Characteristics of terminal hemimelia: What is the difference between terminal hemimelia and classic fibular hemimelia?

Purpose: Fibular hemimelia has denoted a spectrum of postaxial longitudinal deficiency with fibular aplasia/hypoplasia; the term "terminal hemimelia" is reserved for patients with postaxial longitudinal deficiency having a normal fibula. We aimed to delineate the characteristics of terminal hemimelia. Methods: In total, 30 patients with postaxial longitudinal deficiency who had a normal or hypoplastic fibula and visited our institution between 1992 and 2022 were reviewed. Patients were divided into terminal hemimelia and classic fibular hemimelia groups, and their demographic characteristics and clinical and radiographic findings were compared. Results: Femoral shortening, knee valgus, and tibial spine hypoplasia were less common in terminal hemimelia (n = 13) than in classic fibular hemimelia (n = 17) (p = 0.03, p < 0.001, and p = 0.003, respectively). None of the patients in the terminal hemimelia group exhibited knee instability, whereas 12% of patients with classic fibular hemimelia did. Ball-and-socket ankle and absence of lateral rays were commonly observed in both groups. However, tarsal coalition was observed less frequently in terminal hemimelia (p = 0.004). All terminal hemimelia patients exhibited a painless plantigrade foot without ankle instability. Despite limb-length discrepancy at maturity averaging 40.4 mm for terminal hemimelia and 67.0 mm for classic fibular hemimelia (p < 0.001), patients with terminal hemimelia, except for one, exhibited > 20 mm of limb-length discrepancy. However, 46% of them underwent limb-length equalization procedures, mostly single-stage tibial lengthening, at a mean age of 11.2 years. Conclusion: Terminal hemimelia may present with a milder phenotype than classic fibular hemimelia. It mainly overlaps with the symptoms of fibular hemimelia below the ankle joint and manifests as limb-length discrepancy. However, a considerable number of patients with terminal hemimelia required limb-length equalization procedures, for example single-stage tibial lengthening. Level of evidence: level IV.

Improving Prenatal Diagnosis Precision for Congenital Clubfoot by Using Three-Dimensional Ultrasonography.

Prenatal diagnosis of clubfoot traditionally relied on two-dimensional ultrasonography. To enhance diagnosis and predict postnatal outcomes, we examined the parameters that differentiate pathological clubfoot using three-dimensional ultrasonography. In our retrospective study, we examined the findings of prenatal ultrasound and the postnatal outcomes of pregnancies with suspected congenital clubfoot between 2018 and 2021. Based on the three-dimensional perspective, we measured the angles of varus, equinus, calcaneopedal block, and forefoot adduction and compared the sonographic variables between the postnatal treated and non-treated groups. We evaluated 31 pregnancies (47 feet) with suspected clubfoot using three-dimensional ultrasonography. After delivery, a total of 37 feet (78.7%) underwent treatment involving serial casting only or additional Achilles tenotomy. The treated group showed significantly greater hindfoot varus deviation (60.5° vs. 46.6°, p = 0.026) and calcaneopedal block deviation (65.6° vs. 26.6°, p < 0.05) compared to the non-treated group. The calcaneopedal block had an area under the curve of 0.98 with a diagnostic threshold of 46.2 degrees (sensitivity of 97%, specificity of 90%, positive predictive value of 97%, and negative predictive value of 90%). During prenatal evaluation of clubfoot using three-dimensional ultrasonography, the calcaneopedal block deviation has the potential to predict postnatal treatment.

Incidence and Clinical Characteristics of Slipped Capital Femoral Epiphysis in Patients with Endocrinopathy: A Population-Based Cohort Study.

