Clinical, Radiologic, and Manometric Characteristics of Chronic Intestinal Dysmotility: The Stanford Experience
2006; Elsevier BV; Volume: 4; Issue: 7 Linguagem: Inglês
10.1016/j.cgh.2006.05.001
ISSN1542-7714
AutoresLucilene Rosa-e-Silva, Lauren B. Gerson, Marta Davila, George Triadafilopoulos,
Tópico(s)Gastroesophageal reflux and treatments
ResumoBackground & Aims: The clinical spectrum of chronic intestinal dysmotility (CID) is not well known. We determined the spectrum of motor abnormalities, underlying pathology, clinical course, and response to treatment of adults with CID at a tertiary referral center. Methods: This was a descriptive retrospective analysis of a CID cohort conducted at a tertiary referral gastrointestinal (GI) motility center. A total of 113 referred patients underwent gastroduodenal manometry, other motility studies as appropriate, and radiologic and/or endoscopic assessment to exclude mechanical intestinal obstruction. Results: Common symptoms included abdominal distention, abdominal pain, nausea, and constipation. The course was chronic with intermittent symptoms. Gastroduodenal manometry was abnormal in all patients; a pattern suggestive of a neuropathic process was the most common. Other GI motility studies showed delayed gastric, gallbladder, and colonic transit, nonspecific esophageal dysmotility, sphincter of Oddi hypertonicity, and poor rectal balloon sensation/expulsion. Treatment involved nutritional support, prokinetics, analgesics, antinausea agents, and laxatives, with variable response and high morbidity, multiple emergency admissions, need for nutritional support, and poor response to surgery. Nearly 40% of the patients underwent abdominal surgery. Conclusions: Patients with CID have a chronic course and high morbidity. Because any segment of the GI tract may be involved in CID, functional assessment of the entire GI tract is recommended. CID presents several unmet clinical needs even in tertiary centers with expertise. Background & Aims: The clinical spectrum of chronic intestinal dysmotility (CID) is not well known. We determined the spectrum of motor abnormalities, underlying pathology, clinical course, and response to treatment of adults with CID at a tertiary referral center. Methods: This was a descriptive retrospective analysis of a CID cohort conducted at a tertiary referral gastrointestinal (GI) motility center. A total of 113 referred patients underwent gastroduodenal manometry, other motility studies as appropriate, and radiologic and/or endoscopic assessment to exclude mechanical intestinal obstruction. Results: Common symptoms included abdominal distention, abdominal pain, nausea, and constipation. The course was chronic with intermittent symptoms. Gastroduodenal manometry was abnormal in all patients; a pattern suggestive of a neuropathic process was the most common. Other GI motility studies showed delayed gastric, gallbladder, and colonic transit, nonspecific esophageal dysmotility, sphincter of Oddi hypertonicity, and poor rectal balloon sensation/expulsion. Treatment involved nutritional support, prokinetics, analgesics, antinausea agents, and laxatives, with variable response and high morbidity, multiple emergency admissions, need for nutritional support, and poor response to surgery. Nearly 40% of the patients underwent abdominal surgery. Conclusions: Patients with CID have a chronic course and high morbidity. Because any segment of the GI tract may be involved in CID, functional assessment of the entire GI tract is recommended. CID presents several unmet clinical needs even in tertiary centers with expertise. Chronic intestinal dysmotility (CID) is a clinical syndrome characterized by recurrent symptoms and signs of bowel obstruction in the absence of a mechanical occlusion but in the absence of continual bowel dilatation. Although the spectrum of this condition in children has been reported,1Heneyke S. Smith V.V. Spitz L. et al.Chronic intestinal pseudo-obstruction treatment and long-term follow up of 44 patients.Arch Dis Child. 