孤立性额蝶缝早闭：骨性斜头畸形的罕见病因。

Isolated frontosphenoidal synostosis: a rare cause of synostotic frontal plagiocephaly.

作者信息

Sauerhammer Tina M, Oh Albert K, Boyajian Michael, Magge Suresh N, Myseros John S, Keating Robert F, Rogers Gary F

机构信息

Division of Plastic and Reconstructive Surgery, Children's National Medical Center, Washington, DC.

出版信息

J Neurosurg Pediatr. 2014 May;13(5):553-8. doi: 10.3171/2014.1.PEDS1378. Epub 2014 Mar 7.

DOI:10.3171/2014.1.PEDS1378

PMID:24606403

Abstract

OBJECT

Unilateral fusion of the frontoparietal suture is the most common cause of synostotic frontal plagiocephaly. Localized fusion of the frontosphenoidal suture is rare but can lead to a similar, but subtly distinct, phenotype.

METHODS

A retrospective chart review of the authors' craniofacial database was performed. Patients with isolated frontosphenoidal synostosis on CT imaging were included. Demographic data, as well as the clinical and radiographic findings, were recorded.

RESULTS

Three patients were identified. All patients were female and none had an identifiable syndrome. Head circumference was normal in each patient. The mean age at presentation was 4.8 months (range 2.0-9.8 months); 2 fusions were on the right side. Frontal flattening and recession of the supraorbital rim on the fused side were consistent physical findings. No patient had appreciable facial angulation or orbital dystopia, and 2 patients had anterior displacement of the ipsilateral ear. All 3 patients were initially misdiagnosed with unilateral coronal synostosis, and CT imaging at a mean age of 5.4 months (range 2.1-10.8 months) was required to secure the correct diagnosis. Computed tomography findings included patency of the frontoparietal suture, minor to no anterior cranial base angulation, and vertical flattening of the orbit without sphenoid wing elevation on the fused side. One patient underwent CT scanning at 2.1 months of age, which demonstrated a narrow, but patent, frontosphenoidal suture. The patient's condition was assumed to be a deformational process, and she underwent 6 months of unsuccessful helmet therapy. A repeat CT scan obtained at 10.7 months of age demonstrated the synostosis. All 3 patients underwent fronto-orbital correction at mean age of 12.1 months (range 7.8-16.1 months). The mean duration of postoperative follow-up was 11.7 months (range 1.9-23.9 months).

CONCLUSIONS

Isolated frontosphenoidal synostosis should be considered in the differential diagnosis of atypical frontal plagiocephaly.

摘要

目的

额顶缝单侧融合是骨性斜头畸形最常见的病因。额蝶缝局部融合虽罕见，但可导致类似但又略有不同的表型。

方法

对作者的颅面数据库进行回顾性图表审查。纳入CT成像显示孤立性额蝶缝早闭的患者。记录人口统计学数据以及临床和影像学检查结果。

结果

共确定3例患者。所有患者均为女性，且均无明确综合征。每位患者的头围均正常。就诊时的平均年龄为4.8个月（范围2.0 - 9.8个月）；2例融合发生在右侧。融合侧额部扁平及眶上缘后缩是一致的体格检查发现。无患者有明显的面部成角或眶移位，2例患者同侧耳向前移位。所有3例患者最初均被误诊为单侧冠状缝早闭，平均在5.4个月（范围2.1 - 10.8个月）时需行CT成像以明确正确诊断。CT表现包括额顶缝通畅、前颅底轻度成角或无成角，以及融合侧眼眶垂直扁平且蝶骨嵴无抬高。1例患者在2.1个月时行CT扫描，显示额蝶缝狭窄但通畅。该患者的病情被认为是一种变形过程，她接受了6个月的头盔治疗但未成功。10.7个月时复查CT扫描显示缝早闭。所有3例患者均在平均12.1个月（范围7.8 - 16.1个月）时接受了额眶矫正术。术后平均随访时间为11.7个月（范围1.9 - 23.9个月）。