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PubMed2000

Successful airway control with the laryngeal mask in an infant with Beckwith-Wiedemann syndrome and hepatoblastoma for central line catheterization.

We present a case of an infant with severe macroglossia, hypoglycaemia and inguinal hernia associated with hepatoblastoma (Beckwith-Wiedemann syndrome) in which a laryngeal mask airway (LMA) was useful to secure the airway during central line insertion. Carbon dioxide monitoring through LMA proved effective to assess airway patency during positioning for central vein puncture. In this syndrome, where a potentially difficult airway may be encountered, LMA allowed adequate ventilation, avoiding the risk and inconvenience of tracheal intubation.

Beckwith-Wiedemann Syndrome↗
PubMedDate not supplied

Dysarthria as the leading symptom of hypothyroidism.

Typical symptoms of hypothyroidism are lethargy, cold intolerance, slowing of intellectual and motor activity, declining appetite, increasing weight, and dry skin. A 43-year-old man with hypothyroidism presented with dysarthria as the leading symptom. Further symptoms were cramps in the legs after exercise, dizziness, and stunned feeling. He suffered from severe snoring for 4 years, and obstructive sleep apnea syndrome was diagnosed 2 years before. Creatine phosphokinase was elevated. Electromyography was myogenic. Echocardiography showed a thickened myocardium. An otolaryngologic investigation revealed macroglossia and hypertrophy of the uvula. After administration of L-thyroxine, the symptoms rapidly improved. Dysarthria may be the leading symptom of hypothyroidism and can be promptly resolved after hormone substitution.

Adult↗
PubMed2000

Hypoglycemia in Beckwith-Wiedemann syndrome.

Beckwith-Wiedemann syndrome (BWS) is an overgrowth syndrome associated with macrosomia, macroglossia, abdominal wall defects, hypoglycemia in the neonatal period and embryonal cancers of infancy and early childhood. The frequency of hypoglycemia in this population is between 30% and 50%. The majority of infants with hypoglycemia will be asymptomatic and have resolution of the hypoglycemia within the first 3 days of life. Less than 5% will have hypoglycemia beyond the neonatal period requiring either continuous feeding or a partial pancreatectomy. The cause of hypoglycemia is unclear, but direct and indirect evidence supports a hyperinsulinemia as the major factor. Recent identification of the majority of genes associated with BWS in the 11p15 region and the genotype of persistent hyperinsulinemia hypoglycemia of childhood also in the 11p15 region may provide a molecular basis for hypoglycemia in BWS, particularly for the occasional patients with hypoglycemia requiring a partial pancreatectomy. Detailed genotype phenotype evaluations are needed and should provide an insight as to why patients with BWS have hypoglycemia.

Beckwith-Wiedemann Syndrome↗
PubMed2001

Tongue lesions in the pediatric population.

OBJECTIVE: To describe the spectrum of pediatric tongue lesions treated surgically at Columbia-Presbyterian Medical Center from January 1990 to December 1999. STUDY DESIGN AND SETTING: Retrospective case-series at the pediatric hospital of a tertiary care, academic medical center. RESULTS: Seventeen patients were identified. Their ages ranged from 1 to 132 months (median, 7 months). Eight lesions were located anteriorly: mucous cyst (1), polyp (1), chronic inflammatory mass (1), hamartoma (1), squamous papilloma (2), cavernous hemangioma (1), and vascular malformation (1). Four lesions were located posteriorly: teratoma (1), glial choristoma (1), osseous choristoma (1), and benign epithelial cyst (1). Finally, there were 5 diffuse lesions including macroglossia (4) and massively infiltrating congenital lymphatic malformation (1). Symptoms included respiratory distress (3) and dysarthria (3); all other children were asymptomatic. CONCLUSIONS: This series revealed an interesting spectrum of rare solid tumors; compared with other large series, fewer lymphatic and vascular malformations were seen. Presenting symptoms, differential diagnosis, and surgical approach were differentiated according to lesion location.

Child, Preschool↗
PubMed2003

The role of tongue reduction.

Macroglossia may occur as a congenital or acquired condition. The enlarged tongue has both functional and cosmetic deformity, which may affect the oral airway, speech, and the development of the jaws. We discuss the various tongue-reduction procedures and present cases to illustrate the uses of this procedure, its potential complications, and the results.

Adolescent↗
PubMed1998

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.

Adult↗
PubMed2006

Hemimacroglossia caused by isolated plexiform neurofibroma: a case report.

OBJECTIVES: The present case is a diffuse isolated plexiform neurofibroma of the tongue that was not associated with neurofibromatosis that we treated with intraoral surgery. STUDY DESIGN: A case report. METHODS: We present a 5-year-old girl with isolated plexiform neurofibroma of the tongue with infiltration of the tongue base and review treatment approaches for this very rare tumor. RESULTS: An intraoral approach appears to be a good alternative method that allows both an acceptable exposure for total excision and limited postoperative sequelae. CONCLUSIONS: Isolated plexiform neurofibroma of the tongue is very rare. It causes a massive macroglossia that is progressive with advance of years and disturbs the patient's speech, occlusion, and tongue movements. Involvement of the tongue may be associated with infiltration into the deep structures of the neck by the tumor, thereby presenting considerable operative difficulties.

Biopsy, Needle↗
PubMed1995

Massive lingual swelling following palatoplasty. An unusual cause of upper airway obstruction.

