the standardization of terminology of lower urinary tract ... · ing of iccs terminology. despite...

The Standardization of Terminology of Lower UrinaryTract Function in Children and Adolescents: UpdateReport from the Standardization Committee of theInternational Children’s Continence Society

Paul F. Austin,*,† Stuart B. Bauer, Wendy Bower, Janet Chase,Israel Franco,‡ Piet Hoebeke, Søren Rittig, Johan Vande Walle,§Alexander von Gontard, Anne Wright,jj Stephen S. Yangand Tryggve Nev!eus

From the Division of Urology, Washington University in St. Louis, St. Louis Children’s Hospital, St. Louis, Missouri (PFA),Department of Urology, Children’s Hospital and Harvard Medical School, Boston, Massachusetts (SBB), Pediatrics(Nephrology Section), Skejby University Hospital, Aarhus, Denmark (WB, SR), The Children’s Centre, Cabrini Hospital,Melbourne, Australia (JC), New York Medical College, Valhalla, New York (IF), Pediatric Urology and Nephrology,Gent University Hospital, Ghent, Belgium (PH, JVW), Department of Child and Adolescent Psychiatry, SaarlandUniversity Hospital, Homburg, Germany (AvG), Pediatrics, Evelina Children’s Hospital, St Thomas’ Hospital, London,England (AW), Division of Urology, Taipei Tzu Chi Hospital, The Buddhist Medical Foundation, New Taipei, and Schoolof Medicine, Buddhist Tzu Chi University, Hualien, Taiwan (SSY), and Section of Paediatric Nephrology, Departmentof Women’s and Children’s Health, Uppsala University, Uppsala, Sweden (TN)

Purpose: The impact of the original International Children’s Continence Societyterminology document on lower urinary tract function resulted in the globalestablishment of uniformity and clarity in the characterization of lower urinarytract function and dysfunction in children across multiple health care disciplines.The present document serves as a stand-alone terminology update reflectingrefinement and current advancement of knowledge on pediatric lower urinarytract function.

Materials and Methods: A variety of worldwide experts from multiple disciplinesin the ICCS leadership who care for children with lower urinary tract dysfunc-tion were assembled as part of the standardization committee. A critical reviewof the previous ICCS terminology document and the current literature wasperformed. In addition, contributions and feedback from the multidisciplinaryICCS membership were solicited.

Results: Following a review of the literature during the last 7 years the ICCSexperts assembled a new terminology document reflecting the current under-standing of bladder function and lower urinary tract dysfunction in childrenusing resources from the literature review, expert opinion and ICCS memberfeedback.

Conclusions: The present ICCS terminology document provides a current andconsensus update to the evolving terminology and understanding of lowerurinary tract function in children. For the complete document visit http://jurology.com/.

Key Words: terminology, consensus, child, urinary bladder,urination disorders

Abbreviationsand Acronyms

ICCS ¼ International Children’sContinence Society

LUT ¼ lower urinary tract

Accepted for publication January 28, 2014.The complete report is available at http://

jurology.com/.* Correspondence: Pediatric Urology,

Washington University School of Medicine, 4990Children’s Place, Suite 1120, Campus Box 8242,Saint Louis, Missouri 63110-1077 (telephone:314-454-6034; e-mail: [email protected]).

† Financial interest and/or other relationshipwith Allergan and Warner-Chilcott.

‡ Financial interest and/or other relationshipwith Astellas and Allergan.

§ Financial interest and/or other relationshipwith Astellas and Ferring.

jj Financial interest and/or other relationshipwith Ferring Pharmaceuticals.

0022-5347/14/1916-1863/0THE JOURNAL OF UROLOGY®

© 2014 by AMERICAN UROLOGICAL ASSOCIATION EDUCATION AND RESEARCH, INC.

http://dx.doi.org/10.1016/j.juro.2014.01.110Vol. 191, 1863-1865, June 2014

Printed in U.S.A.www.jurology.com j 1863

THE standardization of terminology for pediatricbladder and bowel function is critical in providinga platform for optimal understanding, communica-tion and treatment across multiple health careproviders who care for children and adolescentswith LUT dysfunction. Terminology that is appli-cable internationally is particularly pertinent dueto the global prevalence of pediatric LUT dysfunc-tion and the numerous specialists who treat thesechildren and adolescents. LUT dysfunction is abroad term that encompasses subsets of LUTdysfunction with different manifestations. Theheterogeneity of symptoms is at times overlappingand at other times unique to the subsets of LUTdysfunction. Thus, universally accepted terminol-ogy of pediatric LUT dysfunction is imperative toreduce confusion among providers. Standardizedterms are also critical for comparing research andstudy outcomes to optimally promote the investi-gative understanding of pediatric LUT dysfunction.

The ICCS is a unique organization whose mem-bers comprise multiple disciplines and specialtiesfrom almost every continent who care for childrenwith bladder and bowel incontinence. Thus, theICCS is uniquely positioned to provide guidance inthe standardization of terminology for bladder andbowel dysfunction in children and adolescents.

During the last decade the second report fromthe Standardization Committee of the ICCS1 haspropagated definitions and established standard-ized terminology that allowed for clarity ofcommunication. The impact of the ICCS proposedterminology on the body of literature of pediatricLUT function has been evaluated.2 The importanceof pediatric urinary incontinence is supported bythe finding of a 49% increase in publications from2002e2005 to 2007e2010 (55 to 82 per year) thatfocus on pediatric LUT function. Additionally, therewas approximately a fourfold increase in the likeli-hood of use of ICCS recommended terminologiesafter ICCS guideline publication (OR 4.19, 95% CI3.04e5.78, p <0.001). It is noteworthy that therewas no significant geographical variation in adopt-ing of ICCS terminology. Despite this significantimpact of the global use of ICCS terminology,approximately 25% of studies published between2007 and 2010 contained obsolete terminologies.2

Similar to the dynamic flux of knowledge andunderstanding within medicine, the terminologyfor pediatric bladder and bowel function isdynamic. This document on ICCS terminology forpediatric bladder and bowel function serves as astand-alone terminology update reflecting refine-ment and advancement of knowledge on thesesystems. Adherence to the updated terminology isfollowed at all ICCS courses and workshops, andit is encouraged that all investigators and clinicians

who publish on this topic use the ICCS recom-mended terminology. To delineate manuscriptsand publications that follow the ICCS guidelinesregarding terminology we recommend future man-uscripts include the text, “Terminology adheres tostandards recommended by the ICCS except wherespecifically noted.”

MATERIALS AND METHODSA variety of worldwide experts from multiple disciplineswho care for children with LUT dysfunction were assem-bled. The standardization committee consisted of activemembers and leaders of the ICCS who have publishedextensively on several facets of bladder and boweldysfunction and all of the ICCS documents published inthe last 4 years. Health care disciplines included urology,nephrology, gastroenterology, general and developmentalpediatrics, physical therapy, psychology and psychiatry.The standardization committee came from North andSouth America, Europe, the Middle East, Africa,Australia and Asia. A critical review of the original ICCSterminology document and the current literature wasperformed. Additionally, input from the multidisciplinaryICCS membership was solicited.

This terminology document represents the 3rd pub-lished standardization on terminology for LUT functionand enhances previous ICCS documents.1,3 Recognitionand reference to the terminology on LUT function by theInternational Continence Society4 as well the joint ter-minology for female pelvic floor dysfunction by the Inter-national Urogynecological Association and InternationalContinence Society5 were used to be current and inclusiveof other global organizations and disciplines that also dealwith continence. In addition, terms and definitions usedby the Fifth Edition of the Diagnostic and StatisticalManual of Mental Disorders6,7 were considered and theICD-10 medical classification list from the World HealthOrganization8 was referenced.

This update is not intended to serve as a guidelinefor clinical treatment. There are numerous previousICCS documents outlining treatment for specific LUTand associated comorbid conditions.9e16 This terminol-ogy update follows the prior ICCS terminology outlineof establishing syntax to properly convey symptoms ofLUT dysfunction, and to affirm terminology for inves-tigative tools, signs, conditions and treatment parame-ters as they pertain to LUT function and dysfunction.The reader is referred to the prior ICCS communica-tions for a comprehensive description of the patho-physiology. We have updated the relevance of age tobladder and bowel function, and discuss the common-ality of bowel emptying issues with bladder function.We recognize that we are an organization whose pri-mary expertise is in urinary continence and bladderfunction but also acknowledge a close relationship be-tween bowel and bladder function. Thus, the importanceof bowel related terms in relation to bladder functionis emphasized.

The complete report is available at http://jurology.com/.

