188 Gut 1997; 40: 188-191 Oesophageal manometry in the evaluation of

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 .' ` 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 . 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- , 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). None of four open tipped recording catheters plus a this subgroup of patients had radiographic central lumen (inner diameter 1-8 mm) for dilatation of the oesophagus. Three of them inflation of a rubber rectal balloon with air. At reported oesophageal symptoms, , 60 ml of distension the mean (SD) pressure and regurgitation in two cases and chest pain inside the balloon was 91 (2) mm Hg and the in the other; none of the three had gross diameter was 41 (6) mm. Side openings were oesophagitis at endoscopy. Table I shows the spaced 1 cm apart and were oriented at angles results of oesophageal manometry in controls of 900. The probe was inserted with the and in patients subdivided according to impair- patients lying in the right lateral position, with ment of oesophageal motility. The amplitude hips flexed at 900. The side openings were of waves in the distal oesophagus was signifi- placed in the anal sphincter 0 5, 1-5, 2-5, and cantly reduced and their duration was signifi- 3-5 cm from the anal verge. The caudal edge cantly prolonged in patients with impaired 190 Basilisco, Velio, Bianchi

A R Of the nine patients with normal oeso- ;Ws tWs U Ws aWs phageal motility, five were constipated and one cm cm had a history of pesudo-obstruction. The from from Gut: first published as 10.1136/gut.40.2.188 on 1 February 1997. Downloaded from LOS LOS threshold volumes for the perception of dis- tension, desire to defecate, and rectoanal in- 18 18 hibitory reflex were greater than in healthy subjects, in particular in the three patients with megarectum. 13 13 Discussion Our study showed that five of 14 consecutive 10s 8 los 8 patients with idiopathic longstanding mega- 40 mm Hg 40 mm Hg colon with onset in adult life had disease involving the oesophagus. In four of them, low 3 3 amplitude multiple peaked simultaneous con- tractions were recorded in the distal oeso- Oesophageal manometry in two patients with megacolon, one (A) with simultaneous low amplitude multiple peaked contractions after wet swallows (WS) and one (B) without any phagus and contractions were undetectable in contraction (LOS=lower oesophageal sphincter). the fifth. The severe oesophageal motor impairment cannot be considered a variation of normal. In fact, simultaneous low amplitude TABLE I Oesophageal motility in controls andpatients with megacolon subdivided according to the presence or absence ofperistaltic contractions in the oesophagus (data are multiple peaked contractions were never mean (SD)) observed in our healthy subjects after 10 wet swallows and only in four subjects after one wet Controls Patients swallow of a series of 95 healthy subjects Oesophagealperistalsis Present Present Absent studied with a similar technique.8 Moreover, Subjects (n) 15 8 5 the absence of detectable contractions in the LOS resting pressure (mm Hg) 17 (15) 27 (11) 23 (11) distal oesophagus has not been reported in Absent LOS relaxation (no) 0 0 3* Distal amplitude (mm Hg) 83 (30) 86 (38) 27 (22)** healthy subjects.8 Acute gut distension inhibits Distal duration (s) 3-3 (0-2) 3-9 (0-3) 5-4t (0-3)** the motility of the other tracts of the intestine by reflex relaxation.'0 However, this mechan- *p<005 v controls; **p<0-01 v controls and patients with normal oesophageal motility; ism can hardly be the cause of the severe tmeasured in four patients. impairment of oesophageal motility found in our five patients as colonic distension was TABLE II Anorectal motility in control subjects andpatients with megacolon subdivided according to the presence or absence ofperistaltic contractions in the oesophagus (data are chronic and as in the remaining nine patients http://gut.bmj.com/ mean (SD)) oesophageal motility was entirely normal despite colonic dilatation. Controls Patients Simultaneous contractions occur in patients Oesophageal peristalsis Present Present Absent with diffuse oesophageal spasm and in Subjects (n) 11 8 5 achalasia. " However, even ifthe normal resting Anal pressures (mm Hg) pressure of the LOS in our patients and the resting 79 (21) 78 (11) 62 (17) squeeze 200 (21) 185 (60) 146 (32) normal relaxation observed in two of them on September 25, 2021 by guest. Protected copyright. Rectal sensation threshold (ml) does not exclude a chance association with distension 12 (6) 34 (30) 22 (18) desire to defecate 44 (17) 60 (14) 54 (23) these diseases, '1-3 they make it more unlikely. Rectoanal inhibitory reflex Simultaneous oesophageal contractions and absent (no of subjects) 0 0 3* threshold volume (ml) 15 (12) 41 (26)* 35 (21) megacolon were described in patients with Chagas' disease.'4 A negative complement *p<0 05 v controls. fixation test for Trypanosoma cruzi might have been helpful to exclude this disease. In such peristalsis in comparison with controls and patients however the resting pressure of the patients with normal oesophageal motility. LOS is often raised; moreover in Europe LOS relaxation after swallows was absent in Trypanosoma cruzi is not endemic and Chagas' three patients with impaired oesophageal disease has been reported only in immuno- peristalsis (p<0.05 v controls), one of whom suppressed subjects.'5 had reflux symptoms and one chest pain. The absence of detectable contractions in All five patients with impaired oesophageal the distal oesophagus recalls the pattern of motility had a colonic dilatation sparing the motility observed in patients with visceral rectum and a variable part of the sigmoid. myopathy and megaduodenum'-3 or with end Three of them reported constipation and a stage systemic sclerosis.'6 None of our patients, history of pseudo-obstruction; the other two however, had a dilated duodenum or a family complained of abdominal distension but their history ofthe disease that characterises patients bowel frequency was normal. Table II shows with visceral myopathy and megaduodenum, -3 the results of anorectal manometry. Resting nor did any of them have Raynaud's phe- and squeeze pressures ofthe anal sphincter and nomenon or cutaneous changes, which rectal sensitivity to distension in patients with characterise patients with systemic sclerosis.'6 impaired oesophageal motility were not The absence of anorectal inhibitory reflex in different from those of controls. The reflex three patients may indicate Hirschsprung's relaxation of the internal anal sphincter was disease.'7 One of our patients had spontaneous impaired in the three patients in whom LOS daily evacuations, however, which is very un- relaxation was also impaired. common in adult patients with Hirschsprung's Oesophageal manometry in the evaluation ofmegacolon with onset in adult life 191

