Early Vascularized Fibular Grafts in Infants with Congenital Pseudarthrosis

No abstract available.

Comparison of Treatment Methods in Gartland Type III Pediatric Supracondylar Humeral Fracture: Lateral Entry Pin versus Crossed-Pin Technique

PURPOSE: The aim of this study was to compare the results of the lateral entry pin technique and the crossed pin technique in treatment of Gartland type III humerus supracondylar fracture. MATERIALS AND METHODS: Seventeen patients (group I) underwent surgery using the lateral entry pin technique, and 33 patients (group II) underwent surgery using the crossed pin technique for Gartland type III humerus supracondylar fracture in Hanyang University Seoul Hospital between January 2011 and January 2014. Maintenance of reduction was compared between the 2 surgical techniques by measuring changes in Baumann angle and lateral humerocapitellar angle after surgery and after pin removal in groups I and II. In addition, the final carrying angle and level of loss of functional movement were measured for comparison of clinical results between the 2 groups. Occurrence of ulnar nerve palsy in the 2 groups was also examined. RESULTS: The mean Baumann angle and lateral humerocapitellar angle changes were 3.3degrees and 3.7 in group I and 3.1degrees and 3.4degrees in group II, respectively. No statistically significant differences were found between the 2 groups. Clinical results showed that the changes in the final carrying angle and range of motion were 2.9degrees and 2.6degrees in group I and 2.6degrees and 3.0degrees in group II, respectively, indicating no significant differences between the 2 groups. In terms of nerve damage, 1 patient in group II had temporary iatrogenic ulnar nerve palsy. CONCLUSION: The lateral entry pin technique may be regarded as an appropriate treatment that reduces the risk of iatrogenic ulnar nerve palsy and provides satisfactory results in Gartland type III humerus supracondylar fracture patients.

Diffuse Skeletal Hemangiomatosis Mimicking Sacroiliitis

Hemangiomatosis of the skeletal system is a rare disease without specific symptoms and signs. We describe a 20-year-old patient with low back pain, whose plain radiographs of sacroiliac (SI) joint showed irregular sclerotic lesions. The patient was finally confirmed with skeletal hemangiomatosis by magnetic resonance imaging (MRI) and excisional biopsy of the lesion. The present case suggests that if patients with abnormal lesions of the SI joint in the plain radiographs do not have typical inflammatory back pain, advanced imaging is required to make an accurate diagnosis. Our case also emphasizes the importance of MRI and biopsy in establishing the diagnosis.

Eighteen Year Follow-Up Results of Accessory Lower Limb Disarticulation and Pelvic Bone Reconstruction for Monocephalus Tripus Tribrachius / 대한정형외과학회잡지

Monocephalus tripus tribrachius, a type of conjoined twins with one head and three upper and lower extremities, is a rare congenital disorder. To date, no long-term follow-up results of surgical procedures for this condition have been reported in Korean literature. We experienced a case of monocephalus tripus tribrachius, which had been surgically managed with an accessory lower limb disarticulation and pelvic bone reconstruction to manage this accessory limb and accompanying comorbidities in hip joint and pelvis. Subsequently, ipsilateral Syme amputation was done for intractable deformity of foot, and later, ipsilateral femoral varus derotational osteotomy was done for inadequate coverage of femoral head observed in follow-up. We report 18-year follow-up results of the procedures with a review of literatures.

Intraneural Ganglion Cyst of the Peripheral Nerve: Two Cases Report / 대한골관절종양학회지

Ganglion cysts that occurred within sheaths of peripheral nerves have been documented in literatures, but it is relatively rare condition. The peroneal nerve is the most common site of involvement. Other reported sites of involvement are the radial, ulnar, median, sciatic, tibial and posterior interosseous nerves. We report a case of the intraneual ganglion cyst within peroneal nerve and another case of the intraneural ganglion cyst within a medial plantar nerve that were successfully excised without neurologic complication.

Intramuscular Baker's Cyst in Plantaris: A Case Report / 대한골관절종양학회지

Baker's cyst is found as most frequent cystic mass around the knee occurring between medial head of gastrocnemius muscle and semimembranosus muscle. The proximal or posterolateral extension of the cyst had been rarely reported and the cyst into the surrounding muscular tissue extremely rare. Intramuscular Baker's cyst that we report was found between lateral head of gastrocnemius muscle and popliteus tendon, and then extended into the plantaris muscle. With review of the literature, we here report a very rare case of plantaris muscular extension of Baker's cyst.

