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L O N G - T E R M M E D I C A L M O R B I D I T I E S
F O L L O W I N G L O W E R S P I N A L
S U R G E R Y I N C H I L D R E N
D R . G O P A K U M A R H A R I H A R A N
S E N I O R R E G I S T R A R , P A E D I A T R I C S
N O R T H W E S T R E G I O N A L H O S P I T A L , B U R N I E
T A S M A N I A , A U S T R A L I A
3/5/2016 1
HISTORY
• Sacrococcygeal teratoma – antenatal diagnosis at 32 weeks – confirmed with foetal
MRI
• Polyhydramnios and bilateral moderate hydronephrosis
• LSCS – 34 weeks
• Day 3 – enblock removal with coccyx
• Current presentation – UTI
3/5/2016 2
Anticipated problems???
3/5/2016 3
ANTICIPATED PROBLEMS IN
POSTNATAL PERIOD
• Neuropathic bladder
• Neuropathic bowel – constipation
• Impaired growth and development
• Sensory impairment
• Bone and joint problems – DDH and equinus
• Tumour recurrence ( in this case )
• Tethering of spinal cord
• Obesity
• Adolescent and mental health issues
Morbidities common to lower spinal
conditions
3/5/2016 4
MULTIDISCIPLINARY CARE
• Spina bifida clinic – Orthopaedician, urologist, neurologist
• Allied health – Physiotherapy, occupational therapy and orthotics
• Paediatrician – coordination of care
• General practitioner – community support
• Emergency visits – regional hospital
3/5/2016 5
SACROCOCCYGEAL TERATOMA
• Most common neonatal malignancy
• Arise from primitive streak cells located in the coccyx region
• Comprises solid and cystic areas arising from all three embryonic layers
• Most diagnosed antenatally
• Presentation - mass extending off the caudal end
Any lump over the coccyx of a baby
should be assumed to be a
teratoma until proved otherwise
3/5/2016 6
Altman Classification - Type I tumours are primarily external, while type IV lesions are
completely internal
Type IV SCTs - constipation, abdominal pain, or a palpable mass.
May be asymptomatic/ signs of obstruction of the rectum or bladder.
3/5/2016 7
MANAGEMENT OF SACROCOCCYGEAL
TERATOMA
• MRI- to determine extend of intrapelvic tumour
• Could cause obstetric difficulties due to size.
• Surgical resection
• Differential diagnosis- chordoma, ganglioneuroma
3/5/2016 8
NEUROGENIC BLADDER
• Presented with continuous leakage of urine - high pressure bladder with renal
dilatation – urodynamic studies – neurogenic bladder
• CIC up to 5 times/ day.
• Anticholinergics to relax the bladder muscles.
• MAG3. Good function
• December 2015: vesicostomy
3/5/2016 9
BLADDER FUNCTION
• Evolution of bladder function - involuntary bladder
emptying (urinary incontinence) during infancy to
voluntary control –by five years of age.
• Urinary continence - interrelated network of
autonomic and somatic nerves of the central and
peripheral nervous systems
• Normal coordination – bladder filling and voiding (
based on pressure gradient)
Bladder filling phase - urine storage at low
pressure with high outlet resistance
Voiding phase – sustained detrusor
contraction and low outlet resistance
3/5/2016 10
NEUROGENIC BLADDER
Disruption in the innervation of the bladder or external sphincter.
Congenital anomalies - myelomeningocele
Trauma to the central nervous system (eg, spinal cord injury, post
surgical)
3/5/2016 11
NEUROPATHIC BLADDER
Bladder – neuropathic
Small and noncompliant
Floppy with poor emptying
Sphincter function – determines
Degree of incontinence
Intravesical pressures – backpressure to kidneys
Detrusor- sphincter dysynergia
3/5/2016 12
NEUROPATHIC BLADDER
• Flaccid bladder
• High pressure bladder
• Hyperreflexic bladder
• Open bladder neck, and/or overactive
external/internal sphincter
• Lack of coordination between the bladder
and the external sphincter during voiding
and/or bladder filling (detrusor sphincter
dysynergia)
3/5/2016 13
CAN WE PREDICT URINARY TRACT
DYSFUNCTION IN SPINAL CORD
PATHOLOGY?