1864 ICCS TERMINOLOGY FOR PEDIATRIC LOWER URINARY TRACT FUNCTION

REFERENCES1. Nev!eus T, von Gontard A, Hoebeke P et al: The

standardization of terminology of lower urinarytract function in children and adolescents: reportfrom the Standardisation Committee of the In-ternational Children’s Continence Society. J Urol2006; 176: 314.

2. Dannaway J, Ng H and Deshpande AV:Adherence to ICCS nomenclature guidelines insubsequent literature: a bibliometric study.Neurourol Urodyn 2013; 32: 952.

3. Nørgaard JP, van Gool JD, Hj€alm#as K et al:Standardization and definitions in lower urinarytract dysfunction in children. International Chil-dren’s Continence Society. Br J Urol 1998; 81: 1.

4. Abrams P, Cardozo L, Fall M et al: The stand-ardisation of terminology of lower urinary tractfunction: report from the StandardisationSub-committee of the International ContinenceSociety. Neurourol Urodyn 2002; 21: 167.

5. Haylen BT, de Ridder D, Freeman RM et al:An International Urogynecological Association(IUGA)/International Continence Society (ICS)joint report on the terminology for female pel-vic floor dysfunction. Neurourol Urodyn 2010;29: 4.

6. Fifth Edition of the Diagnostic and StatisticalManual of Mental Disorders (DSM-5), 2013.Available at www.dsm5.org.

7. von Gontard A: The impact of DSM-5 andguidelines for assessment and treatment ofelimination disorders. Eur Child Adolesc Psychi-atry, suppl., 2013; 22: S61.

8. Chase J, Austin P, Hoebeke P et al: The man-agement of dysfunctional voiding in children:a report from the Standardisation Committeeof the International Children’s Continence Soci-ety. J Urol 2010; 183: 1296.

9. Hoebeke P, Bower W, Combs A et al: Diagnosticevaluation of children with daytime inconti-nence. J Urol 2010; 183: 699.

10. Neveus T, Eggert P, Evans J et al: Evaluationof and treatment for monosymptomatic enuresis:a standardization document from the Interna-tional Children’s Continence Society. J Urol2010; 183: 441.

11. von Gontard A, Baeyens D, Van Hoecke E et al:Psychological and psychiatric issues in urinaryand fecal incontinence. J Urol 2011; 185: 1432.

12. Bauer SB, Austin PF, Rawashdeh YF et al:International Children’s Continence Society’srecommendations for initial diagnostic evaluation

and follow-up in congenital neuropathic bladderand bowel dysfunction in children. NeurourolUrodyn 2012; 31: 610.

13. Rawashdeh YF, Austin P, Siggaard C et al:International Children’s Continence Society’srecommendations for therapeutic intervention incongenital neuropathic bladder and boweldysfunction in children. Neurourol Urodyn 2012;31: 615.

14. Burgers RE, Mugie SM, Chase J et al: Man-agement of functional constipation in childrenwith lower urinary tract symptoms: report fromthe Standardization Committee of the Interna-tional Children’s Continence Society. J Urol2013; 190: 29.

15. Franco I, von Gontard A, De Gennaro M et al:Evaluation and treatment of nonmonosymptomaticnocturnal enuresis: a standardization documentfrom the International Children’s ContinenceSociety. J Pediatr Urol 2013; 9: 234.

16. World Health Organization: Multiaxial Classifi-cation of Child and Adolescent PsychiatricDisorders: The ICD-10 Classification of Mentaland Behavioural Disorders in Children andAdolescents. Cambridge: Cambridge UniversityPress 2008; pp viii, 302.

ICCS TERMINOLOGY FOR PEDIATRIC LOWER URINARY TRACT FUNCTION 1865

The Standardization of Terminology of Lower Urinary Tract Functionin Children and Adolescents: Update Report from the StandardizationCommittee of the International Children’s Continence Society

Paul F. Austin,* Stuart B. Bauer, Wendy Bower, Janet Chase, Israel Franco, Piet Hoebeke,Søren Rittig, Johan Vande Walle, Alexander von Gontard, Anne Wright, Stephen S. Yangand Tryggve Nev!eus

From the Division of Urology, Washington University in St. Louis, St. Louis Children’s Hospital, USA (PFA), Department of Urology, Children’s Hospitaland Harvard Medical School, Boston, USA (SBB), Pediatrics (Nephrology Section), Skejby University Hospital, Aarhus, Denmark (WB, SR), The Children’s Centre,Cabrini Hospital, Melbourne, Australia (JC), New York Medical College, USA (IF), Pediatric Urology and Nephrology, Gent University Hospital, Ghent, Belgium,(PH, JVdW), Department of Child and Adolescent Psychiatry, Saarland University Hospital, Germany (AvG), Pediatrics, Evelina Children’s Hospital, St Thomas’Hospital, London, England (AW), Division of Urology, Taipei Tzu Chi Hospital, The Buddhist Medical Foundation, New Taipei, Taiwan and School of Medicine,Buddhist Tzu Chi University, Hualien, Taiwan (SSY) and Section of Paediatric Nephrology, Department of Women’s and Children’s Health, Uppsala University,Uppsala, Sweden (TN).

Purpose: The impact of the original International Children’s Continence Society(ICCS) terminology document on lower urinary tract (LUT) function resultedin the global establishment of uniformity and clarity in the characterization ofLUT function and dysfunction in children across multiple healthcare disciplines.The present document serves as a stand-alone terminology update reflectingrefinement and current advancement of knowledge on pediatric LUT function.

Materials and Methods: A variety of worldwide experts from multiple disciplineswithin the ICCS leadership who care for children with LUT dysfunction wereassembled as part of the standardization committee. A critical review of theprevious ICCS terminology document and the current literature was performed.Additionally, contributions and feedback from the multidisciplinary ICCSmembership were solicited.

Results: Following a review of the literature over the last 7 years, the ICCSexperts assembled a new terminology document reflecting current understand-ing of bladder function and LUT dysfunction in children using the resources fromthe literature review, expert opinion and ICCS member feedback.

Conclusions: The present ICCS terminology document provides a currentand consensus update to the evolving terminology and understanding of LUTfunction in children.

Key words: terminology; consensus; child; urinary bladder/physiology;urination Disorders

INTRODUCTIONTHE standardization of terminologyfor pediatric bladder and bowel func-tion is critical in providing a platformfor optimal understanding, commu-nication and treatment across multi-ple health care providers who care forchildren and adolescents with LUTdysfunction. Terminology that is

applicable internationally is particu-larly pertinent due to the globalprevalence of pediatric LUT dysfunc-tion and the numerous specialistswho treat these children and adoles-cents. LUT dysfunction is a broadterm that encompasses subsets ofLUT dysfunction with differentmanifestations. The heterogeneity of

* Corresponding Author: Paul F. Austin, Washington University School of Medicine, 4990 Children’s Place, Suite 1120, Campus Box 8242,Pediatric Urology, Saint Louis, MO 63110-1077, Phone Number: 314-454-6034; e-mail: [email protected].

Abbreviations Key

ADHD ¼ attention deficithyperactivity disorder

BBD ¼ bladder bowel dysfunction

BC ¼ bladder capacity

BOO ¼ bladder outlet obstruction

CBCL ¼ Child Behavior Checklist(CBCL)

DSD ¼ detrusor sphincterdysfunction

DSM-5 ¼ Fifth Edition of theDiagnostic and Statistical Manualof Mental Disorders

DVSS ¼ Dysfunctional VoidingSymptom Score

EBC ¼ expected bladder capacity

EMG ¼ electromyography

ICCS ¼ International Children’sContinence Society

ICD-10 ¼ InternationalClassification of Diseases-10

ICS ¼ International ContinenceSociety

IUGA ¼ InternationalUrogynecological Association

LPP ¼ leak point pressure

LUT ¼ lower urinary tract

MVV ¼ maximum voided volume

OAB ¼ overactive bladder

PVR ¼ post void residual

1865.e1 ICCS TERMINOLOGY FOR PEDIATRIC LOWER URINARY TRACT FUNCTION

symptoms is at times overlapping and at other timesunique to the subsets of LUT dysfunction. Univer-sally accepted terminology of pediatric LUTdysfunction is thus imperative to reduce confusionamong providers. Standardized terms are also crit-ical for comparing research and study outcomes tooptimally promote investigative understanding ofpediatric LUT dysfunction.

The ICCS is a unique organization whose mem-bers comprise multiple disciplines and specialtiesfrom almost every continent that care for childrenwith bladder and bowel incontinence. Thus, the ICCSis uniquely positioned to provide guidance in thestandardization of terminology for bladder and boweldysfunction (BBD) in children and adolescents.

