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儿童鼻皮样囊肿:基于6例病例的综述

[Nasal dermal sinus in children: a review based on a series of 6 cases].

作者信息

Klein O, Simon E, Coffinet L, Joud A, Ghetemme C, Marchal J-C

机构信息

Service de neurochirurgie pédiatrique, hôpital d'Enfants, CHU de Nancy, université de Lorraine, 4, rue du Morvan, 54500 Vandœuvre-Lès-Nancy, France.

Service de chirurgie maxillo-faciale et plastique, CHU de Nancy, université de Lorraine, 54500 Nancy, France.

出版信息

Neurochirurgie. 2014 Feb-Apr;60(1-2):27-32. doi: 10.1016/j.neuchi.2013.10.123. Epub 2014 Mar 20.

Abstract

INTRODUCTION

Nasal dermal sinus in children (NDSC) is a rare malformation (1/20,000 to 1/40,000). Apart from local infection, they present as median nasal lump or pit on the dorsum and their diagnosis and treatment are often delayed. Consequences of untreated NDSC are: local infection, meningitis, and empyema, due to their frequent intracranial extension.

PATIENTS AND METHODS

Six cases of NDSC were retrospectively reviewed (5 boys and one girl), all treated between 2006 and 2012 in our institution (Nancy University Hospital). All patients underwent a brain and facial CT-scan and MR imaging to check any bony lesions, skull base extension by foramen cæcum, course of the sinus and the possible associated brain malformations. Evolution, treatment and follow-up (FU) were conducted by the same multidisciplinary team (neurosurgeon, ENT surgeon, and plastic surgeon). Children were operated on by a conjoined approach (cranial and facial) for removal of the sinus and its intracranial extension.

RESULTS

Mean age at diagnosis was 12 months (birth-36 months). Initial presentation consisted of three local infections, one dorsum nasal lump, one CSF leakage, and one asymptomatic child. Five children presented with a skull base extension. There were no associated brain malformations. We observed only one surgical complication (bleeding from the anterior part of the superior sagittal sinus during dissection) leading to blood transfusion. Pathology results confirmed three dermoid cysts, one epidermoid cyst, one cyst with granulation tissue, and negative in one case. Average FU was 30.8 months (4-84 months). Two recurrences (same child) occurred, leading to two re-operations. There were no recurrences or complications at the end of FU.

CONCLUSION

NDSC are rare malformations, mostly diagnosed before the age of three years, due to an infectious complication. The aim of the treatment is complete removal to avoid recurrence, and a multidisciplinary strategy is required.

摘要

引言

儿童鼻皮样窦(NDSC)是一种罕见的畸形(1/20,000至1/40,000)。除局部感染外,它们表现为鼻背部的正中肿块或凹陷,其诊断和治疗常常延迟。未经治疗的NDSC的后果包括:局部感染、脑膜炎和脓胸,因为它们常常向颅内蔓延。

患者与方法

回顾性分析6例NDSC患者(5例男孩和1例女孩),均于2006年至2012年在我们机构(南锡大学医院)接受治疗。所有患者均接受脑部和面部CT扫描及磁共振成像检查,以检查是否存在任何骨质病变、通过盲孔的颅底扩展情况、窦道的走行以及可能相关的脑部畸形。由同一多学科团队(神经外科医生、耳鼻喉科医生和整形外科医生)进行病情进展、治疗及随访(FU)。儿童通过联合手术(颅脑和面部)切除窦道及其颅内扩展部分。

结果

诊断时的平均年龄为12个月(出生至36个月)。初始表现包括3例局部感染、1例鼻背部肿块、1例脑脊液漏和1例无症状儿童。5例儿童存在颅底扩展。无相关脑部畸形。我们仅观察到1例手术并发症(解剖过程中上矢状窦前部出血),导致输血。病理结果证实3例为皮样囊肿,1例为表皮样囊肿,1例为肉芽组织囊肿,1例为阴性。平均随访时间为30.8个月(4至84个月)。发生2例复发(同一患儿)并导致2次再次手术。随访结束时无复发或并发症。

结论

NDSC是罕见的畸形,大多因感染性并发症在3岁前被诊断。治疗目的是彻底切除以避免复发,需要多学科策略。

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