Hirschsprung disease

[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]

[size=6]A four-year old boy presents with chronic constipation and numerous failed attempts at medical management. A contrast enema shows a markedly dilated colon. He undergoes a full thickness rectal biopsy with findings consistent with neuronal intestinal dysplasia (NID). The histopathology of NID is best characterized by A hypoplastic ganglion cells. B absence of ganglion cells. C small ganglia within the myenteric plexus. D giant ganglia within the submucosal plexus. E decreased acetylcholinesterase activity in the lamina propria.[/size]

First one with correct answer with justification win. A two-year old boy with trisomy 21 underwent a leveling colostomy for aganglionosis as a newborn. A Duhamel procedure was performed at seven months of age. He has been hospitalized on three subsequent occasions for increasingly severe enterocolitis. His abdomen is chronically distended and his parents have been diligent with rectal irrigations at home. A digital rectal exam is normal. Plain radiographs and a contrast enema are obtained. [center] [/center] [size=20px]Anorectal manometry demonstrates poor relaxation of the internal anal sphincter. The best next step in this postoperative Hirschsprung patie…

[size=6]A 5-year-old child underwent a Yancey-Soave pull through for Hirschsprung disease as a newborn. They present to your office due to concerns about infrequent stools and abdominal distension. A thorough workup reveals no twist, stricture, or retained cuff. There are circumferential ganglion cells at the anastomosis. What is the next best step in management? a Injection of botulinum toxin into the internal sphincter. b Revision of the pull through. c Biofeedback therapy. d Twice daily dilations. e Lateral internal anal sphincterotomy.[/size]

any one want link of HQ video please contribute with one thread to forums, and I will sent it to you by email Edit: available without restrictions in next post

[size=6]A 4-year-old child who underwent a Yancey-Soave pull through for Hirschsprung disease as a newborn continues to have infrequent stools and abdominal distension. There is no twist, stricture, or retained cuff. There are circumferential ganglion cells at the anastomosis. What is the next best step in management? a Twice daily dilations. b Injection of botulinum toxin into the internal sphincter. c Revision of the pull through. d Lateral internal anal sphincterotomy. e Biofeedback therapy.[/size]

see video link in second post

An 11-year-old girl presents to your clinic with a history of chronic constipation. She underwent a Duhamel procedure for Hirschsprung disease when she was a newborn. Previous transition zone was identified in the proximal-descending colon based upon review of medical records. She is markedly underweight and has been undergoing regular rectal irrigation and disimpaction. An abdominal film and contrast enema were obtained. Examination under anesthesia does not reveal a retained Duhamel spur. What is the best next step in the management of this patient? [size=20px]a Diverting ostomy b Revision Duhamel c Rectal irrigation/disimpaction d Revision Swenson e…

Any one want a high quality PDF of this chart, post in this thread and use a personalized picture as an avatar, I will send it to you in a private message Edit: available without restrictions

.

.

Quote from "The SAGES Manual of Pediatric Minimally Invasive Surgery" by Danielle S. Walsh, Todd A. Ponsky, Nicholas E. Bruns - "The diagnosis of HD must be confirmed by an experienced, reliable pathologist with a rectal biopsy. This technique is best suited for short-segment disease, and ideally the patient would have a barium enema study demonstrating a distal transition zone. Longer segment disease is a relative contraindication for this surgical option, and these patients may be better served by a Duhamel procedure l. Prematurity is a relative contraindication, as these patients may not have fully mature ganglion cells and histologic evaluation of biopsies may ther…

see video link in second post

see paper link in second post

see video link in second post

[center]by Dr Hussain kotawala see in next post[/center]

see video HQ link in next post

.

.

.

.

See video link in second post

A 2-day old term baby boy weighing 3.2 kg,who already passed meconium, presents with bilious emesis. Pre-eclampsia occurred during the last week of pregnancy. The abdomen is distended but soft and non-tender. Plain film and contrast enema are shown. How would you manage this patient?