Over the last decade, the second report from theStandardization Committee of the ICCS1 has prop-agated definitions and established standardizedterminology that allowed for clarity of communica-tion. The impact of the ICCS-proposed terminologyon the body of literature of pediatric LUT functionhas been evaluated.2 The importance of pediatricurinary incontinence is supported by the finding of a49% increase in publications from 2002e2005 to2007e2010 (55 to 82 per year) that focus on pedi-atric LUT function. Additionally, there wasapproximately a fourfold increase in the likelihoodof usage of ICCS recommended terminologies post-ICCS guideline publication (OR: 4.19, 95% CI:3.04e5.78, P < 0.001). It is noteworthy that therewas no significant geographical variation in adopt-ing of ICCS terminology. Despite this significantimpact of the global usage of ICCS terminology,approximately 25% of studies published between2007 and 2010 contained obsolete terminologies.2

Similar to the dynamic flux of knowledge andunderstanding within medicine, the terminology forpediatric bladder and bowel function is dynamic.This document on ICCS terminology for pediatricbladder and bowel function serves as a stand-aloneterminology update reflecting refinement andadvancement of knowledge on these systems.Adherence to the updated terminology is followed atall ICCS courses and workshops and it is encour-aged that all investigators and clinicians who pub-lish on this topic utilize the ICCS recommendedterminology. To delineate manuscripts and publi-cations that follow the ICCS guidelines regardingterminology we recommend future manuscriptsinclude the text “Terminology adheres to standardsrecommended by the ICCS except where specif-ically noted”.

MATERIALS AND METHODSA variety of worldwide experts from multiple disciplineswho care for children with LUT dysfunction were

assembled. The standardization committee consisted ofactive members and leaders of the ICCS that haveextensively published on several facets of BBD and all ofthe ICCS documents published in the last 4 years.Healthcare disciplines included urology, nephrology,gastroenterology, general and developmental pediatrics,physical therapy, psychology and psychiatry. The stan-dardization committee emanated from North and SouthAmerica, Europe, the Middle East, Africa, Australia andAsia. A critical review of the original ICCS terminologydocument and the current literature was performed.Additionally, input from the multidisciplinary ICCSmembership was solicited.

This terminology document represents the 3rd pub-lished standardization on terminology for LUT functionand enhances previous ICCS documents1,3. Recognitionand reference to the terminology on LUT function by theInternational Continence Society (ICS)4 as well the jointterminology for female pelvic floor dysfunction by the In-ternational Urogynecological Association (IUGA) andICS5 were employed to be current and inclusive of otherglobal organizations and disciplines that also deal withcontinence. Additionally, terms and definitions employedby the new Fifth Edition of the Diagnostic and StatisticalManual of Mental Disorders (DSM-5)6,7 were consideredand the ICD-10 medical classification list from the WorldHealth Organization8 was referenced.

This update is not intended to serve as a guideline forclinical treatment. There are numerous previous ICCSdocuments outlining treatment for specific LUT andassociated co-morbid conditions.9e16 This terminologyupdate follows the prior ICCS terminology outline ofestablishing syntax to properly convey symptoms of LUTdysfunction and to affirm terminology for investigativetools, signs, conditions and treatment parameters as theypertain to LUT function and dysfunction. The reader isreferred to the prior ICCS communications for a compre-hensive description of the pathophysiology. We haveupdated the relevance of age to bladder and bowel func-tion and discuss the commonality of bowel emptying is-sues with bladder function. We recognize that we are anorganization whose primary expertise is in urinarycontinence and bladder function but equally acknowledgea close relationship between bowel and bladder function.Thus, the importance of bowel related terms in relation tobladder function as emphasized.

TERMINOLOGY

Bladder and bowel dysfunction (BBD)Due to the aforementioned relationship betweenthe bladder and bowel, concomitant bladder andbowel disturbances have been labeled as BBD. Wediscourage using the term dysfunctional eliminationsyndrome (DES) as this connotes a particular ab-normality or condition. We recommend BBD as amore descriptive comprehensive term of a combinedbladder and bowel disturbance that does not explainpathogenesis but rather encompasses this paralleldysfunction. BBD is an umbrella term that can be

ICCS TERMINOLOGY FOR PEDIATRIC LOWER URINARY TRACT FUNCTION 1865.e2

subcategorized into LUT dysfunction and boweldysfunction. (Figure 1).

When the term dysfunction or disorder is used, itrepresents clinical significance and relevance. In aresearch document or reference, authors shouldspecify and provide support for using the term BBD.In the absence of any co-morbid bowel dysfunction,the term LUT dysfunction alone suffices.

Symptomatic termsSymptoms are classified according to their relationto the storage and/or voiding phase of bladderfunction. Although a symptom may occur only onceor rarely, this does not necessarily make it a con-dition. Symptoms are variable and duration of asymptom may alter the perception of its relevance.Nevertheless, duration of time is beneficial incharacterizing symptoms.

Terminology used for LUT symptoms will focuson descriptive rather than quantitative language, asquantitative data to define symptomatic terms islacking. Age of the child is particularly relevantwhen applying terminology for pediatric bladderfunction. Our reference point for LUT symptoms is" 5 years of age as this age is used by the DSM-5and the International Classification of Diseases-10(ICD-10) to characterize urinary incontinence dis-orders.6,8 For functional bowel dysfunction theminimum age is 4.0 years. We recognize the vari-ability and maturational aspect of LUT function17

and fully acknowledge there are children whohave voluntary control over LUT function < 5 yearsof age; therefore, this terminology document may beselectively applicable to younger cohorts of children.Other influences impacting bladder function andcontinence include the developmental level of thechild18 as well as any behavioral disorders12.

Storage symptomsIncreased or decreased voiding frequency. Voidingfrequency is variable and is influenced by age19 aswell as by diuresis and fluid intake20, more so thanbladder capacity. Normative data in populationsurveys are mixed. In a small, cross-sectional

analysis of healthy school-aged children, approxi-mately 95% of 7 - 15 years old children will voidbetween 3 to 8 times per day;21 population surveysin larger sample sizes report that most 7 year oldswill void between 3 to 7 times daily22 whereas inanother large population survey most children be-tween 3 - 12 years of age void 5 to 6 times per day23.Based on the large surveys and the previous ter-minology document1, the panel continues to proposethe definition of increased daytime urinary fre-quency in those children who void "8x per day anddecreased daytime urinary frequency for the oneswho void #3x per day. Voiding frequency may not befully appreciated unless a formal voiding frequency/volume chart or voiding diary is collected.

Incontinence. Urinary incontinence means involun-tary leakage of urine; it can be continuous orintermittent. The subdivisions of incontinenceinclude continuous incontinence, intermittent in-continence, daytime incontinence and enuresis.(Figure 2).

Continuous incontinence refers to constant urineleakage (day and nighttime) usually associated withcongenital malformations (i.e., ectopic ureter, exs-trophy variant), functional loss of the externalurethral sphincter function (e.g. external sphinc-terotomy) or iatrogenic causes (e.g. vesicovaginalfistula). Intermittent incontinence is the leakage ofurine in discrete amounts. Intermittent inconti-nence that occurs while awake is termed daytimeincontinence. When intermittent incontinenceoccurs exclusively during sleeping periods, it istermed enuresis. Enuresis should not be usedto refer to daytime incontinence. A child with com-bined intermittent incontinence during ‘awake’

Figure 1. Bladder and bowel dysfunction subtypes. Figure 2. Incontinence subtypes.


periods and while sleeping is termed daytimeincontinence and enuresis. For subdivisions ofenuresis and daytime incontinence, the reader isreferred to the sections on Conditions/Diagnosis(Enuresis) and LUT symptoms below.

Urgency. Urgency refers to the sudden and unex-pected experience of an immediate and compellingneed to void. The term is not applicable before theattainment of bladder control. The symptom of ur-gency is often a sign of bladder overactivity.

Nocturia. Nocturia is the complaint that the childhas to wake at night to void. Nocturia is commonamong school children21,24 and is not necessarilyindicative of LUT dysfunction or a pathologic con-dition. Unlike enuresis, nocturia does not result inincontinence. Note that nocturia does not apply tochildren who wake up for reasons other than a needto void, e.g. children who wake up after anenuretic episode.

Voiding SymptomsHesitancy. Hesitancy denotes difficulty in initiatingvoiding when the child is ready to void.

Straining. Straining means the child complains ofneeding to make an intense effort to increase intra-abdominal pressure (e.g. Valsalva) in order toinitiate and maintain voiding.

