Abstract
Purpose
Growth and diet are major concerns for patients with Hirschsprung’s disease yet are rarely described. This study aimed to investigate self-reported growth and the dietary impact on gastrointestinal symptoms in children with Hirschsprung’s disease.
Methods
This observational cross-sectional study used a validated self-report questionnaire. All children aged 1–18 years old (n = 85) with Hirschsprung’s disease treated at a national center were invited to participate.
Results
In total, 71 children (84%) participated (median age 6 years [range 1–17]). Stunting was reported in 6 of 71 children (9%), with no significant difference between those with rectosigmoid and long-segment aganglionosis (4/58 [7%] vs. 2/13 [15%], p = 0.328). The majority (55/71; 77%) reported diet-induced gastrointestinal symptoms, again with no significant difference between the groups (43/58 [74%] vs. 12/13 [92%] p = 0.227). Of the 90 food items assessed, the most frequently reported to trigger symptoms were high in fermentable oligosaccharides, disaccharides, monosaccharides, and polyols (FODMAPs), free sugar and fat.
Conclusion
Diet-related gastrointestinal complaints are reported frequently in children with Hirschsprung’s disease, regardless of aganglionic extent. The findings highlight the need to assess growth and dietary factors in follow-up care and support further interventional studies on dietary strategies.
Supplementary Information
The online version contains supplementary material available at https://doi.org/10.1007/s00383-026-06657-3.
Keywords: Hirschsprung’s disease, Diet, Gastrointestinal symptoms, Growth
Introduction
Hirschsprung’s disease is a congenital intestinal disorder characterized by aganglionosis of the intestinal wall, extending proximally from the anus to varying lengths of bowel [1–5]. Rectosigmoid aganglionosis accounts for 80–85% of cases, long-segment aganglionosis for approximately 10%, and total colonic aganglionosis for fewer than 10% of cases [1, 4, 6–8]. Hirschsprung’s disease requires surgical management, with removal of the affected part of the bowel [1, 5]. However, despite surgery, residual gastrointestinal symptoms, such as constipation, outlet obstruction, fecal incontinence, abdominal pain and flatulence are reported frequently [1–8]. To improve bowel function, the European consensus guidelines for the management of rectosigmoid Hirschsprung’s disease recommend dietary modifications as part of the bowel management program [1]. While short-term growth at 1 year of age is generally within normal limits regardless of aganglionic segment length [9], children with long-segment aganglionosis are at increased risk of long-term faltering growth and nutritional deficiencies [6–7, 10–12]. Accordingly, both the European guidelines for rectosigmoid Hirschsprung’s disease and the clinical consensus statements on total colonic and intestinal aganglionosis recommend regular growth monitoring during follow-up [1, 8]. Although dietary concerns are highly relevant to patients and families [13], few studies have examined the impact of diet on gastrointestinal symptoms or the nutritional needs of children with Hirschsprung’s disease [6–12, 14–15]. Evidence-based dietary management strategies remain lacking. Previous research has shown that a substantial proportion (77%) of children with Hirschsprung’s disease report diet-induced gastrointestinal symptoms and often engage in dietary self-management [14–16]. Families have also expressed a strong need for dietary guidance to manage these symptoms [13–15, 17]. Importantly, the dietary impact on gastrointestinal symptoms in children with Hirschsprung’s disease appears to differ significantly from that in healthy peers. These children with Hirschsprung’s disease not only experience symptoms more frequently but also report different types of diet-related effects [15]. This distinction highlights the need for disease-specific dietary guidelines. To improve clinical counseling and inform future interventional studies on growth and dietary management in Hirschsprung’s disease, it is essential to expand our understanding of growth patterns and the role of specific dietary components on gastrointestinal symptomatology.
Aim
This study aimed to investigate self-reported growth status and perceptions of whether diet and specific food items trigger gastrointestinal symptoms in children with Hirschsprung’s disease.
Materials and methods
Study setting
This was an observational, cross-sectional, patient-reported questionnaire study conducted at a pediatric surgery department serving as a national referral center for Hirschsprung’s disease since 2018, covering a catchment area of approximately 5 million residents. Data collection took place from March 2020 to March 2021.
Study questionnaire
The validated questionnaire included 32 questions in total (as shown in Online Resource 1) [18]. Nine questions addressed growth, comorbidities, medications, and allergies. Growth parameters (current length/height and weight) were self-reported by patients or their parents and evaluated according to World Health Organization (WHO) growth classification standards [19–23]. Gastrointestinal symptoms were assessed using the Rintala Bowel Function Score (BFS) [24–25] with additional questions on constipation, abdominal pain, and bothersome gases [7]. The BFS, commonly used in children with Hirschsprung’s disease, consists of seven questions with a total score ranging from 1 to 20 (20 = best function). A score of 18–20 indicates normal bowel function, while 1–17 reflects impaired or moderately impaired bowel function [24–25]. Dietary effects on gastrointestinal symptoms were assessed using the Diet and Bowel Function questionnaire [18], which includes 13 questions and 90 specified food items. Respondents indicated whether each food item caused any gastrointestinal symptoms and, if so, the type (i.e. any, laxative, constipating, abdominal pain, gas, or other). To explore potential mechanisms, food items were categorized based on their content into six groups: Fermentable Oligosaccharides, Disaccharides, Monosaccharides, And Polyols (FODMAPs); fat; free sugar; stringy or hard to digest textures; histamine-releasing or biogenic amine-containing foods. Response frequencies for each category in the questionnaire are shown in Fig. 1. Parents were instructed to complete the questionnaire by proxy for young children and to complete the questionnaire together with their child for children younger than 15 years. Adolescents aged 15–18 years were encouraged to complete the questionnaire independently.
