Pediatric Feeding Disorder in Children With Short Bowel Syndrome

Abstract

Objective: 

We aimed to characterize the prevalence of pediatric feeding disorder (PFD) in short bowel syndrome (SBS) and study factors contributing to the persistence of PFD.

Methods: 

Single-center retrospective study of patients diagnosed with SBS at age 6 months or younger. Data were collected in 3-month intervals through age 2 years, and every 6 months through age 4 years. Demographic information, anthropometric data, and details regarding nutrition support were recorded and analyzed.

Results: 

We reviewed 28 patients. Of the 21 patients who were weaned off parenteral nutrition, 57.1%, 81.0%, 90.5%, and 100.0% achieved this by 12, 24, 36, and 48 months of age, respectively. Of the 13 patients who were weaned off enteral nutrition, 30.8%, 69.2%, 76.9%, and 100.0% achieved this by 12, 24, 36, and 48 months, respectively.

Discussion: 

The prevalence of PFD was 100.0%, 76.5%, 68.8%, and 70.0% at 1, 2, 3, and 4 years of age, respectively. All patients who exhibited resolution of PFD had an underlying etiology of necrotizing enterocolitis. Median small bowel percentage remaining was greater in patients who exhibited resolution of PFD compared to those who did not. Except for the group of patients seen at 4 years of age, a larger percentage of patients with vomiting/history of requirement of postpyloric feeds were seen among patients with PFD compared to those without PFD.

Conclusion: 

PFD is prevalent in children with SBS. Although prevalence decreases over time, children with PFD will continue to require more medical attention than children that do not.

What Is Known/What Is New

What Is Known

• Children with short bowel syndrome typically require parenteral nutrition support for varying periods of time.
• Difficulty advancing feeds is thought to be common in children with short bowel syndrome.

What Is New

• Children with short bowel syndrome continue to require enteral nutrition even after they no longer need parenteral nutrition.
• Pediatric feeding disorder is common in children with short bowel syndrome and is a persistent concern in some children with short bowel syndrome.

Short bowel syndrome (SBS) is the most common cause of pediatric intestinal failure ⁽¹⁾ and is commonly defined as the requirement of parenteral nutrition (PN) for >42 days after resection of bowel, or a residual small bowel length of <25% expected for gestational age ⁽²⁾. Norms for small bowel length are used to define SBS ^(1,3).

In an era wherein the majority of children with SBS will survive, our focus should be on improving other outcomes such as oral feeding. Although commonly encountered in practice, difficulty in advancing oral feeding in this population has not been well described. A recent consensus statement defines pediatric feeding disorder (PFD) as impairment in oral intake that is not age appropriate, and is associated with medical, nutritional, feeding skill, and/or psychosocial dysfunction ⁽⁴⁾. Development of feeding disorder in children with SBS is thought to be a complex process, and is presumed secondary to physical, developmental, and social deviations from normal childhood ⁽⁵⁾.

We aimed to characterize the nutrition support provided to these patients and to study the prevalence of PFD in this population over time. We also aimed to study the impact various factors may pose on the persistence of PFD.

METHODS

This was a single-center retrospective study. Institutional review board approval was obtained for chart review and waiver of consent (IRB 1202639). We studied patients who met criteria for diagnosis of SBS ⁽²⁾ within the first 6 months of life and were followed by our tertiary care Gastroenterology Clinic for at least 1 year after diagnosis.

We excluded patients with intestinal failure secondary to causes other than SBS. At our facility, the standard of practice with SBS is to place a gastrostomy tube shortly after diagnosis, to provide continuous enteral nutrition (EN) and increase the volume until enteral autonomy is attained. Oral feedings are simultaneously provided and advanced as tolerated. As adaptation occurs, we transition continuous delivery of EN to bolus feeds. As oral skills advance, EN is gradually weaned and discontinued. Patients who require PN or EN are seen more frequently in our clinic than patients who are completely fed orally.

