Aged ; Cholangiopancreatography, Endoscopic Retrograde ; Fibrosis ; drug therapy ; pathology ; surgery ; therapy ; Gallstones ; complications ; Humans ; Middle Aged ; Somatostatin ; therapeutic use ; Varicose Veins ; drug therapy ; pathology ; surgery ; therapy

Klippel - Trenaunay syndrome (KTS) is characterized by a cutaneous vascular nevus of the involved extremity, bone and soft tissue hypertrophy of the extremity and venous malformations. We present a case of KTS with splenic hemangiomas and rectal varices. A 29-year-old woman was referred for intermittent hematochezia for several years. She had history with a number of operations for cutaneous and soft tissue hamangiomas since the age of one year old and for increased circumference of her left thigh during the last few months. Abdominal CT revealed multiple hemangiomas in the spleen, fusiform aneurysmal dilatation of the deep veins and soft tissue hemangiomas. There was no evidence of hepatosplenomegaly or liver cirrhosis. Colonoscopy revealed hemangiomatous involvement in the rectum. There were rectal varices without evidence of active bleeding. Upon venography of the left leg, we also found infiltrative dilated superficial veins in the subcutaneous tissue and aneurysmal dilatation of the deep veins. The patient was finally diagnosed with KTS, and treated with oral iron supplementation only, which has been tolerable to date. Intervention or surgery is not required. When gastrointestinal varices or hemangiomatous mucosal changes are detected in a young patient without definite underlying cause, KTS should be considered.
Adult ; Colonoscopy ; Female ; Hemangioma/*complications ; Humans ; Iron, Dietary/therapeutic use ; Klippel-Trenaunay-Weber Syndrome/complications/*diagnosis/drug therapy ; Rectum/blood supply ; Spleen/blood supply ; Tomography, X-Ray Computed ; *Varicose Veins

BACKGROUND/AIMS: Terlipressin and somatostatin decrease portal venous pressure and they are used for the treatment of variceal bleeding. However, only a few studies have compared the efficacy of these drugs in combination with other procedures for hemostasis. Therefore, we performed a prospective study to compare the efficacy of terlipressin and somatostatin for controlling acute variceal bleeding when used in combination with other procedures for hemostasis. METHODS: A total of 98 patients, who presented with variceal bleeding from September 2003 to May 2005, were randomly divided into the somatostatin group or terlipressin group. We compared the 5-day failure rate (defined as failure to control bleeding, rebleeding or death within 5 days of admission) and the 6-week mortality. The prognostic factors for 5-day failure and 6-week mortality were also evaluated. RESULTS: There were no differences in baseline characteristics between the two groups. The overall 5-day failure rate and the cumulative 6-week mortality were 16.3% and 15.8%, respectively. The five-day failure rate and the cumulative 6-week mortality were not significantly different between the somatostatin and terlipressin groups. Hepatocellular carcinoma, the baseline serum creatinine level and endoscopic treatment for hemostasis were the significant predictors of 5-day failure; the baseline serum creatinine level was the predictor of 6-week mortality. CONCLUSIONS: Both somatostatin and terlipressin were effective and showed comparable efficacy for the control of the acute variceal bleeding in the setting of a combined therapeutic approach. The baseline serum creatinine level may be a significant predictor for patient failure at 5 days and the 6-week mortality.
Acute Disease ; Aged ; Carcinoma, Hepatocellular/complications ; Esophageal and Gastric Varices/complications/*drug therapy ; Female ; Gastrointestinal Hemorrhage/complications/*drug therapy ; Hemorrhage/complications/drug therapy ; Hemostasis, Endoscopic ; Humans ; Liver/*blood supply ; Liver Cirrhosis/*complications ; Liver Diseases/drug therapy ; Liver Neoplasms/complications ; Lysine Vasopressin/administration & dosage/*analogs & derivatives/therapeutic use ; Male ; Middle Aged ; Multivariate Analysis ; Somatostatin/administration & dosage/*therapeutic use ; Varicose Veins/complications/drug therapy ; Vasoconstrictor Agents/administration & dosage/*therapeutic use

BACKGROUND: Craniofacial resection (CFR) has been regarded as a standard treatment for various tumors involving the anterior skull base. The purpose of this study was to evaluate the results of CFR for the patients with anterior skull base malignancies in our hospital. METHODS: We retrospectively analyzed 17 patients with anterior skull base malignancies treated with CFR between 2001 and 2012. Mean follow-up duration was 41 months (range, 2-103 months). RESULTS: Intracranial involvement was found in 11 patients (65%) and orbital extension in 6 patients (35%). Classical bifrontal craniotomy was combined with endoscopic endonasal approach in 14 patients and external approach in 3 patients. Vascularized flap was used for reconstruction of the anterior fossa floor in 16 patients (94%). The most common pathological type was squamous cell carcinoma (6 patients). Gross total resection was achieved in all cases. Postoperative complications developed in 4 patients (24%) and included local wound problem and brain abscess. One patient with liver cirrhosis died from unexpected varix bleeding after the operation. Although postoperative treatment, such as radiotherapy or chemotherapy, was performed in 14 patients, local recurrence was seen in 6 patients. The mean overall survival time after the operation was 69.0 months (95% confidence interval: 47.5-90.5 months) with a 1-, 2-, and 5-year survival rate of 82.3%, 76.5%, and 64.7%, respectively. Postoperative radiotherapy was found to be the powerful prognostic factor for favorable survival. CONCLUSION: Considering the higher local control rate and acceptable complication or mortality rate, CFR with adjuvant radiotherapy is a gold standard treatment option for malignant tumors involving anterior skull base, especially with extensive intracranial involvement.
Brain Abscess ; Carcinoma, Squamous Cell ; Cranial Fossa, Anterior ; Craniotomy ; Drug Therapy ; Follow-Up Studies ; Hemorrhage ; Humans ; Intraoperative Complications ; Liver Cirrhosis ; Mortality ; Orbit ; Paranasal Sinus Neoplasms ; Postoperative Complications ; Radiotherapy ; Radiotherapy, Adjuvant ; Recurrence ; Retrospective Studies ; Skull Base* ; Skull* ; Survival Rate ; Treatment Outcome ; Varicose Veins ; Wounds and Injuries