Purpose

Lambdoid synostosis (LS) is a rare condition, which is either isolated; associated with sagittal synostosis, the “Mercedes-Benz” syndrome (MBS); or with synostosis of the coronal sutures (oxycephalic form). In addition, LS is part of the phenotype of a growing number of genetic diseases. The nosology, pathophysiology, and management are controversial. We decided to review our experience with LS.

Methods

We reviewed retrospectively pediatric cases of LS proved on CT-scanner, isolated or associated with other conditions, followed in our craniofacial center during the last 15 years, regarding clinical presentation, anatomical lesions, syndromic associations, surgical management, and outcome.

Results

We reviewed 48 cases: 6 isolated LS, 22 MBS, and 20 oxycephalic. A syndromic context was present in 72% (up to 80% of oxycephalic cases), and faciostenosis was present in 23%, mostly oxycephalic cases (40%). Transverse sinus agenesis was found in 61% of documented patients. A total of 31% of children had a dystocic birth, up to 45% of MBS. Decompressive craniectomy or cranioplasty was needed in a majority of patients, often young infants, while posterior fossa decompression was mostly performed in older children.

Conclusion

LS is rarely isolated and non syndromic; most cases are found in a wide spectrum of diseases, and LS is often associated with sagittal or coronal synostosis. Genetic evaluation is mandatory for LS; conversely, geneticists may require neurosurgical advice for LS in an increasing number of very rare diseases. The surgical management of LS should be tailored according to clinical presentation, age, and syndromic context.

中文翻译：

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菱形缝线的合缝：一个谱

目的

Lambdoid Synos​​tosis (LS) 是一种罕见的疾病，要么是孤立的；与矢状面结合相关的“梅赛德斯-奔驰”综合征 (MBS)；或与冠状缝（oxycephalic 形式）的synostosis。此外，LS 是越来越多的遗传疾病表型的一部分。病原学、病理生理学和治疗是有争议的。我们决定回顾一下我们与 LS 的经历。

方法

我们回顾性地回顾了过去 15 年在我们的颅面中心进行 CT 扫描证实的 LS 儿童病例，这些病例是孤立的或与其他疾病相关的，这些病例包括临床表现、解剖学病变、综合征关联、手术管理和结果。

结果

我们回顾了 48 例：6 例孤立性 LS、22 MBS 和 20 oxycephalic。72%（高达 80% 的 oxycephalic 病例）存在综合征背景，23% 存在面狭窄，主要是 oxycephalic 病例（40%）。在 61% 的记录患者中发现横窦发育不全。共有 31% 的儿童难产，高达 45% 的 MBS。大多数患者需要进行颅骨减压术或颅骨成形术，通常是年幼的婴儿，而后颅窝减压术主要用于年龄较大的儿童。

结论

LS 很少是孤立的和非综合征性的；大多数病例见于广泛的疾病中，LS 常与矢状或冠状面结合。LS 必须进行基因评估；相反，在越来越多的非常罕见的疾病中，遗传学家可能需要为 LS 提供神经外科建议。LS 的手术治疗应根据临床表现、年龄和综合征背景进行调整。