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A case of severe macroglossia, following intraoral surgery, causing respiratory distress is described. Because conventional therapy appeared inadequate, leeches were used and proved an efficient method of reducing this life-threatening swelling of the tongue.
AL amyloidosis was diagnosed in a 56-year-old woman with spontaneous purpura, macroglossia and hepatomegaly, a serum IgGk monoclonal gammopathy and a 25% plasma cell bone marrow infiltration. She was started on high-dose treatment consisting of four monthly cycles of VID chemotherapy, then underwent a stem cell collection after priming with cyclophosphamide + G-CSF. Myeloablative therapy was with melphalan and busulfan. Hematologic recovery was fast and uncomplicated. At follow-up 22 months from ASCT, the patient shows a complete remission of the clonal plasma cell disorder, normalization of liver size and alkaline phosphatase level and a significant improvement in the signs of vascular and soft tissue amyloid infiltration.
Congenital macroglossia is a relatively uncommon condition characterised by enlargement of the tongue disproportionate to the rest of the dentoalveolar structures. It may cause significant symptoms in children. It is important to achieve uniform global reduction of the enlarged tongue for functional as well as aesthetic reasons. It is also important to preserve the mobility and sensation of the tongue. We describe a technique which meets these criteria.
In a sample survey study (questionnaires filled out by parents), all 40 patients with Beckwith-Wiedemann Syndrome (BWS) presented with macroglossia. Articulation problems were reported in 29 of them, which persisted in some following corrective tongue surgery. The impression emerges that the value of surgical treatment needs prospective assessment, that the parents are insufficiently informed, and that the adequacy of direct articulation therapy in BWS needs to be evaluated.
Neonatal diabetes, which can be transient or permanent, is defined as hyperglycemia that presents within the first month of life and requires insulin therapy. Transient neonatal diabetes mellitus has been associated with abnormalities of the paternally inherited copy of chromosome 6, including duplications of a portion of the long arm of chromosome 6 and uniparental disomy, implicating overexpression of an imprinted gene in this disorder. To date, all patients with transient neonatal diabetes mellitus and uniparental disomy have had complete paternal isodisomy. We describe a patient with neonatal diabetes, macroglossia, and craniofacial abnormalities, with partial paternal uniparental disomy of chromosome 6 involving the distal portion of 6q, from 6q24-qter. This observation demonstrates that mitotic recombination of chromosome 6 can also give rise to uniparental disomy and neonatal diabetes, a situation similar to that observed in Beckwith-Wiedemann syndrome, another imprinted disorder. This finding has clinical implications, since somatic mosaicism for uniparental disomy of chromosome 6 should also be considered in patients with transient neonatal diabetes mellitus.
Dentists are aware of the extraordinary influence an active thrusting tongue can have on dental development. In the presence of the profound muscle hypertrophy characteristic of macroglossia in the Beckwith-Wiedemann syndrome, a compelling need exists for surgical reduction of the tongue as early in life as possible. This report describes our clinical experience with 3 children, 1 referred late and the other 2 treated within the first year of life.
Computed tomography of the tongue base and floor of the mouth is an effective means for evaluating and characterizing deep lingual masses. We report two cases of macroglossia secondary to amyloidosis. The findings at CT and differential diagnosis are discussed.
A method is described for the operative correction of unilateral macroglossia. The method permits the tongue to be reconstructed so that it assumes a relatively normal size and shape. The scar is led out to the lateral border of the tongue so that the reconstructed tip retains sensation.
Plastic surgeons are not infrequently required to reduce the size of a large tongue in a child. Macroglossia is one of the main presenting features of the Beckwith-Wiedemann syndrome. This comprises a spectrum of disorders that includes a high incidence of malignancy. Patients with this syndrome should be identified and carefully assessed to exclude serious complications. This paper reports a series of 30 patients presenting to the Royal Children's Hospital over an 8-year period. An incidence of malignancy of 10 percent is recorded.
Unhappy with our postoperative results from standard tongue reduction methods, we have developed a central tongue reduction technique. This technique allows for alteration of the tongue base as well as tongue height and width while minimizing postoperative scarring. Although the technique and its associated surgical complications are in some ways comparable with the standard tongue reduction methods, our postoperative polysomnographic and tongue functioning results are improved. It is noteworthy that none of our patients had worse speech postoperatively and that our one Down syndrome patient had improved speech. Our indication for central tongue reduction is macroglossia of any sort in appropriate patients.
We describe an unusual case of severe postoperative macroglossia after posterior fossa surgery in the prone position, and review potential causative factors and prophylactic measures that may alleviate this serious complication.
An unusual case of massive oropharyngeal swelling and macroglossia occurring after cervical spine surgery performed on a patient in the prone position is described. Possible etiological factors are reviewed, and measures to prevent these complications are suggested.
We describe five cases of macroglossia in patients with posterior fossa disease and suggest that the primary mechanism is neurogenically determined rather than one of vascular obstruction or local trauma.
We describe a patient who had recurrent life-threatening episodes of isolated macroglossia due to the use of an angiotensin-converting enzyme (ACE) inhibitor. No associated facial, labial, pharyngeal, or laryngeal edema was noted. Aggressive treatment with epinephrine, steroids, and antihistamines resulted in rapid resolution of the tongue swelling and respiratory distress. Recurrent isolated angioedema of the tongue is an extremely rare variant of ACE inhibitor-related angioneurotic edema. The widespread use of ACE inhibitors mandates a special awareness by physicians of this potentially life-threatening yet treatable side effect.
Lymphangiomatous macroglossia, or giant tongue, usually presents within the first two years of life. The tongue enlarges to the point of protrusion from the mouth with resultant ulceration and frank necrosis of the tip. There may be associated malocclusion and prognathia produced by the enlargement of the tongue. The pathology and clinical manifestations of lymphangioma of the tongue are discussed in this paper, and a case report is presented.
A four year old white female with a clinical presentation of macroglossia is described. Speech disturbances and occasional episodes of traumatic injury to the tongue with severe bleeding brought the patient to seek dental care. Lymphangioma was diagnosed after incisional biopsy. The differential diagnosis of tongue enlargement in children is discussed including review of the literature relevant to the diagnosis and treatment of lymphangioma.
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.
A kindred is reported in which 8 infants were affected with the macroglossia-omphalocele syndrome. Their characteristics varied from an almost complete clinical picture to nodular hyperplasia with cytomegaly of the adrenals as the only manifestation of the disease. Chromosome analysis was normal. This syndrome appears to be inherited as an autosomal recessive trait, with a high proportion of incomplete clinical forms. The index patient also has signs of the Rubinstein-Taybi syndrome. The simultaneous occurrence of these two syndromes is probably a fortuitous event.