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

Journal of neurosurgery 2001 Vol.94(5) p. 757-64

Guimarães-Ferreira J, Gewalli F, Sahlin P, Friede H, Owman-Moll P, Olsson R, Lauritzen CG

Abstract

[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.

추출된 의학 개체 (NER)

유형영어 표현한국어 / 풀이UMLS CUI출처등장
해부 bicoronal-bilambdoid scispacy 1
해부 blood scispacy 1
합병증 cranial vault scispacy 1
합병증 skull scispacy 1
합병증 brachycephaly scispacy 1
약물 DCB → dynamic cranioplasty for brachycephaly" scispacy 1
질환 brachycephaly C0221356
Brachycephaly
scispacy 1
질환 apert syndrome C0001193
Apert syndrome
scispacy 1
질환 cranial vault deformity scispacy 1
질환 bicoronal synostosis C4021164
Bicoronal synostosis
scispacy 1
질환 bicoronal-bilambdoid synostosis scispacy 1
질환 red cell scispacy 1
질환 ERCM → estimated red cell mass scispacy 1
기타 patients scispacy 1
기타 ERCM → estimated red cell mass scispacy 1

MeSH Terms

Acrocephalosyndactylia; Cephalometry; Female; Follow-Up Studies; Humans; Infant; Male; Parents; Reoperation; Surgery, Plastic; Treatment Outcome