Frontometaphyseal dysplasia is an uncommon genetic syndrome affecting bone and connective tissue. This condition is characterized by the prominence of supraorbital ridges, hyperostosis of the skull, mandibular hypoplasia and malocclusion with antegonial notching, underdeveloped paranasal flaring of the long bone, and a widened iliac wing. Frontometaphyseal dysplasia has an X-linked dominant trait. Clinical manifestations are more severe in males and have extreme variability in females. We present an 11-year-old boy who had frontometaphyseal dysplasia with disfigured face due to bilaterally urinary tract malformation and chronic urinary tract infection. To improve facial appearance, his prominent supraorbital ridges were contoured by ostectomy and burring through bicoronal incision. Urologic operation was performed simultaneously. Histologically, resected bony specimen revealed bony tissue with normal trabecular pattern suggesting exostotic bone. The patient healed without any postoperative complications and he and his parents were satisfied with his final facial morphology.
Child ; Connective Tissue ; Female ; Humans ; Hyperostosis ; Male ; Malocclusion ; Parents ; Postoperative Complications ; Skull ; Urinary Tract ; Urinary Tract Infections

Restoration of anteroposterior projection of the zygomatic body and facial width are key elements in treatment of complex zygomatic fractures. Traditionally, it has been understood that for most fractures of the zygoma, three-point fixation of the zygomaticofrontal suture, inferior orbital rim, and zygomaticomaxillary buttress generally produces exact structural restoration. Recently, the importance of accurate reduction of the zygomatic arch through coronal incision has been favored in complex zygomatic fractures. Though coronal incision has the advantages of accurate reduction and fixation as a result of extensive exposure of the fractured area, this procedure also has many disadvantages including injury to the facial nerve, paresthesia, alopecia, scar formation, longer operating time, protracted hospitalization, etc. As well, there have been no objective data to prove the fact that four-point fixation through coronal incision is superior to traditional three-point fixation. From May, 1994 to December, 1998, the authors treated 45 patients by traditional three-point (n=20) and coronal four-point fixation (n=25) with random sampling. To assess the difference between the two methods the authors measured the axial angle of the zygoma, the axial angle of the zygomatic arch, and the degree of zygomatic arch inclination on submentovertex X-ray, and then analyzed the measurements by the paired T-test(p < 0.05). As a result, the axial angle of the zygoma and zygomatic arch assessed by anteroposterior projection, as well as the facial width showed no statistical difference between the two groups, respectively(p = 0.26, p = 0.18). Mean while, the degree of zygomatic arch inclination representing the local contour of the fractured zygomatic arch was statistically significant between the two groups(p < 0.05). Thus, the traditional three-point fixation method may be widely acceptable in treatment of complex zygomatic fractures with anterior three-point or zygomatic arch comminution.
Alopecia ; Cicatrix ; Facial Nerve ; Hospitalization ; Humans ; Orbit ; Paresthesia ; Sutures ; Zygoma ; Zygomatic Fractures

No abstract available.
Colonoscopy* ; Humans ; Peritoneal Dialysis, Continuous Ambulatory* ; Peritonitis*

No abstract available.
Diagnosis* ; Growth Hormone* ; Humans*

PURPOSE: A colorectal adenoma (CRA) is a well-defined precursor to colorectal cancer (CRC). Additionally, smoking is a potent risk factor for developing a CRA, as well as CRC. However, the association between exposure to environmental tobacco smoke (ETS) and the risk for developing a CRA has not yet been fully evaluated in epidemiologic studies. We performed a cross-sectional analysis on the association between exposure to ETS at the workplace and the risk for developing a CRA. METHODS: The study was conducted on subjects who had undergone a colonoscopy at a health promotion center from January 2012 to December 2012. After descriptive analyses, overall and subgroup analyses by smoking status were performed by using a multivariate logistic regression. RESULTS: Among the 1,129 participants, 300 (26.6%) were diagnosed as having CRAs. Exposure to ETS was found to be associated with CRAs in all subjects (fully adjusted odds ratio [OR], 1.95; 95% confidence interval [CI], 1.08-2.44; P = 0.001). In the subgroup analysis, exposure to ETS in former smokers increased the risk for developing a CRA (fully adjusted OR, 4.44; 95% CI, 2.07-9.51; P < 0.001). CONCLUSION: Exposure to occupational ETS at the workplace, independent of the other factors, was associated with increased risk for developing a CRA in all subjects and in former smokers. Further retrospective studies with large sample sizes may be necessary to clarify the causal effect of this relationship.
Adenoma* ; Colonoscopy ; Colorectal Neoplasms ; Cross-Sectional Studies* ; Epidemiologic Studies ; Health Promotion ; Logistic Models ; Odds Ratio ; Retrospective Studies ; Risk Factors ; Sample Size ; Smoke* ; Smoking ; Tobacco Smoke Pollution ; Tobacco*

