Melorheostosis is a rare disease entity of bone with an etiology and pathogenesis. It cause pain and stiffness in an affected limb, and dense bone formation along the side of bone resembles the flow of candle dripping. Two cases of the disease involving left hand and wrist, and right lower leg are reported with the review of literature.
Extremities ; Hand ; Hyperostosis ; Leg ; Melorheostosis ; Osteogenesis ; Rare Diseases ; Wrist

No abstract available.
Polydactyly

An intraosseous epidermal cyst is a rare benign cystic lesion. It is thought to result from congenital factors or trauma and can lead to bone destruction because the cyst develops at the soft tissue around the bone. Radiological findings of intraosseous epidermal cysts are a well-defined radiolucent lesion, with cortical expansion. It is important to differentiate an intraosseous epidermal cyst with other disease developed at distal phalanx because its clinical and radiological findings are similar. We report two rare cases of intraosseous epidermal cysts that developed at the distal phalanx.
Epidermal Cyst*

Cystinuria is an autosomal recessive inherited defect in renal tubular reabsorption of four amino acids, cystine, ornithine, lysine and arginine. Homozygotes were identified by the formation of urinary tract calculi composed of cystine and by gross hyperexcretion of above mentioned four amino acids. Urinary tract calculi composed primarily of cystine are rare in adults and children. These are usually pure and found exclusively in patients with cystinuria. Herein we report on a 4 and 3/12 years old male child with a right renal stone composed primarily of cystine which was confirmed by chemical analysis method postoperatively. After discharge he has been treated with D-penicillamine, large fluid intake and conversion of urine pH.
Adult ; Amino Acids ; Arginine ; Calculi ; Child* ; Cystine* ; Cystinuria ; Homozygote ; Humans ; Hydrogen-Ion Concentration ; Lysine ; Male ; Ornithine ; Penicillamine ; Urinary Tract

Purpose: To report a case of accidental swallowing of nasal packing gauze during silicone tube intubation under local anesthesia.Case summary: A 58-year-old male patient underwent silicone tube intubation for partial nasolacrimal duct obstruction on the right side. In preparing for surgery, six cottonoids soaked were packed in righ nasal cavity, to facilitate anesthesia and prevent bleeding. Strings detectable by X-ray were attached at the cottonoids and cut 5 cm from the nostrils. After surgical hand scrubbing, the strings were not present. One cottonoid was found in the nasal cavity, but five cottonoids could not be detected by endonasal endoscopic exploration. The patient said he swallowed “something like sputum”. He did not notice any discomfort and the surgery was performed as scheduled. Abdominal X-ray performed immediately after surgery showed the presence of the five strings in the stomach. The strings were not seen on X-ray conducted 4 days later. The patient defecated normal stools three times and did not notice any discomfort over the course of 4 days. Conclusions Surgeons need to be aware of the possibility of accidental swallowing of nasal packing gauze during nasolacrimal duct surgery. Use of gauze detectable by X-ray is helpful to determine its location.

PURPOSE: To evaluate the clinical and radiological outcomes, and the complications of unicompartmental knee arthroplasty (UKA) using a fixed bearing prosthesis after 5-year follow-up. MATERIALS AND METHODS: Twenty-six knees (25 patients) that underwent fixed bearing UKA between May 2003 and August 2011 were included. The subjects were 3 males (3 knees) and 22 females (23 knees), and the average age was 63.5 years. The preoperative diagnosis was osteoarthritis (23 knees) and osteonecrosis (3 knees). The mean follow-up duration was 67 months (from 60 to 149 months). The clinical evaluation included pre- and postoperative American knee society knee and function score, and range of motion. The radiology evaluation included standing antero-posterior, lateral view, and fluoroscopic film to analyze the postoperative alignment and osteolysis. RESULTS: The mean American Knee Society knee score and function score were improved from 42.0 and 57.5 to 87.9 and 85.0, respectively (p < 0.001). The mean preoperative and postoperative range of motion was 132.9° and 132.5°, respectively. The mean femorotibial angle were varus 0.5° preoperatively and valgus 2.2° postoperatively. A radiolucent line was observed in 2 knees; one knee had a stable implant, while in the other knee, patellofemoral arthritis was identified during UKA. Diffuse pain of the knee joint with tenderness of the medial joint line was identified at the follow-up, so conversion to total knee arthroplasty was recommended. No other complications, such as osteolysis, infections, postoperative stiffness, and dislocation, were encountered. CONCLUSION: The midterm results of fixed bearing UKA were clinically and radiologically satisfactory.
Arthritis ; Arthroplasty, Replacement, Knee* ; Diagnosis ; Dislocations ; Female ; Follow-Up Studies* ; Humans ; Joints ; Knee ; Knee Joint ; Male ; Osteoarthritis ; Osteolysis ; Osteonecrosis ; Prostheses and Implants ; Range of Motion, Articular

