Yonsei University College of Medicine, Seoul, Korea Maffucci's syndrome is characterized by dysccondroplasia with hemangiomata. Since Angelo Maffucci (1881) first described this entity, 105 cases and some more were reported in the literature till 1976. We recently observed a patient suffering from this disorder. The patient is 21 year-old girl, who has multiple hemangiomes on her right foot, right buttock, left wrist and enchondromas on both proximal phalanx, distal phalanx of both great toes and proximal, middle phalanx of right second toe. The disease started at age of 11 and these lesions grew rapidly last 3 years. The diagnosis was based on clinical, radiological and histological findings. Because of its rarity, we report it with brief review of literature.
Buttocks ; Chondroma ; Diagnosis ; Female ; Foot ; Humans ; Korea ; Osteochondrodysplasias ; Seoul ; Toes ; Wrist

To investigate the relationship of the density, thickness of lens nucleus and cortex with phacoemulsification time, we measured the preoperative density and length of lens nucleus and cortex of 100 eyes with a Scheimpflug camera. We performed phacoemulsification on the same phacoemulsification technique using a phacoemulsifier aspirator by the same surgeon. The preoperative mean values of the density of lens nucleus and cortex were 127. 09+/-57.44 and 30. 33+/-15. 24, respectively. The mean values of the thickness for both were 3.05+/-0.42mm and 0.65+/-0.17mm, respectively. The mean value of phacoemulsification time was 92.68+/-56.98 seconds. The phacoemulsification time was increased significantly as the density of lens nucleus was increased (P<0.0001). However, the thickness of the lens nucleus and cortex and the density of cortex had no significant effect on the phacoemulsification time (p=0. 235, p=0.811). Preoperative evaluation with a Scheimpflug camera is particularly valuable for cataractous patients with corneal endothelial disease deciding on phacoemulsification.
Cataract ; Humans ; Phacoemulsification*

Labyrinthitis causes damage to inner ear structure, and in turn hearing loss and vertigo. Labyrinthitis is classified as otogenic labyrinthitis and meningogenic labyrinthitis. Otogenic labyrinthitis can be diagnosed early through otoscopic examination. However, when there are no characteristic neurologic symptoms in patients with meningogenic labyrinthitis, clinicians can overlook the underlying meningitis and this may lead to the peripheral vertigo. We encountered an unusual case of meningogenic labyrinthitis that is misdiagnosed as peripheral dizziness.
Dizziness ; Ear, Inner ; Hearing Loss ; Humans ; Labyrinthitis ; Meningitis ; Meningitis, Bacterial ; Neurologic Manifestations ; Vertigo

OBJECTIVE: Obsessive-compulsive disorder (OCD) is a clinically heterogeneous disorder with a bimodal pattern in age onset and treatment outcomes. This study attempted to ascertain the importance of the age factor for a better phenotypic precision. Therefore, the authors compared adult OCD patients with an early symptom onset to adult OCD patients with a later symptom onset. METHODS: One hundred sixty five patients with OCD were evaluated with semistructured interviews;79 with symptom onset before the age of 17 (early onset group) and 86 with symptom onset after the age of 17 (late onset group). The two groups were analyzed in terms of Y-BOCS (Yale-Brown Obsessive Compulsive Scale) scores and demographic data including clinical variables. RESULTS: Early onset group has more comorbidity of tic disorder and lesser of depression and anxiety disorder than late onset group. Early onset group showed more family history of tic disorder than late onset group. The treatment response to SSRI is relatively declined after 18 months of initiation in early onset group. CONCLUSION: The results indicate that age at onset may be an important factor in subtyping OCD. Early onset group may have more biological and familial tendency that might be differentiate the two groups.
Adult ; Age Factors ; Age of Onset ; Anxiety Disorders ; Comorbidity ; Depression ; Humans ; Obsessive-Compulsive Disorder* ; Tic Disorders

BACKGROUND: Knowledge of size and morphology of the normal trachea is important for airway management and tracheal reconstruction. Conventional radiography is a simple method used to measure the tracheal diameter, but it is not accurate because of the artifacts related to image magnification and overlapping by the shoulder. The purpose of this study was to provide the normal values of the tracheal size and anatomy in Korean adults using Computerized Tomography. MATERIAL AND METHOD: There were 43 men and 34 women included in this study. They were divided into three age groups(group 1, 20-39 years ; group 2, 40-59 yeas ; groups 3,>or=60 years). The anteroposterior and transverse diameters and cross - sectional areas of the trachea were measured at the level of the thoracic inlet(Level 1) and the aortic arch(Level 2). These values obtained at each level were compared between age groups and sexes. RESULT: In 43 men, the anteroposterior / transverse diameters(mean SD in millimeters) of the trachea at levels 1 and 2 were 19.95+/-2.99 / 17.72+/-2.13 and 19.77+/-2.57 / 18.02+/-2.19, respectively. In 34 women, those values at levels 1 and 2 were 15.56+/-2.12 / 14.18+/-2.07 and 15.35+/-1.82 / 15.00+/-1.60, respectively. At both levels, the anteroposterior and transverse diameters were significantly greater in men than in women (p<0.05). The cross-sectional area of the trachea at levels 1 and 2 were 279.14+/-61.37 / 281.93+/-63.97 mm2 in men and 173.29+/-35.81 / 181.88+/-34.74 in women, respectively. They also showed significantly greater values in men than in women(P<0.05). There was no significant difference in diameters and cross-sectional areas of the trachea between age groups. CONCLUSION: There are significant differences in the internal diameter and cross- sectional area of the trachea between men and women in normal Korean adults, while the age difference was insignificant. We believed CT is a relatively accurate and safe way to measure the internal diameter and cross-sectional areas of the trachea.
Adult* ; Airway Management ; Artifacts ; Female ; Humans ; Male ; Radiography ; Reference Values ; Shoulder ; Tomography, X-Ray Computed ; Trachea*

No abstract available.

No abstract available.
Cytomegalovirus ; Hepatitis* ; Rickets*

Acute necrotizing esophagitis (AEN), also called "black esophagus," is a rare disorder with an unknown pathogenesis. Endoscopic findings generally show black pigmentation throughout the esophagus. This case also offered rare views of the gross anatomy of this disorder. Histological examination revealed that the mucosal and submucosal layers of the esophagus were involved in the severe necrotizing inflammation. The chief manifestation of this disease is hematemesis from hemorrhage of the upper gastrointestinal tract with a typically multifactorial etiology. AEN is also characterized by a clear boundary at the gastroesophageal junction where the necrosis stops. In this study, we report an autopsy case of a 61-year-old man with necrotizing inflammation throughout the esophagus and esophageal necrosis from the laryngopharynx to the gastroesophageal junction. The patient was a disabled person with a history of alcohol abuse who was also diagnosed with mild coronary arteriosclerosis and fatty liver on the basis of the underlying diseases. In this case, the main etiology for poor perfusion from the distal esophageal area was likely underlying illness, history of alcoholism, and malnutrition.
Alcoholism ; Autopsy* ; Coronary Artery Disease ; Disabled Persons ; Esophagitis* ; Esophagogastric Junction ; Esophagus ; Fatty Liver ; Hematemesis ; Hemorrhage ; Humans ; Hypopharynx ; Inflammation ; Malnutrition ; Middle Aged ; Necrosis ; Perfusion ; Pigmentation ; Upper Gastrointestinal Tract

No abstract available.
Enterovirus ; Meningitis, Aseptic* ; Seoul*

No abstract available.
Mucocutaneous Lymph Node Syndrome