1999; 81: 21-27Crossref PubMed Scopus (122) Google Scholar, 2Fell J.M.E. Smith V.V. Milla P.J. Infantile chronic idiopathic intestinal pseudo-obstruction the role of small intestinal manometry as a diagnostic tool and prognostic indicator.Gut. 1996; 39: 306-311Crossref PubMed Scopus (62) Google Scholar, 3Lapointe S.P. Rivet C. Goulet O. et al.Urological manifestations associated with chronic intestinal pseudo-obstruction in children.J Urol. 2002; 168: 1768-1770Abstract Full Text Full Text PDF PubMed Google Scholar, 4Mousa H. Hyman P.E. Cocjin J. et al.Long-term outcome of congenital intestinal pseudo-obstruction.Dig Dis Sci. 2002; 47: 2298-2305Crossref PubMed Scopus (88) Google Scholar the clinical spectrum of adult CID is not well known.5Mayer E.A. Schuffler M.D. Rotter J.I. et al.Familial visceral neuropathy with autosomal dominant transmission.Gastroenterology. 1986; 91: 1528-1535PubMed Google Scholar, 6Camilleri M. Carbone L. Schuffler M.D. Familial enteric neuropathy with pseudo-obstruction.Dig Dis Sci. 1991; 36: 1168-1171Crossref PubMed Scopus (20) Google Scholar, 7Jones S.C. Dixon M.F. Lintott D.J. et al.Familial visceral myopathy a family with involvement of four generations.Dig Dis Sci. 1992; 37: 464-469Crossref PubMed Scopus (25) Google Scholar, 8Schuffler M.D. Pope C.E. Studies of idiopathic intestinal pseudoobstruction. Hereditary hollow visceral myopathy: family studies.Gastroenterology. 1977; 73: 339-344PubMed Google Scholar, 9Schuffler M.D. Rohrmann C.A. Chaffee R.G. et al.Chronic intestinal pseudo-obstruction a report of 27 cases and review of the literature.Medicine. 1981; 60: 173-196Crossref PubMed Scopus (173) Google Scholar, 10Mann S.D. Debinski H.S. Kamm M.A. Clinical characteristics of chronic idiopathic intestinal pseudo-obstruction in adults.Gut. 1997; 41: 675-681Crossref PubMed Scopus (169) Google Scholar, 11Stanghellini V. Camilleri M. Malagelada J.R. Chronic idiopathic intestinal pseudo-obstruction clinical and intestinal manometric findings.Gut. 1987; 28: 5-12Crossref PubMed Scopus (275) Google Scholar, 12Silk D.B.A. Chronic idiopathic intestinal pseudo-obstruction the need for a multidisciplinary approach to management.Proc Nutr Soc. 2004; 63: 473-480Crossref PubMed Scopus (19) Google Scholar, 13Stanghellini V. Cogliandro R.F. De Giorgio R. et al.Natural history of chronic idiopathic intestinal pseudo-obstruction in adults a single-center study.Clin Gastroenterol Hepatol. 2005; 3: 449-458Abstract Full Text Full Text PDF PubMed Scopus (146) Google Scholar, 14Camilleri M. Balm R.K. Zinsmeister A.R. Determinants of response to a prokinetic agent in neuropathic chronic intestinal motility disorder.Gastroenterology. 1994; 106: 916-923PubMed Google Scholar, 15Camilleri M. Balm R.K. Zinsmeister A.R. Symptomatic improvement with one-year cisapride treatment in neuropathic chronic intestinal dysmotility.Aliment Pharmacol Ther. 1996; 10: 403-409Crossref PubMed Scopus (21) Google Scholar, 16Camilleri M. Appraisal of medium- and long-term treatment of gastroparesis and chronic intestinal dysmotility.Am J Gastroenterol. 1994; 89: 1769-1774PubMed Google Scholar A single center study from Italy presented data on the natural history of chronic intestinal pseudo-obstruction,13Stanghellini V. Cogliandro R.F. De Giorgio R. et al.Natural history of chronic idiopathic intestinal pseudo-obstruction in adults a single-center study.Clin Gastroenterol Hepatol. 2005; 3: 449-458Abstract Full Text Full Text PDF PubMed Scopus (146) Google Scholar including the need for total parenteral nutrition, surgery, and transplantation. The present study investigated the manifestations and management of CID in a US center.CID can be classified as either a primary (idiopathic) or a secondary disorder, resulting from conditions that affect the enteric neuromusculature, such as scleroderma, diabetes mellitus, amyloidosis, and others.14Camilleri M. Balm R.K. Zinsmeister A.R. Determinants of response to a prokinetic agent in neuropathic chronic intestinal motility disorder.Gastroenterology. 1994; 106: 916-923PubMed Google Scholar, 15Camilleri M. Balm R.K. Zinsmeister A.R. Symptomatic improvement with one-year cisapride treatment in neuropathic chronic intestinal dysmotility.Aliment Pharmacol Ther. 1996; 10: 403-409Crossref PubMed Scopus (21) Google Scholar, 16Camilleri M. Appraisal of medium- and long-term treatment of gastroparesis and chronic intestinal dysmotility.Am J Gastroenterol. 