Background: The purpose of this study is to review 10 years of surgical experience in the management of Apert syndrome, focusing on an updated algorithm which includes hand reconstruction and posterior vault distraction osteogenesis (PVDO). Additionally, the authors compare PVDO, which is currently used, with fronto-orbital advancement (FOA), which was utilized in a previous algorithm. Methods: An observational retrospective study was performed on consecutive patients with Apert syndrome who underwent upper and lower limb reconstruction and craniofacial surgery between 2007 and 2017. A modified Clavien–Dindo surgical complication scale was used to stratify complications between PVDO and FOA. Demographic, surgical, and outcome data was also recorded. The blood transfusion rate between PVDO and FOA was also assessed and compared utilizing the Student t test. Results: The present study included 69 patients with Apert syndrome (34 males and 35 females). Craniofacial surgeries were performed on a total of 38 patients. A total of 210 operations were performed on the respective upper and lower limbs of patients included in this study. A total of 18 patients underwent PVDO (n = 9) and FOA (n = 9). Posterior vault distraction osteogenesis required significantly less transfused blood volume than FOA (P < 0.05). Complication rate and length of hospital stay were similar for each procedure. Conclusion: An updated algorithm to treat Apert patients was implemented. Posterior vault distraction osteogenesis incorporated into an updated algorithm results in a lower blood transfusion rate.

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Background: Presenting a wide clinical spectrum and large variety of clinical features, successful treatment of Apert syndrome necessitates performance of sequential multiple surgeries before a patient's facial skeleton growth is complete.The objective of this study is to compare forehead contour asymmetry and clinical outcomes between Apert patients who underwent either fronto-orbital advancement (FOA) or posterior vault distraction osteogenesis (PVDO). Methods: A retrospective study was performed on consecutive patients with Apert syndrome who underwent either FOA or PVDO between 2007 and 2019, and participated in at least 6 months of follow-up care. Forehead contour asymmetry and surgical outcomes for each of the included patients were verified through medical records, clinical photographs, and interviews with the parents of the patients. The need for additional craniofacial procedures based on the surgical outcomes of each patient was graded from I to IV utilizing the Whitaker outcome classification system. Results: Forehead contour asymmetry for all included patients was rated under the Whitaker grading scale as type II (n ¼ 4) 44.4%, type III (n ¼ 2) 22.2%, and type IV (n ¼ 3) 33.3% for FOA, and type I (n ¼ 5) 35.7%, type II (n ¼ 7) 50%, and type III (n ¼ 2) 14.3%, for PVDO (P < 0.05). The average transfused blood volume was 47.77 AE 9.42 mL/kg for patients who underwent FOA, and 22.75 AE 10.31 mL/kg for patients who underwent PVDO (P < 0.05). Conclusions: Patients who underwent PVDO had lower forehead contour asymmetry as per the Whitaker outcome grading scale than patients who underwent FOA.

Interestingly, patients with severe injuries (blast, R.T.A.) had all opted to a one-stage rather than a two-stage reconstruction, Supplementary Digital Content, Table 1, http://links.lww.com/SCS/C535. Even more, a bone graft was only used in the one case with R.T.A. The distribution according to the classification of the defect, Supplementary Digital Content, Table 2, http://links.lww.com/SCS/C538 shows that most patients who agreed to have a second surgery were those without a C component in their Boyd classification. Also, 90% of the patients in the one-stage reconstruction group had injuries elsewhere in the body. These observations highlight that patients with severe injuries are less likely to agree/proceed with a multi-stage reconstruction and tend to be satisfied with the simplest available form of reconstructive treatment. Many of them, however, might have to undergo revision surgeries later according to a recent meta-analysis. 11 This is also supported by the fact that 7 cases which were initially planned for two-stage reconstruction did not go through with the second stage. Most of these cases were males possibly because their esthetic requirements are not as high as females. 12 Bone grafts were successfully involved to bridge defects even larger than the maximum 6 cm limit (2). This decision was made because microvascular reconstruction is not available as a treatment option in our facility. Therefore, nonvascularized bone graft is indicated as long as it would have enough soft tissue cover at the time of wound closure (as determined during the treatment planning stage). 13 Figure 1A shows an example of successful case beyond this limit.The correlation between TMJ/occlusal complications and HL type was apparent (all 8 cases had L component). However, C type correlation with esthetic issues was not clear in our sample (only one of the three cases had a C component). 1,2 In fact, the authors noted that esthetic complains were mainly attributed to permanent facial nerve damage (2 out of the 3). It is therefore prudent not to promise symmetry and to lower patient's expectations in cases of facial nerve damage.The outcome results in Supplementary Digital Content, Table 4, http://links.lww.com/SCS/C541 suggest that one-stage and twostage procedures had nearly equal success rates in the study sample.In this study, sample patients did not attend follow-up appointments if they were unsatisfied with the initial results. This observation contradicts the results of Elegbede et al 14 investigation. This also would explain the disagreement of our failed cases distribution with previous studies stating that lower success is expected with repeated surgery. 2,4 The authors conclude that mandibular reconstruction with nonvascularized bone graft is a successful treatment with a high predictability of possible complications. Most of such complications are temporary, easily managed and rarely reach the necessity for graft removal. It is a good option to consider in the absence of vascularized flaps as opposed to ove...