First World Congress on Spina Bifida Care and Research Is a Success for Pediatric Neurosurgeons

Jeffrey P. Blount, MD

From March 15 to 18, the First World Congress on Spina Bifida Research and Care convened in Orlando, Fla., under the direction of the Spina Bifida Association of America (SBA). This multidisciplinary meeting involved representatives from more than 30 countries around the world and represented a broad range of fields including neurosurgery, orthopedics, urology, developmental pediatrics, nursing and epidemiology/public health. Approximately 20 pediatric neurosurgeons from a wide range of academic and private practices attended and discussed a variety of important topics including the management of hydrocephalus and tethered spinal cord, transitional care and the controversial new procedure, lower urinary tract refunctionalization by somatic/autonomic nerve root transposition in children, commonly known as the Xiao procedure.

The purpose of the meeting was to foster interdisciplinary discussions between groups of professionals interested in and dedicated to spina bifida who might not otherwise interact with one another at professional meetings. To accomplish this interaction, the format of the meeting involved keynote speakers from each of the major disciplines during the morning session with discipline-specific breakout sessions in the afternoon. This format provided rich opportunities for cross-fertilization of ideas and questions.

Highlights of the meeting for pediatric neurosurgeons included the following:

• Marion Walker, MD, from Primary Children’s Hospital in Salt Lake City, Utah, gave the neurosurgery keynote lecture entitled “Observations on the Evolution of Thinking About Spina Bifida in Neurosurgery Across an Academic Career.” Dr. Walker traced the approach in spina bifida from a period of nihilism and despair to an era in which neurological decline is not accepted as a part of the natural history of the disorder. He outlined the evolution in the thinking about treatment of hydrocephalus and the tethered cord, and shared brief insights and ideas regarding the challenging issue of transition to adult care.
  
  
• Judy Woodruff from CNN and PBS gave the first annual Hal Pote Memorial Lecture and shared her insights regarding the challenges, fears, frustrations and victories in raising a child with spina bifida. Ms Woodruff’s son Jeffrey was born with spina bifida, and she and her husband, Al Hunt, for decades have been fierce advocates for spina bifida research. The lecture was named for Harold W. “Hal” Pote, a banker, philanthropist and impassioned and beloved advocate for children with spina bifida and for research and advocacy in spina bifida. He passed away unexpectedly in 2007.
  
  
• David McLone, MD, PhD, from Children’s Memorial Hospital in Chicago was given a Lifetime Achievement Award from the SBA for his monumental accomplishments and dedication to the cause of spina bifida. Dr. McLone was instrumental in fundamentally changing the approach to children with spina bifida and led the program in Chicago for three decades. During this time Children’s Memorial became the world leader for research, training and advocacy regarding spina bifida, and many leaders who were trained in Chicago during that time carry on the tradition today. One such neurosurgeon is Robin Bowman, who introduced Dr. McLone and presented him with the achievement award.
  
  
• Benjamin Warf, MD, from Nemour/Dupont Children’s Hospital in Wilmington, Del., received first prize in the First World Congress Award Papers, which recognize and reward the most significant original research contributions. The title of Dr. Warf’s presentation was “Neurocognitive Outcomes for ETV/CPC (Comparison of Neurocognitive Outcomes and Ventricle Size in Myelomeningocele Infants Treated for Hydrocephalus by Shunting to Those Treated with Combined Endoscopic Third Ventriculostomy/Choroid Plexus Cauterization). In this study Dr. Warf used the Hydrocephlaus Outcome Questionnaire (a standardized and validated scale for assessment) to determine the impact of different modalities of treating hydrocephalus. He concluded, based on longitudinal outcome analysis, that ETV/CPC was preferable but was associated with larger ventricles over the long term.

An update on the Management of Myelomeningocele Study (MOMS) was presented. This multicenter, unmasked randomized trial compares the outcomes of intrauterine closure of myelomeningocele with conventional treatment. As the trial is still ongoing, no outcome data could be released, but recruitment progress and rationale were presented. As of March 2008, 1,130 contacts were established between study centers and potential patient candidates, 840 patients were screened and 152 patients were randomized. Since the target population for randomization was 200, it appears the study has attained a level of recruitment of about 75 percent.

Chuan-Guo Xiao, MD, presented the urology keynote lecture and reviewed outcome data for the “Xiao procedure,” lower urinary tract refunctionalization by somatic/autonomic nerve root transposition in children with spinal cord abnormalities. He reported that 1,320 patients have undergone this procedure in China. Follow-up data were available for 400 patients: 87 percent demonstrated effectiveness of the procedure as measured by freedom from catheterization and freedom from recurrent urinary tract infections. Dr. Xiao then stayed for an additional day at the end of the meeting to present a one-day symposium on the procedure. This represented the first time these techniques had been taught in North America.

The neurosurgery breakout session featured outstanding talks that inspired significant and lively debate. In addition to Dr. Warf’s previously described paper, several papers addressed outcomes in hydrocephalus and tethered cord. Robin Bowman, MD, presented a provocative paper on whether the benign sacral dimple truly exists. Every child at Children’s Memorial clinic who was referred for a coccygeal pit/sacral dimple underwent magnetic resonance imaging of the spine. Only 44 percent were truly normal. Fat was noted in the filum of 23 percent of patients. If a low cord was noted, then a tethered cord evaluation (manual muscle testing, urodynamics) was undertaken. Of the 21 patients evaluated, 14 went on to surgery. Extensive discussion ensued.

Transition of care from childhood to adulthood was extensively discussed, and several successful models were presented both in plenary and breakout sessions.

In summary, the meeting was well attended by pediatric neurosurgeons and was successful in establishing a forum for discussion and review of the most current ideas and controversies surrounding the care of children with spina bifida.

Jeffrey P. Blount, MD, is member-at-large of the AANS/CNS Section on Pediatric Neurological Surgery and chair of the Professional Advisory Board of the Spina Bifida Association of America.