1994; 89: 1769-1774PubMed Google Scholar CID may involve either the entire gastrointestinal (GI) tract or isolated segments to a variable extent and magnitude.17Anuras J. Anuras S. Pseudo-obstruction syndromes.in: Anuras S. Motility disorders of the gastrointestinal tract. Raven, New York1992: 327-344Google Scholar, 18Schuffler M.D. Baird W. Fleming C.R. et al.Intestinal pseudo-obstruction as the presenting manifestation of small cell carcinoma of the lung.Ann Int Med. 1983; 98: 129-134Crossref PubMed Scopus (151) Google Scholar, 19Smith D.S. Williams C.S. Ferris C.D. Diagnosis and treatment of chronic gastroparesis and chronic intestinal pseudo-obstruction.Gastroenterol Clin North Am. 2003; 32: 619-658Abstract Full Text Full Text PDF PubMed Scopus (64) Google Scholar Gastroduodenal manometry is useful in CID11Stanghellini V. Camilleri M. Malagelada J.R. Chronic idiopathic intestinal pseudo-obstruction clinical and intestinal manometric findings.Gut. 1987; 28: 5-12Crossref PubMed Scopus (275) Google Scholar, 20Frank J.W. Sarr M.G. Camilleri M. Use of gastroduodenal manometry to differentiate mechanical and functional intestinal obstruction an analysis of clinical outcome.Am J Gastroenterol. 1994; 89: 339-344PubMed Google Scholar, 21Summers R.W. Anuras S. Green J. Jejunal manometry patterns in health, partial intestinal obstruction, and pseudo-obstruction.Gastroenterology. 1983; 85: 1290-1300PubMed Scopus (197) Google Scholar, 22Stanghellini V. Corinaldesi R. Ghidini C. et al.Reversibility of gastrointestinal motor abnormalities in chronic intestinal pseudo-obstruction.Hepatogastroenterology. 1992; 39: 34-38PubMed Google Scholar, 23Camilleri M. Jejunal manometry in distal subacute mechanical obstruction significance of prolonged simultaneous contractions.Gut. 1989; 30: 468-475Crossref PubMed Scopus (74) Google Scholar, 24Camilleri M. Study of human gastroduodenojejunal motility applied physiology in clinical practice.Dig Dis Sci. 1993; 38: 785-794Crossref PubMed Scopus (82) Google Scholar by showing 1 or more abnormalities in small bowel motor motility, such as absent phase III of the migrating motor complex (MMC), postprandial low-amplitude contractions, bursts of sustained uncoordinated phasic activity, and clusters of contractions. Additional examinations, including esophageal, anorectal, and sphincter of Oddi manometry as well as gastric and gallbladder scintigraphy and colonic transit time, can be performed to assess the extent of GI tract involvement in this disorder.Our aim was to assess the spectrum, underlying pathologies, clinical course, and response to treatment of a large cohort of CID adults evaluated at a US tertiary referral motility center.Materials and MethodsPatientsWe reviewed the medical records of 113 patients with the diagnosis of CID. All patients had recurrent symptoms suggestive of bowel obstruction in the absence of a mechanical occlusion. The manometric criteria for the diagnosis of CID as originally proposed by the Mayo Clinic11Stanghellini V. Camilleri M. Malagelada J.R. Chronic idiopathic intestinal pseudo-obstruction clinical and intestinal manometric findings.Gut. 1987; 28: 5-12Crossref PubMed Scopus (275) Google Scholar (see below) were fulfilled by all 113 patients (19 male, 94 female; median age, 43 years [range, 18–80 years]). These patients were evaluated and managed at the Stanford Hospital GI Motility Center between 1999 and 2004. The study was approved by Stanford University's Institutional Review Board for Human Subjects Research.CID was diagnosed by a combination of clinical, radiologic, surgical, and motility examinations,11Stanghellini V. Camilleri M. Malagelada J.R. Chronic idiopathic intestinal pseudo-obstruction clinical and intestinal manometric findings.Gut. 1987; 28: 5-12Crossref PubMed Scopus (275) Google Scholar, 12Silk D.B.A. Chronic idiopathic intestinal pseudo-obstruction the need for a multidisciplinary approach to management.Proc Nutr Soc. 2004; 63: 473-480Crossref PubMed Scopus (19) Google Scholar, 25Murr M.M. Sarr M.G. Camilleri M. The surgeon's role in the treatment of chronic intestinal pseudo-obstruction.Am J Gastroenterol. 