BACKGROUND: Endocrinopathy is a risk factor for slipped capital femoral epiphysis (SCFE). We aimed to determine (1) the incidence of endocrinopathy-associated SCFE compared with that of non-endocrinopathy-associated SCFE, (2) whether the incidence of SCFE increases with the number of deficient hormones, and (3) the clinical characteristics of endocrinopathy-associated SCFE. METHODS: We conducted a population-based cohort study using a nationwide database in South Korea. All new diagnoses of endocrinopathy or SCFE between 2002 and 2019 in children born between 2002 and 2005 were identified. The incidence of SCFE was calculated for each type of endocrinopathy. The trend of the incidence of SCFE relative to the number of deficient hormones was analyzed. The male:female ratio was compared between endocrinopathy-associated SCFE and non-endocrinopathy-associated SCFE. For endocrinopathy-associated SCFE, the time between the diagnoses of SCFE and endocrinopathy was evaluated. RESULTS: The incidence of SCFE was higher in children with endocrinopathy than in those without endocrinopathy (37.1/100,000 versus 9.0/100,000 children) (relative risk, 4.1 [95% confidence interval, 2.8-6.1]). Among various endocrinopathies, growth hormone deficiency showed the highest incidence of SCFE (583.8/100,000 children). The Cochran-Armitage test showed a linear trend, with an increased number of deficient hormones being associated with a higher incidence of SCFE (p < 0.001). Male sex was dominant in the non-endocrinopathy-associated SCFE group (73%; 117 of 161), whereas female sex was dominant in the endocrinopathy-associated SCFE group (53%; 16 of 30) (p = 0.009). Twenty-two of the 30 cases of endocrinopathy-associated SCFE were diagnosed after the diagnosis of endocrinopathy, with a median time of 3.6 years between the diagnoses. Six (27%) of these 22 children developed SCFE >5 years after the diagnosis of endocrinopathy. CONCLUSIONS: The incidence of SCFE was approximately 4 times higher in children with endocrinopathy than in those without endocrinopathy. The risk of SCFE increased with an increased number of deficient hormones. Long-term monitoring of SCFE occurrence in children with endocrinopathies is strongly recommended. LEVEL OF EVIDENCE: Diagnostic Level III . See Instructions for Authors for a complete description of levels of evidence.

The role of 99mTc-DPD bone SPECT/CT in the management of growth disturbance of the long bones in pediatric patients: a retrospective observational study.

BACKGROUNDS: Determining the precise localization of diseased physes is crucial for guiding the treatment of growth disturbances. Conventional radiography, computed tomography (CT), and magnetic resonance imaging only provide information on physeal anatomy. Planar bone scintigraphy and bone single-photon emission computed tomography (SPECT) resolutions are suboptimal for clinically managing growth disturbances. Bone SPECT/CT, which provides high-resolution functional information, can be a useful tool for evaluating growth disturbances. The purposes of this study were to identify the conditions in which bone SPECT/CT outperforms planar scintigraphy or SPECT for evaluating the location and activity of diseased physes and to assess surgical outcomes using bone SPECT/CT findings in pediatric patients experiencing long bone growth disturbances. METHODS: Fifty-nine patients who underwent bone SPECT/CT between January 2018 and January 2021 to evaluate physeal activity using technetium-99 m-labeled 2,3-dicarboxypropane-1,1-diphosphonate (99mTc-DPD) were included. The proportions of patients for whom certain modalities provided sufficient data for selecting treatment plans for growth disturbances were compared based on the site of the diseased physis, growth disturbance cause, and shape of deformity (i.e., SPECT/CT vs. planar scintigraphy and SPECT/CT vs. SPECT). For assessing surgical outcomes, progression of post-surgical deformity was investigated by measuring the angles reflecting the degree of deformity, iliac crest height difference, or ulnar variance on radiographs. RESULTS: Bone SPECT/CT was sufficient for selecting a treatment plan, but planar scintigraphy or SPECT alone was insufficient in every 10 patients with diseased physes inside the femoral head (p = 0.002) and in every six with physes that were severely deformed or whose locations were unclear on conventional radiography (p = 0.03). In the proximal or distal tibia, where the tibial and fibular physes often overlapped on planar scintigraphy due to leg rotation, bone SPECT/CT was sufficient in 33/34 patients (97%), but planar scintigraphy and SPECT were sufficient in 10/34 (29%) (p < 0.001) and 24/34 (71%) patients, respectively (p = 0.004). No progression or deformity recurrence occurred. CONCLUSIONS: Bone SPECT/CT may be indicated in proximal femoral growth disturbance, when the physis is unclear on conventional radiography or severely deformed, the leg exhibits rotational deformity, or the patient is noncompliant.

When to Combine Acetabular Osteotomy in Patients With Proximal Femoral Deformity Causing Residual Hip Dysplasia/Subluxation After Reduction of Developmental Dysplasia of the Hip.

