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Thai Neurogastroenterology
and Motility Society (TNMS)

Kids corner :
Review articles :
Pathology review : Aganglionosis
Whats new : Endoanal ultrasound for anorectal disorders

Thai Neurogastroenterology and Motility Society (TNMS)

Contents
Kids corner:

Review articles:

Whats new:
Endoanal ultrasound
for anorectal disorders
Pathology review :
Aganglionosis
Interesting case
Events guide

3-6
7-8
9
10 - 13
14 - 15
15

Editors note

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2554

basic

fecal incontinence

thaimotility@yahoo.com
website

motility

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Thai Neurogastroenterology and Motility Society (TMNS)
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2552 - 2554

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Kids corner

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5 20-25
1
18 1,507
5 2
functional disorder functional constipation
(organic constipation) Hirschsprungs disease,
hypothyroidism, anorectal malformations (ARM) spinal anomaly
spina bifida, tethered cord

functional constipation
Rome III Criteria
functional constipation 3,4 2
1 4
2 > 4
1. 3 /
2. (incontinence) 1 /
3.
4.
5. rectum
6.
organic constipation
12 , , ,
, (soling), rectum
, anal sphincter , , ,
,

Vicious cycle functional constipation

functional constipation
withholding behavior
rectum sigmoid
colon muscle tone
fecal
impaction
rectum (soiling)



toilet training
irritability, , , , , soiling,
anal fissure
functional constipation
Hirschsprungs disease, ARM, spina bifida, anal
achalasia slow transit constipation

Hirschsprungs disease5

1 5000 6
ganglion cells myenteric submucous plexuses
80 rectosigmoid
ganglion cells (aganglionic zone)
functional obstruction proximal
transition zone barium enema
90 meconium 24
bilious vomiting, ,
ribbon-like 3
short-segment Hirschsprungs disease
Hirschsprungs disease enterocolitis

2-3 20

massive peristaltic wave , (contracted anal sphincter),

24-48 rectum rectum, rectal examination
rectum rectal distension stretch
rectum Anorectal malformations (ARM)7
(fecal continence) 3
anal stenosis
sensation rectum, imperforate anus
(anal sphincter) ARM Hirschsprungs disease 1 4000-5000
abnormal development urorectal
ARM anal canal septum first trimester incomplete separation
cloaca urogenital anorectal portions ARM

Thai Neurogastroenterology and Motility Society (TNMS)

Kids corner
(VACTERL association)
vertebral anomalies, anorectal malformations, cardiac defects, tracheoesophageal fistula, renal anomalies limb abnormalities
ARM
defects cutaneous perineal fistula
(low defect) anal opening anterior perineum,
base scrotum penis anus interior rectum
vagina anal opening

rectal examination sphincteric
mechanism long-term bowel function
imperforate anus fistula
rectum urinary tract (high defect) rectum vagina

meconium
fistula rectum urinary tract
perineum
high fistula anal dimple poorly developed
gluteal muscles with incomplete midline groove (gluteal cleft)
cloaca rectum, vagina urethra
long common channel
imperforate anus
2 ( slow colonic motility)
rectum ( fast colonic motility)
rectum sigmoid colon abdominoperineal
pull-through procedures endorectal resections
ARM high malformations, sacrum
spinal anomalies

Spina bifida8
neural tube defect 28 gestation
lumbosacral motor sensory deficits lower
extremities
sacral spinal cord spina
bifida rectoanal inhibitory reflex defecation urge
internal anal sphincter
colonic transit time

Anal achalasia9
internal anal sphincter (IAS) dysfunction IAS
resting pressure 100 mm Hg, rectoanal inhibitory reflex

Hirschsprungs disease 10
4.5

Slow transit constipation11

megacolon
meconium , 1 , encopresis 23 ,
, transit study delayed colonic transit
slow transit constipation neuroendocrine
peptides 12 intestinal neuronal
dysplasia dysplasia parasympathetic submucosal plexus1
Approach to constipation

