Investigation into neurogenic bladder in arthrogryposis multiplex congenita

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  • arthrogryposis

    Liubiana Arantes deAndre Ferraz de ArruEdjane de Oliveira D

    bilitae, Feda, Bra

    Received 9 August 2012; acceptedAvailable online 13 March 2013

    Urinary tract Methods: A series of 26 cases were obtained from the electronic medical records of patients

    bladder. It is mandatory to study these symptomatic children with urinary disorders.All rights reserved.

    * Corresponding author. Center for Childrens Rehabilitation of Salvador, SARAH Hospital, Av. Tancredo Neves, 2782, Caminho das Arvores,Salvador, Bahia 41820-900, Brazil. Tel./fax: 55 7132063333.

    E-mail address: (L. Arantes de Araujo).

    1477-5131/$36 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved.

    Journal of Pediatric Urology (2013) 9, 895e899 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd.infections with AMC who were admitted to Hospital Sarah in Salvador between 1994 and 2007. Thepatients had all been diagnosed with neurogenic bladder through clinical symptoms, lowerurinary tract exams, and urodynamic findings.Results: There was urinary incontinence in 21 patients (81%), and 50% had a history of urinarytract infections. Renal function was altered in 4 patients (15%) and normal in 22 (85%). In theurodynamic study, 14 patients (64%) had detrusor overactivity and 6 (27%) had underactivity.Conclusion: Patients with AMC may show changes in the urinary tract, including neurogenicKEYWORDSUrinary bladder;Neurogenic;Arthrogryposis;Urodynamic;

    Abstract Objective: During the follow-up of children who had been diagnosed with arthro-gryposis multiplex congenita (AMC), it was noted that some were experiencing dysfunctionalvoiding. Further investigation into these cases led to a diagnosis of neurogenic bladder. Fewstudies have investigated the relationship between AMC and neurogenic bladder, this beingthe first to describe the clinical characteristics of neurogenic bladder among these patients.Roberto Badaro c

    aCenter for Childrens RehabBahiana School of Medicinc Federal University of Bahimultiplex congenita

    Araujo a,*,da Musegante a,amasceno a, Ubirajara Barroso Jr b,

    tion of SARAH Hospital, Salvador, Brazileral University of Bahia, Brazilzil

    12 December 2012Investigation into neurogenic bladder in

  • Introduction

    Arthrogryposis multiplex congenita (AMC) is defined asa non-progressive congenital rigidity disorder that affectsmultiple joints [1,2]. There are around 150 symptoms thatare associated with multiple congenital contractures.Congenital amyoplasia, the most common type of congen-ital arthrogryposis, usually results from impaired blood flowto the placenta and leads to the death of the embryosmarrow [3e5].

    There are a few publications that establish a correlationbetween arthrogryposis and genitourinary changes, but notwith respect to a possible association with neurogenicbladder [6]. There is a description of concomitancebetween arthrogryposis and the malformation of the geni-tourinary system, as in cryptorchidism, hypospadias, renalagenesis and lithiasis. The presence of neurogenic bladderamong patients with arthrogryposis has been reported, butwithout a description of its characteristics [7,8].

    The objective of this study was to describe the urinarysymptoms and the results of urological examination ofpatients with neurogenic bladder and AMC.

    records, and patients with other associated conditions ofarthrogryposis such as spinal dysraphism, hydrocephalus,cerebral palsy, mental retardation, or paraparesis of otheretiology. Fig. 1 summarizes the selection of patients.

    The data were obtained by review of the electronicmedical records of each patient, following a specific protocol.

    The different levels of impairment were categorizedwith relation to the arthrogryposis classification accordingto Judith Hall [9]:

    Type I e Predominantly limb-related disorders (amyo-plasia, distal forms of arthrogryposis).

    Type II e Disorders involving the limbs as well as someother body parts.

    Type III e Disorders that involve the limbs as well ascentral nervous system (CNS) dysfunction.

    The clinical manifestations investigated were: urgency,daytime incontinence, nocturnal enuresis, and the pres-ence of a urinary tract infection as well as recurrent urinarytract infections. The exams included renal function(through both urea and creatinine tests), kidney andurinary tract ultrasounds, voiding cystourethrography, andurodynamic studies. During the urodynamic study (Med-tronic Duet, Minneapolis, MN, USA), uroflowmetry wasinitially carried out, followed by cystometry and pressure

    t s

    896 L. Arantes de Araujo et al.Figure 1 PatienMaterials and methods

    A series of cases were analyzed in order to describe thecharacteristics of neurogenic bladder in patients witharthrogryposis. Between March of 1994 and January of2007, a total of 226 patients diagnosed with AMC wereadmitted to Hospital Sarah of Salvador, Bahia, Brazil.