BACKGROUND: After the successful reduction of developmental dysplasia of the hip, residual hip dysplasia may persist and lead to early osteoarthritis. Femoral and/or acetabular osteotomy has been used to address this problem. The purpose of this study is to determine the indication of femoral versus combined femoral-acetabular osteotomy in the management of residual hip dysplasia. METHODS: Fifty-five patients with unilateral dislocated-type dysplasia of the hip, who had residual hip dysplasia after reduction, underwent femoral osteotomy with or without acetabular osteotomy before 8 years of age, and were followed for more than 2 years and over 8 years of age, were the subjects of this retrospective study. Twenty-eight patients underwent femoral osteotomy only at a median age of 34 months (group F), and 27 underwent combined femoral-Dega osteotomy at a median age of 49 months (group C). Seventeen patients in group F and 4 in group C had an additional osteotomy due to persistent hip dysplasia. Acetabular index (AI), lateral center-edge angle, and center-head distance difference were measured on serial radiographs. The z-value of AI (Z AI ) was calculated. At the latest follow-up, patients in group F with Severin I/II who did not have an additional osteotomy were considered satisfactory, and patients with Severin III/IV or those who had an additional osteotomy were considered unsatisfactory. Preoperative variables were tested for the difference between satisfactory and unsatisfactory cases. Receiver operating characteristic analysis was performed to delineate a cutoff value of a significant parameter dividing the outcome. RESULTS: AI and Z AI before index osteotomy were significant parameters predicting a satisfactory outcome in group F. Receiver operating characteristic analysis returned a cutoff value of Z AI 2.6 (Area Under the Curve=0.86, P =0.001). Eight of 12 cases (66.7%) with Z AI <2.6 in group F achieved a satisfactory outcome, whereas only 2 of 14 cases with Z AI ≥2.6 in group F did ( P =0.02). CONCLUSION: Z AI 2.6 may serve as a threshold to combine acetabular osteotomy with femoral osteotomy in the management of residual hip dysplasia before 8 years of age. LEVEL OF EVIDENCE: Therapeutic III.

The increased analgesic efficacy of cold therapy after an unsuccessful analgesic experience is associated with inferior parietal lobule activation.

Prior experiences of successful and failed treatments are known to influence the efficacy of a newly applied treatment. However, whether that carry-over effect applies to non-pharmacological treatments is unknown. This study investigated how a failed treatment history with placebo analgesic cream affected the therapeutic outcomes of cold-pack treatment. The neural correlates underlying those effects were also explored using functional magnetic resonance imaging. The effect of the placebo analgesic cream was induced using placebo conditioning with small (44.5 °C to 43.7 °C, negative experience) and large (44.5 °C to 40.0 °C, positive experience) thermal stimuli changes. After the placebo conditioning, brain responses and self-reported evaluations of the effect of subsequent treatment with a cold-pack were contrasted between the two groups. The negative experience group reported less pain and lower anxiety scores in the cold-pack condition than the positive experience group and exhibited significantly greater activation in the right inferior parietal lobule (IPL), which is known to be involved in pain relief. These findings suggest that an unsatisfying experience with an initial pain-relief treatment could increase the expectations for the complementary treatment outcome and improve the analgesic effect of the subsequent treatment. The IPL could be associated with this expectation-induced pain relief process.

Effect of Leg Length Discrepancy on Lateral Center-edge Angle Measurement.

BACKGROUND: In patients with leg length discrepancy (LLD) and consequent pelvic obliquity, either the longitudinal axis of the pelvis or a line perpendicular to the ground may be used as the longitudinal reference line for measuring the lateral center-edge angle (LCEA). We aimed to (1) systematically inspect which longitudinal reference line has been used for measuring the LCEA in previous studies; (2) evaluate the frequency of change in the radiographical classification of acetabular overcoverage or undercoverage per the longitudinal reference line; and (3) validate the trigonometric method, predicting the change in the LCEA according to the LLD. METHODS: Studies investigating the LCEA published between January 1976 and July 2019 in the MEDLINE database were categorized according to the longitudinal reference line used. Further, in a retrospective analysis of 238 patients surgically treated for LLD, the LCEA was first measured on standing pelvic radiographs using the longitudinal axis of the pelvis (pLCEA) and measured again using a line perpendicular to the ground (gLCEA). Femoral head coverage was categorized as undercoverage, normal, or overcoverage based on the pLCEA and gLCEA. The theoretically calculated difference between the pLCEA and gLCEA (dLCEA) as determined using a trigonometric method was compared with the dLCEA measured on radiographs. RESULTS: Of 229 previous studies, 188 did not specify the longitudinal reference line. The number of patients who were diagnosed with acetabular overcoverage using the pLCEA and gLCEA was one and fourteen, respectively (P<0.001). The number of patients who were diagnosed with acetabular undercoverage using the pLCEA and gLCEA was one and zero, respectively (P=1.000). There was no difference (P=0.433) between the theoretically calculated (9±5 degrees) and measured (9±5 degrees) dLCEAs. CONCLUSIONS: The definition of the longitudinal reference line should be clarified when measuring the LCEA. The trigonometric method can accurately predict the change in the LCEA according to LLD in concentric hips without proximal femoral and pelvic deformities. LEVEL OF EVIDENCE: Level IV-diagnostic study.

