Last week I asked you about a 1 year old boy who was ex-premature (32 weeker), with a VSD, weighing 1.5 kg, who had distension in the newborn period and a rectal biopsy showing HD. The contrast study had shown no obvious transition zone. He went to the OR for a planned pull-through but biopsy of the proximal sigmoid on frozen section showed no ganglion cells. The cecum and ileum were biopsied and showed ganglion cells. The surgeon then opened an ileostomy with no further biopsies. I asked you what would you do now?
Many of you chose to map the colon, wait for permanent section and then proceed with the pull-through, and I like that idea very much, particularly if frozen section is not reliable. One technical point that if you do this, is to mark your biopsy sites with a proline (or another permanent) suture so you can find those biopsy sites when you return. In this case that was my plan but I did check the left colon as a first step and I do have very reliable frozen section available to me. That left colon showed ganglion cells so we proceeded with the pull-through. I believe you can close the ileostomy at the same time if you feel confident that the pull-through is healthy, with good blood supply and no tension. Although leaving the ileostomy is also fine, as this allows for a very quick recovery and you could close the ileostomy in 1-2 months, with a check at the same time of the anastomosis. In that case I would also place botox into the anal canal at the same time.
Now for this week, another conundrum:
Giulia Brisighelli and her incredible nurse Catterina Bebington from Johannesburg sent us this case:
“A 2.5 year old boy with total colonic HD presented with severe perianal pain. He had had a successful ileoanal pull through one year previously after demonstrating the capacity for formed stools in his ileostomy bag. Surgery was uneventful and he was discharged 5 days post op. He initially had very frequent bowel movements and a severe rash but with very aggressive local treatment he improved. In the past 6 months he has 4-5 stools a day, mom reports he can hold the stool and he easily communicates when he is about to have a bowel movement. He however has recurrent and incapacitating pain in the anal area. Every 15 minutes or so he has this sudden movement like he is trying to contract his bottom, walks on his toe tips, and jumps around screaming in agony. This happens day and night. He does have a perineal rash with slightly raised red bumps, that is non-bleeding, and not particularly tender to touch. In between lesions the skin is not red or inflamed. On exam he has good tone and no palpable anastomosis, no stricture at all, and the stool is not foul smelling and alternates between soft and liquid. In between pain episodes he is a happy playful and a very smart child. He has a normal weight and height for age and he is a good eater. His abdomen is soft and not distended. We have tried irrigations, augmentin alternating with metronidazole, loperamide and on a constipating diet. He has been on chloromax, vaseline, or abase and castor cream. He has been on paracetamol and ibuprofen with no improvement. The episodes are incapacitating.”
Here is the current list of the Colorectal Quiz podcasts, best viewed via the StayCurrent app:
Colorectal Quiz Episode 1 ARM - Low Bulbar Fistula, https://staycurrentapp.app.link/wK2FhonUEhb
Colorectal Quiz Episode 2 When to redo a PSARP https://staycurrentapp.app.link/RX2eeDqUEhb
Colorectal Quiz Episode 3 Hirschprung Disease https://staycurrentapp.app.link/UnFRkprUEhb
Colorectal Quiz Episode 3.5 Proximal Hirschsprung Disease https://staycurrentapp.app.link/JiaWCGsUEhb
Colorectal Quiz Episode 4 Classic Hirschsprung disease - Surgical Technique https://staycurrentapp.app.link/qGWLoGtUEhb
Colorectal Quiz Episode 5 Proximal Hirschsprung Disease Surgical Technique https://staycurrentapp.app.link/AiGHjcvUEhb
Colorectal Quiz Episode 6 Bowel Management Part 1 https://staycurrentapp.app.link/SVuVYWvUEhb
Colorectal Quiz Episode 7 Bowel Management Part 2 https://staycurrentapp.app.link/b8IhkKwUEhb
Colorectal Quiz Episode 8 Motility Disorders Part 1 https://staycurrentapp.app.link/NRerIoxUEhb
Colorectal Quiz Episode 9 Motility Disorders Part 2 https://staycurrentapp.app.link/s2cVlbyUEhb
Colorectal Quiz Episode 10 Total Colonic Hirschsprung Disease Part 1 https://staycurrentapp.app.link/fEtTMRyUEhb
Colorectal Quiz Episode 11 Total Colonic Hirschsprung's Part 2 https://staycurrentapp.app.link/MNqS9yzUEhb
Colorectal Quiz episode 12 Newborn ARM Part 1 https://staycurrentapp.app.link/x5UL6DBUEhb
Colorectal Quiz Episode 13 Newborn ARM Part 2 https://staycurrentapp.app.link/YqVpTmCUEhb
Colorectal Quiz Episode 14 ARM Newborn Part 3 https://staycurrentapp.app.link/x9mUY7CUEhb
Colorectal Quiz Episode 15 Bowel Management in Spinal Pts. Need for a urologist Part 1 https://staycurrentapp.app.link/etX3mPDUEhb
Colorectal Quiz Episode 16 Bowel Management in Spinal Pts. Need for a urologist Part 2 https://staycurrentapp.app.link/5C7GsJIDEhb
Colorectal Quiz Episode 17 Cloaca Part I https://staycurrentapp.app.link/YGPmYZPEPhb
Colorectal Quiz Episode 18 Cloaca Part II https://staycurrentapp.app.link/vUEzQxG80hb
Colorectal Quiz Episode 19 Hirschsprung Disease - The Obstructed Patient Part 1 https://staycurrentapp.app.link/gBzrAtLKLib
Colorectal Quiz: Episode 20 Hirschsprung Disease – The Obstructed Patient Part 2 https://staycurrentapp.app.link/W7kEcqB6vjb
Colorectal Quiz Episode 21 The History of Hirschsprung Disease https://staycurrentapp.app.link/QXtFg2UlTjb
Colorectal Quiz Episode 22 Hirschsprung Disease - the Soiling Patient Part 1 https://staycurrentapp.app.link/OII38hVhQkb
Colorectal Quiz Episode 23 Hirschsprung Disease The Soiling Patient Part 2 https://staycurrentapp.app.link/DA4WEDIcXlb
Colorectal Quiz Episode 24 Cloaca Part 3 https://staycurrentapp.app.link/3uuEG49mkmb
Colorectal Quiz Episode 25 Perineal Groove https://staycurrentapp.app.link/PxlGWtfswmb
Colorectal Quiz Episode 26 Perianal Crohn's Disease https://staycurrentapp.app.link/qQbadIDjTmb
Colorectal Quiz Episode 27: Delayed Hirschsprung Disease https://staycurrentapp.app.link/v37xYPDSDnb
Some announcements of future meetings – please join for these virtual offerings by the pcplc.