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肢端肥大症在颅骨和颅颈交界处的可变骨骼解剖特征。

Variable skeletal anatomical features of acromegaly in the skull and craniocervical junction.

作者信息

Piagkou Maria, Manolakos Othon, Troupis Theodore, Lazaridis Nikolaos, Laios Konstantinos, Samolis Alexandros, Natsis Konstantinos

机构信息

Department of Anatomy, Medical School, Faculty of Health Sciences, National and Kapodistrian University of Athens, Greece.

Department of Anatomy and Surgical Anatomy, Medical School, Faculty of Health Sciences, Aristotle University of Thessaloniki, Thessaloniki, Greece.

出版信息

Acta Med Acad. 2017 Nov;46(2):162-168. doi: 10.5644/ama2006-124.201.

Abstract

OBJECTIVE

This study adds important information regarding the morphological alterations caused by growth hormone hypersecretion in the skull and craniocervical junction (CCJ). A variably asymmetric skull due to acromegaly coexists with expansion of the paranasal sinuses and multiple Wormian bones.

CASE REPORT

A pathologically asymmetric dry skull of a European male, aged 38 years at death, with cranial vault and skull base thickening is described. The extensive paranasal sinus pneumatization caused a generalized thinning of the bony walls. The sphenoid sinus expanded intraorbitally, leading to sella enlargement. The orbital asymmetry coexisted with platybasia and hypoplasia of the occipital condyles and the odontoid process. Facial skeleton elongation and mandibular overgrowth were combined with prognathism, malocclusion and overbite.

CONCLUSION

Skull and CCJ alterations are of paramount importance when selecting the surgical approach, if surgery is indicated. Consecutively, detailed preoperative evaluation and planning is essential. During surgery, skilled and experienced neurosurgeons recognize anatomical landmarks, use neuronavigation and micro-instrumentation in order to remain on the midline avoiding any potential lethal vascular injury.

摘要

目的

本研究补充了有关生长激素分泌过多导致颅骨和颅颈交界区(CCJ)形态学改变的重要信息。肢端肥大症所致的颅骨不对称程度不一,同时伴有鼻窦扩张和多发缝间骨。

病例报告

描述了一名38岁死亡的欧洲男性的病理性不对称干颅骨,其颅顶和颅底增厚。广泛的鼻窦气化导致骨壁普遍变薄。蝶窦向眶内扩展,导致蝶鞍扩大。眼眶不对称与扁平颅底、枕髁发育不全和齿突发育不全并存。面部骨骼伸长和下颌过度生长伴有下颌前突、咬合不正和深覆合。

结论

如果需要手术,在选择手术入路时,颅骨和CCJ的改变至关重要。因此,详细的术前评估和规划必不可少。手术过程中,技术熟练且经验丰富的神经外科医生要识别解剖标志,使用神经导航和显微器械,以保持在中线位置,避免任何潜在的致命性血管损伤。

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