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Hirschsprung's disease - Coggle Diagram

- - - - Myenteric (Auerbach's) plexus
      - Submucous (Meissner's) plexus
    - - Non-myelinated
      - Space normally occupied by ganglionic cells
    - - Anorectal region to proximal extension
      - Variations in affected segments
- - - - Failure to pass meconium in the first 24 hours
      - Explosive evacuation of gas and foul-smelling stools upon rectal examination
    - - Persistent constipation and chronic abdominal distension
  - - - Over 90% fail to pass meconium in the first 24 hours
      - Normal meconium passage within 24 hours after birth in healthy full-term babies
      - Passage within 48 hours in all healthy full-term babies
    - - Constipation, abdominal distension, vomiting during the first few days of life
      - Explosive evacuation of gas and foul-smelling stools upon rectal examination or introduction of a rectal tube
  - - - Suggestive of enterocolitis
    - - Sudden onset of marked abdominal distension
      - Bile-stained vomiting, fever, signs of dehydration, sepsis, and shock
  - - - May be present in both neonates and older children
    - - Key indicator in neonates
    - - Explosive evacuation of gas and foul-smelling stools
    - - Suggestive of enterocolitis and potential progression to toxic megacolon
  - - - Emphasis on meconium passage and early signs of obstruction
    - - Persistent constipation and abdominal distension
- - - - Dilated loops of bowel with fluid levels and airless pelvis
      - Presence of air in the bowel wall may suggest enterocolitis
      - Pneumoperitoneum possible in cases of perforation
    - - Essential imaging for initial assessment
  - - - Conducted without bowel preparation to preserve transitional zone
      - Typical HD case shows barium flow from undilated rectum through a cone-shaped transitional zone into dilated sigmoid colon
      - Reversed recto-sigmoid index (<1) compared to normal (>1)
      - Delayed film at 24 hours may confirm diagnosis by demonstrating retained barium
    - - Confirmatory diagnostic tool, especially revealing the characteristic transition zone
  - - - Distending the rectal balloon with air elicits recto-anal inhibitory reflex (RAIR)
      - Immediate transient rise in pressure and simultaneous depression of pressure in the internal sphincter
    - - Complete absence of internal sphincter relaxation, i.e., absent RAIR
    - - Provides functional insights supporting the diagnosis of HD
  - - - Suction biopsy in neonates (safe, painless, bedside procedure)
      - Full-thickness open biopsy in older children for more tissue and deeper levels
    - - Biopsy site at least 0.6 in. (1.5 cm) above the dentate line
      - Distal rectum normally lacks ganglion cells
    - - Absence of Meissner and Auerbach's ganglion plexuses
      - Marked hypertrophy of nerve trunks supporting HD diagnosis
      - Essential for definitive confirmation of HD + important medico-legal
- - - - Creation of a diverting colostomy
      - Timing: At the time of diagnosis
      - Purpose: Temporary diversion to alleviate symptoms
    - - Timing: Once the child grows and weighs more than 10 kg
      - Procedure: Surgical correction to remove aganglionic segment
  - - - Procedure performed without diverting colostomy
      - Management: Regular rectal washouts and suppositories
      - Timing: Surgery conducted few months later as the child grows
    - - Variety of procedures applicable
      - Advantages: Reduced invasiveness, quicker recovery
    - - Procedure performed without opening the abdomen
      - Application: Particularly effective in rectosigmoid HD
      - Outcomes: Demonstrates excellent results
  - - - Achieved through appropriate fluid infusion
    - - Essential for babies with life-threatening complications
      - Techniques: Rectal irrigations or life-saving colostomy if necessary
  - - - Essential for postoperative follow-up
      - Identifying complications: Wound infections, anastomotic leak, strictures, necrosis, ileus
    - - Monitoring for late complications: Constipation, enterocolitis, incontinence, anastomotic problems, adhesive bowel obstruction, urogenital complications
- - - - Removal of all aganglionic bowel up to 1 cm from the dentate line posteriorly
      - Anastomosis: Colo-anal anastomosis from the outside
    - - Preservation of a margin of healthy tissue to ensure optimal function
      - Key in addressing the aganglionic segment
  - - - Dissection behind the rectum (to minimize pelvic nerve damage) to create a tunnel
      - Anastomosis: Ganglionic bowel brought through approximately 1 cm above the dentate line, creating a side-to-side anastomosis using a stapler
    - - Emphasizes minimizing pelvic nerve damage
      - Use of stapler for precise and secure anastomosis
  - - - Continuation of colon dissection in the submucosal plane to approximately 1 cm from the dentate line at the pelvic reflection
      - Anastomosis: Ganglionic bowel pulled through the rectal muscle sleeve, anastomosed to anal mucosa
    - - Extramucosal dissection to create anastomosis
      - Effective in rectosigmoid HD