Weak Stream. This term describes an observedstream or uroflow that is weak.

Intermittency. Intermittency implies micturition thatis not continuous but rather has several discretestop and start spurts.

Dysuria. Dysuria is the complaint of burning ordiscomfort during micturition. The timing ofdysuria may be noted during voiding. Dysuria at thestart of voiding suggests a urethral source of painwhereas dysuria at the completion of voiding sug-gests a bladder.

Other SymptomsHolding Maneuvers. These are observable strategiesused to postpone voiding or suppress urgency thatmay be associated with bladder overactivity. Thechild may or may not be fully aware of the purposeof these maneuvers, but they are usually obvious tocaregivers. Common behaviors include standing ontiptoes, forcefully crossing the legs, grabbing orpushing on the genitals or abdomen and placingpressure on the perineum (e.g. squatting with theheel pressed into the perineum or sitting on theedge of a chair).

Feeling of incomplete emptying. This refers to thecomplaint that the bladder does not feel empty after

voiding and may result in the need to return to thetoilet to void again.

Urinary retention. This refers to the sensation of aninability to void despite persistent effort in thepresence of a fully, distended bladder. Duration oftime is particularly beneficial in characterizingretention.

Post micturition dribble. This term is used when thechild describes involuntary leakage of urine imme-diately after voiding has finished. This symptommay be associated with vaginal reflux in girls orsyringocoele in boys (see below).

Spraying (splitting) of the urinary stream. This refers tothe complaint that urine passes as a spray or a splitrather than a single discrete stream. It usuallyimplies a mechanical obstruction at or just belowthe meatus (e.g. meatal stenosis).

Genital and LUT painBladder pain. Complaint of suprapubic pain or pres-sure or discomfort related to the bladder.

Urethral pain. Complaint of pain felt in the urethra

Genital pain. This refers to pain in the genitals.In girls, vaginal pain and vaginal itching arecommonly seen with localized irritation from in-continence. Penile pain and episodic priapism maybe seen in young boys as symptoms associated witha full bladder, constipation or the result of urinetrapping inside a phimotic foreskin.

TOOLS OF INVESTIGATIONA thorough history and physical examination arethe hallmark diagnostic tools for evaluation ofchildren and adolescents with LUT dysfunction.During the evaluation, it is advisable to observe thechild for holding maneuvers, expressions of urgencyor any behavioral issues. Specific tools that aid theevaluation have been published in the ICCS guide-line on diagnostic evaluation of children with day-time incontinence.10 These tools and their relevantterminology will be briefly reviewed and categorizedinto invasive and non-invasive urodynamics.

Non-invasive urodynamicsDiaries. Bladder diary. The objective recording anddocumentation of bladder function involves collect-ing a diary. A complete bladder diary consists of a7-night recording of incontinence episodes andnighttime urine volume measurements to evaluateenuresis, and a 48 hours daytime frequency andvolume chart (not necessarily recorded on 2consecutive days) to evaluate for LUT dysfunction.Details can be found on the ICCS website (http://www.i-c-c-s.org) and guidelines on evaluation for


enuresis and LUT dysfunction.10,11,16 Mobile deviceapplications (apps) may also facilitate bladder diaryrecordings.

Bowel diary. The close relationship between bladderand bowel function requires screening of both sys-tems to rule out BBD. The work up for boweldysfunction in the context of BBD is outlined in theICCS guideline on the management of functionalconstipation in children with LUT symptoms.15 A7-day bowel diary utilizing the Bristol Stool FormScale is preferable. The diagnosis of functionalconstipation in children is controversial; theRome-III criteria are the most commonly acceptedguideline for diagnosis.

QuestionnairesQuestionnaires have emerged as useful adjuncts inthe evaluation of LUT function. This need is largelybased on the symptomatic nature of LUT dysfunc-tion and the importance of objectively translatingsubjective complaints into semi-quantitative data.The scoring of questionnaires allows providers togauge the extent of the dysfunction and provides amethod of monitoring outcomes during treatment.Two types of questionnaires exist - measurements ofLUT function and psychological screening.

LUT function questionnairesAlthough several questionnaires have emerged asassessment tools, 2 stand out as they have beentested across cultures, validated and undergone testand re-testing for reliability.25e29 These include:

Dysfunctional Voiding Symptom Score (DVSS)25:TheDVSS questionnaire quantifies severity of LUTS.

Pediatric Urinary Incontinence Quality ofLife Score (PIN-Q)28: The PIN-Q measures theemotional impact that urinary incontinence has ona child.

Both tools are complementary and provide aclinically appropriate picture of LUTS and impacton quality of life.30

Psychological ScreeningThe high rate of comorbid clinical behavioral disor-ders associated with BBD is well documented andreviewed in detail in the ICCS document on psy-chological and psychiatric issues in urinary andfecal Incontinence.12 The Child Behavior Checklist(CBCL) is a widely used parental questionnaire bypsychiatrists and psychologists that contains 113empirically derived behavioral items. The CBCLhas been translated into several languages. Anyvalidated, normed broadband behavioral question-naire can be used i.e. Strengths and DifficultiesQuestionnaire (SDQ) of the Behavior Assessmentfor Children (BASC).

Short Screening Instrument for Psychological Problemsin Enuresis (SSIPPE)31. The SSIPE is a brief instru-ment derived from the CBCL and recommendedinitially if any psychological problem associatedwith pediatric LUT dysfunction or BBD exists.

Urine Flow MeasurementUroflow studies consist of measuring the rate, vol-ume voided, voiding time and examining the patternduring urination into an uroflowmeter. To obtain anuroflow, a child must obviously be toilet trained.Additionally, it is important (1) the volume of voidedurine is adequate as curves change when voidedvolume is < 50% of expected bladder capacity forage10 and (2) to obtain more than 1 curve to improveaccuracy, reliability and interpretation of the test.

Uroflowmetry may be done with or without elec-tromyography (EMG) testing of the perineal mus-cles. The advantage of combining EMG withuroflowmetry is the ability to appreciate synergy ordyssynergy between the bladder and the pel-vic floor.

Flow rate. Maximum flow rate (Qmax) is the mostrelevant quantitative variable when assessingbladder outflow. Sharp peaks in the curve areusually artifacts, so maximum flow rate should beregistered only when a peak level has a duration of" 2 seconds.32 In studies of normal children andadults, a linear correlation has been found betweenmaximum flow and the square root of voided vol-ume.33 If the square of the maximum flow rate [(ml/s)2] equals or exceeds the voided volume (ml), therecorded maximum flow is most probably normal.

Flow curve shape. The shape of the flow curve isparamount when analyzing the flow pattern. Theprecise shape is determined by detrusor contrac-tility and influenced by abdominal straining, coor-dination with the bladder outlet musculature andany distal anatomic obstruction. Five types of flowpatterns are seen. (Figure 3). Each specific patternis no guarantee of an underlying diagnostic abnor-mality but rather serves as a guide to the existenceof a specific condition.

Bell-shaped curve. The urinary flow curve of ahealthy child is bell-shaped regardless of gender,age and voided volume.

Tower-shaped curve. This is a sudden, high-amplitudecurve of short duration that suggests anoveractive bladder produced by an explosivevoiding contraction.

Staccato-shaped curve. This flow pattern is irregularand fluctuating throughout voiding but the flow iscontinuous, never reaching zero during voiding.This pattern suggests incoordination of the bladder


and the sphincter with intermittent sphincteroveractivity during voiding (i.e. dysfunctional void-ing). It will be seen as sharp peaks and troughs inthe flow curve. To qualify for a staccato label, thefluctuations should be larger than the square root ofthe maximum flow rate.

Interrupted-shaped curve. This flow will displaydiscrete peaks with spikes similar to a staccato-shaped curve but unlike the latter pattern, therewill be segments where zero flow with completecessation between these peaks exists. This flowpattern suggests an underactive bladder; eachpeak represents abdominal muscle strainingcreating the main force for urine evacuation. Inbetween each strain, the flow ceases. It is possible

this flow pattern can be seen with incoordinationbetween the bladder and external urethralsphincter.

Plateau-shaped curve. This is a flattened, low-amplitude prolonged flow curve that is suggestiveof bladder outlet obstruction (BOO). The BOO canbe anatomical (e.g. posterior urethral valves orurethral stricture) or dynamic (e.g. continuous,tonic sphincter contraction). Flow electromyography(EMG) may differentiate between BOO subtypes.A plateau-shaped curve may be seen with anunderactive bladder during a long continuousabdominal strain. Abdominal pressure monitoringduring the uroflow can help delineate an underac-tive bladder condition.