Fig. 1.
Flowchart of participants: children with Hirschsprung’s disease 1–18 years old included and excluded in the study and answer frequency for each category in the questionnaire. Values are n (%) of children
Study population
All children aged 1–18 years treated for Hirschsprung’s disease at the department were invited to participate, along with their parents. Study invitations and questionnaires were distributed by postal mail. Children receiving enteral tube feeding or parenteral nutrition were excluded.
Statistical analysis
A statistician designed the statistical analysis plan. Only questionnaires with at least 90% of questions completed (≥ 29/32) were included. For BFS analysis, only participants aged 4–18 years who had completed all BFS questions were included. Descriptive data are presented as n (%). Data on a continuous variable (i.e. age), is reported as median (range) due to non-normal distribution. The nonparametric Mann-Whitney U test was used for continuous and discrete (number of food items reported to induce gastrointestinal symptoms) variables and for graded data on an ordinal scale. Binary categorical variables were analyzed using the Chi-square test. A p-value < 0.05 was considered statistically significant. Analyses were performed using IBM SPSS Statistics version 29 for MacOS.
Hirschsprung’s disease was classified as rectosigmoid aganglionosis (n = 58), long-segment aganglionosis extending beyond the rectosigmoid colon to the descending or transverse colon (n = 8), or total colonic aganglionosis, including extension of aganglionosis up to 20 cm into the ileum (n = 5). Rectosigmoid aganglionosis served as the reference group, as this is the most common subtype of Hirschsprung’s disease. Long-segment and total colonic aganglionosis were analyzed together, as the phenotypes were few and analyses of the distribution of bowel function outcomes showed no differences between the groups. Univariate subgroup analyses (including BFS, age and sex) were conducted for dietary effects on gastrointestinal symptoms and dietary adjustments. No significant differences were found within subgroups (Online Resource 2). Therefore, subsequent analyses of self-reported growth and dietary effects on gastrointestinal symptoms were stratified only by aganglionic extent (rectosigmoid vs. long-segment aganglionosis). Responses to questions on constipation and outlet obstruction were dichotomized as “No” versus “Yes” (including symptoms without treatment, or treated with diet, medication, enemas, or other methods), soiling (Often/Always), abdominal pain (Often/Always) and bothersome gases (Often/Always).
Ethical considerations
The study was conducted in accordance with the Declaration of Helsinki and was approved by the Swedish Ethical Review Authority (registration number 2018/720). Participants received age-appropriate oral and written information. Informed consent was obtained from both legal guardians for children under 15 years of age, while adolescents aged 15 years or older provided their own consent.
Results
Background information and gastrointestinal symptoms
A total of 71 out of 85 children (84%) met the inclusion criteria (i.e. ≥90% of questions answered and no enteral or parenteral nutrition). A flowchart of participant inclusion and exclusion is shown in Fig. 1. The median age of respondents was 6 years (range 1–17), and 56 of 71 (79%) were boys. Among participants, 58 (82%) had rectosigmoid aganglionosis and 13 (18%) had long-segment aganglionosis, including five (7%) with total colonic aganglionosis. Three participants (4%) had an ostomy. The BFS was completed by 50 children aged 4–18 years. Normal bowel function (BFS 18–20) was reported by 15 of 50 (30%) participants, with no significant difference between children with rectosigmoid and long-segment aganglionosis (14/42 [33%] vs. 1/8 [13%], (p = 0.407). A full overview of BFS outcomes is provided in Online Resource 3. The self-reported gastrointestinal symptoms obstruction, abdominal pain and bothersome gases were analyzed for all 71 participants aged 1–18 years. Obstructive symptoms were reported by 26 (37%), abdominal pain by 14 (20%) and bothersome gases by 37 (52%), with no significant differences between aganglionosis subtypes (Online Resource 4).
Growth and nutritional supplements
Stunting was reported by 6 of 71 children (9%) children, with no significant difference between those with rectosigmoid and long-segment aganglionosis (4/58 [7%] vs. 2/13 [15%], p = 0.328). Growth status based on weight and self-reported history of faltering growth also did not differ significantly between groups (Table 1). Children with long-segment aganglionosis more frequently reported current or previous intake of nutritional supplements compared to those with rectosigmoid aganglionosis (8/13 [62%] vs. 12/58 [21%], (p = 0.007).
Table 1.