In our study, patient charts were reviewed, and data collected in 3-month intervals through 2 years of age, and every 6 months through 4 years of age. Within each interval, we selected the visit that was closest to the middle. If a patient had not been seen during the interval, data were not collected for the interval. Age was corrected for prematurity where applicable. The following data were recorded: gestational age, underlying etiology for bowel resection, percentage of small bowel remaining after resection, presence/absence of the ileocecal valve (ICV) and colon, presumed food allergy, presence of significant emesis (defined as history of vomiting at >50% of reviewed visits, or requirement of postpyloric feeds), history of serial transverse enteroplasty procedure, history of small bowel transplant, history of receiving teduglutide, history of receiving speech therapy, details of oral diet consumed including requirement of oral high-calorie beverage, details of EN and PN support, and anthropometric parameters. For the purpose of our analysis, we considered patients who were on intravenous fluids as being on PN. We did not differentiate the children seen by speech therapy alone from those seen at a multidisciplinary feeding clinic.

We defined PFD based on established criteria ⁽⁴⁾ as reliance on enteral feeds to sustain nutrition, reliance on high-calorie oral supplements to sustain nutrition, or feeding skill dysfunction resulting in not consuming an age-appropriate diet. Patients dependent on PN were considered to have a PFD. We considered the reliance on EN as indication of a feeding disorder, recognizing that this may be an iatrogenic indication, as EN may be considered a medical treatment for underlying SBS. Some analyses were performed with the PFD diagnosis using all 3 criteria, whereas others were performed using only reliance on high-calorie oral supplements and inability to consume an age-appropriate diet. In patients on oral feedings, we reviewed the appropriateness of variety and textures consumed to determine the presence/absence of PFD.

We used descriptive statistics to describe our patients. Due to the small numbers of patients we were unable to conduct more advanced statistical analyses.

RESULTS

A total of 28 patients were studied. Thirteen (46.4%) of the total 28 patients were girls. Twenty-three patients (82.1%) were premature. Median gestational age was 32 weeks (interquartile range [IQR] 25.5, 35). Sixteen (57.1%) patients had an underlying etiology of necrotizing enterocolitis (NEC). The remaining 12 patients (42.9%) underwent intestinal resection due to complicated gastroschisis, intestinal atresia, volvulus, ruptured omphalomesenteric duct, and mesenteric venous thrombosis. Median small bowel remaining was 25% of expected length for age (IQR: 16.6, 33.9). Colon and ICV were intact in 12 (42.9%) patients.

Eleven (39.3%) patients had significant vomiting and/or required postpyloric feeds. Five (17.9%) patients had presumed food allergies. Five (17.9%) patients underwent a serial transverse enteroplasty procedure. One (3.6%) patient received teduglutide therapy; 1 (3.6%) was the recipient of a small bowel transplant.

Throughout the study, across all age intervals median weight z score was −1.1 (IQR: −1.9, −0.3), median length/height z score was −2.0 (IQR: −2.7, −1.1), and median weight-for-length/BMI z score was 0.2 (IQR: −0.5, 0.7); we used corrected age when relevant. Median head circumference z score for patients younger than 2 years of age was −1.0 (IQR −2.2, 0.3).

Of the 28 patients studied, 4 died (all on PN at the time of death). Twenty-one patients were successfully weaned off PN; 3 remained on PN at the time of the study. The median age of discontinuation of PN among patients who weaned off PN was 10.8 months (IQR: 6.0, 18.4). Mean age of discontinuation of PN was 15 months.

Of the 21 patients who were weaned off PN, 3 transferred care to other facilities while on EN, 5 remained on EN under our care, and 13 were successfully weaned to an exclusively oral diet. The median age of discontinuation of EN among patients who weaned off EN was 15.7 months (IQR: 10.5, 27.6). Mean age of discontinuation of EN was 20.5 months.

Among the patients who were weaned off both PN and EN (n = 13), median time lag between the discontinuation of each form of nutrition support was 7.9 months (IQR: 3.47, 13.46).

Figure 1 is a flow diagram illustrating how the total 28 patients were divided into subgroups for our analysis. Age at which patients were weaned from PN and EN are depicted in Figure 1. Figure 2 is a Kaplan-Meier survival curve where curve 1 represents patients on EN and curve 2 represents patients on PN.

