Lymphatic malformations are rare benign tumors that stem from a malformation of the lymphatic vessels in the soft tissues and account for 5–6% of all benign soft tissue tumors in children [1, 5, 7]. The majority (75%) of these lesions develop in the cervicofacial region and the diagnosis can be done before the age of 2 years in 90% of the cases [3, 4]. No significant sexual or racial differences have been reported regarding the incidence of LMs [7]. In this study, 66.7% of the patients had their lesions in the neck, 77.8% of the patients were presented before the age of 2 years, and the male to female ratio was 1:0.8.
Macrocystic lymphatic malformations are defined as those lesions with cysts greater than 2 cm in diameter and having clear boundaries [4, 5]. Asymptomatic mass with parental esthetic concern is the usual presentation of macrocystic lymphatic malformations in infants and children. Even when large, these lesions may not cause symptoms as they grow within an overlying elastic skin and subcutaneous tissue especially in the cervicofacial region where these lesions predominate [7]. Painless mass was the mode of presentation in the majority (66.7%) of patients in this study. Similar findings were reported in previous relevant series [7, 8].
Lymphatic malformations are liable to infection because the abnormal lymphatic vessels have limited ability to clear foreign materials, besides, the high proteinaceous fluid and blood content of the cysts in favor of bacterial overgrowth [9]. Two (22.2%) patients in this study suffered infection of their lesions and both were given injectable antibiotic therapy to resolve the problem preoperatively. Infection rates are comparable to those reported in other studies [8].
Respiratory distress with feeding difficulties are serious complications of LMs that result from a sudden increase in the size of the lesion following infection or intralesional bleeding or secondary to compression of the mass on the upper airway. In our study, these serious complications were reported in one (11.1%) patient with a giant lymphatic malformation involving both sides of the neck with extension into the lingual base. Similar results were observed in similar previous studies [8].
Historically, surgical excision has been the initial therapeutic option for LMs, and since the first description of the macrocystic variety, several therapeutic modalities including intralesional sclerotherapy and laser therapy have emerged; however, surgery remains the initial therapeutic option of choice for many surgeons and has proven effective [3, 8]. Surgical resection of giant macrosystic LMs is challenging. These lesions tend to insinuate major blood vessels, nerves, and muscles, and all surgeons attempting radical surgical excision should be aware of the anatomic details to avoid unnecessary damage to vital structures. Patients with cervicofacial lesions in particular are at risk of airway obstruction, functional damage, and recurrent or persistent lesions [8].
In this study, radical surgical excision was achieved in the majority (88.9%) of patients (Fig. 4a–c). No significant neurovascular damage has been reported in any of the patients operated upon (Fig. 5a, b). All of the patients regained full immediate postoperative recovery following uneventful extubation. Residual lesion was recorded in only one patient with a giant lesion involving both sides of the neck with sublingual extension. Ma et al. and Chen et al. reported complete surgical resection in 82.4% and 89.4% of their case series respectively [7, 8].
major postoperative complications regarding problems with breathing, swallowing, or phonation were recorded in this study. Cosmetic results were satisfactory in the majority (88.9%) of patients (Figs. 6 and 7) and no recurrence was reported after 1 year of follow-up. Similar results were reported in previous other studies [8].
Over the past several decades, several alternative therapeutic modalities emerged including the use of intralesional sclerotherapy. In 2014, Balakrishnan et al. [10] were the first to do a head-head comparison between primary surgery and primary sclerotherapy in the treatment of head and neck LMs and found that both modalities were equally effective in a multisite comparison. In 2007, Okazaki et al. [11] reported serious complications of treating LMs with OK-432 and surgery. They found that sclerotherapy may cause fever, infection, upper respiratory tract obstruction, or anaphylactic shock and suggested that sclerotherapy with OK-432 was not as effective as previously reported.
