Development and validation of a childhood self-efficacy for functional constipation questionnaire.

BACKGROUND: Children with functional constipation fear painful bowel movements leading to stool withholding behavior. Self-efficacy is the belief that an individual can accomplish a given goal. If children with constipation avoid defecation because they think that they are unable defecate comfortably, this low self-efficacy may prevent treatment success. The aim of the current study was to develop and validate a constipation specific self-efficacy scale. METHODS: The self-efficacy for functional constipation questionnaire (SEFCQ) was developed by the authors and evaluated by 10 children and seven experts. Ninety-nine healthy children and 122 children with functional constipation completed the SEFCQ and three other questionnaires measuring related constructs. KEY RESULTS: Minor changes were made in wording based on feedback from experts and children. Factor analysis showed two scales, a 7 item Action scale (Cronbach's α = 0.88) and a 7 item Emotion scale (Cronbach α = 0.86). The SEFCQ total scale correlated positively with general self-efficacy (r = .32, P < .001) and quality of life (r = .20; P < .01) and negatively with anxiety (r = -.15; P < .05). Scores on the SEFCQ were higher in children without functional constipation compared to those with functional constipation (53.33 + 3.38 vs 39.34 + 7.19, P < .001). CONCLUSIONS & INFERENCES: We developed a constipation specific self-efficacy questionnaire with good initial internal reliability, excellent face validity and adequate content validity. A low self-efficacy for defecation, may make the child resist their physical urge to defecate and hence, the need for further studies to assess its effect on treatment outcomes.

Interobserver variability in antroduodenal manometry.

Interobserver variability affects investigations involving assessment of complex visual data, such as histopathology, radiology and motility. This study assessed interobserver variation for interpretation of antroduodenal manometry (ADM), as this has not been previously investigated. Thirty-five ADM recordings from children aged 0.3-18 years were independently evaluated by five experienced paediatric gastroenterologists who were blinded to cases' clinical histories. Intra-class correlation (ICC) was analysed for detection and measurement of phase three of the migrating motor complex (MMC) and Cohen's kappa statistic was calculated between observer pairs for detection of specific motility features and final diagnosis. Observers were unanimous on the differentiation of normal and abnormal motility in 63% of cases. There was excellent interobserver agreement for the number of phase three of the MMC in fasting (ICC = 0.82, P < 0.0001) and for measurements of phase three of the MMC (ICC = 0.9999, P < 0.0001). Detection of other normal and abnormal motility patterns varied more. Objective findings such as the presence of phase three of the MMC correlated more closely than findings that involved the integration of several variables, such as final diagnosis. However, these data overall indicate that agreement between expert observers for the distinction of normal and abnormal antroduodenal motility compares favourably with other standard medical assessments.

Cyclic vomiting syndrome in adults.

Cyclic vomiting syndrome (CVS) was initially described in children but can occur in all age groups. Cyclic vomiting syndrome is increasingly recognized in adults. However, the lack of awareness of CVS in adults has led to small numbers of diagnosed patients and a paucity of published data on the causes, diagnosis and management of CVS in adults. This article is a state-of-knowledge overview on CVS in adults and is intended to provide a framework for management and further investigations into CVS in adults.

Ileal manometry in children following ileostomies and pull-through operations.

Our aim was to analyse the patterns of ileal contractions in children. We reviewed the charts of 23 children who had ileal manometry studies (16 males), mean age 7 years (range 2 months to 17 years). We positioned the manometry catheters with 4-8 recording sites, 5 or 15 cm apart, through ileostomies fashioned for clinically indicated reasons. We studied six additional children with persistent faecal soiling following endorectal pull through for Hirschsprung's disease; the catheters were positioned through the anus and colon into the ileum. We recorded phasic and tonic intermittent contractions in all the subjects, clustered contractions (rate 5-9 min-1, duration 20-120 s) in 19 subjects with ileostomies and four with endorectal pull throughs. In 13 children there were prolonged propagated contractions, > 60 mmHg in amplitude, > 15 s in duration, propagating at rates of 2-6 cm s-1 over at least 20 cm. The migrating motor complex was rare; in 55 h of fasting recording there were two phase III sequences. There are four distinctive features of ileal manometry recordings in children: random intermittent contractions, clustered contractions, prolonged propagated contractions and tonic contractions. The features of ileal motility differ from motility in the proximal small bowel.

