30th ESPU Congress - Lyon, France - 2019

S15: NEUROPATHIC BLADDER 1

Moderators: Rosalia Misseri (USA), Erik Van Laecke (Belgium)

ESPU Meeting on Friday 26, April 2019, 13:50 - 14:56


13:50 - 13:55
S15-1 (LO)

★ ONE HUNDRED CASES OF NEUROGENIC BLADDER AFTER IN-UTERO MMC REPAIR: AN OBSERVATION OF BLADDER PATTERN AND URINARY AND FECAL CONTINENCE.

Antonio MACEDO JR 1, Riberto LIGUORI 2, Gilmar GARRONE 2, Sergio OTTONI 2, Ricardo MATTOS 2 and Marcela LEAL DA CRUZ 2
1) 1. Federal University of São Paulo (UNIFESP) 2. Center for Supporting Children with Urological Anomalies (CACAU), 1. Pediatrics 2. Urology, São Paulo, BRAZIL - 2) Center for Supporting Children with Urological Anomalies - CACAU, Urology, São Paulo, BRAZIL

PURPOSE

To assessthe first one hundred cases ofin uteromyelomeningocele repair in regards to bladder classification and define urinary and fecal continence for patients aged 5 years and over.

MATERIAL AND METHODS

We used a protocol consisting of detailed medical history, urinary tract ultrasound (US), voiding cystourethrography (VCUG) and urodynamic evaluation (UE) to categorize patients into 4patterns: normal, high risk (overactive bladder with DLPP higher than 40 cmH2O), incontinent and underactivity (underactive bladder with post-void residual urine). Patientsaged 5 years and over completed a questionnaire to study aspects of urinary and fecal continence.

RESULTS

We evaluated 100patients at mean age of 5.8 months (median 4 months), classified as high risk in 52.6%, incontinent in 27.4%, with underactive bladder in 4.2% and only 14.7% had a normal bladder profile. Of theses 100 cases, 14 were 5 years or over consisting of 4 (28.6%) males and 10(71.4%) females. Three patients had undergone surgery (2 augmentations and 1 mini-sling urethroplasty). Twelve patients perform CIC(85.7%). Only 3(21.4%) patients had no urinary leakage. Eleven patients(78.6%) use diapers. Eight patients (57,2%) perform retrograde rectal irrigation and 11(78.6%) complained of fecal loss. Eleven patients (78.6%) did not report impair in their  self esteem.

CONCLUSIONS

The high incidence of abnormal bladder patterns suggest poor benefit of in utero MMC surgery towards the urinary tract.  The urinary continence rate is low and 78.6% of patients use diapers.


13:55 - 14:00
S15-2 (LO)

A COMPARATIVE ANALYSIS OF IN UTERO MYELOMENINGOCELE REPAIR VERSUS POSTNATAL SURGERY. IS THERE ANY DIFFERENCE?

João PARIZI 1, Marcela LEAL DA CRUZ 2, Gilmar GARRONE 2, Sérgio OTTONI 2, Riberto LIGUORI 2, Ricardo MATTOS 2 and Antonio MACEDO JR 1
1) 1. Federal University of São Paulo (UNIFESP) 2. Center for Supporting Children with Urological Anomalies (CACAU), 1. Pediatrics 2. Urology, São Paulo, BRAZIL - 2) Center for Supporting Children with Urological Anomalies - CACAU, Urology, São Paulo, BRAZIL

PURPOSE

Myelomeningocele is the result of two factors: failure to close neural tube and continuous injury by exposed neural tissue in intrauterine environment. We aimed to evaluate possible urologic benefits of patients undergoing in utero MMC correction.

MATERIAL AND METHODS

Three groups of patients were analyzed retrospectively in a serial way: Group 1 (fetal MMC repair): 88 patients; Group 2 (MMC correction after birth): 86 patients, and of these, 38 patients, who started follow-up before 12 months of life (Group 3). We looked at urological data, urodynamics, evolution of the bladder pattern and bladder response to treatment.

