Histomorphometric analysis of patients with femoral neck fracture and 25-hydroxyvitamin D deficiency: a cross-sectional study.

INTRODUCTION: Vitamin D deficiency causes osteoporosis, bone mineralization disorders, and osteomalacia. Osteomalacia is diagnosed using blood biochemical tests, clinical symptoms, and imaging; however, accurate detection of mineralization disorders requires tissue observation. We investigated the prevalence of bone mineralization disorders and their relationship with serum 25-hydroxyvitamin D (25OHD) levels in patients with untreated osteoporosis with femoral neck fractures. MATERIALS AND METHODS: A non-demineralized specimen was prepared from the femoral head removed during surgery in 65 patients. Bone histomorphometry of cancerous bone in the femoral head center was conducted. Osteoid volume per bone volume (OV/BV) and osteoid thickness (O.Th) were measured as indicators of mineralization disorder. RESULTS: The mean serum 25OHD level (11.9 ± 5.7 ng/mL) was in the deficiency range (< 12 ng/mL). There were no clinically diagnosed cases of osteomalacia (OV/BV > 10% and O.Th > 12.5 µm); however, one case of mineralization disorder, considered histologically pre-osteomalacia (OV/BV > 5% and O.Th < 12.5 µm), was observed (OB/BV, 17.6%; O.Th, 12.3 µm). Excluding this case, those with severe (25OHD < 12 ng/mL, at risk of osteomalacia; n = 39) and non-severe deficiency (25OHD ≥ 12 ng/mL; n = 25) did not significantly differ in OV/BV (%; 0.77 ± 0.54 vs. 0.69 ± 0.38, p = 0.484) or O.Th (µm; 5.32 ± 1.04 vs. 5.13 ± 0.78, p = 0.410). Further, 25OHD and OV/BV were not significantly correlated (R = - 0.124, p = 0.327). CONCLUSION: This is the first study in the twenty-first century to examine serum 25OHD concentrations and bone mineralization disorders in Japanese patients with osteoporosis. The results indicate that vitamin D deficiency does not necessarily cause bone mineralization disorders and rarely leads to osteomalacia.

A rare pediatric case of McCune-Albright syndrome with acute visual disturbance: Case report.

RATIONALE: McCune-Albright syndrome (MAS) is a rare disorder characterized by clinical findings, which includes fibrous dysplasia (FD). FD is a benign tumor that leads to increased rates of bone fracture. In some MAS cases with FD, facial deformities, severe pain, and orbital neuropathies are complicated. Aneurysmal bone cyst (ABC) is a benign bone tumor and rare complication of FD. PATIENT CONCERNS: A 9-year-old boy was admitted to our hospital because of acute visual disturbance. DIAGNOSIS AND INTERVENTIONS: The patient was clinically diagnosed as ABC complicated with MAS, and he underwent surgery. OUTCOMES: After the surgery, his sight became normal. Recurrence of ABC and visual disturbance was not observed in 3 years. Genetic analysis of a tissue sample from the ABC lesion by next-generation sequencing revealed a somatic activating GNAS mutation. LESSONS: To the best of our knowledge, this is the first case report of MAS causing optic neuropathy complicated with ABC. ABC complicated with MAS is extremely rare, but it should be considered as a possible diagnosis in patients with acute visual loss and facial swelling. In addition, our case had OAS, which is an uncommon syndrome and a rare complication in ABC with MAS, and rapid decompression of the ABC was effective in improving the patient's eyesight.

Surgical Exposure Technique for Volar Locking Plate Fixation of Distal Radius Fractures in Patients with Flexor Carpi Radialis Brevis Muscle Anomaly.

The flexor carpi radialis brevis (FCRB) muscle, considered a rare anomaly, is not well known among orthopedic surgeons. The indications for volar locking plates to treat distal radius fractures have recently expanded, and, as a result, encounters with the FCRB are becoming more common. However, few studies have described how to retract an FCRB. Here, we describe seven of 264 patients with FCRB who underwent surgery for distal radius fractures. In one case, the retracted FCRB interfered with the internal fixation. The presented cases demonstrate that the radial retraction of an FCRB with a large muscle belly enables favorable exposure of the distal radius.

Innovative Fixation Technique for Avulsion Fractures of the Calcaneal Tuberosity.