- meconium
- Onset
- (frequency, consistency, caliber volume)
- pain at defecation, , ,
(withholding behavior), soiling Hirschsprungs disease
soiling
- neurologic deficits

- functional constipation
- reflex perineum perianal exclude spinal dysraphism
- rectal examination (impaction)
postoperative stricture ARM
Hirschsprungs disease

functional constipation
organic constipation
- plain abdominal radiography
contrast enema colonic motility transitional zone
- Colonic transit study radio-opaque markers
markers
- MRI pelvic floor rectum
imperforate anus
- Anorectal manometry balloon
rectum anal canal
inflate balloon rectum anal canal
( relaxation internal anal sphincter) rectoanal inhibitory
reflex Hirschsprungs disease reflex
defecation dynamics sensory threshold to rectal distention
paradoxical contraction external anal sphincter puborectalis muscle
(anismus)
- Rectal suction biopsy Hirschsprungs disease
ganglion cells submucosa hypertrophic nerve trunks
cholinesterase staining

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Kids corner
(anal dilatation)
20-30 anal myectomy myotomy13
14

ARM7
low defect anoplasty anus
(behavior modification) 5-10
gastrocolic reflex anus posterior center sphincter complex
positive reinforcement star chart high defect recto-urinary rectovaginal fistula
colostomy posterior
( + 5) sagittal anorectoplasty 3-12 fistula
fructose sortbitol osmotic rectum neo-anus
colostomy 3-6 neo-anus
3
1. Disimpaction rectum anus anus
ARM
hypertonic phosphate enemas 3 /
1 . ( 135 ) 3 medical dietary management
polyethylene glycol solution 20 / 1 ./ (enema)
2-6 nasogastric tube colonic
anorectal manometry (sensation), motility
anesthesia
2. Maintenance sphincter control MRI
3-12 rectum pelvis
Malone appendico
peristaltic activity PEG cecostomy for antegrade colonic enemas (ACE procedure)
0.8 / 1 ./, milk of magnesia 13 / cecostomy15 nonrefluxing conduit cecum
1 ./, lactulose 13 / 1 ./ ileum enema fluid
stimulant laxative senna ( 1)
enema fluid rectum
drug dependence
3. Weaning ACE
exclude organic stricture leakage
stoma
disease

Functional constipation

Biofeedback therapy

Spina bifida16

lactulose
6 enemas suppositories
2436

5-10
5
Hirschsprungs disease
aganglionic bowel proximal ACE
anal canal definitive treatment rectum
(anorectal stenosis),
defecation dynamics anismus
biofeedback
anorectal manometry
sensory threshold to rectal distention
biofeedback balloon rectum
balloon rectal
distention

Thai Neurogastroenterology and Motility Society (TNMS)

Kids corner
Anal achalasia9
IAS myotomy myectomy
fecal incontinence
Clostridium botulinum toxin

Slow transit constipation11

subtotal colectomy with ileo-rectal anastomosis ACE

functional constipation
3-12
organic constipation

References

1. stoma
(antegrade continence enema)

