Oral health in children with rare diseases and gastrostomy tubes: A retrospective database cohort study

Abstract

Background

The purpose was to investigate oral health in children with rare diseases and gastrostomy tubes (GTs).

Methods

This is a retrospective review of oral health in children with rare diseases and GTs 3–16 years of age who were registered in a database. The children were divided into three cohorts: 1995–2002 (cohort I), 2003–2010 (cohort II), and 2011–2018 (cohort III) with a control group of children with rare diseases without GTs. The presence of gingivitis, calculus, caries, and oral hypersensitivity were obtained from a standardized observation schedule. Frequencies of vomiting and dental visits were obtained from questionnaires completed by legal guardians.

Results

A total of 263 out of 2470 children had a GT. The control group consisted of 2208 children. The presence of gingivitis was significantly lower in cohort III compared with both cohorts I and II. There was no significant difference between calculus and hypersensitivity between the cohorts. The presence of gingivitis, calculus, oral hypersensitivity, and frequency of vomiting were higher in the study group, compared with that of the controls. The study groups showed statistically significant fewer caries in children <12 years of age compared with those without a g‐tube.

Conclusions

Ten percent of the children with rare diseases had a gastrostomy. Children with GTs displayed poorer oral health than children without, except for caries. All children with GTs displayed oral hypersensitivity and a higher frequency of vomiting. Gingivitis decreased when comparing cohort I to cohort III.

INTRODUCTION

There are 296 identified rare diseases compiled from 1109 organizations and 32 international jurisdictions, demonstrating the complexity and diversity of these conditions. ¹ However, a rare disease is a medical condition that affects a small proportion of the general population. ¹ Data show a global variation of 5–80 individuals per 100,000. In over 70% of rare diseases, the origin is genetic. ² The remainder are due to viral or bacterial infections, allergies, and environmental influences. The majority of genetically rare diseases debut and are diagnosed in childhood. ²

Among children with rare diseases, orofacial functions along with eating and swallowing dysfunctions affect the oral health. Impaired oral motor skills, paired with reduced sensation in the oral cavity, diminishes the ability for oral clearance, ³ which increases the risk for caries and gum disease.

Tooth wear, gingivitis, and supragingival calculus are not uncommon in these groups. ³ , ⁴ In children with GTs, the bacterial environment in the oral cavity is affected because of the absence of oral food intake and lack of chewing, both of which are significant for the formation of calculus. ⁵ , ⁶ , ⁷ When oral intake is impaired and metabolic needs exceed energy intake, nutrition support is required. Nutrition can be administered via a gastrointestinal tract (enteral nutrition) or directly into the bloodstream (parenteral nutrition). If enteral nutrition is needed for a longer period, a gastrostomy tube (GT) through the abdominal wall into the stomach is inserted. ⁸ , ⁹ GTs are observed in approximately 9%–13% of patients with rare diseases ³ , ¹⁰ , ¹¹ Performing oral care in children with rare diseases and a GT may be difficult due to impaired oral motor control, in combination with involuntary biting and/or vomiting reflexes. Intellectual and reflex behavioral problems, along with oral hypersensitivity, are additional conditions limiting dental treatment and daily oral care. ³ , ¹²

The purpose of this study was to investigate the oral health in children with rare diseases and GTs.

METHODS

This study is a retrospective review.

Ethics approval and consent to participate

Data collection for the database was approved by the Ethics Committee, Sahlgrenska Academy, University of Gothenburg, and the Regional Data Inspection Board 1995‐05‐31 (Dnr: 189‐95) and 2024 (Dnr 2024‐08132‐02).

Written informed consent was obtained from the participants' parents.

Database

The Mun‐H‐Center (MHC) is a national orofacial resource center for rare diseases in Gothenburg, Sweden. It collects and registers data on oral health and orofacial function in Swedish patients with rare diseases through clinical observations and parental questionnaires. Registrations are kept in the MHC national database, which was created in 1995. The database has given rise to, among others, the following scientific articles. ³ , ¹²

Inclusion criteria

Study group

Between 1995 and 2018, children aged 3–16 years with rare diseases and GTs were documented in the MHC database using clinical observation schedules and questionnaires regarding orofacial function and oral health.

