Long-term urologic outcome in patients with caudal regression syndrome, compared with meningomyelocele and spinal cord lipoma.
Torre M, Buffa P, Jasonni V, Cama A. Paediatric Surgery, Istituto Giannina Gaslini, 16148 Genoa, Italy. micheletorre@hotmail.com J Pediatr Surg. 2008 Mar;43(3):530-3.
BACKGROUND/PURPOSE: The long-term urologic outcome in a large series of patients with neural tube defects was evaluated.
METHODS: The following clinical parameters in 398 patients ranging from 1 to 37 years of age–69 with caudal regression syndrome (CRS), 244 with meningomyelocele (MMC), and 85 with spinal lipoma (SL)–were studied: congenital renal anomalies, renal function, vesico-ureteric reflux, upper tract dilatation, urodynamic pattern, and urinary continence. RESULTS: Single kidney was much more frequent in CRS (20.3%), compared with MMC (1.2%) and SL (0%). Vesico-ureteric reflux was found in 37.7% of patients with CRS, 43.0% of MMC, and 21.2% of SL. Patients with CRS had a higher risk of impaired renal function (8.7%), compared with MMC (5.3%) and SL (1.2%). Neuropathic bladder was found in 61% of patients with CRS, 98% of MMC, and 42% of SL. Among them, clean intermittent catheterization and drugs allowed 30% of patients with CRS, 45% of MMC, and 71% of SL to be dry for more than 4 hours.
CONCLUSIONS: Diagnosis influences the urologic outcome in neural tube defect. In CRS, the incidence of renal agenesis and vesico-ureteric reflux was unexpectedly high. The risk of renal damage and, in those with neuropathic bladder, of urinary incontinence, was similar to patients with MMC.
Minimally invasive approach for treatment of urinary and fecal incontinence in selected patients with spina bifida.
Lorenzo AJ, Chait PG, Wallis MC, Raikhlin A, Farhat WA. Division of Urology and Department of Diagnostic Imaging, The Hospital for Sick Children and University of Toronto, Toronto, Ontario, Canada. Urology. 2007 Sep;70(3):568-71.
OBJECTIVES: At our institution, the use of cecostomy tubes has provided a successful method for managing severe constipation in patients with spina bifida, with good patient and caretaker satisfaction and minimal morbidity. We have developed a modified technique to allow placement of the cecostomy tube under direct vision during laparoscopic appendicovesicostomy. We present our initial experience and technique. METHODS: Patients with a normal bladder capacity and compliance who were scheduled for creation of an appendicovesicostomy and who also had refractory constipation were offered concurrent cecostomy tube placement. At the laparoscopic procedure, we performed percutaneous placement of the cecostomy tube through the abdominal wall under direct visualization. Subsequently, dissection of the appendix with its mesentery was performed. The detrusor muscle was dissected and a trough for the appendix created. Laparoscopic anastomosis of the appendix to the bladder mucosa and approximation of the detrusor over the appendix created a nonrefluxing channel. RESULTS: Three patients have undergone concurrent cecostomy tube placement at appendicovesicostomy. No complications have been encountered thus far. On follow-up, the cecostomy tube scar has been well concealed and appears no different from the ones placed under radiologic guidance. The patients have been using the catheterizable channel to access the bladder and dry performing intermittent catheterization without difficulties.
CONCLUSIONS: In patients with a neurogenic bladder who do not qualify for major bladder reconstructive procedures, such as augmentation cystoplasty or bladder neck repair, social continence and independence can be achieved with minimally invasive surgery. Concomitant laparoscopic appendicovesicostomy and cecostomy tube placement may be a suitable surgical option.
A multicenter evaluation of urinary incontinence management and outcome in spina bifida.
Lemelle JL, Guillemin F, Aubert D, Guys JM, Lottmann H, Lortat-Jacob S, Moscovici J, Mouriquand P, Ruffion A, Schmitt M.
Service de Chirurgie Infantile, Hôpital d’Enfants, Centre Hospitalier Universitaire de Nancy, France. J Urol. 2006 Jan;175(1):208-12.
PURPOSE: We describe urinary continence management and outcome in patients with spina bifida to identify the procedures that are most successful.
MATERIALS AND METHODS: In a multicenter retrospective cohort study medical charts were studied. At the same time in a cross-sectional survey sociodemographic characteristics, orthopedic features and urinary continence were described based on the frequency of leakage from the viewpoint of patients or close relatives using a Likert scale of 5 items, namely 1-leakage permanent to 5-leakage never.
RESULTS: A total of 421 patients were included, of whom 191 (45%) had been medically treated with a normal voiding pattern according to the patient viewpoint in 21%, clean intermittent catheterization in 61% and no specific bladder emptying method in 18%. The mean leakage score +/- SD was 2.74 +/- 1.55. On the other hand, 230 patients (55%) were surgically treated. Except for 23 patients who underwent noncontinent urinary diversion 207 were considered for treatment and continence description. The mean leakage score was 3.45 +/- 1.60. An artificial urinary sphincter in male and females, and a sling or Kropp technique in females were satisfactory when bladder enlargement was not required. In cases of bladder augmentation without continent diversion an artificial urinary sphincter in males and a bladder neck sling or cinch, Kropp and Young-Dees procedures in females have provided the best results. In cases of bladder enlargement with continent urinary diversion bladder neck closure or a wrap have provided the best results whatever the patient sex.
