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Adult Outcomes: Hirschsprung Disease

Video Published 2019-01-11 Updated 2026-06-10

Timestops (6)

Introduction and Study Design

Speaker introduces Finnish national registry advantages (social security number tracking) and describes a population-bas…

Adult Hirschsprung Outcomes

Results show significantly worse bowel function scores in patients (mean 17.1 vs 19.1 in controls), with constipation, s…

Conclusions: Adult Cohort

Bowel function remains impaired in a significant proportion of adults; increasing age worsens outcomes. Overall GI quali…

Long-Segment Aganglionosis Cohort

Retrospective review of 25 patients (1984–2013) with total colonic ± small-bowel aganglionosis. Five patients with near-…

Functional Outcomes After Pouch Procedures

Patients with pouch anal anastomosis have voluntary bowel movements (median 4/day), low rates of soiling, no constipatio…

Conclusions: Long-Segment Cohort

Aganglionosis beyond 50 cm of terminal ileum rarely achieves functional autonomy or long-term survival without transplan…

Topic Overview

A Finnish surgeon presents population-based outcomes data on adults operated for Hirschsprung disease in childhood (1960–1986) and a separate cohort of children with long-segment/total colonic aganglionosis (1984–2013). In the adult cohort (n=143 eligible, 86 matched controls), bowel function remains impaired in a significant proportion: constipation, soiling, and social problems affect 15–33% of patients, and increasing age predicts worse functional scores. However, gastrointestinal quality of life is comparable to controls in most patients, with poor QOL in about one-fifth. In the long-segment cohort (n=25), aganglionosis extending beyond 50 cm of terminal ileum is associated with dependence on parenteral nutrition and poor survival without small-bowel transplantation; patients with purely colonic involvement achieve bowel continuity and reasonable function after pouch procedures, though obstructive episodes and enterocolitis are frequent.

Key Takeaways

One-third of adult Hirschsprung patients have persistent constipation, soiling, or social problems; outcomes worsen with age. (9:16)
Aganglionosis beyond 50 cm of terminal ileum predicts poor survival and parenteral nutrition dependence without transplant. (17:29)
Gastrointestinal QOL is comparable to controls in most adults, but 22% have poor QOL (GIQLI <110). (8:24)
Pouch anal anastomosis in colonic-only aganglionosis yields reassuring function; enterocolitis and obstruction are manageable. (18:10)

Inside this episode

Kai, the Library's AI content creator, listened to this episode and mapped who's speaking, the chapters, key claims, and cases. Every item links to the exact moment in the recording.

AI-enriched

Who's speaking

Speaker 1 — guest
Speaker 2 — host
Speaker 3 — host

Chapters

0:00Introduction and Study Design — Speaker introduces Finnish national registry advantages (social security number tracking) and describes a population-based cross-sectional study of 143 adults operated for Hirschsprung disease 1960–1986, using 86 matched controls and validated bowel function and quality-of-life scores.
4:57Adult Hirschsprung Outcomes: Bowel Function and Quality of Life — Results show significantly worse bowel function scores in patients (mean 17.1 vs 19.1 in controls), with constipation, soiling, and social problems more frequent. Gastrointestinal quality of life is marginally lower but not statistically different; 22% of patients score below 110 (poor QOL threshold). Increasing age is the only predictor of poor functional outcome.
10:42Conclusions: Adult Cohort — Bowel function remains impaired in a significant proportion of adults; increasing age worsens outcomes. Overall GI quality of life is comparable to controls, but decreased in one-fifth of patients.
11:44Long-Segment Aganglionosis Cohort: Patient Characteristics and Outcomes by Extent — Retrospective review of 25 patients (1984–2013) with total colonic ± small-bowel aganglionosis. Five patients with near-total bowel involvement remain on parenteral nutrition; four with mid-small-bowel extension had high mortality. Patients with <50% small-bowel or purely colonic disease achieved bowel continuity and were weaned from PN.
18:10Functional Outcomes After Pouch Procedures — Patients with pouch anal anastomosis have voluntary bowel movements (median 4/day), low rates of soiling, no constipation, but frequent enterocolitis (treated with metronidazole/ciprofloxacin) and obstructive episodes (managed with Botox injections, up to 6 required in some).
19:28Conclusions: Long-Segment Cohort — Aganglionosis beyond 50 cm of terminal ileum rarely achieves functional autonomy or long-term survival without transplantation. Purely colonic disease has reassuring bowel function after pouch, though enterocolitis and obstruction are frequent but manageable. Long-term follow-up is needed.

