Individualized treatment of congenital vascular malformations of the tongue.

BACKGROUND/OBJECTIVE: Oral malformations of the tongue are exceedingly rare. The aim of this study was to evaluate the effectiveness of individualized treatment for patients with vascular malformations of the tongue. METHODS: This retrospective study is based on a consecutive local registry at a tertiary care Interdisciplinary Center for Vascular Anomalies. Patients with vascular malformations of the tongue were included. Indications for therapy of the vascular malformation were macroglossia with the impossibility to close the mouth, bleeding, recurrent infection and dysphagia. Size regression of the malformation (volume measurement) and symptom improvement were investigated. RESULTS: Out of 971 consecutive patients with vascular malformations, 16 patients suffered from a vascular malformation of the tongue. Twelve patients had slow-flow malformations and 4 fast-flow malformations. Indications for interventions were bleeding (4/16, 25%), macroglossia (6/16, 37.5%), and recurrent infections (4/16, 25%). For two patients (2/16, 12.5%), there was no indication for intervention due to absence of symptoms. Four patients received sclerotherapy, 7 patients Bleomycin-electrosclerotherapy (BEST) and 3 patients embolization. Median follow-up was 16 months (IQR 7-35.5). In all patients, symptoms had decreased after two interventions at a median (IQR 1-3.75). Volume reduction of the malformation of the tongue was 13.3% (from median 27.9 cm3 to median 24.2 cm3, p = 0.0039), and even more pronounced when considering only patients with BEST (from 86 cm3 to 59.1 cm3, p = 0.001). CONCLUSION: Symptoms of vascular malformations of the tongue are improved after a median of two interventions with significantly increased volume reduction after Bleomycin-electrosclerotherapy.

Ethosuximide induced macroglossia and oropharyngeal edema.

Acute macroglossia and laryngeal edema are rare adverse side effects that can cause life-threatening airway obstruction. We report a case of acute macroglossia that began after initiation of ethosuximide in a 15-year-old female with severe medically refractory epilepsy. Macroglossia worsened over the next two weeks of ethosuximide administration, preventing extubation. Macroglossia and laryngeal edema improved upon ethosuximide wean, and completely resolved after discontinuation. The patient was extubated successfully, with precautionary nasal trumpet placement and dexamethasone administration prior to extubation. In medically complex patients on multiple pharmacologic agents, anti-epileptic drugs should be suspected as a possible cause of acute macroglossia.

Management of persistent lingual angioedema: a team approach.

A 70-year-old African American man suffered anoxic encephalopathy following a choking episode. He had a history of hypertension, which was being treated with lisinopril, an angiotensin-converting enzyme inhibitor (ACEI). Soon after the patient's admission to an intensive care unit, his tongue began to swell until it reached more than twice its normal size and extended almost 2 inches outside his mouth. When the swelling did not diminish after 2 weeks, a diagnosis of ACEI-induced angioedema was determined. ACEIs have the potential to cause angioedema through an uncommon effect on the angiotensin-renin vascular control system. Lingual angioedema can be life-threatening due to the possibility of severe compromise of the airway and thus may require immediate intubation. After the ACEI is discontinued, swelling may remain if there is continued pressure from the maxillary and mandibular incisors on the dorsal and lingual surfaces of the tongue. In this case, the patient was comatose and unable to voluntarily move the tongue; therefore, relief from pressure was easily accomplished, and the edema was eventually diminished through a team effort in which a dentist instructed the nursing personnel on proper placement of Molt mouth props.

Methylprednisolone sodium succinate-associated macroglossia in a critically ill patient.