• Spinal lesion location and neurologic examination – doesn’t predict the type or severity
of urinary tract dysfunction,
Bladder controlled below the spinal roots directing lower extremity
function
• "low" level (sacral) myelomeningocele may have unfavourable bladder dynamics even
if lower extremity function is normal
• Evaluation required periodically with ultrasonography and urodynamic studies
3/5/2016 14
URODYNAMIC TESTING
• Urethral and rectal catheters - study bladder during filling and voiding in an awake
child.
• Information on detrusor instability, incontinence, compliance, bladder pressure,
bladder capacity, urine flow rate, sphincter activity, and bladder emptying.
• Bladder - filled with contrast during a urodynamic test – allows radiographic
visualization.
• Useful to separate common disorders of overactive bladder (filling phase abnormality)
from those of dysfunctional voiding due to abnormal sphincter or pelvic musculature
contraction during voiding
3/5/2016 15
3/5/2016 16
RECOMMENDED TEST FREQUENCY
• Urodynamic study (VCUG,Cystometrogram and electromyogram)
• At birth, 6 months, 1 year, 3 to 4 years and then 2 to 3 yearly thereafter
3/5/2016 17
ULTRASONOGRAPHY
• Most commonly imaging modality – bladder dysfunction.
• Anatomical abnormalities - hydronephrosis (obstruction or vesicoureteral reflux, a double collecting
system with an ectopic ureter, or renal scarring)
• Detection and measurement of post-void residual volume( more than 20 mL upon repeat measurement
indicates incomplete bladder emptying )
Incomplete emptying - underactive bladder and dysfunctional voiding
• Measurement of bladder wall thickness – The bladder wall is normally less than 3 mm thick when full or
less than 5 mm thick when relatively empty*.
Thickened bladder wall - outlet obstruction (eg, posterior urethral valves) or functional
abnormality (eg, non-neurogenic dysfunctional voiding)
• Most common cause of a thickened bladder wall is an overactive bladder (92 percent of cases in one
series)**.
3/5/2016 18
FOLLOW UP USG
• 3 monthly in first year
• 6 monthly in second year
• Yearly thereafter
• More frequent in
UTI
Noncompliance with CIC
High risk bladder
3/5/2016 19
HIGH PRESSURE BLADDER
• Concern - Renal involvement – Renal failure ( focus of management)
• Lifelong problem- requires ongoing management
• Prevention of renal failure
• Oxybutynin
• CIC and cystostomy
• Monitoring for urinary tract infections
• UTI prophylaxis- Trimethoprim/ Cephalexin
• Regular urodynamic study- detrusor muscle function, residual urine and sphincter-
detrusor dyssynergy
3/5/2016 20
DETRUSOR- SPHINCTER DYSYNERGIA
• Bladder contracts while the sphincter doesn’t relax – affecting the pathway involving
bladder afferents and the motor innervation of the urethral sphincter
• Symptoms – Daytime and nightime wetting, urinary retention, history of UTIs
• Urodynamic studies
• Self catheterisation
• Cystostomy
• Botulinum toxin injection
3/5/2016 21
CLEAN INTERMITTENT
CATHETERISATION
• Recommended early in infancy
Improves outcome
Reduces need for bladder augmentation
Better compliance in late childhood
Could attain independence with CIC by 5 years of age
• Very few complications
Less complications with self catheterisation than assisted
Less complication in boys
3/5/2016 22
CLOUDY OR SMELLY URINE
• Increase fluid intake
• Increase frequency of catheterisation to 1 – 2 hourly
3/5/2016 23
OXYBUTYNIN
• In overactive bladder
• VUR ( secondary VUR related to high pressure bladder)
Dose
• Less than 12 months – 0.1 mg/kg three times daily ( 1 mg/ml )
• 1 – 5 years – 0.2 mg/ kg/dose TDS
• More than 5 years – Tab Ditropan 5 mg TDS
• Alternative anticholinergic agent – Tolterodine – 1 to 2 mg BD (
3/5/2016 24
SURGICAL INTERVENTIONS TO
PROTECT RENAL FUNCTION
• To protect renal function
• Achieve social continence ( not wetting between CIC)
• High bladder pressure causing hydronephrosis despite CIC or in Frequent UTI
Options
• Vesicostomy
• Bladder augmentation
• Mitranoff procedure- conduit between the bladder and abdominal wall – using
appendix – to facilitate independence in catheterisation
3/5/2016 25
RECURRENT UTI
• Recurrent urinary tract infections in the past - on Trimethoprim to prevent urinary tract
infections.
