Sacral Agenesis in Children: 15 Year Experiences

ABSTRACT

PURPOSE: Sacral agenesis may occur as an isolated lesion or in conjunction with other syndromes of caudal regression. Patients with disorder often have varying degrees of vesical dysfunction. Our longitudinal experience with cohort of patients with sacral agenesis provides the model for clinimetrics in care of children with congenital anomalies. MATERIALS AND METHODS: Between 1984 and 1998, we evaluated 33 children(18 boys, 15 girls) with sacral agenesis. All patients were evaluated with renal ultrasound, cystometrogram, and voiding cystourethrogram. RESULTS: The age at initial evaluation ranged from newborns to 18 years of age, with a mean age of 5.5 years. The follow-up period ranged from 1 week to 14 years, with a mean of 7.2 years. Associated genitourinary anomalies were present in 23 patients. Only four children had no associated anomalies. And then 27 patients have maintained native bladder while 6 children underwent surgery for small bladder. Urinary continence was evaluated in 27 patients. Twenty-one patients are complete or partial dry on intermittent catheterization ; 6 patients are managed with pull-ups; none showed normal voiding. Of the 31 patients where pressure based bladder capacity could measured, only 3 patients had age expected bladder capacity; all others had an average of 56% below the age expected bladder capacity. Most patients have maintained stable renal function and renal growth. Two patients had scarring to the refluxing units with one eventually requiring nephrectomy for chronic infection. CONCLUSION: Our experience illustrates that sacral agenesis patients have a high incidence of associated genitourinary and anorectal anomalies. Continence may be achieved with clean intermittent catheterization. The outcomes of consistent upper tact preservation and absence of high-pressure bladder suggest that sequential urodynamic evaluation can be streamlined.