Avulsion fractures of the calcaneal tuberosity, although relatively uncommon, occur more frequently in patients with osteoporosis and in the elderly. The results of closed manipulation are poor in these fractures, usually requiring open reduction and internal fixation. However, it is difficult to fix the bone fragment rigidly, because the avulsed bone fragment is small and thin, and the bone quality of the calcaneal body in the elderly is poor. Hence, it is necessary to limit prolonged weight-bearing after the operation. We performed an innovative surgical procedure of suture fixation to the anchor screw in four cases, following which earlier postoperative rehabilitation with full weight-bearing walking and range of motion exercises was possible, and bony union was achieved without repeated displacement of the fragment in all patients. We believe this technique would prove useful in surgical management of calcaneal tuberosity avulsion fractures.

A pediatric case of anaplastic astrocytoma with a gliomatosis cerebri; the growth pattern and changes in serum VEGF-121 levels after bevacizumab treatment.

Gliomatosis cerebri (GC) is a rare diffusely infiltrating glial neoplasm that carries a poor prognosis. Because tumors are undetectable in most patients at early-stage of the onset, a useful diagnostic method is expected. We compared serum vascular endothelial growth factor (VEGF)-121 levels in patients with GC or glioblastoma and controls. VEGF-121 levels were significantly higher in one patient with GC and patients with glioblastoma than in controls. VEGF-121 levels decreased in a patient with GC after bevacizumab-based therapy. Thus, VEGF-121 may be useful for diagnosing GC, its disease-monitoring and understanding its etiology.

Bilateral atypical femoral subtrochanteric fractures in a premenopausal patient receiving prolonged bisphosphonate therapy: evidence of severely suppressed bone turnover.

We report a case of bilateral atypical femoral fractures that occurred in a patient who had been taking bisphosphonate long-term. A 36-year-old premenopausal female diagnosed with systemic lupus erythematosus and dermatomyositis had been treated with glucocorticoid and alendronate (5 mg/day) to prevent glucocorticoid-induced osteoporosis. She was taken to our hospital because she could not walk immediately after falling down from the standing position. A plain radiograph showed a subtrochanteric fracture of the left femur. Four months later, she fell again and sustained a contralateral subtrochanteric fracture. For each fracture, a femoral intramedullary nail was inserted. Delayed union was detected in both sides, and revision surgery with an iliac bone graft was required for implant breakage in the right side. Histomorphometric findings for the ilium revealed remarkably decreased osteoid volume with no osteoclasts and a minimally eroded surface, suggesting that bone turnover was severely suppressed. However, histology of the delayed union site revealed callus formation and some osteoclast appearance, suggesting that fracture healing was occurring. In total, it took 29 months (left) and 24 months (right) until fracture healing was achieved, showing delayed union. This case is extremely rare in that patient who presented with atypical femoral fractures in spite of her premenopausal status. The bone histomorphometric findings from this case suggest that severely suppressed bone turnover is associated with atypical femoral subtrochanteric fracture and can cause delayed union in patients treated with alendronate long-term.

Improvement of tracheal flap method for laryngotracheal separation.

OBJECTIVES/HYPOTHESIS: Recurrent pneumonia due to intractable aspiration is a life-threatening disease. A tracheal flap method for children without previous tracheostomy has been previously reported. This study reports that improvements of this method and its three subtypes are widely applicable to patients with various conditions. STUDY DESIGN: Surgical technique study. METHODS: The tracheal flap method does not involve transection of the trachea but achieves laryngotracheal separation using the tracheal, mucoperichondrial, and sternohyoid muscle, along with anterior cervical skin flaps. This method can be divided into three subtypes as follows: A-type, utilizing the tracheal flap (for patients without previous tracheostomy); B-type, utilizing the mucoperichondrial and sternohyoid muscle flaps (for patients lacking an anterior tracheal wall); and C-type, utilizing the esophageal flap (for patients with severe hypersalivation). In all three subtypes, the anterior cervical skin flap is employed. RESULTS: The tracheal flap method was performed in 30 patients (24 children and six adults) at risk of developing intractable aspiration pneumonia. In all 30 cases, aspiration pneumonia was prevented without severe complications. No fistula formation was observed. CONCLUSIONS: All three subtypes (A-, B-, and C-type) of the tracheal flap method are effective in preventing the recurrence of aspiration pneumonia. This method is applicable to diverse patient backgrounds regardless of age or previous tracheostomy. It is less invasive than Lindeman procedure. Furthermore, this method is acceptable to patients' families and improves the QOL of both patients and caregivers.