• USG kidneys – hydronephrosis returned to normal in 6 weeks( pressure released after
resection)
• DMSA and DTPA/MAG3 scans
3/5/2016 26
RECURRENT UTI INSPITE OF CIC
Causes
• Non compliance
• Constipation
• Change in bladder dynamics ( ex spinal cord tethering)
Management
Trial of nocturnal bladder emptying – continuously draining catheter overnight ( reduces
UTI frequency, decreased hydronephrosis, increases bladder capacity, improved urinary
continence)
If unsuccessful – Bladder augmentation cystoplasty.
3/5/2016 27
Child – persistent erythrocytes - ? Thoughts
3/5/2016 28
COMPLICATIONS OF CYSTOSTOMY
• UTIs
• Blockage
• Bladder stones
• Bladder cancer
3/5/2016 29
Increased risk of kidney stones
From prolonged immobility – resultant bone
resorption
MALIGNANCY
• Bladder cancer ( more common if indwelling for more than 10 years) –urothelial and
adenocarcinoma
• Squamous cell carcinoma
• Watch for skin changes around Cystostomy site
• CT scan
• Biopsy
3/5/2016 30
UROLOGIC MANAGEMENT
• Renal function- monitor creatinine
• Renal tract ultrasound- hydro nephrosis, bladder trabeculation, to assess renal structure
• Initially 6-12 weeks, then 6 monthly in toddlers and children
• Urinary tract infection- common- midstream or catheter sample for testing
• Urodynamic studies- to establish filling and emptying pressures of the bladder – to gauge risk to
kidneys.
• Clean intermittent catheterisation- started in the neonatal period
• Medication – Oxybutynin 8 – 12 hourly
• Protective clothing
• Sometimes condom drainage
• Lifelong urologic surveillance
3/5/2016 31
NEUROPATHIC BOWEL
• Soiling, incontinence
• Chronic constipation
• Dietary advise, laxatives, enemas,
frequent irrigation
Malone procedure- non-
refluxing
appendicocaesostomy- can
perform ante grade
continence enema
Button caecostomy
CHAIT caecostomy3/5/2016 32
DEVELOPMENTAL DYSPLASIA OF HIPS
• Ultrasound hips
• X ray from 6 months
• Pavlik harness
• Surgery
Follow up scans - satisfactory acetabular
growth.
3/5/2016 33
EQUINUS DEFORMITY
AFO on both legs for equines deformity.
Had right achillo-tendon lengthening
Muscle strengthening exercises to reduce deformity.
