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The Cleft Palate-craniofacial Journal :... Sep 2022Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by surgeons....
INTRODUCTION
Macroglossia occurs in 80% to 99% of patients with Beckwith-Wiedemann syndrome (BWS) and a variety of surgical techniques for tongue reduction are offered by surgeons. The purpose of this study is to evaluate the postoperative outcomes of the anterior "W" tongue reduction technique in patients with BWS.
METHODS
A retrospective review was conducted of all patients diagnosed with BWS that underwent an anterior "W" tongue reduction for macroglossia in the past 7 years, performed by 2 surgeons. Demographics, procedural characteristics, perioperative outcomes, and complications were assessed.
RESULTS
A total of 19 patients met inclusion criteria consisting of 8 male and 11 female patients. The mean age at the time of surgery was 405 days, mean surgeon operating time was 1.06 h, and mean length of follow-up was 467 days. Postoperative oral competence was observed in 100% of patients. There was no reported history of sleep apnea or airway compromise. Speech delay was seen in 4 patients pre- and postoperatively. Feeding issues decreased from 7 patients preoperatively to 1 patient postoperatively. Preoperative prevalence of class III malocclusion (53%) and isolated anterior open bite (26%) decreased postoperatively to 37% and 16%, respectively. The only reported complications were superficial tip wound dehiscence in 3 patients treated with nystatin antifungal therapy. None of the patients required revisional surgery.
CONCLUSION
Patients treated with the anterior "W" tongue reduction technique had low rates of perioperative complications and significant improvements in oral competence. Anterior "W" tongue reduction is safe and effective for the correction of macroglossia in patients with BWS.
Topics: Beckwith-Wiedemann Syndrome; Female; Glossectomy; Humans; Macroglossia; Male; Tongue
PubMed: 34402311
DOI: 10.1177/10556656211036607 -
BMJ Case Reports Jan 2021A 50-year-old African American woman with hypertension, congestive heart failure, chronic kidney disease and prior cerebral vascular accident was transferred from an...
A 50-year-old African American woman with hypertension, congestive heart failure, chronic kidney disease and prior cerebral vascular accident was transferred from an outside hospital after being found unresponsive and subsequently intubated for severe orolingual swelling. Imaging showed left thalamic haemorrhagic stroke, and the lingual swelling was clinically concerning for angio-oedema, with which a lingual biopsy was consistent. Work-up was negative for hereditary or acquired angio-oedema, and imaging was negative for structural causes. Of note, the patient had an episode of severe orolingual swelling 3 months prior to this presentation after suffering left thalamic haemorrhage which self-resolved after approximately 2 months. In both episodes lingual swelling predated receipt of tissue plasminogen activator and she had discontinued ACE inhibitor therapy since her first episode of tongue swelling. Despite medical and supportive management, tongue swelling progressed during admission and the decision was made to allow the patient's tongue swelling to self-resolve.
Topics: Basal Ganglia Diseases; Female; Hemorrhagic Stroke; Humans; Macroglossia; Middle Aged; Recurrence; Tracheostomy
PubMed: 33431468
DOI: 10.1136/bcr-2020-238775 -
The Journal of Clinical Pediatric... 2003A four year old white female with a clinical presentation of macroglossia is described. Speech disturbances and occasional episodes of traumatic injury to the tongue... (Review)
Review
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.
Topics: Child, Preschool; Female; Humans; Lymphangioma; Macroglossia; Sclerosing Solutions; Tongue Neoplasms
PubMed: 12597690
DOI: 10.17796/jcpd.27.2.a466020r10440121 -
Neurological Sciences : Official... Jun 2023Macroglossia is one of the clinical signs that emerges in patients with amyotrophic lateral sclerosis (ALS) who are undergoing invasive ventilation.
BACKGROUND
Macroglossia is one of the clinical signs that emerges in patients with amyotrophic lateral sclerosis (ALS) who are undergoing invasive ventilation.
OBJECTIVE
We aimed to investigate the relationship between the progression of macroglossia and the clinical characteristics during the advanced stages of ALS.
METHODS
Forty-one patients with ALS using invasive ventilation with tracheostomy were prospectively followed over a 3-year period. We evaluated the prevalence of macroglossia, motor disabilities including oral function, communication ability (communication stage I to V) as a marker of disease progression, and nutritional factors. We analyzed the potential factors affecting the progression of macroglossia using a multivariate logistic analysis.
RESULTS
The number of patients with macroglossia was increased during the follow-up (from 24.4 to 53.7%), while the communication stage progressed from 2.6 in average (SD 1.7) to 3.5 (1.4). During the study, body weight increased, while energy intake decreased, suggesting progressive hypometabolism. Eight patients had newly developed macroglossia during the study and showed greater progression of communication impairment than those without macroglossia. Multivariate logistic regression analysis showed that communication impairment was a factor associated with macroglossia.
CONCLUSION
Macroglossia in advanced ALS with invasive ventilation is associated with disease progression.
Topics: Humans; Amyotrophic Lateral Sclerosis; Macroglossia; Clinical Relevance; Disease Progression
PubMed: 36715782
DOI: 10.1007/s10072-023-06646-7 -
Neurocritical Care Feb 2014Macroglossia has been reported in patients undergoing posterior fossa neurosurgical procedures and is thought to be as a result of venous engorgement from intubation or...
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.