[A pitfall of AirWay Scope--an experience of distinctive airway edema after palatal laceration caused by irWay Scope].

Pentax AirWay Scope (AWS) is a new videolaryngoscope which allows indirect visualization of the vocal cords and provides a better laryngeal view compared with the conventional Macintosh laryngoscope. We report a female patient who developed distinctive upper airway edema after palatal laceration at the time of insertion of the AWS. She was scheduled for tympanoplasty. After anesthesia induction, there was technical difficulties in tracheal intubation with Macintosh laryngoscope (her Cormack grade was rated as 3), and we used the AWS, but could not obtain an appropriate view on the monitor. When the device was removed, we detected bleeding derived from the right palatal laceration. Following the aspiration of the blood, we could barely achieve tracheal intubation with a flexible fiberscope without hypoxemic episode. The upper airway including the arytenoid and vocal cords were distinctively edematous after the operation. Then, the tracheal tube was left for three days after the operation, to secure the airway until the reduction of airway edema. On the postoperative day 4, following the fibroscopic confirmation that the airway edema was reduced, the patient was extubated successfully. AWS should be used more carefully, especially at the time of insertion along the palate.

Simple and new surgical procedure for laryngotracheal separation in pediatrics.

OBJECTIVES: In children with severe physical and mental disabilities who repeatedly develop aspiration pneumonia due to intractable aspiration, laryngotracheal separation/tracheoesophageal anastomosis or laryngotracheal separation has been performed in many institutions for the prevention of aspiration, and good results have been reported. However, families sometimes show a marked reluctance to give consent to these surgical techniques because of tracheal transection. A purpose of this study is to evaluate a new surgical procedure for laryngotracheal separation without tracheal transection. STUDY DESIGN: Case-series study. METHODS: As a new, simple, less invasive surgical technique for the prevention of aspiration without tracheal transection, we performed tracheal closure (tracheal flap method) in six children. A U-shaped flap of the tracheal anterior wall from the 2nd to the 4th/5th tracheal ring was produced, bent toward the tracheal lumen, and sutured to the tracheal posterior/lateral walls by mattress stitches for tracheal closure. In addition, the closure was covered with a cutaneous U-shaped flap for reinforcement and a permanent tracheal stoma was constructed. RESULTS: In all six patients, aspiration pneumonia could be prevented without severe complications. CONCLUSIONS: Tracheal closure (tracheal flap method) has effects comparable to those of other surgical techniques for the prevention of aspiration, and may be useful for aspiration prevention in children with severe physical and mental disabilities.

Mucocele in an Onodi cell with simultaneous bilateral visual disturbance.

The Onodi cell is a large pneumatized posterior ethmoid cell and closely related to optic nerve. We present an extremely rare case of retrobulbar optic neuropathy caused by mucocele in an Onodi cell. A 79-year-old man complained of headaches and simultaneous bilateral visual disturbance. A computed tomography (CT) scan demonstrated a mucocele in an Onodi cell, which involved bilateral optic nerves. The surgical treatment with a transnasal endoscopic approach was performed, resulting in the improving of visual acuity. The bilateral optic nerves were identified along each lateral wall into an Onodi cell accompanied with bone defect. In an Onodi cell, even if the lesion is isolated and/or small, it may be closely related to ocular symptoms. Imaging studies should be considered for the differential diagnosis because early diagnosis and prompt surgical treatment for mucocele are needed for recovery of visual impairment.

Radiation therapy for T2N0 laryngeal cancer: A retrospective analysis for the impact of concurrent chemotherapy on local control.