3/5/2016 34
TUMOUR SURVEILLANCE
• Periodic AFP measurements - decreasing trend - normal limits now
• Will take time up to 12 to 18 months in some cases
• Decreasing trend more important
3/5/2016 35
CO MORBID MEDICAL CONDITIONS
• Scoliosis – X ray
• Pressure sores- feet
• Obesity – increased load on joints
• Adolescent issues- sexuality, disability
• Contraception issues
• Mental health – neuropsychology assessments
• Vocational support
• Transition and transfer to adult services
3/5/2016 36
SUMMARY
• Complex medical condition
• Multidisciplinary approach
• Logical evaluation of presenting complaints
• Short term and long management goals
3/5/2016 37
3/5/2016 38

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Long term medical morbidities following lower spinal surgery in children

  • 1. L O N G - T E R M M E D I C A L M O R B I D I T I E S F O L L O W I N G L O W E R S P I N A L S U R G E R Y I N C H I L D R E N D R . G O P A K U M A R H A R I H A R A N S E N I O R R E G I S T R A R , P A E D I A T R I C S N O R T H W E S T R E G I O N A L H O S P I T A L , B U R N I E T A S M A N I A , A U S T R A L I A 3/5/2016 1
  • 2. HISTORY • Sacrococcygeal teratoma – antenatal diagnosis at 32 weeks – confirmed with foetal MRI • Polyhydramnios and bilateral moderate hydronephrosis • LSCS – 34 weeks • Day 3 – enblock removal with coccyx • Current presentation – UTI 3/5/2016 2
  • 4. ANTICIPATED PROBLEMS IN POSTNATAL PERIOD • Neuropathic bladder • Neuropathic bowel – constipation • Impaired growth and development • Sensory impairment • Bone and joint problems – DDH and equinus • Tumour recurrence ( in this case ) • Tethering of spinal cord • Obesity • Adolescent and mental health issues Morbidities common to lower spinal conditions 3/5/2016 4
  • 5. MULTIDISCIPLINARY CARE • Spina bifida clinic – Orthopaedician, urologist, neurologist • Allied health – Physiotherapy, occupational therapy and orthotics • Paediatrician – coordination of care • General practitioner – community support • Emergency visits – regional hospital 3/5/2016 5
  • 6. SACROCOCCYGEAL TERATOMA • Most common neonatal malignancy • Arise from primitive streak cells located in the coccyx region • Comprises solid and cystic areas arising from all three embryonic layers • Most diagnosed antenatally • Presentation - mass extending off the caudal end Any lump over the coccyx of a baby should be assumed to be a teratoma until proved otherwise 3/5/2016 6
  • 7. Altman Classification - Type I tumours are primarily external, while type IV lesions are completely internal Type IV SCTs - constipation, abdominal pain, or a palpable mass. May be asymptomatic/ signs of obstruction of the rectum or bladder. 3/5/2016 7
  • 8. MANAGEMENT OF SACROCOCCYGEAL TERATOMA • MRI- to determine extend of intrapelvic tumour • Could cause obstetric difficulties due to size. • Surgical resection • Differential diagnosis- chordoma, ganglioneuroma 3/5/2016 8
  • 9. NEUROGENIC BLADDER • Presented with continuous leakage of urine - high pressure bladder with renal dilatation – urodynamic studies – neurogenic bladder • CIC up to 5 times/ day. • Anticholinergics to relax the bladder muscles. • MAG3. Good function • December 2015: vesicostomy 3/5/2016 9
  • 10. BLADDER FUNCTION • Evolution of bladder function - involuntary bladder emptying (urinary incontinence) during infancy to voluntary control –by five years of age. • Urinary continence - interrelated network of autonomic and somatic nerves of the central and peripheral nervous systems • Normal coordination – bladder filling and voiding ( based on pressure gradient) Bladder filling phase - urine storage at low pressure with high outlet resistance Voiding phase – sustained detrusor contraction and low outlet resistance 3/5/2016 10
  • 11. NEUROGENIC BLADDER Disruption in the innervation of the bladder or external sphincter. Congenital anomalies - myelomeningocele Trauma to the central nervous system (eg, spinal cord injury, post surgical) 3/5/2016 11