Growth status according to WHO classification, self-reported growth impairment and need of nutritional supplements reported by children with Hirschsprung’s disease 1–18 years old, using the Diet and Bowel function questionnaire
| Growth status and need of nutritional supplements | All children with HSCR n = 71 | Rectosigmoid aganglionosis n = 58 | Long-segment aganglionosis n = 13 | p-value |
|---|---|---|---|---|
| Growth status according to WHO classifications | ||||
| Stunted1 | 6 (9) | 4 (7) | 2 (15) | 0.32813 |
| Missing | 9 (13) | 8 (14) | 1 (8) | |
| Underweight/Wasted2,3 | 2 (3) | 2 (3) | 0 (0) | 0.56714 |
| Healthy weight4,5,6 | 47 (66) | 37 (64) | 10 (77) | |
| Overweight7,8,9 | 9 (13) | 8 (14) | 1 (8) | |
| Obesity10,11,12 | 2 (3) | 2 (3) | 0 (0) | |
| Missing | 11 (15) | 9 (16) | 2 (15) | |
| Self-reported growth impairment | ||||
| Impaired weight gain | 17 (24) | 13 (22) | 4 (31) | 0.49613 |
| Missing | 0 (0) | 0 (0) | 0 (0) | |
| Impaired growth (length/height) | 14 (20) | 11 (19) | 3 (23) | 0.69815 |
| Missing | 2 (3) | 1 (2) | 1 (8) | |
| Need of nutritional supplements | ||||
| Never | 51 (72) | 46 (79) | 5 (39) | 0.00714 |
| Not currently, but have in the past | 13 (18) | 7 (12) | 6 (46) | |
| Occasionally need | 5 (7) | 3 (5) | 2 (15) | |
| Regularly need | 2 (3) | 2 (3) | 0 (0) | |
| Missing | 0 (0) | 0 (0) | 0 (0) | |
WHO, World Health Organization, HSCR Hirschsprung’s disease
1Length/Height for age − 2SD or below
2Children 1 year old: Weight for length − 2SD or below
3Children 2–18 years old: BMI for age − 2SD or below
4Children 1 year old: Weight for length − 2SD – <2 + SD
5Children 2–5 years old: BMI for age − 2SD – <+2SD
6Children 5–18 years old: BMI for age − 2SD – <+1SD
7Children 1 year old: Weight for length +2SD – <+3SD
8Children 2–5 years old: BMI for age +2SD – <+3SD
9Children 5–18 years old: BMI for age +1SD – <+2SD
10Children 1 year old: Weight for length +3SD or above
11Children 2–5 years old: BMI for age +3SD or above
12Children 5–18 years old: BMI for age +2SD or above
13Fischer’s exact test
14Mann-Whitney U Tests, two-tailed
15Chi-square test
Food items inducing gastrointestinal symptoms
The majority of children (55/71; 77%) reported that diet induced gastrointestinal symptoms, with no significant difference between aganglionosis subtypes (43/58 [74%] vs. 12/13 [92%] p = 0.227). In total, 58 of 71 children (82%) identified at least one food item that triggered symptoms. The median number of symptom-inducing food items reported was seven (range 0–66). Children with long-segment aganglionosis reported a significantly higher number of symptom-inducing foods compared to children with rectosigmoid aganglionosis (median 18 [0–66] vs. 6 [0–36] (p = 0.038). Specific food items reported to induce gastrointestinal symptoms varied between children reporting symptoms of soiling, obstruction, gases, constipation, respectively, abdominal pain (Table 2). Banana, corn and beans were reported frequently as inducing gastrointestinal symptoms across all symptom types. Unique triggers for soiling included pear, apple, cauliflower and popcorn; for gases strawberry and orange; and for abdominal pain, lactose-free milk, carrots, peppers, butter, and Swedish crispbread. The three most frequently reported symptom-inducing foods overall were milk, banana and corn (Table 3). The top 10 food items associated with each gastrointestinal symptom type are listed in Table 3.
Table 2.
Food items reported to induce any (unspecified) gastrointestinal effect, reported by more than 20% children with Hirschsprung’s disease (n = 71) 1–18 years old with gastrointestinal symptoms (soiling, obstruction, gases, constipation, abdominal pain) using the Diet and Bowel function questionnaire
| Children with soiling n = 25 | Children with obstruction n = 24 | Children with gases n = 37 | Children with constipation n = 23 | Children with abdominal pain n = 14 | |||||
|---|---|---|---|---|---|---|---|---|---|
| Banana | 10 (40) | Beans | 11 (46) | Milk | 19 (51) | Beans | 11 (48) | Milk | 7 (50) |
| Corn | 10 (40) | Banana | 10 (42) | Banana | 15 (41) | Banana | 9 (39) | Beans | 5 (36) |
| Beans | 9 (36) | Corn | 10 (42) | Corn | 14 (38) | Corn | 9 (39) | Cream | 5 (36) |
| Cream | 9 (36) | Fruit peel | 6 (25) | Cream | 14 (38) | White bread | 7 (30) | Yoghurt | 5 (36) |
| Milk | 8 (32) | Milk | 6 (25) | Yoghurt | 14 (38) | Cabbage | 6 (26) | Bread: grains/seeds | 5 (36) |
| Onion | 8 (32) | Cabbage | 5 (21) | Ice cream | 13 (35) | Onion | 5 (22) | Pastry | 5 (36) |
| Ice cream | 8 (32) | Onion | 5 (21) | White bread | 12 (32) | Chocolate | 5 (36) | ||
| Yoghurt | 8 (32) | Potato | 5 (21) | Candy | 12 (32) | Candy | 5 (36) | ||
| White bread | 8 (32) | Yoghurt | 5 (21) | Clementine | 11 (30) | Corn | 4 (29) | ||
| Pastry | 7 (28) | Beans | 10 (27) | Ice cream | 4 (29) | ||||
| Chips | 7 (28) | Onion | 10 (27) | Lactose-free milk | 4 (29) | ||||
| Bread: grains/seeds | 6 (24) | Fruit peel | 10 (27) | White bread | 4 (29) | ||||
| Candy | 6 (24) | Bread: grains/seeds | 10 (27) | Banana | 3 (21) | ||||
| Deep fried food | 6 (24) | Strawberry | 9 (24) | Clementine | 3 (21) | ||||
| Pear | 5 (20) | Pastry | 9 (24) | Carrot | 3 (21) | ||||
| Apple | 5 (20) | Chips | 9 (24) | Pepper | 3 (21) | ||||
| Cauliflower | 5 (20) | Chocolate | 9 (24) | Potato | 3 (21) | ||||
| Fruit peel | 5 (20) | Deep fried food | 9 (24) | Fruit peel | 3 (21) | ||||
| Chocolate | 5 (20) | Orange | 8 (22) | Butter | 3 (21) | ||||
| Popcorn | 5 (20) | Swedish cracker | 3 (21) | ||||||
| Chips | 3 (21) | ||||||||
| Median (range) number of food items reported per child | |||||||||
| 7 (0–36) | 6 (0–24) | 10 (0–60) | 7 (0–36) | 8 (0–22) | |||||
Values are n (%) of children
Table 3.
