Surgical management of macroglossia due to primary amyloidosis

A case of macroglossia due to primary amyloidosis is described, followed by a discussion of the various aspects of surgical intervention based on a literature review.     

Surgical treatment of macroglossia caused by systemic primary amyloidosis

Amyloidosis is a heterogeneous group of disorders caused by the extracellular deposition of a fibrillar protein called amyloid. Amyloid involvement of the tongue is almost universally secondary to systemic disease. The clinical manifestations result from the progressive extracellular deposition of amyloid within the suprahyoid muscles. In the late stages, the progressive enlargement of the tongue causes hypo- and oropharyngeal blockage, with obstruction of the upper airways. Conservative excision is a satisfactory treatment for local amyloid masses; the role of surgery in systemic forms is controversial. The authors present a case of systemic primary amyloidosis of the tongue treated surgically by a partial glossectomy via a pull-through approach and discuss the indications and rationale for surgical intervention.     

A tongue resection in macroglossia due to primary amyloidosis

A lady of 62 years with longstanding macroglossia from primary amyloidosis had the anterior two-thirds of her tongue successfully resected. She had Factor X deficiency. The surgical, anaesthetic and haematological problems encountered are described.     

The surgical management of severe macroglossia in systemic AL amyloidosis

Amyloidosis is a disease characterised by the deposition in body tissues of amyloid: abnormal protein in a beta pleated sheet formation. It is a systemic disorder and macroglossia may be seen in all forms. Changes to the normal architecture of the tissues and systemic features of the disease and its underlying cause can complicate the surgical management of the enlarged tongue.     

Dysphagia due to macroglossia in a patient with amyloidosis associated with multiple myeloma: A case report

Amyloidosis is a group of heterogeneous conditions characterized by the deposition of extracellular proteinaceous substances called amyloids. The diagnosis is usually confirmed after a particular body organ has been affected. However, oral manifestations have been identified in 39% of affected patients. Systemic amyloidosis may be primary or in association with multiple myeloma and typically affecting elder adults. The diagnosis of amyloidosis is usually associated with clinical features with histopathological findings, which reveal the presence of acellular eosinophilic deposits. The aim of this report is to describe a clinical case of an elderly patient with dysphagia related to macroglossia and enlargement caused by primary amyloidosis associated with multiple myeloma.     

Primary oral and perioral amyloidosis associated with multiple myeloma

Three years after being diagnosed with multiple myeloma, a patient sought treatment for swelling on the floor of the mouth, associated with hardening of the soft tissues on the right perioral region, loss of facial expression, and difficulty opening his mouth. The patient reported improvement following an incisional biopsy for microscopic diagnosis. Eighteen months later, the patient showed no clinical alterations.     

Congenital macroglossia: surgical and orthodontic management

ObjectivesA case of congenital macroglossia is reported. The most important sign of macroglossia is tongue protrusion through the lips. Tongue protrusion might influence skeletal growth and can cause anterior open bite, proclination of upper and lower incisors and development of diastemas.Materials and methodsA 4 year-old female patient was diagnosed with congenital macroglossia. Parents referred an abnormal tongue dimension since birth and the development of a progressive anterior open bite. The treatment of macroglossia included tongue reduction by partial glossectomy. She was seen regularly and at the age of eight years old a lingual frenectomy was performed and an orthodontic treatment was planned.ResultsAt the end of the orthodontic treatment a Class I occlusion was obtained with correct overbite and overjet values.ConclusionsEarly interception of macroglossia and surgical reduction in combination with orthodontic treatment can be seen as preventive measures to avoid the tongue influence on the development of malocclusions.     

Severe macroglossia after posterior fossa and craniofacial surgery in children

Massive swelling of the tongue can occur after posterior fossa and craniofacial surgery. Several hypotheses have been proposed to explain the occurrence of such severe postoperative macroglossia, but this phenomenon is still poorly understood. Severe postoperative macroglossia can be a life-threatening condition due to upper airway obstruction. Three cases of severe postoperative macroglossia that occurred after cervical spine, craniofacial, and posterior fossa surgical procedures are reported here. These cases required specialized maxillofacial management and a prolonged stay in the intensive care unit. Causal factors involved in this condition are reported, in order to highlight appropriate prevention and treatment options adapted to the management of paediatric patients. An overview of the current literature on severe postoperative macroglossia in paediatric populations is also provided.     