  • 12. NEUROPATHIC BLADDER Bladder – neuropathic Small and noncompliant Floppy with poor emptying Sphincter function – determines Degree of incontinence Intravesical pressures – backpressure to kidneys Detrusor- sphincter dysynergia 3/5/2016 12
  • 13. NEUROPATHIC BLADDER • Flaccid bladder • High pressure bladder • Hyperreflexic bladder • Open bladder neck, and/or overactive external/internal sphincter • Lack of coordination between the bladder and the external sphincter during voiding and/or bladder filling (detrusor sphincter dysynergia) 3/5/2016 13
  • 14. CAN WE PREDICT URINARY TRACT DYSFUNCTION IN SPINAL CORD PATHOLOGY? • Spinal lesion location and neurologic examination – doesn’t predict the type or severity of urinary tract dysfunction, Bladder controlled below the spinal roots directing lower extremity function • "low" level (sacral) myelomeningocele may have unfavourable bladder dynamics even if lower extremity function is normal • Evaluation required periodically with ultrasonography and urodynamic studies 3/5/2016 14
  • 15. URODYNAMIC TESTING • Urethral and rectal catheters - study bladder during filling and voiding in an awake child. • Information on detrusor instability, incontinence, compliance, bladder pressure, bladder capacity, urine flow rate, sphincter activity, and bladder emptying. • Bladder - filled with contrast during a urodynamic test – allows radiographic visualization. • Useful to separate common disorders of overactive bladder (filling phase abnormality) from those of dysfunctional voiding due to abnormal sphincter or pelvic musculature contraction during voiding 3/5/2016 15
  • 17. RECOMMENDED TEST FREQUENCY • Urodynamic study (VCUG,Cystometrogram and electromyogram) • At birth, 6 months, 1 year, 3 to 4 years and then 2 to 3 yearly thereafter 3/5/2016 17
  • 18. ULTRASONOGRAPHY • Most commonly imaging modality – bladder dysfunction. • Anatomical abnormalities - hydronephrosis (obstruction or vesicoureteral reflux, a double collecting system with an ectopic ureter, or renal scarring) • Detection and measurement of post-void residual volume( more than 20 mL upon repeat measurement indicates incomplete bladder emptying ) Incomplete emptying - underactive bladder and dysfunctional voiding • Measurement of bladder wall thickness – The bladder wall is normally less than 3 mm thick when full or less than 5 mm thick when relatively empty*. Thickened bladder wall - outlet obstruction (eg, posterior urethral valves) or functional abnormality (eg, non-neurogenic dysfunctional voiding) • Most common cause of a thickened bladder wall is an overactive bladder (92 percent of cases in one series)**. 3/5/2016 18
  • 19. FOLLOW UP USG • 3 monthly in first year • 6 monthly in second year • Yearly thereafter • More frequent in UTI Noncompliance with CIC High risk bladder 3/5/2016 19
  • 20. HIGH PRESSURE BLADDER • Concern - Renal involvement – Renal failure ( focus of management) • Lifelong problem- requires ongoing management • Prevention of renal failure • Oxybutynin • CIC and cystostomy • Monitoring for urinary tract infections • UTI prophylaxis- Trimethoprim/ Cephalexin • Regular urodynamic study- detrusor muscle function, residual urine and sphincter- detrusor dyssynergy 3/5/2016 20
  • 21. DETRUSOR- SPHINCTER DYSYNERGIA • Bladder contracts while the sphincter doesn’t relax – affecting the pathway involving bladder afferents and the motor innervation of the urethral sphincter • Symptoms – Daytime and nightime wetting, urinary retention, history of UTIs • Urodynamic studies • Self catheterisation • Cystostomy • Botulinum toxin injection 3/5/2016 21
  • 22. CLEAN INTERMITTENT CATHETERISATION • Recommended early in infancy Improves outcome Reduces need for bladder augmentation Better compliance in late childhood Could attain independence with CIC by 5 years of age • Very few complications Less complications with self catheterisation than assisted Less complication in boys 3/5/2016 22
