Urological Outcome of the Xiao Procedure in Children with Myelomeningocele and Lipomyelomeningocele Undergoing Spinal Cord Detethering.

PURPOSE: Although previous studies have revealed high success rates (70% to 85%) after an intradural somatic-to-autonomic nerve transfer procedure in children with spinal dysraphism, no study has had a control group or blinded observers. We report a rigorously designed study to investigate the effectiveness of the Xiao procedure. MATERIALS AND METHODS: Children with neurogenic bladder dysfunction related to myelomeningocele or lipomyelomeningocele who required spinal cord detethering were randomized to 2 groups at surgery, with half undergoing only spinal cord detethering and half undergoing the Xiao procedure in addition to detethering. Double-blind evaluations were performed at regular intervals during the 3-year followup. RESULTS: A total of 10 patients underwent spinal cord detethering only and 10 underwent detethering plus the Xiao procedure. The Xiao procedure did not result in voluntary voiding or continence in any patient, but patients undergoing spinal cord detethering plus the Xiao procedure were more likely to have greater improvements in total bladder capacity, bladder overactivity and overall quality of life than those who underwent detethering only. By the end of the study no participant or evaluator was able to accurately predict to which group the patients had been assigned. CONCLUSIONS: The results of this randomized controlled trial are in agreement with recently published similarly poor results of the Xiao procedure in patients with spinal cord injury. Improvements in bladder parameters observed in this study may be related to sacral nerve root section, a necessary portion of the Xiao procedure, instead of reinnervation. Confirmatory animal studies are recommended before further clinical trials of the Xiao procedure are performed in humans.

Lack of efficacy of an intradural somatic-to-autonomic nerve anastomosis (Xiao procedure) for bladder control in children with myelomeningocele and lipomyelomeningocele: results of a prospective, randomized, double-blind study.

OBJECTIVE Xiao et al. and other investigators have studied an intradural somatic-to-autonomic (e.g., L-5 to S3-4) nerve transfer as a method to create a reflex arc to allow bladder emptying in response to cutaneous stimulation (the Xiao procedure). In previous clinical studies of patients with spinal dysraphism who underwent the Xiao procedure, high success rates (70%-85%) were reported for the establishment of a "skin-CNS-bladder" reflex arc that allows spontaneous, controlled voiding in children with neurogenic bladder dysfunction. However, many of these studies did not use blinded observers, did not have control groups, and/or featured only limited follow-up durations. METHODS A randomized, prospective, double-blind trial was initiated in March 2009, enrolling children with myelomeningocele (MM), lipomyelomeningocele (LMM), and neurogenic bladder dysfunction who were scheduled for spinal cord detethering (DT) for the usual indications. At the time of DT, patients were randomized between 2 arms of the study: half of the patients underwent a standard spinal cord DT procedure alone (DT group) and half underwent DT as well as the Xiao procedure (DT+X group). Patients, families, and study investigators, all of whom were blinded to the surgical details, analyzed the patients' strength, sensory function, mobility, voiding, and urodynamic bladder function before surgery and at regular intervals during the 3-year follow-up. RESULTS Twenty patients were enrolled in the study: 10 underwent only DT and the other 10 underwent DT+X. The addition of the Xiao procedure to spinal cord DT resulted in longer operative times (p = 0.024) and a greater chance of wound infection (p = 0.03). Patients in both treatment arms could intermittently void or dribble small amounts of urine (< 20% total bladder capacity) in response to scratching in dermatomes T-9 through S-2 using a standardized protocol, but the voiding was not reproducible and the volume voided was not clinically useful in any patient. Voiding in response to scratching was not more frequent in patients who underwent DT+X compared with those who underwent only DT. Bladder contractions in response to scratching occurred in both treatment arms at various intervals after surgery, but they were not more reproducible or more frequent in the patients who underwent the Xiao procedure than in the patients who did not. No patient in either treatment arm was continent of urine before, during, or after the study. CONCLUSIONS Patients with MM and LMM who underwent the Xiao procedure during spinal cord DT were no more likely to be able to void, to control their urination, to achieve continence, or to have a demonstrable urodynamic bladder contraction in response to cutaneous stimulation than patients who underwent only spinal cord DT. This study, in the context of disappointing results reported in other recent studies of the Xiao procedure, raises doubts about the clinical applicability of this procedure in humans until further basic science research is performed.

Angiography-negative primary angiitis of the central nervous system in childhood.

