Springer-Verlag 1998
REVIEW
Abstract Symptomatic colonic lipomas, although unusual, continue to present difficulties in the preoperative
differential diagnosis between malignant and benign colonic neoplasm. Although new imaging techniques are
available, they are frequently diagnosed at laparotomy, and
definitive histology is required. Local excision is adequate
treatment, but segmental excision may be necessary when
there is doubt about the diagnosis, or when a complication
occurs.
Key words Colonic lipoma Differential diagnosis
Excision
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Colon Rectum 34:10271029
Springer-Verlag 1998
REVIEW
Introduction
Associated tumours
Another feature seen in many of these families is the occurrence of adenocarcinomas at other sites, in particular
the endometrium, ovary, stomach, pancreas, ureter, renal
pelvis and skin [3 5].
Mecklin and Jarvinen [6] looked at the tumour spectrum in 40 HNPCC families and found colorectal (64%),
endometrial (8%), gastric (6%), biliary/pancreatic (4%)
and uroepithelial carcinomas (2%) to be the most frequent.
Vasen et al. [7], in 24 kindreds in the Netherlands, and Watson and Lynch [8], from a study of 23 HNPCC families,
likewise found carcinoma of the endometrium to be the
second commonest malignancy in HNPCC. They also reported an excess of gastric, urinary tract, small bowel, ovarian and hepatobiliary cancers. An increased incidence of
breast cancer and lung cancer have been reported in female
family members [9], but others workers have found no excess of these cancers in HNPCC [8, 10, 11]. Indeed Lynch
et al. [10, 11] found significantly fewer lung cancers oc-
curring in HNPCC families than in control groups. It is obviously more difficult to demonstrate a positive link
between these two cancers and HNPCC because of the relatively high incidence of breast and bronchogenic carcinoma in the general population. Risenger et al. [12], however, described widespread microsatellite instability in several breast carcinomas from known HNPCC kindreds. Furthermore, one family member had a germline hMLH1 mutation along with the wild-type allele in her normal tissues
but was homozygous for the mutant allele in the breast cancer tissue, suggesting that breast cancer may occur as an integral tumour in HNPCC. Beck et al. [13] also found germline HNPCC mutations in individuals from HNPCC kindreds with breast cancer but felt this may simply be the result of chance. Other studies have reported an increased incidence of lymphatic/haemopoietic cancers and sarcomas
in HNPCC [14 16] but this has not been substantiated.
Clinical characteristics
Lynchs early description subdivided HNPCC into two clinical variants: Lynch syndrome I, which is the predisposition to site specific colorectal cancer, and Lynch syndrome
II, which is characterised by the development of extracolonic malignancies. Many authorities now believe that there
is no clear distinction between these two variants, and that
they are merely manifestations of a single syndrome. This
is supported by recent data showing families with extra-colonic cancers and those with site-specific HNPCC to be
linked to the same chromosomes [17, 18].
Unlike familial adenomatous polyposis (FAP), in which
colorectal cancer is preceded by the development of hundreds to thousands of adenomatous polyps, HNPCC has no
such phenotypic features. Evidence suggests colorectal
carcinomas in HNPCC do develop from adenomatous
polyps, but that adenomas do not occur in large numbers.
Jass [19] reviewed 131 cancers from 117 HNPCC family
members. None of the cancers was of the small, superficial, de novo type, but residual adenoma (contiguous with
cancer) was present in 100% in situ cancers, 89% of cancers involving only the submucosa, 29% of cancers limited to the muscle coat and 12% of cancers extending
beyond muscle, suggesting that the majority had arisen
from adenomatous polyps. In an earlier study Jass and
Stewart [20] compared the prevalence of colorectal adenomas in 23 patients with HNPCC to that in an age-matched
forensic autopsy population. Although the incidence of
adenomas was greater in the HNPCC patients than in the
autopsy group (particularly those under 50 years of age;
30% and 5%, respectively) all the adenomas were solitary,
apart from that in one patient who had two synchronous
adenomas. Mecklin et al. [21] evaluated the histopathology of colorectal cancers and adenomas in 75 HNPCC patients with colorectal cancer compared to control patients
with sporadic colorectal cancer. They found a similar incidence of synchronous adenomas in the two groups, 19%
in HNPCC patients compared to 16% in controls. In a colonoscopic screening program Love and Morrissey [22]
be obtained fulfilled the Amsterdam criteria, and that a further 12% satisfied less strict criteria.
Genetics
ment of cancer in these families. This hypothesis is supported by experimental data in which Lazar et al. [51] demonstrated the presence of somatic mutations in the APC and
p53 genes in RER+ tumours from two HNPCC families.
Only three tumours were analysed (one polyp and two carcinomas). All the mutations detected were absent from patient lymphocyte-extracted DNA and could therefore be
considered as somatic mutations. The mutational pattern,
however, was interesting. In the polyp one mutation was
found in the APC gene and no mutations in the p53 gene,
but in the carcinomas six mutations were found in the
APC gene and four in the p53 gene in one and four mutations in the APC gene and none in the p53 gene in the other.
This mutational pattern is strikingly different from that observed in sporadic colorectal tumours, which are characterised by single or, at most, two different mutations in each
gene. This supports the hypothesis that in HNPCC inactivation of a DNA repair gene results in the progressive accumulation of mutations in critical genes known to be involved in colorectal tumourigenesis.
More recently, however, Parsons et al. [52] have looked
again at the non-neoplastic cells of HNPCC patients for
mismatch repair defects using more sensitive methods.
They found evidence of microsatellite instability in nonneoplastic cells in a subset of HNPCC patients. They postulated that this is due to inherited mutations of other genes
that participate in mismatch repair, with multiple germline
mutations leading to a reduction of mismatch repair activity. An alternative hypothesis is that mismatch repair gene
mutations are acting in a dominant-negative fashion; the
product of the abnormal allele interferes with the function
of the normal protein.
HNPCC is thus an end result of defects in one or more
of several mismatch repair genes. hMSH2 and hMLH1 are
thought to account for 70 90% of all cases of HNPCC.
hPMS1 and hPMS2, along with possibly other as yet unidentified DNA mismatch repair genes, account for the remainder.
Pathology
Despite the name non-polyposis colorectal cancer evidence to date suggests that the carcinomas in HNPCC, as
discussed above, arise from benign adenomatous polyps
[20, 53, 54]. Although adenomatous polyps are no more
prevalent in individuals from HNPCC pedigrees than in
the general population [20, 22, 53, 55], they do appear to
show a greater propensity for malignant transformation
[20]. In keeping with this they are also more likely to have
a villous component and to show moderate or severe dysplasia.
Comparison with sporadic colorectal carcinoma
Colorectal carcinomas in HNPCC are more often found in
the proximal colon than sporadic carcinomas, and there is
a higher incidence of synchronous and metachronous tumours. They usually have a normal diploid constitution
rather than being aneuploid, are frequently mucinous and
poorly differentiated, with areas of necrosis [3, 55 58]. It
has been suggested that, paradoxically, stage for stage the
prognosis of HNPCC tumours is relatively good [20, 59,
60], though this may in part be a selection bias by focusing on large pedigrees with surviving gene carriers. One
theory suggested to account for a good prognosis in the
presence of pathologically poor prognostic features is that
the relatively high mutational load that occurs in tumours
with defective DNA repair systems is detrimental to tumour survival [60]. Another possibility is that tumours carrying a large number of mutations stimulate a stronger immune response [31].
The majority of colorectal cancers in HNPCC [>80%)
manifest replication errors and are thus termed replication
error positive (RER+) tumours. Some 10 16% of sporadic
colorectal cancers have also been found to be RER+ [54,
62 63]. Interestingly, RER+ sporadic cancers exhibit
many of the features of colorectal tumours in HNPCC mentioned above, when compared to RER sporadic tumours
[62, 63]. In one study 80% of sporadic RER+ tumours were
proximal to the splenic flexure [54]. Some of these tumours
may represent unrecognised HNPCC or may be due to a
de novo rather than inherited germline mutation. Alternatively somatic mutations in HNPCC genes may have occurred during tumourigenesis.
Cancers at other sites
The RER phenotype is not limited to colon cancers. Peltomaki et al. [64] studied the incidence of microsatellite instability in more than 500 sporadic tumours, representing
six different types of cancer. They found that 18% of gastric cancers and 22% of endometrial cancers were RER+
whereas all of the lung, breast and testicular cancers examined were RER. Importantly, the first two cancers, as
opposed to the latter three, are part of the HNPCC tumour
spectrum. Risenger et al. [12] found microsatellite instability in both sporadic and HNPCC endometrial cancers. Microsatellite instability has also been observed in keratoacanthomas [65], a distinctive skin tumour frequently seen
in Muir Torre syndrome, which is now known to be part of
the HNPCC syndrome.
The reason for the observed site specificity of tumours
in HNPCC is still unclear. It may simply be a reflection of
the frequent exposure of these tissues to specific mutagens. The colorectal, gastric, small bowel and urothelial mucosas are often in contact with potential carcinogens, for
example heterocylic amines, unlike breast and testicular
cancers. If this were the case, however, it is somewhat surprising lung cancer is not part of the HNPCC tumour spectrum. Alternatively, Peltomaki et al. [64] suggested that the
site specificity is due to differences in the vulnerability of
various genes to replication errors. If target genes contain
mutation prone sequences at critical sites in their structure,
tumourigenesis is more likely to occur.
Management
Identification of HNPCC
Family history
Probably the most difficult hurdle in the management of
HNPCC is the identification of gene carriers. This is due
partly to the absence of a recognisable phenotype. The clinician must therefore always be alert to the possibility of
an inherited predisposition. The initial step in identification should be a routine family history of all malignancy.
One major problem with reliance on family history is the
accuracy of patient recall. Studies have shown inaccuracies in patient recollections of relatives illnesses, both
over- and underreporting pathology. Other factors which
should alert the clinican are multiple colonic tumours, extra-colonic cancers, particularly those recognised as part
of the HNPCC tumour spectrum and tumours occurring at
an early age. As discussed above, strict adherence to the
Amsterdam criteria is not appropriate as they are too stringent. All families with a pedigree suggestive of HNPCC
should be identified and referred to a geneticist.
Experience with FAP [66] suggests that the tracing of
pedigrees, counselling and recruitment of individuals for
screening is best performed by a trained genetics nurse,
along with a clinical geneticist. These pedigrees are often
complex and extensive. Contacting a healthy individual to
inform them of a potential health risk must be carried out
with tact and without the time restriction of a busy clinic.
The management of HNPCC is still not uniform, and pedigree tracing is not yet a routine concept as it is with FAP.
It has been adequately demonstrated with FAP that a regional register identifies more individuals at risk [66] and
leads to a more uniform approach to management. Most
FAP registries are now documenting cases of HNPCC, but
the increased workload (four to five times the incidence of
FAP) suggests that establishing regional registers for
HNPCC should be a goal in the near future.
