A 13 years old boy who had typical triad of tuberous sclerosis-adenoma cebaceum, mental deficiency, and epileptic seizure-was presented. He also had some white patches on his right buttock and shagreen patches on his right thigh. The finding of histopathologic biopsy was compatible with an angiofibromatosis. The pedigree of his family was investigated and seemed to be a sporadic.
Adolescent ; Biopsy ; Buttocks ; Humans ; Intellectual Disability ; Male ; Pedigree ; Thigh ; Tuberous Sclerosis*

Vitamin D deficiency rickets is a metabolic disorder of growing bone, which is still important nutritional problem in developing countries. Recently we had expirienced 2 cases of typical vitamin D deficiency rickets, who were fed with only thin rice gruels and not fed any nutrient diets, breast milk, vitamin D supplements and almostly not to be exposured to sunlight for a long period of time due to poor socio-economic condition. Treatments were consisted of 600,000 units of vitamin D injection, high protein, high calorie diets and frequent exposures to sunlight. All of two were improved in clinical and laboratory findings after above therapy.
Developing Countries ; Diet ; Milk, Human ; Rickets ; Sunlight ; Vitamin D Deficiency* ; Vitamin D* ; Vitamins*

The Shikani Optical Stylet(TM) (SOS, Clarus Medical, USA) is another tool to facilitate tracheal intubation. It combines the benefits of a lightwand and a fiberoptic bronchoscope. We report the application of SOS in facilitating the tracheal intubation of two-person with history of difficult airway management. A 25-year-old woman with micrognathia was scheduled to undergo an exploratory laparotomy. Intubation attemps failed with a direct laryngoscope because of difficulty in her mouth opening. Although airway management was re-attempted by a laryngeal mask airway, it also failed for the same reason. Airway management was successfully performed using the SOS instead of a laryngeal mask airway. The second case was a 38-year-old woman with ankylosing spondylitis, scheduled for spinal fusion. She had difficulty in extending her neck. Intubation was successfully performed via the SOS. We believe that intubation by the SOS is a useful and readily available alternative technique for patients with difficult airways.
Adult ; Airway Management ; Bronchoscopes ; Female ; Humans ; Intubation ; Laparotomy ; Laryngeal Masks ; Laryngoscopes ; Mouth ; Neck ; Spinal Fusion ; Spondylitis, Ankylosing

The surgical methods of injured peripheral nerve were limited to end-to-end neurorrhaphy, nerve graft, neurotization, etc. Recently, Several studies were executed about end-to-side neurorrhaphy in peripheral nerve injury. The purpose of this study is to investigate the axonal regeneration of end-to-side neurorrhaphy in rats, as alternative surgical method for peripheral nerve injury comparing with the state of normal, denervated, and end-to-end neurorrhaphy. Sixty female Sprague-Dawley rats were divided into four groups; group I as normal control group, group II as denervated control group, group III as end-to-end neurorrhaphy group, group IV as end-to-side neurorrhaphy group. At postoperative 4, 8, 12, 16, 20 and 24 week, nerve regeneration was assessed through electrophysiologic and histological studies. The results obtained were as follows: 1. In electrophysiologic test, the mean amplitude was higher in normal control group(group I) than either in end-to-end neurorrhaphy group(group III) or in end- to- side neurorrhaphy group(group IV)(p < 0.05). But there is no significant difference between group III and group IV. 2. The mean number of regenerating myelinated nerve fibers was higher in group I than either in group III or in group IV(p < 0.05). But there is no significant difference between group III and group IV, except at postoperative 16 week. 3. The mean number of motor end-plates at postoperative 24 week was 20.5 in group III and 18.2 in group IV, but there is no significant difference between group III and group IV. In conclusion, end-to-side neurorrhaphy through an epineural window could induce distal nerve regeneration by collateral sprouting of main peripheral nerve and positively reflected in functional improvement of the target muscle.
Animals ; Axons ; Female ; Humans ; Motor Endplate ; Nerve Fibers, Myelinated ; Nerve Regeneration* ; Nerve Transfer ; Peripheral Nerve Injuries ; Peripheral Nerves ; Rats* ; Rats, Sprague-Dawley ; Regeneration ; Transplants

Cutaneous metastases rarely develop in patients with internal malignancy. Cholangiocarcinoma, a malignant cancer of the bile duct, is a relatively rare adenocarcinoma and has a poor prognosis. Few reports have mentioned cutaneous metastases of cholangiocarcinoma, and the most of them were due to direct tumor seeding by percutaneous procedures. Herein, we report a case of cholangiocarcinoma with distant cutaneous metastases in a 60-year-old man.
Bile Duct Neoplasms/*diagnosis/pathology ; *Bile Ducts, Intrahepatic ; Cholangiocarcinoma/*diagnosis/pathology/secondary ; Fluorodeoxyglucose F18/diagnostic use ; Humans ; Male ; Middle Aged ; Positron-Emission Tomography ; Skin Neoplasms/*diagnosis/pathology/secondary ; Tomography, X-Ray Computed