Colonic diversion for intractable constipation in children: colonic manometry helps guide clinical decisions.

BACKGROUND: Colonic manometry helps discriminate functional and behavioral causes for childhood constipation from colonic neuromuscular disease. METHODS: Of 375 colonic manometries performed for clinical indications, 12 could not be interpreted because of chronic colonic dilation. Based on colonic manometries that showed either no contractions or an absence of the gastrocolonic response or an absence of high-amplitude propagating contractions, the authors recommended diverting colostomies or ileostomies in 12 chronically constipated children (mean age, 4 years; range, 2-14 years, 5 boys). Before study, medical treatment was ineffective in all children. These children had persistently dilated colons with pathologic diagnoses of intestinal neuronal dysplasia (n = 4), hypoganglionosis (n = 2), hollow visceral myopathy (n = 1), and normal (n = 5). RESULTS: Six to 30 months after diversion, the authors restudied all the children. Eleven of 12 diverted colons were no longer dilated. In two patients, abnormal motility involving the entire colon was unchanged from the initial study, small bowel motility was abnormal, and we recommended no further surgery. In two cases, the colon remained abnormal but small bowel motility was normal, and we recommended subtotal colectomy and ileoproctostomy. In four cases, the left colon remained abnormal, but the right colon was normal, and we recommended reanastomosis after left hemicolectomy. In four cases, motility in the diverted colons was normal, including a gastrocolonic response and high-amplitude propagating contractions, and the authors recommended reanastomosis. Defecation problems resolved in 10 of 12 when followed up 5 to 30 months after treatment. CONCLUSION: These data suggest that in some cases of intractable childhood constipation associated with colonic distention, temporary diversion improved colonic motility. Colonic manometry may be used to predict which patients will benefit from resection or reanastomosis.

Rapid maturation of gastric relaxation in newborn infants.

We studied gastric volume, wall compliance, sensory perception, and receptive relaxation during the first postnatal 80 h in 17 healthy term infants, using a computer-driven air pump and simultaneously measuring pressure and volume within a latex balloon placed through the oropharynx into the stomach. To evaluate gastric compliance, we measured pressures while we infused air into the intragastric balloon at different rates (10, 20, and 60 mL/min) in random sequence. In all infants, there was a linear relationship between intragastric pressure and volume to the maximum pressure tested, 30 mm Hg. Gastric compliance ranged from 0.2 mL/mm Hg to 3.8 mL/mm Hg. Different infusion rates had no effect on compliance. We calculated gastric receptive relaxation by measuring the volume needed to maintain a constant pressure of 10 mm Hg within the balloon for 5 min. Gastric receptive relaxation ranged from 0.5 mL/min to 54 mL/min. Gastric compliance and receptive relaxation increased with postnatal age (r = 0.70, p < 0.005; r = 0.79, p < 0.001, respectively) and with number of feedings (r = 0.80, r = 0.88, respectively, both p < 0.001). There was no correlation between weight or type of feeding (breast versus formula) and either gastric compliance or relaxation. In conclusion, these results may explain the small feedings that neonates ingest in the first days of life. During the first 3 postnatal d, the newborn stomach becomes more compliant and develops more receptive relaxation, associated with a larger volume capacity.

Function of hollow viscera in children with constipation and voiding difficulties.

