Quantitative analysis of anorectal pressures in Hirschsprung's disease

Abstract

Anorectal motility was investigated in 146 children with Hirschsprung's disease and 89 normal control subjects. Pressures were recorded in the rectum and anal canal at rest and during rectal distention. The rectoanal inhibitory reflex was absent in all but four patients. Intraluminal rectal pressure was higher than normal (16.5 vs. 14.6 cm H2O, P less than 0.03), with more frequent (41 per cent vs. 18 per cent, P less than 0.01) pressure waves. In the upper anal canal, there were more frequent (62 per cent vs. 18 per cent, P less than 0.001) spontaneous variations of pressure of lower frequency (9.5 vs. 12.8 cycles/min P less than 0.001) and greater amplitude (5.2 vs. 3.6 cm H2O, P less than 0.001) than normal. The rectoanal contractile reflex occurred in 47 per cent of the patients but in only 21 per cent of the control subjects (P less than 0.001). Aganglionosis was associated with the presence of a rectoanal inhibitory reflex in three patients. This study confirms the value of anorectal manometry in diagnosing Hirschsprung's disease in a large group of patients, and demonstrates other abnormalities that may be useful in cases in which histologic and manometric data are in conflict.

Anal ultraslow waves and high anal pressure in childhood: a clinical condition mimicking Hirschsprung disease

“…We found that USWs were rare in Hirschsprung disease, and there were significant differences in the anal pressure and frequency of anal slow waves between patients with USWs and those with Hirschsprung disease. Our data on the low frequency of anal slow waves in Hirschsprung disease were compatible with the data reported by Faverdin et al [13]. Another clinical entity to be discussed is bultrashort Hirschsprung diseaseQ or internal anal sphincter achalasia [14].…”

Anorectal manovolumetry in the decision making before surgery for slow transit constipation

“…The frequency of SW differed between patients and control subjects. The median (range) frequency was 9 (0-12) per minute in patients and 12 (6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16)(17) in controls (p<0.001). Overall, 22 patients (44%) were outside the 5 th -95 th percentile of controls in at least one of these sphincter function parameters.…”

“…For example, absence of the recto-anal inhibitory reflex (RAIR) suggests Hirschsprung's disease [9]. Other anorectal pressure abnormalities have been described.…”

Benzalkonium chloride–treated anorectums mimicked endothelin-3–deficient aganglionic anorectums on manometry

“…Patients usually have increased anorectal resting pressure (ARP) and do not possess rectoanal inhibitory reflex on manometry [3]. The spastic contraction in the aganglionic segment can be attributed either to the lack of smooth muscle relaxants, including nitric oxide, vasoactive intestinal peptide, and carbon monoxide from nonadrenergic, noncholinergic neurons [4][5][6], or to the excessive activity of excitatory acetylcholine and substance P released from hypertrophic nerves [7,8].…”

“…Hirschsprung disease is characterized by aganglionosis in both the submucosal and myenteric plexuses secondary to the absence of the enteric nervous system, with concurrent hypertrophic nerve trunks thought to mainly originate from extrinsic, parasympathetic nerves [1,2]. Patients usually have increased anorectal resting pressure (ARP) and do not possess rectoanal inhibitory reflex on manometry [3]. The spastic contraction in the aganglionic segment can be attributed either to the lack of smooth muscle relaxants, including nitric oxide, vasoactive intestinal peptide, and carbon monoxide from nonadrenergic, noncholinergic neurons [4-6], or to the excessive activity of excitatory acetylcholine and substance P released from hypertrophic nerves [7,8].…”