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[颈部囊性淋巴管瘤的当前研究进展]

[Current aspects of cystic lymphangioma in the neck].

作者信息

Chappuis J P

机构信息

Service de chirurgie pédiatrique, hôpital Edouard-Herriot, Lyon, France.

出版信息

Arch Pediatr. 1994 Feb;1(2):186-92.

PMID:7987449
Abstract

Cystic hygroma of the neck (CHN) is the result of a defect in the embryonary development of the lymphatic system. In many cases foetal ultrasound allows an early diagnosis before birth, the prognosis being very different according to the gestational age at the time of detection. When discovered before 30 weeks of gestation it is almost always associated with chromosomal abnormality and/or polymalformation leading to spontaneous or therapeutic abortion. On the contrary when appearing after the 30th week of gestation, CHN is usually an isolated malformation as when discovered during infancy or childhood. Approximately 2/3 of these "late" CHN are present at birth; 90% are discovered before 2 years of age. A spontaneous regression occurs in about 15% of the cases. In 70% of cases the CHN is simple without extension to the oropharynx or mediastinum and its complete surgical resection is usually easy. Extension to the oropharynx is present in about 20% of the cases; there is a risk of neonatal respiratory distress and the treatment is difficult. Extension to the mediastinum is found in about 10% of the cases; respiratory distress is rare and a large surgical resection is necessary. Surgery is the primary treatment of CHN after a careful evaluation of the extension of the tumor by ultrasound, scanography or nuclear magnetic resonance, and oropharyngeal endoscopy. It allows a "macroscopically complete" resection in about 80% of the cases, but a recurrence is observed in approximately one every five cases. Following partial resection or important recurrence, treatment includes according to the cases; new attempt of surgical resection, sclerosing therapy, and laser therapy for the oropharyngeal forms.

摘要

颈部囊状水瘤(CHN)是淋巴系统胚胎发育缺陷的结果。在许多情况下,胎儿超声检查可在出生前进行早期诊断,根据检测时的孕周不同,预后差异很大。在妊娠30周前发现时,几乎总是与染色体异常和/或多发畸形相关,导致自然流产或治疗性流产。相反,当在妊娠30周后出现时,CHN通常是一种孤立的畸形,如同在婴儿期或儿童期发现的那样。这些“晚期”CHN中约2/3在出生时就已存在;90%在2岁前被发现。约15%的病例会自然消退。70%的病例中,CHN较为简单,未延伸至口咽或纵隔,其完整手术切除通常较为容易。约20%的病例存在口咽延伸;有新生儿呼吸窘迫的风险,治疗困难。约10%的病例发现有纵隔延伸;呼吸窘迫罕见,需要进行大型手术切除。手术是CHN的主要治疗方法,需通过超声、扫描或核磁共振以及口咽内镜仔细评估肿瘤的延伸情况。在约80%的病例中,手术可实现“宏观上完整”的切除,但约每五例中就有一例会复发。部分切除或重要复发后,治疗方法根据具体情况而定;包括再次尝试手术切除、硬化治疗以及对口咽型进行激光治疗。

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