We wished to investigate the urodynamic characteristics and colonic motility in a group of children with severe chronic constipation and lower urinary tract symptoms. We performed colonic manometry using an endoscopically placed catheter. The urodynamic studies consisted of cystometry, electromyography of the external urethral sphincter, measurement of urinary flow rate, and urethral pressure profile. We found abnormal colonic motility in all patients. Findings included: absent gastrocolonic response (N = 8), absent high-amplitude propagated contractions (HAPCs) (N = 4), and abnormal propagation of HAPCs (N = 7). Urodynamic features were abnormal in 10 children. Findings included: uninhibited bladder contractions (N = 6), hypertonic bladder (N = 2), sphincter dyssynergy (N = 2), small capacity bladder (N = 1). In all children constipation improved, in three after a partial colectomy. Urinary symptoms persisted. We conclude that some children with severe constipation may have a neuropathy affecting both the colonic and lower urinary tracts systems. In this group of patients treatment of constipation does not result in resolution of urinary symptoms.

Colonic motility after surgery for Hirschsprung's disease.

OBJECTIVE: Years after surgery for Hirschsprung's disease, many children continue to suffer from fecal incontinence or constipation. The purpose of the present investigation was to define the physiology underlying the persistent symptoms in children after surgery for Hirschsprung's disease, and to determine the outcome of interventions based on the results of the motility testing. METHODS: We studied 46 symptomatic patients (5.5+/-3.3 yr old, 35 male) >10 months after surgery for Hirschsprung's disease. We performed a colonic manometry with a catheter placed with the tip in the proximal colon. We used a structured questionnaire and phone interview to follow up the patients an average of 34 months after the manometry. RESULTS: We identified four motility patterns: 1) high-amplitude propagating contractions (HAPCs) migrating through the neorectum to the anal sphincter, associated with fecal soiling (n = 18); 2) normal colonic manometry associated with fear of defecation and retentive posturing (n = 9); 3) absence of HAPCs or persistent simultaneous contractions over two or more recording sites (n = 15), associated with constipation (n = 13); and 4) normal colonic motility and a hypertensive internal anal sphincter (>80 mm Hg) (n = 4). We based treatment on results of the motility studies. There was improvement in global health (mean score, 3.9+/-1.1 vs 2.8+/-1.3 at the time of initial evaluation, p < 0.001) and emotional health (3.8+/-1.1 vs 2.6+/-1.1, p < 0.0001). Improvement in the number of bowel movements occurred in 72% of children. Resolution or decreased abdominal pain was reported in 80%. CONCLUSIONS: Colonic manometry clarifies the pathophysiology and directs treatment in symptomatic children after surgery for Hirschsprung's disease.

Rumination syndrome in adolescents.

OBJECTIVES: To evaluate the clinical presentation and to assess the usefulness of antroduodenal manometry (ADM) and the results of multidisciplinary team management in 12 neurologically normal adolescents (9 girls) with rumination. STUDY DESIGN: All patients had extensive investigations that ruled out other causes of their chronic symptoms. We performed ADM in all patients. A multidisciplinary approach was used for the nutritional and behavioral rehabilitation of these patients. RESULTS: The median age at presentation was 14 years (range, 9-19 years), and the average duration of symptoms was 17 months. All patients complained of postprandial, effortless regurgitation, and the majority had weight loss and abdominal pain. Results of fasting ADM were normal in all. The postprandial ADM showed brief, simultaneous pressure increases at all recording sites, associated with regurgitation in 8 patients. No emesis was observed in the other 4 children during the study. Treatment included nutritional support in combination with antidepressants and anxiolytics (n = 6), cognitive therapy with biofeedback or relaxation techniques (n = 7), and pain management (n = 2). Resolution or improvement of symptoms was seen in 10 of the 12 patients, and successful transition to oral feedings was achieved in all during the follow-up period, which ranged from 5 to 36 months. CONCLUSIONS: Rumination is a distinct functional gastrointestinal disorder of otherwise healthy children and adolescents, which can be diagnosed on the basis of clinical features. The ADM shows a characteristic pattern and rules out motility disorders that are often confused with rumination. A multidisciplinary team approach is associated with satisfactory recovery in most patients.

