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[Cutaneous signs of occult cranial and spinal dysraphism]

D Bessis1, 1

  • 1Département de dermatologie, hôpital Saint-Éloi, CHRU de Montpellier, 80, avenue Augustin-Fliche, 34295 Montpellier cedex 5, France; Université Montpellier, 163, rue Auguste-Broussonnet, 34090 Montpellier, France; Inserm 1058, 60, rue de Navacelles, 34394 Montpellier cedex 5, France.

Annales De Dermatologie Et De Venereologie
|April 29, 2020
PubMed

Insights

Skin signs can indicate underlying neural tube defects like cranial dysraphism (CD) and occult spinal dysraphism (OSD). Early identification of these midline lesions is crucial for timely diagnosis and management.

Area of Science:

  • Neurology
  • Dermatology
  • Pediatrics

Background:

  • Dysraphism encompasses neural tube and midline closure defects affecting skin, muscles, vertebrae, and meninges.
  • Cranial dysraphism (CD) and occult spinal dysraphism (OSD) can manifest through distinct skin signs, detectable from birth through adulthood.

Purpose of the Study:

  • To review and categorize the various skin manifestations associated with cranial dysraphism (CD) and occult spinal dysraphism (OSD).
  • To highlight the diagnostic significance of congenital midline skin lesions in suspecting underlying dysraphic conditions.

Main Methods:

  • Literature review focusing on skin signs linked to CD and OSD.
  • Categorization of skin abnormalities based on their association with specific types of dysraphism (e.g., DCEO, DSO).

Main Results:

  • Congenital midline skin lesions, especially on the face, scalp, neck, and lower back, warrant suspicion for dysraphism.
  • Specific skin findings include nodules, swelling, hair abnormalities (collar sign, tuft), dimples, fistulas, hemangiomas, appendages, and lipomas.
  • Magnetic resonance imaging (MRI) is the gold standard for diagnosing suspected DCEO or DSO.

Conclusions:

  • Evocative skin signs are critical indicators of underlying CD and OSD.
  • Prompt medical evaluation of midline skin lesions is essential for early diagnosis and intervention.
  • MRI is indispensable for confirming suspected dysraphic conditions.

Keywords:
Aplasie cutanéeAppendice caudalCapillary malformationCephaloceleCollier de cheveuxCutaneous aplasiaCéphalocèleDermoid cystDermoid fistulaDysraphieDysraphismFistule dermoïdeFossette sacrococcygienneHair collar signHair tuftHuman tailHypertrichoseHypertrichosisHémangiome infantileHétérotopie neuroglialeInfantile haemangiomaKyste dermoïdeLipomaLipomesMalformation capillaireMoelle bas attachéeNeuroglial heterotopiaSacrococcygeal dimpleTethered cordTouffe de cheveux

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