RESULTS

UTI prevalence was higher in patients operated in the postnatal period (45%) whereas in group 1 it was 20%. Hydronephrosis occurred in 20.7% in Group 1, 22.6% in Group 2 and 28.9% in Group 3 (no statistical significance). The presence of VUR occurred in 15% in all groups. The urodynamic data showed a statistically higher prevalence of bladder hyperactivity in Group 1 compared to Groups 2 and 3 (high-risk bladder pattern in the initial evaluation occurred in 56%, 50% and 46% in groups 1, 2 and 3, respectively). There was a trend to decrease high risk bladder pattern in all groups after treatment, assessed in patients with at least three urodynamic exams. Patients with incontinent bladder and hypocontractility tend to maintain the same bladder pattern over time.

CONCLUSIONS

We confirmed that in utero MMC surgery does not improve urological parameters when compared to patients operated in the postnatal period.


14:00 - 14:03
S15-3 (PP)

EVALUATION OF BLADDER AND BOWEL FUNCTION IN CHILDREN AFTER PRENATAL AND POSTNATAL MYELOMENINGOCELE REPARIR. MIDE-TERM AUTCOMS.

Agnieszka PASTUSZKA 1, Janusz BOHOSIEWICZ 2 and Tomasz KOSZUTSKI 3
1) School of Medicine with Division of Dentistry in Zabrze, Medical University of Silesia in Katowice,, Chair and Department of Descriptive and Topographic Anatomy, Katowice, POLAND - 2) School of Medicine in Katowice, Medical University of Silesia in Katowice,, Clinical Department of Children's Surgery and Urology, Katowice, POLAND - 3) Of Medicine in Katowice, Medical University of Silesia in Katowice, Clinical Department of Children's Surgery and Urology, Katowice, POLAND

PURPOSE

Prenatal myelomeningocele (MMC) closure significantly reduces the need for hydrocephalus shunting and improves lower extremity motor function. Urologic benefits, however, remain to be unclear. The aim of study was to assess mid-term outcomes of bladder and bowel function in children after prenatal and postnatal myelomeningocele repair.

MATERIAL AND METHODS

Clinical data were prospectively collected for all patients who underwent fetal or postnatal MMC repair. Assessments include renal and bladder ultrasound, VCUG, urodynamic study, need for CIC, occurrence of UTI, degree of social urinary continence and constipations.
From the group of 104 patients who underwent prenatal MMC closure from 2006 to 2017, 39 patients with a postnatal follow-up of at least 3 years, were included in this study and compared with age-matched 39 MMC patients who did not undergo fetal surgery. The level of the spina bifida was similar in both groups. All children, regardless of the type of operation, were managed according to the same protocol.

RESULTS

Urodynamic and imaging studies showed no differences between the groups.
The incidence of neurogenic bladder dysfunction was similar. Children from the prenatal group showed statistically significant lower number of UTI in all age groups and had a statistically significant better urine continence in age above 3 years and less frequent constipation when compared to postnatally operated patients (p=0.005).

CONCLUSIONS

Prenatal MMC repair improved the clinical condition of the urinary tract, therefore improving significantly the social urinary continence and reducing the risk of constipation.
Children operated prenatally can achieve in the future better quality of life, self-esteem and independence.


14:03 - 14:12
Discussion
 

14:12 - 14:15
S15-4 (PP)

THE VALUE OF INTRAOPERATIVE NEUROPHYSIOLOGICAL MONITORING IN LIPOMYELOMENINGOCELE REPAIR FOR URINARY AND BOWEL FUNCTION: THE PRELIMINARY FINDINGS OF A PROSPECTIVE LONGITUDINAL STUDY

Cem AKBAL 1, Gülden Demirci OTLUOĞLU 2, Müge KOÇAK 3, Elif Ilgaz AYDINLAR 4, Ahmet ŞAHIN 1 and M. Memet ÖZEK 5
1) Acıbadem Mehmet Ali Aydınlar University, Urology, Istanbul, TURKEY - 2) Acıbadem Health Group, Pediatric Neurosurgery, Istanbul, TURKEY - 3) Acıbadem Health Group, Neurology, Istanbul, TURKEY - 4) Acıbadem Mehmet Ali Aydınlar University, Neurology, Istanbul, TURKEY - 5) Acıbadem Mehmet Ali Aydınlar University, Pediatric Neurosurgery, Istanbul, TURKEY