Colonic motility in children with repaired imperforate anus.

Following surgical correction of imperforate anus, voluntary bowel control is frequently poor because of abnormal anorectal function. Using colonic manometry we investigated the role of colonic motility in the pathogenesis of fecal soiling in children following imperforate anus repair. Thirteen children with repaired imperforate anus and fecal soiling underwent motility testing 2-12 years after anoplasty. All had fecal incontinence unresponsive to conventional medical treatment. Colonic manometry was performed using water-perfused catheters. Anorectal manometry was undertaken in 10 patients. Motility study results, treatment and outcomes were compared. All patients had high-amplitude propagating contractions (HAPCs) with an average of 80% propagation into the neorectum. There was no correlation between HAPC number or morphology and any variable. Internal anal sphincter resting pressure was low in 6/10 patients. Relaxation of the internal anal sphincter was present in 6/10 children. Only 1 of 5 patients able to cooperate was capable of generating a normal maximal squeeze pressure. Therapeutic regimens were changed in 11 patients with clinical improvement in five. Fecal soiling in patients with repaired imperforate anus is a multifactorial problem including propagation of excessive numbers of HAPCs into the neorectum as well as internal anal sphincter dysfunction. Colonic manometry in conjunction with anorectal manometry aids in the understanding of the pathophysiology of fecal soiling and guides clinical management in children with repaired imperforate anus.

Effect of sequential erythromycin and octreotide on antroduodenal manometry.

BACKGROUND: In earlier studies, erythromycin stimulated but octreotide inhibited gastric antral contractions, as each drug induced phase 3-like episodes. METHODS: To assess the effect of erythromycin pretreatment on octreotide-induced changes in antroduodenal motility, 16 patients were studied (mean age, 8.7 +/- 1.5 years, 8 male): 6 with severe gastroesophageal reflux, 4 with cyclic vomiting, 3 with gastroparesis, 2 with chronic intestinal pseudo-obstruction, and 1 with Crohn's disease and unexplained nausea and vomiting. After recording fasting antroduodenal motility for 3 hours, 1 mg/kg intravenous erythromycin was administered over 30 minutes. Sixty minutes after the erythromycin infusion, 0.5 microg/kg subcutaneous octreotide was administered, followed 1 hour later by a meal. RESULTS: Phase 3 occurred spontaneously in 10 patients and after erythromycin in 12 patients. When administered after erythromycin, octreotide immediately induced phase 3s contractions in 15 patients, beginning in the antrum. In 7 children, some of the octreotide-induced phase 3s did not propagate. After the meal, antral contractions continued in all patients. The fed pattern was replaced in 14 patients by alternating phase 3 and phase 1 activities. CONCLUSIONS: Pretreatment with erythromycin prevented octreotide-induced inhibition of antral contractions. Inhibition of antral contractions by octreotide may be mediated through either a direct or indirect suppression of motilin release, because antral contractions persist after pretreatment with the motilin receptor agonist erythromycin.

Childhood functional gastrointestinal disorders.

This is the first attempt at defining criteria for functional gastrointestinal disorders (FGIDs) in infancy, childhood, and adolescence. The decision-making process was as for adults and consisted of arriving at consensus, based on clinical experience. This paper is intended to be a quick reference. The classification system selected differs from the one used in the adult population in that it is organized according to main complaints instead of being organ-targeted. Because the child is still developing, some disorders such as toddler's diarrhea (or functional diarrhea) are linked to certain physiologic stages; others may result from behavioral responses to sphincter function acquisition such as fecal retention; others will only be recognizable after the child is cognitively mature enough to report the symptoms (e.g., dyspepsia). Infant regurgitation, rumination, and cyclic vomiting constitute the vomiting disorders. Abdominal pain disorders are classified as: functional dyspepsia, irritable bowel syndrome (IBS), functional abdominal pain, abdominal migraine, and aerophagia. Disorders of defecation include: infant dyschezia, functional constipation, functional fecal retention, and functional non-retentive fecal soiling. Some disorders, such as IBS and dyspepsia and functional abdominal pain, are exact replications of the adult criteria because there are enough data to confirm that they represent specific and similar disorders in pediatrics. Other disorders not included in the pediatric classification, such as functional biliary disorders, do occur in children; however, existing data are insufficient to warrant including them at the present time. For these disorders, it is suggested that, for the time being, clinicians refer to the criteria established for the adult population.

