05503R#3 8/9/03

18th Congress of AAPS

Huge Lymphangioma of the Tongue: A Case Report

Eri Tei, Atsuyuki Yamataka, Yuzo Komuro,1 Kiyohiko Ohshiro, Akira Yanai,1 Geoffrey J. Lane and Takeshi
Miyano, Departments of Pediatric Surgery and 1Plastic Surgery, Juntendo University School of Medicine, Tokyo, Japan.

Lymphangioma of the tongue is relatively rare and may cause facial structural deformity. Using a combination
of a V-shaped and central resection, we successfully treated a 6-year-old girl who had massive lymphangioma of
the tongue. Postoperatively, her tongue was located completely within her mouth with good cosmetic results.
Sensory and motor nerves to the tongue appeared to be intact. Her speech was also improved. [Asian J Surg 2003;
26(4):228–30]

Introduction A

Lymphangiomas are congenital benign tumours of the lym-

phatic system, and 50% to 75% are located in the head and
neck.1 They may arise in the tongue, and are a common cause
of macroglossia in children. Lymphangiomas of the tongue
(LTs) do not regress spontaneously, and they are almost always
of the cavernous type.2 The lesions are not tender or painful.
However, inflammation from trauma or infection may result
in sudden enlargement and severe pain. LT may cause gross
structural deformities of the face and interfere with speech
and swallowing.2 We report a case of massive LT, in which
surgical resection was successfully performed, leading to good
cosmetic and functional results. B

Case report

A girl born with macroglossia was diagnosed with LT and

managed conservatively at a district hospital. When she was 5
years old, the tongue suddenly enlarged after an episode of
infection (Figure 1). Following this, she had many problems;
the tongue could not be withdrawn into the oral cavity, the
mouth could not be closed, and saliva dribbled from the
corners of her mouth. She was eating only soft food with liquid
and enteral nutritional supplementation. Her sensation of
taste was intact and her speech was not severely affected. There Figure 1. Massive lymphangioma in the tongue: A) frontal view;
were no other associated anomalies. At the age of 6, she was B) lateral view.

Address correspondence and reprint requests to Dr. Atsuyuki Yamataka, Department of Pediatric Surgery,
Juntendo University School of Medicine, 2-1-1 Hongo, Bunkyo-ku, Tokyo 113-8421, Japan.
E-mail: yama@med.juntendo.ac.jp • Date of acceptance: 1st May, 2003

© 2003 Elsevier. All rights reserved.

228 ASIAN JOURNAL OF SURGERY VOL 26 • NO 4 • OCTOBER 2003

03502R
 ■ LYMPHANGIOMA OF THE TONGUE ■

cal resection, radiation therapy, cryotherapy, electrocautery,

sclerotherapy, steroid administration, embolization, ligation
and laser surgery are treatment options for LT.1 Lymphangi-
oma is resistant to medical treatment. Radiotherapy is effec-
tive in the early stages, but the tumour recurs in many cases.3
Although the sclerosing agent OK-432 is effective for cystic
lymphangioma, it is not usually effective in the cavernous
type.4,5 Because of this, surgical resection appeared to be the
most effective treatment for LT in this case.
Partial glossectomy of the tongue has been reported to be
an effective treatment for LT. Typical glossectomy can be
roughly divided into three procedures: V-shaped resection,
bilateral marginal resection, or central resection. Taste is de-
tected by the tongue, the soft palate, the pharynx and the
Figure 2. Magnetic resonance image shows that the lymphangioma buccal mucosa. Although the whole tongue can detect taste,
is localized within the tongue (arrows). There is no extension into
the pharynx and neck. most of the taste buds are located on the sides of the tongue.
Thus, when the tongue is resected, it is desirable to leave the
transferred to our hospital for resection of the lesion. Mag- sides intact. To limit injury to taste and motor functions, a
netic resonance imaging (MRI) showed that the LT was local- central resection is preferable;6 however, satisfactory resection
ized within the tongue, with no extension into the pharynx or
neck (Figure 2).
A B
The Köle technique, which is a combination of V-shaped
resection and central resection, was used under general
nasotracheal anaesthesia. Approximately 6 × 5 cm of the
tumour was resected by partial glossectomy in the shape of a
boat’s keel (Figure 3), and care was taken to minimize injury to
the VII, IX and XII cranial nerves. After resection, the tongue
was relatively normal in size and shape, although some lym-
phangiomatous tissue remained in each side of the tongue.
The postoperative course was uneventful. Histopathological
examination of the resected specimen showed the presence of
many cavernous lymphatic spaces within the lamina propria
and the underlying muscle.
The patient was discharged 2 weeks after surgery, with a C D
normal-looking tongue and marked improvement in speech
(Figure 4). At follow-up 6 months after surgery, her tongue was
located completely within her mouth, with good cosmetic
results. Tongue sensory and motor functions were intact and
her speech was continuing to improve. She has a mandible
deformity and anterior open bite secondary to chronic un-
treated macroglossia and will probably require orthodontic
treatment.

Discussion
Figure 3. Intraoperative photographs: A) planned incision;
The goal of treatment of LT is to obtain good cosmesis, B) resection of the tongue; C) resected lymphangioma of the
prevent facial deformity, and prevent speech problems. Surgi- tongue; and D) approximation of the remaining tongue.

229
ASIAN JOURNAL OF SURGERY VOL 26 • NO 4 • OCTOBER 2003 229
 ■ TEI AND OTHERS ■
A phonetic, psychological and cosmetic problems.8 If the mouth
B
Figure 4. Postoperative photographs: A) the size of the tongue is
almost normal; B) although the patient still has a mandible deform-
ity secondary to chronic untreated macroglossia, her tongue is
located completely within her mouth.
cannot be obtained with central resection alone and the results
are usually cosmetically unsatisfactory. The Köle method, 7
which is a combination of V-shaped resection and central
resection, was chosen in our case to reduce the size of the
tongue to as close to normal as possible.
230
Surgical resection of LT is indicated to prevent orthodontic,
cannot close due to an enlarged tongue, this will cause occlusal
imbalance, mandible deformity and delay of speech develop-
ment. It will be difficult to treat these problems after a growth
spurt starts. Thus, surgical reduction of the tongue is recom-
mended before the anterior teeth have been completely
replaced by adult teeth (around 7 years of age).9 In our case,
psychological problems were resolved and quality of life
improved dramatically after surgery. However, careful long-
term follow-up is required because mandible deformity and
anterior open bite still remain, and LT may recur from the
residual lesions.
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