| Journal: | International braz j urol |
| Database: | PERIÓDICA |
| System number: | 000296738 |
| ISSN: | 1677-5538 |
| Authors: | Nicanor, Francisco A1 Cook, Anthony Pippi-Salle, Joao L |
| Institutions: | 1University of Toronto, Hospital for Sick Children, Toronto, Ontario. Canadá |
| Year: | 2005 |
| Season: | Sep-Oct |
| Volumen: | 31 |
| Number: | 5 |
| Pages: | 477-481 |
| Country: | Brasil |
| Language: | Inglés |
| Document type: | Artículo |
| Approach: | Caso clínico, descriptivo |
| English abstract | INTRODUCTION: The urofacial or Ochoa syndrome is a rare disease characterized by the presence of functional obstructive uropathy associated with peculiar facial features when patients attempt to smile or laugh. Unfortunately, many of these patients remain without proper diagnosis or adequate treatment due to lack of recognition of the disease. This can ultimately result in upper tract deterioration and eventual renal failure. We present our experience with this rare syndrome. MATERIALS AND METHODS: We identified 3 patients who presented initially with acute renal failure, urinary tract infection (UTI) and severe dysfunctional elimination. All patients were thoroughly evaluated, including screening for spinal cord anomalies, and were subsequently diagnosed with urofacial syndrome. RESULTS: At the outset, the two older patients (aged 4 and 9 years) presented with the typical facial features when attempting to smile or laugh. One patient in the newborn period presented with urinary and fecal retention and septicemia and, to our knowledge, represents the youngest case of urofacial syndrome reported so far. All patients were evaluated with ultrasonography, renal scan, voiding cystourethrogram (VCUG) and urodynamics. Findings included hydronephrosis and a thick-walled, trabeculated bladder with poor compliance and detrusor hypereflexia respectively in each patient. All were subsequently treated with clean intermittent catheterization (CIC), antibiotic prophylaxis and anticholinergic therapy. One patient required appendicovesicostomy for |
| Disciplines: | Medicina |
| Keyword: | Cirugía, Diagnóstico, Vejiga neurogénica, Insuficiencia renal, Síndrome Ochoa, Facies |
| Keyword: | Medicine, Diagnosis, Surgery, Neurogenic bladder, Renal failure, Ochoa syndrome, Facies |
| Full text: | Texto completo (Ver HTML) |
