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Editor,—We compliment Vasen et al(Gut 1997;40:716–19) on their attempt to quantify the benefit of surveillance of the duodenum in patients with familial adenomatous polyposis (FAP). We should like to make the following points:
The St Marks’s study1 is not strictly comparable to the Scandinavian study in that in the former results are based on the use of a side-viewing endoscope to focus particularly on the high risk periampullary area, whereas the Scandinavian study used forward viewing endoscopes only.
Vasen et al state that pancreaticoduodenectomy has substantial morbidity and mortality; this statement contradicts another within the body of the text referring to the decline in mortality from this operation over the past decade. It would be fair to say that the operation has the potential for substantial morbidity and mortality. However, this potential has not been fulfilled, especially in the context of a performance of a pylorus-sparing pancreaticoduodenectomy (E Tiret and C Penna, Hôpital St Antoine, Paris, France, personal communication), together with encouraging results with regard to the lack of recurrence of duodenal adenomas.
We would suggest that a baseline screening examination be done before the age of 30. This might be done at the time of colectomy. This may be particularly worthwhile in those patients with a family history of duodenal cancer where such an examination would hopefully also reassure them.
There is additional evidence to that cited in favour of the existence of the adenoma–carcinoma sequence in the duodenum.2 For example, adenomas were found in or adjacent to duodenal cancer in 84% (38/45) of patients with FAP.
We agree that the results of surveillance should be collected in a uniform manner at a central registry. In this way the quoted criteria for population screening, namely the natural history of duodenal ademonas, the availability of curative treatment and evidence that this treatment leads to improved prognosis can be assessed. Without surveillance of these individuals, the natural history will remain unknown. Treatments will not be able to be instituted until symptoms, which are usually a marker of advanced disease, occur. The incentive for testing novel treatments, such as Sulindac and its derivatives, COX 2 agents, photodynamic laser therapy, antacid administration or gene therapy, would not be as great.
Finally, a point of personal sensitivity. Vasen et al’s reference to the “so-called” Spigelman classification neglects to say that this classification was “called-so” in the literature by the Scandinavian group itself!3 Whilst we remain in favour of eponymous labelling of conditions4 and are indeed gratified to see that these authors have adopted the term Bussey–Gardner polyposis, the stimulus for labelling the classification of severity of upper gastrointestinal disease in FAP did not come from the St Mark’s group.
Editor,—We thank Drs Spigelman and Phillips for their remarks. We agree that the St Marks’s study and the Scandinavian study are not strictly comparable. Between the two studies, however, there was little significant difference in the detection rate of stage IV duodenal polyposis.
We do not believe that our statements on the mortality associated with pancreaticoduodenectomy are contradictory, as we still consider a mortality of 5% to be substantial, especially if this surgical procedure is performed in a relatively young patient with a disease that is potentially malignant but still benign (stage IV duodenal polyposis). An important issue which was not mentioned in our manuscript is that recent studies indicate that mortality is significantly higher in hospitals with limited experience (fewer than five procedures per year).1 2 We would therefore like to emphasise that such procedures should be performed at centres in which experience is available.
The disadvantage of recommending a baseline duodenoscopy before the age of 30 is that if abnormalities are found, most physicians will decide to continue (frequent) examinations, thereby imposing an additional burden on the patient. Although exceptions can be made for patients seeking reassurance, we prefer to make a decision on the basis of the natural history of the disease, which indicates that duodenal cancer before 30 years of age is extremely rare.
We regret that our comments on the Spigelman classification gave the impression that we dislike the name of this classfication. On the contrary, we believe that the classification proposed by Spigelman is very useful and fully deserves his name.
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