Eosinophilic gastroenteritis is a rare, benign condition, characterized by various gastrointestinal symptoms associated with eosinophilic infiltration of the wall of the any part of the digestive tract, most commonly the stomach and small intestine. Eosinophilic gastroenteritis is generally classified according to the involved layer of the gastrointestinal tract. Serosal type is the rarest form of eosinophilic gastroenteritis that is characteristically accompanied with eosinophilic ascites and responds well to steroid treatment. We have experienced a typical case of serosal type eosinophilic gastroenteritis in a women who complained of abdominal pain. She had peripheral eosinophilia, gastric and small bowel wall thickening with eosionophilic ascites. Her symptom relieved rapidly after starting corticosteroid treatment and she had long been in clinical remission after discontinuation of corticosteroid administration.
Abdominal Pain ; Ascites ; Eosinophilia ; Eosinophils* ; Female ; Gastroenteritis* ; Gastrointestinal Tract ; Humans ; Intestine, Small ; Stomach

BACKGROUND: The greater occipital nerve (GON) block has been frequently used for different types of headache, but performed with rough estimates of anatomic landmarks. Our study presents the values of the anatomic parameters and estimates the effectiveness of the ultrasound-guided GON blockade. METHODS: The GON was detected using ultrasound technique and distance from external occipital protuberance (EOP) to GON, from GON to occipital artery and depth from skin to GON was measured in volunteers. Patients with occipital headache were divided into two groups (ultrasound-guided block: group S, conventional blind block: group B) and GON block was performed. The same parameters were measured on group S and VAS scores were assessed at pretreatment, 1 week and 4 weeks after treatment on both groups. RESULTS: The GON had distance of 23.1 +/- 3.4 mm (right) and 20.5 +/- 2.8 mm (left) from EOP to GON. Its depth below the skin was 6.8 +/- 1.5 mm (right) and 7.0 +/- 1.3 mm (left). The distance from GON to occipital artery was 1.5 +/- 0.6 mm (right) and 1.2 +/- 0.6 mm (left) in volunteers. Initial VAS score of group S and group B patients were 6.4 +/- 0.2 and 6.5 +/- 0.2. VAS score of 4 weeks after injection were 2.3 +/- 0.2 on group S and 3.8 +/- 0.3 on group B (P = 0.0003). CONCLUSIONS: The parameters measured in this study should be useful for GON block and ultrasound-guided blockade is likely to be a more effective technique than blind blockade in occipital headache treatment.
Anatomic Landmarks ; Arteries ; Follow-Up Studies ; Headache ; Humans ; Nerve Block ; Skin

Peutz-Jeghers syndrome is a rare autosomal dominant disorder defined by hamartomatous polyps of gastrointestinal tracts and the occurrence of melanin spots on the mucocutaneous surface. This syndrome has important complications such as malignant transformation, gastrointestinal bleeding, and intussusception. About 50% of patients with Peutz-Jeghers syndrome involved Intussusception. A case of Peutz-Jeghers syndrome with intussusception was recently experienced and herein reported.
Gastrointestinal Tract ; Hemorrhage ; Humans ; Intussusception* ; Melanins ; Peutz-Jeghers Syndrome* ; Polyps

A case with carcinoid tumor of the appendix was encountered incidentally during an elective abdominal surgery for borderline mucinous ovarian tumor in a 16-year old female. Carcinoid tumor of appendix is the most common neoplasm of the gastrointestinal tract in childhood and adolesence. They are usually regarded as a benign neoplasm and an uncommon source of metastasis and recurrence. We report this case with reference to the value of grosss examination of the appendix and incidental appendectomy during gynecologic abdominal operations.
Adolescent ; Appendectomy ; Appendix* ; Carcinoid Tumor* ; Female ; Gastrointestinal Tract ; Humans ; Mucins* ; Neoplasm Metastasis ; Recurrence

Neurilemmomas are relatively uncommon, slowly growing tumors which originate from Schwann cells. Intrathoracic neurilemmomas often occur in the chest wall and posterior mediastinum, but endobronchial neurilemmomas are exceedingly rare. These tumors in trachea or bronchus are usually detected by radiologic examinations, mostly computed tomography scan of chest. An 88-year-old man was admitted for management of pneumonia in left lower lobe and parapneumonic effusion. On bronchoscopic examination, there was a small polypoid nodule less than 1 cm in diameter mimicking an endobronchial inflammatory polyp at the bifurcation of the right anterior segmental bronchus and lateral segmental bronchus and under auto-fluorescence imaging, the nodule showed reddish brown area with defined margin. The bronchoscopic biopsy revealed that the bronchial nodule was endobronchial neurilemmoma. This is an interesting case of endobronchial neurilemmoma mimicking a bronchial polyp that is detected incidentally via bronchoscopy.
Aged, 80 and over ; Biopsy ; Bronchi ; Bronchoscopy ; Humans ; Mediastinum ; Neurilemmoma* ; Optical Imaging ; Pneumonia ; Polyps* ; Schwann Cells ; Thoracic Wall ; Thorax ; Trachea