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婴儿颅骨筋膜炎:基于病例的综述及手术技术

Infantile cranial fasciitis: case-based review and operative technique.

作者信息

Flouty Oliver E, Piscopo Anthony J, Holland Marshall T, Abode-Iyamah Kingsley, Bruch Leslie, Menezes Arnold H, Dlouhy Brian J

机构信息

Department of Neurosurgery, University of Iowa Hospitals and Clinics, Iowa city, IA, 52242, USA.

Department of Biology, University of Iowa Hospitals and Clinics, Iowa city, IA, 52242, USA.

出版信息

Childs Nerv Syst. 2017 Jun;33(6):899-908. doi: 10.1007/s00381-017-3417-y. Epub 2017 Apr 27.

Abstract

BACKGROUND

Cranial fasciitis (CF) is an uncommon benign primary lesion of the skull that typically affects the pediatric age group. Due to the rarity of CF, no prospective studies exist. Earliest description of this condition dates to 1980. The limited scientific and clinical literature regarding CF is dominated by case reports. For these reasons, questions pertaining to the true incidence, genetic risk factors, prognosis, and long-term outcome remain unanswered.

DISCUSSION

Clinically, CF presents as a firm, painless, growing scalp mass that is typically not considered in the differential diagnosis. Preoperative pathognomonic signs and symptoms are absent, and imaging features are often nonspecific. Treatment is typically through complete surgical resection, at which time histopathological examination confirms the diagnosis of CF. Reconstruction of the skull defect in the child is critical. Autograft techniques help maintain a rigid construct that integrates with the native skull while preserving its continued ability to grow. Generally, a good outcome is observed with complete resection.

EXEMPLARY CASE

We report a case of CF in an infant with emphasis on operative nuances and early follow-up results.

CONCLUSION

CF is a rare fibroproliferative disease that has a poorly defined incidence and long-term follow-up. Due to its locally invasive nature and nonspecific presentation, CF is often difficult to differentiate from malignancies and infections. Complete surgical resection is the best approach for diagnosis and cure. Its occult clinical presentation often allows it to achieve considerable growth, leaving a sizeable skull defect following resection. Since CF presents in the pediatric population, allograft reconstruction is preferred over titanium mesh or other synthetic materials to allow osseous integration and continued uninterrupted skull growth.

摘要

背景

颅骨筋膜炎(CF)是一种罕见的颅骨原发性良性病变,通常影响儿童年龄组。由于CF罕见,尚无前瞻性研究。对这种疾病的最早描述可追溯到1980年。关于CF的有限科学和临床文献主要是病例报告。由于这些原因,有关真实发病率、遗传危险因素、预后和长期结局的问题仍未得到解答。

讨论

临床上,CF表现为头皮上一个坚硬、无痛、不断生长的肿块,通常不在鉴别诊断范围内。术前缺乏特征性体征和症状,影像学特征往往不具特异性。治疗通常通过完整的手术切除,此时组织病理学检查可确诊CF。儿童颅骨缺损的重建至关重要。自体移植技术有助于维持一个与颅骨整合的坚固结构,同时保留其持续生长的能力。一般来说,完整切除后预后良好。

典型病例

我们报告一例婴儿CF病例,重点介绍手术细节和早期随访结果。

结论

CF是一种罕见的纤维增生性疾病,发病率和长期随访情况尚不明确。由于其局部侵袭性和非特异性表现,CF常难以与恶性肿瘤和感染相鉴别。完整的手术切除是诊断和治愈的最佳方法。其隐匿的临床表现常使其得以显著生长,切除后留下相当大的颅骨缺损。由于CF发生于儿童人群,同种异体移植重建优于钛网或其他合成材料,以实现骨整合和颅骨的持续不间断生长。

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