1995; 90: 2147-2151PubMed Google Scholar, 26De Giorgio R. Sarnelli G. Corinaldesi R. et al.Advances in our understanding of the pathology of chronic intestinal pseudo-obstruction.Gut. 2004; 53: 1549-1552Crossref PubMed Scopus (191) Google Scholar with symptoms suggestive of subobstruction and manometric abnormalities required for entry into this series. Plain or contrast radiography showing dilatation of all or part of the small intestine in the absence of demonstrable mechanical obstruction, air–fluid levels, impaired gastric emptying, and markedly decreased small intestinal transit time were considered consistent with the diagnosis of CID.Data retrieved from the patients' medical records included demographics, age at symptom onset, family history, underlying pathologies, presenting symptoms, the presence of any extraintestinal manifestations, results of diagnostic examinations (plain/contrast radiography, abdominal ultrasound, abdominal computed tomography [CT], and endoscopy), and previous therapeutic interventions (including nutritional, pharmacologic, surgical, and endoscopic).Assessment of Symptoms and Response to TherapyWe semiquantitatively assessed our patients' symptoms in terms of frequency and severity, using a GI questionnaire that was previously used and validated in our center.27Lin M. Gerson L.B. Lascar R. et al.Features of gastroesophageal reflux disease in women.Am J Gastroenterol. 2004; 99: 1442-1447Crossref PubMed Scopus (71) Google Scholar According to this scoring system, symptom frequency and severity range from 0 to 3, with 0 representing no occurrence, 1 representing mild symptoms < 50% of the time, 2 representing moderate symptoms 50%–75% of the time, and 3 representing severe symptoms 75%–100% of the time. We report the median score for each symptom in our patients.In addition, the patients were classified as either "responders" or "nonresponders" to therapy. Responders were classified as "good" if they experienced significant relief of their symptoms and continued therapy over multiple visits throughout their follow-up care, or as "partial" if they initially responded to therapy but on subsequent assessment had discontinued therapy. Nonresponders were those who experienced no relief of symptoms early after initiation of therapy.Gastroduodenal Motility StudiesAll patients underwent gastroduodenal manometry after an overnight fast. This study was carried out using an 8-lumen perfusion catheter introduced using combined endoscopic and fluoroscopic guidance. Briefly, a proximal enteroscopy was performed, and a super-stiff guide wire (Boston Scientific, Natick, MA) was placed into the proximal jejunum. The endoscope was then removed, and, under fluoroscopy, the motility catheter was thread over the guide wire to reach beyond the ligament of Treitz. The catheter extruding from the patient's mouth was perfused with water at a rate of .1 mL/min by a pneumohydraulic pump and then connected to a transducer (Medtronic, Minneapolis, MN), which recorded pressure profiles from the distal antrum, pylorus, and the duodenal C-loop up to the ligament of Treitz. Pressure activity was recorded continuously in each patient for 3 hours before (fasting period) and 2 hours after (fed period) ingestion of a mixed solid–liquid meal.28Thumshirm M. Bruninga K. Camilleri M. Simplifying the evaluation of postprandial antral motor function in patients with suspected gastroparesis.Am J Gastroenterol. 1997; 92: 496-500Google ScholarTracing analysis was performed visually and semiquantitatively. The mean fasting cycle duration and the site initiation of phase III were determined. The presence of abnormal patterns was identified as follows, consistent with several studies on manometry of neuropathic and myopathic dysmotility reported in the literature9Schuffler M.D. Rohrmann C.A. Chaffee R.G. et al.Chronic intestinal pseudo-obstruction a report of 27 cases and review of the literature.Medicine. 1981; 60: 173-196Crossref PubMed Scopus (173) Google Scholar, 11Stanghellini V. Camilleri M. Malagelada J.R. Chronic idiopathic intestinal pseudo-obstruction clinical and intestinal manometric findings.Gut. 1987; 28: 5-12Crossref PubMed Scopus (275) Google Scholar, 20Frank J.W. Sarr M.G. Camilleri M. Use of gastroduodenal manometry to differentiate mechanical and functional intestinal obstruction an analysis of clinical outcome.Am J Gastroenterol. 