Key claims

0:08Finland uses a national social security number that allows tracking of all patients and access to national medical records. — Speaker 1
0:52There are very few controlled studies in adults operated for Hirschsprung disease in childhood. — Speaker 1
1:23The study cohort comprised patients operated 1960–1986, with 143 eligible after excluding deaths and migrants, and 86 matched controls. — Speaker 1
1:55Most operations were Duhamel procedures, with Soave and Swenson operations in about 10% and 25% respectively. — Speaker 1
4:07The average bowel function score in healthy adults is 1.1 (low score = poor function, high score = good function). — Speaker 1
4:42The average gastrointestinal quality-of-life score (GIQLI) in healthy adults is 125.8, with a maximum of 144. — Speaker 1
5:16Constipation, soiling, and social problems related to bowel function were much more frequent in Hirschsprung patients than controls. — Speaker 1
5:53Mean bowel function score was 17.1 in patients vs 19.1 in controls, a statistically significant difference. — Speaker 1
6:1025% of patients scored full 20 points on bowel function vs 50% of controls. — Speaker 1
6:2413% of patients reported frequent soiling, 2% had accidents, and 10% had complications requiring treatment. — Speaker 1
8:11Gastrointestinal quality-of-life scores were marginally lower in patients but not statistically significant. — Speaker 1
8:2422% of patients had a GIQLI score lower than 110, indicating poor gastrointestinal quality of life. — Speaker 1
8:34Patients scored worse on 11 of 36 quality-of-life items but better on ability to participate in activities and quality of sleep. — Speaker 1
9:16Increasing age was the only significant predictor of poor functional outcome (bowel function score <17). — Speaker 1
10:08Age was inversely related to bowel function score in patients but not in controls, suggesting outcomes may worsen with aging. — Speaker 1
10:32Low bowel function score was the only predictor of poor quality of life (GIQLI <110). — Speaker 1
10:48Bowel function continues to be impaired in a significant proportion of adults with Hirschsprung disease. — Speaker 1
10:56Abnormalities in urge to defecate, fecal soiling, constipation, and social problems affect about one-third to 15% of adult Hirschsprung patients. — Speaker 1
11:13Overall gastrointestinal quality of life is comparable to controls, but decreased QOL was found in one-fifth of patients. — Speaker 1
11:31The long-segment cohort included 25 patients born 1984–2013 with total colonic aganglionosis ± small-bowel extension. — Speaker 1
12:2621 patients were alive at follow-up; 15 male, 6 female. Four had Down syndrome, two had cartilage-hair hypoplasia, one had congenital central hypoventilation syndrome with neuroblastoma, one had Shah-Waardenburg syndrome. — Speaker 1
13:02Median follow-up was 6.5 years. — Speaker 1
13:10Five patients had aganglionosis extending very near the duodenojejunal flexure (essentially total bowel aganglionosis); four are alive, only one weaned from parenteral nutrition, all have bowel continuity, one underwent successful small-bowel transplantation, another is awaiting transplant. — Speaker 1
14:08Four patients had aganglionosis to mid-small-bowel level; two had syndromic disease contraindicating transplant, two died from severe bowel dysmotility and recurrent sepsis, none weaned from parenteral nutrition, only one has bowel continuity. — Speaker 1
16:41Seven patients had aganglionosis involving less than 50% of small bowel; six had pouch procedures, one had Duhamel; all alive, none on parenteral nutrition, all have bowel continuity. — Speaker 1
17:07Five patients had purely colonic aganglionosis; all alive, all weaned from parenteral nutrition, all have bowel continuity. — Speaker 1
17:29Patients with aganglionosis extending to mid-small-bowel or more proximally are rarely weaned from parenteral nutrition, and this extent affects survival. — Speaker 1
18:10All patients with pouch anal anastomosis have voluntary bowel movements; nine are toilet-trained. — Speaker 1
18:25Stooling frequency is median 4 per 24 hours (range 1–10). — Speaker 1
18:32Four of ten patients have bowel movements at nighttime. — Speaker 1
18:36Two patients have fecal soiling to some degree; none suffer from constipation. — Speaker 1
18:45Most patients had at least one episode of enterocolitis, treated with oral metronidazole or ciprofloxacin. — Speaker 1
19:04Obstructive episodes were common and mainly treated by Botox injections; some patients required more than two injections, maximum six. — Speaker 1
19:28Aganglionosis beyond 50 cm of terminal ileum makes achievement of functional bowel continuity or autonomy exceptional and long-term survival poor without intestinal transplantation. — Speaker 1
19:45If aganglionosis is limited to colon, bowel function after pouch anal anastomosis is reassuring, although obstructive symptoms and enterocolitis are frequent but manageable with Botox and metronidazole. — Speaker 1