Predictive testing
The recent identification and cloning of four genes,
hMSH2, hMLH1, hPMS1 and hPMS2, provides the basis
for direct mutation analysis. It is therefore now possible to
offer predictive testing to some families, but at present the
One of the cardinal features of HNPCC is the occurrence of synchronous and metachronous colorectal tumours, and therefore segmental resection is not appropriate in gene carriers. The risk of metachronous cancers is
40% at 10 years if segmental resection is performed [89].
The procedure of choice then is total colectomy with ileorectal anastomosis (IRA), which removes the maximal area
at risk, consistent with avoidance of a stoma, yet has low
morbidity and mortality [3, 10, 90]. The rectum must be
screened sigmoidoscopically following this, and for those
individuals for whom this is unacceptable a restorative
proctocolectomy is an appropriate choice.
Several important factors must be addressed when offering a prophylactic total colectomy, including whether
or not the procedure eliminates the cancer risk, its morbidity and its timing [87]. The cancer risk, although less, still
exists in that the rectum remains in situ. The risk of developing rectal cancer is in the region of 12% 12 years following total colectomy and IRA, and regular surveillance
is therefore mandatory [87]. There is also still the risk of
extra-colonic malignancies developing. Lynch et al. [85]
suggest that female gene carriers should be encouraged to
have their families early so that they can consider the option of hysterectomy and bilateral salpingo-oophorectomy
between the ages of 35 and 40 years, but they must be aware
of the possibility of developing peritoneal cystadenocarcinoma, ovarian in origin, despite having their ovaries removed. The patient must understand that total colectomy
and IRA is a major procedure with significant morbidity,
reported to be in the region of 7.8 10% in FAP [91, 92].
The optimal timing of surgery is not known, and advice
can only be based on the available data. The mean age at
presentation with colorectal cancer is the middle 40s
[3, 83], but the range is wide (14 82 years), [85]. The risks
of elective surgery increase with increasing age, but early
surgery inflicts a not insignificiant operative procedure on
individuals who may not develop cancer until their 60s or
70s, if at all.
It has taken a long time from Warthins first observations on family G to the establishment of the genetic
basis of HNPCC. Recent developments have contributed
greatly to our understanding of HNPCC, but if we are
to reduce morbidity and mortality rates, medical practitioners must become more aware of the syndrome, and
we must look towards the establishment of regional registers and management protocols for such families. Wider
availability of mutation analysis, as techniques improve,
will help clarify the natural history of the disease and
greatly assist in the management of these families in the
future.
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time come? Cancer 78: 199 201
88. Vasen HFA, Wijnen JT (1996) Cancer risk in families with hereditary non-polyposis colorectal cancer diagnosed by mutation
analysis. Gastroenterology 110: 1020 1027
89. Fante R, Roncucci L, Di Gregorio C, Tamassia MG, Losi L, Benatti P, Pedroni M, Percesepe A, De Pietri S, Ponz de Leon M
(1996) Frequency and clinical features of multiple tumours of the
large bowel in the general population and in patients with hereditary nonpolyposis colorectal carcinoma. Cancer 77: 2013 2021
90. Mecklin J-P, Jarvinen H (1993) Treatment and follow-up strategies in hereditary non-polyposis colorectal carcinoma. Dis Colon Rectum 36: 927929
91. Herrera L (1991) Extracolonic manifestations of FAP. Oncology 5: 31 33
92. Nugent KP, Northover J (1994) Total colectomy and ileorectal
anastomosis. In: Phillips RKS, Spigelman AD, Thompson JPS
(eds) Familial adenomatous polyposis, 1st edn. Arnold, London,
79 91
Springer-Verlag 1998
O R I G I N A L A RT I C L E
cas la constipation qui peut tre due dautres raisons mconnues. Ltude prsente a t entreprise pour tudier chez
les patientes porteuses dune rectocle sil existe des lsions colo-rectales concomittantes et dans quelle mesure elles associes la rectocle. Cent-douze femmes souffrant
de constipation svre et de troubles de lvacuation rectale ont t investigues laide de dfcographies, de mesures lectrophysiologiques, de manomtries ano-rectales
et de dterminations du temps de transit colique. Cinquante-six patientes chez lesquelles la dfcographie a mis
en vidence une rectocle ont t compares avec 56 patientes sans rectocle mais porteuses dautres anomalies
la dfcographie. La frquence dune raction paradoxale
du sphincter anal est plus souvent observe chez des patientes porteuses dune rectocle (60%) que chez les patientes sans rectocle (24%). Cette tude supporte lide
dune association entre la rectocle et la raction paradoxale du sphincter anal. Nous suggrons que des patientes
constipes porteuses dune rectocle doivent faire lobjet
dinvestigations compltes avant que lon envisage la correction chirurgicale de la rectocle. Dautres tudes sur
lefficacit du biofeedback en cas de rectocle et de ractions paradoxales du sphincter anal devraient tre entreprises.
Patients with rectocele may present with a variety of symptoms, thereby necessitating consultation with both gynecologists and surgeons. A rectocele may be isolated or part
of a complete genital prolapse.
Symptoms of rectocele include a bearing-down sensation, incomplete rectal emptying, a sensation of rectal pressure and sometimes vaginal symptoms from the herniation
itself [1, 2]. The role of a rectocele in constipation is controversial. At defecography about 25% of patients with defecation disorders have a rectocele, but many women with
rectocele are not constipated [3].
A transvaginal approach for rectocele repair is advocated by several gynecologists [2, 4, 5]. Using this approach gynecologic symptoms are often improved. How-
14
No of patients
20
3
36
0
14
22
0
11
3
2
1
112
(18%)
(3%)
(32%)
(0%)
(12%)
(20%)
(0%)
(10%)
(3%)
(2%)
(1%)
A viscous contrast medium was simultaneously instilled into the vagina. The patient was seated on a commode placed and exposed to a
fluoroscopic unit. Iron plates were used for contrast leveling. Left lateral views of the pelvis were recorded during fluoroscopy by video.
A rectocele was diagnosed when the anterior rectal and posterior
vaginal wall herniated into the lumen of the vagina an by how much?
Rectal intussusception was defined as a circumferential descent
of the entire thickness of the rectal wall, which might extend into the
anal canal but not through the anal verge. Rectal prolapse was defined
as a circumferential descent of the entire thickness of the rectal wall
seen coming out through the anus. An enterocele was diagnosed when
small bowel was present between the vagina and rectum.
Electrophysiology
Electrophysiologic analysis provides information on the function of
the pelvic floor musculature and its innervation. Electrophysiologic
assessment was carried out according to the method described by
Swash et al. [14, 15]. Conventional needle electromyography (EMG)
was recorded in the external anal sphincter (EAS) bilaterally and in
the puborectalis muscle. EMG was considered pathologic indicating
a peripheral nerve lesion if: 1) the activity during maximal voluntary contraction (squeezing) was reduced to such an extent that only single discharges of motor unit potentials were recorded instead
of a normal interference pattern, or 2) a moderately reduced interference pattern contained a considerable number of polyphasic motor unit potentials of high amplitude (> 2 mV).
A paradoxical anal sphincter reaction (PSR) was present if: 1)
maximal straining increased the on-going EMG activity, or 2) maximal straining did not decrease the on-going EMG-activity and no
closing reflex was seen after completed straining. Fibre density (FD)
was measured in the EAS by single fibre EMG recordings at 20 different locations on each side. Normal values for FD were derived
from the literature [14, 16, 17] and the normal limits (mean 2 SD)
for different age groups were 1.52 (< 30 years), 1.82 (30 65 years),
1.88 (66 70 years) and 2.20 (71 85 years). Pudendal nerve terminal motor latency (PNTML) was determined on both sides using a
special electrode (Dantec St. Marks Pudendal Electrode 13L40). The
upper limit of normal was 2.5 ms.
Anorectal manometry
The procedure has been previously described by Holmstrm et al.
[18]. With this method maximal anal resting pressure (MRP) less
than 50 mm Hg, maximal anal squeeze pressure (MSP) less than
65 mm Hg and maximal tolerable volume (MTV) less than 150 ml
or more than 400 ml were considered pathologic. Rectal sensibility was considered abnormal if the patient had no sensation of rectal filling following insufflation of the rectal balloon with 150 ml
of air.
Colon transit time
Colon transit time was estimated according to a modification of the
methods of Hinton et al. [19] and Keighley and Shouler [20]. The
patients ingested a capsule containing markers with a meal and five
days later a plain x-ray of the abdomen was taken. The number of
markers in the colon was estimated. A residue of more than 40% in
the colon was considered pathologic.
Statistical methods
The analyses were performed by the Department of Medical Information Processing, Karolinska Institutet, Stockholm.
Chi-squared analysis was used when comparing defecographic
and electrophysiologic findings, frequency of constipation, previous
15
hysterectomy and delayed colon transit time in patients with and
without rectocele.
Normal distribution of the data was checked and Students unpaired t-test was used when comparing MRP and MSP.
Results
The frequency of previous hysterectomy did not significantly differ between patients with (9%) or without rectocele (11%).
PSRa
Pathologic PNTMLb
Both EMG and FDc
indicate peripheral
nerve damage
a
b
c
Electrophysiology
The frequency of PSR was higher in patients with rectocele (60%) than in patients without (24%) (Table 2). There
were no statistical differences in the frequencies of pathologic PNTML and pathologic findings indicating peripheral neuropathy on EMG or FD between the two groups of
patients (Table 2).
Defecography
Thirty-six percent (20/56) of patients with rectocele had
additional radiological abnormalities on defecography
(Table 1). The frequencies of enterocele, rectal intussusception, and rectal prolapse were lower in patients with
rectocele than in patients without (Table 3).
Patients
with
rectocele
Patients
without
rectocele
60% (26/43)
16% (5/31)
24% (10/41)
32% (8/25)
P<0.001
N.S.
17% (6/35)
29% (9/31)
N.S.
Rectal intussusception
Rectal prolapse
Enterocele
Patients
with
rectocele
(n = 56)
Patients
without
rectocele
(n = 56)
29%
2%
11%
75%
25%
59%
Anorectal manometry
MRP was higher in patients with rectocele than in patients
without (P < 0.01) (Table 4). There was no statistical difference of MSP between patients with and without rectocele (Table 4).
Colon transit time
The proportion of patients with delayed colon transit time
did not differ significantly between the two groups. Eleven
(30%) of the 31 patients with rectocele had delayed colon
transit time compared with 13 (32%) of 41 patients without.
Discussion
P<0.001
P<0.001
P<0.001
Patients
with
rectocele
(n = 56)
Patients
without
rectocele
(n = 56)
72 19
72 26
61 23
64 26
P < 0.01
N.S.
fore been suggested [12, 21, 22], before considering surgical repair.
We have previously reported [23] that paradoxical
sphincter reaction (PSR) is frequently found in patients with
rectocele. The present study demonstrates an association
between rectocele and PSR and this association may indicate that PSR is a causative factor in the formation of the
rectocele. Straining and emptying efforts against a contracted pelvic floor may facilitate development of rectocele.
