No abstract available.
Allografts* ; Anemia, Hemolytic* ; Cadaver* ; Transplantation*

Aneurysm of the internal carotid artery is a rare disease and is known to be associated with congenital arterial anomalies such as neurofibromatosis type I (NF-I). NF-I is an autosomal dominant neurocutaneous disorder characterized by a variety of manifestations that involve the central and peripheral nervous systems, skin, vascular system, and skeleton. In particular, the involvement of vascular abnormalities in NF-I is well known. Any vessel may be affected by this condition, although the renal artery is most frequently involved. The vascular abnormality can be occlusive or an aneurysmal degenerative change. Therefore, symptomatic presentations might assume an indolent pathophysiologic course such as hypertension, or manifest as a catastrophic event such as arterial rupture that could result in sudden death. We report a rare autopsy case of an aneurysmal rupture of the internal carotid artery in a woman with suspected NF-I, who collapsed in her home.
Aneurysm ; Autopsy ; Carotid Artery, Internal ; Death, Sudden ; Female ; Glycosaminoglycans ; Humans ; Hypertension ; Neurocutaneous Syndromes ; Neurofibromatoses ; Neurofibromatosis 1 ; Peripheral Nervous System ; Rare Diseases ; Renal Artery ; Rupture ; Skeleton ; Skin

Many authors reported the etiology of hypoplasia of the nasomaxillary complex as trauma, infection, underdevelopment. To correct these deformities, Le Fort II Osteotomy and its modification has been popularly applied. This method enabled total advancement of nasomaxillary complexes and acquirememt of midfacial esthetics. But it has some limitations such as various occlusal deviation or lateral shifting of nasomaxillary complex in case of nasomaxillary retrusion. We grouped these patients as follows : 1. Nasomaxillary retrusion without shifting of nasomaxillary complex (1) Anteroposterior deviation of occlusal plane (2) Lateral deviation of occlusal plane(including canting) (3) Supero-inferior deviation of occlusal plane (4) Combined disturbance of occlusal plane without shifting of nasamaxillary complex 2. Lateral shifting of nasomaxillary complex with or without deviation of occlusal plane We performed Le Fort II and I combined osteotomy on eleven cases of midfacial deformity from June 1994 to July 1997 and in most of the cases, followed up maximum 36 months and could acquire positional stability and improvement of facial eathetics.
Congenital Abnormalities* ; Dental Occlusion ; Esthetics ; Humans ; Osteotomy*

A female fetus with short rib(polydactyly) syndrome, Beemer-Langer type was terminated at 28 week's gestation because of abnormalities visualized on sonography. We present the clinical, radiological and autopsy findings of this case with a brief review of the literatures.
Autopsy ; Female ; Fetus ; Humans ; Polydactyly* ; Pregnancy ; Ribs*

Blue rubber bleb nevus syndrome is a rare disease of cutaneous hemangioma of a variant of the carvenous type which is clinically characterized by multiple, protuberant, dark blue, generally soft, rubbery, and compressible cutaneous masses usually associated with similar lesions of the gastrointestinal tract. Most cases are sporadic, although autosomal dominant inheritance has been described. We report herein a cases of blue rubber bleb nevus syndrome in a 49-year-old, who had multiple, dark blue, slightly tender, easily compressible vascular tumors on the face, right postauricular area, tongue, neck, and right had and foot, measuring from 0.5 to 1.0 cm in diameter. Endoscopic examination also revealed several similar lesions on the upper third of the esophagus. Biopsy specimen from the lesion of the neck showed irregularly dilated vessels containing red blood cells and fibrinous material. The vascular space was lined by a single layer of flattened endothelial cells.
Biopsy ; Blister* ; Endothelial Cells ; Erythrocytes ; Esophagus ; Fibrin ; Foot ; Gastrointestinal Tract ; Hemangioma ; Humans ; Middle Aged ; Neck ; Nevus* ; Rare Diseases ; Rubber* ; Tongue ; Wills

Weber-Christian disease is an inflammatory disease of subcutneious fat tissue which is clinically characterized by relapsing, febrile, nodular, nonsuppurative pannicutilitiy. The cause is unknown, but the disease has been attributed to an autoimmune reaction, infectioris, halogen compounds, sarcoidosis, SLE and a-antitrypsin deficiency. We report here in a case of Weber-Christian disease in a 8-year-old girl, who had indurated erythemataus subcutaneous nodules on the extremities, buttock and trunk accompanied by various general symptoms such as fever, malaise, arthralgia and edema of the abclorren and legs. This case was successfully treated with dapsone,
Arthralgia ; Buttocks ; Child* ; Dapsone ; Edema ; Extremities ; Female ; Fever ; Humans ; Leg ; Panniculitis, Nodular Nonsuppurative* ; Sarcoidosis