1994; 89: 339-344PubMed Google Scholar: •Fasting state, consisting of (1) bursts of phasic activity of abnormal duration (> 2 minutes), amplitude (> 20 mm Hg), and frequency (10–12/minute) that are nonpropagating and distinct from phase III; (2) sustained (> 30 minutes) poorly coordinated phasic activity, isolated to 1 or more segments of the intestine; (3) low-amplitude contractions (typically < 10 mm Hg in the small bowel); (4) lack of propagation, as well as incomplete or retrograde propagation of phase III complexes covering a distance of at least 30 cm; (5) prolonged (> 3 minutes) increase in basal tone (> 30 mm Hg) during phase III activity.•Fed state, consisting of (1) persistent fasting pattern after a meal; (2) low-amplitude waves in the antrum (< 40 mm Hg)28Thumshirm M. Bruninga K. Camilleri M. Simplifying the evaluation of postprandial antral motor function in patients with suspected gastroparesis.Am J Gastroenterol. 1997; 92: 496-500Google Scholar and small bowel (< 10 mm Hg)28Thumshirm M. Bruninga K. Camilleri M. Simplifying the evaluation of postprandial antral motor function in patients with suspected gastroparesis.Am J Gastroenterol. 1997; 92: 496-500Google Scholar; (3) bursts of nonpropagating phasic contractions; (4) premature return of phase III within 90 minutes after a meal; (5) broad-based clusters of contractions occurring in the presence of increased tone (minute contractions).Low contractility during fasting (phases II and III) and postprandial was considered suggestive of myopathy.28Thumshirm M. Bruninga K. Camilleri M. Simplifying the evaluation of postprandial antral motor function in patients with suspected gastroparesis.Am J Gastroenterol. 1997; 92: 496-500Google Scholar The following abnormalities were considered suggestive of neuropathy: •Enteric neuropathy, marked by abnormal configuration or absent propagation of the MMC phase III and sustained, poorly coordinated phasic activity.•Central nervous system–enteric nervous system dysregulation, marked by altered MMC periodicity, lack of postprandial pattern, and clusters of contractions, as suggested in a consensus document from experts in the field.29Quigley E.M. Deprez P.H. Hellstrom P. et al.Ambulatory intestinal manometry a consensus report on its clinical role.Dig Dis Sci. 1997; 42: 2395-2400Crossref PubMed Scopus (47) Google ScholarOther Motility TestsDepending on patients' symptoms, additional studies were performed to detect the extent of the GI tract involvement. Such studies included esophageal manometry (in 35 patients), gastric scintigraphy (in 70 patients), gallbladder scintigraphy (in 13 patients), sphincter of Oddi manometry (in 6 patients), colonic transit time (in 36 patients), and anorectal manometry (in 22 patients). Conventional methods, as reported in the literature, were used for all such studies.30Castell J.A. Esophageal manometry.in: Castell D.O. The esophagus. Little, Brown, Boston1995: 133-152Google Scholar, 31Charles F. Camilleri M. Phillips S.F. et al.Scintigraphy of the whole gut clinical evaluation of transit disorders.Mayo Clin Proc. 1995; 70: 113-118Abstract Full Text Full Text PDF PubMed Scopus (90) Google Scholar, 32Geenen J.E. Hogan W.J. Dodds W.J. Intraluminal pressure recordings from human sphincter of Oddi.Gastroenterology. 1980; 78: 317PubMed Google Scholar, 33Loening-Baucke VColonic transit studies, colonic and anorectal motility studies, and tests to evaluate defecation and incontinence.in: Anuras S. Motility disorders of the gastrointestinal tract. Raven, Now York1992: 125-156Google ScholarResultsClinical PresentationThe median age of symptom onset was 37 years (range, 8–77 years). Figure 1 shows the distribution of GI symptoms at presentation. The median scores for each symptom were 3 for abdominal distention, abdominal pain, and bloating; 2 for nausea and constipation; 1 for vomiting, diarrhea, and heartburn; and 0 for dysphagia. Systemic or non-GI symptoms included weight loss (in 64%), fatigue (in 12%), arthralgia (in 11%), orthostatic hypotension (in 10%), weakness (in 10%), myalgia (in 10%), urinary incontinence (in 7%), and peripheral dysesthesias (in 6%). Eleven patients (10%) had a family history of GI motility disorders, and 13 patients (12%) had a family history of colon cancer.Past Medical HistoryEighty-four patients had no underlying disease (idiopathic CID), whereas 29 had a systemic disorder (secondary CID), including 17 cases of