UPPER AIRWAY-OBSTRUCTION AND RAISED INTRACRANIAL-PRESSURE IN CHILDRENWITH CRANIOSYNOSTOSIS

In children with craniosynostosis, raised intracranial pressure (ICP) and upper airway obstruction (UAO) are both common features. However, potential interactions between UAO and ICP during sleep are poorly und erstood. The aim of the present study was to compare the levels of ICP during sleep between a group of patients with syndromic craniosynosto sis (with facial involvement and consequent UAO) and a group of contro l patients with isolated unicoronal synostosis (with no facial involve ment and normal upper airways). Polygraphic cardiorespiratory sleep st udies with continuous monitoring of ICP were performed during unsedate d sleep in 13 children with syndromic craniosynostosis and 7 control p atients with isolated unicoronal synostosis only. In the syndromic gro up, UAO was present in 11 out of 13 patients, with 8 out of 13 having frank obstructive sleep apnoea. In contrast, none of the control patie nts showed signs of UAO during sleep. There was no evidence of central apnoeas in any of the patients studied. Clinical histories taken from parents tended to underestimate the severity of the respiratory probl ems, Elevated ICP was seen in 10 of the 13 syndromic patients, with bo rderline raised ICP in the remaining three cases. In contrast, raised ICP was seen in only 3 of the 7 control patients, with borderline rais ed levels in 2 of the 7. For both patient groups, ICP was higher durin g active sleep compared to quiet sleep. Multiple regression analysis s howed that ICP during active sleep was dependent upon disease severity (unicoronal/multiple synostosis) and to the baseline ICP level during quiet sleep. Both raised ICP and airway obstruction were more apparen t during active sleep. There was a significant correlation between sev erity of UAO and increased ICP in active sleep. We conclude that obstr uctive respiratory problems are frequent in the syndromic patients, an d can be severe in a large proportion of cases; intracranial hypertens ion is also frequent in this group. Further studies are required to in vestigate the possibility of a causal relationship between upper airwa y obstruction and raised intracranial pressure.