Intra-articular acetabular osteochondroma in patients with multiple hereditary exostoses.

We report three additional cases of intra-articular acetabular osteochondroma in multiple hereditary exostoses patients in order to raise the awareness of this rare location, to prompt early diagnosis, and to present various treatment options according to the patient's condition. A 2.5-year-old boy presenting with an out-toeing gait had a large acetabular osteochondroma causing lateral displacement of the femoral head and acetabular dysplasia. Mass excision through hip subluxation via anterior approach and concomitant Dega osteotomy resulted in a congruent, well-developed, and well-covered hip joint at 11-year follow-up. A 10-year-old boy showing a pedunculated osteochondroma arising from the triradiate cartilage was successfully treated by arthroscopic excision. Normal development of the hip joint was observed at skeletal maturity. A 6-year-old boy presented with a painless limp. Acetabular osteochondroma could be confirmed by computed tomography scan, which was excised through hip subluxation by anterior approach. Persistent coxa valga and femoral head uncovering were addressed by femoral varization osteotomy at 9 years of age. A high index of suspicion is required to detect a lesion at this rare location. Various procedures may be considered according to the pathoanatomy. Acetabular dysplasia, coxa valga, and femoroacetabular impingement by femur neck mass, if associated, should be addressed in due time.

Prospective study of epigenetic alterations responsible for isolated hemihyperplasia/hemihypoplasia and their association with leg length discrepancy.

BACKGROUND: Hemihyperplasia and hemihypoplasia result in leg length discrepancy (LLD) by causing skeletal asymmetry. Beckwith-Wiedemann syndrome (BWS) and Silver-Russell syndrome (SRS) are opposite growth-affecting disorders caused by opposite epigenetic alterations at the same chromosomal locus, 11p15, to induce hemihyperplasia and hemihypoplasia, respectively. Because of their somatic mosaicism, BWS and SRS show a wide spectrum of clinical phenotypes. We evaluated the underlying epigenetic alterations and potential epigenotype-phenotype correlations, focusing on LLD, in a group of individuals with isolated hemihyperplasia/hemihypoplasia. RESULTS: We prospectively collected paired blood-tissue samples from 30 patients with isolated hemihyperplasia/hemihypoplasia who underwent surgery for LLD. Methylation-specific multiplex-ligation-dependent probe amplification assay (MS-MLPA) and bisulfite pyrosequencing for differentially methylated regions 1 and 2 (DMR1 and DMR2) on chromosome 11p15 were performed using the patient samples. Samples from patients showing no abnormalities in MS-MLPA or bisulfite pyrosequencing were analyzed by single nucleotide polymorphism (SNP) microarray and CDKN1C Sanger sequencing. We introduced a metric named as the methylation difference, defined as the difference in DNA methylation levels between DMR1 and DMR2. The correlation between the methylation difference and the predicted LLD at skeletal maturity, calculated using a multiplier method, was evaluated. Predicted LLD was standardized for stature. Ten patients (33%) showed epigenetic alterations in MS-MLPA and bisulfite pyrosequencing. Of these, six and four patients had epigenetic alterations related to BWS and SRS, respectively. The clinical diagnosis of hemihyperplasia/hemihypoplasia was not compatible with the epigenetic alterations in four of these ten patients. No patients showed abnormalities in SNP array or their CDKN1C sequences. The standardized predicted LLD was moderately correlated with the methylation difference using fat tissue (r = 0.53; p = 0.002) and skin tissue (r = 0.50; p = 0.005) in all patients. CONCLUSIONS: Isolated hemihyperplasia and hemihypoplasia can occur as a spectrum of BWS and SRS. Although the accurate differentiation between isolated hemihyperplasia and isolated hemihypoplasia is important in tumor surveillance planning, it is often difficult to clinically differentiate these two diseases without epigenetic tests. Epigenetic tests may play a role in the prediction of LLD, which would aid in treatment planning.

Efficacy of Differential Diagnosis of Thyroid Nodules by Shear Wave Elastography-the Stiffness Map.