Ranking of the 10 most frequently reported food items to induce any (unspecified) gastrointestinal effect, laxative effect, constipating effect, rising pain and/or rising gases respectively, reported by children with Hirschsprung’s disease (n = 71) 1–18 years old using the Diet and Bowel function questionnaire
| Ranking | Any effect | Laxative effect | Constipating effect | Rising pain | Rising gases | |||||
|---|---|---|---|---|---|---|---|---|---|---|
| 1 | Milk | 26 (37) | Cream | 15 (21) | Banana | 20 (28) | Milk | 9 (13) | Beans | 18 (25) |
| 2 | Banana | 24 (34) | Ice cream | 13 (18) | White bread | 13 (18) | Corn | 8 (11) | Milk | 14 (20) |
| 3 | Corn | 24 (34) | Milk | 12 (17) | Rice | 8 (11) | Cream | 6 (9) | Onion | 11 (16) |
| 4 | Cream | 23 (32) | Candy | 12 (17) | Corn | 7 (10) | Beans | 5 (7) | Cabbage | 11 (16) |
| 5 | Beans | 21 (30) | Yoghurt | 11 (16) | Potato | 7 (10) | Yoghurt | 5 (7) | Cream | 9 (13) |
| 6 | Yoghurt | 21 (30) | Kiwi | 10 (14) | Pasta | 7 (10) | Ice cream | 5 (7) | Yoghurt | 8 (11) |
| 7 | Ice cream | 19 (27) | Pastry | 9 (13) | Flour | 6 (9) | Candy | 5 (7) | Ice cream | 8 (11) |
| 8 | White bread | 19 (27) | Plum | 8 (11) | Apple | 5 (7) | Pastry | 5 (7) | Cauliflower | 8 (11) |
| 9 | Candy | 18 (25) | Chocolate | 7 (10) | Chips | 4 (6) | Chips | 4 (6) | Chips | 6 (9) |
| 10 | Pastry | 17 (24) | Clementine | 7 (10) | Fruit peel | 4 (6) | Chocolate | 4 (6) | Deep fried food | 6 (9) |
Values are n (%) of children
Potential underlying mechanisms of symptom-inducing foods
The most frequently reported food category to induce gastrointestinal symptoms was FODMAPs. Among the 10 most frequently reported symptom-inducing foods, nine were high in FODMAPs. The second and third most reported categories were foods high in free sugar and high in fat, respectively (Table 4). Across all 90 food items assessed, FODMAPs remained the most frequently reported category reported to induce gastrointestinal symptoms (Online Resource 5).
Table 4.
Potential underlying mechanism of inducing gastrointestinal symptoms as reported by children with Hirschsprung’s disease 1–18 years old using the Diet and Bowel function questionnaire
| Potential underlying mechanism | Number of food items in the questionnaire (In total n = 90) | Frequency of children (n = 71) reporting at least one food item | Median number of food items reported per child | Food items included in the 10 most frequently reported food items to induce gastrointestinal symptoms (number and specific food item) | |
|---|---|---|---|---|---|
| FODMAPs | 40 | 46 (65%) | 4 (range 0–32) | 9 | Milk, banana, corn, cream, beans, yoghurt, ice cream, white bread, pastry |
| High content of fat | 6 | 33 (46%) | 1 (range 0–6) | 3 | Cream, ice cream and pastry |
| High content of free sugar | 5 | 30 (42%) | 0 (range 0–5) | 3 | Ice cream, candy and pastry |
| Stringy foods or foods hard to digest | 33 | 37 (52%) | 2 (range 0–24) | 2 | Corn and beans |
| Histamine releasing | 10 | 32 (45%) | 1 (range 0–9) | 1 | Milk |
| Biogenic amines | 8 | 37 (52%) | 1 (range 0–7) | 1 | Banana |
FODMAPs Fermentable Oligosaccharides, Disaccharides, Monosaccharides, and Polyols
Dietary adjustments to improve gastrointestinal complaints
Overall, 49 of 71 children (69%) reported making dietary adjustments to manage gastrointestinal symptoms. The most common reasons were to obtain looser stools (23/49; 47%), reduce flatulence (20/49; 41%) and to obtain a constipating effect (13/49; 27%). A total of 27 children (38%) reported actively choosing specific foods or dietary habits to improve gastrointestinal symptoms. The most frequently chosen foods were fruits (i.e. kiwi, pear, banana, prune, grapes, blueberries, apple, plum, and apricot) reported by 9 of 27 children (33%). Probiotics were used by six (22%) of the children, and a lactose-free diet was followed by eight (30%). Avoidance of specific foods or dietary habits was reported by 44 of 71 children (62%). Of these, 42 children specified the foods avoided. The most avoided foods were lactose-containing foods (16/42; 38%), fruit peel, stringy or hard-to-digest foods (10/42; 24%), and free sugar- or fiber-rich foods (8/42; 19%).