PURPOSE: The impact of concurrent chemotherapy on the local control in patients with T2N0 laryngeal cancer who receive radiation therapy (RT) was evaluated. METHODS AND MATERIALS: Sixty-three patients with T2N0 laryngeal cancer who were treated by definitive RT were analyzed. The primary site of the cancer was the glottis in 50 patients, the supraglottis in 9 patients, and the subglottis in 4 patients. Thirty-six patients were treated by RT alone and the remaining 27 patients received concurrent chemoradiotherapy (CRT). RESULTS: Complete response (CR) was obtained in 92% of the patients who received RT alone and 100% of the patients who received CRT. Voice preservation in the group who received CRT (89%) was significantly higher than that in the group treated by RT alone (61%). The 5-year disease-free survival rates in those who received concurrent CRT was significantly superior to that in the patients who received RT alone, although no significant difference was seen in the cause-specific survival rate between the 2 groups. The multivariate analysis revealed that the treatment method (RT alone vs. CRT) was the most significant risk factor that predicted recurrence after RT. CONCLUSION: Concurrent CRT had a positive impact on the local control of T2N0 laryngeal cancer.

Acute toxicity and preliminary clinical outcomes of concurrent radiation therapy and weekly docetaxel and daily cisplatin for head and neck cancer.

OBJECTIVE: To examine the feasibility and efficacy of concurrent weekly docetaxel and radiation therapy as a definitive treatment for head and neck cancer (HNC). METHODS: Thirty-two patients with primary HNC, who were treated with concurrent weekly docetaxel and radiation therapy, were analysed. The distribution of the disease stage was as follows: Stage II, 18 patients; Stage III, 3 patients; Stage IVA, 7 patients; Stage IVB, 3 patients; the patient of cervical lymph node metastasis with unknown primary tumor was not assessable. The average total dose of radiotherapy was 67.5 Gy. Docetaxel (10 mg/m(2), intravenously, once a week) was given to all patients up to four cycles, and cisplatin (6 mg/m(2), intravenously, five times a week) was also administered to all patients for up to 3 weeks from the beginning of the radiation therapy. RESULTS: Only in two patients did the radiotherapy need to be temporarily interrupted due to the development of acute mucositis. Grade 3 toxicity was observed in six patients. Grade 4 acute mucositis was seen in one patient. The response rate was 100%, and complete response (CR) was observed in 30 patients (94%). At the time of the analysis, the 2 year local control and relapse-free rates in the 30 patients showing CR were 90 and 76%, respectively. CONCLUSIONS: Concurrent weekly docetaxel and radiation therapy did not affect the compliance of the patients for the radiation therapy, indicating that the acute toxicities were within acceptable limits.

Mutational analysis of the MRP2 gene and long-term follow-up of Dubin-Johnson syndrome in Japan.

BACKGROUND: Recent studies have indicated that dysfunction or loss of the multidrug resistance protein 2 (MRP2) is the molecular basis of Dubin-Johnson syndrome (DJS). To clarify the genetic basis of the disease and the long-term stability of serum bilirubin levels, we conducted a mutational analysis of the MRP2 gene and followed up serum bilirubin levels in Japanese DJS patients 30 years after they were originally diagnosed, based on traditional criteria. METHODS: Patients were interviewed by telephone, and blood tests, including a genetic analysis of MRP2, were performed on the patients and family members who gave informed consent. RESULTS: Over the 30 years, hyperbilirubinemia remained unchanged in four of the five patients studied, while it worsened in 1 patient whose DJS was complicated by chronic hepatitis C. From an MRP2 gene mutational analysis, six mutations, including the novel mutation 1177C>T, were found. Three patients of a consanguineous family were homozygotes for three mutations (298C>T, 1967+2T>C, and 2439+2T>C). Two patients were compound heterozygotes (1177C>T/2302C>T and 1967+2T>C/2026G>C). A familial study showed no difference in serum bilirubin levels between mutant/wild heterozygotes and wild/wild homozygotes. CONCLUSIONS: The hyperbilirubinemia of four Japanese patients with DJS, one of whom had a novel mutation, 1177C>T, of the MRP2 gene, had not worsened with aging.

Positron emission tomographic imaging with 11C-choline in differential diagnosis of head and neck tumors: comparison with 18F-FDG PET.