Treatment for Bilateral Congenital Dislocation of the Patella Using Stanisavljevic Procedure / 대한정형외과학회잡지

Congenital dislocation of the patella is a rare clinical entity and is considered to result from a failure in internal rotation when the myotome develops, which forms the femur, the quadriceps muscle, and the extensor mechanism. Several authors have suggested various treatment methods, but there has been no consensus on treatment. A 12-year-old boy complained of knee pain and presented with a deformity due to bilateral congenital dislocation of the patella. The patient was treated with surgical management using the Stanisavljevic procedure. We report this case of congenital dislocation of the patella treated with this procedure that achieved a stable patellofemoral joint and satisfactory clinical results over 6.5 years of follow-up. In addition, we have included a review of the relevant medical literature.

Mini-review: Eosinophils, a Useful Diagnostic Clue in Surgical Neuropathology / 고신대학교의과대학학술지

Eosinophils are one of the polymorphonuclear granulocytes derived from bone marrow stem cells, and they contain many small cytoplasmic granules that stain bright red with eosin or brick-red with Romanowsky staining. Eosinophilic infiltration is also present in various human central nervous system (CNS) diseases such as parasitic infection, transverse myelitis, vasculitis, Langerhans cell histiocytosis, glioblastoma and etc... Due to the morphologic and functional characteristics, the presence of eosinophils in certain lesions may provide useful diagnostic clues in the right clinical setting. Consideration of this finding may facilitate the diagnosis of CNS pathologic lesions, especially in a small specimen such as a stereotactic biopsy.

Coxa Vara with Spondylometaphyseal Dysplasia / 대한정형외과학회잡지

Developmental coxa vara is a rare disease and the symptoms do not appear at birth, but rather, they appear at the age of walking. Clinically, the symptoms include a waddling gait, limb length discrepancy and frequent weariness. Developmental coxa vara is sometimes associated with skeletal dysplasia. Especially, it is associated with spondylometaphyseal dysplsia and the vertebral bodies and long bones are affected. The authors report here on diagnosing and treating this rare disease and we review the relevant literatures.

Juvenile Hemangioma Occurred in Distal Femoral Epiphysis / 대한골관절종양학회지

A hemangioma occurred in the bony epiphysis is extremly rare. A 5-year-old boy visited to our hospital with pain and flexion contracture on the right knee. MRI showed some lesions scattered in the epiphysis of the distal femur and the proximal tibia. Biopsy specimen from the distal femoral epiphysis revealed pathologic findings compatible with hemangioma. On 8 years follow-up, the lesion in the distal femoral epiphysis had been cured, and those in the proximal tibial epiphysis were spontaneously disappeared without surgery. The scanogram shows no leg length discrepancy and angular deformity. We reports a rare case of hemangioma occurred in the bony epiphysis with the results of 8 year follow-up with the review of literatures.

Clinical Heterogeneity in Korean Patients with Nemaline Myopathy

PURPOSE: Nemaline myopathy (NM) is a clinical heterogeneous congenital myopathy characterized by the presence of subsarcolemmal or cytoplasmic rod-like structures that call nemaline bodies in the muscle fibers. The purpose of this study was to investigate the clinical diversity and pathological features of Korean patients with NM. MATERIALS AND METHODS: Eight patients underwent analyses of clinical manifestations by a structured protocol. Diagnoses were established by a muscle biopsy. RESULTS: Two patients had the typical congenital type, which exhibited neonatal hypotonia and delayed motor milestone, and five patients had the childhood onset type, which exhibited mild gait disturbance as a first symptom. One patient had the adult onset type, which showed acute respiratory failure. Limb weakness was proximal-dominant occurred in six patients. Hyporeflexia was observed in most patients. Elongated faces and high arched palates and feet were also observed. On light microscopy, the nemaline bodies were observed in type 1 and 2 fibers. All patients showed type 1 predominance and atrophy. In the two cases in which ultrastructural studies were performed, typical nemaline rods and disorganized myofibrillar apparatus were detected. CONCLUSION: In conclusion, the eight Korean patients in this study with NM shared common clinical expressions such as proximal limb weakness, reduced deep tendon reflex, and dysmorphic features. This study, however, showed that clinical heterogeneity ranged from typical congenital, mildly affected childhood to the adult onset form with acute respiratory failure. The pathological findings in this study were in accordance with those of other previous reports.

Kennedy Disease Showing Prominent Remyelinating Features of Sural Nerve

We report a 55-year-old man with chronic weakness of both legs with recently experienced nasal voice. Despite the absence of sensory symptoms, electrophysiologic studies revealed the presence of sensorimotor polyneuropathy. A sural-nerve biopsy showed remarkable reduction of large myelinated fibers with prominent remyelination. Intravenous immunoglobulin was administered due to suspected chronic demyelinating neuropathy, but had no effect. Abnormal trinucleotide-repeat expansion of the androgen receptor gene was subsequently detected in both the patient and his family. These observation indicate that prominent remyelinating features are not necessarily indicative of demyelinating neuropathy.

