Advances in craniosynostosis research and management.

The purpose of the present paper is to analyze the most recent advances in the field of craniosynostosis basic and clinical research and management, and to give an overview of the more frequently adopted surgical strategies. After reviewing some basic concepts regarding normal craniofacial embryology and growth, aetiopathogenesis of craniosynostosis and craniofacial dysostosis, classification and diagnosis and historical evolution of surgical treatment, the authors elaborate on a selection of topics that have modified our current understanding of and therapeutical approach to these disease processes. Areas covered include advances in molecular biology and genetics, imaging techniques and surgical planning, resorbable fixation technology, bone substitutes and tissue engineering, distraction osteogenesis and the spring-mediated cranioplasties, resorbable distractor devices, minimally invasive surgery and in utero surgery. A review of the main subtypes of craniosynostosis and craniofacial dysostosis is presented, including their specific clinical features and a commentary on the presently available surgical options.

Expander elements in craniofacial surgery: an experimental study in rabbits.

With the inception of craniofacial surgery elaborate surgical procedures were introduced for the treatment of craniosynostosis. Recently the use of implantable springs to aid simple strip craniectomies for these conditions has been described. Having shown the feasibility of using springs for dynamic skull reshaping, several questions remain to be answered, all of them about how to control the spring action clinically. One of the most important questions concerns force. The purpose of this study was to evaluate experimentally the effect of two springs of different strengths on the growth of rabbit calvaria after strip craniectomy. Thirty-two 6-week-old rabbits were randomised into one of four groups: sham-operation where only amalgam markers were inserted after subperiostal dissection; strip craniectomy of the sagittal suture, no expansion; strip craniectomy of the sagittal suture and insertion of an expander element made of titanium molybdenum alloy (TMA); and strip craniectomy sagittal suture and insertion of an expander element made of stainless steel (SS). SS springs delivered a range of forces from 2.42-2.18 N, whereas the TMA expander elements delivered a range from 1.39-1.09 N. The parietal bone marker separation after 12 weeks was 4.9 mm in the TMA group and 7.4 mm in the SS group. This resulted in a significant increase of the calvarian height compared with control groups. Histological examination showed intramembranous bone formation in the bone gap in all groups.

Long-term follow-up of dynamic cranioplasty for brachycephaly--non-syndromal bicoronal synostosis.

We followed up 10 patients whose non-syndromal bicoronal synostosis had been operated on with a dynamic cranioplasty technique developed by this craniofacial unit in 1992. With this technique, the growth of the brain is redirected in an anteroposterior direction as wire-mediated compression and restraint are exerted on the transverse and vertical dimensions of the skull. The mean operating time was 160 minutes (range 120-275) and mean stay in the intensive care unit was 36 hours (range 23-58). There was no operative mortality and few complications. The surgical results were assessed objectively by analysis of cephalometric tracings. The mean (SD) cephalic index was 87.6 (4.9) preoperatively and 77.7 (1.8) postoperatively (p = 0.001). The modified Whitaker scale was used as a subjective outcome measurement, and nine patients were classified as Whitaker grade 1 (no additional surgery). One patient required additional intracranial surgery. A questionnaire was sent to all families to obtain an additional subjective measurement of outcome. Parents' satisfaction was high. We conclude that dynamic cranioplasty is a safe and efficient operation for treatment of brachycephaly.

Clinical outcome of the modified pi-plasty procedure for sagittal synostosis.

