Acquired Lückenschädel in a Toddler with Craniosynostosis and Semilobar Holoprosencephaly

The pathogenesis of Lückenschädel, or lacunar skull deformity, an abnormality of skull development that is found most commonly in children with neural tube defects, remains unknown. Major theories regarding its formation include changes in intracranial pressure and disorganized ossification of the skull. There have been only 2 reports of lacunar skull deformity in children with craniosynostosis and none in children with holoprosencephaly. Here, we present the case of a child with semilobar holoprosencephaly who developed progressive multisutural craniosynostosis and hydrocephalus and eventually demonstrated multiple meningoceles that herniated through acquired skull defects. We postulate a causal relationship between raised intracranial pressure and lacunar skull deformity in our patient and offer a review of the current literature on lacunar skull deformity.