PURPOSE: This study aimed to verify the safety of low-dose topiramate on language development in pediatric patients with migraine. METHODS: Thirty newly diagnosed pediatric patients with migraine who needed topiramate were enrolled and assessed twice with standard language tests, including the Test of Language Problem Solving Abilities (TOPs), Receptive and Expressive Vocabulary Test, Urimal Test of Articulation and Phonology, and computerized speech laboratory analysis. Data were collected before treatment, and topiramate as monotherapy was sustained for at least 3 months. The mean follow-up period was 4.3±2.7 months. The mean topiramate dosage was 0.9 mg/kg/day. RESULTS: The patient's mean age was 144.1±42.3 months (male-to-female ratio, 9:21). The values of all the language parameters of the TOPs were not changed significantly after the topiramate treatment as follows: Determine cause, from 15.0±4.4 to 15.4±4.8 (P>0.05); making inference, from 17.6±5.6 to 17.5±6.6 (P>0.05); predicting, from 11.5±4.5 to 12.3±4.0 (P>0.05); and total TOPs score, from 44.1± 13.4 to 45.3±13.6 (P>0.05). The total mean length of utterance in words during the test decreased from 44.1±13.4 to 45.3±13.6 (P<0.05). The Receptive and Expressive Vocabulary Test results decreased from 97.7±22.1 to 96.3±19.9 months, and from 81.8±23.4 to 82.3±25.4 months, respectively (P>0.05). In the articulation and phonology validation in both groups, speech pitch and energy were not significant, and all the vowel test results showed no other significant values. CONCLUSION: No significant difference was found in the language-speaking ability between the patients; however, the number of vocabularies used decreased. Therefore, topiramate should be used cautiously for children with migraine.
Child* ; Follow-Up Studies ; Humans ; Language Development ; Language Tests ; Migraine Disorders* ; Problem Solving ; Vocabulary

Purpose: We report an atypical case of ischemic oculomotor palsy occurring sequentially in both eyes and then improving, followed by a new abducens palsy in a diabetic patient with poor blood sugar control.Case summary: A 51-year-old woman presented with diplopia, dizziness, and pain in the right eye and was diagnosed with oculomotor palsy in the right eye. Magnetic resonance imaging of her brain was normal but glycated hemoglobin concentration was 13.4%. After 2 months, the third nerve palsy in the right eye had recovered completely but a new third nerve palsy occurred in the left eye. 1 month later, the symptoms had improved, and the patient did not return to our clinic until 18 months later, when she returned with new diplopia. This time, she was diagnosed with a sixth nerve palsy in the left eye and was observed while controlling her blood sugar. She recovered after 4 months. Conclusions Generally, cranial nerve palsies occur unilaterally in situations that can cause ischemia, such as diabetes, high blood pressure, and hyperlipidemia. Rarely, they occur bilaterally, or two or more cranial nerve palsies occur simultaneously. However, it is very rare that cranial nerve palsy occurs sequentially in both eyes under the same systemic blood sugar control. Diabetic patients with poor blood sugar control require long-term monitoring, considering the possibility of symptoms developing in both eyes with a time lag or other forms of ischemic cranial nerve palsy.

PURPOSE: The aim of this study was to compare the clinical characteristics in emergency endotracheal intubation between patients with acute drug intoxication and medical disease. METHODS: Data for airway registry collected in two emergency departments (ED) between April 2006 and March 2010 were reviewed retrospectively. The airway registry data included patient's demographic information and variables such as Cormack-Lehane grade, 3-3-2 finger analysis, success rate, the number of attempts at intubation, complications of intubation, and clinical outcomes after intubation. RESULTS: A total of 1480 patients were enrolled; 62 patients were classified as belonging to the intubation group after the drug intoxication group. No significant differences in Cormack-Lehane grade, 3-3-2 finger analysis, success rate, the number of attempts at intubation, and complications after intubation were observed between patients with acute drug intoxication and medical disease. However, significant difference was observed for indication of emergency endotracheal intubation. While emergency endotracheal intubations were usually performed in medical patients because of failure of airway patency, they were performed in intoxicated patients with the goal of preventing serious complications. CONCLUSION: Anatomical structures related to endotracheal intubation, the process and clinical outcome of intoxicated patients are not significantly different from those for medical patients.
Emergencies* ; Emergency Service, Hospital ; Fingers ; Humans ; Intubation ; Intubation, Intratracheal* ; Poisoning ; Retrospective Studies

Rhadomyosarcoma of the prostate is relatively rare and highly malignant condition. Three major categories of rhabdomyosarcoma exist, which should be kept clearly separated: pleomorphic, embryonal, and alveolar Pleomorphic rhabdomyosarcoma is actually the least common of the three categories. It arises from myotome-derived skeletal muscle and occurs almost exclusively in adults. We make the unequivocal diagnosis of pleomorphic rhabdomyosarcoma only if we can detect cross striations in some of the tumor cells. Herein we experienced a pleomorphic rhabdomyosarcoma of the prostate which is generally not found in the urogenital area and reviewed literature briefly.
Adult* ; Diagnosis ; Humans ; Muscle, Skeletal ; Prostate* ; Rhabdomyosarcoma*