156 Chapter 9 Surgical Interventions Table 2 gives an overview of the surgical procedures for all 19 patients. Figure 2 shows the surgeries divided by medical center location. In Figure 3, the chronology of surgeries per patient over time is shown. The 19 patients were of different ages and in different stages of their treatment; therefore, the graphic may not reflect the final number of surgeries for the younger patients before they reach adulthood. Thirteen patients had a first vault expansion before the age of 1 year, and 6 patients had their first after the age of 1. Eleven patients had a redo vault expansion, and 3 of them needed a third vault expansion. Thirteen patients were treated for hydrocephalus; the first intervention was a VP shunt in 8 patients and an ETV in 5 patients. In 3 of these latter 5 patients, ETV failed and a VP shunt was inserted. Eight of 11 patients needed one or more revisions of their shunt. In 8 patients, hydrocephalus was treated before they underwent cranial vault expansion (Netherlands [NL] 1/6, United Kingdom [UK] 4/8, France [FR] 3/5). Table 2. Overview of surgeries. Vault expansion No. of surgeries (in No. of patients) Fronto-supraorbital advancement 7(7) Fronto parietal expansion 2(2) Occipital expansion* 9(8) Occipito parietal expansion, spring distraction 5(4) Monobloc with distraction** 9(9) Monobloc without distraction 1(1) Facial bipartition distraction 1(1) Foramen Magnum Decompression*** 11(9) Midface surgery Le Fort II distraction 1(1) Le Fort III distraction 4(4) Bimax 1(1) Cerebrospinal fluid diverting surgery Shunt 23(11) Endoscopic third ventriculostomy 6(5) Endoscopic third ventriculostomy converted to shunt (3) Respiratory improvement: non-facial surgery Tracheotomy 10(10) Adenotonsillectomy 13(12) Other Cleft Palate Repair 1(1) * including 5 surgeries in 4 patients combined with Foramen Magnum Decompression ** including 1 mandibular distraction ***including 5 surgeries in 4 patients combined with Occipital Expansion
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