1. Loening-Baucke V. Constipation in children. Curr Opin Pediatr 1994;6:556-61.

2. Chongsrisawat V, Samakchantra S, Poovorawan Y. Prevalence of functional gastrointestinal disorders in Thai children. Presented in 25th International Congress
of Pediatrics, August 25-30, 2007, Athens, Greece (abstract).
3. Hyman PE, Milla PJ, Benninga MA, Davidson GP, Fleisher DF, Taminiau J. Childhood functional gastrointestinal disorders: neonate/toddler. Gastroenterology
2006;130:1519-26.
4. Rasquin A, Di Lorenzo C, Forbes D, Guiraldes E, Hyams JS, Staiano A, Walker LS. Childhood functional gastrointestinal disorders: child/adolescent.
Gastroenterology 2006;130:1527-37.
5. Kessmann J. Hirschsprungs disease: diagnosis and management. Am Fam Physician 2006;74:1319-22.
6. Amiel J, Lyonnet S. Hirschsprung disease, associated syndromes, and genetics: a review. J Med Genet 2001;38:729-39.
7. Rintala RJ. Fecal incontinence in anorectal malformations, neuropathy, and miscellaneous conditions. Semin Pediatr Surg 2002;11:75-82.
8. DiLorenzo C, Benninga MA. Pathophysiology of pediatric fecal incontinence. Gastroenterology 2004;126(1 Suppl 1):S33S40.
9. De Caluwe D, Yoneda A, Akl U, Puri P. Internal anal sphincter achalasia: outcome after internal sphincter myectomy. J Pediatr Surg 2001;36:736-8.
10. Ciamarra P, Nurko S, Barksdale E, Fishman S, Di Lorenzo C. Internal anal sphincter achalasia in children: clinical characteristics and treatment with
Clostridium botulinum toxin. J Pediatr Gastroenterol Nutr 2003;37:315-9.
11. Hutson JM, Mcnamara J, Gibb S, Shin YM. Slow transit constipation in children. J Paediatr Child Health 2001;37:426-30.
12. El-Salhy M, Norrgard O. Colonic neuroendocrine peptide levels in patients with chronic idiopathic slow transit constipation. Upsala J Med Sci 1998;103:223-30.
13. Imseis E, Gariepy C. Hirschsprungs disease. In: Walker W, Goulet O, Kleinman R, Sherman P, Schneider B, Sanderson I, editor. Pediatric gastrointestinal
disease. 4th ed. Ontario: BC Decker, Inc; 2004;p.1031-43.
14. Yanchar N, Soucy P. Long term outcomes of Hirschsprungs disease: The patients perspective. J Pediatr Surg 1999;34:1152-60.
15. Malone PS. The antegrade continence enema procedure. BJU Int 2004;93:248-9. 16. Lemelle JL, Guillemin F, Aubert D. A multicentre study of the
management of disorders of defecation in patients with spina bifida. Neurogastroenterol Motil 2006;18:123-8.

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Articles

...

2
1. (peristalsis)
2. (pelvic floor) (rectum)
(anus)

peristalsis

content
mass movement haustra
peristaltic waves

(parasympathetic nervous system)
(vagus nerve) sacrum (sacral
spinal cord)
(enteric nervous system: ENS)

(gastrocolic reflex)
(pelvic floor) (rectum)
(anus)

18 mmHg

(enteric nervous system:

ENS)
mechanosensory information reflex
(internal anal sphincter)
(external anal sphincter)
( 1)
Passive
Pressure
from stool

Pressure
from rectal
contraction

Rectum
Internal
anal
sphincter
Voluntary
External
anal
sphincter
Defecation

1 reflex
(internal anal sphincter)
(external anal sphincter)
(rectum) 55 mmHg (Berne & Levy, 2010)

levator ani


(anorectal angle)
1-3 cm 55
mmHg
( 2)

Thai Neurogastroenterology and Motility Society (TNMS)

Articles

2 anorectal area A
puborectalis muscle
(anorectal angle) B
puborectalis muscle (anorectal angle)
(Ganong 2010)

puborectalis

mechanoreceptor

(longitudinal muscle)
sigmoid
(circular muscle)


adaptive relaxation

References
1. Ganong WF. Gastrointestinal Physiology: Review of medical physiology. 23rd ed. New York: McGraw-Hill; 2010. P.475-478.
2. Jackie DW. Integrative Functions of the Enteric Nervous System: Johnson LR. Physiology of the Gastrointestinal tract. 4th ed. Boston: Elsevier academic
press; 2006. P. 678-683.
3. Kim EB and Helen ER: Gastrointestinal Physiology: Berne RM, Levy MN, Koeppen BM, Stanton BA, eds. Physiology. 6th ed. St. Louis: Mosby; 2010. p. 539-541.
4. : . 2. 5:
; 2548. 639-641

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Whats new
Endoanal ultrasound for anorectal disorders

.