BACKGROUND: Fine needle aspiration is the gold standard for differential diagnosis of thyroid nodules; however, the malignancy rate for indeterminate cytology is 20% to 50%. OBJECTIVE: We evaluated the efficacy of shear wave elastography added to ultrasonography for differential diagnosis of thyroid nodules. METHODS: We retrospectively reviewed the medical records of 258 consecutive patients. Thyroid nodules were divided into 4 categories according to maximum elasticity (EMax) and nodule depth/width (D/W) ratio: Category 1 (EMax ≥ 42.6 kPa; D/W < 0.9); Category 2 (EMax < 42.6 kPa; D/W < 0.9); Category 3 (EMax ≥ 42.6 kPa; D/W ≥ 0.9); and Category 4 (EMax < 42.6 kPa; D/W ≥ 0.9). The EMax cutoff value was set using receiver operating characteristic (ROC) curve analysis to predict nodular hyperplasia (NH) vs follicular neoplasm (FN). Cutoff value for nodule D/W ratio was set using ROC curve analysis for malignancy. RESULTS: NH was the most prevalent pathology group in Category 1, FN in Category 2, and papillary thyroid carcinoma in Category 3. Category 3 demonstrated the highest rate of malignancy (81.8%) and had 55.4% sensitivity and 90% specificity for predicting malignancy. When assessing the benign pathology of NH in follicular patterned lesion, Category 1 demonstrated the highest NH prevalence of 88.9% (34/37) and had 73.9% sensitivity and 85.0% specificity. CONCLUSION: The performance for malignancy was highest in Category 3 and predictive ability for benign pathology of NH in follicular lesion was highest in Category 1. The information of EMax and nodule D/W ratio was useful to predict the pathology of thyroid nodules.

Pseudo-Protrusio Acetabular Deformity in Osteogenesis Imperfecta Patients.

BACKGROUND: Osteogenesis imperfecta (OI) can develop a protrusio acetabuli deformity. However, the authors observed a pseudo-protrusio-type acetabular deformity (PPAD) on 3-dimensional computed tomography (3D-CT). Hence, we systematically reviewed 3D-CT and pelvis radiographs of OI patients and report the incidence and patterns of acetabular deformity in OI patients and the associated radiographic signs. METHODS: The study included 590 hips of 295 OI patients, who were older than 5 years, and did not have a pelvic fracture. The incidence of a deformed acetabulum (center-edge angle >40 degrees) and its correlation with disease severity were investigated. In 40 hips for which 3D-CT was available, 3-dimensional morphology of the acetabular deformity was analyzed to delineate PPAD. On plain radiographs, PPAD-related signs were determined, focusing on the contour of ilioischial line, iliopectineal line, acetabular line, and their relationship. These radiographic signs were also evaluated in the remaining hips with deformed acetabula that did not have 3D-CT. RESULTS: One hundred twenty-three hips of 590 hips (21%) showed deformed acetabula. The incidence of deformed acetabula was significantly associated with disease severity (P<0.001). Three-dimensional analysis showed that 10 hips had protrusio acetabuli, whereas 17 had PPAD, which showed that the hemipelvis was crumpled, the acetabular roof was rotated upwardly and medially, and the hip center migrated superiorly, uncovering the anterior femoral head. Among the PPAD-related signs, superomedial bulging of the iliopectineal line was the most predictive radiographic sign (73% sensitivity and 100% specificity). This sign was also observed in almost one third of deformed acetabula of those investigated only with plain radiographs. CONCLUSIONS: This study showed that acetabular deformity is common in OI patients and is associated with disease severity. A substantial number of hips showed PPAD, which may not cause femoroacetabular impingement but result in anterior uncovering of the hip joint. Superomedial bulging of the iliopectineal line suggests this pattern of acetabular deformity. LEVEL OF EVIDENCE: Lever IV-prognostic studies.

Late recurrence of papillary thyroid cancer from needle tract implantation after core needle biopsy: A case report.

BACKGROUND: Papillary thyroid cancer (PTC) has good prognosis so that the local recurrence or distant metastasis can occur later on the lifetime follow up. In this study, we report recurrence of PTC in subcutaneous area combined with lymph node metastasis. A suspicion of needle tract implantation after core needle biopsy was found. CASE SUMMARY: A 66-year-old female patients who underwent right thyroid lobectomy for PTC complained of palpable nodule on anterior neck area. The location of the palpable nodule was not associated with her postoperative scar. After excision of the skin tumor, it was diagnosed as recurrence of PTC. Furthermore, results of subsequent imaging showed lymph node metastasis on her right cervical area. According to the previous medical records, the patient received core needle biopsy through the neck of the patient midline and hematoma was noted after the procedure. The time interval from the first diagnosis to local recurrence or metastasis to the skin and lymph nodes was ten years. As treatment, the patient underwent lymph node dissection in the right and completion thyroidectomy for radioisotope treatment. CONCLUSION: Needle tract implantation can occur after core needle biopsy. Further studies are needed to compare core-needle biopsy and fine-needle aspiration.