UNLABELLED: The aim of this study was to evaluate the clinical value of positron emission tomography (PET) with 11C-labeled choline (CHOL) for the differential diagnosis of malignant head and neck tumors from benign lesions as compared with 18F-fluorodeoxyglucose PET. METHODS: We studied 45 patients (28 males, 17 females, age range, 29-84 years) with suspected lesions in the head and neck region using both CHOL and FDG PET within a 2-week period on each patient. All patients fasted for at least 6 hours for both the CHOL and FDG studies. PET imaging was performed 5 min and 50-60 min after intravenous injection of CHOL and FDG, respectively. After data acquisition, PET images were corrected for attenuation, and the reconstructed images were analyzed by visual interpretation. Then, the standardized uptake value (SUV) was calculated for semiquantitative evaluation of tumor tracer uptake. Finally the results of PET scans were compared with the histological diagnoses from surgical specimens or biopsies. RESULTS: With CHOL PET, malignant tumors were correctly detected in 24 (96%) of 25 patients, and benign lesions in 14 (70%) of 20 patients with an accuracy of 84.4%. With FDG PET, malignancy was correctly diagnosed in 23 (92%) of 25 patients, and benign lesions in 13 (65%) of 20 patients resulting an accuracy of 80%. A significant positive correlation between CHOL and FDG SUVs was found for all lesions (r = 0.677, p = 0.004, n = 45). Malignant tumors showed significantly higher tracer accumulation than the benign lesions in both CHOL and FDG studies (5.69 +/- 1.61, n = 25 vs. 2.98 +/- 2.13, n = 20, p < 0.0001; 9.21 +/- 4.23, n = 25 vs. 3.60 +/- 2.57, n = 20, p < 0.0001). The cutoff SUV for differentiating malignant and benign lesions was 3.5 for CHOL and 3.9 for FDG. CHOL showed slightly better differentiation between malignant and benign lesions than FDG although some overlap existed on both studies. But the difference was not statistically significant. CONCLUSION: The results of this study indicate that CHOL PET may be feasible clinically for head and neck tumor imaging. PET imaging with CHOL seems to be able to detect malignant head and neck tumors as effectively as FDG PET. The advantages of CHOL PET were shorter examination period and low uptake in the muscle. However, both CHOL and FDG have some limitations in the evaluation of salivary gland lesions.

A preliminary study on sentinel lymph node biopsy: feasibility and predictive ability in oral cavity cancer.

The main factor that affects the prognosis of patients with head and neck cancer (HNC) is regional lymph node metastases. For this reason, the accurate evaluation of neck metastases is required for neck management. This study investigates the sentinel lymph node identification and the accuracy of the histopathology of the sentinel lymph node in patients with HNC. Eleven patients with histologically proven oral squamous cell carcinoma accessible to radiocolloid injection were enrolled in this study. Using both lymphoscintigraphy and a handheld gamma probe, the sentinel lymph node could be identified in all 11 patients. Subsequently, the sentinel lymph nodes and the neck dissection specimen were examined for lymph node involvement due to tumor. The histopathology of sentinel lymph nodes was consistent with the pathological N classification in all 11 patients. Furthermore, the histopathology of sentinel lymph nodes was superior to physical examination, computed tomography (CT), magnetic resonance imaging (MRI) and positron emission tomography (PET) scan. The results of this study indicate that sentinel lymph node identification is technically feasible and predicts cervical metastases in patients with oral cavity cancer. This may be a useful diagnostic technique for identifying lymph node disease in staging lymph node dissection.

Diagnosis of tumor in the nasal cavity and paranasal sinuses with [11C]choline PET: comparative study with 2-[18F]fluoro-2-deoxy-D-glucose (FDG) PET.

[11C]choline (11C-choline) positron emission tomography (PET) was performed to evaluate its clinical utility in the diagnosis of tumors in the nasal cavity and paranasal sinuses. We studied 22 patients with suspicion of malignant tumors in the nasal cavity and paranasal sinuses. Tumor uptake of 11C-choline was measured with standardized uptake value (SUV) and correlated with the pathological diagnosis. 2-[18F]fluoro-2-deoxy-D-glucose (FDG) PET was performed in all patients for comparison. Both 11C-choline and FDG PET depicted squamous cell carcinoma showing an increased activity significantly higher than that of normal tissue, and these SUVs were significantly higher than those of benign lesions. FDG uptake in malignant tumors as a whole was variable. Although 11C-choline uptake in squamous cell carcinoma was lower than FDG uptake, 11C-choline uptake in malignant tumors was relatively uniform and statistical significance was found. PET with 11C-choline may be useful to diagnosis tumors in the nasal cavity and paranasal sinuses.