Kawasaki disease or mucocutaneous lymph node syndrome is an acute inflammatory illness of childhood characterized by systemic panvasculitis. It presents with high fever, dramatic changes of the skin and mucous membranes, and lymphadenopathy. Adult-onset Kawasaki disease is rare and reports on coronary involvement in adult are even rarer. Herein, we report a case of adult-onset Kawasaki disease complicated by splenic infarction and development of coronary aneurysm even despite of treatment with intravenous gamma globulin. A 20-year-old man presented with fever, erytheatous rash, induration and desquamation of hands and feet, pulmonary edema and shock due to cardiomyopathy, splenic infarction, bilateral conjunctivitis, jaundice, and cervical lymphadenopathy. After Kawasaki disease was suspected, intravenous gamma globulin (2 g/kg once) and aspirin (6 g/day) were administered. On the 30th hospital day, transesophageal echocardiography showed one coronary aneurysm and coronary angiography showed three aneurysms. Eight months after the first admission, follow-up coronary angiography showed normalization of the previous coronary abnormalities.
Adult ; Aneurysm ; Aspirin ; Cardiomyopathies ; Conjunctivitis ; Coronary Aneurysm* ; Coronary Angiography ; Echocardiography, Transesophageal ; Exanthema ; Fever ; Follow-Up Studies ; Foot ; gamma-Globulins ; Hand ; Humans ; Jaundice ; Lymphatic Diseases ; Mucocutaneous Lymph Node Syndrome ; Mucous Membrane ; Pulmonary Edema ; Shock ; Skin ; Splenic Infarction* ; Young Adult

Xanthogranulnmatous pyelonephritis is an unusual chronic renal infection associated with renal calculi, urinary tract infection or obstruction in many cases. It is characterized by orange-yellow nodules of inflamed parenchymal tissue macroscopically and foamy lipid-laden histiocyte microscopically. We report a case of Xanthogranulomatous pyelonephritis in a 74 years old male.
Aged ; Histiocytes ; Humans ; Kidney Calculi ; Male ; Pyelonephritis ; Pyelonephritis, Xanthogranulomatous* ; Urinary Tract Infections

A case of 18-Trisomy mosaicism in a male newborn infant who was born to a mother of 27 years old at Cheil Hospital was presented. This baby had grossly multiple anomalies that were characterized by prominatn occiput, microhpthalmia, palpebral fissure, micrognathia, high arched palate, low set ears, undescended testes, limitation of abduction of hip joint, syndactylism of finger, and overlapping of finger. We have been confirmedby chromosome culture and his mother chromosomal analysis revealed sex chromosomal mosaicism (45xo/46xxx). This is the first case report of 18-Trisomy mosaicism with parental mosaicism in Korea. A brief review of related literature is also pressented.
Adult ; Cryptorchidism ; Ear ; Fingers ; Hip Joint ; Humans ; Infant, Newborn ; Korea ; Male ; Mosaicism* ; Mothers ; Palate ; Parents*

PURPOSE: Extensive midface defect following total maxillectomy with orbital exenteration and cheek skin resection should be three dimensionally reconstructed with a large flap that have a sufficient volume of tissue and multiple skin islands. We describe our transverse rectus abdominis myocutaneous(TRAM) free flap with three skin islands which was successfully used in this situation. METHODS: A 58-year-old man was performed enbloc total maxillectomy including orbital contents and wide cheek skin because of invasive maxillary squamous cell carcinoma. He was immediately reconstructed with TRAM flap that was designed not vertical but transverse fashion for providing sufficient skin area. Also, deepithelialization procedure making for multiple skin islands was done in flap insetting period when appropriate modification according to the intraoperative situation was possible. Dead space was completely obliterated by bulky muscular tissue, and three skin islands were used for lining of lateral nasal wall, palatal surface, and cheek skin restoration. RESULTS: Postoperative course was satisfying. Maintaining of proper ipsilateral nasal airway, loss of rhinolalia and oronasal regurgitation of food particles, and restoration of cheek contour were successfully obtained. CONCLUSION: We report clinical experience of threedimensional reconstruction using free TRAM flap after total maxillectomy with orbital exenteration.
Carcinoma, Squamous Cell ; Cheek ; Free Tissue Flaps ; Humans ; Islands ; Middle Aged ; Orbit ; Rectus Abdominis ; Skin ; Speech Disorders

Hyper-activated depressor septi nasi is an important factor caused a nasal tip drooping and many studies have been carried out, however it still remains controversial. This study presents a surgical modality for its intervention, based on the anatomical study. Eleven fixed and six fresh cadavers were used for the study. We found that the depressor septi nasi was consisted of three fascicles. Medial fascicles were inserted into the dermocartilaginous ligament, and deep fibers of the medial fascicles were attached to the anterior nasal spine. After superficial fibers were interdigitated with the orbicularis oris, they were attached to the alveolar bone. Intermedial fascicles were inserted to the footplates of the medial cruses and the caudal septum. After they were interdigitated with the medial fascicle and the orbicularis oris, they were attached to the alveolar bone. From April to August 2008, five patients had surgical intervention for hyper-activated depressor septi nasi. Medial and intermedial fascicles were detached completely from the anterior nasal spine and the septum through intranasal approach. Tip droopings were improved in all cases. Specific complications were not found. We believe that surgical intervention through intranasal approach is a useful method for correction of tip drooping.