Abstract:
Lymphangiomas of the tongue are very rare tumors usually diagnosed in
infancy and early childhood. Children with Lymphangiomas of the tongue
may require multidisciplinary care and a close collaboration between
physician and dentist to be able to eradicate any sources of dental
infections before undertaking medical treatment.
Keywords: Benign congenital tumors, Lymphangioma, Macroglossia,
Sirolimus, Dental management, Case report.
Key Clinical Message: A multidisciplinary approach, a god
awareness of the patient’s medical history and the oral manifestations
of the underlying conditions are imperative during the oral care of
children with tongue lymphangioma.
Introduction
Lymphangiomas are uncommon developmental anomalies and congenital tumors
of the lymphatic system diagnosed mostly in children under the age of
five years as cysts or lobulated masses, localized in the head or the
neck.1,2 However, the involvement of the tongue, is
considered very rare.2
Lymphangiomas of the tongue are caused by the formation of benign tumors
on the dorsum of the tongue, but it could also occur in the palate,
gingiva, buccal mucosa and, even lips.3
Tongue lymphangiomas typically demonstrated multiple blisters like a
pebbly surface, nodules that appear like a cluster of translucent
vesicles on the enlarged dorsal surface of the tongue, but, these
lesions may also be diffuse involving large portions of the tongue
causing macroglossia.4,5
Aetiological factors can include maternal viral infection, maternal
substance abuse, and environmental factors.6
Lymphangiomas are known to be associated with some syndromes such as
Turner syndrome, Trisomies, fetal alcohol syndrome, cardiac anomalies,
Noonan’s syndrome and, familial hydrops.6
Histopathologically, lymphangiomas have been classified into;
lymphangioma simplex which exhibits small capillary-sized vessels,
cavernous lymphangioma which is composed of large dilated lymphatic
vessels, and cystic lymphangioma or cystic hygroma which consists of
large macroscopic cystic spaces.2,7
These developmental anomalies are the most common cause of macroglossia
in infancy,4 and patients with lymphangioma of the
tongue commonly present with difficulty to retract and to move their
massive tongue which may cause speech disturbances, difficulty in
chewing and swallowing and compromise the mechanical plaque removal
which enhances the caries risk.8
Complications are numerous and frequent, including air-way compromise,
infection, bleeding, and dental caries.9
This case report aimed to discuss the most relevant oral manifestations
and to describe the dental care management of a four-year-old female
patient diagnosed with lymphangioma of the tongue undergoing medical
treatment with Sirolimus.
Case report
This case report was prepared according to the CARE case report
Guidelines.10
A four-year-old female patient was admitted to the Department of
Pediatric and Preventive Dentistry at The Faculty of Dental Medicine of
xxxx.
The patient was referred for general dental care by the Department of
Dermatology at the Hospital of Farhed Hached xxxx, where she had been
diagnostic with microcystic lymphangioma of the tongue and undergone
medical treatment with Sirolimus.
The patient was born to healthy non-consanguineous parents following an
uneventful full-term pregnancy.
The detailed family history revealed no relevant systemic conditions,
and in the head and neck region, no other mass was detected.
The young patient’s medical history revealed that the disease dates to
the first day of birth when the patient presented with dyspnea and was
admitted to the emergency room.
The evolution was then marked by the appearance at the age of five
months of micro-vesicles in the tongue, followed by a swelling of the
tongue interfering with eating and breathing.
The tongue was swollen, bluish in appearance surmounted by microvesicles
with hematic content, and blackish scabs interfering with feeding and
mouth closure.
The patient received several symptomatic treatments without improvement,
and at the age of one and a half, the mother reported that the swelling
of the tongue progressed.
At the age of two years, the diagnosis of microcystic lymphangioma of
the tongue was made. The patient was treated by Sirolimus for six months
with an improvement of the symptoms.
The actual general examination of the patient does not reveal any
abnormalities. Only a notion of recent weight loss was reported because
of the chewing disorder caused by the macroglossia.
The medical history of the patient revealed also that the patient was
admitted twice to the Pediatric Intensive Care Unit for bacterial
pneumonia.
On admission in our department, the patient presented with an enlarged
tongue with nodular, fluffy, white, and infected pink-purple lesions on
the superior and inferior aspects of the tongue (Figure 1). The patient
presented also speech disturbances, swallow inability, breathing
difficulty and complained of burning sensation of the tongue.
The extra-oral examination showed a convex profile with increased lower
facial height.
The intraoral clinical examination revealed, poor oral hygiene with
plaque accumulation, maxillary primary incisors with extensive caries,
an active cavitated carious lesion on teeth 64 (Figure 2), and anterior
open-bite (Figure 3).
A panoramic radiographic examination showed extensive carious lesions
and root resorption in the maxillary incisors.
A mandibular deformity was also seen on the orthopantomography.
The patient presented with an anterior open bite, an increased
mandibular body-ramus angle, an elongated mandibular body, and a
displaced anterior teeth (Figure 4).
Since even brushing teeth was considered to be painful for the patient,
the main objective of the treatment plan was to try to motivate her to
change her behavior, therefore the treatment began with preventive
measures including; diet counseling, basic instructions in oral health
care, and a discussion on improving oral home care skills by including
training on mechanically removing dental plaque and a supervised
toothbrushing.
Because the patient was currently undergoing treatment with Sirolimus to
help decrease the size of the lesion, the dental treatments were carried
out under antibiotic prophylaxis. This decision was made in consultation
with the patient’s physician.