Complications after a median sternotomy incision, which is used currently in most open heart surgery, are serious, although it is infrequent. Reconstruction of the sternal defect resulting from dehiscence of median sternotomy is still big challenge to the most plastic surgeons. Since vascularized greater omentum was transposed to eliminate mediastinal wound problems, many vascularized regional muscle flaps became mainstay in reconstruction of median sternotomy wound. We treated 13 patients with median sternotomy dehiscence between October of 1993 and March of 1998. In two patients, the wound problems were so confined to superficial tissue that debrided and closed primarily. Eleven patients with deep wound infection were managed with vigorous debridement of all necrotic tissues and resultant defects were covered with regional muscle flaps: rectus myocutaneous flap(3) and bilateral pectoralis advancement flap(8). We used the pectoralis major advancement flaps without counter incision at humeral insertion site and the dissections were limited only medial to the anterior axillary line to preserve the axillary fold. In five patients with larger defects, we elevated muscle and cutaneous flaps separately to make these flaps more mobile. Large portion of two rectus abdominis flaps could not survive, whereas pectoralis advancement flaps had mo special wound problems. Only one patient developed fistula due to remained wire, regardless to flap surgery.
Debridement ; Fistula ; Humans ; Omentum ; Rectus Abdominis ; Sternotomy* ; Thoracic Surgery ; Wound Infection ; Wounds and Injuries

We report a case of congenital candidiasis confined to the rail plates in a 57-day-old girl whose infected nails exhibited the rough, yellow-white, thickened, opaque distal ends and pink-colored, normal healthy plates at their proximal bases. Numerous pseudohyphae and spores were demonstrated by KOH microscopic examination. Candida albicans was identified by culture on Sabourauds glucose agar and API 20C Aux system. Histopathologic findings of nail crippings also showed mutiple hyphae beneath the hyperkeratot,ic nail plate on PAS stain. Shedding of the infected distal portion of the ungual plates ws caused by growing out of the healthy nails, resulting in spontaneous healing of the disease rithin 2 months.
Agar ; Candida albicans ; Candidiasis* ; Female ; Glucose ; Humans ; Hyphae ; Spores

We report a case of infantile acute hemorrhagic edema in a 15-month-old boy. The patient showed multiple, bean to walnut-sized, red to purple-colored, indurated purpura with a cockade pattern on the face, earlobes, and extremities. Edema of the hands, feet, and earlobes was also observed. Laboratory examinations showed thrombocytosis, decreased serum CH50, and ketone in the urine. A skin biopsy taken from indurated purpura on the lower leg exhibited findings of acute leukocytoclastic vasculitis. Direct immunofluorescence examination showed deposition of IgM along the dermo-epidermal junction and C3 in the dermal vessel walls. With-out specific management except for fluid therapy to correct dehydration, the skin lesions cleared completely in 2 weeks, and recurrence has not observed.
Biopsy ; Dehydration ; Edema* ; Extremities ; Fluid Therapy ; Fluorescent Antibody Technique, Direct ; Foot ; Hand ; Humans ; Immunoglobulin M ; Infant ; Leg ; Male ; Purpura ; Recurrence ; Skin ; Thrombocytosis ; Vasculitis

Chronic lymphedema of the penis and scrotum is a rare disease in Korea. Lymphedema can be defined as an accumulation of excessive lymph, proteins, chronic inflammation, edema and fibrosis, secondary to the impairment of the lymph vessels, which can be classified into primary, which is the result of inadequate drainage of lymph caused by agenesis and hypogenesis of lymph vessels, and secondary, which could be due to an obstruction or interruption of the lymphatic system, caused by malignancies, post-operative, post-radiation fibrosis, infection, trauma or excision. The treatment method for a lymphedema of the genitalia is determined by cause, which is crucial to the natural course of the disease. In contrast to a primary lymphedema without soft tissue changes and injury, which could be treated conservatively, surgical excision is the best method to treat a chronic lymphedema with soft tissue changes. In this article, a case of penile and scrotal chronic lymphedema of unknown cause, treated with surgical methods, is reported.
Drainage ; Edema ; Fibrosis ; Genitalia ; Inflammation ; Korea ; Lymphatic System ; Lymphedema* ; Male ; Penis* ; Rare Diseases ; Scrotum* ; Surgical Procedures, Operative