[size=20px]A three-year-old boy presents with refractory constipation following an uncomplicated Yancey-Soave endorectal pull-through for Hirschsprung disease. The most common condition requiring redo pull through operation is

 

A twisted pull through.

 

B tight muscular cuff.

 

C anastomotic stricture.

 

D motility disorder.

 

E aganglionosis.

[/size]

E

E

E

E

E

Correct answer

E aganglionosis.

 

Successful surgical correction of Hirschsprung disease involves removal of the aganglionic bowel and restoration of gastrointestinal continuity using proximal normally innervated bowel. Postoperative difficulties may include constipation, soiling or enterocolitis. In the absence of obstruction these symptoms usually resolve. However, ongoing difficulties require further work up including rectal exam, contrast enema and rectal biopsy.

 

Although all of the above can be responsible for refractory obstruction, the most common indication for a redo pull through is residual aganglionosis. Possibilities include initial pathologist error, use of transition zone segment for the pull through or acquired aganglionosis due to loss of ganglion cells. It is essential that an experienced pathologist examine the entire circumference at the initial pull through for both ganglion cells and hypertrophic nerves to minimize this complication.

 

For Hirschsprung disease, the Soave or endorectal pull-through was described in 1964. Yancey described the same technique in 1952 but because of his race was published in a less read journal. The procedure is now being renamed the Yancey-Soave to give proper historical credit.

E

Residual aganglionosis or a transition-zone pull-through is the most commonly reported indication for redo pull-through in many series. The abnormally innervated bowel causes persistent functional obstruction and requires resection, followed by pull-through of normally ganglionated bowel