Effective and Rapid Microbial Identification in Pediatric Osteoarticular Infections Using Blood Culture Bottles.

BACKGROUND: The detection and identification of pathogenic microorganisms are essential for the treatment of osteoarticular infection. However, obtaining a sufficient amount of specimen from pediatric patients is often difficult. Herein, we aimed to demonstrate the effectiveness of the blood culture bottle (BCB) system in pediatric osteoarticular infections. We hypothesized that our BCB culture method is superior to the conventional swab and tissue culture methods in terms of required specimen size, incubation time, and microbial identification rate. METHODS: We analyzed the prospectively collected data of pediatric patients who underwent surgical treatment for osteoarticular infections between August 2016 and October 2019. Four needles were dipped in the infected fluid or tissue during the surgical procedure as soon as the infected area was exposed and were used to inoculate 2 aerobic pediatric BCBs and 2 anaerobic general BCBs. We also collected 2 conventional swab samples and 2 tissue samples from the identical area. The microbial identification rate and the time required for identification were compared between BCB, swab, and tissue cultures. RESULTS: Forty patients constituted the study group; 13 patients had osteomyelitis, 17 patients had septic arthritis, and 10 patients had both. Of these 40 patients, the microbial identification rate was higher with BCB cultures (27 [68%]) than with swab cultures (18 [45%]; p = 0.004) or tissue cultures (15 [38%]; p < 0.001). Nine samples (9 patients [23%]) were only positive in the BCB culture. Positive microbial growth was not detected with conventional culture methods when microorganisms did not grow on the BCB culture. Compared with swab culture (4.3 ± 1.1 days; p < 0.001) or tissue culture (4.4 ± 1.1 days; p < 0.001), the BCB culture reduced the time required for microbial identification (3.5 ± 0.9 days). CONCLUSIONS: In pediatric osteoarticular infections, the BCB culture system improved the microbial identification rate, reduced the time to identification, and permitted a smaller-volume specimen, compared with traditional culture systems. LEVEL OF EVIDENCE: Diagnostic Level II. See Instructions for Authors for a complete description of levels of evidence.

Diagnostic challenges of indolent peripheral T cell lymphoma: A case report and literature review.

INTRODUCTION: Peripheral T cell lymphoma, not otherwise specified (PTCL-NOS) is a heterogeneous group of mature T cell lymphomas which do not correspond to any specific subtype of mature T-cell lymphoma in current classifications. Some researchers have suggested that PTCL with low Ki-67 labeling index should be classified as indolent PTCL PATIENT CONCERNS:: A 58-year old man diagnosed with alcoholic fatty liver 3 months prior complained of tenesmus and abdominal distension. Colonoscopy of the small and large intestines revealed multiple polyps, which were histologically diagnosed as lymphoid hyperplasia. One month later, he re-visited with a weight loss of 3 to 4 kg over 2 months. Radiologic examination revealed numerous small, homogenous, hypovascular lymph node enlargement in the para-aortic, mesenteric, and both inguinal areas, suggesting malignant lymphoma. DIAGNOSIS: Laparoscopic biopsy of an omental lymph node was performed, which was histologically confirmed as PTCL-NOS. INTERVENTIONS: The patient was administered 3 cycles of cyclophosphamide, doxorubicin, vincristine, and prednisone, but his general condition did not improve. Therefore, treatment was changed to ifosfamide, carboplatin, and etoposide -dexamethasone (4 cycles) followed by allogeneic stem cell transplantation. OUTCOME: Even after allogeneic stem cell transplantation, fluorodeoxyglucose uptake in his abdominal lymph nodes and small bowel in positron emission tomography- computed tomography persisted at a Deauville score of 4. The patient has been followed-up for 2 years without progression. CONCLUSION: These indolent PTCLs histologically show diffuse infiltrated small lymphoid cells with low KI-67 labeling index and have a relatively good prognosis, although the epidemiology and pathogenesis are not fully elucidated. We report a case of indolent PTCL with cytogenetic abnormalities and poor response to chemotherapy, along with a brief review of the literature.

Which Acetabular Landmarks are the Most Useful for Measuring the Acetabular Index and Center-edge Angle in Developmental Dysplasia of the Hip? A Comparison of Two Methods.

