anteversion (p < 0.001), and larger alpha angle were identified in DDH. Risk of anterior impingement in retroverted DDH hips is similar to that in retroverted normal hips in excessive adduction but minimal in less adduction. These findings might be borne in mind when considering the possibility of extra-articular posterior impingement in DDH being a source of pain, particularly for patients with a highly anteverted femoral neck.

Consensus in chronic ankle instability: aetiology, assessment, surgical indications and place for arthroscopy.

Ankle sprains are the most common injuries sustained during sports activities. Most ankle sprains recover fully with non-operative treatment but 20-30% develop chronic ankle instability. Predicting which patients who sustain an ankle sprain will develop instability is difficult. This paper summarises a consensus on identifying which patients may require surgery, the optimal surgical intervention along with treatment of concomitant pathology given the evidence available today. It also discusses the role of arthroscopic treatment and the anatomical basis for individual procedures.

Differences in the locations and modes of labral tearing between dysplastic hips and those with femoroacetabular impingement.

We investigated differences in the location and mode of labral tears between dysplastic hips and hips with femoroacetabular impingement (FAI). We also investigated the relationship between labral tear and adjacent cartilage damage. We retrospectively studied 72 symptomatic hips (in 68 patients: 19 men and 49 women) with radiological evidence of dysplasia or FAI on high-resolution CT arthrography. The incidence and location of labral tears and modes of tear associated with the base of the labrum (Mode 1) or body of the labrum (Mode 2) were compared among FAI, mildly dysplastic and severely dysplastic hips. The locations predominantly involved with labral tears were different in FAI and mild dysplastic hips (anterior and anterosuperior zones) and in severely dysplastic hips (anterosuperior and superior zones) around the acetabulum. Significant differences were observed in the prevalence of Mode 1 versus Mode 2 tears in FAI hips (72% (n = 13) vs 28% (n = 5)) and severe dysplastic hips (25% (n = 2) vs 75% (n = 6)). The frequency of cartilage damage adjacent to Mode 1 tears was significantly higher (42% (n = 14)) than that adjacent to Mode 2 tears (14% (n = 3)). Hip pathology is significantly related to the locations and modes of labral tears. Mode 1 tears may be a risk factor for the development of adjacent acetabular cartilage damage.

Grafting with hydroxyapatite granules for defects of acetabular bone at revision total hip replacement: a minimum ten-year follow-up.

The long-term results of grafting with hydroxyapatite granules for acetabular deficiency in revision total hip replacement are not well known. We have evaluated the results of revision using a modular cup with hydroxyapatite grafting for Paprosky type 2 and 3 acetabular defects at a minimum of ten years' follow-up. We retrospectively reviewed 49 acetabular revisions at a mean of 135 months (120 to 178). There was one type 2B, ten 2C, 28 3A and ten 3B hips. With loosening as the endpoint, the survival rate was 74.2% (95% confidence interval 58.3 to 90.1). Radiologically, four of the type 3A hips (14%) and six of the type 3B hips (60%) showed aseptic loosening with collapse of the hydroxyapatite layer, whereas no loosening occurred in type 2 hips. There was consolidation of the hydroxyapatite layer in 33 hips (66%). Loosening was detected in nine of 29 hips (31%) without cement and in one of 20 hips (5%) with cement (p = 0.03, Fisher's exact probability test). The linear wear and annual wear rate did not correlate with loosening. These results suggest that the long-term results of hydroxyapatite grafting with cement for type 2 and 3A hips are encouraging.

A modular femoral neck and head system works well in cementless total hip replacement for patients with developmental dysplasia of the hip.

We compared a modular neck system with a non-modular system in a cementless anatomical total hip replacement (THR). Each group consisted of 74 hips with developmental hip dysplasia. Both groups had the same cementless acetabular component and the same articulation, which consisted of a conventional polyethylene liner and a 28 mm alumina head. The mean follow-up was 14.5 years (13 to 15), at which point there were significant differences in the mean total Harris hip score (modular/non-modular: 98.6 (64 to 100)/93.8 (68 to 100)), the mean range of abduction (32 degrees (15 degrees to 40 degrees )/28 (0 degrees to 40 degrees )), use of a 10 degrees elevated liner (31%/100%), the incidence of osteolysis (27%/79.7%) and the incidence of equal leg lengths (> or = 6 mm, 92%/61%). There was no disassociation or fracture of the modular neck. The modular system reduces the need for an elevated liner, thereby reducing the incidence of osteolysis. It gives a better range of movement and allows the surgeon to make an accurate adjustment of leg length.