Data reconciliation was performed for three 7‐year periods, forming the following cohorts: cohort I: 1995–2002, cohort II: 2003–2010, and cohort III: 2011–2018. Patients in cohort I could be represented in cohort II and cohort III. The cohorts were further divided into three age groups: 3–6 years, 7–12 years, and 13–16 years.

Control group

Between 1995 and 2018, children aged 3–16 years with rare diseases but without GTs were documented in the MHC database using clinical observation schedules and questionnaires regarding orofacial function and oral health. Cohorts and age groups corresponded to the study group.

Oral examination

An oral examination for gingivitis, calculus, and caries was performed by a dentist. Oral hypersensitivity was assessed by a speech and language pathologist at the MHC During the study, data were registered in a standardized observation schedule. ¹²

To ensure clinical feasibility, the following oral variables were selected for inclusion in the documentation of patients with disabilities:

• 1.Gingivitis (The presence of gingivitis was clinically examined and recorded by indicating yes or no).
• 2.Calculus (The presence of calculus [including supragingival and subgingival calculus] was clinically examined and recorded by indicating yes or no).
• 3.Caries (Caries was clinically recorded in the primary and permanent dentition. The incidence of manifest caries was recorded with yes or no).
• 4.Oral hypersensitivity (Oral hypersensitivity was assessed and noted when the patient reacted to face or mouth contact. The presence of oral hypersensitivity was recorded with yes or no).
• 5.Questionnaire (Questionnaires were answered by parents regarding oral health and orofacial function. From the questionnaires, information was obtained on the frequency of vomiting and the number of dental visits per year ³ ).
• 6.General recommendation (All patients were advised to brush their teeth twice daily using either a regular or electric toothbrush, in accordance with general recommendations).

Statistical processing

Basic descriptive statistical analyses were performed using the chi‐square test in IBM SPSS, version 25.

A P value ≤0.05 was considered statistically significant regarding differences between the study and control groups, as well as between cohorts (over time).

RESULTS

A total of 3013 children with rare diseases were registered in the database.

Of these, 2470 children (54% boys and 46% girls) were documented using both observation schedules and questionnaires between 1995 to 2018, including 974 aged 3–6 years, 1085 aged 7–12 years, and 411 aged 13–16 years (Table 1).

Table 1.

Distribution of gastrostomy tubes.

	Children with a gastrostomy				Children without a gastrostomy
	3–6 years old, n (%)	7–12 years old, n (%)	13–16 years old, n (%)	Total	3–6 years old, n (%)	7–12 years old, n (%)	13–16 years old, n (%)	Total
Cohort I, 1995–2002	36 (67)	16 (30)	2 (4)	54	298 (39)	334 (43)	138 (18)	770
Cohort II, 2003–2010	25 (45)	22 (39)	9 (16)	56	234 (36)	292 (45)	130 (20)	656
Cohort III, 2011–2018	82 (54)	56 (37)	15 (9)	153	299 (38)	365 (47)	117 (15)	781
Total	143 (54)	94 (36)	26 (10)	263	831 (38)	991 (45)	385 (17)	2207

Note: Number and proportion of children with rare diseases with and without gastrostomy tubes, by cohort and age group registered in the Mun‐H‐Center database between the years 1995–2018.

Of the 2473 children, 263 (10%) received nutrition via a GT (Table 1).

The control group consisted of 2207 children who received oral nutrition (Table 1). A higher proportion of children with GTs were registered in cohort III compared with cohorts I and II, respectively (P < 0.001; Figure 1).

Figure 1.

