Long-Term Follow-up With Multispecialty Management of a Giant Lymphangioma of an Infant Tongue Contributed to Reduced Complications of the Disease: A Case Report of a 21-Year Follow-up

Lymphangiomas are benign tumors formed from abnormal lymphatic tissue because of congenital lymphatic developmental malformations.¹ Approximately 6% of all lymphangiomas occur on the tongue.² Fifty percent of the cases are detected at birth, and about 90% of all patients have their lymphangioma diagnosed before 2 years of age.^1,2 A lymphangioma of the tongue in children can result in difficulty in swallowing and mastication, airway obstruction, speech disturbances, mandibular prognathism, open bite, and maxillofacial deformities.^2,3

Various treatment modalities are available for the management of lymphangiomas, such as surgical excision, Nd-YAG laser therapy, and cryotherapy.⁴ Other treatments include the use of sclerosing agents, the application of carbon dioxide laser therapy, steroid administration, embolization, and liquid nitrogen cryotherapy. However, such methods are inefficient due to the possibility of lesion relapse.^4,5 In addition, given that this disease occurs in the early years of life and the physical and psychological status of patients change with age, it is necessary to start multidisciplinary management, including dietary consultation, speech therapy, and orthodontic management, at an early stage.

There has been no study on long-term follow-up from baby to adult using multiple treatment approaches. This is the first study to report 21 years of follow-up with multidisciplinary treatments from an early stage of a male infant with a giant lymphangioma of the tongue, showing a positive outcome on ordinary physical and psychosocial life.

A 4400-g 1-month-old infant presented with macroglossia with cervical and anterior thoracic masses. The infant came from healthy parents without any known medical family history or pregnancy-related complications. The first clinical and magnetic resonance imaging (MRI) examinations found a lymphangioma in the tongue (Figure 1). Because there were no subjective or acute symptoms, he was scheduled for follow-up including clinical check-ups and MRI examinations.

At 10 months of age, his tongue was enlarged with lymphangioma, and its surface became dry and triggered constant bleeding. Additionally, lymph liquid was extravasated. He experienced difficulty in withdrawing the tongue into the mouth, as well as in swallowing and speaking. To alleviate recurring exacerbations of the tongue size and bleeding, the infant underwent tracheotomy (inverted U-shaped incision from tracheal cartilage I to IV, which prevented postoperative airway stenosis. This was followed by a partial glossectomy using Obwegeser method via a nasogastric tube to avoid dysphagia, undernutrition, and difficulty in voluntary oral intake after surgery (Figure 2a–c). Then, tracheoplasty was performed to improve airflow in his obstructed airway. These treatments led to improved conditions of the affected area.

To develop speech communication, speech therapy was started under the guidance of a speech therapist. At 1.5 years of age, the patient began to use Makaton sign language, a type of simple gesture language, as an augmentative alternative to traditional speech communication. After 7 months of treatment, his speech was developed, and there was no longer a need for sign language to facilitate communication.

At 4 years of age, a second glossectomy was performed to reduce the thickness of the tongue, combined with1 Klinische Einheit (KE, used to express the strength of the preparation; 1 KE corresponds to 0.1 mg of dried streptococci) injection of a sclerosing agent Picibanil (OK-432) to the lymphangioma on the floor of the oral cavity, which was an extension of the glossal lesion (Figure 3a-d). Thereafter, no recurrence was observed, but training by a speech therapist and dietician was continued.

After this multidisciplinary treatment intervention at an early stage along with continuous training, the patient was able to speak clearly and digest food normally at 10 years of age. Because the quality of life of the patient was remarkably improved, training by the speech therapist and dietician was concluded.

At the age of 10 years, the patient was referred to an orthodontic clinic for an open bite treatment (Figure 4a-f) to control the Class III skeletal growth pattern and the tongue position. Treatments for lateral expansion and anterior protrusion of the maxilla were performed, and the patient was instructed to use a facial mask every night for a year. One year later, multi-bracket treatment was additionally performed, and the patient was instructed to continue using a facial mask at night. The patient underwent these treatments every year for 6 years with regular checkups and oral care to avoid dental caries. His oral occlusions had improved at 17 years of age, and the multi-bracket treatment was concluded. Subsequently, the patient started using a retainer to maintain the occlusions (Figure 5a–f).

