Before the start of the Management of Myelomeningocele Study (MOMS) in 2003, an initial group of 58 patients at The Children’s Hospital of Philadelphia’s Center for Fetal Diagnosis and Treatment underwent in utero repair of their myelomeningocele between 21 and 25 weeks' gestation. The average gestational age at the time of delivery was 34 1/2 weeks' gestation.
Fifty-four survived for long-term follow-up, which included early videourodynamics in our urodynamics suite, using a Laborie Aquarious™ unit. A 5 French dual lumen urodynamic catheter was positioned in the bladder, along with an 8 French feeding tube to transduce intra-abdominal pressure along with patch EMG electrodes to assess pelvic floor and external sphincter activity. Infusion of Cysto-Conray™ II at room temperature was conducted at a rate of 2 to 3 ml per minute. Video fluoroscopy was employed to periodically image the bladder, look for the presence of vesicoureteral reflux and assess the bladder outlet during the filling and voiding phases. Equivocal studies were repeated with a second filling phase, much like a cyclic voiding cystourethrogram study is done in infants.
Repeat urodynamics were performed at 1, 2, 3 and 5 years of age, with the same team utilized for all of the studies. For those children who reported volitional voiding, flow rates were obtained and post-void residuals determined, either with the use of ultrasonography or with catheterization. Families were questioned about the bowel regimens employed in addition to urologic, neurological and orthopedic changes that had occurred since their last visit. Routine renal and bladder ultrasonography, if needed, and evaluation of brain and spine MRI studies were performed as well.
To further assess children who were reported to have successfully toilet trained, families completed a Pediatric Lower Urinary Tract Symptom Score questionnaire used in our Dysfunctional Outpatient Voiding Education (DOVE) Center. The score reflects the child’s day- and nighttime wetting, assesses voiding difficulties and constipation, and provides a reference point so that the intervention enacted can be quantitated at subsequent evaluation. In addition, whenever feasible, flow rates were obtained to further characterize the child’s voiding dynamics along with an assessment of post-void residual. This study was conducted with Institutional Review Board approval.
Ten patients (18.5 percent) reported successful toilet training during the day and variable degrees of continence, while two patients had bowel continence and one patient had bladder continence but required enemas, and two patients who successfully toilet-trained developed spinal dermoid cysts requiring surgical resection or symptomatic retethering of their spinal cord. Both of these patients did ultimately regain control of their bladder function, but required several months of convalescence and rehabilitation before regaining control.
The 44 patients who did not report volitional voiding and continence had either been started on a clean intermittent catheterization (CIC) program, generally with the use of anti-muscarinics, or were still in diapers. The pediatric urologists who oversaw their care at the respective spina bifida clinics made the decision to enact CIC due to clinical concerns with recurrent urinary tract infections, changes to the upper tracts with progressive renal dilation, or to allow children to achieve social continence or dryness.
This original nonrandomized group has demonstrated a greater likelihood to successfully toilet train than historical controls. MOMS II urologic follow-up at ages 6 to 10 years will provide the most meaningful information to assess urologic function in spina bifida patients.
An analysis of an individual L4 level spina bifida patient who has had long-term follow-up at CHOP’s Spina Bifida Clinic is worth noting. His initial newborn videourodynamic evaluation showed a pattern of dyssynergy and incomplete bladder emptying.
At 1 year of age, his bladder appeared smooth-walled on fluoroscopy, he generated a detrusor contraction but did not empty his bladder completely. By 2 years of age, his urodynamic pattern was similar to 1 year of age, but by 3 years of age, his parents reported that he was now using a toilet periodically but had not successfully toilet trained. At 5 years of age, he was generally dry during the day and following successful voiding he emptied his bladder completely. His DOVE score at 6 years of age was 7, due to his persistent nocturnal enuresis.
Now, he is 10 years of age and generally dry both during the day and at night. Thus, if the initial urodynamic studies had been used to predict his urinary continence, he should have required intermittent catheterization and been predicted to do poorly with respect to achieving continence. Hence, long-term follow-up data is critical to provide a complete picture, particularly when assessing urinary continence.
Carr MC. Urological Results After Fetal Myelomeningocele Repair in Pre-MOMS Trial Patients at the Children’s Hospital of Philadelphia. Fetal Diagn Ther. 2014 Jul 10. [Epub ahead of print]