Fig. 13.1
(a, b) T.R. is a 24-year-old man with myelomeningocele status post tether release at 18 years who was lost to follow-up for 6 years then presented to adult clinic with frequency and nocturnal enuresis; (a) urodynamics demonstrating a small-capacity bladder with poor compliance; (b) classic “Christmas tree” bladder
13.3.2.5 Treatment Options
Patients in this category typically have small-capacity bladders with poor compliance and detrusor overactivity. Treatment options include anticholinergics, augmentation, or Botox. In this case, this patient was already on anticholinergics, and the severity of bladder wall changes demonstrated by VUDS and ultrasound often suggest poor prognosis with Botox alone. However, the patient was in college and opted for Botox injections prior to proceeding with surgical management. He has Crohn’s disease, and therefore any bowel operation presents additional risk of postoperative complications, not to mention activation of disease in the augmentation cystoplasty. Autoaugmentation has not proven to be durable in this situation, and bladder substitutes (tissue-engineered bladder) are presently investigational.
13.3.3 Type 2: Hypotonic/Atonic Bladder (With or Without Detrusor Overactivity)
Presenting symptom: A hypotonic or atonic bladder is less commonly seen in patients with myelomeningocele , but often observed in older patients with a history of posterior urethral valves, dysfunctional voiding, cerebral palsy, and congenital neuromuscular disease. Patients typically present with infrequent, large-volume voids. In the case of adults with cerebral palsy, large-volume episodes of enuresis occurring once or twice daily or worsening urinary tract infections are classically reported. While it is distressing to not intervene in such circumstances with initiation of CIC, it is important to note that CIC in many of these patients is quite troublesome and not without risk. For patients with PUV, catheterization can be difficult owing to dilation of the posterior urethra and the classic high bladder neck that these patients often have. Patients with cerebral palsy, neuromuscular disease, and myelomeningocele often have lower extremity contractures and anatomy that makes catheterization very challenging, not to mention that as adults they often do not have reliable and consistent assistance if impaired manual dexterity makes self-catheterization difficult. If technical or social challenges mean that the patient is only able to successfully catheterize every 1–2 days, risk of UTI may actually increase due to reinoculation of the bladder with each introduction of a catheter. For these reasons, characterization of the bladder storage pressures is critical. Even near 1 L capacity, many of these bladders demonstrate storage pressures at 10 mmHg or below and no reflux.
13.3.4 Patient 2: M.E.
13.3.4.1 History
M.E. is a 44-year-old male born with myelomeningocele . In childhood he was ambulatory and performed CIC about every 3 h and was maintained on anticholinergics. Around age 20, he was in a car accident and sustained a closed head injury which left him wheelchair bound without consistent urological care. He presented to adult myelomeningocele clinic at age 44 with a complaint of incontinence leading to ulcers on his legs and buttocks and erectile dysfunction. He reported no use of anticholinergics and Valsalva voided every 3 h with leakage in between (noted most often when transferring in and out of his chair).
13.3.4.2 Physical Examination
General: Obese, in wheelchair, no acute distress
Cardiac: Regular rate and rhythm, no murmurs, rubs, or gallops
Pulmonary: Chest clear to auscultation without wheezing, rales, or rhonchi
Neurological: Paraplegia
Abdominal Soft, non-tender, non-distended, no masses. RLQ scar well healed
Genitourinary: Normal external genitalia, atonic lower extremities, and central obesity
Psychiatric: No signs of depression , anxiety, or agitation
13.3.4.3 Lab Work /Other Studies
BUN/Cr = 13/0.8
Renal ultrasound demonstrated 12 cm and 11.7 cm kidneys without hydronephrosis.
13.3.4.4 UDS
Findings
Filling
The bladder was filled to a capacity of about 550 cm3, with first sensation at 75 cm3, strong desire at 221 cm3, and permission to void at 516 cm3. The bladder demonstrated good compliance and no detrusor overactivity, with maximum filling detrusor pressure of 14 cm H2O. The patient leaked during stress maneuvers during filling with a leak point pressure of 78 cm H2O.
Voiding
The patient voided to completion with Valsalva. Maximum detrusor pressure with Valsalva was 160 cm H2O with Pdet Qmax of 43 cm H2O. Flow rate was unable to be recorded as the patient could not void into the funnel.
Impression
Videourodynamics revealed a large smooth-walled, compliant, and stable bladder that emptied to completion with Valsalva, though the patient leaked with stress maneuvers (Fig. 13.2a–c).
Fig. 13.2
(a–c) M.E. is a 44-year -old man with history of myelomeningocele followed by motor vehicle accident and head injury at age 20. He presented at age 44 with incontinence and Valsalva voiding; (a) urodynamics demonstrating large-capacity bladder that was smooth walled with maximal storage pressure of 12 mmHg at 516 cm3; (b) filling; (c) post void
13.3.4.5 Treatment Options
See next section.
