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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 2  |  Issue : 1  |  Page : 25-26

Myelomeningocele Associated with Posterior Urethral Valve and Dermoid Cyst.


Department of Paediatrics Surgery, Indira Gandhi Institute of Medical Sciences, Sheikhpura, Patna, Bihar, India

Date of Web Publication12-Feb-2016

Correspondence Address:
Zaheer Hasan
Associate Professor Dept. of Paediatric Surgery IGIMS, Patna-14
India
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Source of Support: None, Conflict of Interest: None


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  Abstract 


Patients with myelomeningocele have high incidence of associated anomalies. Among the urological anomalies neurogenic bladder is common. Posterior urethral valve can be a rare comorbid possibility associated with myelomeningocele. It is important for clinicians to be aware of this disease association and if left untreated can significantly increase morbidity and mortality.

Keywords: Myelomenigocele, posterior urethral valve, neurogenic bladder


How to cite this article:
Hasan Z, Kumar V, Thakur VK, Yadav R, Niyazi Z. Myelomeningocele Associated with Posterior Urethral Valve and Dermoid Cyst. J Indira Gandhi Inst Med Sci 2016;2:25-6

How to cite this URL:
Hasan Z, Kumar V, Thakur VK, Yadav R, Niyazi Z. Myelomeningocele Associated with Posterior Urethral Valve and Dermoid Cyst. J Indira Gandhi Inst Med Sci [serial online] 2016 [cited 2022 Oct 2];2:25-6. Available from: http://www.jigims.co.in/text.asp?2016/2/1/25/303364




  Introduction: Top


Spina bifida is a neural tube defect caused by the failure of the vertebral arch to close properly during the first month of pregnancy. There are many congenital anomalies of urinary tract associated with spina bifida but association with posterior urethral valve is extremely rare.


  Case Report: Top


A two year old boy presented to outpatient department with complains of poor urinary flow with occasional dribbling of urine since birth and soft tissue swelling over the scalp. He was operated for lumbosacral myelomeningoele at birth. On examination there was scar mark at lower back of the previous operation and urinary bladder was palpable. There was cystic swelling over the vertex diagnosed as dermoid cyst. Hemogram and biochemistry were within normal limit. Ultrasonography of the abdomen showed thick walled trabeculated bladder with significant right side hydroureteronephrosis likely due to bladder out flow obstruction Micturating cystourethrography showed thickened and trabeculated urinary bladder wall with significant dilatation of posterior urethra suggestive of posterior uretharal valve and right sided vesico uretericreflux. Computerised tomography brain shows ventricular system mildly dilated and contents of posterior fossa and brain stem shows normal appearance and attenuation value. Magneting resonance imaging corresponds with the finding of Computerised tomography brain. MRI spine showed low lying teethering of the cord. Posterior element of sacral vertebra not visualised showing dysraphism. Cystosopy and fulgration was done. Patient was passing urine in good stream in the Post-operative period.


  Discussion: Top


Patients with myelomeningocele have a high incidence of associated anomalies[1]. Among the associated anomalies hydrocephalus associated with 80-90% of cases[2]. Reineret, al studied a series of 530 cases of spina bifida and found urinary anomalies in 26 cases. Majority of the patients suffering from myelomeningocele have Lower urinay tract dysfunction[3]. Neurogenic bladder is the most common urinary tract disorder in patients of Myelomeningocele[4]. Some other diseases associated with neurogenic Lower urinary dysfunction in children are spina bifida occulta (including meningocele, sacral agenesis and primary tethered cord)[5], spinal cord injury[6], central nervous system tumours[7]. Since these supraspinal structures are important for normal Lower urinary tract function, both storage and voiding is often deranged in patients with spinal cord lesions as descending projections from higher centres are partially destroyed. Although the symptoms of the Lower urinary tract symptom depends on the level, the completeness and the maturity of the lesion, suprasacral lesions generally result in neurogenic detrusor overactivity with detrusor-sphincter dyssynergia[8]. Lesions that involve the sacral cord often result in an acontractile urinary bladder, usually accompanied denervated urethral sphincter. Similarily, posterior urethral valve can cause lower urinary tract dysfuction causing deletarious effect on bladder and its function[9]. Although, neurogenic bladder caused by myelomeningocele is functional, it can be combined with other anatomical disorder causing neurogenic bladder which creates a high risk for substantial urologic morbidity like high anorectal malformation, urogenitalsinus, bladder and cloacal extrophy and prune belly syndrome. Although, Posterior urethral valve is the most common form of urethral obstruction in boys[10] its association with meningomyelocele is rare. Neurogenic bladder caused by meningomyelocele and valve bladder syndrome resulting from Posterior urethral valve can be a lethal combination for early appearance of end stage renal disease. Early detection of Posterior urethral valve in patients of meningomyelocele by performing micturating cystourethrography and promt treatment by fulgration can relieve further backpressure change of upper renal system.



 
  References Top

1.
L. Pantanowitz, M. Sur: Malformations Associated With Spina Bifida. The Internet Journal of Pediatrics and Neonatology. 2004; 4  Back to cited text no. 1
    
2.
Fischer R G, Uihlein A, and Keith H. M. 1952, Proc. Mayo Clin. 27,33  Back to cited text no. 2
    
3.
Reiner I, Jones M, Donnell S, Rickwood AM. Incidence of normal micturition in myelomenin-gocelepatients. Arch Dis Child1992, 67:640-641  Back to cited text no. 3
    
4.
Bowel and bladder control of children with myelomeningocele-a Nordic study. Dev Med Child Neurol 1985,33:1053-1061  Back to cited text no. 4
    
5.
Johnston LB, Borzyskowski M. Bladder dysfunction and neurological disability at presentation in closed spina bifida. Arch Dis Child 1988, 79:33-38  Back to cited text no. 5
    
6.
Fanciullacci F, Zanollo A, SandriS,Catanzaro F. The neuropathic bladder in children with spinal cord injury. Paraplegia 1988, 26:83- 86  Back to cited text no. 6
    
7.
Soler D, Borzyskowski M Lower urinary tract dysfunction in children with central nervous system tumors. Arch Dis Child 1998, 79:344- 347  Back to cited text no. 7
    
8.
Abrams P, Cardozo L, Fall M, Griffiths D, Rosier P, Ulmsten U, van Kerrebroeck P, Victor A, Wein A. The standardisation of terminology of lower urinary tract function: report from the standardisation subcommittee of the international continence society. NeurourolUrodyn 2002,21:167-178  Back to cited text no. 8
    
9.
BauerSB,DieppaRA,Labib KK. The bladder in boys with posterior urethral valve; an urodynamic assesssment. J of Urology 1979,121:769  Back to cited text no. 9
    
10.
Yohannes P, Hanna M. Current trends in the management of posterior urethral valves in the pediatric population. Urology 2002;60:947-53  Back to cited text no. 10
    




 

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