Lymphangiomas are uncommon congenital hamartomas of the lymphatic system with a marked predilection for the head-and-neck region accounting for 75% of all cases. Lymphangiomas rarely affect the oral cavity. Affected sites in the oral cavity may include the tongue, palate, gingiva, lips, and alveolar ridge of the mandible. The anterior two-thirds on the dorsal surface of the tongue is the most common site for intraoral lymphangiomas leading to macroglossia, similar to the presentation in our case. Congenital macroglossia is a rare clinical condition characterized by an enlarged tongue. This enlargement can lead to a variety of oral and facial problems, with structural deformities such as diastema, disproportionate mandibular growth, and functional disorders such as difficulty in eating and swallowing, speech impairment, or even difficulty in breathing if it is severe. Macroglossia may be attributed to multiple underlying causes. These include conditions such as idiopathic muscular hypertrophy (notably in Beckwith-Wiedemann syndrome), vascular malformations (such as angiomas and lymphangiomas), tumors, and endocrine disorders. Among these causes, lymphangiomatous macroglossia is particularly rare and is characterized by diffuse involvement. This type of macroglossia typically progresses chronically, reaching a developmental plateau at puberty without any spontaneous regression. We report a case of congenital macroglossia in an 8-year-old male due to lymphatic malformation and managed successfully.

Macroglossia, an uncommon anatomical anomaly, can manifest as either congenital or acquired. The size of the tongue undergoes variations with age, peaking at 8 years and reaching full maturity at 18 years. Congenital macroglossia stems from diverse conditions, such as muscular hypertrophy, hemangioma, lymphangioma, Down syndrome, and others. Acquired macroglossia can result from malignancies, endocrine and metabolic disorders, chronic infectious diseases, and head and neck infections, among other factors. Additionally, extended-prone surgery can lead to its development. The incidence of macroglossia is likely underreported. This presentation is rare with only six reported cases in the literature.

Sturge- Weber syndrome (SWS), is a rare neuro-cutaneous angiomatosis which affects male and females alike. The clinical manifestations include angiomas, haemangiomas of the lips, tongue and palatine region. The oral manifestations are usually unilateral and are susceptible to bleed. Patients can also present with macroglossia and maxillary bone hypertrophy which can lead to malocclusion of the oral cavity. Food accumulation due to occlusion can cause growth of bacteria which can intensify infections and can cause gingival hyperplasia. A case of a middle-aged 39 year old female was reported in the Ziauddin Hospital, Karachi on 2nd of February,2022 with the presenting complaints of intermittent fever and drowsiness for 10 days. On examination she had massive tongue enlargement, drooling, malocclusion, difficulty in eating and breathing. She was a known case of Sturgeweber syndrome. Based on the clinical and radiological findings, she was managed along the lines of prelaryngeal soft tissue and submandibular infection.

Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder that causes developmental defects as well as an elevated risk of malignancies. Macroglossia, or an enlarged tongue, is a common symptom of BWS that may have a negative influence on a person's quality of life. The aim of this systematic review is to look at the present state of knowledge about the repercussions of macroglossia, as well as the influence of the timing of surgical resection, or glossectomy, in the treatment of severe cases of macroglossia (Ref. 35). Keywords: macroglossia, Beckwith-Wiedemann syndrome, glossectomy.

Objective: This review was conducted to define the natural history of unoperated Beckwith-Wiedemann syndrome (BWS) macroglossia and the effect of tongue reduction surgery upon breathing, eating, speaking and dentoskeletal development in individuals having BWS. Design: This is a retrospective study of medical records. All patients were evaluated and treated in one of two Children's Hospitals by an ACPA approved Craniofacial Team. Medical records were reviewed of 526 individuals having a diagnosis of BWS and evaluated in-person by a single craniofacial surgeon between 1986 and 2014 in conjunction with a series of multi-disciplinary craniofacial team colleagues. 28 individuals were excluded having had multiple tongue reductions elsewhere. 498 individuals comprise the "pre tongue-reduction group". The "post tongue-reduction group" consists of 391 individuals who underwent surgical tongue reduction by one surgeon using one technique between 1986 and 2014. The primary outcome measure was change in anterior dental occlusion following tongue reduction surgery. Tongue reduction surgery was performed on the assumption that it would improve dentoskeletal relationships. Secondary outcome measures were: breathing, feeding/swallowing, and speech. Results: A significant difference (p<0.001) over time between the two groups was found with less anterior occlusal abnormality in the tongue reduction group. Tongue reduction surgery had no mortality and minimal morbidity for breathing, feeding/swallowing, and speech and can ameliorate obstructive sleep apnea. Conclusions: Surgical tongue reduction for BWS macroglossia is recommended for the infant or child in primary dentition with a grossly abnormal anterior tooth/jaw relationship and/or obstructive sleep apnea.