Happy Monday all. I hope all who celebrate had a wonderful holiday weekend – a rare calendar confluence of Easter, Passover, and Ramadan!
Last quiz I asked you about an 11 year old boy who we saw with chronic distension and constipation, with severe failure to thrive. An anorectal manometry failed to demonstrate a RAIR, but the rectal biopsy did show ganglion cells – so no Hirschsprung disease. The colon is massively distended on plain xray and on contrast study with no area of obstruction.
This patient likely has diffuse colonic dysmotility. It is a confounding diagnosis and without an obvious treatment plan in the published literature. Given his malnutrition I believe he needs a diversion – typically that would be an ileostomy to divert the nonfunctional colon. He will need assessment of the colonic motility, I expect that a colonic manometry (CMAN) when done will likely show no HAPCs throughout. I anticipate that with diversion he will thrive. Some of you suggested antegrade flushes, which could be attempted, but the malnutrition makes me want to get him better quickly, which will occur with a diversion. Based on the colonic motility evaluation, the stoma could likely be closed in the future with a simultaneous colonic resection – a Deloyer’s procedure plus a Malone or a subtotal colectomy with an ileorectal anastomosis are the two surgical options. He will need AMAN assessment of his ability to empty the rectum (balloon expulsion test), and then will need pelvic floor physiotherapy if he needs to improve his ability to empty the rectum.
OK, for this week’s quiz, we have a 4 year old boy previously operated on for total colonic Hirschsprung disease, with an ileo Duhamel reconstruction. He has had recurrent episodes of obstructive symptoms and several admissions for enterocolitis. His contrast study is below. What is the problem and what would be your next step?
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
Colorectal Quiz Episode 28: Female ARM Management - Perineal Fistula https://staycurrentapp.app.link/lLH7KQr20nb
Colorectal Quiz Episode 30: Tethered Cord https://staycurrentapp.app.link/sE57H6IPzob
Colorectal Quiz Episode 31: Müllerian Anomalies in patients with ARM
https://staycurrentapp.app.link/FuQyrjuC8ob