This association might also be one of the reasons for suboptimal results of surgical repair.
Different approaches to the treatment of PSR have been
proposed [24 27]. The best results are reported using EMG
feedback [28 30], aiming to teach patients to relax the pelvic floor muscles during straining. The present study indicates that some patients with rectocele might benefit from
biofeedback.
The present study does not support an association
between rectocele and pathologic reduced colon transit although this was abnormal in a high frequency of the patients. It may nevertheless be useful to include colonic transit studies in the preoperative assessment since these pa-
16
Conclusions
The present study supports an association between rectocele and paradoxical anal sphincter reaction. Constipated
patients with rectocele should be investigated thoroughly
before surgical repair is considered. Defecography will confirm the clinical diagnosis and might show concurrent abnormalities. Electrophysiologic assessment might demonstrate PSR and transit studies will help to identity patients
with a less favorable outcome after surgical repair.
Further prospective studies on the effect of biofeedback
in patients with rectocele and PSR are indicated.
Acknowledgements We are indebted to Elisabet Berg, Department
of Medical Information Processing, Karolinska Institutet, Stockholm
for help with the statistical analyses.
References
1. Sehapayak S (1985) Transrectal repair of rectocele: an extended armamentarium of colorectal surgeons. A report of 355 cases. Dis Colon Rectum 28: 422433
2. Nichols DH, Randall CL (1989) Vaginal surgery. 3rd edn. Williams & Wilkins, Baltimore
3. Nichols DH (1991) Surgery for pelciv floor disorders. Surg Clin
N Am 71: 927946
4. Marek CB (1969) Transverse repair for rectocele. South Med J
62: 749752
5. ster S, Astrup A (1981) A new vaginal operation for recurrent
and large rectocele using dermis transplant. Acta Obstet Gynecol Scand 60: 493495
6. Marks MM (1967) The rectal side of the rectocele. Dis Colon
Rectum 10: 387388
7. Pitchford CA (1967) Rectocele: a cause of anorectal pathologic changes in women. Dis Colon Rectum 10: 464466
8. Sullivan ES, Leaverton GH, Hardwick CE (1968) Transrectal
perineal repair: an adjunct to improved function after anorectal
surgery. Dis Colon Rectum 11: 106114
9. Capps WF (1975) Rectoplasty and perineoplasty for the symptomatic rectocele: a report of fifty cases. Dis Colon Rectum
18: 237244
10. Khubchandani IT, Sheets JA, Stasik JJ, Hakki AR (1983) Endorectal repair of rectocele. Dis Colon Rectum 26: 792796
11. Block IR (1986) Transrectal repair of rectocele using obliterative suture. Dis Colon Rectum 29: 707711
12. Arnold MW, Stewart WR, Aguilar PS (1990) Rectocele repair.
Four years experience. Dis Colon Rectum 33: 684687
13. Brodn B, Snellman B (1968) Procidentia of the rectum studied
with cineradiography: a contribution to the discussion of causative mechanism. Dis Colon Rectum 11: 330347
14. Neill ME, Swash M (1980) Increased motor unit fibre density
in the external and sphincter muscle in ano-rectal incontinence:
a single fibre EMG study. J Neurol Neurosurg Psychiatry 43:
343347
15. Kiff ES, Swash M (1984) Slowed conduction in the pudendal
nerves in idiopathic (neurogenic) faecal incontinence. Br J Surg
71: 614616
16. Percy JP, Neill ME, Kandiah TK, Swash M (1982) A neurogenic factor in faecal incontinence in the elderly. Age Ageing
11: 175179
17. Henry MM, Snooks SJ, Barnes PRH, Swash M (1985) Investigation of disorders of the anorectum and colon. Ann R Coll Surg
Engl 67: 355360
18. Holmstrm B, Brodn G, Dolk A, Frenckner B (1986) Increased
anal resting pressure following the Ripstein operation: a contribution to continence? Dis Colon Rectum 29: 485487
19. Hinton JM, Lennard-Jones JE, Young AC (1969) A new method for studying gut transit time using radioopaque markers. Gut
10: 842847
20. Keighley MRB, Shouler PJ (1984) Abnormalities of colonic
function in patients with rectal prolapse and faecal incontinence.
Br J Surg 71: 892895
21. Siproudhis L, Dautrme S, Robert A, Bretagne J, Heresbach D,
Raoul J, Gosselin M (1993) Dyschezia and rectocele a marriage of convenience? Physiologic evaluation of the rectocele in
a group of 52 women complaining of difficulty in evacuation.
Dis Colon Rectum 36: 10301036
22. Mellgren A, Anzn B, Nilsson B-Y, Johansson C, Dolk A, Gillgren P, Bremmer S, Holmstrm B (1995) Results of rectocele
repair, a prospective study. Dis Colon Rectum 38: 713
23. Johansson C, Nilsson B-Y, Holmstrm B, Dolk A, Mellgren A
(1992) Association between rectocele and paradoxical sphincter response. Dis Colon Rectum 35: 503509
24. Wasserman IF (1964) Puborectalis syndrome (rectal stenosis due
to anorectal spasm) Dis Colon Rectum 7: 8798
25. Keighley MRB, Shouler P (1984) Outlet syndrome: is there a
surgical option? J R Soc Med 77: 559563
26. Kamm MA, Hawley PR, Lennard-Jones JE (1988) Lateral division of the puborectalis muscle in the management of severe
constipation. Br J Surg 75: 661663
27. Hallan RI, Melling J, Womack NR, Williams NS, Waldron DJ,
Morrison JFB (1988) Treatment of anismus in intractable constipation with botulinum A toxin. Lancet II: 714717
28. Lestr B, Penninckx F, Kerremans R (1991) Biofeedback defaecation training for anismus. Int J Colorectal Dis 6: 202207
29. Kawimbe BM, Papachrysostomou M, Binnie NR, Clare N,
Smith AN (1991) Outlet obstruction constipation (anismus)
managed by biofeedback. Gut 32: 11751179
30. Wexner SD, Cheape JD, Jorge JMN, Heymen S, Jagelman DG
(1992) Prospective assessment of biofeedback for the treatment
of paradoxical puborectalis contraction. Dis Colon Rectum
35: 145150
31. Nichols D (1972) Types of enterocele and principles underlying choice of operation for repair. Am J Obstet Gynecol
40: 257263
32. Kelvin FM, Maglinte DDT, Hornback JA, Benson JT (1992) Pelvic prolapse: assessment with evacuation proctography (defecography). Radiology 184: 547551
33. Mellgren A (1994) Enterocele, rectocele and constipation,
Stockholm
Springer-Verlag 1998
O R I G I N A L A RT I C L E
Introduction
18
Technique
Two-staged procedure: This procedure was indicated for patients
having chronic colitis without high dose long term administration of
steroids. In the first stage, nearly all of the colon was excised leaving the caecum and about 5 cm of ascending colon. A rectal mucosectomy was performed to just above the dentate line, leaving about
a 5 cm rectal muscle cuff (Fig. 1). The right colic and ileocolic vessels were preserved in order to assure an adequate blood supply to
the terminal ileum. A 50 to 70 cm ileal segment was isolated,
20 to 40 cm from the ileocecal valve, with a vascular pedicle and a
J-shaped pouch 15 cm long at its distal portion was constructed. Mesenteric lengthening was performed by division of the vascular arcades and transverse incision of the peritoneum in a J pouch. The
proximal end of the isolated ileal segment was closed and attached
to the tenia of the cecum. An appendectomy was usually performed.
The apex of the J pouch was anastomosed to the anus. Finally, an
end ascending colostomy was constructed in the right lower quadrant. In the second stage, more than three months later, the opening
made by the ascending colostomy was closed, the retained colon was
excised to complete the proctocolectomy, and the terminal ileum was
anastomosed to the proximal end of the interposed ileal segment to
maintain intestinal continuity (Fig. 1 D).
Three-staged procedure: This procedure was indicated for patients either receiving high doses of steroids for severe colitis or in
association with a anal fistula. As first, a subtotal colectomy was performed, followed by a Brooke type end ileostomy and mucous fistula of the sigmoid colon (Figure 1 B). Each patient with an anal fis-
Fig. 1 A D Counterclockwise direction shows the two-staged operation and clockwise direction the three-staged operation. A The
first stage of the two-staged operation. Ascending colostomy and isolated ileal J pouch-anal anastomosis are performed following subtotal colectomy. The proximal end of the isolated ileum is closed.
B The first stage of the three-staged operation. A Brooke type ileostomy and mucous fistula of the sigmoid colon are constructed. C The
second stage of the three-staged operation. An isolated ileum with a
J pouch is anastomosed to the dentate line after excision of the retained colon and rectum. D Completion of ileoanal anastomosis. Finally, the terminal ileum is transposed above the isolated ileal segment
tula underwent a subtotal colectomy, followed by a fistulectomy
combined with an ascending colostomy and mucous fistula of the
sigmoid colon. For those patients receiving high dose steroids, one
month after cessation of steroids, the retained colon and rectal mucosa was excised, and an isolated ileal segment anastomosed to the
dentate line with creation of J pouch (Fig. 1 C). The proximal end of
the isolated ileal segment was closed and fixed to the abdominal wall
near the ileostomy. More than three months after the second operation, the opening made by the ileostomy or ascending colostomy was
closed and a proctocolectomy completed followed by an end to end
anastomosis between the terminal ileum and the proximal end of the
interposed ileal J pouch (Fig. 1 D).
Results
Of twelve patients, eight underwent the two-staged procedure and four the three-staged procedure. One of patients
who underwent the three-staged procedure had an anal fistula with chronic ulcerative colitis. The mean length of the
transposed ileum was 29 (range 20 40) cm, whilst the
mean length of the isolated ileal segment was 60 (range
50 70) cm. These lengths were determined by the branching of the mesenteric vessels.
