Mayer-Rokitansky-Kuster-Hauser Syndrom is characterized by the absence of the vagina and the uterus, the presence of apparently normal tubes and ovaries, feminine appearance, normal female secondary sexual characteristics, a normal 46, XX karyotypes, and a feminine psychosexual orientation. Absence of the vagina results from an embryological arrest in the development of the lower portion of the Mullerian system. Various methods of surgical treatment for the vaginal absence in this syndrom have been introduced but the ideal method to restore the original dimension and function of the normal vagina was not found. The two cases reviewed in this paper were all treated with modified McIndoe operation using full thickness skin grafts. Postoperatively both women were satisfied without complications, i,e. lack of skin graft, bleeding, urethrovaginal fistula, perforation of the rectum, rectovaginal fistula and significant vaginal stricture. This paper reveals the satisfactory results that were uniformly good.
Constriction, Pathologic ; Female ; Fistula ; Hemorrhage ; Humans ; Karyotype ; Ovary ; Rectovaginal Fistula ; Rectum ; Skin* ; Transplants* ; Uterus ; Vagina

Occult syringoma means a clinically inapparent tumor of the swea gland that are histologically similar but not identical with the common syringoma. They may occur in a variety of inflammatory skin lesions and cutaneous neoplaams as a reactive process. We report herein a case of diffuse alopecia areata associated with occult syringoma in the scalp of a 57-year-old man. Our patient developed progressive, fairly well circ amscribed patches of alopecia and diffuse hair loss over a 1 month period. Histopathological examinaiion revealed non-scaring alopecia and a proliferation of eccrine ductal structures in the upper dermis. These ductal structures resembled syringoma. Only minimal focal fibrosis was occasionally observed arcund the hair follicles. We believe that occult syringona is a reactive process and is secondary to active inflarnmation or dermal fibrosis. It has been most commonly observed in scarring alobiecias but plays no significant role in the etiology of hair loss.
Alopecia Areata ; Alopecia* ; Cicatrix ; Dermis ; Fibrosis ; Hair ; Hair Follicle ; Humans ; Middle Aged ; Scalp ; Skin ; Syringoma*

To determine the types of metabolic a cidosis using anion gap in acute infantile diarrhea and to correlate it with clinical outcome, we examined 103 infants admitted with acute diarrhea. The serum electrolytes (sodium, potassium, chloride, phosphorus), creatinine, CO2 content and anion gap were measued on first admission day. They were classified group A with normal anion gap (8~16mEq/L) and group B with increased anion gap (>16mEq/L). The results were as follows. 1) The number of group A with normal anion gap (11.6+/-3.3mEq/L) was 62 and the number of group B with incresed anion gap(21.1+/-5.5mEq/L) was 38. 2) The duration of diarrhea was significantly prolonged in group B (9.0+/-2.5 days), compared with group A (5.9+/-1.1 days)(p<0.001). 3) The duration of admission was significantly prolonged in group B(5.6+/-2.2 days), compared with group A (4.+/-01.4days)(p<0.001). 4) Infants in group B, compared with group A, were significantly more severe dehydrated (p.
Acid-Base Equilibrium ; Acidosis* ; Creatinine ; Diarrhea ; Diarrhea, Infantile* ; Electrolytes ; Humans ; Infant ; Potassium

Subcutaneous angiolymphoid hyperplasia is an uncommon disease characterized by eosinophilia, proliferation of the capillary vessels and lymphoid tissue and infiltration of the inflammatory cell, especially eosinophils. The etiology of the disease are obscure, but probably trauma, lower grade infection, nervous factor & hormonal status etc. The predilection sites are face, ears, scalf & neck, but rarely reported in the extremities. We experienced a case of subcutaneous angiolymphoid hyperplasia of the inguinal region and anteromedial side of the proximal thigh in a 10 year-old man who complained pain and tenderness on the above region. The patient was treated by excision with satisfactory result.
Capillaries ; Ear ; Eosinophilia ; Eosinophils ; Extremities ; Humans ; Hyperplasia ; Lymphoid Tissue ; Neck ; Thigh

Verrucous hemangioma is a variety of capillary, cavernous or mixed hemangioma that can develop into acanthosis, hyperkeratosis, and papillomatosis. Most verrucous hemangiomas appear on the lower extremities, and are present at birth or appear during infancy. It is important, from a prognostic and therapeutic point of view, to make a correct diagnosis between verrucous hemangioma and angiokeratoma circumscriptum. The former needs a large and deep excision, while the latter responds to the common means of physical therapy. We observed two patients who showed typical clinical and histopathological findings of verrucous hemangioma. Treatment was by wide sugical excision and skin graft. The two patients responded positively to this tratment.
Angiokeratoma ; Capillaries ; Diagnosis ; Hemangioma* ; Humans ; Lower Extremity ; Papilloma ; Parturition ; Skin ; Transplants

