Background Faecal incontinence is usually attributed to pelvic-floor d
enervation of striated muscle or direct sphincter trauma. We have iden
tified a cause of passive faecal incontinence related to degeneration
of the internal anal sphincter smooth muscle, in the absence of denerv
ation, structural damage, external-sphincter weakness, or sensory abno
rmalities. Methods Patients were included on the basis of: passive fae
cal incontinence, no urge faecal incontinence, low anal pressure whils
t at rest, normal anal-squeeze pressure, endosonographically confirmed
circumferentially intact internal and external anal sphincters, and n
ormal pudendal nerve terminal nerve latencies. In a second analysis do
ne to assess the proportion of patients with this disorder, we recorde
d the cause of incontinence in consecutive patients seen during a 6-mo
nth period. Findings 45 patients (35 women, median age 63 years, range
23-80 years) fulfilled the diagnostic criteria. Median duration of sy
mptoms was 2 years (3 months to 20 years). Nine of the 35 women were n
ulliparous. The median resting anal pressure was 40 cm water (16-56 cm
water, normal >60 cm water). Endosonography revealed an internal sphi
ncter that was thin and hyperechogenic, and had a poorly defined edge.
The normal increase in the thickness of the internal anal sphincter w
ith age was not seen, Anal-squeeze pressure, sensitivity, and pudendal
nerve latencies were normal. In the second analysis the condition was
identified in eight of 230 patients, representing 4% of new referrals
. Interpretation Primary degeneration of the internal anal sphincter s
mooth muscle is a discrete clinical condition causing passive faecal i
ncontinence.