Childhood primary angiitis of the CNS is a recently characterized, potentially reversible disease process. A favorable outcome requires early diagnosis and appropriate treatment. The histological findings of childhood primary angiitis of the CNS are characterized by a lymphocytic, nongranulomatous vasculitis. This disorder can lead to neurological deficits, seizures, and strokes. Laboratory and radiographic investigation are part of the evaluation, but are often nonspecific. Conventional angiography can fail to show any abnormality, and biopsy may ultimately be required for diagnosis. Although there can be significant rates of morbidity and mortality if untreated, patients who receive appropriate therapy can experience excellent outcomes, and in many cases will demonstrate near-complete or total clinical and radiographic resolution. The case of a previously healthy 13-year-old girl with new-onset generalized tonic-clonic seizures is presented, with a review of the literature.

Attempted bladder reinnervation and creation of a scratch reflex for bladder emptying through a somatic-to-autonomic intradural anastomosis.

An intradural somatic-to-autonomic anastomosis, or Xiao procedure, has been described to create a "skin-CNS-bladder" reflex that improves bladder and bowel function in patients with neurogenic bladder and bowel dysfunction. The authors present their experience with a 10-year-old boy with chronic neurogenic bladder and bowel dysfunction related to spinal cord injury who underwent the Xiao procedure. After undergoing a left L-5 ventral root to left S2-3 intradural anastomosis, the patient reported that his bladder and bowel dysfunction improved between 6 and 12 months. Two years after the procedure, however, he reported that there was no change in his bladder or bowel dysfunction as compared with his condition prior to the procedure. Frequent, systematic multidisciplinary evaluations produced conflicting data. Electrophysiological and histological evaluation of the previously performed anastomosis during surgical reexploration 3 years after the Xiao procedure revealed that the anastomosis was in anatomical continuity but neuroma formation had prevented reinnervation. Nerve action potentials were not demonstrable across the anastomosis, and stimulation of the nerve above and below the anastomosis created no bladder or perineal contractions. This is the first clinical report on the outcome of the Xiao procedure in a child with spinal cord injury outside of China. It is impossible to draw broad conclusions about the efficacy of the procedure based on a single patient with no demonstrable benefit. However, future studies should carefully interpret transient improvements in bladder function, urodynamic findings, and the patient's ability to void in response to scratching after the Xiao procedure. The authors' experience with the featured patient, in whom reinnervation could not be demonstrated, suggests that such changes could be related to factors other than the establishment of a skin-CNS-bladder reflex as a result of a somatic-to-autonomic anastomosis.

The use of transcutaneous thermal convection analysis to assess shunt function in the pediatric population.

BACKGROUND: The diagnosis of shunt malfunction is largely made by subjective clinical history and assessment in association with neurodiagnostic imaging. OBJECTIVE: : To evaluate the use of a transcutaneous thermal convection device for the diagnosis of shunt malfunction. METHODS: We present the results of a trial of a commercially available device under an Institutional Review Board-approved protocol. All patients had neurodiagnostic studies that defined their shunt function at the time of transcutaneous thermal convection measurement. Thirty-seven shunts were studied in 35 patients. To be included, patients had to be between 0 to 18 years of age, had to be due within a 3-month period for routine follow-up evaluations, and had to have neurodiagnostic imaging (computed tomography or magnetic resonance imaging) as part of this visit and a shunt series. All patients were seen in routine follow-up, and none had clinical symptoms of shunt malfunction. RESULTS: Three patients had fractured shunts. The remaining 32 patients had functioning shunts as determined by clinical criteria, computed tomography or magnetic resonance imaging scans, and, when appropriate, a shunt series. In these remaining patients, flow was initially confirmed in only 40%. After some filtering of the data, this was increased to 51%. Although these results are disappointing, they outline the current issues with the technique and the state of its utility and point to the need for further refinement. CONCLUSION: Our current research suggests that cerebrospinal fluid flow as detected by thermoconvection analysis is not a reliable indicator of shunt function in the pediatric population.

Congenital dermoid tumor in a child at initial myelomeningocele closure: an etiological discussion.

The authors report on a case of a full-term infant with lumbar myelomeningocele who was found to have an intramedullary mass at the time of surgical repair of the defect. The intramedullary mass was consistent with a dermoid tumor both macroscopically and microscopically. This case provides evidence that dermoid tumors occurring at the site of previous surgical myelomeningocele repair are not always a consequence of incomplete excision of the dermal elements.

Primary closure of open myelomeningocele.

OBJECT: The purpose of this paper is to describe the technique of closing an open myelomeningocele in the newborn infant. METHODS: The anatomical rationale behind the closure techniques will be specifically reviewed. Avoidance of complications will be discussed. The management of unusual developmental anatomical abnormalities, such as the management of unusually large skin defects and kyphotic deformities will be discussed. Complications and the postoperative care will be reviewed and specifics regarding postoperative care will be summarized. CONCLUSIONS: A multidisciplinary team approach is critical to the long-term management of these patients.