Complications of congenital melanocytic naevi in children: analysis of 16 years' experience and clinical practice
Complications of congenital melanocytic naevi in children: analysis of 16 years' experience and clinical practice
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DOI：
10.1111/j.1365-2133.2008.08775.x
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发表时间：
2008-10-01
影响因子：
10.3
通讯作者：
Atherton, D. J.
中科院分区：
文献类型：
--
作者：
Kinsler, V. A.;Chong, W. K.;Atherton, D. J.
背景先天性黑色素细胞痣（CMNs）可能与中枢神经系统（CNS）异常和/或黑色素瘤有关。引用这些并发症的发病率在literature中有所不同，做调查和follow-up.Objectives的建议，以确定并发症的发生率，并确定与并发症的风险较高的表型特征。方法我们回顾了224例CMN患者在皮肤科诊所1991年和2007年之间的记录。如果患者在转诊时有并发症，则将其排除。根据CMN表型提供CNS的磁共振成像（MRI）。结果120例患者（男54例，女66例）均接受了中枢神经系统MRI检查。MRI检查时的平均年龄为2.46岁（中位数1.20）。平均随访时间为8.35年（中位数7.86年）。65%的患者有痣> 20 cm，预计成人大小或多个CMN（40% > 40 cm），83%的患者在出生时有卫星病变。结局指标为MRI异常、临床神经系统异常、任何肿瘤、恶性黑色素瘤和死亡。出生时无卫星病变的16例患者中未观察到并发症。在22名患者（18%）中发现MRI和/或临床神经系统异常，并且与CMN的预计成人大小（特别是> 40 cm）显著相关，并且与男性性别无关。5例患者发生肿瘤，其中2例为恶性黑色素瘤（1.7%）。由于数量少，表型和肿瘤发生之间没有显着关联。3例患者（2.5%）死亡（MRI扫描正常的患者中，1例死于神经黑变病，2例死于黑色素瘤）。死亡与CMN大小> 40 cm显著相关。重要的是，CMN分布（包括后方轴向位置）和不良outcomes.Conclusions这是最大的研究中枢神经系统成像患者CMNs之间没有显着的关联。我们报告了一个新认识的男性性别和神经系统并发症之间的关联，争议之前报道的CMN网站和神经系统并发症之间的关联，并量化CMN大小，卫星病变和神经系统并发症之间的关联。我们对这些患者的管理提出建议。
Background Congenital melanocytic naevi (CMNs) can be associated with abnormalities of the cental nervous system (CNS) and/or with melanoma. Quoted incidences for these complications vary in the literature, as do recommendations for investigations and follow-up.Objectives To determine the incidence of complications, and to identify phenotypic features associated with a higher risk of complications.Methods we reviewed records of 224 patients with CMNs seen in Dermatology clinic between 1991 and 2007. Patients were excluded if they had a complication at the time of referral. Magnetic resonance imaging (MRI) of the CNS was offered on the basis of CMN phenotype. Follow up was in clinic and/or by postal questionnaires.Results One hundred and twenty patients (54 boys and 66 girls) who had MRI of the CNS were included in the analysis. Mean age at MRI was 2.46 years (median 1.20). Mean follow up was 8.35 years (median 7.86). Sixty-five per cent had naevi > 20 cm projected adult size or multiple CMNs (40% > 40 cm), and 83% had satellite lesions at birth. Outcome measures were MRI abnormality, clinical neurological abnormality, any tumour, malignant melanoma, and death. No complications were seen in the 16 patients with no satellite lesions at birth. MRI and/or clinical neurological abnormalities were found in 22 patients (18%) and were significantly associated with projected adult size of the CMN (particularly > 40 cm), and independently with male gender. Tumours occurred in five patients, two of which were malignant melanoma (1.7%). Due to small numbers there was no significant association between phenotype and occurrence of tumours. Three patients (2.5%) died (one from neuromelanosis and two from melanoma in patients with normal MRI scans). Death was significantly associated with CMN size > 40 cm. Importantly, there was no significant association between CMN distribution (including posterior axial location) and adverse outcomes.Conclusions This is the largest study of CNS imaging in patients with CMNs. We report a newly recognized association between male gender and neurological complications, dispute the previously reported association between CMN site and neurological complications, and quantify the associations between CMN size, satellite lesions and neurological complications. We make recommendations for the management of these patients.