We report a case of cutaneous calcinosis following extravasation of calcium calcium chloride in a 3-week old neonate, After extravasation of 3% calcium chloride infusion, rapid and marked swelling, erythema, and signs of soft tissue necrosis developed over the extensor surface of both upper extremities. Through the small channels of the destroyed epidermis, tiny white pieces of calcific masses were eliminated spontaneously. Histologically the calcium deposits were found as amorphous masses in the upper dermis and the discharge of calcium deposits also took place by means of transepidermal elimination. The central bone formation and surrounding calcium deposition were present along with dense lymphohistiocytic cell infiltration in the deep dermis. Complications of extravasation of calcium solution during intravenous infusion included localized calcification and, ocasionally necrosis. The incidence of compllcations was about eight percent. This is a case which again emphasises of the danger of using calcium chloride intravenously in the neonate or young infant.
Calcinosis* ; Calcium Chloride* ; Calcium* ; Dermis ; Epidermis ; Erythema ; Humans ; Incidence ; Infant ; Infant, Newborn ; Infusions, Intravenous ; Necrosis ; Osteogenesis ; Upper Extremity

Schonlein-Henoch purpura is a generalized small vessel vasculitis characterized by nonthrombocytopenic purpura, arthritis, abdominal pain and nephritis. In 1914 Osler described an allergic purpura associated with hemiplegia. After then Lewis et al. reported the cases of Schonlein-Henoch purpura associated with convulsion, coma, confusion, intracranial hemorrhage, and chorea, CNS complication has been reported in 1-8% of children and subsided spontaneously in most cases. Headache is a remarkable sympton and appears nonspecific nature. In 1991 Ostergaard and Storm reported that headache occured during the first week following skin rash and frequently showed abnormal EEG findings. We investigated prospectively the presence of a possible cerebral and renal involvement in the case of Schonlein-Henoch purpura. EEG abnormality demonstrated in 52.6% of all cases, and headache or irritability in 47.4% of all cases. A significant association was found between abnormal EEG finding and presence of headache, but was not found between EEG findings and presence of renal involvement and hypertension. Patients with abnormal EEG had no Past or famity history of febrile convulsion or ididopathic epilepsy.
Abdominal Pain ; Arthritis ; Child ; Chorea ; Coma ; Electroencephalography* ; Epilepsy ; Exanthema ; Headache ; Hemiplegia ; Humans ; Hypertension ; Intracranial Hemorrhages ; Nephritis ; Prospective Studies ; Purpura, Schoenlein-Henoch* ; Seizures ; Seizures, Febrile ; Vasculitis

We present a case of systemic lupus erythematosus(SLE) asseci ted with vitiligo in a 57 year-old male patient. Physical examination revealed two distinct skin lesions those of itiligo showed as centrally located depigmented patches surrounded by erythernatous patch on the right cheek, scalp and left dorsurn of hand and those of SLE showed as erythematous papules and patchs with partial adherent scales on the face, scalp, neck, both extensor surface of arm, and left dorsun of hand. Their coexistence lends credence to the contention that altered autoimrnunity may play a role in the pathogenesis of each of these diseases. In Western countries and Japen, patients with lupus erythema osis have been observed to develop vitiligo or depigmented spots on the skin, while in Korea, its has not been previously observed.
Arm ; Cheek ; Erythema ; Hand ; Humans ; Korea ; Lupus Erythematosus, Systemic* ; Male ; Middle Aged ; Neck ; Physical Examination ; Scalp ; Skin ; Vitiligo* ; Weights and Measures

The congenital diverticulum of ventricle is a rare cardiac malformation arising from the left or right ventricle, the former being more common. Ventricular diverticulum is usually associated with other anomalies including intracardiac, midline thoracic, diaphragmatic and abdominal wall defect. The authors experienced a case of congenital diverticulum of left ventricle in nine month-old female infant. Left Blolock-Taussig shunt operation was done and total correction will be done later. Thereafter we presented a case with brief review of the related literatures.
Abdominal Wall ; Diverticulum* ; Female ; Heart Ventricles ; Humans ; Infant ; Pulmonary Atresia*

Bilateral coronary artery fistula is very uncommon congenital anomaly which occupy small percentage of all congenital coronary artery fistula. About 20% of the cases associated with additional congenital heart disease. And it may cause congestive heart failure, especially severely in neonate. We experienced a case of neonate who was 1 day-old-male with bilateral coronary artery-right ventricular fistula associated with atrial septal defect and congestive heart failure. The Patient was admitted because dyspnea, cyanosis since birth. 2D ecgicardiogram and cardiac catheterization revealed that a fistulous communicating ; forming a large aneurysm, was noted from bilateral coronary artery emptied into the right ventricle, and there was oxygen step-up in right ventricle.
Aneurysm ; Cardiac Catheterization ; Cardiac Catheters ; Coronary Vessels ; Cyanosis ; Dyspnea ; Fistula* ; Heart Defects, Congenital ; Heart Failure ; Heart Septal Defects, Atrial* ; Heart Ventricles ; Humans ; Infant, Newborn* ; Oxygen ; Parturition