Macroglossia associated with brainstem injury.

BACKGROUND: Macroglossia has been reported in patients undergoing posterior fossa neurosurgical procedures and is thought to be as a result of venous engorgement from intubation or mechanical positioning during these prolonged procedures. METHODS: We report three patients who developed macroglossia and dysautonomia of central neurogenic origin following brainstem injury. RESULTS: The three patients developed macroglossia and dysautonomia with wide hemodynamic fluctuations in the setting of posterior fossa injury of the lower brainstem structures, necessitating tracheostomy placement. Macroglossia was managed with dexamethasone and there was complete resolution of dysautonomia while treated with beta-blockers and gabapentin. CONCLUSIONS: Neurointensivists should be aware of macroglossia with dysautonomia complicating brainstem injury, which may have perilous consequences in the setting of cerebral edema or intracranial hypertension.

Unilateral macroglossia as sole presenting manifestation of internal carotid artery dissection.

A patient presented with acute-onset, painless, unilateral enlargement of the tongue. Steroid treatment for angioedema was ineffective, and a biopsy of an apparent mass of the tongue base showed normal tissue. Subsequent magnetic resonance imaging showed enlargement, enhancement, asymmetric T2 hyperintensity of the left half of the tongue, and dissection of the left cervical internal carotid artery (ICA) at the skull base. Unilateral enlargement of the tongue due to acute neurogenic denervation may be the sole clinical presentation of a spontaneous arterial dissection at the skull base. The hypoglossal neuropathy resulted from compression by the pseudoaneurysmal ICA dilatation or ischemia from interruption of the vasa nervorum.

Focal myositis of the tongue presenting as macroglossia.

Focal myositis (FM) is a benign localized inflammatory process of unknown aetiology, which presents as a rapidly enlarging soft-tissue mass that evolves into a localized lesion. It is most often mistaken for a neoplasm. Histologically, it is characterized by marked myopathic changes of the muscle fibres, lymphocytic infiltration and interstitial fibrosis. We describe a case of focal myositis of the tongue and review this rare condition.

Dental care management of a young patient with extensive lymphangioma of the tongue: a case report.

Lymphangiomas are benign tumors resulting from a congenital malformation of the lymphatic system. Relatively uncommon, lymphangiomas are usually diagnosed at birth and develop within the first years of life. When these tumors occur in the oral cavity, the tongue is the most frequently affected site. Lymphangioma of the tongue is a common cause of macroglossia in children, which may lead to a dry/cracked tongue with ulcerating secondary infections, difficulty in swallowing and mastication, speech disturbances, exclusive nasal breathing, airway obstruction, mandibular prognathism and other possible deformities of maxillofacial structures. This paper discusses the most relevant features, clinical manifestations, disease-related impairments and treatment options for lymphangioma of the tongue. It presents the case report of a five-year-old child diagnosed with this lesion, including a description of the patient's dental care management.

Angioedema of the tongue due to acquired C1 esterase inhibitor deficiency.

We describe the management of an 83-year-old woman who presented with upper airway obstruction due to angioedema of the tongue. Following definitive airway management, investigation showed a diagnosis of acquired C1 esterase inhibitor deficiency (acquired angioedema) that was considered to be subsequent to haematological malignancy. Resolution of the macroglossia followed treatment with C1 esterase inhibitor concentrate, but the patient failed to wean from ventilatory support and died in the Intensive Care Unit. This case report highlights the potential for acquired angioedema to cause upper airway obstruction. The various treatment modalities for acquired C1 esterase inhibitor deficiency are summarized.

The clinical features of immunoglobulin light-chain (AL) amyloidosis with heart involvement.

We reviewed clinical presentation, investigations, therapy, prognosis and outcome of 232 patients with primary (AL) cardiac amyloidosis. There were 142 men and 90 women. Median age at presentation was 59 years (range 29-85). AL heart disease was unusual both in patients under the age of 40 (3.0%) and in non-Caucasians (6.5%). Fatigue and weakness were the commonest presenting symptoms. Hallmark features of periorbital ecchymoses and macroglossia were present in 12.5% and 27.2%, respectively. AL cardiac amyloidosis was unusual in isolation (3.9%), and most frequently patients had features of multiorgan dysfunction; heavy proteinuria and features of malabsorption predominating in this respect. Heart involvement represents the worst prognostic indicator, with a median survival from diagnosis of 1.08 years, falling to 0.75 years with the onset of heart failure. Current therapeutic procedures appear to prolong survival, with left ventricular wall thickness, mass and ejection fraction on echocardiography and late potentials on signal averaged electrocardiography of use in prognostic stratification. Cardiac involvement from AL amyloidosis is rapidly fatal. It should be suspected in all patients with heart failure who have wall thickening on echo, normal chamber sizes, low EKG voltages and evidence suggesting a multisystem disease.

[Macroglossia in apparently primary amyloidosis]. / Macroglossie au cours d'une amylose apparemment primitive.

The authors report a case of apparently primary amyloidosis in which macroglossia was the clinical presentation, confirmed by lingual biopsy. They take advantage of this case to review the therapeutic possibilities, though stating that from a stomatological standpoint there is virtually nothing available.