Intractable bronchopleural fistula caused by radiofrequency ablation: endoscopic bronchial occlusion with silicone embolic material.

A 58-year-old man with primary lung cancer underwent lung radiofrequency (RF) ablation. Pneumothorax developed 12 days after lung RF ablation. Despite chest drainage for 1 month, air leakage continued through a bronchopleural fistula. Bronchial occlusion was performed with a silicone embolus, causing cessation of the air leakage.

Association of epidermal growth factor receptor mutations in lung cancer with chemosensitivity to gefitinib in isolated cancer cells from Japanese patients.

Somatic mutations in the tyrosine kinase domain of the epidermal growth factor receptor (EGFR) gene are reported to be associated with clinical responsiveness of lung cancer to gefitinib, an EGFR tyrosine kinase inhibitor. To elucidate the association between somatic mutations and the pharmacological actions of gefitinib, the chemosensitivity of isolated cancer cells from the lungs of Japanese patients to gefitinib was examined by the collagen gel-droplet embedded culture drug sensitivity test in vitro. In 30 specimens isolated from non-small-cell lung cancer patients, mutations were observed in eight tumour specimens (27%) and chemosensitivity to gefitinib was observed in seven specimens (23%). However, somatic mutations were not predominantly associated with chemosensitivity to gefitinib in vitro. Both mutation and chemosensitivity frequencies in this study were higher than those reported in studies from the United States, indicating a possible ethnic difference. Moreover, both frequencies were much higher in females than in males. Since a gender difference in chemosensitivity to gefitinib was observed in isolated cancer cells in vitro, this suggests that gefitinib works in part through the suppression of EGFR signalling, but that other factors, including sex-related factors, may participate in gefitinib action.

Disorders of acetabular labrum and articular cartilage in hip dysplasia: evaluation using isotropic high-resolutional CT arthrography with sequential radial reformation.

OBJECTIVE: Acetabular labral tear may predispose to adjacent articular cartilage disorder and hip osteoarthritis in patients with hip dysplasia. We evaluated the diagnostic ability of isotropic computed tomography (CT) arthrography with radial reformation technique for detection of acetabular labral and articular cartilage disorders, and evaluate those interactions in hip dysplasia. METHODS: Forty-one hips in 29 patients with hip dysplasia received CT arthrography with isotropic spatial resolution of 0.5mm. After processing of multiplanar radial reformation over the whole acetabular circumference, frequencies of labral tear and acetabular cartilage disorder were evaluated at six divided zones of the weight-bearing areas. Of the 41 hips, 20 hips underwent arthroscopic examinations, and sensitivity, specificity and accuracy for detecting labral tear and acetabular cartilage disorder by CT arthrography were calculated using the arthroscopic findings as the standard of reference. RESULTS: The sensitivity, specificity and accuracy of CT arthrography were 97%/87%/92% for labral tear and 88%/82%/85% for acetabular cartilage disorder, respectively, using arthroscopic findings as the reference. The CT arthrography showed significantly higher frequency of labral and acetabular cartilage disorders at the anterior zones. Those zones with labral tear had significantly higher frequency of adjacent cartilage disorder than zones without labral tear. CONCLUSIONS: Isotropic CT arthrography with radial reformation technique allowed simultaneous, accurate assessment of labral and cartilage disorders in the whole acetabular circumference. Our findings indicated that labral tear is closely associated with adjacent cartilage disorder in hip dysplasia.

[Clinicopathologic study of thymic epithelial tumors].

OBJECTIVE: We analyzed clinicopathologic characters and long-term results of 11 thymic epithelial tumors. METHODS: Five cases of thymic carcinoma and 6 cases of thymoma treated in our hospital from September 1991 to June 2002 were retrospectively analyzed. RESULTS: The histological subtypes of thymic carcinoma were basaloid carcinoma in 2 cases, epidermoid non-keratinizing carcinoma in 1 case, undifferentiated carcinoma in 1 case and sarcomatoid carcinoma in 1 case. Four cases underwent chemotherapy and radiotherapy. Three cases underwent midsternal thoracotomy, 1 had total resection and 2 had exploratory thoracotomy due to tumor invasion of the right upper lobe and cardiac sac. Two cases of basaloid carcinoma had been alive more than 10 years since the operation. The histological subtypes of thymoma were 1, 2, 1, 1 and 1 cases with type A, AB, B 1, B 2 and B 3. All cases underwent midsternal thoracotomy, 4 cases had thymothymectomy and 2 cases had extended thymothymectomy. Five cases have been alive since the operation. Strong immunoreactivity for bcl-2 and p 53 expression of epidermoid non-keratinizing carcinoma and undifferentiated carcinoma were seen. ki-67 labeling index of epidermoid non-keratinizing carcinoma and undifferentiated carcinoma and type B 3 thymoma were higher than those of the other carcinomas and thymomas.

