P E Hyman

Use of colonic manometry to differentiate causes of intractable constipation in children

We evaluated colon manometry as a means of differentiating causes of intractable constipation in children. We studied pressure changes in the transverse, descending, and rectosigmoid colons of 23 children with intractable constipation. All patients had a history of less than one bowel movement per week for longer than 2 years without resolution after conventional medical management. The possibility of Hirschsprung disease was excluded in all. On the basis of pathologic and manometric studies of the upper gastrointestinal tract, 10 patients had a diagnosis of gastrointestinal neuropathy and two had a diagnosis of myopathy. The other 11 patients had functional fecal retention; this diagnosis was based on history and outcome of therapy. On the day of study we used endoscopy to place a manometry catheter into the transverse colon and recorded intraluminal pressure for longer than 4 hours. After obtaining a baseline recording, we gave the patient a meal to assess gastrocolonic response. Colonic contractions were recorded in 21 of 23 children. Children with functional fecal retention could be differentiated from those with neuropathy by examination of the postprandial record. After a meal children with functional fecal retention had (1) an increase in motility index (3.4 +/- 0.5 while fasting vs 9.1 +/- 1.3 postprandially; p less than 0.001), and (2) at least one high-amplitude propagated contraction (in 10 of 11 children). The patients with neuropathy had no high-amplitude propagated contractions (p less than 0.001 vs group with functional fecal retention) and motility index in these children did not increase significantly after a meal (2.7 +/- 1.0 while fasting vs 2.9 +/- 1.3 postprandially). The two children with hollow visceral myopathy had no contractions. We conclude that in children with severe chronic constipation the colonic results of manometry differentiate patients with functional fecal retention from those with neuropathy or myopathy of the colon.

Colonic motility after surgery for Hirschsprung's disease.

OBJECTIVE:Years after surgery for Hirschsprungs 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 Hirschsprungs 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 Hirschsprungs 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 Hirschsprungs disease.

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.

Colonic manometry in children with chronic intestinal pseudo-obstruction.

Pressure changes were evaluated in the transverse, descending, and rectosigmoid colon of 30 children with chronic intestinal pseudo-obstruction. Twenty two had severe lifelong constipation and eight had symptoms suggesting a motility disorder exclusively of the upper gastrointestinal tract. Based on prior antroduodenal manometry, 24 children were diagnosed as having a neuropathic and six a myopathic form of intestinal pseudo-obstruction. On the day of study, endoscopy was used to place a manometry catheter into the transverse colon and intraluminal pressure was recorded for more than four hours. After a baseline recording, we gave a meal to assess the gastrocolonic response. Colonic contractions were noted in 24 children. The six children with no colonic contractions had a hollow visceral myopathy and constipation. In the children with colonic contractions, fasting motility did not differentiate children with and without constipation. After the meal, in all eight children without constipation there was (1) an increase in motility index (3.2 (SEM 0.3) mm Hg/min basal v 8.4 (SEM 1.1) mm Hg/min postprandial; p < 0.001), and (2) at least one high amplitude propagated contraction (HAPC). In the 16 constipated children with colonic contractions the motility index did not significantly increase after the meal (2.1 (SEM 0.3) mm Hg/min basal v 3.1 (SEM 0.4) mm Hg/min postprandial) and 12 of them had no HAPCs (p < 0.01 v group without constipation). In summary, in children with a clinical diagnosis of chronic intestinal pseudo-obstruction, constipation is associated with absence of HAPCs, and the gastrocolonic response or with total absence of colonic contractions. It is concluded that studies of colonic manometry are feasible in children and may document discrete abnormalities in those with intestinal pseudo-obstruction with colonic involvement.

Chronic intestinal pseudoobstruction associated with fetal alcohol syndrome

Alcohol acts as a teratogen in the fetus,resulting in prenatal or postnatal growth failure,characteristic facial dysmorphic features, and centralnervous system dysfunction. The toxic effects of alcohol on the developing brain are well recognized,but gastrointestinal neuropathy has not been describedin fetal alcohol syndrome (FAS). Five children with FASpresented in infancy with signs and symptoms suggestive of chronic intestinal pseudoobstruction. Theywere not able to sustain adequate caloric intake bymouth, and all required prolonged special methods ofalimentation. We performed antroduodenal manometry in these children to determine whether theirsymptoms were associated with a gastrointestinalmotility disorder. All patients had abnormallypropagating phase III-like episodes during fasting(retrograde in four, simultaneous in two). Persistentclusters of stationary contractions were a prominentfeature in two patients. In utero neurotoxicity ofalcohol may not be limited to the central nervoussystem, but may also cause an enteric neuropathypresenting in infancy as chronic intestinalpseudoobstruction.

Is Electrogastrography a Substitute for Manometric Studies in Children with Functional Gastrointestinal Disorders

We performed simultaneous fasting and fedantroduodenal manometry and EGG in 25 children withfunctional bowel disorders. Three patients (12%) had anuninterpretable EGG. The manometric studies showedsevere neuropathy in six patients; milder neuropathicchanges in five patients; postprandial hypomotility inone patient; myopathy in four patients, and normalmotility in the remaining six patients. The percentage of tachygastria time (frequency >3.5cycles/min) was higher in the patients 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 thepercentage of tachygastria and total arrhythmia timeamong the different study groups. The ratio of post- to preprandial power was significantly higher (2.5± 0.07) in children with normal motility than inthe other patients groups. Every child with totalarrhythmia time <35% and a ratio of post-topreprandial power >2.4 had normal manometry. In summary,EGG differentiated groups of children with normalmanometry from others with neuropathic or myopathicchanges, but in a minority of patients the study was not interpretable and there was overlap in EGGresults between children with normal and abnormalmanometry.

Antroduodenal Manometry in Children with No Upper Gastrointestinal Symptoms

BACKGROUND The interpretation of antroduodenal manometry in children with gastrointestinal motility disorder has been limited by a paucity of data from normal children. The purpose of this study was to define antroduodenal manometry findings in children with no upper gastrointestinal symptoms. METHODS We reviewed 260 consecutive antroduodenal manometries and selected 18 studies from subjects aged 2-12 years with no symptoms referable to a gastrointestinal motility disorder involving the stomach and/or the small bowel. RESULTS During fasting, phase 3 of the migrating motor complex (MMC) was present in 14 of 18 children, and it was induced by erythromycin in 4 who failed to have a spontaneous phase 3. Phase 3 propagation velocity increased significantly with age. The cycle length between MMCs showed no age-dependent variation. Phase 3 occupied 3%; phase 1, 10%; and phase 2, 87% of the fasting recording time. CONCLUSIONS Antroduodenal manometry findings in children with no upper gastrointestinal symptoms are similar to those in adults.

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.

Pediatric Functional Gastrointestinal Disorders.

Opinion statementMany of the childhood functional gastrointestinal disorders are extremely common.Using symptom-based diagnostic criteria for pediatric functional gastrointestinal disorders will improve patient care, enhance family satisfaction, and reduce costs.Using symptom-based diagnoses, the emphasis shifts from evaluations to rule out rare diseases to family education and symptom management.Well-meaning clinicians may co-create disability by failure to recognize and appropriately manage functional pediatric gastrointestinal disorders.