BACKGROUND: The acetabular index and center-edge angle are widely used radiographic parameters. However, the exact landmarks for measuring these parameters are not clearly defined. Although their measurement is straightforward when the lateral osseous margin of the acetabular roof coincides with the lateral end of the acetabular sourcil, where these two landmarks disagree, recommendations have differed about which landmark should be used. Using a radiographic parameter with high reliability for predicting residual hip dysplasia helps avoid unnecessary treatment. QUESTIONS/PURPOSES: We aimed to (1) compare two landmarks (the lateral osseous margin of the acetabular roof and the lateral end of the acetabular sourcil) for measuring the acetabular index and center-edge angle with respect to intraobserver and interobserver reliability and the predictability of residual hip dysplasia in patients with developmental dysplasia of the hip (DDH) and (2) evaluate longitudinal change in the acetabular edge's shape after closed reduction with the patient under general anesthesia. METHODS: Between February 1985 and July 2006, we performed closed reduction with the patient under general anesthesia as well as cast immobilization in 116 patients with DDH. To be included in this study, a patient had to have dislocated-type DDH. We excluded patients with a hip dislocation associated with neuromuscular disease, arthrogryposis, or congenital anomalies of other organs or systems (n = 9); hips that underwent osteotomy within 1 year since closed reduction (n = 8); hips that underwent open reduction because of re-dislocation after closed reduction (n = 4); and hips with Type III or IV osteonecrosis according to Bucholz-Ogden's classification (n = 4). Ninety-one patients were eligible. We excluded 19% (17 of 91) of the patients, who were lost to follow-up before they were 8 years old, leaving 81% (74 of 91 patients) with full preoperative and most-recent data. Ninety-seven percent (72 patients) were girls and 3% (two patients) were boys. The mean ± standard deviation age was 14.0 months ± 6.4 months (range 3-40 months) at the time of closed reduction and 12.1 years ± 2.3 years (range 8.0-16.0 years) at the time of the latest follow-up examination, the duration of which averaged 11 years ± 2.2 years (range 6.5-15.4 years). To investigate whether longitudinal change in the acetabular edge's shape differed among hips with DDH, contralateral hips, and control hips, we identified control participants after searching our hospital's database for patients with a diagnosis of congenital idiopathic hemihypertrophy from October 2000 to November 2006 who had AP hip radiographs taken at 3 years old and then at older than 8 years. From 29 patients who met these criteria, we randomly excluded two male patients to match for sex because girls were predominant in the DDH group. We excluded another female patient from the control group because of a hip radiograph that revealed unacceptable rotation. Eventually, 26 patients were assigned to the control group. Control patients consisted of 24 girls (92%) and two boys (8%). The demographic characteristics of control patients was not different from those of 67 patients with unilateral DDH, except for laterality (left-side involvement: 64% [43 of 67] in the DDH group versus 38% [10 of 26] in the control group; odds ratio 1.7 [95% confidence interval, 1.0-2.8]; p = 0.035). The acetabular index and center-edge angle at 3 years old were measured using the lateral osseous margin of the acetabular roof (AIB and CEAB) and the lateral end of the acetabular sourcil (AIS and CEAS). The treatment outcome was classified as satisfactory (Severin Grade I or II) or unsatisfactory (Grade III or IV). The intraclass correlation coefficient (ICC) was used to compare the intraobserver and interobserver reliability of each method. We compared the predictability of residual hip dysplasia of each method at 3 years old as a proxy using the area under the receiver operating characteristic (AUC) curve. To evaluate longitudinal change in the acetabular edge's shape, we compared the proportion of hips showing coincidence of the two landmarks between 3 years old and the latest follow-up examination. To investigate whether the longitudinal change in the acetabular edge's shape differs among hips with DDH, contralateral hips, and control hips, we compared the proportion of coincidence among the three groups at both timepoints. RESULTS: Intraobserver and interobserver reliabilities were higher for the CEAB (ICC 0.96; 95% CI, 0.94-0.98 and ICC 0.88; 95% CI, 0.81-0.92, respectively) than for the CEAS (ICC 0.81; 95% CI, 0.70-0.88 and ICC 0.69; 95% CI, 0.55-0.79, respectively). The AIB (AUC 0.88; 95% CI, 0.80-0.96) and CEAB (AUC 0.841; 95% CI, 0.748-0.933) predicted residual hip dysplasia better than the AIS (AUC 0.776; 95% CI, 0.67-0.88) and CEAS (AUC 0.72; 95% CI, 0.59-0.84) (p = 0.03 and p = 0.01, respectively). The proportion of hips showing coincidence of the two landmarks increased from 3 years old to the latest follow-up examination in hips with DDH (37% [25 of 67] to 81% [54 of 67]; OR = 8.8 [95% CI, 3.1-33.9]; p < 0.001), contralateral hips (42% [28 of 67] to 85% [57 of 67]; OR = 16.5 [95% CI, 4.2-141.9]; p < 0.001), and control hips (38% [10 of 26] to 88% [23 of 26]; OR = 14 [95% CI, 2.1-592.0]; p = 0.001). The proportion of coincidence in hips with DDH was not different from that in the contralateral hips and control hips at both timepoints. CONCLUSIONS: Measuring the acetabular index and center-edge angle at 3 years old using the lateral osseous margin of the acetabular roof has higher reliability for predicting residual hip dysplasia than that using the lateral end of the acetabular sourcil in patients with DDH treated with closed reduction. Measuring the acetabular index and center-edge angle at an early age using the lateral end of the sourcil may lead to overdiagnosis of residual hip dysplasia and unnecessary treatment. LEVEL OF EVIDENCE: Level III, diagnostic study.