Open questions

Will adult Hirschsprung patients' bowel function continue to decline with further aging beyond the current cohort's age range?
What are the very long-term outcomes after pouch anal anastomosis in patients with total colonic aganglionosis?

This episode was analyzed and enriched by Kai, the Library's AI content creator. Every item links to the moment it comes from — click a timestamp to listen in context.

Written for:

Hirschsprung Disease: What Happens to Patients Who Reach Adulthood

The episode's main topic retold as a plain-language walkthrough — what it is, why it matters, and what the speakers concluded. Written by Kai from the episode transcript and reviewed before publishing.

For trainees · Explainer · AI-written, human-reviewed

Why This Matters

Hirschsprung disease is a congenital absence of ganglion cells in the distal bowel, typically diagnosed and surgically corrected in infancy. Most pediatric surgeons follow these patients through childhood, but few know what happens decades later. The Finnish national registry — which assigns every citizen a social security number and tracks medical records across all institutions — makes it possible to answer this question without the selection bias that plagues most long-term outcome studies 0:08.

The Core Problem

Even after anatomically successful pull-through operations, many patients continue to have disordered bowel function into adulthood. The question is how many, how severe, and whether outcomes deteriorate with age. Controlled data have been scarce: there are very few studies comparing adult Hirschsprung patients to matched healthy controls 0:52.

What the Finnish Cohort Shows

A population-based study of 143 adults operated between 1960 and 1986 — most with Duhamel procedures, about 25% Swenson, 10% Soave — used validated bowel function scores and quality-of-life instruments against 86 matched controls [c3, c4]. The bowel function score runs from low to high, with higher scores indicating better function; healthy adults average 19.1 4:07.

Patients scored significantly worse: mean 17.1 versus 19.1 in controls 5:53. Only 25% of patients achieved the full maximum score, compared to 50% of controls 6:10. Constipation, soiling, and social problems related to bowel function were all more frequent in the Hirschsprung group 5:16. Thirteen percent reported frequent soiling, 2% had accidents, and 10% required treatment for complications 6:24.

Gastrointestinal quality of life, measured by a 36-item validated instrument (maximum score 144, healthy adult average 125.8), was marginally lower in patients but did not reach statistical significance [c6, c11]. However, 22% of patients scored below 110, a threshold indicating poor gastrointestinal quality of life 8:24. Patients scored worse on 11 of 36 items but paradoxically reported better ability to participate in activities and better sleep quality 8:34 — a reminder that questionnaire studies sometimes yield counterintuitive results.

The most concerning finding: increasing age was the only significant predictor of poor functional outcome (score below 17) 9:16. Age was inversely related to bowel function score in patients but not in controls, suggesting that outcomes may worsen as these patients move through middle age 10:08. Low bowel function score was the only predictor of poor quality of life 10:32.

Long-Segment Disease: A Different Problem

When aganglionosis extends beyond the colon into the small bowel, the clinical picture changes entirely. A retrospective review of 25 patients with total colonic aganglionosis ± small-bowel extension, followed for a median 6.5 years, stratified outcomes by anatomic extent [c20, c22].

Five patients had aganglionosis extending nearly to the duodenojejunal flexure — essentially total bowel involvement. Four are alive; only one has been weaned from parenteral nutrition. All have bowel continuity. One underwent successful small-bowel transplantation; another is awaiting transplant 13:10.

Four patients had mid-small-bowel involvement. Two had syndromic disease that contraindicated transplantation. Two died from severe bowel dysmotility and recurrent sepsis. None were weaned from parenteral nutrition; only one achieved bowel continuity 14:08.

Seven patients had less than 50% small-bowel involvement. Six underwent pouch procedures, one a Duhamel. All are alive, none require parenteral nutrition, all have bowel continuity 16:41. Five patients had purely colonic disease; all are alive, weaned from parenteral nutrition, with bowel continuity 17:07.

