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Hair collar sign

Hair collar sign. BY: MOHAMMED ALSAIDAN. Hair collar sign. Ring-like hypertrichosis consisting of coarser and longer hair surrounding a congenital alopecic lesion on the midline of the scalp.

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Hair collar sign

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  1. Hair collar sign BY: MOHAMMED ALSAIDAN

  2. Hair collar sign • Ring-like hypertrichosisconsisting of coarser and longer hair surrounding a congenital alopecic lesion on the midline of the scalp. • A marker of cranial dysraphism, including encephaloceles, meningoceles, Cutaneous meningothelial tumors (heterotropic brain tissue), dermoid cyst , aplasia cutis congenita, Dandy Walker malformation or agenesis of the corpus callosum • Due to neuroectodermal abnormalities in embryonic development of the cranial neural tube

  3. Hair collar sign • Encephaloceles and meningocelescommunicate directly to the central nervous system by herniation of intracranial structures through a scalp defect • Heterotropicbrain tissue is found with an intact cranium and no connectionto the brain

  4. Associations • Port wine stain • Subcutaneous hemangioma • the presence of a vascular malformation with a second cutaneous marker heightens the likelihood of dysraphism • Cardiac defect ( e.g. VSD) • Oculoectodermal syndrome : include epibulbardermoids and other ocular abnormalities, skin hyperpigmentation, congenital heart defects, urinary tract malformations, and neoplasms • Syndactyly, cleft lip and palate, sclerocornea, scalp epidermal nevus, lipodermoid also have been reported

  5. Approach • Present at birth ? Growing in size ? Bulging with crying ? • Thorough physical examination with neurologic evaluation • Brain MRI • Screening for spinal abnormalities (spine, spinal cord) • Genetic counseling ? Translocation t(1;4) (q44;q10). • Abdominal ultrasound, metabolic screening

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