Early postoperative complications consisted of compartment syndrome of the lower extremity in one patient
19
Discussion
References
1. Mibu R, Makino I, Chijiiwa K (1995) Gallstones and their composition in patients with ileoanal anastomosis. J Gastroenterol
30: 413 415
20
2. MKoma AE (1994) Follow-up results of hematology data before and after restorative proctocolectomy (clinical outcome). Dis
Colon Rectum 37: 932 937
3. Stringel G (1985) Pull-through with isolated jejunal loop for ulcerative colitis. J Pediatr Surg 20: 661 663
4. Mibu R, Itoh H, Nakayama F (1987) Effect of total colectomy
and mucosal proctectomy on intestinal absorptive capacity in
dogs. Dis Colon Rectum 30: 47 51
5. Nakahara S, Itoh H, Mibu R, Ikeda S, Nakayama F (1988) Regional difference in intestinal adaptation after total colectomy as
judged by the changes of mucosal Na-K ATPase, cyclic AMP,
and transmural potential difference. Dis Colon Rectum
31: 523 528
6. Hotokezaka M, Nakahara S, Nakamura K, Mibu R (1994) Morphology following proctocolectomy in dogs: effect of introduction of a neocolon using an interposed jejunal segment. Eur Surg
Res 26: 179 186
7. Chu KU, Tsuchiya T, Ishizuka J, Uchida T, Townsend CM,
Thompson JC (1995) Trophic response of gut and pancreas after
ileojejunal transposition. Ann Surg 221: 249 256
8. Hotokezaka M, Nakahara S, Iwamoto T, Chijiiwa K, Mibu R
(1996) Effect of terminal ileal transposition on intestinal absorption following proctocolectomy. Br J Surg 83: 486 492
9. Santos MC, Thompson JS (1993) Late complications of the ileal
pouchanal anastomosis. Am J Gastroenterol 88: 3 10
Springer-Verlag 1998
O R I G I N A L A RT I C L E
Abstract The study investigated the relationship between anal canal size and anal canal pressure measured simultaneously by anal endosonography and an electronic
pressure probe. Twelve normal subjects were studied. Anal
endosonography was performed using a 7.5-Mhz rotating
transducer of 2 5 cm focal length covered with a sylastic
balloon filled with degassed water (25 ml). During anal endosonography an electronic manometric probe was passed
along the side of the probe and positioned in the anal canal. The ultrasonic image was frozen when maximal anal
pressure was seen at basal, squeeze, and minimal pressure
during straining. An image was also obtained at maximal
anal relaxation after rectal distension with a balloon filled
with 150 ml air. The results showed that anal canal pressure was significantly and linearly correlated with anal canal diameter (P<0.001).
Key words Anal canal Pressure Endosonography
Sphincter Squeezing Straining
Rsum Cette tude analyse les relations entre la dimension et la pression du canal anal mesure simultanment
par chographie endo-anale et enregistrement lectronique
des pressions. Douze sujets normaux ont t tudis.
Lendo-sonographie anale a t ralise au moyen dune
sonde de 7,5 Mhz rotative avec une longuer focale da 2
5 cm recouverte dun ballon de silastic rempli de 25 ml
deau dgazee. Durant lchographie endo-anale, un capteur lectronique de pression a t pass le long de la sonde
et positionn dans le canal anal. Limage chographique a
t fige lorsque la pression anale maximale tait obenue
au repos et leffort de contraction et lorsque la pression
a t minimale durant lexpulsion. Une image a t obtenue
L. Marzio () F. A. Ciccaglione M. Falcucci
M. G. Malatesta L. Grossi N. Travaglini
Unit di Medicina Interna and Gastroenterologia,
G. dAnnunzio University, Casa di Cura Pierangeli,
Largo L. Pierangeli 1, I-65100 Pescara, Italy
S. Guerri
Urology Unit, Casa di Cura Pierangeli,
Largo L. Pierangeli 1, I-65100 Pescara, Italy
galement en phase maximale de relaxation aprs distension rectale par un ballonnet rempli de 150 ml dair. Ces
rsultats montrent que la pression du canal anal est corrle
de manire significative et linaire avec le diamtre du canal anal (P<0.001).
Introduction
22
Fig. 1 Anal manometry and
endosonography simultaneously taken in basal state in a normal subject. The endosonographic image was taken at
open air (A, arrow) and when a
steady anal canal pressure was
observed at manometry (B, arrow). Anal canal diameters
(A, B) were calculated as indicated in the endosonographic
picture. The endosonographic
probe was covered with a latex
balloon filled with 25 ml of water
latex and the water within it, any change in anal canal size at rest and
during straining, squeezing, and relaxation in response to rectal balloon distension could be easily observed and measured on the ultrasonographic screen.
The ultrasonographic and mounted pressure probes were introduced into the rectum, and were slowly withdrawn until the maximal
anal canal pressure was recorded. After stabilization of the pressure
at that level the subject was asked to squeeze and strain alternatively on three consecutive occasions at an interval of 3 min. The endosonographic image was frozen at the maximal basal resting pressure
(Fig. 1), at maximum squeeze pressure (Fig. 2), at minimal pressure
on straining (Fig. 3), and at minimal and canal pressure induced by
rectal distention (Fig. 4). Finally, an image was taken after withdrawing the probe from the anal canal to calculate the diameter of the water-filled cover at atmospheric pressure (Fig. 1). On ultrasonography
the anal canal assumed an ellipsoid shape and the anteroposterior and
transverse diameters were recorded (mm; Fig. 1). Data were computed by one of the authors (F. C.) who was unaware of the pressure
Results
23
Fig. 2 Anal manometry and
endosonography in a normal
subject. The endosonographic
pictures are taken during stable
anal canal pressure (A, arrow)
and at maximal squeezing pressure (B, arrow)
Discussion
Basal
Squeezing
Straining
Rectal distention
* P<0.05 vs. basal
Pressure
(mBar)
Diameter
(mm)
8718
14512 *
3810 *
239 *
195
133 *
297 *
268 *
24
Fig. 3 Anal manometry and
endosonography in a normal
subject. The endosonographic
pictures are taken during stable
anal canal pressure (A, arrow)
and at minimal straining pressure (B, arrow)
Table 2 Pressure of the anal canal (mBar) measured with and without the endosonographic probe (n = 12)
Normal subjects
Basal
Squeezing
Straining
Rectal distension
* NS vs. with probe
With probe
Without probe
8718
14512
3810
239
8911 *
15218 *
346 *
228 *
25
Fig. 4 Anal manometry and
endosonography in a normal
subject. The endosonographic
pictures are taken during stable
anal canal pressure (A, arrow)
and at minimal pressure recorded during rectal distension with
150 ml of air (B, arrow)
26
muscle thickness and reduced capability to generate pressure in the anal canal. This is supported by evidence that
the lower the electrical activity of the external anal sphincter during squeezing, the thicker is the internal anal sphincter muscle [9]. While the determination of internal and
sphincter thickness is easy, imaging by ultrasound the external anal sphincter is less clear [5]. Our study shows that
the anal canal diameter is correlated well with the pressures generated by the combined internal and external
sphincters. It is appreciated that the diameter measured by
ultrasound is essentially a distortion due to the latex balloon. The presence of the endosonographic probe does not,
however, appear to influence anal canal pressures since no
differences were found when the pressures were measured
with or without the endosonographic probe.
Our study provides evidence that the anal canal of normal human subjects changes in size at variations in pressure in an inverse linear manner.
Acknowledgement Part of this paper was presented at the American Gastroenterological Association Meeting, San Francisco,
California, USA, 19 22 May 1996.
References
1. Law PJ, Bartam CI (1989) Anal endosonography: technique and
normal anatomy. Gastrointest Radiol 14: 349 353
2. Pittmann JS, Benson TB, Sumners JE (1990) Physiologic evaluation of the anorectum a new ultrasound technique. Dis Colon
Rectum 33: 476 478
3. Sultan AH, Kamm MA, Talbot IC, Nicholls RJ, Bartam CI (1994)
Anal endosonography for identifying sphincter defects confirmed
histologically. Br J Surg 81: 463 465
4. Felt-Bersma RJF, Cuesta MA, Koorevaar M, Srijers RLM, Meuwissen SGM, Derksen EJ, Wesdorp RIC (1992) Anal endosonography: relationship with anal manometry and neurophysiologic
tests. Dis Colon Rectum 35: 944 949
5. Emblem R, Dhaenens G, Stien R, Morkrid L, Aasen AO, Bergan
A (1994) The importance of anal endosonography in the evaluation of idiopathic fecal incontinence. Dis Colon Rectum
37: 42 48
6. Schafer A, Enck P, Furst G, Kahn T, Frieling T, Lubke HJ (1994)
Anatomy of the anal sphincters. Comparison of anal endosonography to magnetic resonance imaging. Dis Colon Rectum
37: 777 781
7. Burnett SJD, Bartram CI (1991) Endosonographic variations in
the normal internal sphincter. Int J Colorect Dis 6: 2 4
8. Swash M, Gray A, Lubowski DZ, Nicholls RJ (1988) Ultrastructural changes in internal anal sphincter in neurogenic fecal incontinence. Gut 29: 1692 1698
9. Law PJ, Kamm MA, Bartrams CI (1991) Anal endosonography
in the investigation of fecal incontinence. Br J Surg 78: 312 314
Springer-Verlag 1998
O R I G I N A L A RT I C L E
S. Carter M. Winslet
S. Carter () M. Winslet
University Department of Surgery,
Royal Free Hospital, School of Medicine,
Pond Street, London NW3 2QG, UK
Introduction
Patient delay
28
discussed their symptoms with relatives or friends but only 22% had consulted a physician. On the positive side,
there is some evidence that patient delay has decreased
over the past decade, with one series reporting a median
delay of 16 weeks in 1984 compared to 6 weeks in 1993
[16], although the reasons for this are unclear.
29
Table 1 Dukes stage at presentation and delay at general practitioner (CP) and hospital levels (percentages) in carcinoma of the
colon (n = 100) and carcinoma of the rectum (n = 100; from [22] with
kind permission of the Lancet)
Delay
Dukes stage
A
Hospital delay
Colon
None
GP or hospital
78
22 a
17
2
41
9
20
11
Rectum
None
GP or hospital
78
22 b
16
2
39
6
23
14
a
b
The study by Rowe-Jones [22] clearly illustrates how delay can affect the stage at presentation. One hundred patients with carcinoma of the colon and 100 with carcinoma
30
Summary
There is considerable evidence that there is delay at patient, general practitioner and hospital levels prior to the
diagnosis of colorectal carcinoma. This has been present
for several years and does not seem likely to decrease in
the near future but rather to increase particularly in the
case of delay at hospital level. There are potential means
to try and reduce this delay at all three levels, but there
does appear to be a group of colorectal tumours that are
biologically aggressive and associated with a shorter duration of symptoms, and it seems unlikely that the prognosis in this group of patients will be improved by earlier
diagnosis.
31
18. Springall R, Todd I (1988) General practitioner referral of patients
with lower gastrointestinal symptoms. J R Soc Med 81: 87 88
19. Hennigan T, Franks P, Hocken D, Allen-Mersh T (1990) Rectal
examination in general practice. BMJ 301: 478 480
20. MacArthur C, Smith A (1983) Delay in the diagnosis of colorectal cancer. J R Coll Gen Pract 33: 159 161
21. Rubin G (1992) Endoscopy facilities in general practice. BMJ
304: 1542 1543
22. Rowe-Jones D, Aylett S (1965) Delay in treatment in carcinoma of colon and rectum. Lancet II: 973 976
23. Dixon A, Thornton-Holmes J, Cheetham N (1990) General
practitioners awareness of colorectal cancer a 10 year review.
