Fifty percents of patient with achondroplasia present neurological disturbances of varying degree. Congenital narrowing of the spinal canal in achondroplastics seems to be the main cause of the cord disturbance, and there are several other causes such as prolapse of intervertebral discs, spondyloarthitic degenerative manifestations and wedging of vertebral body. Surgical treatment of the cord disturbance consist of anterior decompression with fusion and posterior decompression. We experienced L1-2 disc herniation in achondroplasia with rapid progression of neurologic symptoms and treated with posterior decompression. Two years after operation, the patient had good result.
Achondroplasia ; Decompression ; Humans ; Intervertebral Disc ; Neurologic Manifestations ; Prolapse ; Spinal Canal

No abstract available.
Osteoarthropathy, Primary Hypertrophic* ; Peptic Ulcer* ; Polyps* ; Stomach*

No abstract available.
Goldenhar Syndrome*

The effects of unilateral sciatic neurectomy on gastrocnemius muscle were studied in adult male rats. The morphological changes of both gastrocnemius muscles were observed by light and electron microscopy. Western blot analysis was performed to study the protein expression. Following the denervation, the affected muscle weights decreased significantly than normal. And the denervation led to a significant reduction in the area and diameter of muscle fibers on light microscopy. The affected muscle fibers showed the decreasing in size and the irregularity of myofibrilar arrangement on electron microscopy. On transverse view, the area of affected muscle fibers were smaller than normal. Their myofibrils were smaller and very irregular in size. The thin and thick myofilaments were not regular and partially lost. Mitochondria between myofibrils and subsarcolemmal area in affected muscle fibers were damaged and partially lost. The sacoplasmic reticulum and T-tubules were mostly lost and irregular. Some satellite cells were observed adjacent the muscle fiber, but they were inactive morphologically. On longitudinal view, most of myofibrils in affected muscle fibers were lost generally or partially although the their most sarcomeres were regularly arranged. Z line and myofilaments were lost partially and were partially irregularly arranged. The loss of thin myofilaments was more than that of thick myofilaments. Much debris of cell organelles were scattered among myofibrils. The expression of MyoD and myogenin were decrease significantly and the expression of p-mTOR and p-FOXO1 were decreased, too. On the other hand, MuRF1 was increased significantly. Taken together, the main effect of gastrocnemius muscle by sciatic neurectomy is the atrophy as a result of the loss of myofilaments and cell organelles through the decrease of protein synthesis and the increase of protein degradation.
Adult ; Animals ; Atrophy ; Blotting, Western ; Denervation ; Hand ; Humans ; Light ; Male ; Microscopy ; Microscopy, Electron ; Mitochondria ; Muscle, Skeletal ; Muscles ; Myofibrils ; Myogenin ; Organelles ; Proteolysis ; Rats ; Reticulum ; Sarcomeres ; Sciatic Nerve ; Weights and Measures

OBJECTIVE: This retrospective study compared the three-dimensional (3D) structure of mandibular condyles between adults with and without facial asymmetry, and whether it influences menton deviation. METHODS: Sixty adult patients were classified into symmetry and asymmetry groups based on the menton deviation on postero-anterior radiographs. The right/left differences of 3D measurements were compared between the two groups, and measurements were compared separately on the right and left sides. The correlations between menton deviation and the right/left differences were analyzed. RESULTS: The mediolateral dimension, neck length, condylar angles to the anteroposterior reference (PO) and midsagittal reference planes, and neck and head volumes showed significantly larger right/left differences in the asymmetry group compared to the symmetry group. Separate comparisons of the right and left sides between the two groups showed that the neck was significantly shorter and neck and head volumes were significantly smaller on the left side, which was deviated side in the asymmetry group. Pearson's correlation analysis showed significant positive correlations of menton deviation with right/left differences in neck length, condylar angle to the PO plane, and neck and head volumes in the asymmetry group. CONCLUSIONS: In individuals with facial asymmetry, menton deviation is associated with the right/left differences caused by a smaller condyle on the deviated side, particularly in neck length and neck and head volumes.
Adult* ; Facial Asymmetry* ; Head ; Humans ; Mandibular Condyle* ; Neck ; Retrospective Studies*

Purpose: This study was designed to evaluate the radiological and clinical outcomes of a new surgical technique—customized staple fixation using K-wire—in displaced metacarpal neck or base fractures. Materials and Methods: From November 2016 to May 2017, 13 unstable metacarpal neck and base fractures (10 patients) were treated with II-shaped customized K-wire staples fixation, after performing open reductions through minimal dorsal incisions. The radiological and clinical outcomes were retrospectively evaluated. Results: A mean of 2.6 staples were used for each fracture fixation. Preoperative angulation of 36.3°was reduced to 3.1° postoperatively. A week after surgery, the volar short arm splint was replaced with a dorsal splint to initiate active range of motion exercise, and the splint was subsequently removed after 3 weeks. The radiologic union was achieved at a mean of 5.1 weeks, and total active motion was recovered at a mean of 7.4 weeks. On a mean, K-wire staples were removed at 16.5 weeks after the surgery, and the mean treatment took 18.6 weeks. At the final follow-up (at mean 27.3 weeks), no significant difference was observed for total active motion of the digits and grip strength, when compared to the contralateral hand. Complete union was achieved in all fractures without deformity, or complications such as infection or nerve injury. All patients were satisfied with the cosmetic and functional outcomes. Conclusion K-wire stapling is an effective alternative modality in treating unstable displaced metacarpal neck or base fractures. It requires minimal incision to enable open reduction. In addition, early mobilization is ensured through the rigid fixations. Moreover, it prevents postoperative joint stiffness and reduces the time needed for treatment.