Figure 3. Uroflow curve patterns. A. Bell-shaped. B. Tower-shaped. C. Staccato-shaped. D. Interrupted-shaped. E. Plateau shaped.


Pelvic UltrasoundPelvic ultrasound is a key tool in the evaluation ofpediatric LUT function10. Ultrasonographic bladderscan machines calculates bladder volume, and thusare useful in measuring pre- and post void residual(PVR) or as a B-mode sonographic probe thatprovides anatomical details of the LUT and adja-cent rectum.

Post-void residual. PVR measurements in neurologi-cally intact children are highly variable. Recentlyinvestigation of 1,128 healthy Taiwanese childrenbetween 4-12 years of age with a bell-shapeduroflow pattern and a voided volume of >50 mlsupport the following normative 95th percentilevalues for an abnormally elevated PVR34:

Children 4 - 6 years old: Single PVR >30 ml or>21% of bladder capacity (BC) where BC is deter-mined as voided volume (VV) þ PVR and expressedas percent of the expected bladder capacity (EBC ¼[age (yrs) þ 1] x 30 ml)1. It is recommended that arepeat PVR be performed with dual measurements,a repetitive PVR >20 ml or >10% BC is consideredsignificantly elevated.

Children 7 - 12 years old: A single PVR >20 ml or15% BC, or repetitive PVR >10 ml or 6% BC isconsidered elevated.

Standard conditions should be applied tomeasuring PVR: the bladder should not be under-distended (<50%) nor over-distended (>115%) inrelation to the EBC; PVR must be obtained imme-diately after voiding (<5minutes). Further valida-tion is needed for the above nomograms in similarcohorts across cultures.

Bladder wall thicknessIn daily clinical practice a thickened bladder wallalerts the clinician to longstanding problems withurine storage and emptying.10 Bladder wall thick-ness can be measured with a full and emptybladder. However, normal values do not exist.Bladder wall thickness depends on degree ofbladder filling. It is likely that bladder wall thick-ness correlates with LUT dysfunction.35

Rectal distensionThere is insufficient evidence that the transversediameter of the rectum can be used solely as apredictor of constipation and fecal impaction.15 Innon-constipated and constipated children, a diam-eter >30 mm on pelvic ultrasound correlated with afinding of rectal impaction on a digital rectalexamination.36

Invasive urodynamicsUrodynamic studies are not routinely used toevaluate LUT function in neurologically intactchildren10 but are employed regularly in children

suspected of having a neuropathic bladder13. Afuture ICCS document will detail pediatric urody-namic guidelines.

Urodynamic (cystometric) techniques. Urodynamicstudies investigate filling and emptying phases ofbladder function. In the pediatric setting, thereshould be specific adaptations regarding stafftraining, environment, child and parental support sothe entire examination is child-friendly. If bladderdynamics are measured via a suprapubic catheter,a delay of time is recommended between catheterinsertion and urodynamic recording. If a transure-thral catheter is used, catheter size needs to be assmall as possible to avoid outflow obstruction.

Cystometry is used to describe the urodynamicinvestigation during the filling phase of the mictu-rition cycle. Before filling is started, the bladdermust be emptied completely. The filling phase be-gins with the flow of fluid into the bladder andceases when instillation ends. Several parametersduring this phase should be identified in the clinicalreport that includes the filling rate, temperature ofthe infusate and the final volume instilled. Thefilling rate should be close to physiologic fillingapproaching 5 to 10% of EBC. Fluid temperatureshould be between 25 and 37

%C and the volume of

instilled fluid should not exceed an amount thatcauses pain or results in prolonged passive detrusorpressures >40 cm H2O.

Natural fill (ambulatory) cystometry provides themost physiologic simulation of bladder filling; thetime and volumes should be identified during theevaluation.

Bladder storage function should be described interms of bladder sensation, detrusor activity,bladder compliance and bladder capacity.

Bladder sensation during filling cystometry. Bladdersensation is subjective in infants and toddlers butless so in older children and adolescents. Physicalcues (e.g. holding maneuvers) will be the signs inyounger children who cannot express the sensationor a desire to void.

Reduced bladder sensation is defined as dimin-ished awareness throughout bladder filling, andabsent bladder sensation as no bladder sensationwhatsoever. Both can be observed in children withan underactive detrusor, a neuropathic bladder or aco-morbidity of diabetes mellitus.

Detrusor function during filling cystometry. Normaldetrusor function allows bladder filling with little orno change in pressure, and without involuntarydetrusor contractions despite provocation such ascoughing or positional changes. In infants andchildren any detrusor activity observed beforevoiding is considered pathological.


Detrusor overactivity is the occurrence of invol-untary detrusor contractions during filling cystom-etry. They may be spontaneous or provoked andproduce a waveform of variable duration andamplitude. Contractions may be phasic or terminal.Symptoms of urgency and/or urgency incontinencemay or may not occur. Similar to the latest IUGA/ICS terminology5, if a relevant neurological causeis present, then neurogenic detrusor overactivity isnoted, otherwise idiopathic detrusor overactivity isthe preferred term.

Bladder capacity during filling cystometry. Cystometriccapacity is the bladder volume at the end of fillingcystometry, when ‘‘permission to void’’ is givenduring the urodynamics study. This endpoint andthe level of the child’s bladder sensation at that time(‘‘normal desire to void’’) should be noted.

Maximum cystometric capacity is the bladdervolume when the child is no longer able to delaymicturition.

Bladder capacity during filling cystometry. Bladdercompliance describes the relationship betweenchanges in bladder volume and changes in detrusorpressure. Compliance is calculated by dividing thevolume change (DV) by the change in detrusorpressure (DPdet) during that change in bladdervolume (C ¼ DV/DPdet).

Compliance is expressed as ml per cm H2O.Bladder compliance can be affected by several fac-tors that should be standardized during the studysuch as the rate of filling and the reference pointsfor compliance calculations. A faster filling rate ismore provocative and should not exceed 5 to 10%of EBC or 20 ml/min. The starting point forcompliance calculations is the detrusor pressure atthe initiation of bladder filling and the correspond-ing bladder volume (usually zero). The end point forcompliance calculations is the passive detrusorpressure (and corresponding bladder volume) atcystometric capacity or immediately before thestart of any detrusor contraction that causes sig-nificant leakage (that causes the bladder volumeto decrease).

In addition to the quantitative calculation, theshape of the filling curve is important; it providesinsight into bladder compliance. Normally, detrusorpressure remains relatively stable throughoutbladder filling resulting in a linear shaped curve. Anon-linear shaped filling curve will be seen withrising detrusor pressure during filling. The changein shape of the compliance curve should be noted atthe corresponding bladder volume and time of thestudy as change may occur early or later duringbladder filling. The overall quantitative compliancemay be similar in two studies but one study has anonlinear curve during the onset of filling whereas

another has a nonlinear curve that occurs towardthe end of bladder filling.

Urethral function during filling cystometry. Urethralfunction is usually assessed in children by pelvicfloor EMG with skin or (less commonly) needleelectrodes. Urethral closure pressure is rarelymeasured. For centers using pressure measure-ments IUGA/ICS definitions are applicable.5 Theoccurrence of urethral leakage may differ whendoing urodynamic studies in a supine as comparedto an upright position; thus body position mustbe noted.

Incompetent urethral closure mechanism isleakage of urine occurring during activities thatraise intra-abdominal pressure in the absence of adetrusor contraction.

Urethral relaxation incontinence is defined asleakage due to urethral relaxation in the absence ofraised abdominal pressure or detrusor contraction.

Urodynamic stress incontinence is the involun-tary leakage of urine during filling cystometry,associated with increased intra-abdominal pressure(e.g. coughing or sneezing), in the absence of adetrusor contraction. In children, urodynamic stressincontinence is a less common condition ascompared to adult females.

Leak point pressures. There are two types of leakpoint pressure measurement; the terminology forpediatrics is identical to IUGA/ICS terminology5.The pressure values at leakage should be measuredat the moment it occurs.

Detrusor leak point pressure (detrusor LPP):This static test is the lowest value of detrusorpressure at which leakage is observed in theabsence of increased abdominal pressure or adetrusor contraction. High detrusor LPP (e.g.>40cmH2O) is associated with reduced bladder musclecompliance and poses risk for upper urinary tractdeterioration. High detrusor LPP is commonlydenoted in children with a neuropathic bladder, i.e.spina bifida or related neurological disorders. Itshould be noted that if a patient has little to nobladder neck or intrinsic sphincter function, thenthe DLPP is not an accurate reflection of detrusorcompliance. Subsequently, bladder wall complianceis further assessed with maneuvers to increase theoutlet resistance. There is no data on correlationbetween detrusor LPP and upper tract damage inchildren with a non-neuropathic bladder.