Topics: Adolescent; Adrenergic beta-Antagonists; Aged; Amines; Anti-Inflammatory Agents; Brain Injuries; Brain Stem; Calcium Channel Blockers; Cranial Fossa, Posterior; Cyclohexanecarboxylic Acids; Dexamethasone; Female; Gabapentin; Humans; Macroglossia; Male; Middle Aged; Primary Dysautonomias; Tracheostomy; Treatment Outcome; gamma-Aminobutyric Acid
PubMed: 24002892
DOI: 10.1007/s12028-013-9901-7 -
Journal of Ultrasound in Medicine :... Jan 2023Abnormal fetal tongue size is a phenotypic feature of various syndromes including Beckwith-Wiedemann, Pierre-Robin, oromandibular limb hypoplasia, chromosomal...
OBJECTIVES
Abnormal fetal tongue size is a phenotypic feature of various syndromes including Beckwith-Wiedemann, Pierre-Robin, oromandibular limb hypoplasia, chromosomal aberrations, etc. Current data regarding normal fetal tongue size are limited. Hence, micro/macroglossia are subjectively determined. The aim of the study was to construct a contemporary fetal tongue nomogram and to assess its clinical contribution.
METHODS
A prospective cross-sectional study was performed in well dated, low risk, singleton pregnancies. Fetal tongues were measured by 5 trained sonographers. Highest quality images were selected. Intra- and interobserver variability was assessed. Tongue length, width, area, and circumference 1st to 99th centiles were calculated for each gestational week. Based on the normal tongue size charts, we created a Tongue Centile Calculator.
RESULTS
Over 18 months, 664 tongue measurements were performed. A cubic polynomial regression model best described the correlation between tongue size and gestational age. The correlation coefficient (r ) was 0.934, 0.932, 0.925, and 0.953 for tongue length, width, area, and circumference, respectively (P < .001). Intra- and interobserver variability had high interclass correlation coefficients (>0.9). Using the new charts, we were able to identify 2 cases of macroglossia, subsequently diagnosed with Beckwith-Wiedemann, and 4 cases of microglossia, 3 associated with Pierre-Robin sequence, and 1 associated with persistent buccopharyngeal membrane.
CONCLUSIONS
We present novel fetal tongue size charts from 13 to 40 weeks of gestation. Clinical application of these nomograms may be beneficial in the prenatal diagnosis of syndromes or malformations associated with abnormal fetal tongue size.
Topics: Pregnancy; Female; Humans; Macroglossia; Beckwith-Wiedemann Syndrome; Cross-Sectional Studies; Prospective Studies; Tongue
PubMed: 35396717
DOI: 10.1002/jum.15983 -
Anesthesia and Analgesia Aug 1999
Topics: Adult; Humans; Macroglossia; Male; Postoperative Complications; Prone Position
PubMed: 10439787
DOI: 10.1097/00000539-199908000-00064 -
BMJ Case Reports Jul 2021We report the case of a 59-year-old woman who developed acute macroglossia following prolonged prone positioning for management of COVID-19. We found that Biotene mixed...
We report the case of a 59-year-old woman who developed acute macroglossia following prolonged prone positioning for management of COVID-19. We found that Biotene mixed in glycerin was effective at removing keratinised lingual plaques and better tolerated than Biotene alone. Additionally, uniform tongue compression applied via tubular elastic dressing yielded more efficacious results than uneven tongue compression via Coban.
Topics: COVID-19; Female; Humans; Macroglossia; Middle Aged; Patient Positioning; Prone Position; Respiratory Insufficiency; SARS-CoV-2
PubMed: 34230050
DOI: 10.1136/bcr-2021-244224 -
The Cleft Palate-craniofacial Journal :... Jan 2022Macroglossia is a characteristic feature of Beckwith-Wiedemann syndrome (BWS), commonly treated with reduction glossectomy to restore form and function. There exists no...
OBJECTIVE
Macroglossia is a characteristic feature of Beckwith-Wiedemann syndrome (BWS), commonly treated with reduction glossectomy to restore form and function. There exists no consensus on the perioperative management of these patients undergoing tongue reduction surgery, including anecdotal information regarding how long postoperative intubation should be maintained. The aim of this study is to evaluate the necessity of prolonged postoperative intubation in patients receiving tongue reduction surgery via the surgical and anesthetic management methods at our center.
DESIGN
Retrospective case series.
SETTING
Institutional care at Level I Children's Hospital.
PARTICIPANTS
All children less than 18 years old with BWS and congenital macroglossia who underwent tongue reduction surgery over 5 consecutive years at our center (N = 24).
INTERVENTIONS
Tongue reduction surgery via the "W" technique.
MAIN OUTCOME MEASURES
Success of immediate postoperative extubation and related surgical complications.
RESULTS
Immediate, uncomplicated postoperative extubation was successfully performed in all patients who received tongue reduction surgery for congenital macroglossia.
CONCLUSIONS
Prolonged postoperative intubation for tongue reduction surgery may not be necessary as immediate, uncomplicated postoperative extubation was achieved in 100% of patients who received tongue reduction surgery at our center.
Topics: Adolescent; Beckwith-Wiedemann Syndrome; Child; Glossectomy; Humans; Intubation, Intratracheal; Macroglossia; Retrospective Studies
PubMed: 33550827
DOI: 10.1177/1055665621991739 -
Journal of Perinatology : Official... 1996Transient neonatal diabetes mellitus is an uncommon disorder. Macroglossia in association with transient neonatal diabetes mellitus has been reported only twice before.... (Review)
Review
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.
Topics: Diabetes Mellitus, Type 1; Humans; Hyperglycemia; Hypoglycemic Agents; Infant, Newborn; Insulin, Isophane; Macroglossia; Male; Remission, Spontaneous; Somatomedins
PubMed: 8866300
DOI: No ID Found