2. • 21 years young girl presented with difficulty in passing
urine, intermittent and poor stream of urination and
recurrent urinary retention for 2 years (from the age of 18
years)
• She does not feel urge to pass urine but feels an
uncomfortable sensation when the bladder is full
• There is no incontinence
• She was treated for recurrent UTI over the past 2 years
• She has normal appetite and the bowel habits were normal
• She attained menarche at the age of 11 years and her
menstrual cycles were normal
• At the age of 15 years she was investigated for a watery
breast discharge but the investigations were normal
3. • No significant history of trauma or surgery
• She is not on opioids, antidepressants, NSAIDS
• At the age of 6 years her mother met with a RTA
and refused the child. So she was angry with the
mother
• 8 years – highly aggressive, refused mother,
suicidal ideas, treated for depression for 2 years
• A/L - A 2,C1
• Only sister is healthy
4. Examination
• CVS – No postural drop
• Respiratory – NAD
• Abdomen – Normal, normal anal tone
• Gynae – no UVP, no cystocele, normal external
genitalia
• B/L optic fundi were normal
• Cranial nerves were clinically normal
• Rest of the NS is normal
• Normal sacral sensation
6. • Real Time PCR TB – Negative
• Serum creatinine – 0.63mg/dl
• CPK – Normal
• FT4, TSH – Normal
• S. Prolactin – 11.93ng/ml
7. USS Abdomen
• Liver – Normal
• GB – Normal
• R/ Kidney – BPL 9.3cm
• L/ Kidney – BPL 9.2cm
• Both kidneys are normal in size and shape.
Outlines are smooth. No cortical scarring.
Cortico medullary pattern appears normal. No
evidence of intra renal calculi or
hydronephrosis seen.
8. • Pancreas – Normal
• Spleen – Normal
• Bladder is smooth in outline. No bladder wall
thickening, diverticulae or vesical calculi seen.
• Pre void bladder volume – 311ml , post void
bladder volume – 200ml ( significant).
• Uterus – Normal
• Both ovaries – normal
9. • Comment : Normal USS of abdomen and
pelvis except for residual urine.
11. Uroflowmetry
• Average flow – 5.4ml/s
• Time to peak flow – 13.6 s
• Voided volume – 183 ml
• Flow time – 33.6 s
• Pattern – intermittent
• Void time – 36.2 s
12. Cystoscopy and UDL
• UDL done up to28Fr
• Bladder – normal
• UOO seen
• No cystocele or urethrocele
13. Analysis report of urodynamic
Bladder capability Pdet
First desire to void 225.8 ml 2.9 cm/H2O
Strong desire to void 271.5 ml 5.7 cm/H2O
Urgency 305.2 ml 5.38 cm/H2O
CC 335.8 ml 6.11 cm/H2O
Residual urine
15. • Routine cystometry usually demonstrates a
large capacity bladder without the usual
sensations during the filling phase.
• The patient is then unable to pass urine –
technically, and what is often written in the
report is that there is “no rise in detrusor
pressure”. Thus little can be concluded from
this result except that the patient has a large
bladder capacity and cannot pass urine.
20. • Earlier isolated urinary retention in young women
with no structural or neurological abnormality
found were purported to be psychogenic or
hysterical origin although a disorder of spincter
relaxation had been recognized for several years
• Wrong right from the beginning
• Moor - urethral spincter hypertrophy
• Raz – elevated urethral closure pressure and
postulated the retention is due to spasticity of
striated urethral spincter or pelvic floor
21. Fowler's Syndrome is
the commonest cause
of urinary retention in
young women
Prof Clare J. Fowler
FRCP
Emeritus Professor of
Uro-Neurology, Institute
of Neurology, UCL and
Consultant in Uro-
Neurology, National
Hospital for Neurology
and Neurosurgery,
Queen Square, London
22. • Fowler and college then demonstrated a
myotonia like EMG activity from the striated
spincter and proposed that retention is due to
the primary impairment of spincter relaxation
25. • The striated urethral sphincter or the so called
Intrinsic Rhabdosphincter is a definite mass of
striated muscles which have a circular
orientation.
• Indeed this muscle is made of “slow twitch”
striated fibers, which are capable of prolonged
contraction.
26. • Although it's an intrinsic urethral muscle, it is
supplied entirely by the pudendal nerve. The
third component is of course the muscle mass
of the pelvic floor which essentially surrounds
the external rhabdosphincter but is made
mainly of “fast twitch” fibers like other
skeletal muscles.
27.
28.
29.
30.
31. Voiding Urine - Micturition
• Micturition reflex
1) 300-400 ml urine in bladder, stretch receptors
send signal to spinal cord (S2, S3)
2) parasympathetic reflex arc from spinal cord,
stimulates contraction of detrusor muscle
3) relaxation of internal urethral sphincter
4) this reflex predominates in infants
32.
33. • Infants
–Spinal reflex
• Adults
–Spinal reflex
–Higher control
• (pelvic muscles and external urethral
sphincter)
34. • Micturition center is
located in the
– Frontal lobe
• Function of micturition
center
– Send tonically inhibitory
signals to the detrusor
muscle to prevent the
bladder from emptying
(contracting) until a socially
acceptable time and place to
urinate is available.