At the age of 19 years, a small lymphangioma (3 cm in diameter, purple in color) was found in the right lower mandibular region (Figure 6). To destroy the lesion tissue, a total 2.5 KE Picibanil injection was administered to the affected region. The adverse effects included slight fever and swelling, which eased gradually after few days. In addition, there was no influence on the speech function. After the injection, the swelling of the right lower mandibular region subsequently subsided. At 22 years of age, there was no recurrence, and the patient was no longer under observation.

Due to the nature of lymphangiomas, medical intervention from early stage with multidisciplinary management and long-term follow-up is indispensable. Several surgical excisions, sclerotherapy, and long-term follow up were performed, along with speech therapist, dietician, and orthodontic management. This resulted in no recurrence after 22 years, and the patient is able to lead an ordinary life both physically and socially.

This is the first report of reduced oral obstacles in a male infant who presented with a giant tongue lymphangioma including long-term follow-up for over 21 years. A multispecialty management that included surgical excision and sclerotherapy, as well as a multidisciplinary approach including dietary consultation, speech therapy, and orthodontic treatments, resulted in diminished complications (Figure 7). Previous observations indicated that treating complications of a tongue lymphangioma is difficult after a growth spurt starts.³ Specifically, when lymphangiomas enlarge, they may result in complications such as airway constriction, dysphagia, difficulty in speech, occlusion deformity, mandibular prognathism, open bite, and other maxillofacial structural deformities.^2,6 Therefore, we planned to provide regular tumor check-ups with concomitant multidisciplinary management, including dietary consultation, speech therapy, and orthodontic therapy, from the initial visit of the patient.

Various treatments for tongue lymphangioma, including surgical excision, sclerotherapy, radiation therapy, steroid administration, and embolization, have been reported,¹ but the treatment of choice is the subject of discussion, as there are no established guidelines. Surgical excision is effective in cases of tumor enlargement, frequent infections, debilitating functions, and/or life-threatening symptoms.⁷ It was expected that malocclusion, mandibular deformity, and delay of speech development would occur if this patient was left untreated. Surgical excision was considered the best alternative for lesions that presented localized growth. At 10 months after birth, a tracheotomy was conducted to prevent the postoperative complication of airway stenosis, and a partial glossectomy using the Obwegeser method was performed via a nasogastric tube. This excision alleviated recurring exacerbations of the tongue size and bleeding and avoided dysphagia, undernutrition, and difficulty in voluntary oral intake.

Picibanil is one of the most recently available sclerosing agents⁵ and is recommended for use in recurrent and unresectable cases.⁸ In this case, Picibanil was administered to the patient for sclerotherapy of lymphangioma at 4 and 19 years of age. In the latter case, a small lymphangioma was found in the right lower mandibular region and Picibanil was administered to the lymphangioma on the floor of the oral cavity and the right lower mandibular area. After these injections, the swelling subsided, and no further recurrence was noted.

The present results strongly suggest that Picibanil injection combined with a partial glossectomy at an early age is an effective treatment option for lymphangioma. However, it must be noted that sclerotherapy is not recommended as an initial treatment in cases of tongue lymphangiomas because it may result in tongue sclerosis with poor speech, taste, and swallowing functions.

This study demonstrated a successful long-term multidisciplinary approach for follow-up owing to the highly recurrent nature of the disease and its effects on tongue shape, speech, diet, and orthodontic and psychological health. This treatment did not focus only on controlling the tumor and protecting physical functions but also included multidisciplinary treatments, including dietary consultation, speech therapy, and orthodontic treatment; therefore, the combined medical and extra-medical managements must be considered to improve the quality of life of patients with lymphangioma.

The current study on the follow-up of a case of tongue lymphangioma over 21 years led not only to the diminishment of the tumor and preservation of physical integrity but also to the improvement of oral conditions, including dietary intake, speech, and alignment of teeth. Through long-term follow-up with multispecialty management, the patient was able to live an ordinary life both physically and socially. Due to the high risks of lymphangiomas, such as high recurrence rate and early onset, physicians should start treatment at an early age and provide long-term follow-up care after considering all aspects.

Affiliations: ¹Kansai Medical University, Department of Plastic and Reconstructive Surgery, Osaka, Japan; ²Yamamoto Dental Clinic, Orthodontic Clinic, Hyogo, Japan; ³Fukuda Plastic Surgery Clinic, Osaka, Japan

Correspondence: Tae Nagama, MD; tae.buttle@gmail.com

Ethics: Written informed consent was obtained from the patient for the publication of this report.

Disclosures: This research did not receive any grant from funding agencies in the public, commercial, or not-for-profit sectors, and the authors report no conflicts of interest.