The threshold appears to be at a specific point in the terminal ileum: aganglionosis beyond this point rarely permits functional autonomy or long-term survival without intestinal transplantation 17:29.

Functional Outcomes After Pouch Procedures

For patients with colonic or limited small-bowel disease who underwent pouch anal anastomosis, functional results are reassuring but not problem-free. All have voluntary bowel movements; nine are toilet-trained 18:10. Median stooling frequency is 4 per 24 hours (range 1–10) 18:25. Four of ten have nighttime bowel movements 18:32. Two have some degree of fecal soiling; none suffer from constipation 18:36.

Enterocolitis is common — most patients had at least one episode, treated with oral metronidazole or ciprofloxacin 18:45. Obstructive episodes are also frequent, managed primarily with Botox injections to the pouch; some patients required more than two injections, with a maximum of six documented 19:04. These are manageable complications, not treatment failures, but they require ongoing surveillance 19:45.

When to Involve Long-Term Follow-Up

Any adult patient with a history of Hirschsprung repair who reports worsening bowel function, new-onset soiling, or declining quality of life warrants formal assessment. The finding that outcomes deteriorate with age means these patients need lifelong access to specialized care, not discharge after childhood follow-up. For patients with long-segment disease, the extent of aganglionosis determines whether the goal is functional bowel continuity or preparation for transplantation. Mid-small-bowel involvement carries high mortality and poor functional outcomes; purely colonic disease has a much better prognosis but still requires management of enterocolitis and obstruction.

Takeaways from this story

Bowel function worsens with age in Hirschsprung patients but not controls—these patients need lifelong follow-up, not discharge after childhood.
Aganglionosis extending beyond a critical point in the terminal ileum rarely permits survival without transplantation; purely colonic disease has much better outcomes.
After pouch procedures, enterocolitis and obstruction are common but manageable with antibiotics and Botox—not reasons to avoid the operation.
22% of adult Hirschsprung patients score below the threshold for poor gastrointestinal quality of life despite comparable mean scores to controls.

Adult Outcomes After Hirschsprung Repair: Function Deteriorates With Age

The essential version of this episode — what it covers, the points that matter most, and what it changes for you. Written by Kai from the episode transcript and reviewed before publishing.

For specialists · Core brief · AI-written, human-reviewed

Standard-Length Disease

A Finnish population-based study tracked 143 adults operated for Hirschsprung disease between 1960 and 1986, comparing them to 86 matched controls 1:23. Most underwent Duhamel procedures; Soave and Swenson operations comprised approximately 10% and 25% respectively 1:55.

Mean bowel function score was 17.1 in patients versus 19.1 in controls, a statistically significant difference 5:53. Only 25% of patients achieved the maximum score of 20 points compared to 50% of controls 6:10. Thirteen percent reported frequent soiling, 2% had accidents, and 10% required treatment for complications 6:24. While overall gastrointestinal quality-of-life scores were marginally lower in patients, the difference did not reach statistical significance 8:11. However, 22% of patients scored below 110 on the GIQLI, indicating poor gastrointestinal quality of life 8:24.

The critical finding: increasing age was the only significant predictor of poor functional outcome (bowel function score <17) 9:16. Age was inversely related to bowel function score in patients but not in controls, suggesting outcomes may worsen with aging 10:08.

Long-Segment Disease

A separate cohort of 25 patients with total colonic aganglionosis ± small-bowel extension, born 1984–2013, revealed stark differences based on extent 11:31. Five patients with aganglionosis extending nearly to the duodenojejunal flexure: four alive, only one weaned from parenteral nutrition, one underwent successful small-bowel transplantation 13:10. Four patients with mid-small-bowel involvement: two died from severe dysmotility and sepsis, none weaned from parenteral nutrition, only one retained bowel continuity 14:08.

Patients with less than 50% small-bowel involvement or purely colonic disease fared better: all alive, all weaned from parenteral nutrition, all with bowel continuity 16:41 17:07. Aganglionosis beyond 50 cm of terminal ileum makes functional bowel continuity or autonomy exceptional and long-term survival poor without intestinal transplantation 19:28.

