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Most often autosomal dominant inherited disorder
characterized by palmoplantar keratosis in combination with gastrointestinal
cancer.
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Tylosis with Esophageal Cancer; Keratosis Palmoplantaris
with Adenocarcinoma of the Colon/Esophagus; Clarke Howel-Evans Syndrome;
Clarke Howel-Evans-McConnell Syndrome; Howel-Evans Syndrome; Bennion
Patterson Syndrome.
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First reported by Clarke and McConnell in 1954 and further
studied by William Howel-Evans et al. in 1958.
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Unknown. Originally described in two families from
Liverpool, UK. The syndrome has now also been described in many other
countries and ethnicities.
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A wide heterogeneity and variety of genes
have been implicated. It is most often inherited as an autosomal dominant
trait with full penetrance. The tylosis with esophageal cancer gene (TOC) is
located in the 17q23-qter region, telomeric to the type I keratin gene
cluster. Regions 17q24 and 17q25 may also be involved.
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The cause of tylosis and esophageal carcinoma is
unknown, but the association with cancer may represent a contiguous gene
syndrome.
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Based on the findings of palmoplantar keratosis, oral
leukoplakia, and squamous cell carcinoma of the esophagus. Hyperkeratosis
limited to palms and soles, with the keratoderma stopping abruptly at the
lateral margins and not extending onto the dorsum. The epidermis from
biopsies of affected skin sites shows gross acanthosis, hyperkeratosis, and
hypergranulosis.
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The association of hyperkeratosis of soles and
palms with various malignancies in several members of a family is
characteristic for this syndrome. Tylosis usually appears between 5 and 15
years of age. The typical neoplastic manifestation is carcinoma of the
esophagus and colon, but there is also an increased risk for other
malignancies such as endometrial carcinoma or melanoma. Hyperkeratosis can
also be found in other areas that are repeatedly exposed to mechanical
trauma, and enoral leukoplakia is common. Typically, hyperkeratosis appears
in adolescence, and malignancies occur after the third decade of life.
Development of esophageal carcinoma after the third decade is almost
certain, estimated to be 95% by age 65 years. Recognition of tylosis
allows better surveillance for the development of esophageal cancers.
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During childhood, the syndrome is
unlikely to have any specific implications for anesthesia, as these are
related to the presence of malignancies and their medical and surgical
treatment. Determine the competency of the lower esophageal sphincter. A history of
heartburn, reflux, or regurgitation may be elicited. Determine whether
obstruction of the esophagus is present. Malnutrition is common in patients
with esophageal carcinoma, and preoperative parenteral nutrition may have a
role in improving the reserves and immunity status. Preoperative
investigations in these patients should include chest radiographs, lung
function tests including arterial blood gas analysis to evaluate if one-lung
anesthesia would be tolerated, a complete blood count, coagulation status,
urea and electrolyte levels, nutritional markers (e.g., serum protein, albumin),
and an electrocardiogram.
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During childhood, the syndrome is
unlikely to have any specific implications for anesthesia, as these are
related to the presence of malignancies and their medical ...