FIGURE 1: Flow diagram depicting patient analysis. EN = enteral nutrition; PN = parenteral nutrition.

FIGURE 2: Kaplan-Meier survival curve. EN = enteral nutrition; PN = parenteral nutrition.

Prevalence of PFD using all 3 criteria is detailed in Table 1. Of the patients followed at 1, 2, 3, and 4 years of age, 100.0%, 76.5%, 68.8%, and 70.0% met criteria for PFD. Because enteral feeding can lead to an iatrogenic diagnosis of PFD in this population, the prevalence of feeding disorders using only the other 2 criteria (reliance on oral supplements and/or failure to consume an age-appropriate diet) was also included in Table 1. Prevalence of PFD decreased to 80.0%, 70.6%, 62.5%, and 50.0% at 1, 2, 3, and 4 years while excluding reliance on EN as a criterion for PFD.

TABLE 1 - Prevalence of pediatric feeding disorder at 1, 2, 3, and 4 years age

	1 y	2 y	3 y	4 y
Patients seen in this interval (n)	25	17	16	10
Patients with PFD (n)	25 (100%)	13 (76.5%)	11 (68.8%)	7 (70%)
PFD criteria met
Criterion 1: reliance on enteral feeds − N (% of total patients seen)	21 (84%)	10 (58.8%)	7 (43.8%)	5 (50%)
Criteria 2 or 3: reliance on high-calorie oral supplements or feeding skill dysfunction − N (% of total patients seen)	20 (80%)	12 (70.6%)	10 (62.5%)	5 (50%)
Patients on parenteral nutrition (n)	12	3	4	2

PFD = pediatric feeding disorder.

Of the patients who continued to be followed, those with and without a PFD diagnosis were compared at 1, 1.5, 2, 3, and 4 years age (Table 2). At each age interval, it was noted that all patients who exhibited resolution of PFD were premature and had an underlying etiology of NEC. Median small bowel percentage remaining was greater in patients who exhibited resolution of PFD compared to those who did not. With the exception of the group of patients seen at 4 years of age, a larger percentage of patients with vomiting/history of requirement of postpyloric feeds was seen among patients with PFD compared to those without PFD. There was a higher percentage of patients who underwent a bowel-lengthening procedure among patients with PFD. No trends were noted with regards to intactness of colon/ICV, history of food allergies, and history of receiving speech/swallowing therapy.

TABLE 2 - Comparison of patients with and without PFD at 1, 1.5, 2, 3, and 4 years age

	1 y		1.5 y		2 y		3 y		4 y
	25		16		17		16		10
Patients seen in this interval	With PFD	Without PFD	With PFD	Without PFD	With PFD	Without PFD	With PFD	Without PFD	With PFD	Without PFD
Number of patients	25	0	15	1	13	4	11	5	7	3
Underlying etiology NEC − n (%)	14 (56.0%)	N/A	9 (60.0%)	1 (100.0%)	7 (53.9%)	4 (100.0%)	7 (63.6%)	5 (100.0%)	3 (42.9%)	3 (100.0%)
% SB left based on age − median % (IQR %)	24.8 (16.1–28.7)	N/A	26.3 (15.6–32.2)	61.1 (N/A)	26.6 (14.9–34.7)	39.1 (33.9–45.1)	21.7 (14.9–29.0)	39.7 (28.5–59.6)	18.8 (14.9–29.8)	29.3 (24.7–45.2)
Significant vomiting/requirement of postpyloric feeding − n (%)	10 (40.0%)	N/A	6 (40.0%)	0 (0.0%)	5 (38.5%)	0 (0.0%)	3 (27.3%)	0 (0.0%)	1 (14.3%)	1 (33.3%)
Prematurity − n (%)	21 (84.0%)	N/A	10 (66.7%)	1 (100.0%)	9 (69.2%)	4 (100.0%)	9 (81.8%)	5 (100.0%)	5 (71.4%)	3 (100.0%)
Colon and ileocecal valve intact – n (%)	11 (44.0%)	N/A	6 (40.0%)	0 (0.0%)	6 (46.2%)	1 (25.0%)	5 (45.5%)	1 (20.0%)	3 (42.9%)	0 (0.0%)
History of food allergies − n (%)	4 (16.0%)	N/A	3 (20.0%)	1 (100.0%)	2 (15.4%)	1 (25.0%)	2 (18.2%)	1 (20.0%)	2 (28.6%)	1 (33.3%)
Underwent bowel lengthening procedure – n (%)	5 (20.0%)	N/A	3 (20.0%)	0 (0.0%)	2 (15.4%)	0 (0.0%)	2 (18.2%)	0 (0.0%)	2 (28.6%)	0 (0.0%)
Received speech/swallowing therapy – n (%)	16 (64.0%)	N/A	11 (73.3%)	0 (0.0%)	9 (69.2%)	1 (25.0%)	7 (63.6%)	2 (40.0%)	4 (57.1%)	2 (66.7%)

