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Year : 2019  |  Volume : 24  |  Issue : 1  |  Page : 21-26

Encouraging results of bowel and bladder management in spina bifida aperta in South India with quality of life scores in a tertiary care institution in South India

Department of Paediatric Surgery, Christian Medical College, Vellore, Tamil Nadu, India

Date of Web Publication19-Dec-2018

Correspondence Address:
Dr. Tarun John K. Jacob
Department of Paediatric Surgery, Christian Medical College, Vellore - 632 004, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_195_17

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Context: It is often a challenge to counsel parents with children operated for spina bifida aperta in developing countries. Data regarding the efficacy of simple measures and preventive are scarce.
Aims: The aim of this study is to study such children for the incidence, prevalence of bowel bladder dysfunction, and the quality of life (QOL) in children who are involved with a multidisciplinary team in India.
Materials and Methods: All children with spina bifida occulta were followed – QOL questionnaires (PIN Q, modified Barthels activities of daily living , and the visual analog score [VAS]) were used. Interventions, such as clean intermittent catheterization (CIC), bowel enemas, and surgical procedures, were studied.
Results: A total of 68 children were assessed. Twenty-nine of these children over five were evaluated with QOL scores. The prevalence of incontinence of bowel and bladder was studied. The primary outcomes included the QOL scores, and the various surgical options help bowel and bladder management. Hydronephrosis in 17.95% of children <5 years and 65.5% of children over 5 years was noted. Nineteen children were socially independent for their bowel management. The Barthel index and PIN-Q showed a poor QOL in 27.6% and the VAS in 34.5% had the same. This translated to an acceptable QOL for over two-thirds of the children.
Conclusions: Simple procedures and training for bowel management translate to a significant number of children being able to independently manage bowel care. About 30% of children develop hydronephrosis by 5 years; the decision to teach CIC must be made by then. We believe that positive counseling is given to the parents of children with spina bifida aperta as the children are capable of a reasonable QOL.

Keywords: Bladder management, bowel management, meningomyelocele, neurogenic bladder, neurogenic bowel, quality of life study, spina bifida

How to cite this article:
Kurian JJ, K. Jacob TJ, Mathai J. Encouraging results of bowel and bladder management in spina bifida aperta in South India with quality of life scores in a tertiary care institution in South India. J Indian Assoc Pediatr Surg 2019;24:21-6

How to cite this URL:
Kurian JJ, K. Jacob TJ, Mathai J. Encouraging results of bowel and bladder management in spina bifida aperta in South India with quality of life scores in a tertiary care institution in South India. J Indian Assoc Pediatr Surg [serial online] 2019 [cited 2021 Dec 9];24:21-6. Available from: https://www.jiaps.com/text.asp?2019/24/1/21/247900

   Introduction Top

The outlook of spina bifida aperta has undergone a sea change over the past few decades. This condition had such poor prognosis in the 1960's that these children were refused treatment. A better understanding of long-term morbidity of this congenital condition with improvement in medical and surgical techniques over the years has resulted in successful management using a multidisciplinary approach. Current survival, in this condition, had improved to 85% from the dismal 10% in the 1960's.[1]

The worldwide occurrence of spina bifida is about 1–3 per 1000 births,[2] while the incidence in India is about 3.9–8.8 per 1000 births.[3] The occurrence of this anomaly has been on the decline in the developed countries; on account of better nutrition, food fortification, and timely folic acid supplementation.[4] The advanced antenatal sonography screening services that paradoxically offer termination to affected individuals also contribute to this decline.

The disease burden in India alone would translate to thousands of children born every year with this disease. Live-birth prevalence of neural tube defects in North India was 17.4/10,000 live births.[5]

In a developing country such as India, parents are often unaware of the options that are available for children with spina bifida. Children in developing nations can have an improved quality of life (QOL) with simple interventions and lifestyle modifications. Data that support this is unfortunately missing in the Indian scenario, and we believe that the availability of this data can translate to better health information in similar resource-poor countries.