The aim of the study was to evaluate the modified pi-plasty procedure for the treatment of sagittal synostosis, assessing the issues of safety, complications, morphological outcome, and degree of parental satisfaction. A retrospective evaluation of 110 patients with nonsyndromal single suture sagittal synostosis operated on with the modified pi-plasty procedure was undertaken. Cephalometric radiographs were obtained preoperatively and postoperatively at ages 3 and 5 years in three standardized projections. The Cephalic Index and the Axial Width Ratio were determined and used as objective outcome measures. An evaluation of the radiographic digital markings was carried out using a Beaten Copper Score. A parental questionnaire was used to obtain a subjective esthetical outcome assessment. The patient population consisted of 76% boys and 24% girls with a 20% incidence of a positive familial history of craniosynostosis. The mean age at surgery was 7.73 months. Morbidity from the procedure was minimal and there were no mortalities. The Cephalic Index changed from a mean preoperative value of 65% to a postoperative mean value of 72% (P = 0.00004). The mean Axial Width Ratio changed from a preoperative 80% to 72% at the 3-year evaluation (P = 0.00029). The Beaten Copper score changed from a mean preoperative value of 2.35 to 5.42 postoperatively at 3 years (P = 0.00001). The response rate to the questionnaire was 86%, and there were significant postoperative improvements in all studied aspects of the skull shape. The modified pi-plasty is a safe technique, and it induces significant objective changes in skull morphology toward normality. It also yields a high degree of parental satisfaction with regard to aesthetic outcome, as evaluated by a written questionnaire.

Dynamic cranioplasty for brachycephaly in apert syndrome: long-term follow-up study.

OBJECT: Brachycephaly is a characteristic feature of Apert syndrome. Traditional techniques of cranioplasty often fail to produce an acceptable morphological outcome in patients with this condition. In 1996 a new surgical procedure called "dynamic cranioplasty for brachycephaly" (DCB) was reported. The purpose of the present study was to analyze perioperative data and morphological long-term results in patients with the cranial vault deformity of Apert syndrome who were treated with DCB. METHODS: Twelve patients have undergone surgery performed using this technique since its introduction in 1991 (mean duration of follow-up review 60.2 months). Eleven patients had bicoronal synostosis and one had a combined bicoronal-bilambdoid synostosis. Perioperative data and long-term evolution of skull shape visualized on serial cephalometric radiographs were analyzed and compared with normative data. Changes in mean skull proportions were evaluated using a two-tailed paired-samples t-test, with differences being considered significant for probability values less than 0.01. The mean operative blood transfusion was 136% of estimated red cell mass (ERCM) and the mean postoperative transfusion was 48% of ERCM. The mean operative time was 218 minutes. The duration of stay in the intensive care unit averaged 1.7 days and the mean hospital stay was 11.8 days. There were no incidences of mortality and few complications. An improvement in skull shape was achieved in all cases, with a change in the mean cephalic index from a preoperative value of 90 to a postoperative value of 78 (p = 0.000254). CONCLUSIONS: Dynamic cranioplasty for brachycephaly is a safe procedure, yielding high-quality morphological results in the treatment of brachycephaly in patients with Apert syndrome.

Mental development after modified pi procedure: dynamic cranioplasty for sagittal synostosis.

A prospective developmental assessment was performed on 26 patients operated on with dynamic cranioplasty for sagittal synostosis. Because this technique entails the application of compressive force, it was of great concern to assess the effect of surgery on development and mental status. The surgical technique used was a modified pi procedure. Perioperative variables were recorded. Six patients underwent preoperative intracranial pressure (ICP) measurements. To evaluate objectively the developmental outcome, the Griffiths' Mental Development Scales was used for analysis before and after surgery. A parental questionnaire was used for subjective outcome measurement. Preoperative ICP recordings during sleep ranged from 12.8 to 22.8 mmHg (mean, 16.1 mmHg). The mean age at the time for surgery was 6.9 months (range, 4-16 months; standard deviation [SD], 2.32 months). The surgical technique included shortening of the anteroposterior diameter of the skull by a mean of 16.6 mm. The mean global development quotient (GDQ) preoperatively was 104.5 (range, 82-144; SD, 12.4) and the mean GDQ postoperatively was 101.4 (range, 62-129; SD, 13.6). Mean age at follow-up was 16.3 months (range, 9-40 months; SD, 4.04 months). There was no significant correlation between the amount of intraoperative shortening and mental development. In comparison of means, the GDQ preoperatively did not differ significantly from the GDQ postoperatively. The modified pi procedure is safe and efficient. When surgery was performed before 1 year of age, no significant (p = 0.33) effect on mental development-either detrimental or beneficial-was demonstrated.