159 Clinical Course | Crouzon AN Fourteen patients had a monobloc advancement or midface correction (NL 5/6, UK, 4/8, FR 5/5). The UK treated 1 patient with 1 monobloc advancement; the NL and FR treated the majority of their patients with a monobloc advancement. Seven patients underwent an FMD (NL 1/6, UK 2/8, FR 4/5). Five of these 7 patients required treatment for hydrocephalus either before or after the FMD. The timeline of events shows that most children underwent the majority of surgical events in the range of 0–3 years old. Differences in the timing and order of interventions exist among the centers based on protocol and on the presentation of the patient. Although the order of interventions differed, the total number of interventions per patient was quite similar and the number of skull expansions showed small variations. Patients receiving a VP shunt first had a mean of 2 skull expansions during follow-up. Each patient receiving a VP shunt after initial skull expansion had a total number of 1.5 vault expansions. Each patient who never received a shunt or never underwent an ETV had 1.3 skull expansions. Among the 9 patients who underwent early (before the age of 1) cranial expansion as the initial intervention, 4 needed subsequent hydrocephalus treatment. The patient numbers were too small for statistical analysis. DISCUSSION In this review, we found that CAN follows a complex clinical course mainly dictated by the recurrence of raised intracranial pressure and repeated interventions for hydrocephalus. Treatment strategies differed among the three contributing centers. All patients in this cohort required multiple surgeries to treat ICH or to treat conditions that cause ICH such as OSA, hydrocephalus, and Chiari malformation.6, 8 Interventions to improve airway obstruction were often the first intervention in a course of interventions that would follow. In this cohort, 10 of 19 patients needed a tracheostomy, which is more than the 3 tracheostomies in a cohort of 19 patients with Crouzon described by Arnaud et al.17 It is difficult to compare our findings with the percentages from other CAN studies because of the small cohorts and case reports in the literature. The treatment of progressive ventriculomegaly in patients in the present cohort is below the range found in CAN studies (43%–75%) and above the published prevalence of progressive ventriculomegaly in Crouzon patients (6%–26%).1, 4, 18, 19 Shunting and ETV in this cohort are comparable to figures in the Crouzon literature.8, 20-22 The treatment of hydrocephalus is challenging because of a low success rate with ETV and high shunt failure rate. This may be expected in complex craniosynostosis and is related to young age and multiple cranial surgeries.6, 8, 21 9
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