Allergic hypersensitivity reactions are a rare adverse effect of corticosteroids. Previous reports have identified patients who developed symptoms of urticaria, dyspnea, hypotension, bronchospasm, and angioedema occurring within minutes to an hour after corticosteroid administration. A 35-year-old woman is described who developed an atypical reaction of isolated macroglossia after receiving intravenous methylprednisolone sodium succinate for myasthenic crisis. Macroglossia was identified on day 2 of therapy and worsened through day 5. On day 5, she was transitioned to prednisone 50 mg daily administered by feeding tube. Tongue swelling improved by day 7 and on day 10, the patient was extubated. The patient required reintubation due to stridor, but received a tracheostomy and was weaned off mechanical ventilation by day 15. The reaction was not confirmed with skin-prick tests, intradermal tests, or a drug rechallenge; however, she had previously received and tolerated all other drugs administered during this time. Due to the timing of administration and onset of symptoms, we feel this adverse drug reaction was likely due to administration of methylprednisolone. Applying the Naranjo adverse drug reaction probability scale to this case, a score of six was obtained, indicating a probable association between the administration of methylprednisolone and the development of macroglossia. As intravenous corticosteroids are often used in the treatment of allergic reactions, they may be overlooked as a cause of macroglossia and other allergic reactions; therefore, practitioners need to be aware of the possibility of this adverse effect secondary to corticosteroid administration. In the event of methylprednisolone sodium succinate-induced macroglossia, alternative nonesterified corticosteroids, such as dexamethasone or prednisone, should be considered if continuation of therapy is required.

Response of glandular organs to experimental macroglossia.

Response of glandular organs to tongue enlargement was studied in 16 outbred male rats; each of them received injections of hydrophobic polyacrylamide gel (0.05 ml) in midline of the tongue. Changes in the studied glandular organs of external secretion (salivary glands) and internal secretion (thyroid and adrenal glands) were morphometrically detected.

Angioedema associated with dihydropyridine calcium-channel blockers in a child with Burkitt lymphoma.

PURPOSE: A case of severe macroglossia and angioedema in a child with Burkitt lymphoma who was treated with two dihydropyridine calcium-channel blockers is reported. SUMMARY: An eight-year-old white boy arrived at the pediatric emergency department with complaints of abdominal pain and distention after an episode of mild abdominal trauma. Physical examination results were significant for diffuse abdominal tenderness and distention, with a large palpable mass in the right quadrants. Computed tomography revealed a large abdominal mass, and a biopsy confirmed a diagnosis of Burkitt lymphoma. Before initiation of chemotherapy, the child developed tumor lysis syndrome, with subsequent renal failure and cardiorespiratory compromise. Once the patient was stabilized and sedated on mechanical ventilation, tumor-directed chemotherapy was initiated, and rapid tumor regression ensued. To control episodes of hypertension, nicardipine was initiated and titrated to achieve the blood pressure goals. Three days after initiation of nicardipine therapy, the child developed facial swelling and significant, protruding macroglossia. Eight days after nicardipine initiation, a tracheotomy was required due to upper airway obstruction; at that time, the patient was converted to amlodipine administered via nasogastric tube for continued blood pressure control. The boy's macroglossia persisted for another 18 days, until a multi-disciplinary drug therapy review resulted in the discontinuation of amlodipine. Within one week of the withdrawal of amlodipine, the child's macroglossia was completely resolved. CONCLUSION: An eight-year-old boy with Burkitt lymphoma developed severe macroglossia and angioedema when treated with nicardipine. The reaction persisted throughout treatment with amlodipine and resolved quickly after amlodipine was withdrawn.

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.

Episodic macroglossia as the sole manifestation of angiotensin-converting enzyme inhibitor-induced angioedema.

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.

Lingual angioedema after perindopril use.

We report a case of severe lingual edema and airway compromise associated with angiotensin-converting enzyme inhibitor use. Although angiotensin-converting enzyme inhibitors are generally considered as safe drugs, angioedema may induce severe respiratory distress and death.

Upper airway compromise in acute chlorpromazine ingestion.

A 16-year-old boy developed symptoms of acute upper airway compromise after chlorpromazine ingestion. The patient took six 100-mg Thorazine tablets "to help him sleep". Laryngeal-pharyngeal dystonia is a life-threatening form of dystonic reaction that is dose-dependent. In children, acute upper airway compromise should raise the suspicion of exposure to phenothiazines.

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.