hypothyroidism and 12 cases of mixed connective tissue disease. There was a high incidence of affective disorders (50%), with depression the most common (in 31 patients). A history of gynecologic surgery was present in 54 patients (48%), with hysterectomy the most common procedure (in 35 patients). Other conditions included previous GI surgery (in 35%), gastroesophageal reflux disease (in 29%), allergies (in 27%), and urologic tract disorders (in 17%). This latter group consisted of 14 patients with recurrent urinary tract infections, 2 patients with uretero-pelvic-calyceal dilatation by CT or ultrasound imaging, and 3 patients with neurogenic bladder exhibiting delayed emptying. Ten of these 19 patients had 1 urologic abnormality, and 9 had more than 1 abnormality. Twenty-eight patients (25%) had multiple emergency room visits because of CID symptoms or dehydration. Eleven patients (10%) had symptomatic orthostatic hypotension, and 7 patients (6%) had peripheral dysesthesias. Two patients in this group had autonomic neuropathy confirmed by formal testing.Radiologic StudiesEleven patients (10%) exhibited abnormal findings consistent with CID on plain abdominal radiographic studies. Twenty-six other patients (23%) had abnormal findings consistent with CID on contrast radiography, ultrasound, or CT that revealed gastroparesis and/or delayed small bowel transit in 20 patients and small bowel dilation in 6. All of the remaining 76 patients (67%) had normal plain abdominal radiographs and at least 2 abnormal motility tests, including gastroduodenal manometry. Twenty-six of these 76 patients (34%) experienced severe symptoms suggestive of subacute or chronic obstruction and underwent abdominal surgery that revealed no mechanical obstruction.Gastroduodenal Motility StudiesNeuropathy alone was present in the majority (87) of the 113 patients (Table 1). Neuropathy was also noted in conjunction with features suggestive of partial obstruction (minute contractions) in 11 patients. In 9 patients, the amplitude was borderline and may have suggested a myopathic disorder. Myopathy alone was reported in the remaining 6 patients. The cycle duration of MMC (requiring 2 MMC phase III episodes in the 3-hour fasting period) was determined in only 55 of the 113 patients (49%). The median duration was 60 minutes (range, 10–190 minutes). In 33 patients (30%), no phase III episode was present, whereas in the remaining 25 (21%), only 1 phase III episode was identified. The site of phase III initiation was assessed in 75 patients; 70 (93%) had gastric initiation, and 5 (7%) had duodenal initiation. Table 2 reports the prevalence of the gastroduodenal manometric abnormalities observed.Table 1Gastroduodenal Manometry Reports in 113 Adult Patients With CIDGastroduodenal manometry suggestive ofNo. of patientsPercentage of totalNeuropathy without myopathy or partial small bowel obstruction8777%Neuropathy with some evidence of partial small bowel obstruction (nonpropagated clusters)1110%Neuropathy with borderline amplitude98%Myopathy without evidence of neuropathy or partial small bowel obstruction65% Open table in a new tab Table 2Gastroduodenal Manometric Abnormalities in Patients With CIDGastroduodenal manometric abnormalitiesPrevalenceBursts of uncoordinated phasic activity Fasting51% Postprandial38%Lack of postprandial pattern35%Low contractility Fasting13% Postprandial32%Abnormal duration and/or propagation of phase III31%Lack of propagation, incomplete or retrograde propagation of phase III of the MMC30%Premature return of phase III after meal23%Clusters of contractions of longer than 30 minutes22%Increased tone during phase III19%Nonpropagated clusters (minute) contractions10% Open table in a new tab Distribution of the Gastroduodenal Manometric AbnormalitiesAll patients exhibited at least 1 of the 10 abnormal patterns described in the Methods section. Ninety-six patients (85%) had at least 2 abnormal patterns, 68 (60%) had at least 3, 40 (35%) had at least 4, 22 (20%) had at least 5, 8 (7%) had at least 6, and 3 (2.6%) had 7 abnormal patterns.Other Motility TestsEsophageal manometryOf the 35 patients who underwent esophageal motility evaluation, 7 (20%) had a normal pattern. In the remaining 28 patients, nonspecific esophageal motility disorder was the