(Endoanal ultrasound, EAUS) 20



1
1

Anal and rectal tumor

Pararectal and perianal tumor
Anorectal abscess & fistula
Fecal incontinence
Anorectal pain

EAUS EAUS
EAUS

1
EAUS
anorectal physiology test pudendal nerve terminal
motor latency

External anal sphincter

Internal anal sphincter
1

Thai Neurogastroenterology and Motility Society (TNMS)

Pathology review

Aganglionosis
(Hirschsprungs disease-HSCR)
..
Hirschsprungs disease
enteric nervous system
ganglion cells submucosal myenteric plexus
rectum
hypoganglionosis ( ganglion
cells ) segmental aganglionosis (
rectum)
ganglion cells HSCR

HSCR sex-linked recessive

disorder 4:1 10%
Downs syndrome 5%
ophthalmologic defects, intestinal malrotation
duodenal stenosis1


meconium

(distal contracted
segment) (proximal dilatation)

90% short segment HSCR
rectum distal sigmoid2
(ultrashort) anus
10%

1) ganglion cells 2) hypertrophied

extrinsic nerve bundles 3) muscular coat
(contracted distal segment)

10

ganglion cells submucosa

myenteric plexus hypertrophied nerve bundles
submucosa mucosa

neurons glia neural crest cells

5
6
10 123
neurons
histologic section

neurons section
neuronal density
neurons
H&E slide


neurons neurons
neuronal maturation

immature neurons glia
neurons nucleoli
mature neurons3-4
neurons
ganglion cells
neurons
step sections ganglion cells
step sections

ganglion cells
myenteric
neurons
ganglion cells
neurons

neurons

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Pathology review
control tissue
ganglion cells
3-5 microns Smith 1
neurons
neurons frozen
myenteric plexus

agangliosis submucosal biopsies

agangliosis submucosal
myenteric ganglion
ano-rectal squamocolumnar junction
1 1.5 . squamous epithelium
ano-rectal junction ganglion
suboptimal biopsy sample
submucosal ganglion HSCR
2 3 .
submucosa 1 3
5-7 squamous epithelium
submucosal tissue
( 1-3)
75 100 levels
H&E submucosal ganglion
HSCR ganglion cells

neurons basophilic Nissl
substance ganglion cells
glia lymphocytes

ganglion cells ( 4)
nerve fiber hypertrophy
HSCR8 extrinsic autonomic and sensory fibers
nerve fibers rectum
mucosa, submucosa
myenteric plexus HSCR
submucosal ganglion
500 micron 2 7 ganglion cells

20 30 micron
3 5 micron
ganglion cells


suboptimal biopsy submucosa
artifacts
acetylcholinesterase (AChE)
AChE AChE positive fibers
muscularis mucosae
lamina propria ganglion cells
hypertrophic nerves HSCR (
AChE muscularis mucosae
lamina propria)9-10 ( 5) extrinsic
nerve fibers frozen section
fix
AChE
frozen section
HSCR

agangliosis intraoperative seromuscular biopsies

transanal suction rectal biopsy
ostomy proximal aganglion
ganglion cells ostomy

ganglion cells
seromuscular biopsy distal
proximal 5 .
3 5 . circular muscularis propria
longitudinal muscle orientation
tissue ganglion cells
2 5 sections
ganglion cells ()

11

Thai Neurogastroenterology and Motility Society (TNMS)

