Congenital scalp/calvarial defects classification



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CONGENITAL SCALP/CALVARIAL DEFECTS
Classification (Perlyn PRS 2005)
Class I: Open defects of the scalp alone

A. Less than 2 cm in diameter

B. Two to 5 cm in diameter

C. Greater than 5 cm in diameter

Class II: Open defects of the skull alone

A. Less than 2 cm in diameter

B. Two to 5 cm in diameter

C. Greater than 5 cm in diameter

D. Any above size with:

(+) herniated meningeal/neural tissue

(-) no herniated meningeal/neural tissue

Class III: Open defects of the skull and scalp

A. Less than 2 cm in diameter

B. Two to 5 cm in diameter

C. Greater than 5 cm in diameter

D. Any above size with:

(+) herniated meningeal/neural tissue



(-) no herniated meningeal/neural tissue

Aetiology
Aplasia Cutis Congenita

  • heterogenous group of disorders characterized by the absence of a portion of skin in a localized or widespread area at birth.

  • most commonly (70%) manifests as a solitary defect on the scalp, but sometimes it may occur as multiple lesions.


Pathogenesis

  • Theories

    1. Neural tube defects

    2. amniotic bands

    3. placental infarcts

    4. thrombosed arteriovenous malformations in utero

  • Defects in the skin that form early in gestation may heal before delivery and appear as an atrophic, membranous, or parchmentlike scar with associated alopecia, whereas less mature defects present as ulcerations.


Incidence

  • 3 in 10,000 births

  • Sporadic, AD and AR reported

  • Associations

    • Adams-Oliver syndrome

      • AD

      • distal limb reduction abnormalities (hypoplastic or absent distal phalanges) are found in association with solitary midline scalp defects.

    • epidermal and sebaceous (organoid) nevi, which also involve the scalp, usually adjacent to the cutis aplasia.

    • simplex, junctional, or dystrophic types of epidermolysis bullosa


Histopathology

  • lesions are noninflammatory and well demarcated

  • thin layer of thin fibrous membrane composed of dermal collagen without overlying epithelium or adnexal structures. There also is marked absence of elastic fibers


Clinical

  • appearance of the lesions varies, depending on when they occur during intrauterine development.

  • At birth, the lesions may have already healed with scarring or may remain superficially eroded to deeply ulcerated, occasionally involving the dura or the meninges.

  • Most areas of involvement are located over the parietal regions and over the vertex between the anterior and posterior fontanels; 80% are midline

  • Defects may also occur on the face, the trunk, or the limbs, sometimes symmetrically.

  • Average size of an affected area is 1 to 2 cm in diameter, although cases of greater than 10 cm in diameter have been reported

  • Distorted hair growth around a scalp lesion, known as the hair collar sign, is a marker for underlying defects.

  • 20% of infants with aplasia cutis congenita will also have osseous defects

  • Exposure of the dura matter, which is often attenuated or absent, may expose the sagittal sinus as well. All such defects increase the possibility of potentially fatal complications such as hemorrhage and meningeal infection

  • Fatal bleeding from the sagittal sinus has been described; this is often heralded by several smaller bleeds before a fatal event occurs

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