A randomized phase III trial of adjuvant chemotherapy with UFT for completely resected pathological stage I non-small-cell lung cancer: the West Japan Study Group for Lung Cancer Surgery (WJSG)--the 4th study.

PURPOSE: To examine the efficacy of UFT, an oral 5-fluorouracil derivative agent, as post-operative adjuvant therapy for pathologic (p-) stage I non-small-cell lung cancer (NSCLC), because a previous randomized study had suggested it was efficacious for early-stage NSCLC patients. PATIENTS AND METHODS: Patients with completely resected p-stage I, adenocarcinoma or squamous cell carcinoma were eligible. A total of 332 patients were randomized to the surgery-alone group (control group) and the treatment group (UFT 400 mg/m(2) for 1 year after surgery, UFT group) after stratification by the histologic types. RESULTS: For all patients, the 5- and 8-year survival rates for the UFT group were 82.2% and 73.0%, and those for the control group were 75.9% and 61.2%, respectively; no statistically significant improvement of survival was achieved by UFT administration (P=0.105). For Ad patients, the 5- and 8-year survival rates of the UFT group (n=120) were 85.2% and 79.5%, respectively, which seemed better than those of the control group (n=121) (79.2% and 64.0%, respectively; P=0.081). For squamous cell carcinoma patients, there was also no difference in survival between the control group (n=48) and the UFT group (n=43) (P=0.762). For all pT1 patients, the 5- and 8-year survival rates of the UFT group were 83.6% and 82.1%, respectively, significantly better than those of the control group (77.9% and 57.6%, respectively, P=0.036); UFT was not significantly effective for pT2 patients. For pT1 adenocarcinoma patients, UFT administration markedly improved the survival (P=0.011). CONCLUSION: Post-operative UFT administration did not significantly improve post-operative survival of p-stage I NSCLC patients. Subset analyses suggested that UFT might be effective in pT1N0M0 adenocarcinoma patients.

Intracellular ferritin accumulation in neural and extraneural tissue characterizes a neurodegenerative disease associated with a mutation in the ferritin light polypeptide gene.

Abnormal accumulation of ferritin was found to be associated with an autosomal dominant slowly progressing neurodegenerative disease clinically characterized by tremor, cerebellar ataxia, parkinsonism and pyramidal signs, behavioral disturbances, and cognitive decline. These symptoms may appear sequentially over a period of 4 decades. Pathologically, intranuclear and intracytoplasmic bodies were found in glia and subsets of neurons in the central nervous system as well as in extraneural tissue. Biochemical analyses of these bodies isolated from the striatum and cerebellar cortex revealed that ferritin light polypeptide (FTL) and ferritin heavy polypeptide (FTH1) were the main constituents. Molecular genetic studies revealed a 2-bp insertion mutation in exon 4 of the FTL gene. The resulting mutant polypeptide is predicted to have a carboxy terminus that is altered in amino-acid sequence and length. In tissue sections, the bodies were immunolabeled by anti-ferritin and anti-ubiquitin antibodies and were stained by Perls' method for ferric iron. Synthetic peptides homologous to the altered and wild-type carboxy termini were used to raise polyclonal antibodies. These novel antibodies as well as an antibody recognizing FTH1 immunolabeled the bodies. This study of this disorder has provided additional knowledge and insights in the growing area of ferritin-related neurodegeneration.

[A rational approach of limited resection for small peripheral lung adenocarcinoma with curative intent; analyses of multiple primary adenocarcinomas].

Hundred and forty-one small peripheral adenocarcinomas 2 cm or less in diameter were retrospectively studied to determine the rationale of limited resection with curative intent. We used a conventional computed tomography (CT) which used 2.5 mm thick sections to examine only the main tumor during from March 1985 to May 1999 and a spiral CT which produced 2.5 mm thick sections of the entire lung field during from June 1999 to July 2003. The incidence of small peripheral adenocarcinoma significantly increased from 12.6% to 29.1%, suggesting an increase in the rate of detection with spiral CTs. During the spiral CT era, the percentage of females, pathological stage I a tumors, predominant ground-glass opacity (GGO) tumors and limited resection were significantly higher. The incidence of multiple adenocarcinomas 2 cm or less in diameter significantly increased 2.6% to 14.1%. It increases to 21.9% in small adenocarcinomas and 63.6% in predominant GGO type, when minute GGO lesion which have been followed in 5 patients by a watch and wait policy would be bronchioloalveolar carcinoma (BAC). In conclusion, a paradigm shift of the treatment for small peripheral adenocarcinoma should be warrant, because localized BAC as noninvasive cancer is not rare and often found as multiple BACs.

