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.

Automated summary

This study aimed to describe and compare syndrome-related potential complications and outcomes associated with PVDO. The results showed that PVDO is an effective technique in addressing elevated intracranial pressure in SC patients at midterm follow-up, reducing the need for secondary procedures. Apert syndrome patients had higher total blood transfusion rates compared to Crouzon syndrome patients, who underwent surgery at a later age and weighed less.