Fibromuscular Dysplasia/diagnostic imaging* ; Humans ; Hypertension/complications* ; Hypertension, Renovascular/etiology* ; Kidney ; Renal Artery/diagnostic imaging*

Infant, Newborn ; Humans ; Fibromuscular Dysplasia/complications* ; Renal Artery ; Hypertension, Renovascular/etiology* ; Renal Artery Obstruction/complications* ; Hypertension/complications*

Two cases of primary intimal fibroplasia of the renal artery with renovascular hypertension are described. Case 1 was 27 year old female who was incidentally found to have blood pressure of 210/130 mmHg on routine physical examination. Renal arteriogram revealed tubular narrowing of the mid protion of the left renal artery. Both patients showed lateralization in renin activity at the involved side renal vein. Case 2 was a 10 year old girl who was first noted to have a hypertension of 180/120mmHg after a sudden attack of seizure, vomiting and altered consciousness. Renal arteriogram showed concentric narrowing of the proximal half of the right renal artery. Histopathologic examination of the affected arterial segments from both cases showed essentially same findings, i,e., diffuse fibrous thickening of the intima occluding the lumen, focal fragmentation, duplication and disappearance of the internal elastic membranes. There were no deposit of lipid and inflammatory cells. The media and adventitia remained intact. The blood pressure of both patients became normal, after the surgery and the patients are in good health up to this time.
Adult ; Arterial Occlusive Diseases/*pathology ; Child ; Female ; Fibromuscular Dysplasia/complications/epidemiology/*pathology ; Humans ; Hypertension, Renovascular/*etiology/surgery ; Korea/epidemiology ; Renal Artery/*pathology/surgery

OBJECTIVE: To evaluate mid-term imaging, clinical follow-up, and restenosis rates from patients that had undergone percutaneous transluminal renal artery angioplasty (PTRA) for symptomatic renal artery fibromuscular dysplasia (FMD). MATERIALS AND METHODS: Between March 1999 and July 2006, 16 consecutive renal artery FMD patients underwent PTRA for poorly controlled hypertension. The patients were enrolled into this retrospective study after receiving 19 primary and four secondary PTRAs in 19 renal artery segments. Follow-up monitoring of blood pressure, use of antihypertensive medication, and the serum creatinine level after PTRA were assessed at 1, 3, 6, 9, 12 months, and each following year. The degree of restenosis was evaluated with computed tomographic angiography (CTA) after PTRA at 6, 12 months, and every year if possible. Technical and clinical success rates for the treatment of FMD, and restenosis rates for the renal artery were evaluated. RESULTS: The technical success rate for primary PTRA was 79% (15/19) and the complication rate was 16% (3/19). Hypertension improved in 80% (12/15) of the patients after four weeks follow-up, and was finally cured or improved in 93% (14/15) during the mean follow-up period of 23.6 months. There was a cumulative 22% (4/18) restenosis rate during the follow-up period. All of the patients were treated with a second PTRA without complications and all of the patients were cured of hypertension after the second PTRA. CONCLUSION: Percutaneous transluminal renal artery angioplasty for clinically symptomatic renal FMD is technically and clinically successful and safe to perform. For all patients with restenosis, there was a good response after undergoing a second PTRA.
Adolescent ; Adult ; Angiography ; *Angioplasty, Balloon ; Child ; Female ; Fibromuscular Dysplasia/complications/radiography/*therapy ; Humans ; Male ; Middle Aged ; Recurrence ; Renal Artery Obstruction/etiology/radiography/*therapy ; Retrospective Studies ; Stents ; Treatment Outcome

No abstract available.
Adult ; Angiography, Digital Subtraction ; Angioplasty, Balloon ; *Blood Pressure ; Female ; Fibromuscular Dysplasia/*complications/diagnosis ; Humans ; Hypertension, Renovascular/diagnosis/*etiology/physiopathology/therapy ; Renal Artery Obstruction/diagnosis/*etiology/physiopathology/therapy ; Treatment Outcome

PURPOSE: Congenital esophageal stenosis (CES) is a rare cause of esophageal narrowing due to intrinsic esophageal wall abnormalities such as tracheobronchial remnants (TBR), web, and fibromuscular dysplasia (FMD). It is sometimes associated with esophageal artesia (EA). METHODS: The medical records of children, who underwent operation due to CES at the Asan Medical Center from Jan 1990 to Dec 2007, were retrospectively reviewed. RESULTS: Among 12 patients (M : F=6 : 6), 9 patients had TBR and 3 had FMD. The median age of operation was 25 months (7 mo~6 years). Four patients underwent operation for esophageal atresia at neonate, one underwent operation for duodenal atresia and one for ventricular septal defect. Recurrent vomiting or dysphagia was developed at weaning period or introduction of solid foods. The lesions of stenosis were low-esophagus in 11 and mid-esophagus in 1. Segmental resection of lesion and end-to-end anastomosis of esophagus were completed successfully by means of laparotomy in 9 and thoracotomy in 3 (left in 1, right in 2). One patient treated with ballooning at first, suffered from mediastinitis due to esophageal perforation. Postoperative complications were anastomosis leakage in 2 and lung abscess in 1. Esophageal ballooning for anastomosis stricture was performed in 2 patients and an esophageal stent was needed in 1 patient. All patients are relieved from dysphagia and able to eat solid food. CONCLUSION: CES is rare but should be considered for recurrent vomiting and dysphagia, especially for those who underwent operation for EA. Differential diagnosis from other causes such as gastro-esophageal reflux is essential and surgical treatment is treatment of choice.
Child ; Constriction, Pathologic ; Deglutition Disorders ; Diagnosis, Differential ; Duodenal Obstruction ; Esophageal Atresia ; Esophageal Perforation ; Esophageal Stenosis ; Esophagus ; Fibromuscular Dysplasia ; Gastroesophageal Reflux ; Heart Septal Defects, Ventricular ; Humans ; Infant, Newborn ; Laparotomy ; Lung Abscess ; Mediastinitis ; Medical Records ; Postoperative Complications ; Retrospective Studies ; Stents ; Thoracotomy ; Vomiting ; Weaning