Gastrointestinal sounds and migrating motor complex in fasted humans.

OBJECTIVE: We investigated the relationships among gastrointestinal sounds, gastrointestinal manometric findings, and small intestinal transit time in healthy fasted humans. METHODS: Gastrointestinal sounds acquired with two microphones attached to the upper and lower abdominal walls of healthy subjects were quantified with a computer-aided sound analysis program. Antroduodenal contractions were recorded by manometry. Small intestinal transit time was measured by breath hydrogen testing after intraduodenal administration of lactulose. RESULTS: The sum of the gastrointestinal sound amplitudes (sound index) in both the upper and lower abdomen changed with time, coinciding with the gastric phases of the migrating motor complex. The sound indices in the upper and lower abdomen were 59.0+/-24.8 and 98.1+/-21.6 mV/min in phase 1, 95.5+/-27.9 and 127.4+/-34.9 mV/min in phase 2, and 132.8+/-12.4 and 188.5+/-73.4 mV/min in phase 3, respectively. There were no significant differences among motility phases in terms of the mean duration or frequency of each sound event. Intravenous erythromycin induced phase 3 in the stomach and doubled the sound index. Somatostatin analogue induced phase-3-like clustered contractions in the duodenum, but inhibited antral contractions and decreased the sound index. The small intestinal transit time was shorter and the sound index increased after intravenous metoclopramide, compared with controls. Scopolamine delayed small intestinal transit time and decreased the sound index. CONCLUSIONS: This study is the first to document the relationships between gastrointestinal sounds and the migrating motor complex. The chronological relation between antral motility and gastrointestinal sounds, and the dissimilar effects of erythromycin and somatostatin, suggest that antral contractions increase gastrointestinal sounds, perhaps by supplying gas into the intestine.

Family studies of infantile visceral myopathy: a congenital myopathic pseudo-obstruction syndrome.

We conducted family studies of a rare congenital myopathic pseudo-obstruction to provide recurrence risks to families of affected children. This infantile visceral myopathy (IVM) involves the smooth muscles of the digestive tract and frequently the urinary bladder. Family and pregnancy histories from 16 families were evaluated to identify possible environmental or genetic components. The families were ethnically and geographically diverse within the United States. Eleven of the children were alive, four had died, and the status of one was unknown. The sex ratio was 5 females to 11 males. The pregnancy histories provided no evidence of a teratogenic cause. In one family, the disorder passed from parent to child. There were no consanguineous matings, no similarly affected sibs, and except for one case, the family histories did not suggest affected relatives. We suspect a new dominant mutation may be responsible for some cases of IVM, whereas in others, IVM may be caused from a dominant gene with variable expressivity and incomplete penetrance. Therefore, we predict the recurrence risk of severely affected children is much less than the 25 or 50% risk sometimes given families based on the assumption of autosomal recessive or autosomal dominant inheritance. When counseling IVM families, a thorough family history is essential. Subsequent pregnancies should be monitored by ultrasound for megacystis that was detected prenatally in seven of these cases.

Effect of octreotide on gastrointestinal motility in children with functional gastrointestinal symptoms.

