RESUMO
OBJECTIVES: Complete or partial sacral agenesis is a rare malformation consisting in the absence of one or more sacral vertebrae. It is part of a caudal regression syndrome and it may be associated with other congenital anomalies (Currarino Syndrome). It does not have an established etiology but is associated with insulin-dependent diabetes mellitus in the mother (1%). The objective of this is study was to retrospectively analyze the urological outcome of patients with sacral agenesis in our case series. METHODS: Retrospective analysis of 14 patients between 1975 and 2005. We evaluated reason for consultation, urological status, continence outcome, urological complications, hospital admissions and number of office visits. RESULTS: No patient had history of diabetic mother The number of male/female patients were similar. Mean age at first visit was 13.2 years and main reason for consultation was urinary incontinence. 60% of the patients presented associated myelomeningocele. 70% had a normal upper urinary tract at the beginning of follow-up; 10 patients presented some degree of incontinence (70%). Mean follow-up was 19.7 years (7-30): 50% of the patients keep a normal upper urinary tract. All of them have presented symptomatic urinary tract infection. The main urological reason for hospital admission was programmed surgery (7). The mean number of visits per year was 1.9. CONCLUSIONS: Sacral agenesis is a rare congenital malformation requiring an early diagnosis to avoid mid-term urological complications. These patients need life-long urological follow-up. The mean reason for consultation is urinary incontinence secondary to neurogenic bladder which may be satisfactorily treated in most cases.