There is also sparse data from developing countries on the QOL with spina bifida, the various coexisting morbidities such as hydrocephalus and mental retardation. More importantly, there is a paucity of data on the various implications of neurogenic bowel and bladder in spina bifida, such as the onset of renal damage, and surgical adjuncts that help parents of these children improve the QOL.

Our data captures the experience; drawn from a cross-sectional cohort of children with spina bifida aperta who presented to a tertiary care facility in Southern India. Treatment was achieved using a multidisciplinary approach, the facility for pediatric surgery, neurosurgery, and rehabilitation services with physiatrists and therapists. These children also benefited from the support services of a good radiology department.

   Materials and Methods Top

All children, below the age of 16 who attended our pediatric services between January 2003 and January 2014 with a diagnosis of spina bifida and a dorsal midline scar or defect, were enrolled. All in all 68 children with spina bifida aperta were recruited. Children with spina bifida occulta were excluded from the study.

Five years of age is a very significant threshold in the life of a child when the child leaves the protected confines of home for the more demanding environs of school. Urinary and fecal continence is an essential prerequisite for an uneventful transition to school. Hence, those over 5 years of age (n = 29) were called back to administer the four QOL instruments. The questionnaires were administered to the parents of the children. To assess the functionality with regard to activities of daily living (ADL), modified Barthels ADL and with regard to continence (PinQ) were administered. QOL was assessed using a visual analog score (VAS) and patient-generated index.[6],[7],[8],[9],[10]

The Barthel ADL index is comprised of a ten-point assessment score with regard to ADL. Scores of this index range from 0 to 20, with the best possible score being 20.

PIN-Q also called the pediatric incontinence QOL score measures the QOL with regard to urinary incontinence. There are 20 questions, each having a score from 0 to 4. The maximum score is 80, and the minimum score is 0, with 0 being the best possible score.

VAS measures the general QOL with 0 being the least score and 100 being the best and the maximum possible score. This is the most easily administered, comprehended, and validated global measure of QOL.

A fourth questionnaire used was a patient-generated index, that was an open-ended questionnaire to assess issues affecting the QOL, that the above scales did not assess, and the caregivers felt were of importance. This was a qualitative assessment tool; however, it was found to have a poor response rate from parents – who either felt the above tools were sufficient or were unable to express other issues of care in this format. This tool was hence removed from the analysis.

The number of children in each quartile was then calculated for all the indices. It was presumed that the children whose scores were below the median value will have a poor QOL.

Procedures in children that contributed to a change in their QOL were studied. These procedures included clean intermittent catheterization (CIC) through naturalis or a mitrofanoff, double voiding, enemas, and the use of a Malone's antegrade continent enema.

Data were entered into Microsoft Excel and analyzed using SPSS (IBM Corp. IBM SPSS Statistics for Windows, Version 24.0. Armonk, NY, USA). The QOL was assessed by three instruments in children over 5 years of age. The Barthels score, the PIN-Q score, and the VAS were used and found to be the most reproducible in the Indian population. The prevalence of incontinence of bowel and bladder was studied. Primary outcomes included the QOL scores, and the various surgical options for bowel and bladder management.

   Results Top

A total of 68 children were assessed, 43 were male and 25 were female. Twenty-nine of these children who were 5 years and over were evaluated with the four QOL scores. Bowel and bladder incontinence as a morbidity was evaluated and recorded both by structured interviews and by the above-mentioned scoring systems.

Bladder management

Two groups (age less than and greater than 5 years) were studied to look for the prevalence of hydronephrosis. Seven (n = 39) or 17.95% of children <5 years and 19 (n = 29) or 65.5% of children over 5 years had hydronephrosis when assessed.

The time of the development of upper tract dilatation for children (incidence) who were beyond infancy was obtained from the records. Two children (2/49 - 4.08%) developed upper tract dilatation by the age of 1 year. Out of 49 children, 15 (30.61%) developed upper tract dilatation by the time they were 5 years old. These figures offer us a rough estimate of the incidence and prevalence of hydronephrosis – a potentially preventable complication of spina bifida aperta and a harbinger of renal failure later in the lives of these children.

