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自发性消退的大囊型淋巴管畸形:预测变量与结局

Spontaneously Resolved Macrocystic Lymphatic Malformations: Predictive Variables and Outcomes.

作者信息

Phang Michael J, Courtemanche Douglas J, Bucevska Marija, Malic Claudia, Arneja Jugpal S

机构信息

University of British Columbia, Vancouver, British Columbia, Canada.

Division of Plastic Surgery, BC Children's Hospital, Vancouver, British Columbia, Canada.

出版信息

Plast Surg (Oakv). 2017 Feb;25(1):27-31. doi: 10.1177/2292550317693815. Epub 2017 Mar 13.

Abstract

INTRODUCTION

Lymphatic malformations are benign, low-flow vascular malformations that typically present at or near birth. Due to morbidity associated with operative treatment, nonoperative treatment with injection of sclerosant has become the mainstay of therapy. Over the past 15 years, several patients at our centre with macrocystic (>2 cm cyst size) lymphatic malformations have seen their lesions resolve spontaneously while awaiting treatment. In this study, we review features of these patients that may contribute to spontaneous resolution.

METHOD

A retrospective chart review was conducted from our Vascular Anomalies Clinic database (1999-2014) of all macrocystic lymphatic malformations; characteristics of patients with spontaneous resolution were reviewed.

RESULTS

Of 61 patients with macrocystic lymphatic malformations, 7 cases (11.5%; 4 females, 3 males) resolved spontaneously. Median age at malformation appearance was 2 years (range: 0-6.5 years), with median age at resolution of 4 years (range: 10 months-7 years). Median time from appearance to resolution was 24 months (range: 3-43 months), with a median follow-up time of 4 years (range: 1-15 years). All but 1 case was associated with local or upper respiratory tract infection antecedent to resolution. Six of the 7 lesions were located in the neck.

CONCLUSION

Among the cases reviewed, there was a common theme of upper respiratory tract or local infection antecedent to spontaneous lesion resolution. Compared to the literature, our proportion of malformations presenting after birth and the proportion of malformations presenting in the neck region were higher than those of other series. Although side effects associated with treatment are generally mild and/or rare, risks related to sclerotherapy and the accompanying requirement for general anesthesia in pediatric populations nevertheless exist. As the median time from lesion appearance to resolution was 24 months, it may be reasonable to observe these malformations for up to 24 months before proceeding with treatment if the lesion does not impair function and disfigurement is not considerable, particularly if the lesion presents after birth and/or is located in the neck region.

摘要

引言

淋巴管畸形是一种良性、低流量的血管畸形,通常在出生时或出生后不久出现。由于手术治疗存在相关并发症,注射硬化剂的非手术治疗已成为主要治疗方法。在过去15年中,我们中心的几名患有大囊型(囊肿大小>2 cm)淋巴管畸形的患者在等待治疗期间,其病变自行消退。在本研究中,我们回顾了这些患者可能有助于自发消退的特征。

方法

从我们的血管异常诊所数据库(1999 - 2014年)中对所有大囊型淋巴管畸形进行回顾性病历审查;对病变自发消退患者的特征进行了分析。

结果

在61例大囊型淋巴管畸形患者中,7例(11.5%;4例女性,3例男性)自发消退。畸形出现的中位年龄为2岁(范围:0 - 6.5岁),消退的中位年龄为4岁(范围:10个月 - 7岁)。从出现到消退的中位时间为24个月(范围:3 - 43个月),中位随访时间为4年(范围:1 - 15年)。除1例病例外,所有病例在消退前均伴有局部或上呼吸道感染。7处病变中有6处位于颈部。

结论

在所审查的病例中,病变自发消退前存在上呼吸道或局部感染这一情况较为常见。与文献相比,我们的病例中出生后出现畸形的比例以及颈部出现畸形的比例高于其他系列。虽然治疗相关的副作用通常较轻和/或罕见,但硬化治疗相关风险以及儿科人群所需的全身麻醉风险仍然存在。由于从病变出现到消退的中位时间为24个月,如果病变不影响功能且外观畸形不严重,特别是如果病变在出生后出现和/或位于颈部区域,在进行治疗前观察这些畸形长达24个月可能是合理的。

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