Comprehensive treatment of massive macroglossia due to venous and lymphatic malformations

This study evaluated the outcomes of comprehensive treatment for massive macroglossia. Eleven patients with massive macroglossia due to venous malformations (VMs) and lymphatic malformations (LMs) underwent incision excision of two-thirds of the central wedge of the tongue; two of these patients underwent resection of lesions in the lower lip or floor of the mouth during initial treatment. In subsequent treatment, three patients underwent orthognathic surgery or resection of lesions in the lower lip and submental region, and four patients underwent one or two sclerotherapies. No patient had complications resulting from anaesthesia or surgery. Patients were followed up at 6 months postoperative, and all signs associated with the lesions had disappeared. The mean follow-up duration was 30.8 months. The aesthetic outcome was excellent for nine patients and satisfactory for two. Nine patients could take a soft diet and two a solid diet. Eight patients presented normal speech and three presented intelligible speech. Comprehensive treatment including incision excision of two-thirds of the central wedge of the tongue, as well as orthognathic surgery, the resection of lesions in the oral and maxillofacial region, and sclerotherapy, is effective for patients with massive macroglossia due to VMs and LMs.     

Relationship between dental erosion and respiratory symptoms in patients with gastro-oesophageal reflux disease

Objectives

Both dental erosion and respiratory symptoms are extra-oesophageal manifestations of gastro-oesophageal reflux disease (GERD). The aim of this study was to determine whether dental erosion was correlated with respiratory symptoms in GERD patients.

Methods

88 GERD patients were recruited and assigned to three groups mainly according to the frequency of respiratory symptoms: Group I: never; Group II: occasional (1–2 days a week or less); Group III: frequent (3–5 days a week or more). All patients underwent medical evaluations, including medical history, questionnaire answering and alimentary tract examinations. Dental examinations were carried out on these patients and 36 healthy controls. Dental erosions were measured by modified method of Smith and Knight Tooth Wear Index (TWI). Location and severity of dental erosion were recorded.

Results

The prevalence of dental erosion in Group III (64.52%) was higher (p < 0.05) than that in Groups I (36.67%) and II (44.44%). GERD patients were presented with dental erosion with TWI scores ranging from 1 to 4. Though proportion of dental erosion with Score 2 (7/20) in Group III was higher than that in Group I (2/11) and Group II (3/12), there was no statistical significance in the proportions of erosion scores among three patient groups. Correlation coefficient between airway symptoms and scores of dental erosion was 0.231 (p < 0.05). Palatal erosion of upper incisor was seen in 8 persons (72.7%) in Group I, 9 persons (75%) in Group II and 16 persons (80%) in Group III (p > 0.05). Labial erosion of upper incisors was found in 1 person in Groups I and II respectively and 4 persons in Group III. All patients with labial erosion on upper incisors had palatal erosion, except 1 patient in Group III.

Conclusions

In GERD patients, dental erosions are more prevalent in patients with frequent respiratory symptoms than those in patients with occasional and without respiratory symptoms. Palatal erosion of upper incisor is the main manifestation in patients. Acid reflux is the main causative factor of dental erosion in GERD patients with airway symptoms.     

Oral amyloidosis: A strategy to differentiate systemic amyloidosis involving the oral cavity and localized amyloidosis

Amyloidosis is a rare metabolic disorder caused by the extracellular deposition of fibrillar proteins in important organs. The prognosis of oral amyloidosis associated with systemic involvement is generally poor, but is much more favourable in localized cases. Limited information is available regarding the differential diagnosis between localized and systemic amyloidosis involving the oral mucosa, and also the prognosis. In this study, we summarize the strategy for correctly discriminating between localized and systemic oral amyloidosis, to give the right advice during the diagnostic workup.     

Cyst of the floor of the mouth causing respiratory obstruction. Report of one case

Three major groups of lesions can cause swelling in the floor of the mouth: infectious, neoplastic, and cystic. We present the case of a patient with inflammatory cyst in the floor of the mouth that reached, in a little while, huge dimensions. Needle aspiration of the lesion caused an infectious process that enlarged the cyst and led to progressive difficulties in breathing. Treatment was by emergency tracheostomy and enucleation of the lesion using an intraoral approach. The enucleation was performed through a mucosal incision along the midline of the floor of the mouth, starting just behind the lingual fold, and extending along the ventral surface of the tongue.