Synonym(s)
HistoryThis section has been translated automatically.
DefinitionThis section has been translated automatically.
Very rare, non-familial, non-juvenile polyposis syndrome (see hereditary polyposis syndromes below), which often occurs in combination with gastrointestinal carcinomas, skin changes, eye malformations, spasticity and severe developmental delay.
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EtiopathogenesisThis section has been translated automatically.
ManifestationThis section has been translated automatically.
After the age of 50.
Clinical featuresThis section has been translated automatically.
Generalized intestinal polyposis: malabsorption, therapy-resistant diarrhoea with electrolyte and protein losses. Universal, non-scarring alopecia of all terminal hairs, onychodystrophy and skin pigmentation in the form of pinhead-sized, roundish, brownish lentigines on the palms of the hands and soles of the feet. Also periungual. Also hypopigmentation. Mental retardation with cramps, spasticity, scoliosis, microphthalmia, secondary ectropion, amaurosis, cryptorchidism, growth retardation.
DiagnosisThis section has been translated automatically.
Digital rectal examination, recto/colonoscopy, HD endoscopy (=high definition endioscopy). If necessary, rectal endosonography for larger rectal adenomas to clarify endoscopic ablation and depth of penetration (carcinoma formation). Possibly MRI sellink of the small intestine and video capsule endoscopy to detect small intestinal polyps in hereditary polyposis syndrome.
TherapyThis section has been translated automatically.
Progression/forecastThis section has been translated automatically.
Unfavourable: lethal outcome in 50% of cases, on average 18 months after the onset of refractory diarrhoea. Risk of colorectal cancer is not increased.
LiteratureThis section has been translated automatically.
- Courtens W, Broeckx W, Ledoux M, Vamos E (1989) Oculocerebral hypopigmentation syndrome (Cross syndrome) in a gypsy child. Acta Paediatr Scand 78: 806-810
- Cronkhite LW, Canada WJ (1955) Generalized gastrointestinal polyposis: An unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N Engl J Med 252: 1011
- Cross HE, McKusick VA, Breen W (1967) A new oculo-cerebral syndrome with hypopigmentation. J Pediatr 70: 398-406
- Liu Y et al. (2020) Cronkhite-Canada syndrome: report of a rare case and review of the literature. J Int Med Res 48:300060520922427.
- Lynch HT, de la Chapelle A (2003) Hereditary colorectal cancer. N Engl J Med 348: 919-932
- Marra G, Jiricny J (2003) Multiple colorectal adenomas - is their number up? N Engl J Med 348: 845-847
- Miyawaki K et al. (2022) Cronkhite-Canada Syndrome Mimicking COVID-19-related Symptoms. Acta Derm Venereol 102:adv00812
- Yood MU et al (2003) Colon polyp recurrence in a managed care population. Arch Intern Med 163: 422-426
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Incoming links (6)
Eye diseases, skin changes; Intestinal diseases, skin changes; Onychodystrophy (overview); Peutz-jeghers syndrome; Polyposis, diffuse gastrointestinal with ectodermal changes; Polyposis, skin pigmentation, alopecia, and fingernail changes;Outgoing links (1)
Polyposis syndromes, hereditary;Disclaimer
Please ask your physician for a reliable diagnosis. This website is only meant as a reference.
Prof. Dr. med. Peter Altmeyer
Prof. Dr. med. Guido Gerken
