Seashore J H, Gardiner L J, Ariyan S
Am J Surg. 1985 Apr;149(4):459-65. doi: 10.1016/s0002-9610(85)80040-4.
Giant cystic lymphangioma is a rare lesion that presents major therapeutic challenges. Four infants presented in the first weeks of life with diffuse cysts in the neck. The tongue was involved in three, the mediastinum in one, and the pharynx and supraglottic larynx in two. These lesions are characterized by hundreds of cysts that infiltrate in and around muscles, nerves, and vessels. Complete excision is not possible without damaging the normal structures. All four infants initially had partial removal with unroofing and drainage of the remaining cysts and temporary tracheostomy to protect the airway. One infant died from a thrombosed sagittal sinus. She had severe venous congestion of the head and neck postoperatively which may have been caused by an overzealous attempt to remove all of the cysts. The other three have required nine additional operations for removal of recurrent neck cysts (one operation), removal of mediastinal cysts (one operation), partial glossectomy (three operations), and laser excision of lymphangiomas of the pharynx and supraglottic larynx (four operations). Two patients have required partial glossectomy which should be performed early to protect the airway, to allow normal speech development, and to prevent malocclusion and prognathism from constant pressure of the tongue against the teeth. Removal of wedges of tissue from each side and from the center of the dorsum of the tongue reduces bulk with minimal risk to the lingual arteries and nerves. Recurrent tongue enlargement is common and can be treated by repeated partial glossectomy. Lymphangiomatosis of the pharynx and supraglottic larynx is difficult to treat. The carbon dioxide laser allows very precise excision of individual cysts with minimal drainage to adjacent mucosa, but in our limited experience it has not prevented recurrence. Despite the extensive nature of these lesions, the impossibility of complete excision, and the need for multiple operations, the long-term results in the three surviving patients have been satisfactory. Asymmetry of the neck and face due to lymphedema tends to improve with time. All three children have an acceptable appearance and normal speech for their ages.
巨大囊性淋巴管瘤是一种罕见的病变，带来了重大的治疗挑战。四名婴儿在出生后的头几周出现颈部弥漫性囊肿。三名婴儿的舌头受累，一名婴儿的纵隔受累，两名婴儿的咽部和声门上喉受累。这些病变的特征是数百个囊肿浸润于肌肉、神经和血管及其周围。不损伤正常结构就不可能完全切除。所有四名婴儿最初都进行了部分切除，包括囊肿去顶和引流剩余囊肿，并进行临时气管切开术以保护气道。一名婴儿死于矢状窦血栓形成。她术后头颈部出现严重静脉充血，这可能是由于过度积极地试图切除所有囊肿所致。其他三名婴儿还需要进行另外九次手术，分别用于切除复发性颈部囊肿（一次手术）、切除纵隔囊肿（一次手术）、部分舌切除术（三次手术）以及激光切除咽部和声门上喉的淋巴管瘤（四次手术）。两名患者需要进行部分舌切除术，应尽早进行以保护气道、促进正常语言发育，并防止因舌头持续压迫牙齿导致错颌和下颌前突。从舌头两侧和舌背中央切除楔形组织可减少体积，同时对舌动脉和神经的风险最小。复发性舌肿大很常见，可通过重复进行部分舌切除术进行治疗。咽部和声门上喉的淋巴管瘤难以治疗。二氧化碳激光可非常精确地切除单个囊肿，并将对相邻黏膜的引流降至最低，但根据我们有限的经验，它并不能防止复发。尽管这些病变范围广泛、无法完全切除且需要多次手术，但三名存活患者的长期结果令人满意。由于淋巴水肿导致的颈部和面部不对称往往会随着时间改善。所有三名儿童的外观和语言能力与其年龄相符，均可接受。
