Successful treatment of post-shunt craniocerebral disproportion by coupling gradual external cranial vault distraction with continuous intracranial pressure monitoring

Abstract

<jats:p>A subset of hydrocephalic patients in whom shunts are placed at an early age will develop craniocerebral disproportion (CCD), an iatrogenic mismatch between the fixed intracranial volume and the growing brain. The lack of a reliable, reproducible method to diagnose this condition, however, has hampered attempts to treat it appropriately. For those practitioners who acknowledge the need to create more intracranial space in these patients, the lack of agreed-upon therapeutic end points for cranial vault expansion has limited the use of such techniques and has sometimes led to problems of underexpansion. Here, the authors present a definition of CCD based primarily on the temporal correlation of plateau waves on intracranial pressure (ICP) monitoring and headache exacerbation. The authors describe a technique of exploiting continued ICP monitoring during progressive cranial expansion in which the goal of distraction is the cessation of plateau waves. Previously encountered problems of underexpansion may be mitigated through the simultaneous use of ICP monitors and gradual cranial expansion over time.</jats:p>

Chronic Debilitating Headache in Adults Caused by Craniocerebral Disproportion: Treatment by Cranial Vault Expansion

“…The term slit-ventricle syndrome is used for patients with CSF shunts and a constellation of symptoms and signs that include frequent and severe headaches, lethargy, intracranial hypertension, and small ventricles [ 2 , 3 ]. These patients have a history of having CSF shunts with low outflow resistance from infancy [ 2 - 4 ]. The chronic exposure to abnormally low intracranial pressure during infancy and childhood is thought to predispose to multisutural synostosis, which halts further expansion of their cranial vaults at an age when the brain is still growing [ 2 - 5 , 7 , 10 ].…”

“…The multisutural craniosynostosis then led to craniocerebral disproportion as the enclosed brains grew larger. Cranial vault expansion decompressed the brain parenchymas and provided more space for CSF, thereby improving the overall quality of life [ 4 , 9 ]. The fact that these three patients benefited greatly from cranial vault expansion is consistent with the position that all had insufficient cranial space before their cranial vaults were expanded.…”

Shunt-induced craniosynostosis: topicality of the problem, choice of the approach, and features of surgical treatment

“…При инвазивном мониторинге ВЧД у таких пациентов A. Sandler и соавт. [31] выявили корреляцию между головными болями и появлением плато-волн повышенного ВЧД.…”

“…Дистракционный остеогенез можно определить как рост костей, хирургически индуцированный за счет приложения раздвигающих сил в области остеотомии [39]. Одновременное использование датчиков инвазивного мониторинга ВЧД и постепенная дистракция костей черепа позволяют индивидуализировать методику по отношению к каждому пациенту, выполняя дистракцию на необходимую величину, при которой нормализуются показатели ВЧД [31].…”

Comment on the Article by Hart et al. Entitled ‘Combined Intracranial Pressure Monitoring and Cerebrospinal Fluid Infusion Study to Guide Management of Slit Ventricle Syndrome'

“…We suggest, instead, that the initial symptoms of headaches and papilledema, coupled with small noncompliant ventricles, bespeak of a child that had begun to manifest signs of craniocerebral disproportion (CCD) - an iatrogenic sequela of overdrainage in infancy described by us [4] and others [5,6] - before the distal revision. When the authors changed the valve, the child experienced headaches during recumbency, worsening papilledema, and abnormally prolonged ICP spikes that were slow to normalize, because the new valve effectively altered the resistance to drainage, a condition to which the child's brain had grown accustomed.…”