BMJ 301: 152 153
Springer-Verlag 1998
O R I G I N A L A RT I C L E
33
Introduction
34
Table 1 Follow-up regimen: frequency of examinations per year
Dukes
stage
A
B
C1
C2
Tumor
marker
Pelvis
4
4
4
4
Barium enema
or CF
US
CT
MRI
1
1
2
2
1
1
2
2
1
1, 2
1, 2
Chest
X-ray
1
1
1, 2
1, 2
Liver
1
1
2
2
US
CT
1
3
2
3
1
2
1
2
Table 2 Treatment of pelvic recurrence. (n1 corresponds to Dukes C1 , n2 ~ corresponds to Dukes C2 , including lateral lymph nodes metastases)
Total (n)
Curative resection
Palliative resection
Conservative therapy
Total
Group 1
Group 2
58
36
22
27
20
7
46.6
55.6
31.8
9
4
5
15.5
11.1
22.7
22
12
10
37.9
33.3
45.5
7
10
41
24
17
12
4
7
16
10
6
3
57.1
70.0
39.0
41.7
35.3
25.0
1
1
7
7
0
0
2
2
18
7
11
9
Table 3 Operating procedure in pelvic recurrence. (Conservative therapy: SPO n = 4, APR n = 18; resection rate: SPO 20/25, 89%; APR
16/33, 48.5%; P<0.05)
Initial procedure
Total (n)
Secondary procedure
Tumor resection
(n)
APR
TPE
Curative resection
Total
SPO
APR
27 a
15
12
3
2
1
10 b
10
37.0
71.4
14 b
3
11
51.9
84.5
Palliative resection
Total
SPO
APR
9
5
4
4
4
2
1
1
a
b
Results
35
Table 4 Sites of failure of curative resection for recurrent rectal cancer
Treatment
Total
Local
regional recurrence
Liver
metastases
Lung
metastases
Bone
metastases
Total
12 (44.2%)
6 a (22.2%)
Secondary procedure
XRT+surgery b (n = 15)
Surgery alone c (n = 10)
5
7
2
4
2
4
3
2
3
2
Initial procedure
SPO (n = 15)
APR (n = 12)
7
5
4 (26.7%)
2 (16.7%)
4
2
1
4
3
2
a
b
c
in 15 patients with initial SPO. There were two local re-recurrences (16.7%) in 12 with initial APR: There was no significant difference between initial procedures. However, all
three patients who underwent local tumor resection in the
secondary procedure developed locally re-recurrent tumors.
Overall survival curves in patients undergoing this treatment are shown in Fig. 1. In the 27 patients with curative
resection there were six (22.2%) 5-year survivors, four of
whom had received XRT + surgery (TPE, one; TPE with
sacral resection, one; APR with sacral resection, two), and
two received surgery alone (TPE, one; TPE with sacral resection, one). One of the two patients in the latter group
died of local re-recurrence with distant metastases
65 months after the operation. Five are alive and well without evidence of disease. The estimated 5-year survival in
this group of 27 patients was 45.6%. There were no 5-year
survivors among those receiving palliative resection or
conservative therapy. There was a significant difference in
the survival curves between the patients with curative resection and the patients with palliative resection and conservative therapy. Actuarial survival curves of the patients
with curative resection for treatment group (XRT + surgery, surgery alone) are shown in Fig. 2. The estimated
5-year survival in XRT + surgery group was 61.2% and
that in the surgery alone group was 29.6%; there was no
significant difference in the curves between the two groups.
Although no perioperative deaths occurred in this series, complications were common in those undergoing TPE
or TPE with sacral resection (n = 14), with ten patients
(71.4%) having perioperative complications (Table 5).
Seven of eight patients with XRT + TPE (87.5%) and all
patients with TPE and sacral resection had serious complications. The most common complications were infection
and poor wound healing. Serious complications such as fistula and cardiovascular abnormalities were observed in the
groups receiving XRT + TPE and TPE with sacral resection. The morbidity rate (71.4%) was significant. Intraoperative blood loss was extensive (average 6900 ml, range
4000 12 600 ml), and the duration of operation was long
(average 13 h, range 8.5 26 h) in these procedures, especially in TPE with sacral resection.
36
Table 5 Morbidity in 14 patients with TPE with or without sacral
resection for pelvic local recurrence. (Intraoperative blood loss: average 6900 ml, range 4000 12,600 ml; duration of operation: average 13 h, range 8.5 26 h)
Complications
n
a
Perioperative morbidity
Infection
Abscess
Sepsis
Wound complications
Infection
Separation
Fistula
Small bowel/large bowel
Ureteral
Cardiovascular complications
Deep venous thrombosis
Postoperative bleeding
Pulmonary insufficiency
Hepatic failure
a
10 (71.4%)
7
3
6
4
3
2
3
2
3
1
Discussion
Local pelvic recurrence of rectal cancer is a major problem. The prognosis for patients who develop pelvic recurrence is generally poor, with 5-year survival ranging from
2% to 30% [18, 33, 34]. Surgical resection remains the only
potentially curative treatment in the majority of these patients. As long ago as 1959 Bacon and Berkley reported a
group of 93 patients with local recurrence [35]. In this report 38 patients underwent a curative resection, and
5-year survival was 37%. Recent studies suggest that the
addition of external or intraoperative XRT surgical resection improves survival and local control. However, the risk
of further local recurrence remains approximately 50%
[36 40]. The study by Wanebo et al. [41] on pelvic resection of recurrent rectal cancer reported that pelvic recurrence was resected safely with the expectation of long-term
survival in 33% of cases.
Resection of local recurrence probably also offers the
best chance for improved local control and palliation. For
recurrence at the anastomotic site resection can be curative or palliative by APR with or without sacral resection.
However, there is less chance of resection in local recurrence following radical APR. In our study the resection
rate (curative and palliative) was 80.0% in the SPO group
and 48.5% in the APR group. There was a significant difference in the resectability between two initial treatment
groups. This may in part be due to the fact that it is sometimes difficult to detect local recurrence early and to distinguish local recurrence from scar tissue formation. Most
studies report the role of early detection of local recurrence in resectability and prognosis after resection. In our
study the rate of curative resection in local recurrence was
46.6%: 55.6% in group 1 but only 31.8% group 2. There
was a significant difference in resectability between two
groups. The mean size of recurrent tumors in the resected
specimen was 3.6 cm (range 2.2 5.1 cm) in group 1 and
4.9 cm (range 3.8 6.9 cm) in group 2. These results suggest that our follow-up (group 2) was performed more intensive and was useful for the early detection of local recurrence. However, a simple and more effective followup must be considered. There were few chances for curative resection in the patients with local recurrence who
had undergone an extended radical surgery for the lateral
spread lymph node metastases in initial treatment since
most had local recurrence with the demonstrable extensive involvement of the lateral pelvic wall with distant
metastases. There were few chances for curative resection in recurrent cases that had had an extended radical
resection for a considerable advanced tumor in the initial
treatment.
In the curative procedure for local pelvic recurrence
APR with or without sacral resection was the standard procedure for patients with previous SPO, and TPE with or
without sacral resection was the standard operation for patients with previous APR, because several foci of local recurrence were frequently found in the pelvis, and it was
impossible to obtain sufficient surgical margins by other
procedures. In our experience these pathological findings
are observed in 4 of 16 specimens resected by TPE. Recurrence invading the urogenital organs can be resected
successfully by TPE. When recurrence invades or is adjacent to the sacrum, a combined sacral resection below S2
or S3 is required to obtain radical (curative, negative) margins. All of the patients receiving local excision for pelvic
recurrence developed local re-recurrent tumors in our
study. Local excision (tumor resection) was not a curative
procedure for local recurrence after rectal cancer surgery.
TPE with or without sacral resection was performed in
11 of 12 patients with previous APR in this study. However, this radical procedure for local recurrence had extensive perioperative morbidity (pelvic abscess, sepsis,
wound infection, and separation, bowel/urinary fistula,
pulmonary insufficiency, postoperative bleeding, deep venous thrombosis, hepatic failure), extensive blood loss (average 6900 ml, range 4000 12 600 ml), and long duration
of operation (average 13 h, range 8.5 26 h).
Complications were common in patients receiving TPE
for local recurrence (9 of 14 patients, 64.3%), and there
were four operative deaths (perioperative mortality 9.5%)
in the series using by this procedure. In the study of pelvic resection by Wanebo et al. [41] perioperative mortality was 8.5%, and the majority of their cases had operative
complications. Therefore careful selection of patients is
necessary for these procedures. All three of our patients
with local tumor resection for recurrence developed local
re-recurrence in the pelvis within 2 years of re-operation.
Local tumor resection therefore does not seen an appropriate procedure for local recurrence as curative margins (whether microscopically clear) were not obtained by this
procedure. Since there was the potential for microscopic
or minimal gross residual disease in the pelvis, TPE with
or without sacral resection was a viable procedure for patients who developed pelvic recurrence following previous APR. Complete removal of recurrence resulted in improved survival.
37
Conclusions
Pelvic local recurrence of rectal cancer can be resected curably by APR and TPE with or without sacral resection in
selected patients. TPE with or without sacral resection
should be standard procedure for local recurrence following previous APR. The estimated 5-year survival in our curative patients was 45.6%. Survival following surgery
combined with preoperative XRT also appears superior to
that after surgery alone. Although relatively few patients
received curative resection for local pelvic recurrence in
this study, it seemed that early detection of local recurrence, careful patient selection, and wide excision with preoperative radiotherapy were important for obtaining good
results.