BACKGROUND: The somatostatin analogue octreotide has been proposed as a possible therapeutic agent in patients with abnormal gastrointestinal motility. This study was conducted to study the effects of 0.5 microg/kg and 1.0 microg/kg subcutaneous octreotide on antroduodenal motility in children with chronic gastrointestinal disorders. METHODS: Twenty-three children were studied, eight with intestinal pseudo-obstruction, six with nonulcer dyspepsia, six with gastroesophageal reflux disease, and three with intractable constipation. After recording fasting motility for more than 4 hours, the children were randomized to receive 0.5 microg/kg or 1 microg/kg of subcutaneous octreotide. Motility was recorded for another hour after feeding in 12 children. RESULTS: Phase III of the motor migrating complex was present in 13 of 23 children before and in 21 after octreotide (p < 0.02). All phase III episodes after administration of octreotide except one originated in the small intestine. Phase IIIs after octreotide were longer and were propagated faster than the spontaneous phase IIIs. There were no antral contractions during fasting after octreotide. There was a significant decrease in phase II intestinal motor activity in the hour after administration of octreotide (p < 0.001). There was no difference in effect between the two doses. After feeding, antral contractions were present in all children, and intestinal phase IIIs were not abolished. CONCLUSIONS: In children with chronic bowel disorders, subcutaneous octreotide induced phase IIIs that differed from spontaneous phase IIIs and were not inhibited by meals. Octreotide decreased antral motility during fasting and inhibited intestinal phase II. Feeding abolished the inhibitory effect of octreotide on antral motility.

Bisacodyl and high-amplitude-propagating colonic contractions in children.

BACKGROUND: The purpose of these studies was to determine the suitability of bisacodyl for stimulating high-amplitude-propagating contractions in pediatric studies of colonic manometry. METHODS: Water-perfused manometry catheters were inserted into the right colon of children referred for evaluations related to defecation disorders. Colonic motility was measured in a 3-hour test session: an hour fasting, an hour after a meal, and 30 minutes after administration of a provocative agent. RESULTS: Bisacodyl was superior to edrophonium as a stimulant for inducing high-amplitude-propagating contractions. Bisacodyl-induced high-amplitude-propagating contractions were similar in amplitude, duration, propagation velocity, and sites of origin and extinction to naturally occurring high-amplitude-propagating contractions. The effect of intrarectal bisacodyl was similar to that of intracecal bisacodyl, except for a delay of 10 minutes in onset. Bisacodyl induced high-amplitude-propagating contractions in all 28 children (22 with spontaneous high-amplitude-propagating contractions) without evidence of neuromuscular disease and in 2 of 9 children with a colonic neuromuscular disorder and no spontaneous high-amplitude-propagating contractions. CONCLUSIONS: Bisacodyl-induced high-amplitude-propagating contractions were quantitatively and qualitatively similar to naturally occurring high-amplitude-propagating contractions. In selected cases, such as in children receiving total parenteral nutrition or restricted fluid intake, it may be possible to shorten diagnostic colonic manometry using bisacodyl rather than waiting for spontaneous high-amplitude-propagating contractions.

Is electrogastrography a substitute for manometric studies in children with functional gastrointestinal disorders?

We performed simultaneous fasting and fed antroduodenal manometry and EGG in 25 children with functional bowel disorders. Three patients (12%) had an uninterpretable EGG. The manometric studies showed severe neuropathy in six patients; milder neuropathic changes in five patients; postprandial hypomotility in one patient; myopathy in four patients, and normal motility in the remaining six patients. The percentage of tachygastria time (frequency > 3.5 cycles/min) was higher in the patiens with mild (44.1 +/- 15.8%) and severe (48 +/- 19.1%) neuropathy than in the patients with myopathy (20 +/- 16.2%, P < 0.05) or with normal motility (23 +/- 13.3%, P < 0.05). There was a considerable overlap in the percentage of tachygastria and total arrhythmia time among the different study groups. The ratio of post- to preprandial power was significantly higher (2.5 +/- 0.07) in children with normal motility than in the other patients groups. Every child with total arrhythmia time < 35% and a ratio of post- to preprandial power > 2.4 had normal manometry. In summary, EGG differentiated groups of children with normal manometry from others with neuropathic or myopathic changes, but in a minority of patients the study was not interpretable and there was overlap in EGG results between children with normal and abnormal manometry.