Free-text comments on the influence of diet on gastrointestinal symptoms
Free-text comments were provided by 46/71 (65%) respondents and revealed three main themes. Portion size was most frequently reported (24/46, 52%), with respondents describing limiting rather than excluding foods associated with symptoms. Selective eating (11/46, 24%) made it difficult to evaluate foods that were rarely consumed, or not part of the child’s diet, particularly fruits and vegetables. In addition, 9/46 (20%) respondents reported difficulty in identifying consistent associations between specific foods and gastrointestinal symptoms.
Discussion
This self-reported study found that 9% of children with Hirschsprung’s disease reported growth status classified as stunted growth and 28% required nutritional supplementation. Supplement use was significantly more common among children with long-segment aganglionosis compared to children with rectosigmoid extension. Overall, 77% of participants reported that diet induced gastrointestinal symptoms, with no differences based on aganglionic length. Foods high in FODMAPs, free sugar and fat were most frequently reported as gastrointestinal symptom triggers.
The prevalence of stunting was higher in children with long-segment aganglionosis (15%) than in those with rectosigmoid extension (7%), and both rates exceeded the 2.2% prevalence reported in the general pediatric population, based on the WHO definition (≥ 2 standard deviations below the mean) [26]. Previous studies have reported faltering growth in 23–55% of children with total colonic aganglionosis [6–7, 10–12], and an association between faltering growth and aganglionic extent has been suggested [10]. The lower prevalence observed in our study may be due to the inclusion of only children with total colonic aganglionosis extending no more than 20 cm into the small bowel, who were grouped with those with milder long-segment aganglionosis. Additionally, 61% of children with long-segment aganglionosis in our study reported using nutritional supplements, compared to 47% in a previous Nordic study [7]. At our center, all children receiving supplements are followed regularly by both a pediatric dietitian and a pediatrician, which may have contributed to better growth outcomes. Interpretation of the growth findings is limited by the use of self-reported data. Objective measurements are needed to assess the prevalence of faltering growth accurately. The etiology of faltering growth in Hirschsprung’s disease is likely to be multifactorial, including repeated surgical interventions and admissions for enterocolitis, familial short stature, endocrine or genetic conditions, and dietary restrictions to manage gastrointestinal symptoms may all play a role. Further studies are needed to investigate the relationship between dietary intake, nutritional status, and growth in this population.
A key finding was that 77% of children reported diet-induced gastrointestinal symptoms, consistent with previous studies in children and adults with Hirschsprung’s disease (64–77%) [16, 27–29]. A novel observation was that symptom-triggering foods varied according to the type of gastrointestinal complaint, supporting individualized dietary counseling. Dietary modifications may be particularly relevant in relation to soiling. Families may modify the diet to promote bowel motility and facilitate stool passage; however, the same foods may increase stool frequency or loosen stool consistency and thereby contribute to soiling. Conversely, foods perceived to aggravate soiling may already have been limited or excluded from the child’s diet. This makes the relationship between dietary intake, bowel function, and soiling complex and potentially bidirectional, and should be considered when interpreting the reported associations between specific foods and gastrointestinal symptoms. Dietary modifications in response to gastrointestinal symptoms were reported for 69% of the children, consistent with the previously reported rate of 61–72% [16, 29]. In our cohort, 38% reported selecting specific foods, whereas 62% avoided certain foods. However, whether these dietary modifications were associated with symptom improvement was not assessed. Future studies could explore associations between individual foods, or combination of foods, and gastrointestinal symptoms. The rationale for dietary modifications, including whether they were medically advised or self-initiated, was not assessed. However, European and Latin American studies have suggested that dietary modifications among patients with Hirschsprung’s disease were prominently self-directed [16, 29]. This calls for awareness in clinical counseling. Foods high in FODMAPs, free sugar, and fat were most frequently reported as symptom triggers, although overlap between food categories complicates interpretation. The observed associations are biologically plausible. FODMAPs increase luminal water content and undergo bacterial fermentation, resulting in loose stools, gas production, and abdominal distension [30]. Free sugar and fat may also exacerbate symptoms through stimulation of the gastrocolonic response and postprandial colonic motility [31], which may be particularly problematic in children with persistent bowel dysfunction after Hirschsprung’s disease surgery [1–7]. A small exploratory study reported improved fecal soiling in 9 of 10 children with Hirschsprung’s disease and fecal incontinence following a low-FODMAP diet [32]. Although low-FODMAP diets may benefit children with irritable bowel syndrome, routine use is not currently recommended because of their restrictive nature and limited evidence base [30]. When implemented in children, the diet should follow a structured approach consisting of a 2–6-week FODMAP-restriction phase followed by systematic reintroduction to identify individual trigger foods and achieve the least restrictive long-term diet [30]. Dietary assessment and follow-up by a dietitian are recommended to ensure nutritional adequacy [30]. Whether low-FODMAP or other dietary approaches are beneficial in Hirschsprung’s disease should be evaluated in well-designed interventional studies. The present findings provide a basis for such research.
