No abstract available.
Neurofibromatoses*

A case of porphyria cutanca tarda is reported, in which the photosensitive cutaneous symptoms developed in association with impaired hepatic function possibly related to chronic consumption of alcohol. Wood's light examination of the urine revealed coral red fluorescence, and qualitative chemical urinalysis was strong positive for uroporphyrin and coproporphyrin. Biopsy of the skin showed subepidermal bulla with festooning arrangement of the naked papillae and basophilic degeneration of the dermis. The patient's favorable response to alkalinization therapy by sodium bicarbonate per os is encouraging, even for a short period's observation. Clinical, histopathological, and biochemical features of this disease are also discussed.
Anthozoa ; Basophils ; Biopsy ; Dermis ; Fluorescence ; Porphyrias ; Skin ; Sodium Bicarbonate ; Urinalysis

Two cases of tuberous sclerosis are reported. Case 1: A 13 years-old girl had a triad of adenoma sebaceum, mental deficiency and epileptic seizures associated with ichthyosis vulgaris, but family history revealed no evidences of the disease except ichthyosis in her mother. Case 2 : A 21 year-old woman had a adenoma sebaceum, shagreen patch, periungual fibroma and fihromatous plaque, showing intracranial calcificatins on roentgenographic examination, but has not demonstrated any neurological manifestations as yet. Family history also revealed adenoma sebaceum in her father and two younger sisters. We feel this case is a "forme fruste" of tuberous sclerosis.
Adolescent ; Epilepsy ; Fathers ; Female ; Fibroma ; Humans ; Ichthyosis ; Ichthyosis Vulgaris ; Intellectual Disability ; Korea* ; Mothers ; Neurologic Manifestations ; Siblings ; Tuberous Sclerosis* ; Young Adult

A case of dermatitis herpetiformis assaciated with erythema annulare centrifugum is reported. The patient was a 21 year-old male, who had papulovesieles, erythematous lesions, pigmentation, and erythema annulare centrifugum lesions on the trunk, shoulders, buttocks and extensor surfaces of the extremities, and severe itching sensation of 9 months' duration. The skin biopsy from the vesicular and erythema annulare centrifugum lesion showed subepidermal bulla and vesicles with neutrophil and eosinophil infiltrations.
Biopsy ; Buttocks ; Dermatitis Herpetiformis* ; Dermatitis* ; Eosinophils ; Erythema* ; Extremities ; Humans ; Male ; Neutrophils ; Pigmentation ; Pruritus ; Sensation ; Shoulder ; Skin ; Young Adult

Recently posterior stabilization with various instrumentation and fusion has been used for fractures of the thoracolumbar spine involving anterior and middle columns. However, these methods are sometimes inadequate to gain anatomical reduction and complete decompression especially in burst fractures. So anterior decompression and interbody fusion were frequently added as a second operation. Biomechanically a rigid anterior spinal instrument such as kaneda device can provide enough anterior decompression through partial or total corpectomy and adequate correction of kyphosis as an one stage operation. We have experienced 27 cases of Kaneda instrumentation via anterior approach for thoracolumbar fracture, most of them were burst fractures, from Jan. 1989 to June 1990. Of there 27 cases, 12 were followed up at least 1 year and reviewed. The results were as followed: l. Anterior spinal approach using Kaneda instrumentation provided sufficient anterior neural decompression and adequate correction of kyphotic deformity. It eliminates the second posterior procedure in most cases and enhances early solid union. Simultaneously, it allows early mobilization of patient with an application of brace. 2. Post-operative courses were eventful in most cases except a case of pathological fracture. 3. This procedure seemed to be one of the most suitable method for the treatment of burst fractures of the thoracolumber region. 4. Complications by Kaneda device itself were not developed in the early evaluation stage. However, on longer follow-up study over 1 year revealed some complications including 2 cases of screw breakage and one lateral wedging deformity.
Braces ; Congenital Abnormalities ; Decompression ; Early Ambulation ; Follow-Up Studies ; Fractures, Spontaneous ; Humans ; Kyphosis ; Methods ; Spine

No abstract available.
Fractures, Spontaneous* ; Prostheses and Implants*

No abstract available.
Spine*

No abstract available.

Since the procedure of a full thickness vaginal wall sling for stress urinary incontinence was first described, concern has been raised regarding the potential formation of inclusion cysts due to buried vaginal mucosa. Recently, two female patients, who underwent an in-situ vaginal wall sling procedure, visited our department presenting with painless large anterior vaginal wall masses. The masses were cystic, and cyst excisions performed. The cyst fluid was aseptic and the cyst wall lined with stratified squamous epithelium. At the 1 year follow up, the patient remained continent and the cyst had not reformed. To our knowledge, this is the first reported case of inclusion cyst formation after an in-situ vaginal wall sling procedure in Korea. An epithelial inclusion cyst is an under-recognized complication of the in-situ advancing vaginal wall sling. The vaginal surgeon should be aware of the potential for an epithelial inclusion cyst and actively search for their formation at the postoperative examination following an in-situ sling placement.
Cyst Fluid ; Epithelium ; Female ; Follow-Up Studies ; Humans ; Korea ; Mucous Membrane ; Urinary Incontinence ; Urinary Incontinence, Stress ; Vagina

Leiomyosarcoma of the rectum is a extremely rare disease without well documented report on its management and prognosis. The most complicated problem lies on the correct diagnosis. Many pathologic and histologic criteria have been proposed to make it clear. The treatment of rectal leiomyosarcoma is controversial. Some authors recommand wide local excison for low-grade tumors as much as 2 cm in diameter. However, radical abdominoperineal resection is the procedure of choice. Leiomyosarcoma of the rectum is resistant to radiotherapy, and no single effective chemotherapeutic drug has been found yet, although adriamycin is effective in one third of all cases. The local recurrence rate was much higher in patients receiving wide local excision and the overall 5-year or 10-year survival rate is similar. We report a case of rectal leiomyosarcoma and review the literature.
Diagnosis ; Doxorubicin ; Humans ; Leiomyosarcoma* ; Prognosis ; Radiotherapy ; Rare Diseases ; Rectum* ; Recurrence ; Survival Rate