Pathology review
agangliosis HSCR resection
HSCR map
transitional zone ganglion cells

ganglion cells submucosal myenteric ganglia
proximal
HSCR

suction rectal biopsy HSCR

Suction rectal biopsy Inadequate

Optimal

Suboptimal

Ganglion cells Ganglion cells Ganglion cells

absent
absent
absent
(HRCR)
(not HRCR)
(HRCR)
Intraoperative
seromuscular
biopsy

Re-biopsy

Conservative
management

Without hypertrophic nerves

Without hypertrophic nerves

Intraoperative
seromuscular
biopsy

AChE

1: suction rectal biopsy

2 3 . submucosa
1 3

2: suction rectal biopsy inadequate

submucosa

12

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Pathology review

3: suction rectal biopsy inadequate

ulcerative squamous epithelium
submucosa

4: resection specimen
myenteric ganglion

5: AChE (acetylcholinesterase
activity) muscularis mucosae
lamina propria

References:
1. Blisard KS, Kleinman R. Hirschsprungs disease: a clinical and pathologic view. Human Pathol 1986; 17:1189-1191
2. Bodian M, Carter CO. A family study of Hirschsprungs disease. Ann Hum Genet 1963; 26:261-277
3. Smith VV. Intestinal neuronal density in childhood: a baseline for the objective assessment of hypo- and hyperganglionosis. Pediatr Pathol. 1993; 13:225-237
4. Heinicke EA, Kiernan JA, Wijsman J. Specific, selective, and complete staining of neurons of the myenteric plexus using cuprolinic blue. J neurosci Meth 1987;21:45-54
5. Aldridge RT, Cambell PE. Ganglion cell distribution in the normal rectum and anal canal: a basis for the diagnosis of Hirschsprungs disease by anorectal biopsy. J Pediatr Surg 1968; 3:475-489
6. Weinberg AG. The anorectal myenteric plexus: Its relation to hypoganglionosis of the colon. Am J Clin Pathol. 1970;54:637-642
7. Venugopal S, Mancer K, Shandling B. The validity of rectal biopsy in relation to morphology and distribution of ganglion cells. J Pediatr Surg 1981;16:433-437
8. Monforte-Munoz H, Gonzales-Gomez I, Lowland JM, et al. Increased submucosal nerve trunk caliber in aganglionosis. A positive and objective finding in
suction biopsies and segmental resections in Hirschsprungs disease. Arch Pathol Lab Med. 1998;122:721-725
9. Meier-Ruge W, Lutterbeck PM, Herzog B, et al. Acetylcholinesterase activity in suction biopsies of the rectum in the diagnosis of Hirschsprungs disease. J
Pediatr Surg 1972;7:11-16
10. Chow CW, Chan WC, Ue PCK. Histochemical criteria for the diagnosis of Hirschsprungs disease in in rectal suction biopsies by acetylcholinesterase activity.
J Pediatr Surg 1977;12:675-680

13

Thai Neurogastroenterology and Motility Society (TNMS)

Interesting case
30
C.C. 13
P.I. 13 1







P.H.

PE. Unremarkable

Anorectal manometry: ()
- Anal canal length 3 cm.
- Symmetrical anal vector was documented.
- Normal anal sphincter resting and squeezing pressure.
- Paradoxical increased anal sphincter pressure during
straining.
- Normal sensation.
- RAIR was positive

( )

(paradoxical anal
contraction during pushing)

Symmetrical anal vector

RAIR present

14

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Interesting case
Balloon expulsion study: 3 30 ( 1 )
Colonic transit study: normal (no marker retained at day 5)
Discussion:

colonoscopy anorectal manometry

anorectal manometry balloon expulsion study


dyssynergic defecation (anismus)
(biofeedback therapy)

biofeedback 1

Relaxation of anal
sphincter during straining
(biofeedback therapy)

Event Guide
DDW 2011

Chicago, USA

7-10 May

European Society of Pediatric

Gastroenterology, Hepatology and Nutrition
Meeting 2011

Serrento, Italy

25-28 May

GAT Meeting

Hua hin

7-9 July

GI Motility Day

TBA

2-4 September

St. Louis, Missouri, USA

16-18 September

APDW 2011

Singapore

1-4 October

UEGW 2011

Stockholm, Sweden

22-26 October

Orlando, Florida

20-23 October

16th Neurogastroenterology+Motility
Meeting

North American Society of Pediatric

Gastroenterology, Hepatology and Nutrition
Meeting 2011

15

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E-mail : thaimotility@yahoo.com