For purely colonic disease, pouch anal anastomosis yielded reassuring function: median 4 stools per 24 hours (range 1–10), the majority toilet-trained, minimal soiling, no constipation 18:10 18:25 18:36. Enterocolitis and obstructive episodes were frequent but manageable with metronidazole and Botox injections 18:45 19:04 19:45.

Takeaways from this story

Age inversely correlates with bowel function in Hirschsprung patients but not controls—expect deterioration over time.
Aganglionosis beyond 50 cm of terminal ileum: functional autonomy exceptional, survival poor without transplant.
Mid-small-bowel involvement: none weaned from parenteral nutrition, 50% mortality from dysmotility and sepsis.
Purely colonic disease with pouch: median 4 stools/day, most toilet-trained, enterocolitis manageable with metronidazole.

Hirschsprung Disease: Functional Outcomes Persist Into Adulthood

The essential version of this episode — what it covers, the points that matter most, and what it changes for you. Written by Kai from the episode transcript and reviewed before publishing.

For the care team · Core brief · AI-written, human-reviewed

Standard-Length Disease

Two Finnish registry studies tracked adults who underwent pull-through procedures for Hirschsprung disease in childhood, comparing 143 patients (operated 1960–1986) against 86 matched controls 1:23. Most underwent Duhamel procedures, with Soave and Swenson operations comprising roughly 10% and 25% respectively 1:55.

Mean bowel function scores were significantly lower in patients (17.1) than controls (19.1), though 25% of patients achieved perfect scores compared to 50% of controls 5:53 6:10. Constipation, soiling, and social limitations related to bowel function occurred far more frequently in the Hirschsprung cohort 5:16. Thirteen percent reported frequent soiling, 2% had accidents, and 10% required treatment for complications 6:24. Overall gastrointestinal quality-of-life scores showed no significant difference between groups, but 22% of patients scored below 110, indicating poor GI-specific quality of life 8:11 8:24.

The most concerning finding: increasing age was the only predictor of poor bowel function in patients but not controls, suggesting outcomes may deteriorate over time 9:16 10:08. Low bowel function scores directly predicted poor quality of life 10:32.

Long-Segment Disease

A separate cohort of 25 patients with total colonic aganglionosis ± small-bowel extension (born 1984–2013) revealed stark differences by disease extent 11:31. Five patients with aganglionosis extending nearly to the duodenojejunal flexure: four alive, only one weaned from parenteral nutrition, one underwent successful small-bowel transplant 13:10. Four with mid-small-bowel involvement: none weaned from parenteral nutrition, two died from dysmotility and sepsis 14:08. Seven with <50% small-bowel involvement: all alive, none on parenteral nutrition 16:41. Five with purely colonic disease: all alive and off parenteral nutrition 17:07.

Patients with pouch anal anastomosis achieved voluntary bowel movements (median 4 stools per 24 hours), though four of ten had nighttime stools and two had some degree of soiling 18:10 18:25 18:32 18:36. Enterocolitis episodes were common but manageable with metronidazole or ciprofloxacin; obstructive symptoms often required Botox injections, with some patients needing up to six 18:45 19:04.

Aganglionosis beyond 50 cm of terminal ileum makes functional bowel continuity or autonomy exceptional without intestinal transplantation 19:28.

Takeaways from this story

One-quarter of adults with standard Hirschsprung achieve normal bowel function; outcomes may worsen with age.
Aganglionosis beyond 50 cm of terminal ileum rarely permits weaning from parenteral nutrition without transplant.
Pouch procedures for total colonic aganglionosis yield voluntary bowel movements but frequent enterocolitis and obstruction.
22% of adult Hirschsprung patients report poor GI-specific quality of life despite comparable overall QOL scores.

Keywords

Hirschsprung disease total colonic aganglionosis long-segment aganglionosis constipation fecal soiling enterocolitis Down syndrome cartilage-hair hypoplasia congenital central hypoventilation syndrome neuroblastoma Shah-Waardenburg syndrome bowel dysmotility sepsis Duhamel operation Soave operation

Hashtags

#Gastroenterology #BowelHealth #QualityOfLife #Surgery #PatientCare #Research #Healthcare #FinlandStudies #LongTermOutcomes #PediatricSurgery #ClinicalResearch #HealthData #PatientFollowUp #GastrointestinalDisorders #SurgicalOutcomes #HealthTracking #PublicHealth #ChronicConditions #PatientSafety #EvidenceBasedMedicine #HirschsprungDisease #BowelFunction

