A brief presentation.

1. HIRSCHPRUNGS
DISEASE
Dr Reshma Chandrasekaran
DNB General Surgery Resident

2. Hirschsprung disease (HD):
Congenital anomaly of enteric nervous system :
Absence of ganglion cells in myenteric & submucosal
plexus of intestine due to neuro-cristopathy (failure of
neural crest cells - precursors of enteric ganglion cells to
migrate completely during intestinal development during
fetal life).
Lack of peristalsis → functional bowel obstruction

5. Aganglionic segment extends retrograde from anus for
variable length with gradual transition to innervated
colon.
Level of transition may be
–Rectosigmoid: Short-segment HD (70-80%)
–Proximal to rectosigmoid: Long-segment HD (15-25%)
–Entire colon: Total colonic HD (4-13%)
–Colon & small bowel (SB): Total intestinal HD (very rare)
–Just above anorectal verge: Ultrashort-segment HD (very
rare)

8. • The diagnosis is made on a suction rectal biopsy.
• A contrast enema may show the narrow
aganglionic segment, a dilated proximal bowel.
• Daily rectal washouts may allow a period of growth
at home before surgery.
• If decompression fails, a stoma is fashioned using
frozen section histopathology to identify ganglionic
bowel.

11. Surgical treatment
Definitive surgery removes the aganglionic segment
and brings ganglionic bowel to the anus; Swenson,
Duhamel Yancey- Soave and transanal 'pull-throughs'
are options.
Most children achieve reasonable bowel control, but
some have residual constipation, incontinence or
episodes of enterocolitis.

20. OPEN RECTAL BIOPSY

39. > 5 months and
weight > 8kg

50. Steps :
Submucosal dissection
Mobilisation of rectum
Resection of aganglionic segment
Anastamosis with ganglionic bowel