Cronkhite-canada syndromeQ87.82

Authors:Prof. Dr. med. Peter Altmeyer, Prof. Dr. med. Guido Gerken

All authors of this article

Last updated on: 29.10.2020

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Synonym(s)

Alopecia and fingernail changes; Diffuse gastrointestinal with ectodermal changes; Polyposis diffuse gastrointestinal with ectodermal changes; Polyposis skin pigmentation alopecia and fingernail changes

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HistoryThis section has been translated automatically.

Cronkhite and Canada, 1955

DefinitionThis section has been translated automatically.

Very rare, non-familial, juvenile polyposis syndrome (see below hereditary polyposis syndromes), which often occurs in combination with gastrointestinal carcinomas, skin changes, eye malformations, spasticity and severe developmental delay.

EtiopathogenesisThis section has been translated automatically.

Unsolved.

ManifestationThis section has been translated automatically.

After the age of 50.

Clinical featuresThis section has been translated automatically.

Generalized intestinal polyposis: malabsorption, therapy-resistant diarrhea 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 hypopigmentations. Mental retardation with cramps, spasticity, scoliosis, microphthalmia, secondary ectropion, amaurosis, cryptorchidism, growth retardation.

TherapyThis section has been translated automatically.

Treatment by internists and surgeons. Due to the severity of the gastrointestinal disorders, treatment of the skin symptoms is usually of secondary importance. They rather serve the diagnostic classification of the clinical picture.

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.

  1. Courtens W, Broeckx W, Ledoux M, Vamos E (1989) Oculocerebral hypopigmentation syndrome (Cross syndrome) in a gypsy child. Acta Paediatr Scand 78: 806-810
  2. Cronkhite LW, Canada WJ (1955) Generalized gastrointestinal polyposis: An unusual syndrome of polyposis, pigmentation, alopecia and onychotrophia. N Engl J Med 252: 1011
  3. Cross HE, McKusick VA, Breen W (1967) A new oculo-cerebral syndrome with hypopigmentation. J Pediatr 70: 398-406
  4. Lynch HT, de la Chapelle A (2003) Hereditary colorectal cancer. N Engl J Med 348: 919-932
  5. Marra G, Jiricny J (2003) Multiple colorectal adenomas - is their number up? N Engl J Med 348: 845-847
  6. Yood MU et al (2003) Colon polyp recurrence in a managed care population. Arch Internal Med 163: 422-426

Authors

Last updated on: 29.10.2020

Authors: Prof. Dr. med. Peter AltmeyerProf. Dr. med. Guido Gerken