Abdominal leak point pressure (abdominal LPP):This is a dynamic test that measures the lowestvalue of intentionally increased intravesical pres-sure that provokes urinary leakage in the absence ofa detrusor contraction. Coughing or Valsalva areexamples of inducing increased pressure. A lowabdominal LPP is suggestive of poor urethral


function. Abdominal LPP supplants the terms Val-salva or stress LPP.

Voiding cystometry (Pressure flow studies)Voiding cystometry is the pressure-volume rela-tionship of the bladder during micturition. Voidingcystometry can be evaluated in neurologically intactor near-intact infants and children but is lessfrequently performed due to its invasive nature andresultant distress.

Detrusor function during voiding. Normal detrusorfunction is characterized by an initial (voluntary)relaxation of the external urethral sphincter/pelvicfloor followed immediately by a continuous detrusorcontraction that leads to complete bladder emptyingwithin a normal time span, in the absence ofobstruction.

Detrusor underactivity denotes a voidingcontraction of reduced strength and/or duration,resulting in prolonged bladder emptying and/or afailure to achieve complete emptyingwithin a normaltime span. An acontractile detrusor is seen when nocontraction whatsoever occurs during urodynamictesting; the term neurogenic acontractile detrusorshould be used where a neurological cause exists.

There are selective times when pressure-flowstudies are of clinical value in children in order todistinguish between two clinical conditions that willresult in low flow on uroflowmetry - an underactivebladder versus BOO. With the former, there isdetrusor underactivity whereas with BOO, thedetrusor pressure is elevated. An underactivebladder may require abdominal straining to achievecomplete micturition; consequently abdominalpressure may be elevated during voiding resultingin an interrupted uroflow curve.

Urethral Function During Voiding Cystometry. Normalurethral function: The urethra opens and is con-tinuously relaxed to allow micturition at a normalpressure and flow with no PVR.

Dysfunctional voiding is characterized by anintermittent and/or fluctuating flow rate due tointermittent contractions of the peri-urethralstriated or levator ani muscles during voiding inneurologically normal children. An uroflow withEMG or a videourodynamic study is requiredto document dysfunctional voiding. The EMG isnecessary to distinguish an interrupted or inter-mittent uroflow pattern secondary to an acontractileor underactive detrusor with abdominal voiding.

Detrusor sphincter dyssynergia (DSD) is incoor-dination between detrusor and external urethralsphincter muscles during voiding (i.e., detrusorcontraction synchronous with contraction of theurethral and/or periurethral striated muscles). Thisis seen in neurological disorders on urodynamic

evaluation and is characterized by increased EMGsphincter activity during a detrusor contractionand by either a “spinning-top” configuration ofthe proximal urethra or a narrowing of the externalsphincter area on videocystourethrography (VCUG)or videourodynamics.

A “spinning-top” urethra may also be seen inneurologically intact children with incoordination ofthe external sphincter and bladder during voiding(i.e. dysfunctional voiding) on VCUG. Additionally,it should be noted that patients with OAB withoutdysfunctional voiding might exhibit a “spinning-top”urethral appearance due to habitual guarding orholding maneuvers during increased bladder pres-sure or urgency.

Four hour voiding observationFour hour voiding observation is a validated tech-nique used to evaluate bladder function duringinfancy.1 This involves continuous observation ofthe freely moving infant with frequent ultrasoundmeasurement of bladder filling and residual urinebefore and after each void. Voided volumes may alsobe calculated by weighing of diapers.

SignsSigns related to voided volumes. The term voidedvolume is used to characterize the volume of urinemeasured with micturition and is recorded on thevoiding diary. Voided volume is non-invasive andreflective of real life. It is of utmost importancebecause it is easy to obtain and influences follow-up treatment. Any other measure of bladder vol-ume should explain the method used to obtain ite.g. ultrasound, urodynamic, catheterized, cysto-graphic or cystoscopic volume.

The term maximum voided volume (MVV) refersto the largest volume of voided urine measured onthe frequency volume chart throughout a 24-hourcycle. It is variable if the first morning void isincluded. It is recommended that inclusion orexclusion of the first morning void be noted duringinvestigation of the MVV. The term expectedbladder capacity (EBC) is used as a reference orstandard for comparison. The EBC is defined by theformula: (30 x [age in yrs þ 1] ml).37,38 This EBCformula was recently validated when the firstmorning void was disregarded on the frequencyvolume chart.24 The EBC is applicable for childrenbetween 4 and 12 years as it reaches a level of 390ml at 12 years. Finally, MVV, excluding the firstmorning void, is considered small or large if found tobe < 65% or > 150% of EBC, respectively.

Signs related to urine output. Normal urine outputis difficult to define in childhood, due to great intra-and inter-individual variation and to a lack oflarge-scale investigations. As the IUGA/ICS noted,


the term polyuria is used to describe excessiveexcretion of urine resulting in profuse andfrequent micturition.5 Polyuria is defined asvoided urine volumes of > 40 ml/kg body weightduring 24 hours or > 2.8 L urine for a child oradolescent weighing "70 kg .

Nocturnal urine output excludes the last voidingbefore sleep but includes the first morning void. Inenuretic children, urine voided during sleep iscollected in diapers and the change in diaper weightis measured. Nocturnal polyuria is relevant inchildren suffering from enuresis and is defined inthis cohort as a nocturnal urine output exceeding130% of EBC for age. There is a need to investigatethe quantitative threshold of this definition. In arecent population-based study of 148 healthy chil-dren with 1,977 overnight recordings, nocturnalpolyuria was found when urine volume was > than20 x (age þ 9) in ml.24 This latest formula may beapplicable for a population based nocturnal poly-uria, but its clinical usefulness has yet to be tested.Accordingly, nocturnal polyuria will result in noc-turia or enuresis. However due to the necessaryarbitrariness of this definition, it is recommendedfor authors studying these conditions to reportnocturnal urine output and EBC, or the ratios be-tween them, rather than merely classifying thechildren as polyuric or non-polyuric.

Conditions/DiagnosisUsing the ICD-10 and DSM-V definitions andcriteria6,8, the symptom of incontinence requires aminimum age of 5.0 years, a minimum of one episodeper month and a minimum duration of 3 months tobe termed a condition. Applying the criteria set forthby the DSM-5 and ICD-10, enuresis and daytimeurinary incontinence is a significant condition ifit occurs >1 episode per month and a frequency of3 episodes over 3 months We further propose toqualify the significance of enuresis as frequent("4 per week) or infrequent (<4 per week).

Enuresis. Enuresis is both a symptom and a condi-tion of intermittent incontinence that occurs duringperiods of sleep.

SubgroupsThere is ample evidence that enuretic children withconcomitant symptoms of LUT dysfunction differclinically, therapeutically and pathogenically fromchildren without such daytime symptoms.11,16

Enuresis without other LUT symptoms (nocturiaexcluded), and without bladder dysfunction, isdefined as monosymptomatic enuresis. Childrenwith enuresis and any LUT symptoms are said tohave non-monosymptomatic enuresis. Subgroupingof enuresis in this manner is essential and basedon the current clinical situation. In patients with

non-monosymptomatic enuresis, the type of LUTdysfunction condition should be reported, becausethis information will influence the treatment andthe reproducibility of the data. Once daytime LUTsymptoms have abated, the enuresis switches fromnon-monosymptomatic to monosymptomatic.

If enuresis is subdivided according to its onset,secondary enuresis is reserved for those childrenwho have had a previous dry period of >6 months.11

Otherwise it is termed primary enuresis. A caveatfor subtyping secondary enuresis is its associationwith behavioral co-morbidities that necessitateinvestigation.

Daytime conditionsThe classification of daytime LUT conditions is morecomplex than enuresis due to the heterogeneity ofsymptoms of LUT dysfunction and the considerableoverlap between conditions. Borderline cases arecommon; the rationale for grouping various symp-tom complexes into specific LUT dysfunction is oftennot adequately evidence-based.

To provide a framework to classify daytime LUTdysfunction, assessment and documentation shouldbe based on the following parameters:

1) Incontinence (presence or absence, and symp-tom frequency)

2) Voiding frequency3) Voiding urgency4) Voided volumes5) Fluid intakeThis is more important than subgrouping the

children into various recognized conditions listedbelow. Although the age of reference for symptomsand LUT conditions is "5 years6,8, these conditionsincluding incontinence are applicable to the age ofattained bladder control.