    All patients with AMC who showed lower urinary tractsymptoms underwent urodynamic study to investigate forneurogenic bladder, independent of their agewhen admittedto the hospital. The criteria for exclusion were: patientswithout urological data on electronic medical records, or ifurodynamic study was done in another institution, butwithout a proper description in the electronic medicalflow studies. An anal electrode was used to record theelectromyographic data of the external urethral sphincterand perineal muscles. Methods and definitions of urody-namic evaluations followed the guidelines of the Interna-tional Continence Society [10]. The treatment methodswere: guidance on voiding at scheduled times and inter-mittent bladder catheterization with or without the use ofanti-cholinergic drugs.

    Statistical analysis

    The data were analyzed using the statistical program SPSSv16.0 (SPSS, Chicago Inc., IL, USA). The program analyzed themean, standard deviation and frequency of each variable.

    election process.

  • The Ethics Research Network of Hospital Sarah approvedthis investigation.


    Among the 26 selected patients, 18 (69%) were male. Themean age at time of admission was 2 years, with a rangebetween 0 and 9 years.

    With respect to Halls classification, 25 (96%) wereclassified as Type I, 3 with symmetrical impairment to theupper limbs and 22 with symmetrical impairment to thelower limbs. One patients (4%) arthrogryposis was linked tomultiple pterygium syndrome.

    Twenty-one patients (81%) had urinary incontinence,since the parents reported diaper use at all times. Fiftypercent of the patients had a previous history of urinarytract infections, recurrent in 3 cases (12%).

    Pregnancy history showed that eight individuals (31%)wereexposed tomisoprostolwith the intendion toabort. TheX2 testshowed that therewas no significant differencewith regard toincontinence (pZ0.25), urinary tract infection (pZ0.72)andrenal function (p Z 0.74) between those individuals and 15others (69%) who were not exposed to the drug.

    compromises the vascularization of the embryo and is

    Table 1 Characteristics of urological examinations andclinical data of 26 patients with arthrogryposis multiplexcongenita.

    Number %

    UltrasoundEctasia that returns tonormal after the bladderhas been emptied

    7 27

    Ectasia/dilation that doesnot return to normal

    2 8

    Post-voiding residual 1 4

    Neurogenic bladder in arthrogryposis 897Normal 16 61UrodynamicDetrusor underactivity 6 23Detrusor overactivity 14 54Detrusor normal 6 23Sphincter deficiency 2 9Normal sphincter 24 91Low detrusor compliance 15 58Normal detrusor compliance 9 35Unmeasured detrusor compliance 2 8Normal bladder capacity 6 23Decreased bladder capacity 20 77Voiding cystourethrogramVesicoureteral reflux 0 0Thickened bladder wall 4 15Irregular bladder shape 3 11Irregular bladder shape/thickened bladder wall

    13 50

    Normal 6 23SymptomsUrinary incontinence 21 81Previous UTI 13 50Recurrent UTI 3 12detrimental to the development of neuronal tissues, espe-cially those of the spinal cord [7]. Damage to the neurons ofthe anterior horn of the spinal cord interferes with normalmuscular development, resulting in joint changes [4], andadversely affects neuronal control of the lower urinarytract. Although suspected, this drug is not a statisticallysignificant cause of the neurogenic bladder dysfunction inour cohort of patients.

    There are few investigations regarding the associationbetween arthrogryposis and genitourinary tract changes,and both the clinical and urological characteristics of thesepatients have yet to be catalogued in the literature. Goksenet al. [18] talks about the instance of neurogenic bladder inone patient with neonatal diabetes, and Quinn et al. [8]describes the presence of a hypertrophic bladder in theautopsies of patients with AMC.Electromyography was performed on 20 patients (77%)and showed a neurogenic disorder that suggested involve-ment of the anterior horn of the spinal cord.

    The urological examination results can be found in Table1 and Fig. 2. Among the identified changes, note thedetrusor overactivity followed by detrusor underactivity.

    The voiding cystourethrogram did not identify ves-icoureteral reflux. Renal function was evaluated throughboth urea and creatinine tests, and was found to be alteredin 4 patients (15%).

    With regard to their treatment, 11 patients (42%)received conservative treatment, such as scheduled urina-tion. Intermittent bladder catheterization was used in 13patients (50%) and 9 adhered to the procedure. The anti-cholinergic oxybutynin was prescribed for 19 patients, 11 ofwhom used it regularly.


    Neurogenic bladder can be found in patients with arthrog-r


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