A 1 month female neonate presented with gross abdominal distension and history of not passing  feces   since  a week.Her X-ray is given below.What would be the most probable cause for her condition?




ANSWER -Hirschsprung's disease

Hirschsprung's disease is caused by a developmental failure of the parasympathetic plexuses of Auerbach and Meissner in the gut, leading to constipation and gut dilation.This condition commonly seen in the neonatal period by the failure of passage of meconium followed by obstructive constipation.
CLINICAL FEATURES
 Usually manifests itself in the immediate neonatal period.
Delay in passing meconium followed by obstructive constipation with abdominal distension. 
When performing rectal examination, It usually produces large quantities of gas and faeces.
In mild form of disease  chronic and severe constipation, with gross abdominal distension & Failure to thrive are common.

INVESTIGATIONS
  1. Rectal biopsy
  2. Anorectal manometry: rectal distention demonstrates an absence of the normal rectoanal inhibitory reflex
  3. Barium enema

TREATMENT
Surgical Intervention  -excision of the aganglionic segment and subsequent anastomosis to the anal canal if possible
Definitive Rx  performed at between six to nine months of age
Initial treatment in the neonatal period is by colostomy to relieve obstruction and prevent enterocolitis



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4 comments

  1. Anonymous Says:
  2. multiple air-fluids levels sign for intestinal obstruction, hurscsprung's disease

     
  3. Anonymous Says:
  4. Hirschsprung's disease

     
  5. Anonymous Says:
  6. hirschsprungs disease

     
  7. Anonymous Says:
  8. inguinal hernia vs hirschsprung disease

     

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