As this study was designed to evaluate patient-reported experiences regarding diet and gastrointestinal symptoms, the findings should be interpreted in the context of other factors known to influence gastrointestinal symptoms in children with Hirschsprung’s disease. Several disease-specific mechanisms contribute to postoperative gastrointestinal symptoms. Surgical treatment alters anorectal anatomy and physiology, which may impair continence and bowel function [2, 5]. Reduced anal sensation, absence of internal anal sphincter relaxation, and a preserved aganglionic cuff may contribute to fecal incontinence and/or outlet obstruction [2, 5]. In addition, many patients exhibit accelerated colonic transit, lack of the rectal brake, an exaggerated colonic response, and a hyperreactive colon, which may increase postprandial urgency, stool frequency, abdominal distension, and fecal soiling [33–34]. Therefore, the results do not imply that dietary factors are the sole or primary drivers of gastrointestinal symptoms.
The strengths of this study include the use of a validated, disease-specific questionnaire [18], detailed data on food-related gastrointestinal symptoms, and a high response rate from a national referral center. These factors ensured broad representation across age, sex, symptom severity, and aganglionic extent. However, potential response bias cannot be excluded, as families with food-related concerns may have been more likely to participate. Several limitations should be considered. The main limitation was the small sample size in subgroup analyses, which may have limited the ability to detect differences between aganglionosis types. Due to the small sample sizes, patients with long-segment aganglionosis and total colonic aganglionosis were combined into one group, as no functional outcomes were observed between these subgroups. However, we acknowledge that grouping these phenotypes may be questioned, as they may have distinct clinical courses. The findings should therefore be interpreted with caution. Larger, multicenter studies are needed to confirm whether dietary effects on gastrointestinal symptoms vary by disease severity. The information provided with the questionnaire instructed parents and school-aged children up to 15 years of age to complete the questionnaire together. For the youngest children, parents were instructed to respond by proxy, while the oldest adolescents were encouraged to complete the questionnaire independently. As only one questionnaire was distributed per household, parents and children did not provide separate responses, precluding comparisons between parent-proxy and child-reported responses. Furthermore, whether these instructions were followed was not recorded. Consequently, the extent to which the responses reflect the children’s own experiences cannot be determined. These aspects should be considered potential limitations of the study. Furthermore, the cross-sectional design precludes conclusions regarding causality. Notably, some respondents reported that they were unable to evaluate the effects of specific foods because these foods were not consumed by the child. This highlights an inherent challenge in assessing and reporting food-related effects, as dietary habits may already have been modified in response to previous gastrointestinal symptoms, or dietary restrictions might be due to avoidant/restrictive food intake disorder. Consequently, the reported effects of specific foods may not fully reflect their potential impact on symptoms. Postoperative complications, such as bowel obstruction or anastomotic strictures, were not assessed in the present study. Such complications may influence both gastrointestinal symptoms and dietary intake, for example by leading to dietary modifications or avoidance of certain foods. The lack of information on previous postoperative complications, and the frequency of these in the cohort, could therefore be considered a limitation of the study. Gastrointestinal symptoms were assessed prospectively using the Rintala Bowel Function Score, which is used widely in pediatric surgery and has been shown to capture key domains of bowel function in patients with Hirschsprung’s disease. The use of the Rome IV criteria [35] would have facilitated broader interpretation and comparison of our findings, as these criteria are well established and used widely among pediatricians. The lack of assessment according to the Rome IV criteria should therefore be considered a limitation, particularly when comparing our findings with studies from the field of pediatric gastroenterology.
Children with Hirschsprung’s disease present with a wide range of gastrointestinal symptoms. For effective clinical counseling, it is essential to recognize that not all symptoms can be managed through diet alone. For example, pain and gases may result from outlet obstruction requiring surgical intervention, and incontinence may be the result of a low anastomosis, necessitating bowel management strategies beyond dietary changes [2, 5]. A multidisciplinary approach, including individualized dietary counseling by a pediatric dietitian experienced in Hirschsprung’s disease, is essential. To develop evidence-based dietary recommendations, interventional studies are needed. The present findings offer a strong foundation for future clinical trials.
Conclusion
Diet-related gastrointestinal symptoms are reported frequently in children with Hirschsprung’s disease, regardless of aganglionic extent. Foods with a high content of FODMAPs, free sugar and fat appear to be particularly problematic. The findings support the inclusion of multidisciplinary discussions on diet and routine assessment of growth and dietary habits in follow-up care. Interventional studies on dietary treatment strategiesare warranted.
Supplementary Information
Below is the link to the electronic supplementary material.
Acknowledgements
Representatives from the Swedish patient organization for Hirschsprung’s disease. Ros Kenn for language editing.
Author contributions
Dr Lovisa Telborn conceptualized and designed the study, collected data, carried out the analyzes and drafted the initial manuscript. Assoc. Prof. Irene Axelsson conceptualized and designed the study and critically reviewed and revised the manuscript.Dr Christina Granéli designed the study and critically reviewed and revised the manuscript. Prof. Pernilla Stenström conceptualized and designed the study, supervised data collection and analyzes, and critically reviewed and revised the manuscript for important intellectual content.All authors approved the final manuscript as submitted and agree to be accountable for all aspects of the work.