Transcript

OK, good. Because there's something wrong with the other phone. This is the 3rd phone out. I, I've, I've, I've tried but hope, hopefully this works. So I was telling you why these studies can be made in Finland studies, and the main reason is that we have a social security number. For all Finns. And we can track all our patients back. We know their whereabouts and we also know their sort of medical history from the national records. Let's go on with the talk. We did a study on adults. Uh, with this disease, um, we've studied the bowel function and we assessed also the gastrointestinal quality of life. Um, uh. The basis for this study was that actually there are no or very few controlled studies in adults who have been operated on for her for her in their childhood. And There are some follow up studies. There are many follow up studies, but no studies that have that have used control populations of healthy healthy individuals. This was a population-based cross-section study and operate. In our institution in 1960 and 1986 of the patients have died and had migrated left us 143 patients who were eligible for the study. Uh, most of the patients had clinical interview and we used 86 match controls without any previous surgery for our patients. The operations that were performed. We mainly Duhamel operations. about the life that the figures have changed the other operation used with the founder and also the the European operation the operation in about 10% of our patients and 25% of patients had. OK. But what happened to the flight? Sorry about that. Hold on one second. OK, Um We compared the patients also the patients with the responders who took part in the study with the non the nonresponders, and in this study we didn't find any, any difference between the between the responders and on the nonsense in terms of surgery, the type of surgery. And the and the and the and the and the. We used the function of validate score for the function in in health the average score is 1.1. The low score means that that function and the high score means a very good bow function. We also a score score that is 36 valid score for health related quality of life. This it records the physical and social function and also most states of all the patients. The average score in healthy adults is 125. 8. The maximum score is 144. Uh, you, you can see from this slides, uh, slide that, that, uh, that, uh. There were problems in the, in the, in the bowel function when compared with the with the with the controls. Uh, the The Uh, constipation was much more frequent, with the, uh, uh, patients, uh, sodium was much more frequent. The certificate was, was much more frequent and also the problems were much more frequent in the patients than in the controls. The mean bowel function score of the adult patients with 17.1. And it was 19.1, and this this difference was statistically significant. Uh, however, uh, uh. 25% of all the patients scored the full 20 points uh as opposed to 50% of the con con con to controls. 13% of the patients report frequent soiling. 2 had accidents and 10% had complication that requires treatment. And this figure shows how the Uh It's disappeared again, but you control it? Can we just control it and advance the size. So Doctor, I'll be moving your slides. You just tell me when you, when you want the next slide. I'll be moving them, OK, OK. Oh sorry, thanks. So I don't touch that. OK, that's OK. So uh from this uh figure you can see that um. That definitely the bowel functions of the patients with with the with the bowel function is not at the same level than in health in individuals. The the gas of life on the other hand is mostly at the same level than in the in the health population. There are some, some individuals that had the worst outcome in terms of life, but the people who did worse also in the healthy population. Um In the patients, the GI scores, the scores were marginally lower in the patients, but this difference was not statistically significant. However, 22% of the patients had a score lower than 110 that indicates poor get different quality of life. The patients scored worse in 11 on the on the 36 items and and these items are on the on the on the on the on the table. Um Interestingly, the patients scored better. In the ability to participate in this activities and and and also in the quality of sleep and control. This is very difficult to explain but this kind of questionnaire type studies can give results like this. Uh By doing a sort of multivariate logistic regression analysis, we assess the predictors of poor outcomes, which means the score lower than 17. And the poor quality of life, which means score lower than 110. And this where age surgery after surgery. and primary diversion and in the in the in the quality of life issues that predicts what age, gender, the body mass index and bowel function score and what we found out that the increasing age was the only significant predictor of poor functional outcome. And we also found that age was inversely related to the score in the patients but not in controls, and this is sort of a worrying finding, which means that we may expect that our patients that might do quite well when they're young adults might not do so well when they age. And in terms of quality of life, the low bowel function score was the only predictor of poor quality of life. Next slide please. We conclude that the bowel functions continues to be impaired in a significant proportion of adults with er. Abnormalities in the urge to defecate, fecal soiling, constipation, and social problems in relation to bowel function affect about 1/3 to 15% of the patients. Increasing age increases the risk of poor functional outcome. However, the