PURPOSE

Lipomyelomeningocele (LMM) is the one of the commenest forms of spinal dysraphism. Because LMM and Myelomeningocele (MM) are different in many ways, it is important to separately assess the long-term urologic outcomes of LMM from MM, in order to incorporate realistic continence and bladder management scenarios when counseling families. Many individuals affected by LMM retain a higher functional status than individuals born with Myelomeningocele (MM), and acceptance of Clean Intermitant Catheterisation (CIC) has been difficult in some LMM patients. Intraoperative neurophysiological monitoring (IONM) in lipomyelomeningocele repair was developed to identify the functional nervous structure of the urinary tract and continuously ensure the integrity of the sacral motor roots, thereby protecting bladder and bowel function. In the current study the surgical outcomes with IONM of 13 patients, and in particular those pertaining to urinary and bowel function, were prospectively analyzed in this study. 

MATERIAL AND METHODS

Lipomyelomeningocele repair and duraplasty were performed for all cases by a single surgeon in a 1.5-year period, i.e., between 2016 and 2018. Standard IONM was applied, comprising motor-evoked potentials obtained by transcranial electrical stimulation, bulbocavernosus reflex, or direct nerve root stimulation. Three-monthly postoperative urinary ultrasound results were recorded, along with the number of postoperative urinary tract infections (UTIs). Constipation was evaluated using the Bristol Stool Scale.

RESULTS

IONM responses were obtained for all patients, except one. Temporary intraoperative nerve deterioration was identified in two patients, one of whom experienced ≥ 2 UTI/year and need CIC after one year of operation. Constipation was seen in two patients. Upper urinary tract deterioration was not evident in any patients on ultrasound. 

CONCLUSIONS

The use of IONM is feasible in lipomyelomeningocele patients. IONM helps to ensure the short-term safety of the urinary tract through the continuous regulation of urinary tract function and ensuring the integrity of the sacral nerve roots.


14:15 - 14:18
S15-5 (PP)

BLADDER FUNCTION OUTCOMES IN LUMBOSACRAL LIPOMA SUBTYPES

Neetu KUMAR 1, Susan MAESTRI 1, Navroop JOHAL 1, Dominic THOMPSON 2 and Divyesh DESAI 1
1) Great Ormond Street Hospital, Paediatric Urology, London, UNITED KINGDOM - 2) Great Ormond Street Hospital, Paediatric Neurosurgery, London, UNITED KINGDOM

PURPOSE

Lumbosacral lipoma (LSL) is a common pathology within the spectrum of spinal dysraphisms. There is paucity of bladder functional outcomes in this cohort of patients. We aim to evaluate the bladder function outcomes against LSL subtypes.

MATERIAL AND METHODS

A single centre prospective database of patients born from 2005 to 2010 was used. The lipomas had been primarily classified into dorsal, caudal or transitional types (in relation to the conus medullaris). Other subtypes included were the filum terminale and chaotic lipomas (fat extending anterior to the spinal cord). Bladder Function Assessments (BFA) had been periodically carried out, 6-monthly until the child was toilet trained and then annually. The age matched control group for this study was the standardised International Children’s Continence Society (ICCS) nomograms with post void residual. Outcomes were measured in terms of a normal or abnormal BFA.

RESULTS

Forty-two patients, 12 boys and 30 girls were included. Median age was 10 years (range 7-12 years) with a mean follow up of 9.5 years.  The table below correlates BFAs with LSL subtypes. Since the observed outcomes were better in the dorsal lipomas compared to other subtypes, a Pearson Chi-Square test was conducted to test for significance in this observed difference (P value was <0.05).