Overexpression of Nrf2 promotes colon cancer progression via ERK and AKT signaling pathways.

PURPOSE: We investigated the expression of Nrf2 in colorectal cancer and its correlation with clinicopathological characteristics as well as mechanisms and roles of Nrf2 expression including cell signaling pathway, survival, proliferation, and migration. METHODS: Nrf2 expression was measured in 12 and 30 different colorectal cancer (CRC) tissues by western blot (WB) and immunohistochemistry (IHC), respectively. SW480 cells were used for cell proliferation and cell migration tests. The correlation between the expression of Nrf2 and clinicopathologic parameters were evaluated using the chi-square or Fisher exact test. Data are expressed as the mean ± standard deviation for 3 independent experiments. P < 0.05 was considered statistically significant. RESULTS: Analysis of WB demonstrated that Nrf2 proteins were increased in CRC tissues, and decreased in normal tissues. IHC staining showed that the Nrf2 expression was elevated in CRC tissues, compared to matched normal tissues. When SW480 cells were suppressed with small interfering RNA of Nrf2, cell viability was inhibited, and cell apoptosis was increased. These results were found along with suppression of the phosphorylated form of extracellular signal-regulated kinase 1/2 and AKT. CONCLUSION: This study suggests that overexpression of Nrf2 may be related to carcinogenesis and progression of CRC.

Functional Outcomes of Hip Arthroscopy for Pediatric and Adolescent Hip Disorders.

BACKGROUND: There is a paucity of literature on the use of hip arthroscopy for pathologic conditions in skeletally immature patients. Thus, the indications and safety of the procedure are still unclear. The purpose of this study was to investigate the safety and functional outcomes of hip arthroscopy for pediatric and adolescent hip disorders. We further attempted to characterize arthroscopic findings in each disease. METHODS: We retrospectively reviewed 32 children and adolescents with hip disorders who underwent 34 hip arthroscopic procedures at a tertiary care children's hospital from January 2010 to December 2016. We evaluated functional limitations and improvement after operation by using the modified Harris hip score (HHS), the Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC), subjective pain assessment with a visual analog scale (VAS), and range of hip motion as well as the complications of hip arthroscopy. Arthroscopic findings in each disease were recorded. RESULTS: Hip arthroscopy was performed for Legg-Calvé-Perthes disease (n = 6), developmental dysplasia of the hip (n = 6), slipped capital femoral epiphysis (n = 5), idiopathic femoroacetabular impingement (n = 6), sequelae of septic arthritis of the hip (n = 3), hereditary multiple exostosis (n = 2), synovial giant cell tumor (n = 3), idiopathic chondrolysis (n = 2), and posttraumatic osteonecrosis of the femoral head (n = 1). Overall, there was a significant improvement in the modified HHS, WOMAC, VAS, and range of hip motion. Symptom improvement was not observed for more than 18 months in four patients who had dysplastic acetabulum with a labral tear (n = 2) or a recurrent femoral head bump (n = 2). There were no complications except transient perineal numbness in five patients. CONCLUSIONS: Our short-term follow-up evaluation shows that hip arthroscopy for pediatric and adolescent hip disorder is a less invasive and safe procedure. It appears to be effective in improving functional impairment caused by femoroacetabular impingement between the deformed femoral head and acetabulum or intra-articular focal problems in pediatric and adolescent hip disorders.