COLORECTAL QUIZ: March 7, 2022

Ron Reeder • March 7, 2022

Last week I asked you about a case that Giulia Brisighelli sent us from Johannesburg:

“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.”

 

Many of you were confounded by this case, perhaps not having seen such a situation before. This is intractable pain related to sphincter spasm is called proctalgia fugax. This is something that our adult colleagues see a lot of, but we rarely see in pediatrics. It was one of the main reasons I started doing my total colonic pull-throughs at an earlier age, rather than waiting for potty training, because I had several patient who experience these terrible painful episodes because they were desperately holding in the stool leading to sphincter spasm. The treatment is botox, and this dramatically improved this child.

 

For this week we have a very challenging case sent to us from Akemi Kawaguchi, our good friend at the University of Texas at Houston, who also supplied the below very helpful diagrams. A partner of hers took care of a newborn with free air and found an ileal and cecal perforation, requiring a right colon plus very distal ileal resection and an ileostomy. A biopsy send from the lower sigmoid showed no ganglion cells confirming the suspicion that this was due to Hirschsprung disease. After the baby recovered, Akemi performed a laparoscopic mapping and found ganglion cells in the transverse colon, but no ganglion cells at the splenic flexure or distal.

What would you do?

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


Some announcements of future meetings – please join for these virtual offerings by the pcplc.

 

Colorectal Conundrum: January 29, 2024

By Ron Reeder • January 29, 2024
Colorectal Conundrum for January 29: A male with a rectoperineal fistula undergoes a PSARP with mobilization of the rectum, both anterior and posterior rectal walls. In the days following surgery he starts to drain urine around the anoplasty consistent with a urethral injury. He is also voiding via the penis. A cystogram is shown below. How would you manage this situation? Answer: This patient has suffered from a urethral injury, and urine is draining out the posterior urethra into the perineum around the anoplasty. The key first step is to divert the urine with a suprapubic tube. A colostomy is not necessarily needed. The fistula might heal with diversion. If after a month or so a cystogram shows the persistence of the fistula, then a redo is needed with re-mobilization of the rectum, fully lifting the anterior rectal wall off of the urinary tract, and repair the urethra, with coverage of the posterior urethra with an ischiorectal fat pad. Then a voiding trial a month later with ultimate removal of the SP tube.

Colorectal Conundrum: January 22, 2024

By Ron Reeder • January 22, 2024
Colorectal Conundrum for January 22nd: In the previous week’s case, of an imperforate hymen, the MRI showed dilation all the way down to the perineum. Management involved a perineal – introital – incision to drain the fluid. If that were not the case and you had a dilated vagina but the distal extent was far away from the introital area, how might you handle that hydrocolpos? Answer: If this were a case of hydrolpos with normal anus and normal urethra, and the vagina cannot be drained by the introitus like an imperforate hymen, then drainage needs to be from above. IR or laparoscopy is an ideal approach to get a drain in the dilated structure (remember this could be bilateral, so both sides may need to be drained. Drainage is to relieve distension and most concerning its potential cause of distal ureteral obstruction causing hydronephrosis. Once the material hormones have dissipated the hydrocolpos will resolve and can be dealt with later in life via a laparoscopic distal vaginal pull-through, after the onset of puberty. In such a patient, I would follow closely with ultrasound 6 months after thelarche (breast budding).

Colorectal Conundrum: January 16, 2024

By Ron Reeder • January 16, 2024
Colorectal Conundrum for January 16: A newborn female is noted to have abdominal distension. On exam the uretha and anus are normal, and there appears to be a bulge in the hymen. The has an MRI with the image shown below. What would be your treatment plan? What are the variations in such a case and how would you manage each? Answer: This could be a case of imperforate hymen, but the MRI is key – how high up is the obstruction? How large is the hydrocolpos? In this case, it appears that a perineal incision might all that is needed as the dilation extends all the way down to the perineum. That is the most common scenario. An incision at the level of the hymen should drain out the fluid and no further intervention should be needed. Of course a careful exam needs to check to be certain you see a urethra – sometimes such a bulge could be a paraurethral cyst. For the next week’s question – what would you do if the perineal approach was inadequate – i.e. could not reach the dilated lumen.
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