overall gastrointestinal quality of life appears to be comparable its controls, uh, but decreased, um, uh, Gaston's quality of life was found in 1/5 of the patients. And then I continue with the with the next slide. But some of our more recent studies concerning the outcomes of long segment gangliosisgnosis affecting the entire colon with or without small. in this next slide please. We looked at all the concept of patients who were born between 1984 and 2013 and treated. In our institution and these patients suffered from total colonic anosis with a very extension to the small bowel. This is sort of retrospective case note review. And the patient characteristics bowel function dependence on nutrition growth and survival. Next slide please. In total we identified 25 patients, 21 patients, 15 of these patients were male and 6 female. of the patient for me. And syndrome were found in 4 patients. 2 had causes hair ablation, typical Finnish syndrome which occurs also in the Amish population in the US. 21 patient had a, had a central uh hyperventilation syndrome, had that syndrome with a neuroblastoma, and one patient had a Sch-Wardenberg syndrome. The median follow-up of these patients were 6.5 years. This gives you the next like gives you idea what so how how the patient were doing by the level of 5 patients had the anosis extending very near. To do it, do it in general uh uh uh uh lecture. So they, they have basically sort of total bow egg in their nose. all the patients and for us to alive. Only one has been weaned from parental. The and and all of these patients have a bowel continue. One of the patients and underwent successful small bowel transportation you know and the other patient is waiting for for small about transportation. There were 4 patients who were where the agnosis extended to the mid small bowel level. Uh, I think the phone, his phone went out again. Um Mark, we bring the microphone back up. I, why don't you try to call back in. I think the phone looks like the phone crashed again. So while we are getting Doctor Rintala back, I just, I don't know how many people were able to hear why Finland has such good long-term outcomes, but they have a social security number that is national and they can track medical history, so even if your patient have an operation in another hospital, you can still look at that because when we try to do our long-term follow-up, we always. Have the risk of the ones responding. Many people would say are the ones doing well or are the ones not doing well, so we have a kind of a, a biased view. And in this case they can track all patients from life until death. They can have access to the entire medical history, and I think this is fantastic for learning and to learn what truly happens with a disease as it progresses over years. So hello, hello, yeah. We can hear you. Do you hear me? Yes, perfect. You can go ahead, Dr. Intala. OK, yeah, yeah. Andrea, can you, can you check the previous slide, OK. Yeah, OK, And so I was left, I was left to the in patients who had a gangnosis extending to the mid small bowel. And these patients have a genuine you know pull through um uh uh. But Two of these patients had had a syndromatic disease that makes small transplantation contraindicated two of these patients died because of of of severe bowel motility and the recurrent septic septic infections. None of these patients have been weaned on on nutrition and only one of the patients have about continue continue. There were several patients with the was was less than 50% of. of the patients had pouch and one had a left mark type operation. All the patients patients are alive. None of these patients are on the end and all and and all patients have continuity. They were. 5 patients with the purely colony to the and the notices this patient and they all alive that we can be and and and they all still have about continue nextli please. Um This shows the the the the the duration of of the uh of the uh you can see from this slide that it extends to the mid uh meet the small bowel or more approximately very few of the patients can uh can uh can be weaned from uh from. and also this affects the survival the longer the the the second the survival next like this. And these are the functional outcomes. In patients who had a pouch anal anastomosis, all of the patients have voluntary bowel movement. Motions of the 9 which have been toilet trained. The stooling frequency for 24 hours is 4, ranging between 1 and 210. 4 of the 10 patients have bowel motions at nighttime. 2 of them have uh have have have fe something uh to some degree. None of the patients is suffering from constipation. Um, most of the patients had, had at least one episode of enteritis, um, in these patients, um, uh, the episodes were treated by oral, uh, and, and antibiotics, mainly, uh, uh, metronidazole or cifloxacillin. Obstru episodes were common also and these were mainly treated by by by the injections some of the patients require more than 2 injection and maximum 6 next. To conclude, Intended their noses beyond 50 centimeters of of this island, the achievement of functional continuity or autonomy is exceptional and long-term survival poor without interstellar transportation. If the egg gangnosis is, this is outlook bowel function is reassuring after uh uh a pouch anal anastomosis, although obstructive symptoms and enter colitis are frequent but manageable with Botox and metronidazole. We need to continue, uh, to follow up these patients to assess the long-term outcomes, outcomes, um, after, after, uh, other pouch, anastomosis. Thank you very much. Thank you so much Doctor Rintala. If anybody has any questions.

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