35. Next stop is the…..
Pons
• The major relay center
between the brain and the
bladder
• Pontine micturition center
– The PMC coordinates the
urethral sphincter
relaxation and detrusor
contraction to facilitate
urination
36. Pontine Micturition Center
• Bladder filling detrusor
muscle stretch receptors
signal to the pons brain
– Perception of this signal (bladder
fullness) as a sudden desire to go
to the bathroom
– Normally, the brain sends an
inhibitory signal to the pons to
inhibit the bladder from
contracting until a bathroom is
found.
• Brain deactivating signal to
PMC
– Urge to urinate disappears
– At appropriate time, brain sends
excitatory signals to the pons,
allowing voiding
37. Pathogenesis of retention
• That contraction of the striated urethral
sphincter can inhibit detrusor contraction and
suppress bladder afferents is known from
animal experiments, although this has been
little studied as it is a difficult phenomenon to
investigate in animals.
38. Pathogenesis of retention
• urethral afferents are hard-wired in the spinal cord to
suppress sensation, inhibit bladder activity and moderate
ascending bladder signals. This is the neural basis for the
“pro-continence reflex”
• whereby voluntary contraction of the sphincter reduces
urgency, and it is enhancement of this reflex that is the
basis for physiotherapy exercises to encourage pelvic floor
contractions to control urgency incontinence.
• Feed forward from the guarding reflex may further activate
the pro-continence reflex in health, both mechanisms
combining to maintain bladder control as the bladder fills.
39. • In FS it is hypothesized that extreme
involuntary sphincter contraction results in
accentuation of the pro-continence reflex to
the point that bladder sensation is suppressed
and detrusor contraction completely inhibited.
40. • Jitter analysis of the components of the
complex repetitive discharges shows that this
is so low that it must be due to ephaptic
transmission between muscle fibres (Fowler,
Kirby et al. 1985) generating repetitive,
circuitous, self-excitation. It is this abnormal
activity which is thought to prevent relaxation
of the sphincter and cause urinary retention
or voiding dysfunction (Fowler, Kirby et al.
1985).
41. • Certainly an absence of sensation with gross
bladder filling is characteristic of this condition
and further implies that signals from the
bladder reaching the brain are abnormally
weak.
• The recent surprising results of an fMRI
research study provide confirmation of this
hypothesis.
42.
43. ESSENTIAL KEY FEATURES
• Female
• No evidence of urological disease,
gynaecological or neurological disease
• Retention with a volume in excess of >1000 ml
• No sense of urinary urgency despite high
bladder volumes - Discomfort yes, but not
urgency
• Straining does not help emptying
44. ESSENTIAL KEY FEATURES
• Sense of “something gripping” or difficulty on
removing the catheter which has been used
for urinary drainage
• No history of urological abnormalities in
childhood or associated abnormalities of the
urinary tract
• Association with polycystic ovarian syndrome
and endometriosis
45.
46. • The women often tell of an event prior to the
onset of their retention, such as an obstetric,
gynaecological or urological surgical
procedure using regional or general
anaesthesia
48. • The Presence of Fowler’s Syndrome Predicts
Successful Long-Term Outcome of Sacral
Nerve Stimulation in Women with Urinary
Retention (Dirk De Ridder , Dieter Ost , Frans
Bruyninckx)
• european urology 51 (2007) 229–234
49.
50. Sacral
Neuromodulation
The first stage is usually
done under a short general
anaesthetic and a
stimulating lead is inserted
through the third sacral
foramen (S3) as it is
commonly known.
52. stage 2
a complex and
sophisticated
permanent
stimulating battery is
implanted and
connected to the lead
already in place
53. complications
• Leg pain
• Battery pain
• Lead displacement
• Lead fracture
• Loss of efficacy or battery site infection
54. Fowler’s Syndrome & Opiates
• Quarter of the women with Fowler’s
syndrome were taking opiate medications
55. Spinal Cord Intoxication by Encephalins
• She now hypothesize that Fowler’s syndrome
is the result of spinal cord intoxication by
enkephalins
56. occult dysautonomia in Fowler's
syndrome
• Evidence of : alteration of cardiovascular
autonomic function tests in female patients
presenting with urinary retention.
• Amarenco G, Raibaut P, Ismael SS, Rene-Corail
P, Haab F.
• There was an occult impairment of the
autonomic system in women with FS; this
condition might be a pure bladder expression of a
generalized but occult dysautonomia, which in
some cases might be diagnosed using CAFTs.
57. Fowler's syndrome in two sisters
• Neurourol Urodyn. 2006;25(7):739-41; discussion
742-3.
• Podnar S, Barbic M.
• Diagnosis of Fowler's syndrome was made in
both sisters. Due to very low incidence rate of
this syndrome (0.2/100.000 per year), we think
that it is highly unlikely to find it in two sisters just
by chance. We suggest that the probable
explanation is a genetic predisposition to
polycystic ovaries, with which this condition has
been shown to be associated.
58. • At the end of the day, bladder outlet
obstruction in females remains a challenging
urological condition and demands expertise in
its investigations and management.