Incidence of delayed intracranial hypertension in children with isolated sagittal synostosis following open calvarial vault reconstruction

McClugage, Samuel G.; Lepard, Jacob; Ray, Peter D.; Grant, John H.; Blount, Jeffrey P.; Rozzelle, Curtis J.; Johnston, James M.

doi:10.1007/s00381-019-04406-7

Cited by 6 publications

(17 citation statements)

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“…Delayed intracranial hypertension is defined as a postoperative pathologic elevation of elevated ICP in which clinical symptoms appear after a period of symptom-free follow-up. 5 The cause of DIH is widely unknown; it is speculated that a combination of risk factors and genetic factors such as fibroblast growth factor receptor mutations, premature fusion or restenosis of the same or other sutures, or venous outflow alterations may predispose a child to DIH. 20,21 Some studies report age and specific surgery type as risk factors with inconsistent findings.…”

Section: Discussionmentioning

confidence: 99%

“…Typical treatment for DIH is reoperation to expand the cranial vault. 5,6 Treatment for DIH is crucial as DIH may lead to chronic headaches, progressive neurocognitive deficits, visual changes, and developmental delays in children. 4,6 The need for reoperation for DIH following nonsyndromic single-suture sagittal craniosynostosis can occur at an incidence anywhere from 0.95% to 6.2%.…”

Section: Discussionmentioning

confidence: 99%

“…4,6 The need for reoperation for DIH following nonsyndromic single-suture sagittal craniosynostosis can occur at an incidence anywhere from 0.95% to 6.2%. 5,6,21 CM1 has long been associated with craniosynostosis, primarily multisuture and/or syndromic forms of the disease. 11 The mechanisms behind the association are thought to be craniocephalic disproportion and potentially anomalous intracranial venous pressure.…”

Section: Discussionmentioning

confidence: 99%

“…This phenomenon is usually addressed with repeat open CVR surgery. 5,6 Posterior vault distraction osteogenesis (PVDO) remains an option for postoperative DIH as well. 7 Unlike CVR, PVDO permits a more controlled and gradual expansion of the skull and scalp providing significant volumetric expansion.…”

mentioning

confidence: 99%

“…Delayed intracranial hypertension remains important clinically due to the association with chronic headaches, progressive neurocognitive deficits, developmental delay, and visual difficulty. This phenomenon is usually addressed with repeat open CVR surgery 5,6 . Posterior vault distraction osteogenesis (PVDO) remains an option for postoperative DIH as well 7 .…”

mentioning

confidence: 99%

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Chiari Malformation Type 1 and Syringomyelia in a Patient With Prior Surgically-Treated Sagittal Synostosis Effectively Treated With Posterior Cranial Vault Distraction Osteogenesis

Feller

Jensen

Lew

2021

Journal of Craniofacial Surgery

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The presenting report describes the use of a posterior cranial vault distraction procedure in resolving a Chiari malformation with associated syringomyelia following a surgically-treated nonsyndromic craniosynostosis. Chiari malformations are typically treated with posterior fossa decompression with or without expansion duraplasty. The objective of this report is to describe the effectiveness in resolving both Chiari malformation and secondary syringomyelia with posterior cranial vault distraction osteogenesis. A 5-year-old male, with a history of surgery for sagittal synostosis during infancy, presented with daily severe headaches and dysesthesias in the hands and feet. Imaging demonstrated a copper-beaten calvarium and a Chiari 1 malformation with a 7 mm diameter C4-T1 syrinx. He underwent posterior cranial vault distraction osteogenesis over 3 months to increase his intracranial volume. The patients' headaches improved significantly, and his dysesthesias resolved postoperatively. A magnetic resonance imaging performed 7 months after completion of distraction demonstrated resolution of the Chiari malformation and decompression of his syrinx. A computed tomography scan at 9 months postdistraction showed resolution of the copper-beaten calvarium. Patients with a history of craniosynostosis can develop inadequate cranial volume over time due to abnormal skull growth, leading to secondary Chiari malformation with or without syringomyelia. Posterior vault distraction is an effective strategy to address these conditions and can be employed later in childhood to treat the underlying pathology. The mechanism potentially expands both calvarium and dura, which in turn addresses both the Chiari malformation and secondary syringomyelia.

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Section: Discussionmentioning

confidence: 99%