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胸椎皮肤窦道伴髓内脓肿及脑神经功能缺损

Dermal Sinus Tract of the Thoracic Spine Presenting with Intramedullary Abscess and Cranial Nerve Deficits.

作者信息

Papaevangelou Georgios, Tsitsopoulos Parmenion P, Flaris Nikolaos, Iliadis Charalampos, Tsonidis Christos

出版信息

Pediatr Neurosurg. 2015;50(6):339-43. doi: 10.1159/000439539. Epub 2015 Oct 13.

Abstract

Congenital dermal sinus tract of the spine is an unusual developmental defect which represents a failure of the surface ectoderm and dermal elements to separate from the neuroectoderm. A 15-month-old female presented with high fever, severe right hemiparesis, difficulty breathing and cranial nerve deficits. Magnetic resonance imaging (MRI) of the spine revealed a congenital dermal sinus tract at the Th6 level, an intramedullary collection extending up to the brainstem and a probable intramedullary cystic lesion. The child was operated acutely with ligation of the sinus tract, drainage of the abscess and partial removal of the intramedullary lesion. Due to abscess recurrence, she was reoperated with complete excision of the dermal sinus tract, abscess redrainage and subtotal excision of the dermoid cyst (retaining a part of its capsule). Pus culture isolated Corynebacterium species and Peptococcus species and histology of the lesion showed a dermoid cyst. Postoperatively, after an initial neurologic deterioration, she progressively improved. An MRI scan at 15 months neither showed recurrence of the collection nor regrowth of the lesion. Spinal dermal sinus tracts that remain unnoticed or untreated can result in serious complications and should be operated as soon as possible to prevent undesirable sequelae.

摘要

先天性脊柱皮样窦道是一种罕见的发育缺陷,表现为表面外胚层和真皮成分未能与神经外胚层分离。一名15个月大的女性出现高热、严重右侧偏瘫、呼吸困难和脑神经功能缺损。脊柱磁共振成像(MRI)显示胸6水平有先天性皮样窦道、一个延伸至脑干的髓内积液以及一个可能的髓内囊性病变。患儿接受了急症手术,结扎窦道、引流脓肿并部分切除髓内病变。由于脓肿复发,她再次接受手术,完整切除皮样窦道、再次引流脓肿并次全切除皮样囊肿(保留部分囊壁)。脓液培养分离出棒状杆菌属和消化球菌属,病变组织学检查显示为皮样囊肿。术后,起初有神经功能恶化,之后逐渐好转。15个月时的MRI扫描既未显示积液复发,也未显示病变再生长。未被发现或未治疗的脊柱皮样窦道可导致严重并发症,应尽早手术以防止出现不良后遗症。

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