References
1. Nicholls RJ, Ritchie JK, Wadsworth J, Parks AG (1979) Total
excision or retrative resection for carcinoma of the middle third
of the rectum. Br J Surg 66: 625 627
2. Luke M, Kirkegaard P, Lendorf A, Christiansen J (1983) Pelvic
recurrence rate after abdominoperineal resection and low anterior resection for rectal cancer before and after introduction of
the stapling technique. World J Surg 7: 616 619
3. Williams NS, Johnson D (1984) Survival and recurrence after
sphincter saving resection and abdominoperineal resection for
carcinoma of the middle third of the rectum. Br J Surg
71: 278 282
4. Gillen P, Peel AL (1986) Comparison of the mortality, morbidity and incidence of local recurrence in patients with cancer treated by either stapled anterior resection or abdominoperineal resection. Br J Surg 73: 339 341
5. Vlasak JW, Wagner D, Passaro E, Wilson SE (1989) Local recurrence after curative resection of rectal cancer: a comparison
of low anterior resection and abdominoperineal resection. J Surg
Oncol 41: 236 239
6. Fick TE, Baeten CG, von Meyenfeldt MF, Obertop H (1990) Recurrence and survival after abdominoperineal and low anterior
resection for rectal cancer, without adjunctive therapy. Eur J Surg
Oncol 16: 105 108
7. Wolff BG (1992) Lateral margins of resection in adenocarcinoma of the rectum. World J Surg 16: 467 469
8. MacFarlane JK, Ryall RD, Heald RJ (1993) Mesorectal excision
for rectal cancer. Lancet 341: 457 460
9. Sanella NA (1976) Abdominoperineal resection following anterior resection. Cancer 38: 378 381
10. Segall MM, Goldberg SM, Nivatvongs S, Balcos EG, Nemer
FD, Schottler JL, Christenson CE, Rothenberger DA (1981) Abdominoperineal resection for recurrent cancer following anterior resection. Dis Colon Rectum 24: 80 84
11. Vassilopoulos PP, Yoon JM, Ledesma EJ, Mittelman A (1981)
Treatment of recurrence of adenocarcinoma of the colon and rectum at the anastomotic site. Surg Gynecol Obstet 152: 777 780
12. Pahlman L, Glimelius B (1990) Pre- or postoperative radiotherapy in rectal and rectosigmoid carcinoma: report from a randomized multicenter trial. Ann Surg 211: 187 195
13. Stockholm Rectal Cancer Study Group (1990) Preoperative
short-term radiation therapy in operable rectal cancer. Cancer
66: 49 55
38
14. Marsh PJ, James RD, Schofield PF (1994) Adjuvant preoperative radiotherapy for locally advanced rectal carcinoma. Dis Colon Rectum 37: 1205 1214
15. Fujimoto S, Takahashi M, Endo F, Shrestha RD, Kokubun M,
Takai M, Okui K (1991) A clinical pilot study combining surgery with intraoperative pelvic hyperthermochemotherapy to
prevent the local recurrence of rectal cancer. Ann Surg
213: 43 47
16. Korenaga D, Matsushita T, Adachi Y, Mori M, Matsuda H, Kuwano H, Sugimachi K (1992) Preoperative hyperthermia combined with chemotherapy and radiotherapy for patients with rectal carcinoma may prevent early local pelvic recurrence. Int J
Colorect Dis 7: 206 209
17. Willett CG, Shellito PC, Tepper JE, Eliseo R, Convery K, Wood
WC (1991) Intraoperative electron beam radiation therapy for
primary locally advanced rectal and rectosigmoid carcinoma.
J Clin Oncol 9: 843 849
18. Cass AW, Million RR, Pfaff WW (1976) Patterns of recurrence
following surgery alone for adenocarcinoma of the colon and
rectum. Cancer 37: 2861 2865
19. Polk HC, Spratt JJ (1979) Results of treatment of perineal recurrence of cancer of the rectum. Cancer 43: 952 956
20. Hojo K (1986) Anastomotic recurrence after sphincter saving
resection for rectal cancer; length of distal clearance of the bowel. Dis Colon Rectum 29: 11 14
21. Schiessel R, Wunderlick M, Herbst F (1986) Local recurrence
of colorectal cancer; effect of early detection and aggressive surgery. Br J Surg 72: 342 344
22. Nymann T, Jess P, Christiansen J (1995) Rate and treatment of
pelvic recurrence after abdominoperineal resection and low anterior resection for rectal cancer. Dis Colon Rectum 38: 799 802
23. Wanebo HJ, Gaker DL, Whitehill R, Morgan RF, Constable WC
(1987) Pelvic recurrence of rectal cancer; options for curative
resection. Ann Surg 205: 482 495
24. Takagi H, Morimoto T, Hara S (1986) Seven cases of pelvic exenteration combined with sacral resection for locally recurrent
rectal cancer. J Surg Oncol 32: 184 188
25. Pearlman NW, Donahue RE, Steigmann GV, Ahnen DJ, Sedlacek SM, Braun TJ (1987) Pelvic and sacropelvic exenteration
for locally advanced or recurrent anorectal cancer. Arch Surg
122: 537 541
26. Touran T, Frost DB, OConnell TR (1990) Sacral resection: operative technique and outcome. Arch Surg 179: 555 560
27. Temple WJ, Ketchman AS (1991) Sacral resection for control
of pelvic tumors. Am J Surg 163: 370 374
28. Wanebo HJ, Marcove RC (1981) Abdominosacral resection of
locally recurrent rectal cancer. Am Surg 194: 458 471
Springer-Verlag 1998
O R I G I N A L A RT I C L E
Abstract The frequency of duodenal adenomas in patients with, familial adenomatous polyposis is high.
Duodenal adenoma has malignant potential, and duodenal
adenocarcinoma is one of the main causes of death in patients who have had previous proctocolectomy. A conservative approach to the treatment of duodenal adenomas
(nonsteroidal anti-inflammatory drugs, endoscopy, polypectomy through duodenotomy) is inefficient and unsafe.
When invasive cancer occurs in duodenal adenomas, the
result of surgery is poor. We have performed prophylactic
pancreaticoduodenal resection (PDR) for nonmalignant
severe duodenal polyposis in five patients since 1991. No
operative mortality was observed. One patient developed
a pancreatic fistula which was successfully managed by
medical treatment. The mean follow-up was 35 months.
All five patients are still alive and have a good functional
outcome. Prophylactic PDR may be indicated in familial
adenomatous polyposis when duodenal polyposis is severe. Stages III and IV of Spigelmans classification, periampullary adenoma, age above 40, and family history of
duodenal cancer are factors that may lead to the decision
to perform prophylactic PDR.
Key words Familial adenomatous polyposis Duodenal
adenomas Prophylactic pancreaticoduodenal resection
Rsum Lincidence dadnomes duodnaux chez des
patients atteints de polypose familiale (F. A. P.) est levee. Les adnomes du duodnum ont un potentiel de malignit et les adnocarcinomes du duodnum sont une des
causes principales de dcs chez les patients qui avaient
subi au pralable une procto-colectomie. Une approche
conservatrice du traitement des adnomes duodnaux (mdication anti-inflammatoire non strodienne, endoscopie,
S. Causeret Y. Franois J. B. Griot F. N. Gilly J. Vignal ()
Department of Surgery, Hpital Lyon Sud,
F-69495 Pierre Benite Cedex, France
B. Flourie
Department of Gastroenterology, Hpital Lyon Sud,
F-69495 Pierre Benite Cedex, France
Introduction
40
Table 1 Patient characteristics: age (years) at diagnosis of different stages of the disease and intestinal status when PDR was performed. (IRA Ileo-rectal anastomosis, IAA Ileo-anal anastomosis)
Patient
no.
FAP
Duodenal
disease
PDR
Intestinal
status
1
2
3
4
5
16
40
19
21
28
38
52
27
38
33
38
53
27
38
36
Ileostomy
Ileostomy
IRA
IRA
IAA
Adenomas
Size (mm)
Histology
Dysplasia
14
14
Tubulous
Mild
5 20
5 10
Tubulovillous
Moderate
>20
>10
Villous
Severe
Duodenal polyposis was diagnosed by routine gastroduodenoscopy in three patients; another patient presented with a history of
gastroesophageal reflux and another with an episode of cholangitis.
Gastroduodenoscopy was performed with forward and side-viewing
endoscopes. The endoscopic appearance of the papilla was noted,
and biopsies were taken systematically from the polyps. The histopathological examination was performed by the same pathologist.
To assess the severity of the duodenal polyposis the classification of
Spigelman was used (Table 2), and endoscopic aspect of the papilla was noted (Table 3).
Before PDR the duodenum was inspected directly two patients
by duodenotomy. In one of them enteroscopy was used to identify
possible adenomas in the small intestine. Resection of the first jejunal loop was performed in four cases because of distal duodenal
involvement. The reconstruction, according to Child, started with
the pancreatic anastomosis. In all cases pancreatic tissue was friable, and the diameter of the Wirsung duct was small (2 3 mm) in
all cases but one. Wirsungojejunostomy was performed in four cases and was intubated by a unexteriorised silastic catheter. Two anterior and posterior layers secured the pancreatic section to the jejunal serosa. The fifth patient, in whom no Wirsung duct was seen,
Table 4 Histopathological
examination of the specimens
and stage (Spigelmans
classification)
Results
Histopathological examination of the specimens confirmed the absence of cancer and revealed a more severe
polyposis (as regards number of polyps, size, and dysplasia) than expected preoperatively. Four patients were classified stage IV postoperatively compared to only two preoperatively (Tables 3, 4).
No mortality was observed. The postoperative course
was uneventful in three patients. In one it was complicated
by a delay in bowel transit that required nasogastric suction for 16 days. A pancreatic fistula, with a daily output
up to 500 ml, occurred in one patient; this was successfully
managed by suction, inhibition of pancreatic secretion
(Sandostatine for 23 days), and total parenteral nutrition.
The five patients were discharged respectively on the 13th,
14th, 14th, 28th, and 66th postoperative days.
Late outcome
The mean follow-up of the five patients was 35 months (respectively 2, 19, 42, 42, and 69 months). All five patients
are still alive and have undergone regular endoscopy of the
upper digestive tract; this has also been performed of the
retained rectum in two patients. After PDR no gastric adenomas were observed. On the other hand, the rectum displayed recurrent polyposis which was dealt with either by
endoscopic electrocoagulation (in one patient) or by proctectomy and a J ileal pouch (in another).
Table 5 shows the functional digestive assessment, need
for special died and weight before and after PDR.
Patient
no.
Duodenal
adenomas (n)
Size
(mm)
Papilla
aspect
Histology
Dysplasia
Stage
1
2
3
4
5
3
6
3
3
>20
>10
>10
>10
>10
>10
Abnormal
?
Abnormal
Abnormal
Abnormal
Tubulovillous
Tubulovillous
Tubulovillous
Tubulovillous
Tubulovillous
Moderate
Moderate
Moderate
Moderate
Severe
III
IV
III
III
IV
Patient
Adenomas
(n)
Max. size
(mm)
Papilla
aspect
Ampulla
localization
Histology
Dysplasia
Stage
1
2
3
4
5
6
3
5
15
>20
40
18
30
30
70
Tumor
Normal
Tumor
Tumor
Tumor
Yes
No
Yes
Yes
Yes
Tubulovillous
Tubulovillous
Tubulovillous
Tubulovillous
Tubulovillous
Moderate
Moderate
Severe
Moderate
Severe
IV
III
IV
IV
IV
41
Table 5 Functional digestive
assessment, requirements for
special diet and weight, before
and after PDR
Patient
1
2
3
4
5
Bowel movement
(n/24 h)
Ileostomy output
(l/24 h)
Special diet
Before
Before
After
Before
After
Before
After
<1
<1
<1
<1
No
No
No
No
No
No
No
Yes
No
No
51
65
66
44
50
51
58
69
45
44
1, 2
13
4, 5
After
1, 2
13
5, 6
Comments
Weight
(kg)
under evaluation [27]. Endoscopic methods appear generally to be restricted to small, few, and easily accessible
lesions.
Polypectomy through duodenotomy has the same limits as endoscopic treatment. Penna et al. [28] studied 12
patients and reported recurrence of duodenal polyposis in
all patients after a mean follow-up of 13 months.