We report a case of upper airway obstruction as a result of delayed massive lingual swelling following routine cleft palate repair in an otherwise healthy 12-month-old girl. We believe that ischaemia and venous congestion were the causes of macroglossia, after prolonged use of the Digman Dott tongue retractor. In any difficult and lengthy repair, we recommend the prophylactic insertion of a nasopharyngeal airway under direct vision by the surgeons after surgery to prevent potential upper airway obstruction.

Acute Disease↗
PubMed1985

Oral and dental development in X chromosome aneuploidy.

Though frequently noted, orofacial anomalies in poly X syndromes have been seldom reviewed. In a study of patients with an X chromosome aneuploidy (XXXXX, XXXX, XXXXY, XXXY) the following facial and dental defects were found to be characteristic features of these syndromes: mainly bifid uvula and macroglossia, enamel defects, dental taurodontism and abnormal roots. These observations are discussed in the light of the possible role of X-linked genes.

Adolescent↗
PubMed1990

Laband syndrome: a case report.

A case of Laband syndrome in an 8-yr-old girl is presented. The case is sporadic. The patient manifests enlargement of the soft tissue of the hard palate and the gingiva, which partly or completely covers the crowns of the teeth and macroglossia. The cartilagenous part of the nose and the ears is large and soft. She has synophrys and thick, straight hair. The nails of the fingers and toes are dysplastic. The girl exhibits no other abnormality, except an IQ of 61.

Abnormalities, Multiple↗
PubMedDate not supplied

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.

Child, Preschool↗
PubMed1990

Amyloidosis with oral involvement. Case report.

A patient with chronic renal failure was investigated after complaining of oral discomfort which was found to be due to macroglossia and generalized involvement of the oral soft tissues by amyloidosis. A search for multiple myeloma proved to be positive. She also had a previous history of Carpal-tunnel syndrome. Despite an initial good response to treatment with phenylalanine nitrogen mustard (melphalan hydrochloride), she finally succumbed to end-stage renal failure.

Amyloidosis↗
PubMed1992

Congenital hypothyroidism detected by neonatal screening: relationship between biochemical severity and early clinical features.

The relationships between biochemical severity of hypothyroidism (as judged by plasma thyroxine) and the clinical and radiographic findings at diagnosis were evaluated in 449 infants born in 1982-4 with congenital hypothyroidism identified by neonatal screening. Details of pregnancy, delivery, and the neonatal period were also examined and compared with the findings in a normal population of 36,727 infants born in 1988. Infants with plasma thyroxine values of 30 nmol/l or less had a significantly higher incidence of prolonged jaundice, feeding difficulties, lethargy, umbilical hernia and macroglossia, showed more severe delay of bone maturation on a knee radiograph, and had a higher proportion of thyroid agenesis on isotope scan. In contrast, an ectopic or hypoplastic gland was more common in infants with plasma thyroxine values above 30 nmol/l. Prevalence of illness in pregnancy and mode of delivery was not related to severity of hypothyroidism and were similar to figures for the normal population. Induction of labour, gestation over 40 weeks, and birth weight above 3500 g were significantly more common in the hypothyroid infants. Perinatal illness and congenital malformations were more common in the infants with low plasma thyroxine values at diagnosis.

Adult↗
PubMed1995

Sweet's syndrome and acute myelogenous leukemia in a patient who presented with a sudden massive swelling of the tongue.

We report a case of Sweet's syndrome in a patient in whom an acute myeloblastic leukemia was subsequently diagnosed. Approximately 10-20% of the reported cases of Sweet's syndrome occur in patients with a malignancy, the neoplastic condition most commonly reported being acute myelogenous leukemia. This case is of interest because of its unusual clinical presentation: the cutaneous lesions were accompanied by a massive swelling of the tongue. We suggest that this sudden macroglossia was caused by an infiltration of mature neutrophils, as seen in cutaneous lesions of Sweet's syndrome. There have been several reports recently of extracutaneous manifestations of Sweet's syndrome, and neutrophilic infiltrates have been found both in several internal organs and at mucosal locations.

Female↗
PubMedDate not supplied

Beckwith-Wiedemann syndrome: difficulties with prenatal diagnosis.

Beckwith-Wiedemann syndrome (BWS), though a well-delineated clinical and morphological entity, can be difficult to diagnose by prenatal ultrasound examination when incomplete forms occur. We present a case with sonographic results including hydronephrosis, cardiomegalia, hepatomegalia, macroglossia, and prominent forehead. No abdominal wall defect was detected. Karyotype was normal. In spite of intensive prenatal diagnostics, BWS could not be diagnosed definitely until birth.

Adult↗
PubMed2003

Acute edema of the tongue: a life-threatening condition.

Acute macroglossia is rare, but may cause upper airway obstruction requiring emergency intervention. The cause of the problem is often obscure. Edema of the tongue may be due to angioedema or to allergy. We present several cases of acute edema of the tongue, in 3 cases causing life-threatening airway obstruction. Among these, we present the first case of acute enlargement of the tongue due to the ingestion of artichoke.

Acute Disease↗
PubMed1985

A case report of lingual lymphangioma presenting as recurrent massive tongue enlargement.

Lymphangiomas are benign congenital tumors that frequently result in macroglossia. Tongue involvement may lead to airway obstruction, swallowing difficulties, mandible-dental deformities, and speech disturbances. This case report demonstrates the many difficulties in diagnosis and management. Reduction in tongue size with preservation of motor and sensory function is best accomplished by wedge resection of the tip of the tongue.

Child, Preschool↗
PubMed2003

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.

Aged↗