Study population and controls distributed over different periods. The number of individuals 3–16 years, with rare diseases, with and without gastrostomy tubes according to data from the MHC database in the period 1995–2018, divided by cohorts. There is an increase in the number of registered children with gastrostomy tubes between Cohort I‐Cohort III and between Cohort II‐Cohort III, *** = P < 0.001 (chi‐square).

Oral health in children with rare diseases

Gingivitis

In the study group, 48% had gingivitis (Table 2). The proportion of children with gingivitis was unchanged between 1995 to 2002 (cohort I) and 2003 to 2010 (cohort II) but decreased when compared with 2011 to 2018 (cohort III; Figure 2). In the control group, 36% had gingivitis (Table 2). There was a statistically significantly higher frequency of gingivitis in the total study group compared with the control group (P < 0.01), most notably in the youngest group aged 3–6 years (P < 0.001; Table 2).

Table 2.

Symptoms in children with rare diseases compared with those without GT.

	Children with a gastrostomy n = 263		Children without a gastrostomy n = 2208, n (%)		P value
Age groups	n (%)	Number of answers	n (%)	Number of answers
3–6 years	143 (54%)	—	831 (38%)	—	—
7–12 years	94 (36%)	—	992 (45%)	—	—
13–16 years	26 (10%)	—	385 (17%)	—	—
Gingivitis
3–6 years	42 (51%)	82	80 (18%)	448	<0.001
7–12 years	26 (42%)	62	250 (41%)	609	NS
13–16 years	9 (56%)	16	150 (53%)	283	NS
Total	77 (48%)	160	482 (36%)	1340	<0.01
Calculus
3–6 years	21 (23%)	92	18 (3%)	584	<0.001
7–12 years	18 (29%)	62	58 (7%)	831	<0.001
13–16 years	5 (31%)	16	35 (10%)	354	<0.01
Total	44 (26%)	170	106 (6%)	1769	<0.001
Caries
3–6 years	6 (7%)	84	81 (15%)	543	<0.001
7–12 years	3 (7%)	43	106 (16%)	665	<0.05
13–16 years	2 (17%)	12	51 (18%)	286	NS
Total	11 (8%)	139	299 (20%)	1494	NS
Oral hypersensitivity
3–6 years	106 (100%)	106	20 (3%)	659	<0.001
7–12 years	67 (100%)	67	35 (4%)	878	<0.001
13–16 years	20 (100%)	20	10 (3%)	346	<0.001
Total	193 (100%)	193	75 (4%)	1883	<0.001
Frequency of vomiting
3–6 years	48 (37%)	131	40 (5%)	812	<0.001
7–12 years	17 (21%)	81	19 (2%)	975	<0.001
13–16 years	3 (13%)	23	8 (2%)	377	<0.001
Total	68 (29%)	235	65 (3%)	2164	<0.001

Note: P value represents the differences between the study group and the control group (chi‐square).

Abbreviation: NS, non‐significant.

Figure 2.

Proportion of children 3–16 years old with rare diseases and gastrostomy tubes showing gingivitis, calculus, caries, oral hypersensitivity, and vomiting frequency in Cohort I (1995–2002), Cohort II (2003–2010), and Cohort III (2011–2018). Gingivitis was seen to a lesser extent in Cohort III compared with Cohort I and compared with Cohort II, *** = P < 0.001 (chi‐squared).

Calculus

In the study group, 26% had calculus (Table 2). No statistically significant difference was observed over time (Figure 2). In the control group, 6% were found to have calculus (Table 2).

The study group had a statistically significantly higher frequency of calculus compared with the control group (P < 0.01) across all age groups (Table 2).

Caries

In the study group, 7% of the 3–6 year olds had caries in the primary dentition, whereas 7% of the 7–12 year olds and 17% of the 13–16 year olds had caries in the permanent dentition (Table 2).

In the control group, 15% of the 3–6 year olds had caries in the primary dentition, whereas 16% of the 7–12 year olds and 18% of the 13–16 year olds had caries in the permanent dentition (Table 2). The study group exhibited statistically significantly fewer cases of caries compared with the control group in the younger age groups (P < 0.05; Table 2).