Bladder and bowel dysfunction (BBD). BBD is a com-bination of bladder and bowel disturbances. SevereBBD is characterized by LUT and bowel dysfunctionseen in children with neurologic conditions whohave no identifiable or recognizable neurologic ab-normality. When severe BBD results in changes inthe upper urinary tract (e.g. hydronephrosis and/orvesicoureteral reflux), it may be synonymous withthe historical term ‘Hinman syndrome’.

Overactive bladder. Overactive bladder (OAB): Uri-nary urgency, usually accompanied by frequencyand nocturia, with or without urinary incontinence,in the absence of urinary tract infection (UTI) orother obvious pathology. Children with OAB usuallyhave detrusor overactivity, but this label can only beapplied with cystometric evaluation (see above).Urgency incontinence is the complaint of involun-tary loss of urine associated with urgency and isthus applicable to many children with OAB.


Voiding postponement. Children who habituallypostpone micturition using holding maneuvers suf-fer from voiding postponement. This behaviorderived by clinical history is often associated with alow micturition frequency, a feeling of urgency andpossibly incontinence from a full bladder. Somechildren learn to simultaneously restrict fluids so asto reduce their incontinence. The rationale fordelineating this entity lies in the observation thatthese children often suffer from psychological co-morbidity or behavioral disturbances such as oppo-sitional defiant disorder (ODD).12

Underactive bladder. This clinical term is reserved forchildren who need to raise intra-abdominal pressureto initiate, maintain or complete voiding i.e.straining. The children may have low voidingfrequency in the setting of adequate hydration butmay also have frequency due to incomplete emptyingwith prompt refilling of the bladder. These childrenoften produce an interrupted uroflow pattern andare usually found to have detrusor underactivity ifexamined with invasive urodynamics. Flow patternsmay be plateau-shaped; pressureeflow studies willdistinguish it from bladder outlet obstruction.

Dysfunctional voiding. The child with dysfunctionalvoiding habitually contracts the urethral sphincteror pelvic floor during voiding and demonstrates astaccato pattern with or without an interrupted flowon repeat uroflow when EMG activity is concomi-tantly recorded. Note: This is a term associated witha neurologically intact patient.

Bladder outlet obstruction (BOO). BOO refers to animpediment of urine flow during voiding. It may bemechanical or functional, static or phasic and ischaracterized by increased detrusor pressure anda reduced urinary flow rate during pressure-flowstudies.

Stress incontinence. Stress incontinence is theinvoluntary leakage of small amounts of urine witheffort or physical exertion that increases intra-abdominal pressure e.g. coughing or sneezing.During urodynamic investigation, leakage isconfirmed in the absence of a detrusor contractionand termed urodynamic stress incontinence.

Vaginal reflux. Toilet-trained girls who consistentlyexperience daytime incontinence in moderateamounts shortly after voiding and have no otherLUT symptoms or nighttime incontinence havevaginal reflux. It is a consequence of voiding withadducted legs leading to urine entrapment insidethe introitus. It may be associated with labialadhesions due to localized inflammation.

Giggle incontinence. Giggle incontinence is a rarecondition in which extensive emptying or leakage

occurs during or immediately after laughing.Bladder function is normal when there is nolaughter.

Extraordinary daytime only urinary frequency. Thisapplies to a toilet-trained child who has the frequentneed to void that is associated with small micturitionvolumes solely during the day. The daytime voidingfrequency is at least once per hour with an averagevoided volume of < 50% of EBC (typically 10 e15%). Incontinence is rare and nocturia is absent.Co-morbidities, i.e. polydipsia, diabetes mellitus,nephrogenic diabetes insipidus, daytime polyuria,UTI or viral syndrome, should be excluded.

Bladder neck dysfunction. Bladder neck dysfunctionrefers to impaired/delayed opening of the bladderneck resulting in reduced flow despite an adequateor elevated detrusor contraction.39 The prolongedopening time, i.e. the time between the start of adetrusor voiding contraction and the start of urina-tion can be seen andmeasured on videourodynamics.Alternatively bladder neck dysfunction can be diag-nosed non-invasively with a uroflow/EMG when aprolonged EMG lag time is noted, i.e. the timeinterval between the beginning of pelvic floor relax-ation and the actual start of flow.39 The EMG lagtime remains to be further defined and validated.

ComorbidityIt is not the task of the ICCS to suggest definitionsand terminology for areas beyond the LUT. We do,however, find it useful to list comorbid conditionsthat are relevant and important, especially forresearchers studying the LUT in children. Theseinclude the following:

& Constipation and fecal incontinence& Urinary tract infection& “Asymptomatic” bacteriuria& Vesicoureteral reflux& Neuropsychiatric conditions (attention deficithyperactivity disorder (ADHD), oppositionaldefiant disorder etc.)

& Intellectual disabilities& Disorders of sleep (sleep apneas, parasomnias)& Obesity

Of special relevance are behavioral disorders,which affect 20-40% of children with enuresis and30-40% with daytime incontinence. These includeexternalizing disorders (ADHD and ODD), andinternalizing disorders (depressive and anxietydisorders).12

TreatmentDefinitions of treatment methods. ICCS treatmentguidelines have beenpublished in documents defining


various LUT conditions and comorbidities.9,11,12,14e16

This document conveys definitions and guidelinesregarding terminology alone.

We strongly advise not using terms such as“standard therapy” or “maintenance therapy”without defining the design of these treatments.

Pharmacological therapy, surgical therapy. Thesepertain to any therapy based on drugs or surgery.

Neuromodulation. This refers to therapy that reducesLUT symptoms or restores LUT function by thealteration and modulation of nerve activity throughcentral and/or peripheral electrical stimulation orchemical agents to targeted sites.

Alarm treatment. Alarm treatment is therapy basedon a device that gives a strong sensory signal eusually, but not necessarily, acoustic e immediatelyafter an incontinence episode. It can be used duringday- or nighttime, although the latter usage is morecommon.

Urotherapy. Urotherapy is conservative-basedtherapy and treatment of LUT dysfunction thatrehabilitates the LUT and encompasses a verywide field of healthcare professionals. Urotherapycan be divided into standard therapy and specificinterventions.

Urotherapy encompasses the following standardcomponents:

1) Information and demystification. Explanationabout normal LUT function and how the particularchild deviates from normal.

2) Instruction in how to resolve LUT dysfunction;i.e. behavioral modification with regular voidinghabits, proper voiding posture, avoidance of holdingmaneuvers, regular bowel habits, etc.

3) Life-style advice. Encompasses balanced fluidintake and diet, diminished caffeine, regularbladder and bowel emptying patterns, etc.

4) Registration of symptoms and voiding habits,using bladder diaries or frequency-volume chartsand potentially mobile apps.

5) Support and encouragement via regular follow-up with the caregiver

Specific interventions of urotherapy are definedsimilar to ICS guidelines4 that include variousforms of pelvic floor muscle retraining (biofeedback),neuromodulation and intermittent catheterization.Additional interventions of urotherapy involve

cognitive behavioral therapy (CBT) andpsychotherapy.

Psychotherapy encompasses all non-surgical,non-pharmacological treatments aimed at comor-bid behavioral and emotional disorders accompa-nying incontinence (but not aimed at enuresis orurinary incontinence themselves). These evidence-based techniques are indicated following thoroughpsychological or psychiatric assessment and onlyif a behavioral disorder is present. They can beaugmented by pharmacotherapy (stimulants inADHD). The treatment of these comorbid emotionaland behavioral disorders does not only alleviatesuffering for the child and his/her family, but canincrease compliance and adherence to urotherapy eleading to improved outcomes.12

Definitions of treatment outcomeIn the clinical scenario, the affected childand family are the ones who decide appropriatecriteria for treatment success. In the researchsetting, however, a uniform standard is necessary,so that studies and treatment options can becompared.

Researchers should recognize three basic princi-ples of treatment outcomes:

1) The symptom frequency during baseline andfollowing treatment should each be documented.

2) The assessment of treatment response oroutcome must be based on pretreatment baselineregistration of the frequency of symptoms.

3) The response during treatment should benoted as well as the response after cessation oftreatment for a specified period of time. These re-sponses may not be the same.

Initial success. No-response: <50% reductionPartial response: 50 to 99% reduction.Complete response: 100% reductionNote: The term ‘Response’ (>90% reduction) has

been dropped and rolled into the term ‘Partialresponse’ to simplify and strengthen the term‘Complete response’.”

