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[Macroglossia. Considerations on a case load of 16 patients].

The Authors have examined sixteen cases of macroglossia and after hinting at the various causes they dwell upon clinic and therapeutic problems presented by patients. From the analysis of individual cases it results that muscular idiopathic hypertrophy is the most recurrent cause of macroglossia. It is furthermore emphasized the relation between macroglossia and occlusal disharmony. The kinds of therapy used and compared are two: surgical and orthodontic.

Adolescent↗

[Macroglossia as the initial symptom of amyloidosis].

Amyloidosis is a term that describes a heterogeneous group of disorders in which various tissues contain the characteristic insoluble fibrillar protein known as amyloid. A number of different forms of presentation have been described and include senile, familial or hereditary, systemic, localized, secondary and primary forms. We now report our management of a 79-year old woman who suffered from primary A-lambda-light chain amyloidosis. Amyloid changes first presented in the tissues of the tongue and floor of mouth and were seen clinically as a macroglossia. Further investigation led to a primary diagnosis of multiple myeloma IIa, as categorized by Durie and Salmon. Several cases have been reported to date describing secondary development of macroglossia due to long-term known and treated multiple myeloma. However, there has been only one report of macroglossia as a primary finding leading to the diagnosis of multiple myeloma.

Aged↗

[Differential diagnosis of macroglossia].

In addition to a survey of differential diagnoses to be considered in cases of macroglossia, rare general disorders connected with macroglossia are pointed out. As shown especially in a case of primary amyloidosis, recognition of and differential diagnostic considerations with regard to macroglossia sometimes make it possible to diagnose a general disorder not yet recognized.

Acromegaly↗

Transient neonatal diabetes mellitus and macroglossia.

Transient neonatal diabetes mellitus is an uncommon disorder. Macroglossia in association with transient neonatal diabetes mellitus has been reported only twice before. We report the case of a 21-day-old male infant referred from a peripheral hospital for management of hyperglycemia. The mother was a 21-year-old primigravid in good health. There was no history of diabetes or drug or alcohol exposure. The pregnancy was complicated by intrauterine growth retardation and oligohydramnios from 30 weeks' gestation and the birth weight at 38 weeks' gestation was only 1480 gm. Physical examination revealed dysmorphic features and asymmetric growth retardation. The admission weight (1840 gm) and length (40.5 cm) were 5 SDs less than the mean and head circumference (32.5 cm) was 1 SD less than the mean. Dysmorphic features included macroglossia, large fontanelles, hypospadias, umbilical hernia, and bilateral inguinal hernias. Hyperglycemia had been noted on day 1 of life with an initial blood glucose value of 16 mmol/L (288 mg/dl). Despite treatment with regular insulin blood glucose control continued to be erratic. Therefore a regimen of daily NPH insulin was begun, which has a smoother action. Interestingly, from day 41 to day 47 the infant did not receive insulin and a crude control of the blood glucose was demonstrated. Peak levels of blood glucose in excess of 20 mmol/L (360 mg/dl) were followed by drops to levels less than 2 mmol/L (36 mg/dl) without insulin administration. This abnormal pattern of glucose control may represent poorly regulated release of endogenous insulin. However, because of unsatisfactory glucose levels administration of daily NPH insulin was reintroduced. The infant was discharged from the hospital on day 50 and administration of insulin was discontinued uneventfully at 9 months. At 1 year the hemoglobin A1c level was still normal and the infant's weight was at the 10th percentile. Macroglossia was less pronounced. Development showed mild delay in gross motor milestones.

Diabetes Mellitus, Type 1↗

Macroglossia. An unusual presentation of pemphigus vulgaris.

A 19-year-old woman presented with macroglossia of five months' duration and without bullae or erosions. A biopsy revealed a picture consistent with pemphigus vulgaris. The patient subsequently developed typical oral erosions. Her tongue enlargement and oral ulcerations improved dramatically with topical and systemic steroid therapy. Pemphigus vulgaris should be considered in the differential diagnosis of macroglossia.

Adult↗

Macroglossia: prenatal ultrasonographic diagnosis and proposed management.

A case in which macroglossia was the sole unusual prenatal sonographic finding in a fetus affected with trisomy 21 is presented. The differential diagnosis of fetal macroglossia is given, emphasizing its strong relationship to fetal aneuploidy, together with the principles for evaluation and management.

Adult↗

Autosomal dominant macroglossia in two unrelated families.

Two unrelated families, one with 15 and the other with 3 members affected with macroglossia as a sole trait, are described. It is concluded that this entity differs from previously reported syndromes presenting macroglossia and is inherited in an autosomal dominant fashion.

Female↗

Posttraumatic macroglossia complicated by hyaluronidase injection.

A patient with posttraumatic macroglossia has been presented. A literature review reveals that this is a rare sequela of tongue injury and is usually of acute onset. The delayed symptom of swelling in this patient and the exacerbation of swelling in association with hyaluronidase suggest a causal relationship between the two events. Macroglossia has not been reported in association with hyaluronidase injection, and it is recommended that the drug not be used to treat swelling after tongue injury.

Adult↗

Episodic macroglossia in Down's syndrome.

We report a unique case of recurrent gross enlargement of the tongue associated with lymphangiectasia and tongue protrusion in an adult male with Down's syndrome. There were three episodes of acute macroglossia with no clinically identifiable cause. Spontaneous resolution always occurred after five to six days. Histological examination of the tongue, at post mortem, revealed a primary lesion of the lingual lymphatics. The possible mechanisms of acute macroglossia and the surgical techniques which could have been used to reduce the tongue bulk are discussed.