Oral hypersensitivity

The entire study group (100%) exhibited oral hypersensitivity, which remained consistent over time between cohorts (Figure 2). In contrast, 4% of the control group showed hypersensitivity (Table 2). The study group had a statistically significantly higher frequency of oral hypersensitivity compared with the control group (P < 0.001) across all age groups (Table 2).

Questionnaire

In the study group, 29% of parents reported that their child vomits frequently (Table 2).

The proportion who frequently vomited remained unchanged between the years 1995 to 2002 (cohort I) and tended to decrease between 2003 to 2018 (cohorts II and III; Figure 2). In the control group, 3% of parents reported frequent vomiting (Table 2). The study group showed a statistically significantly higher frequency of vomiting compared with the control group (P < 0.001) across all age groups (Table 2).

Responses regarding dental visits were recorded for 250 individuals in the study group. The number of visits were reported as: one visit per year (5%), two visits per year (31%), three visits per year (32%), and four visits per year (32%). The number of annual dental visits remained unchanged over time. In the control group, 2072 individuals reported their dental visits as: one visit per year (8%), two visits per year (40%), three visits per year (24%), and four visits per year (28%), with two individuals reporting more than four visits per year. There was no statistically significant difference between the study and control groups in the number of dental visits.

DISCUSSION

This study shows that all children with rare diseases and GTs exhibited oral hypersensitivity, which remained unchanged from 1995 to 2018. The children also experienced more gingivitis and calculus than those without GTs.

The findings of an increased incidence of gingivitis and calculus in children with GTs, compared with those without, are consistent with previous studies. ³ , ⁵ , ⁶ , ¹² , ¹³ Calculus is often rapidly forming. ⁵ , ⁶ , ¹⁴ One interpretation for the increased incidence of gingivitis and calculus is an imbalance in the oral bacterial flora, which may be due to inactivity in the oral cavity, orofacial dysfunction, altered eating frequency, or a lack of chewing. ⁶ , ⁷ , ¹³

The pH value in the oral cavity does not reach acidic values due to the absence of ingested food via the oral cavity, thus becoming creating an environment more favorable to calculus formation. Difficulties with tooth brushing for this patient group are also important to note. ⁴ Another possible explanation for the increased gingivitis and calculus in children with GTs is that oral care may be forgotten or not prioritized in a stressful life situation where the focus is on nutrition. ¹⁵ , ¹⁶ When the child is not using the mouth for eating, parents may not perceive tooth brushing as necessary.

Gingivitis observed in some of the youngest children with GTs may be the oral characteristics of the rare disease itself. ¹⁷ It may also result from regular tooth brushing habits never been established before. Moreover, tooth brushing may not be part of the daily routine for a child with a GT because it may seem unnecessary when food is not ingested orally.

This finding highlights the need for dental care to be included in guidelines for children even before gastrostomy insertion, regardless of age, to emphasize the importance of good oral health despite the reduced activity of various oral functions.

Children with GTs had lower incidence of caries than those without GTs. This is explained by the fact that caries develops through acid attacks from the breakdown of ingested sugar via bacteria. When food is not consumed orally, the risk of caries is generally low. This aligns with the findings of the previously mentioned studies by Jawadi and Hidas, who showed that children with disabilities and GTs had a lower incidence of cariogenic bacteria compared with children with disabilities but without GTs. ⁵ , ⁶

The children with rare diseases and GTs showed a higher degree of oral hypersensitivity compared with those without GTs. Studies have shown that long‐term enteral nutrition causes oral hypersensitivity, which is also supported by the results of this study. ¹⁸ , ¹⁹ , ²⁰ , ²¹ These studies also showed that desensitization affects the ability to eat by increasing the acceptance of various foods in the mouth. For young children with a GT, regular training in oral stimulation and desensitization, such as daily tooth brushing and oral care in the home, as well as during dental visits, can be beneficial. Because toothbrushing becomes more difficult due to hypersensitivity, early exposure to it can be considered as part of the desensitization process. In addition to maintaining good oral hygiene, tooth brushing can support the development of sensory functions in the oral cavity. Further studies may determine whether tooth brushing twice a day affects oral hypersensitivity in young children with a GT.

