Renal damage and renal failure are among the most severe complications of spina bifida. Over the past decades, a comprehensive treatment strategy has been applied that results in minimal renal scaring. In addition, the majority of patients can be dry for urine by the time they go to primary school. To obtain such results, it is mandatory to treat detrusor overactivity from birth onward, as upper urinary tract changes predominantly start in the first months of life. This means that new patients with spina bifida should be treated from birth by clean intermittent catheterization and pharmacological suppression of detrusor overactivity. Urinary tract infections, when present, need aggressive treatment, and in many patients, permanent prophylaxis is indicated. Later in life, therapy can be tailored to urodynamic findings. Children with paralyzed pelvic floor and hence urinary incontinence are routinely offered surgery around the age of 5 years to become dry. Rectus abdominis sling suspension of the bladder neck is the first-choice procedure, with good to excellent results in both male and female patients. In children with detrusor hyperactivity, detrusorectomy can be performed as an alternative for ileocystoplasty provided there is adequate bladder capacity. Wheelchair-bound patients can manage their bladder more easily with a continent catheterizable stoma on top of the bladder. This stoma provides them extra privacy and diminishes parental burden. Bowel management is done by retrograde or antegrade enema therapy. Concerning sexuality, special attention is needed to address expectations of adolescent patients. Sensibility of the glans penis can be restored by surgery in the majority of patients.
PurposeTo evaluate if a three‐component model correctly describes the diffusion signal in the kidney and whether it can provide complementary anatomical or physiological information about the underlying tissue.Materials and MethodsTen healthy volunteers were examined at 3T, with T
2‐weighted imaging, diffusion tensor imaging (DTI), and intravoxel incoherent motion (IVIM). Diffusion tensor parameters (mean diffusivity [MD] and fractional anisotropy [FA]) were obtained by iterative weighted linear least squares fitting of the DTI data and mono‐, bi‐, and triexponential fit parameters (D
1, D
2, D
3, f
fast2, f
fast3, and f
interm) using a nonlinear fit of the IVIM data. Average parameters were calculated for three regions of interest (ROIs) (cortex, medulla, and rest) and from fiber tractography. Goodness of fit was assessed with adjusted R2 (
normalRadj2) and the Shapiro‐Wilk test was used to test residuals for normality. Maps of diffusion parameters were also visually compared.ResultsFitting the diffusion signal was feasible for all models. The three‐component model was best able to describe fast signal decay at low b values (b < 50), which was most apparent in
normalRadj2 of the ROI containing high diffusion signals (ROIrest), which was 0.42 ± 0.14, 0.61 ± 0.11, 0.77 ± 0.09, and 0.81 ± 0.08 for DTI, one‐, two‐, and three‐component models, respectively, and in visual comparison of the fitted and measured S0. None of the models showed significant differences (P > 0.05) between the diffusion constant of the medulla and cortex, whereas the f
fast component of the two and three‐component models were significantly different (P < 0.001).ConclusionTriexponential fitting is feasible for the diffusion signal in the kidney, and provides additional information.
Level of Evidence: 2
Technical Efficacy: Stage 1J. MAGN. RESON. IMAGING 2017;46:228–239
The transverse diameter of the rectum measured by lower abdominal ultrasound provides an additional accurate parameter with which to diagnose constipation in patients with nonneurogenic bladder-sphincter dyssynergia.
Pediatric urodynamics taught us that detrusor-sphincter dyssynergia creates a bladder outlet obstruction in about 50% of any population of children with myelomeningocele. This functional obstruction causes renal damage due to obstructive uropathy, exactly the same way as a congenital anatomical urethral obstruction does. Pediatric urodynamics also taught us that in children with myelomeningocele pelvic floor activity and detrusor activity can be abnormal (hyperactive or inactive) completely independent from each other. These insights have changed the management of myelomeningocele. Children with overactivity of the pelvic floor can be singled out at infant age, and started on clean intermittent catherization, to prevent obstructive uropathy and preserve renal function. Children with detrusor overactivity can be singled out too at very early age, and treated with anticholinergics, to prevent irreversible structural damage to the detrusor and preserve normal bladder capacity and compliance.
Arterio-ureteral fistula (AUF) is a rare, but potentially lethal, clinical entity. In spite of an increase in reported cases and clinical awareness, AUF is not always recognized in time. Delay between first presentation and treatment may adversely affect clinical outcome. What We Found: In this systematic review we found 245 published articles with 445 patients and 470 AUFs. Most patients had a medical history of chronic indwelling ureteral stents (80%), pelvic oncology (70%), irradiation (53%) or vascular surgery (26%), presenting with intermittent (micro) hematuria or building up to massive hematuria. The pathophysiologic hypothesis is presented in the figure . AUF was located at the crossing between ureter and artery, mostly the common iliac artery. The best modality to diagnose this entity was an angiography, with a sensitivity of 62%. Endovascular stent graft placement is preferred over open surgical repair in terms of AUF-related mortality (4% vs 11%). AUF-specific mortality before 2000 vs after 2000 is 19% vs 7%, coinciding with increasing use of endovascular stent graft placement.Limitations: The retrospective nature of this study, including many single case reports, results in a low level of evidence and high risk of bias. Furthermore, report bias could impact our findings such as better medical registration, electronic patient files, better imaging tests, less invasive treatment options, and better outcome. Interpretation for Patient Care: Clinical awareness as well as multidisciplinary approach is important to decrease unnecessary delay between first presentation and treatment of AUF. The best diagnostic tool is an angiography and the preferred treatment is endovascular stent graft placement.
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