188 Gut 1997; 40: 188-191 Oesophageal manometry in the evaluation of megacolon with onset in adult life Gut: first published as 10.1136/gut.40.2.188 on 1 February 1997. Downloaded from G Basilisco, P Velio, P A Bianchi Abstract them may be asymptomatic despite the Background-Oesophageal motility is enlarged duodenum.' ` Idiopathic megacolon often impaired in patients with megaduo- in adult age may manifest acutely or there may denum and other forms of intestinal be a long history of bowel disturbances. Some pseudo-obstruction in which a visceral patients with the latter have few symptoms, myopathy or neuropathy may be present. others complain of constipation, and a few with Idiopathic longstanding megacolon with pseudo-obstruction.6 The condition is still onset in adult life is still a poorly defined poorly defined but in some cases at least colonic entity, which may also be part of a more dilatation is caused by an underlying visceral widespread motility disorder but in which myopathy or neuropathy.3 6 In this study we oesophageal motility has not been yet explored the possibility that a systematic systematically studied. assessment of oesophageal motility might be Aim-To assess oesophageal motility in helpful to detect cases in which megacolon is patients with longstanding idiopathic a part of a more widespread motor disorder. We megacolon with onset in adult life. assessed oesophageal motility in a series of Patients-14 consecutive subjects with 14 consecutive patients with longstanding idiopathic megacolon whose symptoms idiopathic megacolon with onset in adult life. began after the age of 10 and a clinical Anorectal manometry was also performed in all history of2-22 years. patients. Results were compared with those Methods-Standard barium enema, water obtained in healthy subjects. perfused oesophageal manometry, and also anorectal manometry. Results-Oesophageal motility was im- Methods paired in five patients (36%; 95% con- fidence intervals 16 to 61%). Normal Patients http://gut.bmj.com/ peristalsis was substituted by low ampli- Fourteen consecutive patients (10 men, mean tude multiple peaked simultaneous con- age 38 years, range 16-69) with longstanding tractions in four subjects and by un- idiopathic megacolon were studied. The diag- detectable contractions in one. In three of nosis ofmegacolon was made from radiographs them the lower oesophageal sphincter did obtained during barium enema examination not relax after swallows; in the same using a standard double contrast technique. patients anal relaxation after rectal dis- Colonic width was over 9 cm in at least one on September 25, 2021 by guest. Protected copyright. tension was also undetectable. All five segment. Three patients had megarectum - patients with impaired oesophageal that is, rectal width of over 6 5 cm measured motility had a colonic dilatation sparing from lateral pelvic radiographs at the pelvic the rectum. Three of them reported con- brim.7 Symptoms, when present, began after stipation and a history of pesudo- the age of 10 years. The mean interval of time obstruction and the other two only from radiological diagnosis or symptoms onset abdominal distension. was nine years, range 2-22. All but two Conclusions-Oesophageal manometry patients complained of abdominal distension. should be performed in patients with long- Eight patients reported two or less bowel standing idiopathic megacolon with onset movements per week; four of them had a in adult life, in particular if the rectum is history of intestinal pseudo-obstruction. None not dilated and even in absence ofpseudo- of them experienced soiling. Two patients had obstruction. This simple test may disclose a partial colectomy for megacolon. Three Cattedra di a more widespread visceral neuropathy or reported oesophageal symptoms; reflux symp- Gastroenterologia, myopathy. Such a diagnosis helps to toms in two and chest pain in one. Istituto di Scienze better understand the cause ofthe colonic None of the patients had travelled in Mediche, UniversitA degli Studi di Milano, dilatation and may be clinically relevant countries where Chagas' disease is endemic or IRCCS-Ospedale for treatment ofthe patients. in east Africa. Neoplastic and collagen diseases Maggiore di Milano, (Gut 1997; 40: 188-191) and diseases of the central nervous system such Milan, Italy G Basilisco as myotonic dystrophy, parkinsonism, and P Velio Keywords: megacolon, oesophageal motility, multiple sclerosis were excluded as were spinal P A Bianchi pseudo-obstruction. cord lesions, diabetic visceral neuropathy, Correspondence to: hypothyroidism, hypoparathyroidism, and Dr G Basilisco, Departnent of amyloidosis. None of the patients had mega- Gastroenterology, Padiglione Oesophageal motility is impaired in patients duodenum or small bowel dilatation or took Granelli, Via F Sforza 35, 20122 Milan, Italy. with visceral myopathy and megaduo- drugs delaying gastrointestinal motility. were Accepted for publication denum.' Most of these patients present with Routine laboratory investigations un- 28 August 1996 intestinal pseudo-obstruction,1-3 