disease.'7 A higher frequency of oesophageal 1 Faulk DL, Anuras S, Gardner D, Mitros FA, Summers RW, Christensen J. A familial visceral myopathy. Ann Intern simultaneous contractions was observed in Med 1978; 89: 600-6. children with this disease,'8 suggesting a more 2 Schuffier MD, Rohrmann CA, Chaffee RG, Brand DL,

Delaney JH, Young JH. Chronic intestinal pseudo- Gut: first published as 10.1136/gut.40.2.188 on 1 February 1997. Downloaded from widespread neuropathy. Our results show that obstruction. A report of 27 cases and review of the in presence of a Hirschsprung's disease-like literature. Medicine 1981; 60: 173-96. 3 Rodrigues CA, Shepherd NA, Lennard-Jones JE, impairment of anorectal inhibitory reflex, Hawley PR, Thompson HH. Familial visceral myopathy: extracolonic impairment of gut motility is also a family with at least six involved members. Gut 1989; 30: 1285-92. possible. 4 Shaw A, Shaffer H, Teja K, Kelly T, Grogan E, Bruni C. Three of our five patients with impaired A perspective for pediatric surgeons: chronic idiopathic intestinal pseudoobstruction. J Pediatr Surg 1979; 14: oesophageal motility had constipation and 719-27. pseudo-obstruction. Both Watier et al'9 and 5 Eaves ER, Schmidt GT. Chronic idiopathic megaduo- denum in a family. Aust NZJ Med 1985; 15: 1-6. Reynolds et al20 pointed out that oesophageal 6 Lennard-Jones JE. Clinical features of idiopathic mega- manometry may be a simple test to detect a rectum and megacolon in adults. In: Kamm MA, Lennard-Jones JE, eds. Constipation Petersfield: generalised motor disorder in subjects with Wrightson Biomedical. 1994: 225-31. constipation. The latter authors found ab- 7 Preston DM, Lennard-Jones JE, Thomas B. Towards a radiologic definition of idiopathic megacolon. Gastrointest normal oesophageal motility in six of 25 patients Radiol 1985; 10: 167-9. (25%) with severe constipation, one of them 8 Richter JE, Wu WC, Johns DN, Blackwell JN, Nelson JL, Castell JA, et al. Oesophageal manometry in 95 healthy with diffusely dilated small and large bowel.20 adult volunteers. Variability of pressures with age and Moreover, oesophageal motility is often im- frequency of "abnormal" contractions. Dig Dis Sci 1987; 32: 583-92. paired in patients with pseudo-obstruction,2 a 9 Simon R. Confidence intervals for reporting results of clinical picture that may have many causes.2' clinical trials. Ann Intern Med 1986; 105: 429-35. 10 Basilisco G, Phillips SF. Ileal distention relaxes the canine Interestingly our study showed that the colon: a model of megacolon? Gastroenterology 1994; 106: presence of megacolon represents in itself a 606-14. 11 Vantrappen G, Janssens J, Hellemans J, Coremans G. hallmark of a motor disorder not limited to Achalasia, diffuse esophageal spasm and related motility the colon independently of associated symp- disorders. Gastroenterology 1979; 76: 450-7. 12 Katz PO, Richter JE, Cowan R, Castell DO. Apparent toms. complete lower esophageal sphincter relaxation in All our patients with oesophageal motor achalasia. Gastroenterology 1986; 90: 978-83. 13 Tokumine F, Muto Y, Okushima N, Kusano T, Nakaci A, impairment had localised megacolon sparing Yamazato M, et al. A rare case of achalasia coexistent with the rectum and a varying part of the sigmoid. sigmoid megacolon and associated with epilepsy. J7 Gastroenterol 1994; 29: 637-41. It has been speculated that localised megacolon 14 Ferreira-Santos R. Megacolon and megarectum in Chagas' could be explained by hindgut dysgenesis or by disease. Proc R Soc Med 1961; 54: 1047-53. 15 Villalba R, Fornes G, Alvarez MA, Roman J, Rubio V, ischaemia in the vascular territory of the inferior Fernandez M, et al. Acute Chagas' disease in a recipient mesenteric artery.22 Our results suggest a more of a bone marrow transplant in Spain: case report. Clin InfectDis 1992; 14: 594-5. widespread visceral neuropathy or myopathy, 16 Stevens MB, Hookman P, Siegel CI, Esterly JR, Shulman

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