Very high bladder pressure is considered as the most important causative factor for upper tract deterioration, and therapeutic measures are directed toward reducing bladder pressures. Of the total 68 children, 42 were either treated with drugs or CIC or underwent diversion procedures or augmentation of the bladder to offset the upper tract changes that resulted from high bladder pressures based on cystometrography. Twenty-six children underwent CIC alone, 20 children were put on bladder relaxing drugs such as oxybutynin and amitriptyline, 14 children who failed the above measures and were appropriately counselled underwent bladder augmentation after consent, with CIC, and 2 children underwent other diversion procedures such as vesicostomy and ureterostomy when unwilling for augmentation.

Of the 14 children, who had a bladder augmentation, an appendicular Mitrofanoff was used for bladder catheterization in 13 children, and the urethra was used for bladder catheterization in one child. Of the 26 children, who were treated with CIC without augmentation, the urethra was used for catheterization in 25 while in one a Mitrofanoff was used. [Table 1] displays the PIN-Q scores of the >5 years group. It stratifies them among different age groups with the bladder management interventions used. PIN-Q scores are within the mid-quartile range indicating that interventions were done as a need to be done basis, ensuring an acceptable QOL over that age groups.
Table 1: Subgroup of ages with bladder assessment and if bladder management initiatives made

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Of the total 68 children, 28 children were not on CIC of which 14 of whom were infants.

Urinary incontinence

The prevalence of urinary incontinence in children with spina bifida aperta without intervention has varied from 46% to 80% as per different studies.[11],[12],[13] In our cohort, 20 of the 29 children (68.96%) who were 5 years or more had urinary incontinence, and only one child was able to void without accidents.

Stool incontinence

A child is expected to be potty trained by 5 years of age. The total number of children in this study who were more than 5 years old was 29. Twelve children were continent to stools with three of them being administered daily enemas; 11 children had occasional soiling with six of them being on daily enemas; six children had severe incontinence that interfered with ADL; and none of this group was on daily enemas. Children not on enemas either did not require them or were not compliant with administering them. Nineteen children were socially independent with their ability to manage their stool continence and did not require assistance from a parent for their bowel management programs. None of the 29 children required any drugs such as loperamide or codeine as an adjunct for bowel management [Table 2].
Table 2: Continence to stool and outcomes with rectal washes

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Quality of life scores

The main contributors to the QOL in children with spina bifida are incontinence, difficulty in ambulation, and its resultant consequences. Since otherwise normal children become proficient in these domains by 3 to 4 years, the QOL study was focused on children who were 5 years or more. [Table 3] discusses a few of the coexisting conditions with spina bifida that significantly affect QOL and a brief capture of what was done for these conditions.
Table 3: Associated conditions with neurological and orthopedic conditions associated tabulated with age subgroups

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QOL in 29 children who were 5 years or more was assessed by sequentially administering the Barthel ADL index; the PIN-Q for urinary incontinence related QOL measure; and a VAS for general QOL.

The median value for VAS was 70 out of a maximum of 100, Barthel was 17 out of 20, and PIN-Q score was 45 out of 80. The children whose scores were within the 25th centile were presumed to have an extremely poor QOL, while those above the 75th centile were presumed to have a good QOL.

According to the Barthel index and PIN-Q, eight of the 29 children (27.6%) were having a poor QOL, while according to the VAS 10 of the 29 (34.5%) had the same. The children who had poor QOL were seen to correlate across all QOL instruments. This translated to an acceptable QOL for over two-thirds of the children with spina bifida aperta across the three scores. Age substratification did not reveal any significant changes, although a larger sample size would have improved the results.

