154 Chapter 9 following cranial expansion is not progressive on subsequent scans, a wait and see policy is followed. If ICH occurs, a second vault expansion is preferred instead of shunt insertion. FMD is performed when tonsillar herniation becomes symptomatic or when a syrinx develops. In general, a shunt is avoided before or shortly after cranial vault expansion to prevent growth reduction induced by shunting. In cases of uncontrolled shape development, programmable shunts are used to gradually bring down the ventricle size to prevent over-shunting and skull growth failure. John Radcliffe Hospital, Oxford, United Kingdom The Oxford Craniofacial Unit has followed an evolving pathway for these patients. They always identify and treat the primary cause of ICH first. For example, any patient identified with primary hydrocephalus will first be treated with a VP shunt regardless of any additional causes of ICH such as craniocerebral disproportion. In the absence of hydrocephalus, patients with ICH caused by primary craniocerebral disproportion are usually treated with posterior cranial vault distraction followed by frontal surgery. The latter will be in the form of either a standard fronto-orbital advancement and remodeling (FOAR) or, more recently, an FOAR with distraction where appropriate monobloc advancements are used. FMD is performed when needed, that is, only when there is a sign of clinical progression. Oxford does not offer a prophylactic FMD surgery. Hôpital Necker-Enfants Malades, Paris, France Management of Crouzon patients at Hôpital Necker- Enfants Malades usually requires multiple surgical interventions. In general, Paris hospital starts with posterior vault expansion before the patient age of 6 months. When central apnea or severe syringomyelia appears, cerebellar tonsillar herniation will be treated, normally around the age of 12 months. A patient will be treated with frontofacial monobloc advancement until they reach an age of at least 18–24 months. Ventricular shunting is preferably avoided or delayed. Consider, for example, that in this study, in 2 of 5 cases with the cloverleaf form of Crouzon, the most severe, a shunt had to be inserted earlier. Primary fronto-orbital advancement or early facial osteotomy Le Fort type III may compromise the subsequent fronto-facial monobloc advancement. However, this salvage secondary monobloc procedure may be undertaken in some instances despite previous anterior osteotomies with a higher morbidity.16 RESULTS Patient Characteristics Nineteen patients with CAN were included in this study: 6 from Erasmus Medical Centre, 8 from Radcliffe Hospital, and 5 from Hôpital Necker-Enfants Malades. Patient characteristics are presented in Table 1. These 19 patients (14 female) presented with exorbitism (n = 15), midface hypoplasia (n = 16), and hypertelorism (n = 8). Table 1 also shows the presence of choanal atresia, aperture piriformis stenosis, and OSA
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