13.3.5 Patient 3: B.R.
13.3.5.1 History
B.R. is a 56-year-old female with history of cerebral palsy, managed with Crede voiding her entire life. In general, she had experienced approximately two nonfebrile UTIs per year. She voids about five times during the day and once at night. She was referred by another urologist for “slowly rising creatinine, mild bilateral pelvicaliectasis, prevoid volume of 732 cm3, and post-void volume 277 cm3.”
13.3.5.2 Physical Examination
General: Contracted lower extremities, well nourished
Cardiac: Regular rate and rhythm, no murmurs, rubs, or gallops
Pulmonary: Chest clear to auscultation, no wheezes, rubs, or rhonchi
Neurological: Severe lower extremity contractures
Abdominal: Soft, non-tender, non-distended, no masses
Genitourinary: Pelvic exam deferred
Psychiatric: Anxious, impaired cognition
13.3.5.3 Lab Work /Other Studies
Cr = 1.14 (2012)
Cr = 1.31(2015)
13.3.5.4 UDS
Findings
Filling
The bladder was filled to a capacity of 700 cm3, with no first sensation or feeling of coolness, strong desire at 590 cm3, and permission to void at 600 cm3. The bladder demonstrated good compliance and no detrusor overactivity, with maximum filling detrusor pressure of 4 cm H2O. The patient had no leakage with stress maneuvers.
Voiding
The patient was unable to void with catheters in place but voided 350 cm3 on uroflow with a maximum flow of 33 mL/s. Maximum detrusor pressure with straining was 15 cm H2O. Her post-void residual was approximately 350 cm3.
Impression
Urodynamics demonstrated a compliant bladder with a capacity around 700 cm3, no leakage, and a post-void residual after straining to void of 350 cm3.
See Fig. 13.3.
Fig. 13.3
B.R. is a 56-year-old woman with a history of cerebral palsy, managed with Crede voiding her entire life. She was referred to us for gradual increase in creatinine, mild bilateral pelvicaliectasis, and PVR of about 300 cm3. UDS revealed no leak with stress and a stable, compliant bladder through filling to 600 cm3. PVR was 350 cm3 after she voided 346 cm3 after the catheters were removed
13.3.5.5 Treatment Options
Both patients 2 and 3 have low-pressure, weak bladders with minimal detrusor overactivity. Terminal DO is often present in patients with cerebral palsy, and phasic DO can be seen in patients with several different underlying etiologies. Cognitive state and the status of the bladder outlet often drive management for symptomatic DO. In both of these cases, neither patient demonstrated DO on UDS or clinical evaluation. Patient 2 (M.E.) was distressed by his leakage and had already had significant sequelae (skin breakdown) related to incontinence. Artificial urinary sphincter is used in the management of bladder neck incompetence for many individuals with myelomeningocele ; although for those using CIC, we typically recommend it be placed at the bladder neck to minimize the risk of urethral erosion. This operation can be quite technically challenging and potentially morbid in an adult with myelomeningocele, particularly if he mobilizes via wheelchair and is obese. Fortunately for patient 2, he is able to empty entirely and quite quickly by Valsalva and is an excellent candidate for a bulbar urethral AUS. Patient 3 demonstrated limited baseline cognitive function and severe lower extremity contractures. Moreover, she was being cared for by her adult sibling who worked outside the home and depended on home care aides and friends during the working hours, which precluded reliable intermittent catheterization. Due to these factors, CIC was not a sustainable option. The patient underwent suprapubic tube placement after serial creatinine demonstrated progression of chronic kidney disease.
13.3.6 Type 3: DSD , Neurogenic
Detrusor sphincter dyssynergia can coexist with any bladder phenotype . It is critical to establish whether it is present, as treatment involves sphincterotomy or botulinum A injection into the sphincter and in some scenarios, may convert a partially continent patient to entirely continent, or prevent the need for intermittent catheterization. Suggestions that DSD may be present on clinical history include straining and positional voiding, Crede voiding, and/or voiding in a staccato pattern. It is our practice to utilize videourodynamics in all situations where DSD is entertained, since patch EMG is insensitive. The classic finding is a dilated posterior urethra and non-opening or poor opening of the sphincter and a staccato pattern on uroflow.
13.3.7 Patient 4: J.G.
13.3.7.1 History
J.G. is a 20-year-old with myelomeningocele who was managing his bladder with timed voiding and CIC twice daily on bactrim prophylaxis and anticholinergics. He experienced very few problems for several years on this regimen. The rationale for twice daily CIC did not make a lot of medical sense, but had worked well for him. We made a plan to wean CIC to once daily and then discontinue altogether and follow his renal ultrasounds, Cr, and symptoms. Over the next couple of years, his creatinine slowly increased, which prompted urodynamic studies.
Related posts:
Iatrogenic Female Bladder Outlet Obstruction
Pelvic Organ Prolapse
Male Stress Urinary Incontinence
Equipment, Setup, and Troubleshooting for Basic and Advanced Urodynamics
Bladder Outlet Obstruction: Female Non-neurogenic
Terminology/Standard Interpretative Format for Basic and Advanced Urodynamics
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