   Discussion Top

Parents of children with spina bifida aperta are often worried about the QOL their children will enjoy. Simple interventions and routine follow-up in our cohort have ensured that over two-thirds have a good QOL and are on surveillance to prevent and detect early renal complications of a neurogenic bladder.

The answer to the question “When do the renal changes set in, and when must bladder management be initiated?” is often unclear. The early familiarization with the procedure has both advantages and its limitations. Many parents find passing a catheter in a small infant difficult, especially as the child, is recovering from surgery and the caregivers are coming to terms with the disease. Anecdotal experience suggests early exposure to CIC has better compliance and adherence by the caregivers and the child, but may result in a few normal children undergoing CIC. This question requires further study.

The untreated high-pressure neurogenic bladder would progressively worsen renal function as the child grows.[14] Children with open bladder necks are safer for renal function preservation but are constantly wet regardless of pharmacological interventions and CIC.[15] We found that a significant number of children developed the upper tract changes in early childhood. Since about 30% of children develop hydronephrosis by 5 years, the decision to teach CIC must be made by then. There is a definite evidence to suggest in neurogenic bladders after spina bifida surgery, it would be detrimental to wait beyond 5 years in view of the unacceptable incidence of the upper tract changes. The jury is still out on advocacy of early CIC. Strong arguments can be made for either case.[16],[17] There are studies from the Indian subcontinent on neurogenic bladder that do not address the long-term outcomes of spina bifida surgery, possibly limited by poor follow-up.[18]

Bowel management is another area that frustrates parents and children alike. Scarce data in the Indian subcontinent exist – however, there is a benefit when advocated in neurogenic bowel in literature.[14],[15] The early initiation of bowel management protocols by simple rectal washes helps these children to stay clean and dry.[19],[20] Our series shows that a good number of children have socially acceptable continence to stool – rectal washes are a useful adjunct to keep them socially continent. Rectal enemas however, did not help prevent accidents.

Data provided here on the incidence and prevalence of hydronephrosis will help guide healthcare professionals to refer spina bifida children to institutes with a clear, focused program to prevent renal damage.

   Conclusions Top

There are a significant number of children after surgery for spina bifida aperta in the Indian subcontinent. Unfortunately, the documentation of the urinary and bowel morbidity, associated conditions, and QOL is inadequate and sketchy. Apart from describing various ways to close defects and untether neural tissue, long-term outcomes are difficult to quantify and just not available.

This cohort sheds light on the prevalence of urinary and bowel morbidity in a subset of Indian patients who sought tertiary care. It also lists the methods employed in our institution to improve the QOL of children affected. We feel that QOL improvements are facilitated by simple measures such as drugs, clean catheterization, and enemas as well as a few complex urological constructions such as the Mitrofanoff and bladder augmentation.

We believe that a regular follow-up of such children with a focus on early screening for urinary and bowel morbidity is needed. Clean catheterization requires the assessment for upper tract changes and initiation of CIC will need to be initiated as soon as required – preferably around 1 year of age. Many of these children can be easily managed in the community and only require referral to tertiary care if there is a requirement for a procedure (neurosurgical, orthopedic, bowel and bladder, or rehabilitative training) that can improve their QOL, or for developmental screening that can help identify subtle developmental delay.

It is possible for children with spina bifida aperta in a developing county to lead a fairly good QOL. We believe that positive counseling should be given to the parents of children with spina bifida aperta as the children are capable of a reasonable QOL.

Financial support and sponsorship

FLUID RESEARCH GRANT, Christian Medical College Vellore.

Conflicts of interest

There are no conflicts of interest.