Long-term success. Relapse: more than one symptomrecurrence per month

Continued success: no relapse in 6 months afterinterruption of treatment

Complete success: no relapse in two years afterinterruption of treatment

REFERENCES1. Neveus, T., von Gontard, A., Hoebeke, P. et al.:

The standardization of terminology of lowerurinary tract function in children and adoles-cents: report from the Standardisation

Committee of the International Children’sContinence Society. J Urol, 176: 314, 2006

2. Dannaway, J., Ng, H., Deshpande, A. V.: Adher-ence to ICCS nomenclature guidelines in

subsequent literature: a bibliometric study.Neurourol Urodyn, 32: 952, 2013

3. Norgaard, J. P., van Gool, J. D., Hjalmas, K.et al.: Standardization and definitions in lower


urinary tract dysfunction in children. Interna-tional Children’s Continence Society. Br J Urol,81 Suppl 3: 1, 1998

4. Abrams, P., Cardozo, L., Fall, M. et al.: Thestandardisation of terminology of lower urinarytract function: report from the StandardisationSub-committee of the International ContinenceSociety. Neurourol Urodyn, 21: 167, 2002

5. Haylen, B. T., de Ridder, D., Freeman, R. M. et al.:An International Urogynecological Association(IUGA)/International Continence Society (ICS)joint report on the terminology for female pelvicfloor dysfunction. Neurourol Urodyn, 29: 4, 2010

6. Association, A. P.: Fifth Edition of the Diagnosticand Statistical Manual of Mental Disorders(DSM-5), pp. The http://www.dsm5.org websitehas been reorganized to serve as a resource forclinicians, researchers, insurers, and patients.,2013

7. von Gontard, A.: The impact of DSM-5 andguidelines for assessment and treatment ofelimination disorders. Eur Child Adolesc Psychi-atry, 22 Suppl 1: S61, 2013

8. Chase, J., Austin, P., Hoebeke, P. et al.: Themanagement of dysfunctional voiding in chil-dren: a report from the Standardisation Com-mittee of the International Children’s ContinenceSociety. J Urol, 183: 1296, 2010

9. Hoebeke, P., Bower, W., Combs, A. et al.:Diagnostic evaluation of children with daytimeincontinence. J Urol, 183: 699, 2010

10. Neveus, T., Eggert, P., Evans, J. et al.: Evaluationof and treatment for monosymptomatic enuresis:a standardization document from the Interna-tional Children’s Continence Society. J Urol, 183:441, 2010

11. von Gontard, A., Baeyens, D., Van Hoecke, E.et al.: Psychological and psychiatric issues inurinary and fecal incontinence. J Urol, 185: 1432,2011

12. Bauer, S. B., Austin, P. F., Rawashdeh, Y. F. et al.:International Children’s Continence Society’srecommendations for initial diagnostic evalua-tion and follow-up in congenital neuropathicbladder and bowel dysfunction in children.Neurourol Urodyn, 31: 610, 2012

13. Rawashdeh, Y. F., Austin, P., Siggaard, C. et al.:International Children’s Continence Society’srecommendations for therapeutic interventionin congenital neuropathic bladder and boweldysfunction in children. Neurourol Urodyn, 31:615, 2012

14. Burgers, R. E., Mugie, S. M., Chase, J. et al.:Management of Functional Constipation inChildren with Lower Urinary Tract Symptoms:

Report from the Standardization Committee ofthe International Children’s Continence Society.J Urol, 190: 29, 2013

15. Franco, I., von Gontard, A., De Gennaro, M.et al.: Evaluation and treatment of non-monosymptomatic nocturnal enuresis: a stan-dardization document from the InternationalChildren’s Continence Society. J Pediatr Urol, 9:234, 2013

16. World Health Organization.: Multiaxial classifi-cation of child and adolescent psychiatric disor-ders : the ICD-10 classification of mental andbehavioural disorders in children and adoles-cents. Cambridge ; New York: CambridgeUniversity Press, pp. viii, 302 p., 2008

17. Duong, T. H., Jansson, U. B., Holmdahl, G. et al.:Urinary bladder control during the first 3 years oflife in healthy children in Vietnamea comparisonstudy with Swedish children. J Pediatr Urol, 9:700, 2013

18. Wu, H. Y.: Achieving urinary continence in chil-dren. Nat Rev Urol, 7: 371, 2010

19. Bower, W. F., Moore, K. H., Adams, R. D. et al.:Frequency-volume chart data from incontinentchildren. Br J Urol, 80: 658, 1997

20. Mahler, B., Hagstroem, S., Rittig, N. et al.: Theimpact of daytime diuresis on voiding frequencyand incontinence classification in children.J Urol, 179: 2384, 2008

21. Mattsson, S. H.: Voiding frequency, volumes andintervals in healthy schoolchildren. Scand J UrolNephrol, 28: 1, 1994

22. Hellstrom, A. L., Hanson, E., Hansson, S. et al.:Micturition habits and incontinence in 7-year-oldSwedish school entrants. Eur J Pediatr, 149: 434,1990

23. Bloom, D. A., Seeley, W. W., Ritchey, M. L. et al.:Toilet habits and continence in children: anopportunity sampling in search of normalparameters. J Urol, 149: 1087, 1993

24. Rittig, S., Kamperis, K., Siggaard, C. et al.: Agerelated nocturnal urine volume and maximumvoided volume in healthy children: reappraisalof International Children’s Continence Societydefinitions. J Urol, 183: 1561, 2010

25. Farhat, W., Bagli, D. J., Capolicchio, G. et al.: Thedysfunctional voiding scoring system: quantita-tive standardization of dysfunctional voidingsymptoms in children. J Urol, 164: 1011, 2000

26. Farhat, W., McLorie, G. A., O’Reilly, S. et al.:Reliability of the pediatric dysfunctionalvoiding symptom score in monitoring responseto behavioral modification. Can J Urol, 8:1401, 2001

27. Upadhyay, J., Bolduc, S., Bagli, D. J. et al.: Useof the dysfunctional voiding symptom score topredict resolution of vesicoureteral reflux inchildren with voiding dysfunction. J Urol, 169:1842, 2003

28. Bower, W. F., Sit, F. K., Bluyssen, N. et al.: PinQ: avalid, reliable and reproducible quality-of-lifemeasure in children with bladder dysfunction.J Pediatr Urol, 2: 185, 2006

29. Chang, S. J., Chen, T. H., Su, C. C. et al.:Exploratory factory analysis and predicted prob-abilities of a Chinese version of DysfunctionalVoiding Symptom Score (DVSS) questionnaire.Neurourol Urodyn, 31: 1247, 2012

30. Thibodeau, B. A., Metcalfe, P., Koop, P. et al.:Urinary incontinence and quality of life inchildren. J Pediatr Urol, 9: 78, 2013

31. Van Hoecke, E., Baeyens, D., Vanden Bossche, H.et al.: Early detection of psychological problemsin a population of children with enuresis:construction and validation of the ShortScreening Instrument for Psychological Problemsin Enuresis. J Urol, 178: 2611, 2007

32. Schafer, W., Abrams, P., Liao, L. et al.: Goodurodynamic practices: uroflowmetry, fillingcystometry, and pressure-flow studies. NeurourolUrodyn, 21: 261, 2002

33. Szabo, L., Fegyverneki, S.: Maximum and averageurine flow rates in normal childrenethe Miskolcnomograms. Br J Urol, 76: 16, 1995

34. Chang, S. J., Chiang, I. N., Hsieh, C. H. et al.:Age- and gender-specific nomograms for singleand dual post-void residual urine in healthychildren. Neurourol Urodyn, 32: 1014, 2013

35. Yeung, C. K., Sreedhar, B., Leung, Y. F. et al.:Correlation between ultrasonographic bladdermeasurements and urodynamic findings inchildren with recurrent urinary tract infection.BJU Int, 99: 651, 2007

36. Joensson, I. M., Siggaard, C., Rittig, S. et al.:Transabdominal ultrasound of rectum as adiagnostic tool in childhood constipation. J Urol,179: 1997, 2008

37. Hjalmas, K.: Micturition in infants and childrenwith normal lower urinary tract. A urodynamicstudy. Scand J Urol Nephrol Suppl: 1, 1976

38. Koff, S. A.: Estimating bladder capacity inchildren. Urology, 21: 248, 1983

39. Combs, A. J., Grafstein, N., Horowitz, M. et al.:Primary bladder neck dysfunction in children andadolescents I: pelvic floor electromyographylag timeea new noninvasive method to screenfor and monitor therapeutic response. J Urol,173: 207, 2005


the standardization of terminology of lower urinary tract ... · ing of iccs terminology. despite...

Documents