Funding
This research was funded by Skåne County Council’s Research and Development Foundation, funding number 43980 and by Skåne University Hospital Funding, funding number 95908. The funding bodies had no role in the design and conduct of the study.
Data availability
All data supporting the findings of this study are available on request.
Declarations
Conflict of interest
The authors have no competing interests to declare that are relevant to the content of this article.
Ethics approval
The research involved Human Participants. The study was conducted in accordance with the Declaration of Helsinki and was approved by the Swedish Ethical Review Authority (registration number 2018/720).
Consent
Participants received age-appropriate oral and written information. Informed consent was obtained from both legal guardians for children under 15 years of age, while adolescents aged 15 years or older provided their own consent.
Standards of reporting
Reporting guidelines for observational studies (STROBE) were followed.
Footnotes
Publisher’s Note
Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.
References
- 1.Rossi D, Irvine W, Granstrom AL et al (2026) Updated ERNICA guidelines for the management of rectosigmoid Hirschsprung’s disease 2025. J Pediatr Gastroenterol Nutr 83:185–207. 10.1002/jpn3.70442 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 2.Bokova E, Prasade N, Janumpally S et al (2023) State of the art bowel management for pediatric colorectal problems: Hirschsprung’s disease. Children 10:1418. 10.3390/children10081418 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 3.Davidson J, Kyrklund K, Eaton S et al (2021) Long-term surgical and patient-reported outcomes of Hirschsprung’s disease. J Pediatr Surg 56:1502–1511. 10.1016/j.jpedsurg.2021.01.043 [DOI] [PubMed] [Google Scholar]
- 4.Bjornland K, Pakarinen MP, Stenström P et al (2017) A Nordic multicenter survey of long-term bowel function after transanal endorectal pull-through in 200 patients with rectosigmoid Hirschsprung’s’s disease. J Pediatr Surg 52:1458–1464. 10.1016/j.jpedsurg.2017.01.001 [DOI] [PubMed] [Google Scholar]
- 5.Ambartsumyan L, Patel D, Kapavarapu P et al (2023) Evaluation and management of postsurgical patient with Hirschsprung’s disease neurogastroenteology & motility committee: position paper of North American society of pediatric gastroenterology, hepatology and nutrition (NASPGHAN). J Pediatr Gastroenterol Nutr 76:533–546. 10.1097/MPG.0000000000003717 [DOI] [PubMed] [Google Scholar]
- 6.Stenström P, Brautigam M, Borg H, Granéli C, Llja HE, Wester T (2017) Patient-reported Swedish nationwide outcomes for children and adolescents with total colonic aganglionosis. J Pediatr Surg 52:1302–1307. 10.1016/j.jpedsurg.2016.11.033 [DOI] [PubMed] [Google Scholar]
- 7.Stenström P, Kyrklund K, Bräutigam M, Engstrand Lilja H, Juul Stensrud K, Löf Granström A et al (2020) Total colonic aganglionosis: multicentre study of surgical treatment and patient-reported outcomes up to adulthood. Br J Surg Open 4:943–953. 10.1002/bjs5.50317 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 8.Granström AL, Irvine W, Hoel AT et al (2024) ERNICA clinical consensus statements on total colonic and intestinal aganglionosis. J Pediatr Surg 59(10):161565. 10.1016/j.jpedsurg.2024.04.019 [DOI] [PubMed] [Google Scholar]
- 9.More K, Rao S, McMichael J, Minutillo C (2014) Growth and developmental outcomes of infants with Hirschsprung’s’s disease presenting in the neonatal period: a retrospective study. J Pediatr 165:73–77. 10.1016/j.jpeds.2014.02.062 [DOI] [PubMed] [Google Scholar]
- 10.Ikawa H, Masuyma H, Hirabayashi T, Endo M, Yokoyama J (1997) More than 10 years’ follow-up to total colonic aganglionosis – severe iron deficiency anemia and growth retardation. J Pediatr Surg 32:25–27. 10.1016/S0022-3468(97)90085-3 [DOI] [PubMed] [Google Scholar]
- 11.Urla C, Lieber J, Obermayr F et al (2018) Surgical treatment of children with total colonic aganglionosis: functional and metabolic long-term outcome. BMC Surg 18:58. 10.1186/s12893-018-0383-6 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 12.Roorda D, Witvliet MJ, Wellens LM et al (2018) Long-term outcome and quality of life in patients with total colonic aganglionosis in the Netherlands. Colorectal Dis 20:719–726. 10.1111/codi.14095 [DOI] [PubMed] [Google Scholar]
- 13.Wittmeier KD, Hobbs-Muriosn K, Holland C, Crawford E, Loewen H, Morris M et al (2018) Identifying information needs for Hirschsprung’s’s disease through caregiver involvement via social media: a prioritization study and literature review. J Med Internet Res 20:e297. 10.2196/jmir.9701 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 14.Telborn L, Tofft L, Hallström I, Axelsson I, Brandström F, Stenström P (2021) Diet plays a central role in parent’s self-treatment of children with Hirschsprung’s’s disease – a focus group study. Acta Paediatr 110(9):2620–2617. 10.1111/apa.15952 [DOI] [PubMed] [Google Scholar]
