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A transitory disease characterized by punched-out
skull defects.
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Lacunar Skull; Lückenschädel.
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Craniolacunia is considered a mesenchymal
dysplasia affecting the calvarial ossification. It may be an isolated
finding; however, most often it is associated with defects in neural tube
closure, such as Arnold-Chiari Syndrome Type II, myelomeningocele, and
encephalocele. Occasionally it occurs with other cerebral lesions. The exact
etiology of the defects is unknown, however, a suggestion has involved the
lack of cerebral ventricular distension as a result of the open neural tube,
which then results in a lack of distension of the fetal brain, which is
necessary to induce normal development of the membranous plates of the fetal
calvaria. Instead, abnormally organized collagen fibers are produced and
their ossification results in the defects seen in craniolacunia. The fact
that the defects resolve by 6 months of age has been explained with remodeling
in response to either normal expansion of cerebral tissue or development of
hydrocephalus. The most commonly affected bones are the parietal and
occipital bones. The lesions appear as multiple, “punched-out” skull defects
with well-defined borders and bony trabecular ridges involving the inner
table and the diploe (less commonly the outer table) with a diameter of up
to 1 cm. Generally, craniolacunia is not associated with increased
intracranial pressure and appears not to affect neurologic outcome.
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Craniolacunia per se should not affect
anesthesia. However, it seems wise to avoid any pressure on the skull,
especially when handling or positioning the baby. Other anesthesia
considerations are guided by the underlying disease (e.g., Arnold-Chiari
Syndrome, myelomeningocele).
Wilson WG, Alford BA, Schnatterly PT: Craniolacunia as the result of
compression and decompression of the fetal skull.
Am J Med Genet 27:729, 1987.
[PubMed: 3307412]
Coley BD: Ultrasound diagnosis of luckenschadel (lacunar skull).
Pediatr Radiol 30:82,
2000.
[PubMed: 10663516]