OBJECTIVE: This study aimed to investigate the effect of pre-applied orthodontic force on the regeneration of periodontal ligament (PDL) tissues and the underlying mechanisms in tooth replantation. METHODS: Orthodontic force (50cN) was applied to the left maxillary first molars of 7-week-old male Sprague–Dawley rats (n = 32); the right maxillary first molars were left untreated to serve as the control group. After 7 days, the first molars on both sides were fully luxated and were immediately replanted in their original sockets. To verify the effects of the pre-applied orthodontic force, we assessed gene expression by using microarray analysis and real-time reverse transcription polymerase chain reaction (RT-PCR), cell proliferation by using proliferating cell nuclear antigen (PCNA) immunofluorescence staining, and morphological changes by using histological analysis. RESULTS: Application of orthodontic force for 7 days led to the proliferation of PDL tissues, as verified on microarray analysis and PCNA staining. Histological analysis after replantation revealed less root resorption, a better arrangement of PDL fibers, and earlier regeneration of periodontal tissues in the experimental group than in the control group. For the key genes involved in periodontal tissue remodeling, including CXCL2, CCL4, CCL7, MMP3, PCNA, OPG, and RUNX2, quantitative RT-PCR confirmed that messenger RNA levels were higher at 1 or 2 weeks in the experimental group. CONCLUSIONS These results suggest that the application of orthodontic force prior to tooth replantation enhanced the proliferation and activities of PDL cells and may lead to higher success rates with fewer complications.

Objective: This study aimed to evaluate the association between low tongue position (LTP) and the volume and dimensions of the nasopharyngeal, retropalatal, retroglossal, and hypopharyngeal segments of the upper airway. Methods: A total of 194 subjects, including 91 males and 103 females were divided into a resting tongue position (RTP) group and a LTP group according to their tongue position. Subjects in the LTP group were divided into four subgroups (Q1, Q2, Q3, and Q4) according to the intraoral space volume. The 3D slicer software was used to measure the volume and minimum and average cross-sectional areas of each group. Airway differences between the RTP and LTP groups were analyzed to explore the association between tongue position and the upper airway. Results: No significant differences were found in the airway dimensions between the RTP and LTP groups. For both retropalatal and retroglossal segments, the volume and average cross-sectional area were significantly greater in the patients with extremely low tongue position. Regression analysis showed that the retroglossal airway dimensions were positively correlated with the intraoral space volume and negatively correlated with A point-nasion-B point and palatal plane to mandibular plane. Males generally had larger retroglossal and hypopharyngeal airways than females. Conclusions Tongue position did not significantly influence upper airway volume or dimensions, except in the extremely LTP subgroup.

BACKGROUND: Congenital ocular motor apraxia (COMA) is characterized by impaired voluntary saccades and abnormal head thrusts. However, mechanism of this disorder remains to be elucidated. METHODS: This study analyzed the eye movements and imaging findings in 16 patients with COMA, who had been recruited from 2003 to 2009 at the Neuro-Ophthalmology Clinic of Seoul National University Bundang Hospital. RESULTS: All the children showed impaired saccades and smooth pursuit in the horizontal direction. One of them also exhibited impaired vertical saccades and smooth pursuit. Eight children showed excessive blinks in association with an attempt to generate saccades. The typical head thrust usually developed around the age of eight months and had resolved by the age of 6-7 years. History of spasmus nutans was confirmed in seven children. Fourteen children showed cerebellar vermian hypoplasia, mostly in the inferior portion, and five of them also had dysgenesis of the corpus callosum. The severity of cerebellar vermian hypoplasia was correlated with developmental delay, as determined by the age of independent walking. CONCLUSION: Cerebellar vermian hypoplasia is a frequent finding in COMA. Dysfunction of the oculomotor vermis may responsible for the impaired saccades and smooth pursuit in COMA. The occasional association of COMA with spasmus nutans indicates a common pathophysiology of these benign developmental disorders.
Apraxias ; Cerebellum ; Child ; Cogan Syndrome ; Coma ; Corpus Callosum ; Eye Movements ; Head ; Humans ; Infant ; Infant, Newborn ; Pursuit, Smooth ; Saccades ; Spasms, Infantile

Cutaneous ossification is an unusual event, and it may be primary or secondary to either inflammatory or neoplastic processes. It is classified as primary when it occurs in the absence of a demonstrable preexisting lesion. Secondary ossifications have most commonly been reported to occur with pilomatricoma, basal cell carcinoma, acne vulgaris and melanocytic nevi (nevus of Nanta). Nevus of Nanta is a pigmented nevus associated with secondary ossification. The histopathological features of an osteo-nevus of Nanta are nests of nevus cells, and bony spicules are observed beneath a melanocytic nevus. We report here two cases of osteo-nevus of Nanta.
Acne Vulgaris ; Carcinoma, Basal Cell ; Neoplastic Processes ; Nevus ; Nevus, Pigmented ; Pilomatrixoma