After administering local anesthesia, tooth 64 was isolated using a
rubber dam. The entire carious tissue was removed and the tooth was
sealed with glass ionomer cement GIC (Riva self-cure, SDI, Australia)
than the extraction of the upper incisors (Teeth: 51-52-61-62) was done.
The surgical and conservative treatments were preceded by professional
prophylaxis and topical applications of 2% Neutral Sodium Fluoride Gel
(Master-Dent®, USA).
Follow-up visits, every three months, in collaboration with the
Dermatology Department have been scheduled for the restorative treatment
re-evaluation, observation for the onset of new lesions, monitoring the
tumor evolution and the size of the patient’s tongue to achieve a
removable partial denture to maintain the space of the incisors while
correcting the functions, improving the aesthetics and correcting the
anterior open bite (Figure 5).
Discussion
Lymphangiomas are a relatively rare benign congenital tumors of the
lymphatic system.11
Most of the cases are detected since birth and about 80% are developed
before the age of 2 years.12,13
In the present case report, the patient presented a history of an
enlarged tongue since the age of five months, the tongue continued to
swell, and thereafter caused feeding and breathing but the diagnosis of
microcystic lymphangioma of the tongue was confirmed not until the age
of two years.
When lymphangiomas involve the tongue, a condition frequently referred
to “macroglossia” can occur and can be associated with a set of
problems unique to this anatomic location.14
Macroglossia may lead to two main types of complications: it can cause
dentoskeletal problems, such as anterior open bite, mandibular
prognathism with an increase in mandibular length, higher gonial angle,
anterior facial height and excessive proclination of the mandibular
incisors; and functional deficits such as difficulty in drooling,
swallowing, obstruction of the upper airway, and alterations in
speech.15
In the present case report in addition to the dentoskeletal and
functional problems, the patient presented poor oral hygiene associated
with extensive carious lesions.
The clinical appearance of lymphangioma depends essentially on the
lesion extension. The superficial lesion presents as papillary lesions
with pebbly surface due to the occurrence of several translucent
vesicles with the same color as that of adjacent mucosa or occasionally
with a mild reddish hue. These lesions can sometimes present a
particular aspect such as a tapioca pudding or frog eggs like
appearance. While the deeper lesions manifest as diffuse nodules which
are soft in consistency and with negligible alterations in color or
texture.16
Due to infection or hemorrhage, lymphangiomas in a child can grow
suddenly but they can also shrink spontaneously.4
These lesions can be described as localized or diffuse
growth.12
Treatment’s objectives of tongue lymphangioma are essentially the
restoration of tongue size, the preservation of taste, correction of
mandibular and dental deformities.4
These congenital malformations can be treated with radiofrequency
ablation, surgery, sclerotherapy, interferon, and
corticosteroids.17
Conserved approaches like surgical excision with low relapse rates,
cryotherapy, and laser can be considered for the treatment of
superficial and/ or localized lymphangiomas.13,17
While the management of diffuse lymphangioma can be more complicated
because the lesion can involve a more extensive area which was the case
in the present report.12
Microcystic lesions do not respect tissue planes, are diffuse and
generally difficult to eradicate.16
In the present report, due to the diffuse nature of the lesion, the
patient was treated with Sirolimus; 1.5 mg/kg/day administered in two
divided daily doses.
The Sirolimus is an immunosuppressive and antitumor agent that belong to
the Mammalian target of rapamycin (mTOR) inhibitors group which plays an
important role in cellular anabolism, catabolism, cell growth, and
angiogenesis.17,18
This molecule has been used in patients with tuberosclerosis to decrease
the size of the lesions of angiomyolipoma and lymphangioleiomyomatosis
(LAM).17,19
Most recently, oral sirolimus, which can inhibit lymphatic vessel
regeneration, invasion, LAM growth, and vascular endothelial growth
factor VEGF secretion, has been reported to be efficacious for patients
with lymphangioma of the tongue.17,20
As an immunosuppressant, sirolimus can cause neutropenia and may
predispose the patient to an increased risk of
infection.21
Ideally, all dental procedures should be completed before
immunosuppressive therapy is initiated but when the dental needs cannot
be treated before therapy, priorities should be infections, extractions
and periodontal care.22
In the present case report, because the patient was undergoing treatment
with Sirolimus, the dental treatment was carried out under antibiotic
prophylaxis.
Clamoxyl ® with a dose of 50mg/kg orally as a single dose one hour
before to the procedure was prescribed for the patient.
For dental procedures, the decision regarding the need for antibiotic
prophylaxis should be made in consultation with the patient’s physician.
Immunosuppressive therapy may cause many acute and long-term side
effects especially in the oral cavity, and any existing or potential
sources of dental infections can compromise the medical treatment.22
In this report, giving the general health status of the patient, to be
able to perform a removable partial denture and to correct the anterior
open-bite, check-ups every three months have been scheduled to control
the reduction of the size of the tongue.
Conclusion
Young patients with lymphangioma of the tongue, usually presenting with
macroglossia, may not only have a significant chewing and breathing
problems but also malocclusions and speech disorders, and dental
problems. The enlarged tongue with partial or total immobility
compromises the mechanical removal of the dental plaque which can lead
to an increase of the caries risk in these children. For this reason,
dental intervention must be done promptly and efficiently, with
attention to the patient’s medical history, treatment protocol, and
health status.