CONCLUSIONS: Many factors may influence the choice of a technique, such as patient sex, bladder characteristics or orthopedic conditions. However, since to our knowledge no randomized, controlled study has been yet performed, definitive conclusions on the best way to achieve urinary continence in patients with spina bifida cannot be established.
A multicentre study of the management of disorders of defecation in patients with spina bifida.
Lemelle JL, Guillemin F, Aubert D, Guys JM, Lottmann H, Lortat-Jacob S, Moscovici J, Mouriquand P, Ruffion A, Schmitt M.
Service de Chirurgie Infantile, Hôpital d’Enfants, CHU de Nancy, Vandoeuvre les Nancy, France. jl.lemelle@chu-nancy.fr Neurogastroenterol Motil. 2006 Feb;18(2):123-8.
Patients with spinal dysraphism may have severe constipation and faecal incontinence. The impact of antegrade colonic enema (ACE) in the management of patients with spina bifida (SB) is analysed. In a multicentre cross-sectional study, constipation, faecal incontinence and faecal management were described. Cases surgically treated were identified. Data were collected from 423 patients, of whom 230 did not use any manoeuvre or laxatives to assist evacuation. Conventional treatment was used in 193 patients, including digital extraction in 39%, retrograde enema in 21% and oral laxatives in 52%. For intractable constipation and overflow of faecal incontinence, 47 patients were treated with ACE, of whom 41 used the method at a mean time of interview of 4.1 +/- 1.9 years after ACE operation; six abandoned ACE for conventional management. With ACE, faecal continence was significantly improved compared with conventional management, and neither retrograde rectal enema nor digital extraction were required. The conduit was fashioned to the right colon in 32 cases and to the left colon in nine cases. This study provides information on a multicentre experience in bowel management in SB patients. Whatever the technique used, ACE has improved faecal status compared with conventional therapy.
Non-traditional management of the neurogenic bladder: tissue engineering and neuromodulation.
Lewis JM, Cheng EY. Children’s Memorial Hospital, The Feinberg School of Medicine at Northwestern University, Chicago, IL, USA. jlewis@childrensmemorial.org ScientificWorldJournal. 2007 Aug 17;7:1230-41
Patients with spina bifida and a neurogenic bladder have traditionally been managed with clean intermittent catheterization and pharmacotherapy in order to treat abnormal bladder wall dynamics, protect the upper urinary tract from damage, and achieve urinary continence. However, some patients will fail this therapy and require surgical reconstruction in the form of bladder augmentation surgery using reconfigured intestine or stomach to increase the bladder capacity while reducing the internal storage pressure. Despite functional success of bladder augmentation in achieving a low pressure reservoir, there are several associated complications of this operation and patients do not have the ability to volitionally void. For these reasons, alternative treatments have been sought. Two exciting alternative approaches that are currently being investigated are tissue engineering and neuromodulation. Tissue engineering aims to create new bladder tissue for replacement purposes with both “seeded” and “unseeded” technology. Advances in the fields of nanotechnology and stem cell biology have further enhanced these tissue engineering technologies. Neuromodulation therapies directly address the root of the problem in patients with spina bifida and a neurogenic bladder, namely the abnormal relationship between the nerves and the bladder wall. These therapies include transurethral bladder electrostimulation, sacral neuromodulation, and neurosurgical techniques such as selective sacral rhizotomy and artificial somatic-autonomic reflex pathway construction. This review will discuss both tissue engineering techniques and neuromodulation therapies in more detail including rationale, experimental data, current status of clinical application, and future direction.
Patients with spina bifida and bladder cancer: atypical presentation, advanced stage and poor survival.
Austin JC, Elliott S, Cooper CS. Department of Urology, University of Iowa, Iowa City, Iowa 52242, USA. chris-austin@uiowa.edu J Urol. 2007 Sep;178(3 Pt 1):798-801. Epub 2007 Jul 16.
PURPOSE: Patients with neurogenic bladder dysfunction due to spina bifida have been reported to be at increased risk for bladder cancer. Recent publications suggest that bladder augmentation is also a significant risk factor. We reviewed our experience with treating patients with spina bifida and bladder cancer.
MATERIALS AND METHODS: Patients with spina bifida treated for bladder cancer between 1995 and 2005 were identified. Patient demographics, mode of bladder management, risk factors and presenting symptoms were recorded along with therapy, pathological findings and outcome. This patient cohort was combined with all prior known published studies for analysis.
RESULTS: Eight patients with a median age of 41 years were treated. Only 1 patient (13%) had undergone bladder augmentation. Locally advanced stage (T3 or greater) or lymph node metastases were present in 88% of cases. Median survival was 6 months with only 1 patient alive with no evidence of recurrence at 20 months. A total of 11 prior published cases were identified and combined with this series. Transitional cell carcinoma was present in 58% of patients. Median survival was 6 months. Only 37% of patients had undergone bladder augmentation.