   References Top

Bomalaski MD, Teague JL, Brooks B. The long-term impact of urological management on the quality of life of children with spina bifida. J Urol 1995;154:778-81.  Back to cited text no. 1
Venkataramana NK. Spinal dysraphism. J Pediatr Neurosci 2011;6:S31-40.  Back to cited text no. 2
  [Full text]  
Cherian A, Seena S, Bullock RK, Antony AC. Incidence of neural tube defects in the least-developed area of India: A population-based study. Lancet 2005;366:930-1.  Back to cited text no. 3
Clemmensen D, Thygesen M, Rasmussen MM, Fenger-Grøn M, Petersen OB, Mosdal C. Decreased incidence of myelomeningocele at birth: Effect of folic acid recommendations or prenatal diagnostics? Childs Nerv Syst ChNS J Int Soc Pediatr Neurosurg 2011;27:1951-5.  Back to cited text no. 4
Kant S, Malhotra S, Singh AK, Haldar P, Kaur R, Misra P, et al. Prevalence of neural tube defects in a rural area of North India from 2001 to 2014: A population-based survey. Birth Defects Res 2017;109:203-10.  Back to cited text no. 5
Wade DT. Measurement in neurologic rehabilitation. Curr Opin Neurol 1993;6:778-84.  Back to cited text no. 6
Paul-Dauphin A, Guillemin F, Virion JM, Briançon S. Bias and precision in visual analogue scales: A randomized controlled trial. Am J Epidemiol 1999;150:1117-27.  Back to cited text no. 7
McCormack HM, Horne DJ, Sheather S. Clinical applications of visual analogue scales: A critical review. Psychol Med 1988;18:1007-19.  Back to cited text no. 8
Bower WF, Wong EM, Yeung CK. Development of a validated quality of life tool specific to children with bladder dysfunction. Neurourol Urodyn 2006;25:221-7.  Back to cited text no. 9
Collin C, Wade DT, Davies S, Horne V. The barthel ADL index: A reliability study. Int Disabil Stud 1988;10:61-3.  Back to cited text no. 10
Verhoef M, Lurvink M, Barf HA, Post MW, van Asbeck FW, Gooskens RH, et al. High prevalence of incontinence among young adults with spina bifida: Description, prediction and problem perception. Spinal Cord 2005;43:331-40.  Back to cited text no. 11
Lie HR, Lagergren J, Rasmussen F, Lagerkvist B, Hagelsteen J, Börjeson MC, et al. Bowel and bladder control of children with myelomeningocele: A nordic study. Dev Med Child Neurol 1991;33:1053-61.  Back to cited text no. 12
Malone PS, Wheeler RA, Williams JE. Continence in patients with spina bifida: Long term results. Arch Dis Child 1994;70:107-10.  Back to cited text no. 13
Verpoorten C, Buyse GM. The neurogenic bladder: Medical treatment. Pediatr Nephrol Berl Ger 2008;23:717-25.  Back to cited text no. 14
Lee HE, Bae J, Oh JK, Oh SJ. Is concomitant bladder neck reconstruction necessary in neurogenic incontinent patients who undergo augmentation cystoplasty? Korean J Urol 2013;54:42-7.  Back to cited text no. 15
Dik P, Klijn AJ, van Gool JD, de Jong-de Vos van Steenwijk CC, de Jong TP. Early start to therapy preserves kidney function in spina bifida patients. Eur Urol 2006;49:908-13.  Back to cited text no. 16
Woo J, Palazzi K, Dwek J, Kaplan G, Chiang G. Early clean intermittent catheterization may not prevent dimercaptosuccinic acid renal scan abnormalities in children with spinal dysraphism. J Pediatr Urol 2014;10:274-7.  Back to cited text no. 17
Agrawal A, Sampley S. Spinal dysraphism: A challenge continued to be faced by neurosurgeons in developing countries. Asian J Neurosurg 2014;9:68-71.  Back to cited text no. 18
[PUBMED]  [Full text]  
Puri B, Harjai MM, Kale R. The malone antegrade continence enema procedure, an Indian perspective. Med J Armed Forces India 2002;58:214-6.  Back to cited text no. 19
Mattsson S, Gladh G. Tap-water enema for children with myelomeningocele and neurogenic bowel dysfunction. Acta Paediatr 2006;95:369-74.  Back to cited text no. 20


  [Table 1], [Table 2], [Table 3]

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