IQR = interquartile range; NEC = necrotizing enterocolitis; PFD = pediatric feeding disorder.

DISCUSSION

Our data show that patients with SBS who wean from PN continue to need EN support. Although the median lag between discontinuation of PN and then of EN was about 8 months, about a third of the patients continued to require EN past the age of 2 years. The majority of patients were able to wean off EN by the age of 4 years. Differences in the median and mean times to wean off PN and EN are due to outliers.

Irrespective of the need for EN, about 3 quarters of the children with SBS who continued to be followed in clinic demonstrated symptoms of a feeding disorder at age 2 and about 70% continued to have these symptoms at the age of 4 years. These prevalence rates are based on a denominator of the number of patients seen in a particular age interval, and hence cannot be generalized across the entire pediatric SBS population. These prevalence rates do, however, tell us that a large number of the SBS patients who are regularly followed in clinic have a PFD.

Although our data are limited by our inability to conduct more statistical analyses, the factors that we attempted to correlate with persistence of PFD provide some important clues to be pursued in future studies. It is likely that the factors that we studied were either correlates of development (prematurity) or factors that directly influence severity of SBS (NEC vs non-NEC etiologies, presence of ICV, etc) and 1 other factor that we suspected may influence the severity of PFD (the presence of significant vomiting or need for postpyloric tube feeding). The association of NEC and resolution of PFD is likely due to the prevalence of dysmotility in patients with small bowel atresia and gastroschisis. Impaired development and maturation of the enteric nervous system and interstitial cells of Cajal are presumed to contribute to dysmotility in these conditions ^(6,7). This observation aligns with findings that correlate enteral autonomy with underlying NEC etiology for SBS ⁽⁸⁾. Our study did not find an association between the presence of colon and ICV with resolution of PFD. This factor was also found to not correlate with enteral autonomy in a recent meta-analysis ⁽⁹⁾.

We have reported data from a single institution with a defined EN progression protocol. It is unclear if children fed using more aggressive oral feeding protocols or children fed using bolus feeds as opposed to continuous feeds (as is customary in our protocol) will have different severities of PFD.

An obvious limitation of our study is the small number of patients in our cohort. In addition, the diagnosis of feeding disorder can be made using other criteria (feeding skill–based criteria such as use of modified feeding equipment or strategies and psychosocial criteria) that we were unable to glean from our chart review. Addition of these criteria is likely to increase the numbers of children with SBS that have PFD. We believe that future studies should incorporate these criteria to provide a more comprehensive understanding of feeding problems in this population. Another important limitation of our study is the lack of developmental data to correlate with feeding outcomes. Use of prematurity is only an indirect measure of developmental abilities. Clearly, developmental status has a huge bearing on oral feeding skill development and needs to be a major consideration when considering the diagnosis of PFD.

CONCLUSIONS

Improved mortality among pediatric SBS patients has been reported at centers with established Intestinal Rehabilitation Programs ⁽¹⁰⁾. This trend is expected to improve with the rising prevalence of these programs. As more pediatric SBS patients age and enter childhood and adolescence, focus of care shifts toward improving the quality of life among patient survivors. The development or persistence of PFD in this population will likely have a significant impact on quality of life.

This study demonstrates the prevalence of PFD in children with SBS. Although the prevalence does decrease over time, it is likely that children with PFD will continue to require more medical attention than children who do not.