Long-segment Hirschsprung: Aganglionic bowel proximal to midtransverse colon
Embryology and Etiology
Ganglion cells originate from the neural crest tissues by 13 weeks postconception from proximal to distal GIT.
2 theories:
- Failure of migration of neural crest tissues.
- Failure of migrated ganglionic cells to mature and proliferate
Associated Syndromes
Down syndrome
Neurocristopathy syndromes (ie. Waardenberg-Shah syndrome)
Malrotation
Congenital heart disease
Urinary tract anomalies
Central nervous system anomalies
Congenital central hypoventilation syndrome (Ondine curse)
Clinical Features
Neonates:
- Delayed passage of meconium after 24 hours
- Abdominal distension
- Bilious vomiting
- Feed intolerance
- Diarrhea, fever, abdominal distension (Hirschprung-associated enterocolitis [HAED])
Investigations
Radiographic
- Abdominal X-ray
- Barium enema
- Transition zone between normal and aganglionic bowel
- Reversed recto-sigmoid index (< 1.0)
- Recto-anal inhibitory reflex (RAIR). Reflex that causes the internal anal spinchter to relax when the rectum is distended.
- Balloon inserted into the rectum and inflated. If reflex present detected by sensor.
- Test more useful with older children.
- Absence of ganglionic cell
- Hypertrophied nerve trunk
- Acetylcholinesterase staining
- Absence of calcitonin on immunochemical testing with calretinin
Resuscitation
- Fluids
- Nasogastric tube
- Antibiotics
May be done through laparotomy or more popular, pull-through surgery via the anus.
- Swenson (end-to-end anastamosis of the ganglionic segment with the internal anal sphincter. Rectum is excised)
- Soave (As Swenson but rectum is preserved becoming the outer 'cuff' of the pulled ganglinic segment)
- Duhamel (The ganglinic segment is joined to the rectum in a end-to-side anastamosis)
- Enterocolitis
- Perforated bowel
- Very dilated proximal bowel
- Ileostomy
- Small bowel pull-through to anastamose with the anal sphincter
- Side-to-side anastamosis between small bowel and large bowel (to preserve absorptive roles). Several techniques: Martin and Kimura
- Anal calibration 4 to 6 weeks
- Buttock care with barrier cream to prevent perineal wound breakdown
References:
1. Coran, Arnold G., et al., Pediatric Surgery, 7th Ed., Elsevier
Both pictures from http://radiopaedia.org/cases/hirschsprung-disease
very interesting my daughter who is 12 has Congenital Central Hypoventilation Syndrome however it was just found out that she also has HD.. crazy that even though we always knew she had CCHS it wasn't until 2 months ago we realized she also had HD
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