Type of LSL

Normal BFA

Age 5 years or more

Abnormal BFA

Age 5 years or more

Dorsal (n=8)

6

2 (25%)

Caudal (n=9)

6

3 (33%)

Transitional (n=21)

7

14 (66%)

Others (n=4)

1

3 (75%)

CONCLUSIONS

Dorsal Lumbosacral lipomas have better bladder function outcomes followed by the caudal subtype. The majority of transitional (two-thirds), filar and chaotic types have poor bladder function outcomes. These observations were constant irrespective of conservative monitoring or surgical untethering.  Our findings may be useful in counselling families and discussing management options.


14:18 - 14:21
S15-6 (PP)

IMPACT OF HYPNOSIS ON INTERMITTENT CATHETERIZATION AND URODYNAMIC STUDIES IN CHILDREN: A PRELIMINARY STUDY

Eliane JOSSET RAFFET, Lise NATIO, Marie Elise JEREMIE, Alaa ELGHONEIMI and Annabel PAYE JAOUEN
Robert Debré University Hospital, AP-HP, University Paris Diderot. Centre de référence de Malformations Rares des voies Urinaires (MARVU), Paediatric Urology, Paris, FRANCE

PURPOSE

Clean Intermittent catheterization (CIC) and urodynamic studies (UDS) in children could be painful and stressful. Our objective was to assess the impact of hypnosis on acceptance and the realisation of the studies or the CIC.

MATERIAL AND METHODS

The study was conducted prospectively in our centre between June 2016 and June 2018. Children needing CIC were included. Hypnosis sessions were coordinated by therapeutic education (TPE) team at the outpatient clinic. Children with Mitrofanoff, psychiatric trouble, not speaking English or French were excluded. For CIC: feasibility of the catheterization, number of hypnosis and TPE sessions, anxiety and pain (self or hetero assessment by digital scale) were collected at the first day, one week, one month and six months. For UDS, we assessed feasibility of the study, anxiety and pain, children had only one hypnosis session.

RESULTS

31 children (9 girls, 22 boys) mean age 9 years (1-17) were included. 14 children had CIC, 57% were anxious or painful on first session. At one month, CIC was done daily by 100% of parents or children. Anxiety and pain disappeared in 92% of patients. Six months later, no patient had pain. 17 patients had hypnosis session during UDS. The study was feasible for all. 35% were anxious and 41% had pain.

CONCLUSIONS

Our preliminary study showed the feasibility of hypnosis in the daily practice of the outpatient clinic of pediatric urology. Hypnosis seems to improve the acceptance rate for CIC. UDS under hypnosis was also feasible. These results need to be validated compared to control groups. In our daily practice, its use is also extended to another indications.


14:21 - 14:24
S15-7 (PP)

VALIDATION OF A NEW SCORING SYSTEM (UNION) FOR THE CLINICAL ASSESMENT AND FOLLOW-UP OF SPINA BIFIDA PATIENTS

Ilker Zeki ARUSOGLU 1, Ibrahim ULMAN 1, Sibel TIRYAKI 1, Hasan CAYIRLI 1, Ali AVANOGLU 1, Huseyin GUNAY 2, Tuncer TURHAN 3 and Kazim CAPACI 4
1) EGE UNIVERSITY FACULTY OF MEDICINE, PEDIATRIC SURGERY, PEDIATRIC UROLOGY, Izmir, TURKEY - 2) EGE UNIVERSITY FACULTY OF MEDICINE, ORTHOPEDICS AND TRAUMATOLOGY, Izmir, TURKEY - 3) EGE UNIVERSITY FACULTY OF MEDICINE, BRAIN AND NERVE SURGERY, Izmir, TURKEY - 4) EGE UNIVERSITY FACULTY OF MEDICINE, PHYSICAL MEDICINE AND REHABILITATION, Izmir, TURKEY

PURPOSE

Efficacy of specialized clinics in the follow-up of spina bifida patients have been documented. But a scoring system for overall evaluation of these patients for different specialties is lacking. The objective of this study was to develop, to test clinically, and to validate a new scoring system easily applicable and reproducible in the follow-up evaluation of spina bifida patients.

