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Huge neck lymphangioma removed in toto with minimal access.

A 16-year-old female patient presented with a huge neck swelling that had been present since she was 2 years of age. On examination, the swelling was found to measure approximately 20x15 cm. The swelling was mostly solid with cystic areas in between, and it was hanging down to the upper chest with the overlying skin stretched and discolored (figure 1).

After considering all the differentials of cystic neck swelling, such as lymphangioma, cavernous hemangioma, branchial cleft cyst, and teratoma, we decided to perform fine-needle aspiration cytology, which revealed benign cystic swelling with the possibility of lymphangioma. Contrast-enhanced computed tomography (CT) showed a huge lesion reaching superiorly to the patient's skull base and inferiorly to the suprasternal notch. The lesion involved the parapharyngeal space, with attenuation of the great vessels (figure 2).

As expected, sclerotherapy with multiple injections of bleomycin to the swelling produced no response because of the lesion's size. Because of the risk of the patient's developing airway obstruction if the swelling expanded farther, she was taken to surgery for excision via a transcervical approach without a mandibulotomy. The gross specimen after removal weighed 1.42 kg and measured 20 x 15 x 9 cm in its largest dimensions (figure 3). Histopathology of the specimen was consistent with lymphangioma. To the best of our knowledge, this is the world's largest neck lymphangioma ever removed in toto with minimal access. The patient was allowed to eat and drink orally within 24 hours, and she could speak normally almost immediately. She remained free of the disease at her 2-year follow-up.

Lymphangiomas usually result from sequestration or obstruction of developing lymph vessels. The clinical effects with which these patients present depends on their relationship with vital structures, although most patients present with a painless lump and no other symptoms.

The management of such lesions is still a matter of great debate. Various treatment modalities are available, including sclerotherapy, cryotherapy, excision, radiotherapy, and embolization. Complete surgical excision is traditionally considered the treatment of choice, (1,2) although sclerotherapy also has been considered appropriate. (3) If the planes are well defined, surgical excision remains the treatment of choice.

Treatment selection should be based on the size, depth, and location of the lesion, as well as potential morbidity and surgical complications. In our case, we initially tried sclerotherapy because lymphangiomas generally are infiltrating in nature, making surgical excision difficult, but it was not effective. Considering the possibility of airway obstruction and death with such a large swelling, we decided to perform surgical excision despite the vital structures involved.

In our case, the transcervical surgical approach without mandibulotomy was significant, demonstrating that huge lesions can be removed without mandibulotomy and with minimal access, thereby avoiding postoperative feeding and speech difficulties and leading to faster recovery. Because such lesions have a propensity for recurrence, our patient remains on a rigorous monthly follow-up. Early detection of lymphangiomas is essential for better management.

Vikas Malhotra, MBBS, MS(ENT), DNB; Nikhil Arora, MBBS, MS(ENT), DNB; Pankhuri Mittal, MBBS, MS(ENT), DNB

References

(1.) Riechelmann H, Muehlfay G, Keck T, et al. Total, subtotal, and partial surgical removal of cervicofacial lymphangiomas. Arch Otolaryngol Head Neck Surg 1999;125(6):643-8.

(2.) Mandel L. Parotid area lymphangioma in an adult: Case report. J Oral Maxillofac Surg 2004;62(10):1320-3.

(3.) Sichel JY, Udassin R, Gozal D, et al. OK-432 therapy for cervical lymphangioma. Laryngoscope 2004; 114(10): 1805-9.

Caption: Figure 1. Photo shows the huge mass hanging from the patient's neck to her upper chest.

Caption: Figure 2. Contrast-enhanced coronal (A) and saggital (B) CT scans reveal the large swelling.

Caption: Figure 3. The excised specimen measures 20 x 15 x 9 cm.
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Title Annotation:HEAD AND NECK CLINIC
Author:Malhotra, Vikas; Arora, Nikhil; Mittal, Pankhuri
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Date:Dec 1, 2018
Words:613
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