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A transitory disease characterized by punched-out skull defects.

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Lacunar Skull; Lückenschädel.

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Unknown.

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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]

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