CONCLUSIONS: Patients with spina bifida and bladder cancer present at a young age with variable tumor histology and advanced stage, and they have poor survival. Presenting symptoms are often atypical and bladder cancer should be a consideration in this patient population, even in young adults. Due to poor survival further study is warranted in this population to determine whether screening would be beneficial for earlier detection and improved outcomes.
Surgical implantation of the new FlowSecure artificial urinary sphincter in the female bladder neck.
García-Montes F, Vicens-Vicens A, Ozonas-Moragues M, Oleza-Simo J, Mora-Salvá A. University Hospital Son Dureta, Palma de Mallorca, Spain. fgarcia@hsd.es Urol Int. 2007;79(2):105-10.
OBJECTIVE: To report implantation of the new FlowSecure artificial urinary sphincter with conditional occlusion in a female bladder neck, describe surgical technique and suggest minor modifications to accommodate the device for universal female implantation.
PATIENT AND METHODS: A spina bifida female patient with urodynamically proven stress incontinence due to sphincteric incompetence associated to atonic detrusor was implanted with the new artificial sphincter. Operating time was one and a half hours. The cuff was adjusted to the bladder neck with no problems. Excess belt removed from the cuff was preserved and used for loose fixation of tubing and reservoirs in the right paravesical space. The control pump was placed in the right labia. The prosthesis was implanted at atmospheric pressure zero.
RESULTS: The device was easily implanted. There were no perioperative complications. Catheter was removed 24 h post-implantation and patient achieved immediate total continence. There was no need for device pressurization at subsequent follow-up. The patient needed intermittent self-catheterization for emptying her bladder because of impaired detrusor contractility.
CONCLUSIONS: Despite that the new FlowSecure artificial urinary sphincter has only been used for bulbar urethral implantation, we have successfully implanted the device in a female bladder neck with excellent clinical results. Cuff lengthening and connecting tubes shortening would probably enable all female patients to be suitable for implantation. To the best of our knowledge, this is the first time the device has been implanted in a bladder neck.
Urinary bladder adenocarcinoma arising in a spina bifida patient.
Bitar M, Mandel E, Kirschenbaum AM, Unger PD. Department of Pathology, The Mount Sinai School Of Medicine, New York, NY 10029, USA. mbitar5@aol.com Ann Diagn Pathol. 2007 Dec;11(6):453-6. Epub 2007 Jul 24.
Urinary bladder adenocarcinomas are rare malignancies accounting for approximately 2.5% of all urothelial neoplasms. Intestinal metaplasia of the urothelium indicates the presence of intestinal-type goblet cells and was generally observed to coexist with or to precede the diagnosis of bladder adenocarcinomas. Controversy continues of whether intestinal metaplasia is an acquired precancerous lesion, secondary to different insults to the urothelium, or a concomitant lesion in glandular carcinogenesis. Patients with neurogenic bladders are particularly at risk for developing bladder cancer, mostly squamous cell carcinoma and rarely adenocarcinoma. In these patients, chronic irritation of the urothelium as well as long-term indwelling urinary catheters were the most significant risk factors. Spina bifida is a congenital developmental abnormality that may result in neurogenic bladder. There is only one previously reported case of urothelial carcinoma with associated squamous metaplasia of the bladder occurring in a spina bifida patient. We report the first case of bladder adenocarcinoma associated with intestinal metaplasia occurring in a spina bifida occulta patient. The patient had a complicated clinical course and suffered recurrent urinary tract infections, renal calculi, and urinary incontinence and was managed with intermittent as well as indwelling catheterization.
Urological follow-up of adult spina bifida patients.
Ahmad I, Granitsiotis P. Department of Urology, Southern General Hospital, Glasgow, Scotland, United Kingdom. imranahmad@doctors.net.uk Neurourol Urodyn. 2007;26(7):978-80.
AIMS: The vast majority of the current urological literature understandably has concentrated on the management of children with spina bifida, because in the past the majority did not survive into adulthood. With improvements in the understanding and multidisciplinary care of spina bifida patients it has become a disease of adults. Our aim was to evaluate the current literature to attempt to formulate evidence based guidelines for the management of this difficult group of patients.
METHODS: We reviewed the literature on adult urological management of spina bifida, all relevant articles which concentrated on adults and long-term management were studied in full.
RESULTS: Renal function may begin/continue to deteriorate into adulthood, becoming the leading cause of adult death. This is thought to occur because of changes in the adult bladder, with increases in storage pressure. Medical and surgical management should aim to preserve renal function as well as the maintenance of continence in the face of the growing and changing urinary tract. Follow-up should be regular and in the context of a specialist multidisciplinary clinic. Despite being unvalidated in the follow-up of adult spina bifida patient’s annual serum creatinine, ultrasound and urodynamics are currently the best tools available.
CONCLUSIONS: There is no reason why the majority of spina bifida sufferers cannot use their own kidneys for the rest of their lives. This however relies on urological treatment being instigated soon after birth and continuing into adulthood.