  • 23. CLOUDY OR SMELLY URINE • Increase fluid intake • Increase frequency of catheterisation to 1 – 2 hourly 3/5/2016 23
  • 24. OXYBUTYNIN • In overactive bladder • VUR ( secondary VUR related to high pressure bladder) Dose • Less than 12 months – 0.1 mg/kg three times daily ( 1 mg/ml ) • 1 – 5 years – 0.2 mg/ kg/dose TDS • More than 5 years – Tab Ditropan 5 mg TDS • Alternative anticholinergic agent – Tolterodine – 1 to 2 mg BD ( 3/5/2016 24
  • 25. SURGICAL INTERVENTIONS TO PROTECT RENAL FUNCTION • To protect renal function • Achieve social continence ( not wetting between CIC) • High bladder pressure causing hydronephrosis despite CIC or in Frequent UTI Options • Vesicostomy • Bladder augmentation • Mitranoff procedure- conduit between the bladder and abdominal wall – using appendix – to facilitate independence in catheterisation 3/5/2016 25
  • 26. RECURRENT UTI • Recurrent urinary tract infections in the past - on Trimethoprim to prevent urinary tract infections. • USG kidneys – hydronephrosis returned to normal in 6 weeks( pressure released after resection) • DMSA and DTPA/MAG3 scans 3/5/2016 26
  • 27. RECURRENT UTI INSPITE OF CIC Causes • Non compliance • Constipation • Change in bladder dynamics ( ex spinal cord tethering) Management Trial of nocturnal bladder emptying – continuously draining catheter overnight ( reduces UTI frequency, decreased hydronephrosis, increases bladder capacity, improved urinary continence) If unsuccessful – Bladder augmentation cystoplasty. 3/5/2016 27
  • 28. Child – persistent erythrocytes - ? Thoughts 3/5/2016 28
  • 29. COMPLICATIONS OF CYSTOSTOMY • UTIs • Blockage • Bladder stones • Bladder cancer 3/5/2016 29 Increased risk of kidney stones From prolonged immobility – resultant bone resorption
  • 30. MALIGNANCY • Bladder cancer ( more common if indwelling for more than 10 years) –urothelial and adenocarcinoma • Squamous cell carcinoma • Watch for skin changes around Cystostomy site • CT scan • Biopsy 3/5/2016 30
  • 31. UROLOGIC MANAGEMENT • Renal function- monitor creatinine • Renal tract ultrasound- hydro nephrosis, bladder trabeculation, to assess renal structure • Initially 6-12 weeks, then 6 monthly in toddlers and children • Urinary tract infection- common- midstream or catheter sample for testing • Urodynamic studies- to establish filling and emptying pressures of the bladder – to gauge risk to kidneys. • Clean intermittent catheterisation- started in the neonatal period • Medication – Oxybutynin 8 – 12 hourly • Protective clothing • Sometimes condom drainage • Lifelong urologic surveillance 3/5/2016 31
  • 32. NEUROPATHIC BOWEL • Soiling, incontinence • Chronic constipation • Dietary advise, laxatives, enemas, frequent irrigation Malone procedure- non- refluxing appendicocaesostomy- can perform ante grade continence enema Button caecostomy CHAIT caecostomy3/5/2016 32
  • 33. DEVELOPMENTAL DYSPLASIA OF HIPS • Ultrasound hips • X ray from 6 months • Pavlik harness • Surgery Follow up scans - satisfactory acetabular growth. 3/5/2016 33
  • 34. EQUINUS DEFORMITY AFO on both legs for equines deformity. Had right achillo-tendon lengthening Muscle strengthening exercises to reduce deformity. 3/5/2016 34
  • 35. TUMOUR SURVEILLANCE • Periodic AFP measurements - decreasing trend - normal limits now • Will take time up to 12 to 18 months in some cases • Decreasing trend more important 3/5/2016 35
  • 36. CO MORBID MEDICAL CONDITIONS • Scoliosis – X ray • Pressure sores- feet • Obesity – increased load on joints • Adolescent issues- sexuality, disability • Contraception issues • Mental health – neuropsychology assessments • Vocational support • Transition and transfer to adult services 3/5/2016 36
  • 37. SUMMARY • Complex medical condition • Multidisciplinary approach • Logical evaluation of presenting complaints • Short term and long management goals 3/5/2016 37