Syndrome-related outcomes following posterior vault distraction osteogenesis.

PURPOSE: The most commonly occurring syndromic craniosynostoses are Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, and Saethre-Chotzen syndrome. There is insufficient data regarding postoperative syndrome-related outcomes following the posterior vault distraction osteogenesis (PVDO) procedure, as well as data addressing whether or not additional procedures will be subsequently necessary to comprehensively treat children who undergo PVDO. Thus, the objective of this study is to describe and compare syndrome-related potential complications and outcomes associated with the PVDO procedure. METHODS: An observational retrospective study was performed on consecutive patients (n=24) with Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, or Saethre-Chotzen syndrome, respectively, who underwent PVDO between 2012 and 2019. Demographic data (patient gender and age when the PVDO procedure was performed), diagnosis, surgery-related data, and outcome data (perioperative and midterm complications and need for additional surgery) were verified. RESULTS: Total relative blood transfusion volumes per kilogram for the patients were as follows: 22.75 ± 9.30 ml for Apert syndrome, 10.73 ± 2.28 ml for Crouzon syndrome (Apert versus Crouzon, p<0.05), 18.53 ± 8.08 ml for Pfeiffer syndrome, and 19.74 ± 9.12 ml for Saethre-Chotzen syndrome. None of the patients required a secondary procedure to alleviate intracranial pressure except for a Saethre-Chotzen patient. CONCLUSION: PVDO is an effective technique to address elevated intracranial pressure in SC patients that alleviates the need for secondary procedures at midterm follow-up. Apert syndrome patients presented relatively higher total blood transfusion rates than Crouzon syndrome patients who were operated on at a later age and weighed more.

Hand Function in Apert Syndrome.

BACKGROUND: The Michigan Hand Questionnaire is widely used to assess hand outcomes in congenital hand deformities. The purpose of the present study is to compare Apert syndrome hand outcomes according to Upton hand type and age stratification with age-matched unaffected controls. METHODS: The Brief Michigan Hand Questionnaire was administered to 39 Apert patients after completion of the digit separation surgical regimen, and 140 age-matched unaffected controls. Patients were divided into 3 groups according to age. In group 1 (from 4 months to 7 years of age), responses were provided by parents of Apert patients, and in group 2 (from 8 to 17 years of age), patients responded with assistance from their parents, and in group 3 (18 years of age or older), responses were provided by the patients themselves. Groups were substratified according to Upton hand type (type I, II, and III). Comparisons were made among groups, subgroups, and controls. RESULTS: Comparisons of hand types for intragroups 1, 2, and 3, did not demonstrate any statistically significant differences (P > 0.05) between hand outcomes according to Upton hand type, regardless of patient age. Comparisons between Apert patients and their age-matched controls demonstrated statistically significant differences (P < 0.05), as the control group had higher outcome scores. CONCLUSIONS: Similar hand outcomes scores were achieved by all Apert patients regardless of hand type. Following completion of the digit separation regimen, Apert patients presented hand outcome scores that were lower than those of the patients in the normative control group.