Introduction 

What is macroglossia? 

Macroglossia is a rare condition, occurring mostly in children rather than adults. It is characterised by tongue enlargement due to muscular hypertrophy, tumour or other endocrine disorder. In which the tongue in its normal resting position is protruding beyond the dental arch (the area of bone and gum that houses the teeth) or, in more severe cases, it lies outside of the patient’s mouth.¹

Mostly, it's not a life-threatening condition, but it may result in complications like airway obstruction and difficulty breathing. It is clinically diagnosed (by visual inspection). Treatment is either conservative or surgical, according to the case. 

What are the causes of macroglossia? 

Macroglossia is a multifactorial condition in which multiple factors may play a role. According to the aetiology of the development, macroglossia is divided into three main types: true macroglossia, relative macroglossia (Pseudo Macroglossia) and functional macroglossia.² 

True macroglossia 

In true macroglossia, there is an underlying disease or condition that results in an obvious enlargement of the tongue​. 

True Macroglossia is either:

• Congenital Macroglossia
• Acquired Macroglossia

Congenital macroglossia might be caused by due to several conditions and syndromes, such as: 

• Lymphangioma 
• Hemangioma 
• Down syndrome 
• Idiopathic muscular hypertrophy
• Adenoid hyperplasia and other conditions

Acquired macroglossia, including but not limited to, caused by: 

• Metabolic disorders: Diabetes Mellitus (DM), Acromegaly, Hypothyroidism
• Inflammatory Conditions: Glossitis, Tuberculosis, Pneumonia, infections of the head and neck
• Radiation 
• Truma 
• Lymphangioma 
• Venal congestion²

Relative macroglossia 

Also called pseudo macroglossia or false macroglossia. It’s unlike true macroglossia as it’s difficult to be recognised because the tongue has no actual increase in size but appears to be larger as compared to other adjacent oral structures, or is slightly larger than normal, and it has an unclear diagnosis. 

Relative macroglossia, as well, is either congenital or acquired.¹  

Functional macroglossia 

There is a lack of adaptation of the tongue to the oral cavity after performing an oral cavity reduction surgery.

Diagnosis of macroglossia 

There are several clinical and cephalometric features and signs that are used to identify if macroglossia is truly present or not. These signs aren’t always necessary to occur together for every individual​. 

Signs and symptoms include:

• Enlarged, protruded, broad and flat tongue 
• Glossitis 
• Diastema 
• Development of a space between the front or back upper and lower teeth (Anterior and posterior open bite) 
• Outside tipping of the teeth 
• Difficulties in eating, swallowing, feeding and talking 
• Increased width of the upper and lower jaws 

Biopsy is a must for diagnostic investigation to exclude the possibilities of tongue tumours and cancers. 

What is lymphangioma? 

It is a congenital abnormality that is characterised by excessive growth of lymphatic tissues during the prenatal or postpartum period of child development. It is a benign (non-cancerous) condition. 

There is 1 in every 4000 births affected by lymphangioma, most of the patients are diagnosed before the age of two years, but there is no difference according to race or gender.³ It most commonly occurs in the head and neck region (about 75%) with a predilection in the tongue, followed by lips, buccal mucosa, palate and the floor of the mouth.⁴ 

It's usually a slow and steadily growing condition, but due to trauma, infection or hormonal changes, it may grow rapidly, ending in life-threatening conditions that require urgent intervention. 

Causes of lymphangioma 

Scientists do not fully understand the cause of the development of lymphangioma, but they believe that it may be due to abnormalities in cell division during the prenatal period that result in the formation of a lymphatic system that doesn’t function effectively. 

Additionally, it is found to be associated with syndromes in which there is an increase in the chromosome count (Down syndrome) or a decrease (Noonan syndrome).

Clinical manifestations of lymphangioma 

Clinically, lymphangioma has a varying manifestation, ranging from a superficial, localised small swelling to a large lymphatic infiltration that results in elephantiasis in the most severe cases.³  Signs and symptoms of lymphangioma depend on the affected part of the body; for example, lymphangioma of the eye and orbit leads to vision problems and lack of eye muscle movement and ptosis​​. Generally, the patient will experience pain and discomfort with a chance of complications developing, like infections. 