Repair of neglected Achilles tendon rupture using gastrocnemius fascial flaps.

BACKGROUND: Neglected Achilles tendon rupture is a rare disorder. In this article, we discuss the results of 10 patients with neglected Achilles tendon ruptures who were treated surgically. METHODS: Between 1980 and 1997, we treated 10 patients (6 men and 4 women) using gastrocnemius fascial flaps according to the method described by Lindholm. The mean age of the patients at the time of the operation was 51 years (range 38-57 years). They were followed-up for 26-192 months. RESULTS: There were significant differences between the American Orthopaedic Foot and Ankle Society (AOFAS) scale score before the operation (72.6+/-5.3) and the score at the most recent follow-up (98.1+/-2.5) (p<0.0001). On Cybex isokinetic strength testing, the peak torque deficiencies in plantar flexion ranged from 8% to 68% at the low setting and from 19% to 33% at the high setting preoperatively, and ranged from -9% to 17% at the low setting and from -13% to 23% at the high setting postoperatively. There were no re-ruptures. CONCLUSION: Our data indicate that the reconstructive technique using gastrocnemius fascial flaps can result in an excellent clinical and functional outcome.

Diagnosis of a tear of the tibiofibular syndesmosis. The role of arthroscopy of the ankle.

In 52 patients we compared the accuracy of standard anteroposterior (AP) radiography, mortise radiography and MRI with arthroscopy of the ankle for the diagnosis of a tear of the tibiofibular syndesmosis. In comparison with arthroscopy, the sensitivity, specificity and accuracy were 44.1%, 100% and 63.5% for standard AP radiography and 58.3%, 100% and 71.2% for mortise radiography. For MRI they were 100%, 93.1% and 96.2% for a tear of the anterior inferior tibiofibular ligament and 100%, 100% and 100% for a tear of the posterior inferior tibiofibular ligament. Standard AP and mortise radiography did not always provide a correct diagnosis. MRI was useful although there were two-false positive cases. We suggest that arthroscopy of the ankle is indispensable for the accurate diagnosis of a tear of the tibiofibular syndesmosis.

Hereditary prion protein amyloidoses.

Prion protein (PrP) amyloid accumulation is the pathologic hallmark of some inherited prion diseases such as Gerstmann-Sträussler-Scheinker disease (GSS) and PrP cerebral amyloid angiopathy (PrP-CAA). In GSS, parenchymal amyloidosis may coexist with spongiform degeneration or neurofibrillary tangles, whereas in PrP-CAA, vascular amyloid coexists with neurofibrillary tangles. In GSS, N-truncated and C-truncated proteinase K-resistant PrP isoforms are present in the brain.

Astroglial swelling in the neuronal depolarization ensemble.

The coupling between neuronal depolarization and astroglial swelling was examined. First, previous in vitro data for the swelling of cultured neurons (N18; rat neuroblastoma) and astroglia (C6; rat astroglyoma) upon exposure to a hypoosmotic solution were reappraised. Neurons swelled rapidly, forming blisters, and easily burst. whereas astroglia resisted swelling and slowly assumed a large full-moon shape. The time constant of swelling was 35.2 +/- 7.8 s for N18 and 594.8 +/- 554.0 s for C6. The glial plasmic membrane was found to be much stronger than the neuronal one, presumably due to a well-developed cytoskeleton. To overcome such neuronal membranous weakness, strong astroglial processes need to cover the neurons including the cell body and synapses, as demonstrated electron-microscopically. Next, in situ astroglial swelling was investigated in rats. During K(+)-induced cortical spreading depression, increases or decreases of a wave-ring spread of light (550 nm) transmission through a 1 mm-thick cerebral cortical layer was observed. The moving local optical density decrease in the cortex was attributable to local vascular bed compression induced by astroglial swelling, since concomitant occurrence of colocated dynamic capillary flow stall was confirmed by a hemodilution technique. Astroglial swelling may occur in an ensemble acting during neuronal depolarization, suggesting that neurons and astroglia behave like a unit complex.