Amyloidosis is characterized by extracellular deposition of amyloid material in various tissues and organs. Deposition of amyloid in the tongue is rare but often occurs in multiple myeloma. Here we present the case of a 44-year-old woman under treatment for multiple myeloma who complained of lateral tongue pain. Intraoral examination revealed an ill-defined, firm, whitish swelling with an ulcerated surface on the bilateral borders of the tongue. Histopathological analysis revealed a lympho-mononuclear infiltration in the lamina propria with the presence of acellular and amorphous material positive for Congo red staining. Further examination revealed amyloidosis in a cervical mass associated with neural compression. Thorough examination of the oral cavity in patients with multiple myeloma is critical for early detection of amyloidosis. A biopsy is mandatory to confirm the diagnosis, thereby facilitating intervention and management if necessary.

Sturge-Weber syndrome (SWS) is a rare congenital neurocutaneous disorder presenting unique anesthetic challenges due to extensive vascular malformations. Key considerations include difficult airway management due to extensive facial and oral angiomas, seizure control, and maintaining stable intracranial and intraocular pressures. This case report discusses the perioperative management of a 57-year-old female with SWS undergoing dental surgery under general anesthesia and synthesizes the current literature on anesthetic considerations and management. Preoperative examination revealed a facial port-wine stain covering more than 75% of the face, extensive oral angiomas, macroglossia, and severe lip hypertrophy, raising concerns for difficult airway management and risk of hemorrhage. A rapid sequence induction was performed following upright preoxygenation, and video laryngoscopy provided a grade I view of the glottis. Endotracheal intubation was accomplished atraumatically, and the case proceeded uneventfully. The patient was extubated but experienced mild desaturation in the post-anesthesia care unit, prompting overnight observation before discharge at baseline oxygenation. This case highlights the importance of anticipating and planning for difficult airway management, maintaining vigilance for perioperative seizure control, and implementing strategies to minimize elevations in intracranial and intraocular pressures.

Background: Beckwith-Wiedemann syndrome (BWS) is a rare congenital overgrowth disorder caused by genetic and epigenetic alterations on chromosome 11p15.5. While macroglossia, abdominal wall defects, and tumor predisposition are well recognized, hearing impairment has been sporadically reported. Objectives: The aim of this study is to review audiological features, surgical management, and rehabilitation in BWS, and we additionally present three cases with comprehensive longitudinal audiological follow-up. Methods: A systematic review of PubMed and Scopus was conducted according to PRISMA guidelines, including studies reporting audiological findings in patients with confirmed BWS. Studies without audiological data or reporting only normal-hearing patients were excluded. Data on hearing loss type, severity, genetics, clinical features, imaging, surgical interventions, and outcomes were extracted. A narrative synthesis was conducted; no meta-analysis was performed due to the heterogeneity and limited number of available studies. Data extraction was performed independently by two reviewers who independently screened titles, abstracts, and full texts, with disagreements resolved by discussion. In addition, three original case reports from our institution were included to further illustrate the clinical and rehabilitative variability of hearing impairment in BWS. Results: We identified 40 patients from the review, but only 12 of them reported audiological data (e.g., hearing thresholds, type of hearing loss, or diagnostic tests). Ossicular chain anomalies, particularly stapes fixation, were frequently observed. Surgical management improved hearing in selected cases, while bone conduction devices (BCD) or conventional amplification were effective alternatives when surgery was contraindicated. Genetic analyses revealed CDKN1C mutations or imprinting defects in nine patients. Conclusions: Hearing impairment in BWS is clinically relevant and often conductive, likely related to middle-ear anomalies. Early, multidisciplinary audiological evaluation-including imaging when indicated-and individualized rehabilitation can optimize auditory and communicative outcomes. The evidence is limited by the small number of studies and heterogeneous reporting of audiological outcomes.

Mucopolysaccharidosis type II (MPS II; also known as Hunter syndrome) is an X-linked multisystem disorder characterized by glycosaminoglycan (GAG) accumulation. The vast majority of affected individuals are male; on rare occasion heterozygous females manifest findings. Age of onset, disease severity, and rate of progression vary significantly among affected males. In those with early progressive disease, CNS involvement (manifest primarily by progressive cognitive deterioration), progressive airway disease, and cardiac disease usually result in death in the first or second decade of life. In those with slowly progressive disease, the CNS is not (or is minimally) affected, although the effect of GAG accumulation on other organ systems may be early progressive to the same degree as in those who have progressive cognitive decline. Survival into the early adult years with normal intelligence is common in the slowly progressing form of the disease. Additional findings in both forms of MPS II include: short stature; macrocephaly with or without communicating hydrocephalus; macroglossia; hoarse voice; conductive and sensorineural hearing loss; hepatosplenomegaly; dysostosis multiplex; spinal stenosis; and carpal tunnel syndrome.

Lymphangioma is a congenital malformation of the lymphatic system, commonly affecting the head and neck region. Macroglossia due to lymphangioma in adolescence is uncommon, making this case noteworthy. This condition can affect oral function, speech, and quality of life, making early diagnosis and intervention essential. This report highlights a 14-year-old Balouch female presenting with progressive tongue enlargement beginning at age five. Intraoral examination revealed an enlarged tongue with a pebbly surface, particularly involving the ventral side of the tongue, without osseous involvement on radiographs. Differential diagnoses included hemangioma and lymphangiomegaly; however, histopathological analysis confirmed lymphangioma. The patient underwent sclerotherapy, resulting in a significant reduction in tongue size, improved oral function, and resolution of related symptoms. Follow-up revealed no recurrence or major complications. Early recognition and appropriate management of lymphangioma-induced macroglossia are essential to prevent functional limitations and improve quality of life. While surgical excision remains the conventional treatment, sclerotherapy offers a less invasive, effective alternative with lower morbidity. This case contributes to the limited literature on adolescent-onset oral lymphangioma and underscores the value of a multidisciplinary approach in ensuring optimal outcomes.

Fissured tongue is a benign oral condition characterized by grooves or fissures on the dorsal surface of the tongue and is often considered idiopathic. However, it has occasionally been reported in association with systemic disorders, including endocrine abnormalities. We report the case of an 18-year-old male who presented with fatigue, cold intolerance, weight gain, and progressive fissuring of the tongue over several years. Intraoral examination revealed multiple prominent fissures on the dorsal surface of the tongue with mild macroglossia. Laboratory investigations demonstrated elevated thyroid-stimulating hormone levels with borderline low free thyroxine and mildly raised antithyroid peroxidase antibodies, findings suggestive of hypothyroidism possibly related to autoimmune thyroid disease. The patient was started on levothyroxine therapy, resulting in improvement of systemic symptoms and normalization of thyroid function tests within 6 months. However, the fissured appearance of the tongue persisted despite biochemical euthyroidism. This case is noteworthy because the patient was a young adult with progressive tongue fissuring preceding diagnosis, a positive family history of thyroid disease, and persistence of fissures despite appropriate thyroid hormone replacement therapy. The report highlights the potential association between oral findings and systemic endocrine disorders and emphasizes the importance of careful oral examination in patients presenting with suggestive systemic symptoms.