Acute Disease↗

[Traumatic macroglossia--a rare indication for tracheotomy].

Hemorrhage into the tongue caused by a trauma is able to produce dangerous hematomas. During an hypertensive crisis a 17 year old patient suffered from an ictus with a bite on his tongue. A macroglossia developed with shortness of breath, so that only a tracheotomy could grand a sufficient ventilation. The macroglossia decreased within 6 days, the nasal breathing was no longer prevented, so the tracheostoma could be closed. An operative intervention is necessary, if conservative therapy is without success referred to diminuation of the tongue.

Adolescent↗

The efficacy of tongue resection in treatment of symptomatic macroglossia in the child.

Nine patients with symptomatic macroglossia, 3 with Beckwith-Wiedemann syndrome, 3 with Down's syndrome, and 3 with lymphatic malformation of the tongue, were evaluated for postoperative improvement following partial glossectomy. Symptoms evaluated were speech intelligibility, oral competence at rest, drooling, and normal deglutition. All patients with Down's syndrome and Beckwith-Wiedemann syndrome were improved postoperatively. All patients with Down's syndrome exhibited improved deglutition and reduced drooling. Only 1 patient (33%), however, achieved oral competence. Speech was improved postoperatively in 2 patients (67%) with Down's syndrome. Patients with lymphatic malformation of the tongue did not exhibit consistent long-term improvement postoperatively, suggesting that conservative management of macroglossia in this patient group may be indicated.

Adolescent↗

Treatment of macroglossia in Beckwith-Wiedemann syndrome.

A case of macroglossia caused by Beckwith Wiedemann syndrome is reported. Beckwith-Wiedemann Syndrome is an overgrowth disorder characterized by a constellation of congenital anomalies. The most common manifestations are omphalocele, macroglossia, gigantism, and visceromegaly. When the tongue reaches a huge dimension, clinical symptoms are represented by dysphagia, alterations in speech, difficulty in chewing, obstruction of the upper airways, and psychologic consequences derived from the patient's physical appearance. The authors describe the surgical strategy performed in the reported case.

Beckwith-Wiedemann Syndrome↗

Traumatic macroglossia: a life-threatening complication.

OBJECTIVE: To describe the use of muscle relaxants and a bite raiser to avoid continued tongue trauma. DESIGN: Case report. SETTING: A tertiary general intensive care unit (ICU). INTERVENTIONS: Muscle relaxation and bite raiser. MAIN RESULTS: Muscle relaxation and a bite raiser were used in a 17-yr-old male with traumatic macroglossia, which allowed for rapid resolution of edema and prevented additional trauma to the tongue. CONCLUSION: Early use of a bite raiser together with muscle relaxants allows for more rapid solution of edema and prevention of additional trauma to the tongue in patients with traumatic macroglossia.

Adolescent↗

Acquired macroglossia due to lopinavir/ritonavir treatment.

A HIV-positive patient, 3 months after the treatment initiation with lopinavir-/ritonavir (LPV/r) acquired macroglossia. The tongue biopsy revealed mature adipose tissue accumulated into submucosa. The drug was discontinued and the patient showed a significant improvement. This case is the first case in the medical literature of acquired macroglossia because of LPV/r, a drug causing changes in body fat composition.

Aged↗

Massive macroglossia, amyloidosis and myeloma.

A 74 year old man with light-chain myeloma developed amyloidosis with macroglossia after 10 years of therapy with alkylating agents. Over a 2-year period his tongue enlarged to persistently protrude from his mouth, inhibit his speech, interfere with normal swallowing and eventually threaten his airway. As a life-saving procedure the tumorous anterior two-thirds of the tongue was resected, with excellent primary healing. Within two weeks the patient's speech became comprehensible and his ability to eat returned to normal. Although rare in amyloidosis, massive macroglossia may occur and surgical correction is easily achieved.

Amyloidosis↗

Macroglossia and amyloidoma of the buttock: evidence of systemic involvement in dialysis amyloid.

A 48-year-old male on cuprophane haemodialysis for 18 years, with a history of dialysis arthropathy and recurrent carpal tunnel syndrome developed macroglossia and bilateral buttock tumoral masses. The tongue and buttock masses were biopsied. Histology of both biopsies showed amyloid deposits of the beta 2-microglobulin (B2M) variety. Amyloidomas in the gluteal region and macroglossia have not been previously described in amyloid derived from B2M. These findings suggest that systemic B2M amyloidosis can have a similar tissue distribution to AL amyloidosis. This case also stresses the importance of inspection of the tongue, and palpation of the gluteal region for masses, in the assessment of patients with dialysis arthropathy.

Amyloid↗

Traumatic macroglossia.

A case of severe macroglossia resulting from trauma (tongue biting) during eclampsia and causing respiratory obstruction is described. Despite medical treatment with steroids and antibiotics for a week, followed by tracheostomy, no significant improvement was observed. After an energetic but cautious maneuver of reducing and restraining the tongue in the oral cavity, the swelling reduced dramatically in 24 to 48 hours. Earlier manual replacement of the tongue into the oral cavity is advised in order to arrest the cycle of venous and lymphatic obstruction and congestion that leads to further edema and increased tongue swelling. The mechanism of traumatic macroglossia is discussed.

Adult↗