- 15.Telborn L, Granéli C, Axelsson I, Stenström P (2023) Children with Hirschsprung’s’s disease report dietary effects on gastrointestinal complaints more frequently than controls. Children 10:1543. 10.3390/children10091543 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 16.Lindert J, Day H, de Andres Crespo M, Amerstorfer E, Alexander S, Backes M, de Filippo C, Golebiewski A, Midrio P, Mohideen M et al (2024) Influence of diet on bowel function and abdominal symptoms in children and adolescents with Hirschsprung’s disease – a multinational patient-reported outcome survey. Children 11:1118. 10.3390/children11091118 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 17.Berrios C, Bollinger J, Yan J, Bisecker B, Cakravarti A (2022) Identifying needs, challenges and benefits among adults and parents of children with Hirschsprung’s disease. J Pediatr Gastroenterol Nutr 74:e103–e108. 10.1097/MPG.0000000000003411 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 18.Telborn L, Kumlien C, Granéli C, Axelsson I, Stenström P (2023) Diet and bowel function in children with Hirschsprung’s’s disease: development and content validation of a patient-reported questionnaire. BMC Nutr 9:78. 10.1186/s40795-023-00737-6 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 19.World Health Organization https://www.who.int/toolkits/child-growth-standards/standards/body-mass-index-for-age-bmi-for-age Accessed 15 September 2026
- 20.World Health Organization https://www.who.int/tools/growth-reference-data-for-5to19-years/indicators/bmi-for-age Accessed 15 September 2026
- 21.World Health Organization https://www.who.int/tools/child-growth-standards/standards/length-height-for-age Accessed 15 September 2026
- 22.World Health Organization https://www.who.int/tools/growth-reference-data-for-5to19-years/indicators/height-for-age Accessed 15 September 2026
- 23.World Health Organization https://www.who.int/tools/child-growth-standards/standards/weight-for-length-height Accessed 15 September 2026
- 24.Rintala RJ, Lindahl H (1995) Is normal bowel function possible after repair of intermediate and high anorectal malformations? J Pediatr Surg 30:491–494. 10.1016/0022-3468(95)90064-0 [DOI] [PubMed] [Google Scholar]
- 25.Rintala RJ, Lindahl HG, Rasanen M (1997) Do children with repaired low anorectal malformations have normal bowel function? J Pediatr Surg 32:823–826. 10.1016/S0022-3468(97)90628-X [DOI] [PubMed] [Google Scholar]
- 26.https://www.rikshandboken-bhv.se/halsobesok/halsoundersokning/tillvaxt/avvikande-langdtillvaxt/#section-31828 Accessed 15 September 2025
- 27.Catto-Smith AG, Trajanovska M, Taylor RG (2007) Long-term continence after surgery for Hirschsprung’s’s disease. J Gastroenterol Hepatol 22:2273–2282. 10.1111/j.1440-1746.2006.04750.x [DOI] [PubMed] [Google Scholar]
- 28.Catto-Smith AG, Coffey CM, Nolan TM et al (1995) Fecal incontinence after the surgical treatment of Hirschsprung’s disease. J Pediatr 127:954–957. 10.1016/s0022-3476(95)70036-6 [DOI] [PubMed] [Google Scholar]
- 29.Scaramella C, Telborn L, Davidson J et al (2026) Gastrointestinal symptoms and association to food items in children with Hirschsrpung disease: a multinational patient-reported outcome study from Latin America and Spain. Pediatr Surg Int 42:380. 10.1007/s00383-026-06605-1 [DOI] [PMC free article] [PubMed] [Google Scholar]
- 30.Thomassen RA, Luque V, Assa A et al (2022) An ESPGHAN position paper on the use of low-FODMAP diet in pediatric gastroenterology. J Pediatr Gastroenterol Nutr 75:356–368. 10.1097/MPG.0000000000003526 [DOI] [PubMed] [Google Scholar]
- 31.Dorfman L, EL-Chammas K, Mansi S et al (2022) Gastrocolonic Response. Curr Gastroenterol Rep 24:137–144. 10.1007/s1189f4-022-00849-2 [DOI] [PubMed] [Google Scholar]
- 32.Stathopoulos L, King Sm Southwell B et al (2016) Nuclear transit study in children with chronic faecal soiling after Hirschsprung’s disease (HSCR) surgery has revealed a group with rapid proximal colonic treatment and possible adverse reactions to food. Pediatr Surg Int 32:773–777. 10.1007/s00383-016-3919-9 [DOI] [PubMed] [Google Scholar]
- 33.Kaul A, Garza JM, Connor FL et al (2011) Colonic hyperactivity results in frequent fecal soiling in a subset of children after surgery for Hirschsprung disease. J Pediatr Gastroenterol Nutr 52:433–436. 10.1097/MPG.0b013e3181efe551 [DOI] [PubMed] [Google Scholar]
- 34.Langer JC, Rollins MD, Levitt M et al (2017) Guidelines for the management of postoperative obstructive symptoms in children with Hirschsprung’s disease. Pediatr Surg Int 33:523–526 [DOI] [PubMed] [Google Scholar]
- 35.Hyams JS, DiLorenzo C, Saps M et al (2016) Childhood functional gastrointestinal disorders: Child/adolescent. Gastroenterol 150(6):1456–1468 [Google Scholar]
Associated Data
This section collects any data citations, data availability statements, or supplementary materials included in this article.
Supplementary Materials
Data Availability Statement
All data supporting the findings of this study are available on request.