but some of remarkable and, in particular, serum concen- Oesophageal manometry in the evaluation ofmegacolon with onset in adult life 189 trations of potassium and calcium were of the distending rectal balloon was 7 cm from normal. Two patients were mentally retarded; the anal verge. Anorectal motility was recorded one of them, a 28 year old woman, refused for 15 minutes in resting conditions. After the oesophageal manometry. resting period, subjects were asked to contract Gut: first published as 10.1136/gut.40.2.188 on 1 February 1997. Downloaded from the anal sphincter maximally. This was re- peated twice with a gap of one minute between Study design contractions. The rectal balloon was then Oesophageal and anorectal manometry were serially inflated with air to 10, 20, 30, 40, 50, performed after an overnight fast with multi- 60, and 70 ml using a hand held syringe. lumen catheters perfused with distilled water Inflations were performed rapidly (less than using a low compliance perfusion system two seconds), maintained for one minute, and (Arndorfer Specialties, Greendale, Wisconsin, separated by one minute intervals. Imme- USA) at a rate of 0.5 ml/min. Pressures were diately after the introduction of each new transmitted to external pressure transducers volume, the subject was asked whether he or and recorded on a computer. she felt the distension or the desire to defecate. Oesophageal manometry was performed The following measurements were recorded: according to Richter et al.8 An eight lumen (a) the resting pressure of the anal canal, as the polyvinyl tube (outer diameter 4-5 mm; inner mean pressure recorded in the recording diameter of each lumen 0-8 mm; Arndorfer channel with the highest pressure at the end of Specialties, Greendale, Wisconsin, USA) was the resting period; (b) the squeeze pressure, as introduced through the nose into the oeso- the mean maximal pressure in the recording phagus. With the distal four openings spaced channel with the highest pressure during 1 cm apart at 900 and radially oriented, lower squeeze; (c) the threshold volume of the rectal oesophageal sphincter (LOS) pressure was balloon eliciting the feeling of distension or the recorded by the station pull through technique desire to defecate; if the feeling of distension (0 5 cm increments/30 seconds). The tube was or the desire to defecate was not reached at the then positioned to record oesophageal motility maximal distending volume of 70 ml, this 3, 8, 13, 18, and 23 cm above the upper border volume was used for calculation purposes; (d) of the LOS. Water perfusion of the first two the volume eliciting the rectoanal inhibitory orad openings of the tube was stopped to avoid reflex, as the presence of a detectable reduction pharyngeal stimulation by water. For the in pressure of the anal canal after rectal assessment of oesophageal peristalsis the distension in at least one recording channel. subjects were asked to swallow 5 ml of water Anorectal manometry was also performed in 10 times at 30 second intervals. The mean 1 1 healthy subjects (nine men, mean age 38 station pull through pressure of the LOS was years, range 19-58). http://gut.bmj.com/ calculated as the average ofthe values obtained All patients with impaired oesophageal from the four radial openings, measured from motility underwent barium oesophagography the gastric baseline to the mid-point of and in the presence of oesophageal symptoms, respiratory variations. The amplitude and also endoscopy of the upper gastrointestinal duration of oesophageal contractions were tract. Results are given as mean (SD). Con- calculated as the average of the values obtained tinuous variables were compared by analysis of during the 10 wet swallows; the amplitude was variance followed by Tukey's test for multiple on September 25, 2021 by guest. Protected copyright. measured from the intraoesophageal baseline comparisons. Fisher's exact test was used to to the peak of the pressure wave and the compare frequency data. Ninety five per cent duration from the point where the upstroke of confidence intervals of percentages were also the contraction left the baseline to the point calculated.9 where the downstroke of the contraction returned to baseline. The individual values for the recording sites 3 cm and 8 cm above the Results LOS were averaged and considered as ampli- Oesophageal motility was normal in nine tude and duration of distal oesophageal con- patients whereas in four normal controls oeso- tractions.8 Oesophageal manometry was also phageal peristalsis was substituted by simul- performed in 15 healthy subjects (nine women, taneous low amplitude multiple peaked con- mean age 33 years, range 23-59). tractions and in one by the absence of Anorectal manometry was performed using detectable contractions (Figure). Thus oeso- a polyvinyl tube (outer diamter 4-8 mm; inner phageal peristalsis was impaired in five patients diameter of each lumen 0-8 mm; Arndorfer (36%; 95% confidence intervals 16 to 61%) Specialities, Greendale, Wisconsin, USA) with and in none of the controls (p<0.05).