An Autopsy Case of Multiple System Atrophy

Multiple-system atrophy (MSA) is an adult-onset, sporadic, progressive neurodegenerative disease. Clinically, the cardinal features include autonomic failure, parkinsonism, cerebellar ataxia, and pyramidal signs in any combination, of which autonomic failure is an integral component in the diagnosis. Pathologically, MSA is characterized by alpha-synuclein-positive glial cytoplasmic inclusions and neuronal loss, predominantly in the basal ganglia, brainstem, cerebellum, and intermediolateral cell columns of the spinal cord. We report the first case of MSA confirmed by autopsy in Korea.

Factor Analysis Affecting the Leakage of Bone Cement After Vertebroplasty

STUDY DESIGN: Retrospective study OBJECTIVE: This study examined the causative factors of cement leakage in an osteoporotic compression fracture that had received percutaneous vertebroplasty. SUMMARY OF LITERATURE REVIEW: Percutaneous vertebroplasty is simple and safe for the treatment of osteoporotic compression fractures. However, serious complications, such as pulmonary emboli and paraplegia, can occur if the bone cement leaks into the pulmonary artery or spinal canal. MATERIALS AND METHODS: Between Oct. 2002 and Apr. 2008, 95 patients (148 vertebral bodies) underwent percutaneous vertebroplasty for the treatment of an osteoporotic compression fracture. The presence of cement leakage was evaluated by plain radiography and computed tomography. The correlations between cement leakage and gender, age, level of fractured vertebra, fracture type, bone density, procedure, injecting amount, preoperative vertebral body compression rate, timing of surgery, and the existence of an intravertebral cleft on magnetic resonance imaging (MRI) were analyzed. RESULTS: Leakage was found in 37 bodies on plain radiography and 56 on the CT-scan. A comparison of the leakage and non-leakage groups revealed the bone density (p=0.046) and amount injected (p=0.000) to be related to cement leakage. Multivariate logistic regression showed that injecting more than 4.0ml was related to cement leakage with an odds ratio of 2.23(95% CI, 1.476~3.377). CONCLUSIONS: Cement leakage after percutaneous vertebroplasty is associated with the amount injected. Therefore, the cement volume should be restricted to the amount required for pain relief.

Bilateral Slipped Capital Femoral Epiphysis Due to Primary Hyperparathyroidism: A Case Report / 대한정형외과학회잡지

Slippage of the upper femoral epiphysis can occur in association with endocrine disorder. A 14-year-old male patient, who complained of pain on multiple joints including both hips, was diagnosed with bilateral slipped femoral epiphysis due to primary hyperparathyroidism. At a single operation, the epiphyses were pinned-in situ, and the tumor was removed. After parathyroidectomy, the calcium and the parathyroid hormone levels promptly resolved. The pinning-in situ of both femoral heads was done for the purpose of preventing further displacement of the femoral epiphyses, and we aimed for epiphysiodesis. The permanent biopsy of the tumor was parathyroid adenoma. The physes of both femoral heads were closed and the widened physes of both distal tibias resolved postoperatively.

Contributing Factors of Radial Nerve Palsy Associated with Humeral Shaft Fracture

PURPOSE: To analyze related factors of radial nerve palsy in patients with humeral shaft fractures. MATERIALS AND METHODS: We reviewed 107 paients with humeral shaft fracture between January 2000 and June 2007. Thirteen patients had radial nerve palsy after trauma and 9 patients after the operation. We analyzed contributing factors of radial nerve palsy associated with humeral shaft fracture including the cause of trauma, location and pattern of fracture, surgical approach and tourniquet application in cases of plate fixation, the exploration for the nerve and the time for operation. RESULTS: The difference in the incidences of radial nerve palsy after trauma and operation was not significant according to the location and pattern of fracture. The tendency of higher rate of radial nerve palsy after trauma in oblique or comminuted fractures, and after operation in spiral fractures was observed. The operation using intramedullary nailing and radial nerve exploration significantly reduced the incidence of radial nerve palsy after operation (p=0.01 and p=0.02). Posterior approach in open reduction and plate fixation showed a tendency of lower incidence of radial nerve palsy after operation (p=0.78). In logistic regression analysis, radial nerve exploration was the only significant factor that reduced the possibility of radial nerve palsy after operation (17.27: odds ratio, p=0.02). CONCLUSION: In humeral shaft fractures, we should take into consideration whether intramedullary nailing is possible or not. In cases of anterior or anterolateral approach of open reduction and plate fixation, radial nerve should be carefully inspected. In most cases, we recommend radial nerve exploration in order to minimize the possibility of radial nerve palsy after operation.