most common abnormality, seen in 19 patients (68%), followed by low lower esophageal sphincter (LES) pressure in 15 (54%), "nutcracker" esophagus in 5 (18%), incomplete LES relaxation in 2 (7%), hypertensive LES in 2 (7%), and esophageal manometric features of scleroderma in 2 (7%).Gastric scintigraphyOf the 70 patients who underwent gastric emptying evaluation, 21 (30%) had a normal pattern. All but 1 of the remaining 49 patients exhibited delayed gastric emptying.Gallbladder scintigraphyThirteen patients underwent a gallbladder emptying evaluation, and 5 exhibited delayed gallbladder emptying.Sphincter of Oddi manometryIn all 6 patients who underwent this procedure, all had sphincter hypertonicity, 3 had tachy-Oddia, and 3 had high-amplitude, high-frequency, and long-duration phasic sphincter of Oddi contractions.Colonic transit timeOf the 36 patients who underwent colonic transit evaluation, 21 patients exhibited delayed transit time.Anorectal manometry and sensationOf the 22 patients who underwent this procedure, 2 (9%) exhibited a normal pattern. All but 1 of the remaining 20 patients had poor rectal balloon sensation and/or expulsion; 13 patients (65%) also had low internal anal and/or external anal sphincter profiles. Of note, these patients did not have an evacuation disorder as the main cause of their GI symptoms, but all had constipation.All of the 113 patients in the study group had at least 1 abnormal motility test, with 36 of them (32%) having gastroduodenal manometry as the only abnormal motility test. Seventy-seven patients (68%) had at least 2 abnormal motility tests, 36 (32%) had at least 3 abnormal tests, 10 (9%) had at least 4 abnormal tests, and 4 (3.5%) had 5 abnormal motility tests.NutritionTwenty-nine of the 113 patients (26%) required nutritional support; 14 of these received total parenteral nutrition (TPN) as the only means of nutritional support. Two patients received nasoenteral feedings, with 1 experiencing a good response and the other subsequently requiring TPN. Thirteen patients had a feeding gastrostomy or jejunostomy; 8 had a good response, whereas in the other 5 the feeding tube was removed and TPN initiated because of infection or tube obstruction. In total, 20 patients required TPN.Drug TherapyProkinetic agentsFigure 2 shows the proportion of patients who had good or partial response to prokinetic drugs, as well as those patients who experienced side effects. Patients receiving the 5-HT4 agonist tegaserod had the highest probability of response. Ten of 19 patients (53%) responded to tegaserod, with 5 reporting a good response. Domperidone and erythromycin had similar responses to treatment; 4 of 8 patients (50%) responded to domperidone, whereas 8 of 16 (50%) responded to erythromycin. For both drugs, the rate of partial response was high (3 of 4 patients for domperidone and 7 of 8 patients for erythromycin). Patients receiving cisapride, octreotide, and metoclopramide had lower responses to treatment. Seventeen of 46 patients (37%) responded to cisapride, with 10 of 17 exhibiting only partial response. Three of 11 patients (27%) responded, although only partially, to octreotide. Nine of 38 patients (24%) responded to metoclopramide, with 8 exhibiting partial response. Metoclopramide also had the highest proportion of drug-related side effects (18%).Figure 2Response to prokinetic therapy in adult patients with CID.View Large Image Figure ViewerDownload (PPT)Pain-control agentsMost patients receiving opiates, nonsteroidal anti-inflammatory drugs (NSAIDs), and antispasmodics responded to treatment. Twenty-six of 31 patients (84%) responded to opiates, with the majority (22) experiencing good symptom relief. Twenty-six of 36 patients (72%) responded to NSAIDs, with 17 having a good response. Nineteen of 29 patients (65%) responded to antispasmodics, with 11 of them experiencing good symptom relief. Only 8 of the 18 patients (44%) responded to tricyclic antidepressants, with 4 of them having a good response. Side effects were rare for all pain control drugs, occurring in only 2 of 31 patients for opiates, in 1 of 29 patients for antispasmodics, in 1 of 18 patients for antidepressants, and in none for NSAIDs.Antinausea agentsThe patients exhibit
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