MATERIAL AND METHODS

In this prospective study; different specialists designed a simple, objective, convenient assessment tool to evaluate spina bifida patients during their clinic visits. Five systems and functional status affected frequently in spina bifida; urinary, neurologic, intellectual, orthopedic, and nutritional status were separately scored in four different grades of clinical severity by different specialists and the parents (UNION Score). The scores obtained from various evaluators were compared statistically using Cronbach's alpha reliability test.

RESULTS

One hundred twenty two patients were scored separately by five evaluators. The scores for each patient demonstrated a high correlation among evaluators (Alpha=0.966). The highest correlation was noted between pediatric urologist and neurosurgeon (Alpha=0.941). The correlation between family scores and the scores of healthcare givers was also noteworthy (Alpha=0.804-0.917).

CONCLUSIONS

The new scoring system was easy to use and it was convenient among evaluators. Changes in the scores during follow-up of spina bifida patients may help warning patients, parents, and health care providers. It also may ease evaluation of spina bifida patients between different specialties. Acquaintance of scores between family and medical group may be important to motivate the patients socially, and increase their compliance during follow-up.


14:24 - 14:27
S15-8 (PP)

CLINICAL PROPERTIES OF SPINA BIFIDA PATIENTS WITH JARCHO LEVIN SYNDROME

S. Kerem OZEL 1, Ibrahim ALATAS 2, Huseyin CANAZ 2 and Rukiye CAYMAZ 2
1) Istanbul Medeniyet University Faculty of Medicine, Paediatric Surgery, Istanbul, TURKEY - 2) Istanbul Bilim University Spina Bifida Center, Paediatric Neurosurgery, Istanbul, TURKEY

PURPOSE

Jarcho Levin Syndrome (JLS) is diagnosed when vertebral and costal anomalies are associated with spina bifida (SB). The aim of this study was to delineate the clinical properties of JLS.

MATERIAL AND METHODS

Patients treated in our Spina Bifida Center were enrolled retrospectively. Patients who had diagnosis of JLS and randomly selected SB patients were compared. Age, gender, presence of VP shunt, urinary tract infection, clean intermittent catheterization (CIC), constipation, anticholinergic usage, presence of hydronephrosis, renal scarring, vesicoureteral reflux, bladder wall thickness, bladder capacity, compliance, detrusor activity, sphincter activity and residual urine were compared with student’s t test and chi square test.

RESULTS

A total 60 patients were diagnosed as JLS among 900 SB patients. Randomly selected 100 patients were chosen for comparison. JLS was found in girls in a rate of  40%, in SB 17%  (p=0,001), in JLS need for VP shunt was 41,6%, in SB 22% (p=0,01), CIC was necessary in 30% of JLS  and 36% in SB (p=0,045), anticholinergic was used in 25% of JLS and 29% in SB (p=0,026). Mean bladder wall thickness was 2,75±1,29 mm in JLS and 3,38±1.1mm in SB (p=0,05).

CONCLUSIONS

This is the largest case series of JLS in the literature. Female gender is more prominent in JLS and more VP shunting may be needed in these patients. More need for CIC, anticholinergic and increased bladder wall thickness in SB may show that bladder is more affected in SB. It is possible to say that JLS may affect  bladder less from urological point of view.


14:27 - 14:30
S15-9 (PP)

★ CLINICAL AND URODYNAMIC OUTCOMES OF SECONDARY UNTETHERING IN SPINA BIFIDA PATIENTS.

Beatriz BANUELOS MARCO 1, Luise CIESLA 2, Ulrich SEIDL 2, Angella KAINDL 3, Ulrich-Wilhem THOMALE 4, Joanna SCHNEIDER 5 and Anja LINGNAU 6
1) Charité-Universitätsmedizin Berlin, Kinderurologie/Paediatric Urology, Berlin, GERMANY - 2) Charité-Universitätsmedizin Berlin, Sozialpädiatrisches Zentrum, Neuropädiatrie, Berlin, GERMANY - 3) Charité-Universitätsmedizin Berlin, Klinik für Pädiatrie m.S. Neurologie, Berlin, GERMANY - 4) Charité-Universitätsmedizin Berlin, Klinik für pädiatrische Neurochirurgie, Berlin, GERMANY - 5) Charité-Universitätsmedizin Berlin, Institut für Zell- und Neurobiologie, Berlin, GERMANY - 6) Charité-Universitätsmedizin Berlin, Kinderurologie/Paediatric Urology, Berlin, GERMANY

PURPOSE

Secondary tethering of the spinal cord (TSC) can lead to worsening of bladder and lower extremity function (LEF). The indications and outcomes of are seldom described in the literature.

