3/8/2014

Disclosures
Long Term Function after
Pediatric Colorectal
Surgery

I have none to disclose

March 7th, 2014

Ankit Sarin MD, MHA

Assistant Professor of Surgery
University of California San Francisco

Pediatric Colorectal Conditions

Pediatric Colorectal Conditions

Hirschprungs disease
Total Colonic aganglionosis
Ultrashort Hirschsprungs

Anorectal Malformations

Midgut Malrotation

Small left colon syndrome

Necrotising enterocolitis

Meconium syndromes

Unlikely to have
continued
consequences
requiring
evaluation as an
adult

Intussusceptions

3/8/2014

Surgery for Hirschprungs

Surgery for Anorectal malformations

B- Svenson
Procedure

C Duhamel
Procedure

D Suave
Procedure

Posterior Sagittal Anorectoplasty PSARP

What are the issues for adult

clinicians ?

HD - Issues for adult clinicians

Specific problems with the various procedures
have been identified
enterocolitis with the Swenson procedure,
diarrhea and incontinence with the Soave procedure
and constipation with the Duhamel procedure

Ultra-Short segment Hirschprungs if undiagnosed

in infancy may be encountered in patients with
lifelong outlet constipation

Even after repair fecal Incontinence in 30 35%
and severe fecal incontinence in 5-10%

3/8/2014

ARM - Issues for adult clinicians

Both Constipation and Fecal incontinence can
occur after definitive repair of anorectal
malformations constipation is more likely

Loss of rectal reservoir or worsening sphincter
function may result in fecal incontinence

Severe constipation may result in overflow
incontinence

Worse stooling behaviour correlates with poorer
quality of life

Rectoanal inhibitory reflex

Rapid rectal distention
with balloon (30 mL)
induces a transient
increase in rectal
pressure, followed by
a more prolonged
reduction in anal
pressure due to
relaxation of the
internal anal sphincter.

Evaluation

History and physical

Type of defect and nature of repair

Bowel movement and voiding pattern,

Type of perineum, location of rectal opening,
presence of an anal dimple, and

Strength of sphincter contraction.

Water-soluble enema or defecography,

Sacral films, MRI with a rectal coil

Manometry, anal ultrasound, and pudendal
nerve latency tests

Evaluation

Normal anal resting pressure and the presence
of a RAI reflex, have been correlated with a
good clinical outcome in both high and low
anorectal malformations.
Preserved RAIR reflex in ~ 60 % of patients after both
PSARP, and LAARP for high imperforate anus

Similarly Colonic Transit time may be of benefit

in patients following Hirschprungs surgery

3/8/2014

Management

Management

Group one appears untrainable.

Group two need revision

They have a poor sacrum, flat perineum, poor

muscles, no sensation, and poor bowel
movement pattern and usually are incontinent to
both urine and all types of stool.

Clinical & MRI evidence of a mislocated rectum

with a good sacrum and well-developed muscles.

Good candidates for a bowel management

program.

A permanent stoma is usually best suited and
truly appreciated by these patients.

Management

Group three has severe constipation

A contrast enema may shows a severely dilated
mega rectosigmoid in which case they benefit
from a sigmoid resection.

This may decrease the requirement of an
enormous amounts of daily laxatives to keep
their colons clean.

A new pull-through operation should be avoided
to preserve the rectal reservoir as its loss could
lead to the worse problem of incontinence
related to diarrhea.

They benefit from a secondary pull through

procedure by an experienced surgeon with the aid
of the Pea stimulator.

A second pull-through procedure if loss of
ganglion cells in the pulled segment (ischemia)
results in severe obstruction

Management

Group four are patients of the good prognostic
type and have a well-located rectum, good
sacrum, and good muscles but are still incontinent
or constipated.

No obvious pathology is noted on imaging or
biopsy

They may benefit from biofeedback or other
behavior modification programs to help them
evacuate the rectum at controlled and predictable
times.

3/8/2014

Thank you

References

Rintala R: Postoperative internal sphincter function in anorectal malformations-A manometric study. Pediatr Surg Int 5:127- 130,1990
Frenckner B, Husberg B: Internal anal sphincter function after correction of imperforate anus. Pediatr Surg Int 6:202-206, 1991
Taylor I, Duthie HL, Zachari RB: Anal continence following surgery for imperforate anus. J Pediatr Surg 8:497-503, 1973
Scharli AF, Kiesewetter WB: Imperforate anus: Anorectosigmoid pressure studies as a quantitative evaluation of postoperative continence. J
Pediatr Surg 4:694-704,1969
Pena A, de Vries PA. Posterior sagittal anorectoplasty; important technical considerations and new applications. J Pediatr Surg 1982;17:796811.
Scharli AF, Kiesewetter WB. Imperforate anus: anorectosigmoid pressure studies as a quantitative evaluation of postoperative continence. J
Pediatr Surg 1969;4:694-704.
Husberg B, Lindahl H, Rintala R, et al. High and intermediate imperforate anus: Results after surgical correction with special respect to
internal sphincter function. J Pediatr Surg 1992;27:185-9.
Mollard P, Meunier P, Mouriquand P, et al. High and intermediate imperforate anus: functional results and postoperative manometric
assessment. Eur J Pediatr Surg 1991;1:282-6.
Mishalany HG, WooUey MM: Postoperative functional and manometric evaluation of patients with Hirschsprung's disease. J Pediatr Surg
22:443-446, 1987
Khan O, Nixon HH: Results following surgery for Hirschsprung's disease: A review of three operations with a reference to neorectal Capacity.
Br J Surg 67:436-438, 1980
Weitzman JJ: Management of Hirschsprung's disease with the Swenson procedure with emphasis on long-term follow-up. Pediatr Surg Int
1:100-104, 1986
Soave F: Endorectal pull-through: 20 years experience. Address of the Guest Speaker, APSA, 1984. J Pediatr Surg 20:568-579, 1985
Foster P, Cowan G, Wrenn EL: Twenty-five years experience with Hirschsprung's disease. J Pediatr Surg 25:531-534, 1990
Iwanaka T, Arai M, Kawashima H, et al. Findings of pelvic musculature and efficacy of laparoscopic muscle stimulator in laparoscopy-assisted
anorectal pull-through for high imperforate anus. Surg Endosc 2003;17:278- 81.
Iwanaka T, Arai M, Kawashima H, et al. Laparoscopically assisted anorectal pull-through for rectocloacal fistula. Pediatr Endosurg Innov Tech
2002;6:261- 7.
Pakarinen MP, Koivusalo A, Lindahl H, et al. Prospective controlled long-term follow-up for functional outcome after anoplasty in boys with
perineal fistula. J Pediatr Gastroenterol Nutr 2007;44:436-9.
Yeung CK, Kiely EM. Low anorectal anomalies: a critical appraisal. Pediatr Surg Int 1991;6:333-5.
West KW, Grosfeld JL, Rescorla FJ, Vane DW. Acquired agangliono- sis: a rare occurrence following pull-through procedures for
Hirschsprung's disease. J Pediatr Surg. 1990;25:104-109.