Hirschsprung Disease Introduction
- Hirschsprung Disease is the most common cause of lower intestinal obstruction in Neonates.
- Hirschsprung Disease also known as ganglionic megacolon
- Hirschsprung disease occurs due to the congenital absence of ganglionic nerve cells in a muscular and submucosal layer in the distal part
- It causes the excessive dilation of a proximal end of the distal part
- Most common site – RECTOSIGMOID COLON
- Hirschsprung disease is mainly associated with the – DOWN SYNDROME
Incidence of Hirschsprung Disease
- 1 in 5000 live birth
- 70% – 80% in boys
- 95% of cases are full-term babies.
Hirschsprung Disease Types
- Ultra-short segment – Below the rectosigmoid junction
- Short segment – Up to Sigmoid colon
- Long Segment – Up to splenic flexure or beyond.
- Total segment – Affect the whole colon.
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Hirschsprung Disease Clinical feature
In newborn Hirschsprung Disease Clinical Feature
- The child does not pass meconium up to 24 hours
- Abdominal distension and Bilious vomiting.
- Enterocolitis (Inflammation of large intestine and small intestine)
- Hypovolemic shock – Due to severe vomiting
- Neonatal intestinal obstruction
In Older child Hirschsprung Disease Clinica Feature
- Ribbon like stool
- Failure to weight gain and delay growth
- Abdominal distention and bilious vomiting
- Constipation and failure to thrive
- Dehydration and Explosive Diarrhea
Hirschsprung Disease Diagnosis
- History collection and physical examination
- Barium Enema
- Anorectal manometry -> Useful in neonates
- Ultrasonography -> For associated anomalies
- Rectal Biopsy -Most confirmatory tests, Done by suction method
Hirschsprung Disease Treatment
- For Constipation
- Laxatives and stool softener
- Isotonic Enema
- Low residue Diet
- Avoid milk and milk products
- Daily fibers requirement of child – 7-10 gm/day
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Hirschsprung Disease Surgical Intervention
- Pull throw procedure is the main surgical procedure of Hirschsprung disease.
- These cut and remove the disease portion and end to end anastomosis, or replace at normal site.
- The producer done by different methods
Swenson procedure pulls the colon and removes the disease portion by oblique cut. The remaining portion of colon attach by end-to-end anastomosis.
In this procedure transverse cut the disease portion and end to end anastomosis by surgical stapler.
These procedures leave outer wall of colon and end to anastomosis.
Hirschsprung Disease Pre-operative Care
- Monitor the abdominal girth
- Low residue diet provides
- Maintain semi fowler position for lungs expansion
- Rectal irrigation by normal saline
- Provide pain reliever
Hirschsprung Disease Post Operative Care
- Assess the surgical site à Redness, swelling and drainage
- Maintain NPO status (within 48-72 hours)
- Monitor the abdominal girth
- Provide the IV fluid when child does not tolerate oral feeding.
- Assess the Hydration status and electrolyte balance
- Instruct to parents about colostomy care.
Hirschsprung Disease Complication
- Excessive Hemorrhage
- Hypovolemic shock
- Fecal incontinence
Hirschsprung Disease Key Points
- A most common cause of lower intestinal obstruction in the Neonate – Hirschsprung Disease
- Most common site of Hirschsprung disease – Rectosigmoid colon
- The Hirschsprung Disease is mainly associated with – Down syndrome
- A most common complication of Hirschsprung Disease – Enterocolitis
- Ribbon-like stool seen in the Hirschsprung Disease
- Most Confirmatory test of Hirschsprung Disease – Rectal Biopsy
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