MATERIAL AND METHODS

A retrospective study on a cohort of 306 spina bifida patients was performed. Patients who underwent a video urodynamics 12 months before and after the procedure were included. We analyzed the indication for detethering as well as bladder capacity, detrusor pressure, leak point pressure and compliance. The degree of severity was assigned according to the Abrahamsson classification (2007) and the modified Hostility Score (AWMF guideline). The indications leading to surgical intervention included worsening of the bladder function resistant to therapy and/or of LEF and adhesiolysys before spondylolisthesis.

RESULTS

58 patients fulfilled the inclusion criteria (n=6 spina bifida occulta). Mean age was 9,1 years at the time of the operation (range 0,5-22,5). The indications for surgery were worsening of the bladder function in 10 patients(17,2%), worsening of LEF in 26 (44,8%), worsening of both bladder and LEF in 17(29,3%) and other in 5 (8,6%).
Bladder function before and after the operation, improved as frequently as it worsened, each 11 (19%), no changes are observed in 36 (62%). In contrast, the hostility score improved in 27 cases (46,6%) , worsened in 17 (29,3%) and remained unchanged in 14 (24,1%). Most of the patients with a postoperative worsening of the bladder function had been operated due to worsening LEF.

CONCLUSIONS

We conclude that the hostility score can be improved in 46% of the cases after detethering but in patients operated solely for LEF changes the operation tended to worsen bladder function.


14:30 - 14:33
S15-10 (PP)

APPENDICOVESICOSTOMY USING SPLIT APPENDIX TECHNIQUE HAS HIGHER RATE OF COMPLICATION COMPARED TO INTACT APPENDIX OR MONTI CHANNEL

Anja ZANN 1, Yuri SEBASTIAO 1, Christina CHING 1, Daniel DAJUSTA 1 and Venkata JAYANTHI 2
1) Nationwide Children's Hospital, Urology, Columbus, USA - 2) Nationwide Children's Hospital, Section of Urology, Columbus, USA

PURPOSE

Many patients with neurogenic bowel/bladder require an appendicovesicostomy for bladder management and a Malone for antegrade bowel management.  The split appendix technique enables the use of this organ for both procedures, obviating the need for a bowel anastomosis. We compared complication rates of the split appendix Mitrofanoff vs use of an intact appendix or a Monti channel.

MATERIAL AND METHODS

We retrospectively reviewed consecutive patients who underwent a continent catheterizable channel . We recorded demographics, diagnosis, body mass index, stoma location, surgical technique and outcomes.  

RESULTS

From Jan 2014-July 2018, 109 patients underwent creation of a continent catheterizable channel Mitrofanoff procedure. 86 (79%)  also underwent a Malone. Median age was 7 years; 66 (60%) were females. Intact appendices were used in 48 (44%), a Monti channel in 26 (24%), and a split-appendix technique in 35 (32.1%). Overall, 37 (34%) patients had complications, of which 27 (25%) required surgical revision. The complication rate requiring surgical revision was 17%, 23%, and 37% for intact appendix, Monti, and split appendix, respectively.  After multivariable analysis, the only independent factor associated with an increased risk of revision was split appendix technique. There was no significant difference between intact appendix and Monti groups.

CONCLUSIONS

Our data suggest that there is an increased risk of complication and need for secondary surgery in children who undergo a Mitrofanoff procedure using a split appendix technique.  Prospective studies, objectively considering the length of the appendix may be helpful in determining which patients may safely undergo a split appendix procedure.


14:33 - 14:56
Discussion