Types of lymphangioma 

Based on the size of the tumour, lymphangioma is categorised into two main groups​: 

1. Microcystic lymphangioma:
   • Less than 2 cm in size 
   • Have poor and ill-defined borders 
   • Grow into tiny bristles 
   • Also known as lymphangioma circumscriptum and capillary lymphangiomas 
2. Macrocystic lymphangioma
   • More than 2 cm in size
   • Have a well-demarcated border
   • According to its depth, it ranges from bluish to reddish colours
   • Have a spongy texture
   • Also known as Cavernous lymphangioma and Cystic hygroma

Diagnosis of lymphangioma 

Some cases of lymphangiomas can be detected through an ultrasound before birth, or they may be detected immediately after birth. In this case, further investigations and imaging, like an MRI and a CT scan, are required. 

In other cases, lymphangioma isn't discovered until the age of 2 years, as it grows and becomes noticeable. 

Macroglossia and lymphangioma 

Lymphangioma of the tongue appears as a defined mass of pebbly, vesicle-like nodules or as a macroglossia that is commonly present from birth, after the eruption of the dentition or even after puberty.⁴  

The most affected area of the tongue is the anterior two-thirds dorsal area. 

Most lymphangiomas are superficial with a yellowish translucent shape that resembles a frog's egg.  

Diagnosis of tongue lymphangioma 

Lymphangioma of the tongue is diagnosed clinically (by identifying the signs and symptoms) or histologically (by observing the biopsy under a microscope)⁴ 

Signs and symptoms of tongue lymphangioma:

• Difficulties in speech  
• Feeding and eating problems  
• Burning sensation in the lesion  
• Bleeding due to minor trauma of the lesion during normal function, like eating  
• In severe cases, airway obstruction and breathing difficulties may occur 

Histological features 

• Proliferation of multiple lymphatic channels of different sizes that determine the size of the clinical lesion  
• Sometimes, red blood cells are also seen in the biopsy, which indicates the presence of trauma and bleeding 

Management of tongue lymphangioma 

There are multiple treatment options for managing lymphangioma; the choice between them depends on the size, location and extent of the lesion.⁵

Treatment options are either:

1. Medical treatment
2. Surgical treatment
3. Others

Medical treatment

By using oral medical drugs it has been found to be effective in treating lymphangioma​.

Surgical treatment

The first choice of treatment for lymphangioma is the surgical excision of the swelling, but if the lesion is invasive and close to an important structure, this will restrict the complete removal of the swelling.⁴ 

Surgical excision is associated with multiple complications, like:  

• high recurrence rate (high chances that the lesion will recur) 
• bleeding 
• Infection 
• Facial injury 
• Deformities like scar formation that are left after the procedure, and compromise the aesthetics 

Other 

• Sclerotherapy: It’s one of the most common and preferred ways of treating lymphangioma by injecting the lesion with a sclerosing agent that controls swelling and pain. It’s more effective in treating macrocystis type⁵ 
• Radiofrequency Ablation: depends on destroying the tissues by low temperature (40° - 70°) with the minimum trauma to adjacent tissue

It’s effective mostly in treating microcystic types.

• Propranolol: an alternative treatment for children, as it was found to be effective in congenital cases of lymphangioma and resulted in significant improvement of the lesion when the child is still in the uterus by administering it to the mother

There are many treatment options, like lasers, which are also used, and combinations of them may also be applied to reach a satisfactory result. 

If the lesion is asymptomatic and doesn’t affect function, treatment is not needed.⁴ 

Summary

Macroglossia is a rare condition in which the tongue is enlarged; it mostly affects children. Multiple causes are associated with macroglossia, such as tumours, endocrine disorders and muscular hypertrophy. Macroglossia is diagnosed clinically and treated either conservatively or surgically. 

Lymphangioma of the tongue appears as macroglossia, in which the lymphatic tissues of the tongue undergo overgrowth. Generally, lymphangioma is rare and affects 1 in 4000 births, (75%) of it is found in the head and neck area, with a predilection. The treatment of lymphangioma is either medical by using drugs or removing the swelling by surgical excision (recurrence, bleeding, infection, and scars are complications of surgery), or sclerotherapy, radiofrequency ablation, and propranolol are other methods of treatment. 

Asymptomatic cases may not require treatment.