The results of this study showed that children with GTs vomited more frequently than those without GTs. It has previously been observed that gastroesophageal reflux can be problematic in children with neurological impairment following GT insertion. ²²

The decrease in the proportion of individuals with GTs who vomited over the years may be a result of increased use of medication for gastroesophageal reflux and gastroesophageal reflux disease. ²² , ²³ Oral hypersensitivity during tooth brushing in children with GTs could also contribute to their higher frequency of vomiting compared with children without GTs, further highlighting the importance of desensitization of the oral cavity at an early age.

The strength of this study was that the analyzed data was collected over a long period and included a large number of patients, which is important in detecting variations over time. The dichotomized observation schedule made it possible to study this patient group. Recordings were conducted by various therapists with regular calibrations, which may have resulted in both overdiagnoses and underdiagnoses. A limitation of this retrospective study is that the data was obtained from an existing database. ²⁴ The data used was not detailed but dichotomized to reduce the risk of misinterpretations. Despite the binary nature of the data, it still reveals differences between the study group and the control group.

The number of children with GTs in the database has increased in recent years, which is consistent with other findings. ¹⁰ , ²⁴ , ²⁵ In this study, GTs were more commonly found in younger children, a pattern that aligns with the findings by Wong. ²⁶ No increase in gastrostomy insertions was found in any specific age group over time. Other studies have shown that the greatest increase occurred during the first years of life. ¹⁰ , ²⁴ , ²⁵ , ²⁷ One explanation for the absence of an observed increase in any specific age group may be that very young children were not included. Additionally, the participants in this study were children with rare diseases, which limits comparisons with other studies that describe gastrostomies more generally. ¹⁰ , ²⁴ , ²⁸

Dental care in Sweden is free of charge up to and including 19 years of age. It is provided regularly and individually adapted. ²⁹ Almost all children with rare diseases and GTs required more than one dental visit per year, which remained consistent over time, suggesting that this patient group has received increased care over an extended period. Nevertheless, the results of this study showed that children with rare diseases and GTs require increased dental care. The findings indicate a need of preventive care focused on hypersensitivity, gingivitis, and calculus in this vulnerable group. The result that all patients exhibited oral hypersensitivity is of significant interest. This study highlights the need for early dental intervention in children with rare diseases and a GT. As a result of this study, an oral care program with advice and support for dental staff caring for this patient group has been implemented in Sweden. ³⁰ The insertion of a gastrostomy affects both general and oral health, making it essential that patients receive information on oral and dental care prior to a gastrostomy. Comprehensive, multiprofessional care is necessary for this group. Oral health information should be included in healthcare guidelines for these patients.

CONCLUSION

Ten percent of the children with rare diseases had a gastrostomy. Children with GTs exhibited poorer oral health than those without GTs, except in the case of caries. All children with GTs showed oral hypersensitivity and a higher frequency of vomiting. Gingivitis decreased when comparing cohort I to cohort III.

AUTHOR CONTRIBUTIONS

The study was designed by Anna Nielsen Magnéli and Agneta Robertson; Anna Nielsen Magnéli has conducted data collection; Anna Nielsen Magnéli, Agneta Robertson, Nina Sabel and Larisa Krekmanova have analyzed and interpreted the data; and all authors contributed actively and equally to the drafting and reviewing of the data.

CONFLICT OF INTEREST STATEMENT

None declared.

Supporting information

Nielsen Magnéli et al.

Magnéli AN, Sabel N, Krekmanova L, Robertson A. Oral health in children with rare diseases and gastrostomy tubes: a retrospective database cohort study. Nutr Clin Pract. 2025;40:1158‐1165. 10.1002/ncp.70006