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三叶草颅骨畸形分期矫正的三个原则。

Three tenets for staged correction of Kleeblattschädel or cloverleaf skull deformity.

作者信息

Jarrahy Reza, Kawamoto Henry K, Keagle Jennifer, Dickinson Brian P, Katchikian Hurig V, Bradley James P

机构信息

Los Angeles, Calif. From the Division of Plastic and Reconstructive Surgery, David Geffen School of Medicine, University of California, Los Angeles.

出版信息

Plast Reconstr Surg. 2009 Jan;123(1):310-318. doi: 10.1097/PRS.0b013e3181934773.

DOI:10.1097/PRS.0b013e3181934773
PMID:19116567
Abstract

BACKGROUND

Patients with cloverleaf skull deformity are known to have high morbidity and poor outcome. Physical anomalies include a misshapen, trilobar skull, with a high "bossed" forehead, a bulging temporal region, and a flat posterior skull from multiple cranial suture fusion.

METHODS

Patients with cloverleaf skull deformity treated at the University of California, Los Angeles from 1990 to 2006 (n = 14) underwent early cranial vault remodeling (group 1) or staged correction with ventriculoperitoneal shunt (neonate), fronto-orbital advancement (3 to 6 months), and posterior vault remodeling (1 year) (group 2). Morbidity, necessary revisions, and neurologic (developmental testing) and aesthetic (Whitaker score) outcomes were assessed at follow-up.

RESULTS

Diagnoses included Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, Saethre-Chotzen syndrome, and nonsyndromic. Early cranial vault remodeling patients had more complications than staged correction patients [pneumonia, meningitis, and excessive bleeding (each 66 percent versus 9 percent); wound infection (66 percent versus 18 percent); and seizure (100 percent versus 0 percent)]. Early cranial vault remodeling patients had prolonged intensive care unit and hospital stays compared with staged correction patients (13 versus 2 days and 27 versus 5 days, respectively). The Whitaker score showed acceptable results at 18 months in group 2 (1.4, no revisions necessary) but not in group 1 (2.8, minor to major bony recontouring). Developmental tests showed that all early cranial vault remodeling patients had lower scores in both preschool tests and global evaluations compared with normative data (mean preschool receptive scores, 95 versus 85; mean preschool expressive scores, 94 versus 87).

CONCLUSION

Staged correction of cloverleaf skull provided acceptable neurologic and aesthetic outcomes in the authors' series of patients.

摘要

背景

已知患三叶形头盖骨畸形的患者发病率高且预后不良。身体异常包括畸形的三叶形颅骨、高耸的“隆起”前额、鼓起的颞部区域以及因多条颅骨缝融合导致的扁平后颅骨。

方法

1990年至2006年在加利福尼亚大学洛杉矶分校接受治疗的三叶形头盖骨畸形患者(n = 14)接受了早期颅骨穹窿重塑(第1组)或分期矫正,包括脑室腹腔分流术(新生儿期)、额眶前移术(3至6个月)和后颅骨穹窿重塑术(1岁)(第2组)。在随访时评估发病率、必要的翻修情况以及神经学(发育测试)和美学(惠特克评分)结果。

结果

诊断包括Apert综合征、Crouzon综合征、Pfeiffer综合征、Saethre - Chotzen综合征和非综合征性。早期颅骨穹窿重塑患者比分期矫正患者有更多并发症[肺炎、脑膜炎和出血过多(分别为66%对9%);伤口感染(66%对18%);以及癫痫发作(100%对0%)]。与分期矫正患者相比,早期颅骨穹窿重塑患者的重症监护病房和住院时间延长(分别为13天对2天和27天对5天)。惠特克评分显示,第2组在18个月时结果可接受(1.4,无需翻修),但第1组不可接受(2.8,表示需要进行小至大的骨性重塑)。发育测试表明,与标准数据相比,所有早期颅骨穹窿重塑患者在学前测试和整体评估中的得分均较低(学前平均接受性得分,95对85;学前平均表达性得分,94对87)。

结论

在作者的一系列患者中,三叶形头盖骨的分期矫正提供了可接受的神经学和美学结果。

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