Conclusion
PDR in duodenal polyposis must not be a routine procedure but rather should be reserved for patients at high risk
of cancer. This risk is related to the severity of polyposis
42
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21. Offerhaus GJ, Giardiello FM, Krush AJ, Booker SV, Tersmette
AC, Kelley NC, Hamilton SR (1992) The risk of upper gastrointestinal cancer in familial adenomatous polyposis. Gastroenterology 102: 1980 1982
22. Noda Y, Watanabe H, Iida M, Narisawa R, Kurosaki I, Iwafuchi M, Satoh M, Ajioka Y (1992) Histologic follow-up of ampullary adenomas in patients with familial adenomatosis coli.
Cancer 70: 1847 1856
23. Beckwith PS, Van-Heerden JA, Dozois RR (1991) Prognosis of
symptomatic duodenal adenomas in familial adenomatous polyposis. Arch Surg 126: 825 827
24. Sanabria JR, Croxford R, Berck TC, Cohen Z, Bapat BV, Gallinger S (1996) Familial segregation in the occurrence and severity of periampullary neoplasms in familial adenomatous
polyposis. Am J Surg 171: 136 140
24. Debinski HS, Trojan J, Nugent KP, Spigelman AD, Phillips RKS
(1995) Effect of sulindac on small polyps in familial adenomatous polyposis. Lancet 345: 855 856
26. Nugent KP, Farmer KCR, Spigelman AD, Williams CB, Phillips RKS (1993) Randomized controlled trial of the effect of
sulindac on duodenal and rectal polyposis and cell proliferation
in patients with familial adenomatous polyposis. Br J Surg
80: 1618 1619
27. Mlkvy P, Messmann H, Debinski H, Regula J, Conio M,
MacRobert A, Spigelman AD, Phillips RKS, Bown SG (1995)
Photodynamic therapy in familial adenomatous polyposis: a
pilot study. Eur J Cancer 31A: 1160 1165
28. Penna C, Phillips RKS, Tiret E, Spigelman AD (1993) Surgical
polypectomy of duodenal adenomas in familial adenomatous
polyposis: experience of two European centres. Br J Surg 80:
1027 1029
29. Balladur P, Penna C, Tiret E, Vaillant JC, Gailleton R, Parc R
(1993) Pancreatico-duodenectomy for cancer and precancer in
familial adenomatous polyposis. Int J Colorectal Dis 8: 151 153
30. Scates DK, Venitt S, Phillips RKS, Spigelman AD (1995) High
pH reduces DNA damage caused by bile from patients with familial adenomatous polyposis. Gut 71: 354 358
31. Spigelman AD, Owen RW, Hill MJ, Phillips RKS (1991)
Biliary bile acid profiles in familial adenomatous polyposis.
Br J Surg 78: 321 325
32. Chung RS, Church JM, van Stolk R (1995) Pancreas-sparing
duodenectomy: indications, surgical technic and results. Surgery
117: 254 259
Springer-Verlag 1998
O R I G I N A L A RT I C L E
Introduction
44
Results
Open
resection
(n = 34)
121 (75 205)
352 (160 410)
16
74
24.4 (21 27)
* P < 0.05
Reoperation
Bleeding
Ureter damage
Anastomotic insufficiency
Pneumonia
Urinary tract infection
Thrombosis
Wound infection
a
Laparoscopic
resection
(n = 25)
Open
resection
(n = 34)
2/25 a
2/25
2/34 a
4/34
6/34
2/34
7/34
Clinically symptomatic leak successfully treated by i. v. antibiotics and parenteral nutrition, no reoperation required
45
Table 3 Postoperative course following laparoscopic and conventional sigmoid resection for diverticular disease
Laparoscopic
resection
(n = 25)
Open
resection
(n = 34)
3.7 *
(3 5)
5.3
(3 7)
4.1 *
(3 10)
5.8
(4 10)
1/25 *
16/34
0.05 *
1.5
(1 14)
Length of postoperative
stay (days)
7.9 *
(6 14)
14.3
(9 23)
* P<0.05
Discussion
71% and 74% of laparoscopic and nonlaparoscopic patients. The length of the resected bowel did not differ
between the two techniques.
Postoperative complications in each of the treatment
groups are shown in Table 2. Four were recorded in the la-
Laparoscopic techniques should be assessed critically. Frazier and Mosteller [6] emphasize such outcomes of treatment as morbidity, stabilization or restoration of function,
pain, the financial impact of treatment, applicability of the
technique, patient satisfaction, overall quality of life, and
unwanted side effects related to the treatment itself. The
outcome of patients undergoing laparoscopic sigmoid resection for diverticular disease is not well documented in
the literature, however. Only a few publications [7, 8] have
examined at laparoscopic treatment of diverticular disease
in depth. Few series address morbidity, mortality, outcome,
or cost specifically.
A substantial selection bias is found in all laparoscopic
series. Ramos and coworkers [9] considered 200 patients
with various colorectal diseases and concluded that laparoscopic surgery was inappropriate in 105, who were operated on by standard surgery. Laparoscopic surgery, was attempted in 95 cases and was successful in 62, with convertion in 33. Thus only 62 of 200 patients (31%) were suitable for laparoscopic surgery. Until now no precise data on
laparoscopy for diverticular disease have been published.
In our own series we were able to perform a laparoscopic sigmoid resection in 25 of 29 cases (86%). More experience, better instruments, and more training may in-
46
Operative time
Instruments
Clinic costs
(surgeon, nurse)
Hotel costs
Wound morbidity
Time off work
Total costs
Conventional
resection
Laparoscopic
resection
14 days = 1750 DM
1000 DM
20 days = 2200 DM
8975 DM
8 days = 960 DM
15 days = 1600 DM
7185 DM
the incisional hernias subsequently require surgery and relaparotomy for wound dehiscence, which is also expensive. This increased wound morbidity would cost about
100,000 DM per 100 patients operated on. Thus 1000 DM
would be saved per patient if patients underwent laparoscopic procedure [19]. In calculating the cost of a laparoscopic sigmoid resection, this wound morbidity must be
considered (Table 4). Liberman [7] and Bruce [8] published comparable data. However, lost-benefit and cost-effectiveness analyses for laparoscopic and conventional
techniques are still missing
References
1. Mennigen R, Vestweber KH, Ure B, Troidl H (1990) Kolondivertikulitis: Wann operieren? Wie operieren? Medwelt 41:
721 725
2. Corman ML (1983) Colon and rectal surgery, 3rd edn. Lippincott, Philadelphia
3. Rothenberger D, Waltz O (1993) Surgery for complicated diverticulities. Surg Clin North Am 73: 975 992
4. Schauer P, Ramos R, Ghiatas A (1992) Virulent diverticular disease in young obese men. Am J Surg 164: 443 451
5. Forde KA, Hultn L (1996) Laparoscopy in colorectal surgery.
Surg Endosc 10: 1039 1040
6. Frazier HS, Mosteller F (1995) Medicine worth paying for. Assessing Medical Innovations. Havard University Press, Cambridge
7. Liberman MA, Phillips EH, Carroll BJ, Fallas M, Rosenthal R
(1996) Laparoscopic colectomy vs traditional colectomy for diverticulitis. Surg Endosc 10: 15 18
8. Bruce CJ, Coller JA, Murray JJ, Schoetz DJ, Roberts PL, Rusin
LC (1996) Laparoscopic resection for diverticular disease. Dis
Colon Rectum 39: S1 S6
9. Ramos JM, Beart RW, Goes R, Ortega AE, Schlinkert RT (1995)
Role of laparoscopy in colorectal surgery. Dis Colon Rectum
38: 494 501
10. Slim K, Pezet D, Riff Y, Clark E, Chipponi J (1995) High morbidity rate after converted laparoscopic colorectal surgery. Br J
Surg 82: 1406 1408
11. Ortega AE, Beart RW, Steele GD (1995) Laparoscopic bowel
surgery registry. Dis Colon Rectum 38: 681 686
12. Begos DG, Arsenault J, Ballantayne GH (1996) Laparoscopic
colon and rectal surgery at a VA hospital. Surg Endosc
10: 1050 1056
13. Lacy AM, Garcia-Valdecasas JC, Piqu JM (1995) Short-term
outcome analysis of a randomized study comparing laparoscopic vs. open colectomy for colon cancer. Surg Endosc 9:
1101 1105
47
14. Tate JJT, Kwok S, Dawson JW, Lau WY, Li AKC (1993) Prospective comparison of laparoscopic and conventional anterior
resection. Br J Surg 80: 1396 1398
15. Turner JA, Deyo RA, Loeser JD, Von Korff M, Fordyce WE
(1994) The importance of placebo effects in pain treatment and
research. JAMA 271: 1609 1614
16. Gellmann L, Salky B, Edye M (1996) Laparoscopic assisted colectomy. Surg Endosc 10: 1041 1044
17. Wexner SD, Reissmann P, Pfeifer J, Bernstein M, Geron N
(1996) Laparosocopic colorectal surgery. Surg Endosc 10:
133 136
Springer-Verlag 1998
O R I G I N A L A RT I C L E
I. N. Nomikos
Introduction
49
Table 1 Data on patients with Fourniers disease. (IRF Ischiorectal fossa, FIR femoro-inguinal region, SCR scrotum, LAW lower abdominal wall, LM labia majora, PRS pararectal space)
Patients
Sex
Age
(years)
Predisposing
factors
Septic
shock
on admission
Extent of soft
tissue necrosis
Postoperative
complications
Colostomy
Perineal
reconstruction
Outcome
29
Obesity,
delayed hosp.
transfer
Yes
IRF, FIR
SCR
Septicemia
No
Surgical
Survived
67
Delayed hosp.
transfer
Yes
IRF, SCR
LAW
Liver dysfunction,
paralytic ileus,
temporary anal
incontinence
No
Surgical
Survived
77
Latent rectal
perforation
No
IRF, LM
Paralytic ileus,
pulmonary
embolism
Yes
49
Obesity,
delayed hosp.
transfer
Yes
IRF, PRS
SCR
Cecum perforation,
gangrenous
cholecystitis
No
Surgical
Survived
57
Diabetes,
delayed hosp.
transfer
Yes
IRF, PRS
LM
Septicemia,
temporary anal
incontinence
Yes
Spontaneous
Survived
42
Unknown
Yes
No
Spontaneous
Survived
57
Unknown
No
IRF, SCR
No
Surgical
Survived
Died
scrotum or the space underlying the labia majora and/or the lower
abdominal wall. The wounds were packed open, and the dressings
were changed at least twice daily. During each dressing replacement
course the wound was irrigated with normal saline (or tap water, if
the patient was able to walk to the bathroom). Depending on the extent of the perineal or perineoscrotal wound either spontaneous healing by secondary intention or reconstructive surgical procedure was
attempted after the appearance of healthy granulation tissue at the
base of the wound.
Results
There were five men and two women, ranging in age from
29 to 77 years (mean 54). A factor predisposing to fulminant progression of perineal gangrene was found in all
but two patients. A delay in initial admission to hospital and
consequent surgical intervention were considered in four
patients as a determining factor. In contrast to the mean
interval between initial symptoms and diagnosis of 6.3 days
(range 3 11), for these four patients (patients 1, 2, 4, and
5 of Table 1) this was 9.2 days. Two of them were obese,
and one suffered from insulin-dependent diabetes. One patient developed perineal gangrene due to accidental rectal
perforation made during a cathartic enema (Fig. 1).