Expressions of Nitric Oxide Synthases and Akt in the Tibialis Anterior Muscle of the Spontaneously Hypertensive Rat after Ischemic Preconditioning / 체질인류학회지

Nitric oxide (NO) generated during ischemic-reperfusion phase is known to activate various transcriptional factors. NO is generated by 3 nitric oxide synthase (NOS) isoforms, nNOS, iNOS, eNOS. Akt plays a important role of the cell survival. Ischemic preconditioning (IP) reduces ischemic injury by changing of the expression of the NOSs and Akt. The alterations of the NOSs is related to the development of hypertension, and hypertension has been known to induce the skeletal muscle changes including decreased fatigue resistance and contractile force. The purpose of this study was to observe the effect of IP on the expressions of the NOSs and Akt in the skeletal muscle of the spontaneously hypertensive rat (SHR). Nine weeks old male normotensive rat (Wistar-Kyoto rat, WKY) and SHR were divided into control and IP groups. The IP group was further divided into 3 or 10 cycles of IP. For IP, left common iliac artery was occluded 3 or 10 cycles of 5 min of ischemia/5 min of reperfusion using rodent vascular clamp. The animals were sacrificed at 0, 0.5, 1, 3 and 24 hours after reperfusion and the tibialis anterior muscles were removed. The expressions of nNOS, iNOS, eNOS, and Akt were examined with immunohistochemical methods and Western blot analysis. In the SHR, the expression of nNOS was decreased, iNOS was increased and the expressions of eNOS and AKT were decreased, compare to WKY. The expression of iNOS was increased, and nNOS, eNOS and Akt were decreased in SHR by 10IP. In summary, IP increased the expression of iNOS, and decreased the expression of eNOS and Akt in the SHR. It is suggested that hypertension may aggravate injury of the tibialis anterior muscle induced by repetitive ischemic conditioning.

A Dumbbell-Shaped Solitary Fibrous Tumor of the Cervical Spinal Cord

A 40-year-old Asian female presented with a 2-month history of right shoulder pain and right triceps weakness. MRI revealed an extramedullary, extradural, dumbbell-shaped spinal cord tumor with C6 to C7 iso- and hyperintensity on T1 and T2 weighted imaging, respectively. Histological examination revealed monomorphous spindle cells with a storiform pattern. Immunohistochemistry was positive for CD34, CD99, and negative for EMA, SMA, and S100; solitary fibrous tumor (SFT) was confirmed.

Vimentin and Survivin Expression Rates as Prognostic Factors in Medulloblastoma

BACKGROUND: A medulloblastoma is a primitive neuroepithelial tumor of the cerebellum that occurs in children and metastasizes through the cerebrospinal fluid. It is highly malignant and invasive, and the 5-year survival rate is only 60%. Surgical resection techniques, radiation, and chemotherapy have improved the overall survival but the patients suffer life-long cognitive dysfunctions or endocrine abnormalities as the side effects of treatment. Therefore it is essential to identify prognostic markers to determine the appropriate treatment strategy in order to minimize the side effects. METHODS: This study evaluated the immunohistochemical differentiation and survival rate with synaptophysin, glial fibrillary acidic protein, epithelial membrane antigen, vimentin and primitive neuroepithelial marker nestin of 55 paraffin-embedded medulloblastomas, using a tissue microarray. The expression of survivin, the apoptotic inhibitor, and the survival rate with regard to the proliferation index of Ki-67 were also investigated. RESULTS: The group testing positive to vimentin, a mesenchymal differentiation marker, had a worse prognosis and there was a strong correlation between vimentin expression and nestin expression. Patients with a survivin expression rate >35% had a significantly poorer clinical course and there was a correlation between the survivin expression rate and Ki-67 expression rate. CONCLUSION: In conclusion, vimentin and survivin are negative prognostic markers in medulloblastomas.

A Case of Adult Polyglucosan Body Disease

Adult polyglucosan body disease (APBD) is a rare neurological disease, characterized by adult onset (fifth to seventh decades), progressive sensorimotor or pure motor peripheral neuropathy, upper motor neuron symptoms, neurogenic bladder, and cognitive impairment. APBD is confirmed by a sural nerve biopsy that shows the widespread presence of polyglucosan bodies in the nerve. We report a 70 year old male patient who exhibited progressive weakness in all extremities and dementia. His electrodiagnostic studies showed sensorimotor polyneuropathy and muscle pathology that consisted of polyglucosan bodies located in small peripheral nerves. This is the first case of APBD reported in Korea.