In all patients the infection originated from the anorectal area and proved to be polymicrobial in nature (Table 2).
Broad-spectrum antibiotics active against gram-positive
cocci, facultative anaerobic gram-negative rods, and anaerobic organisms were administered immediately after
samples of pus or tissue were taken for culture. The patients received intravenous antibiotic therapy for a mean
period of 11.5 days. The most common combination used
Fig. 1 Edema, redness, and necrosis of overlying skin were the local findings in a 77-year-old woman with Fourniers disease after accidental rectal perforation during a cathartic enema
50
Table 2 Bacteriology of patients with Fourniers gangrene (n = 7)
n
Aerobes
E. coli
S. faecalis
Proteus mirabilis
Klebsiella pneumoniae
S. epidermidis
5
3
3
2
1
Anaerobes
Bacteroides fragilis
Bacteroides melaninogenicous
Gram positive cocci
Clostridium spp.
6
3
3
2
deemed necessary in two patients. Temporary anal incontinence appeared in two patients. Two patients developed
long-lasting adynamic ileus. One patient underwent a right
hemicolectomy and 10 days later had a cholecystectomy
for gangrenous alithiasic cholecystitis. One patient developed hepatic dysfunction during his septic syndrome.
A spontaneous closure of the wound was achieved only
in two patients (patients 5 and 6 of Table 1). No reconstructive procedure was undertaken in the others, but a delayed
primary closure of the scrotal and perineal skin was possible without tension after the appearance of granulation
tissue at the base of the wound. There was one death due
to pulmonary embolism. The remaining six patients survived the septic process and were discharged from hospial
(mortality 14.2%). During the follow-up period (from
6 months to 12 years) only one of the survivors developed
fisulae in ano 4 months after his initial management.
Table 1 presents the basic data of our patients with
Fourniers disease.
Discussion
Fourniers gangrene is a potentially fatal soft tissue infection of the perineum that rapidly invades the surrounding
tissues. Alcoholism, diabetes mellitus, and obesity have
been cited as main predisposing factors. However, delay
in seeking medical treatment seemed to be a major factor
in our series. In aged, immunocompromized, or debilitated
patients the disease may be silent, whereas the clinical picture of septic syndrome predominates in younger adults.
The potential causes that are directly related to the disease
can be classified into two groups: (a) infections of cutaneous, urogenital, or rectal origin and (b) complications of
surgical procedures such as hemorrhoidectomy, orchiectomy, herniorrhapy, or vasectomy [1, 2]. No apparent cause
is detected in 30% of cases. Although the disease predominates in males, its appearance in two women in this
series may be due to an accidental event (rectal perforation by a cathartic enema) in one patient and in the other
to delay in hospital admission (9 days after the onset of
symptoms) and the coexistence of insulin-dependent diabetes mellitus which caused an insidious course.
Scarpas fascia necrosis allows for the spread of inflammation to the abdominal wall (as occurred in two of our
patients) [3]. Usually the scrotal skin is affected by the septic process (due to obliterating endarteritis) secondary to
microbial invasion of subcutaneous tissue. In contrast, the
testes are invariably spared of the infection because of their
separate blood supply [1].
Persistent paralytic ileus, as seen in three of our patients,
is an interesting finding not mentioned in the literature.
The etiology of ileus remains obscure; reflex sympathetic
activity mediated by extensive scrotal inflammation
probably plays a role. Elevated levels of circulating catecholamines may also inhibit peristalsis and cause
adynamic ileus, as has been suggested previously [3, 4].
Gram stain is most helpful in demonstrating the characteristic synergism of aerobes (gram-positive cocci and
gram-negative bacilli) and anaerobes. Proper and extensive dbridement of all necrotic tissue is an absolute requirement for a successful outcome. Likewise, sinus tracts
must be fully unroofed. The incision may require extension into the buttocks, highs, perineal region, or inguinal
canal depending upon the areas involved. The wound
should remain open and covered with dressings, which
should be changed two or more times daily. The wound
must be irrigated each time with normal saline or tap water with simultaneous dbridement of all necrotic tissue.
Simultaneous adjuvant supportive treatment is a prerequiste for a successful outcome.
With appropriate treatment, granulation tissue appears
in the base of the wound, and primary wound closure is
possible in most of the cases. Since the scrotal skin is elastic and has the capability of rapid regeneration, large defects in this area can heal spontaneously. However, may
need to be reconstructed surgically by one of the following techniques: delayed primary surgical closure of the
scrotum, skin grafting, implantation of the testes in a subcutaneous abdominal or medial thigh pocket, and rotating
flaps from the inguinal region.
None of our patients received hyperbaric oxygen as an
adjuvant treatment although this has been suggested as an
initial step for the control of local disease. The value of hyperbaric oxygen therapy has been debated in the literature,
with some studies finding a survival benefit from its use
and others finding no benefit. Hyperbaric oxygen could
play a favorable role as an adjuvant modality of treatment
to lower the mortality and morbidity of the disease. Its use
is based mainly on: (a) the presence of anaerobic and
facultative anaerobic bacteria in most of the cases,
(b) the presence of endarteritis and septic vasculitis as a
pathological feature leading to ischemia, and (c) the
marked edema which is due to the inflammatory process
and to venous thrombosis [5]. However, although evidence
for decreased mortality seems convincing when hyperbaric
oxygen is used for clostridial myonecrosis, no survival
benefit has been well demonstrated by a prospective, randomized, controlled trial for hyperbaric oxygen used for
nonclostridial necrotizing soft tissue infections [6].
Despite optimal medical and surgical treatment, mortality rates in Fourniers gangrene range between 8% and
51
References
1. Lauks SS II (1994) Fourniers gangrene. Surg Clin North Am
74: 1339 1352
Springer-Verlag 1998
L E T T E R TO T H E E D I TO R
Dear Sir,
Electrical stimulation by superficial
electrodes has been established in the
diagnostic workup of pudendal neuropathy [1 3]. The latency of the terminal segment of the pudendal nerve
can be determined by this method
with St Marks pudendal electrode
(Dantec Company, Skovlunde, Denmark). With this standard electrode,
the stimulating electrodes are placed
at the tip of the examining finger and
the recording electrodes at the base.
This arrangement can lead to a methodical error, owing to the fact that
the different and indifferent electrodes are attached to the base of the
finger such that stimulation of the
right pudendal nerve gives a negative
recording of the potential. In contrast,
stimulation of the left pudendal nerve
yields a positive response since the
finger changes sides. In this case, considerable portions of the right external anal sphincter muscle are adding
to this potential. This may explain
why many researchers are measuring
the latency of the right pudendal
nerve only.
B
5
References
B
6
Springer-Verlag 1998
L E T T E R TO T H E E D I TO R
Sir,
We enjoyed reading the article by
Leroi et al. (Int J Colorect Dis 12:
243245), which clearly demonstrates that the management of anorectal incontinence in patients with
isolated internal anal sphincter injury is difficult. In keeping with the
findings of the above study, we have
disappointing failures with direct
internal anal sphincter repair. By
contrast, our results with anoplasty
[1] which comprise filling the defect
in the anal canal with skin and sub-
Reference
1. Morgan AR, Patel B, Beynon J, Carr ND
(1997) Surgical management of anorectal incontinence due to internal anal
sphincter deficiency. Br J Surg 84:
226230
Yours sincerely,
F. Abbasakoor () A. R. Morgan
J. Beynon N. D. Carr
Department of Surgery
Singleton Hospital
Swansea SA2 8QA
UK
Springer-Verlag 1998
L E T T E R TO T H E E D I TO R
Dear Sir,
We appreciated very much the paper
by Dworak et al. [1] and feel flattered that their classification of rectal cancer regression after preoperative radiotherapy (RT) and chemotherapy (CT) is quite similar to that
which we reported [2] 2 years ago in
this journal for rectal cancer patients
receiving preoperative RT. This
system was adopted by our pathologist (S.A.) strictly following Mandards [3] proposal for staging the response of the esophageal carcinoma
to the preoperative therapy.
To date we have treated 53 patients, with the following results: (a)
a complete pathological disappearance of the tumor in 3 patients (6%)
and (b) a regression greater than 50%
in an other 36 patients. These results
are in agreement with data in the literature [4 8] which report a complete response rate in 9% of patients
(range 5 14%) receiving preoperative RT.
However, the main questions that
we are now faced with differ from
those of Dr. Dworak. We think that it
is important to demonstrate whether:
(a) preoperative RT and CT are able
to decrease local recurrence and distant metastases compared to a control
group, (b) tumor regression after preoperative therapy is associated with
a more favorable outcome, and (c) it
is possible to predict in some way
(e.g., by biological markers) the response to therapy prior to initiating
it. We cannot overemphasize the re-
F. Bozzetti ()
Department of Digestive Tract Surgery
Istituto Nazionale per lo Studio e la Cura
dei Tumori
Via Venezian, 1
I-20133 Milano, Italy
S. Andreola
Department of Pathology
Istituto Nazionale per lo Studio e la Cura
dei Tumori
Via Venezian, 1
I-20133 Milano, Italy
L. Bertario
Department of Digestive Tract Surgery
Istituto Nazionale per lo Studio e la Cura
dei Tumori
Via Venezian, 1
I-20133 Milano, Italy
References
1. Dworak O, Keilholz L, Hoffmann A
(1997) Pathological features of rectal
cancer after preoperative radiochemotherapy. Int J Colorect Dis 12:1923
2. Bozzetti F, Andreola S, Rossetti C,
Zucali R, Meroni E, Baratti D, Bertario
L, Doci R, Gennari L (1996) Preoperative radiotherapy for resectable cancer
of the middle-distal rectum: its effect on
the primary lesion as determined by
endorectal ultrasound using flexible
echo colonoscope. Int J Colorect Dis
11: 283286
3. Mandard AM, Dalibard F, Mandard JC,
Marnay J, Henry-Amar M, Petiot JF,
Roussel A, Jacob JH, Segol P, Samama
G, Ollivier JM, Bonvalot S, Gignoux M
(1994) Pathologic assessment of tumor
regression after preoperative chemoradiotherapy of esophageal carcinoma.
Cancer 73: 26802686
4. Minsky BD, Cohen AM, Ender WE,
Paty P (1995) Sphincter preservation
with preoperative radiation therapy and
coloanal anastomosis. Int J Radiat
Oncol Biol Phys 31: 553559
55
5. Mohiuddin M, Marks G (1991) High
dose preoperative irradiation for cancer
of the rectum, 19761988. Int J Radiat Oncol Biol Phys 20: 3743
6. Marks G, Motriuddin M, Masoni L
(1993) The reality of radical sphincter
preservation surgery for cancer of the
distal 3 cm of rectum following highdose radiation. Int J Radiat Oncol Biol
Phys 27: 779783