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The aim of this study was to evaluate the postoperative course and long-term functional and aesthetic outcomes in patients with Beckwith-Wiedemann syndrome (BWS) following surgical reduction of macroglossia, using multiple questionnaires. Patients with BWS who underwent keyhole reduction for macroglossia were included in this study. The postoperative course for each patient was recorded, and multiple questionnaires were administered to evaluate aesthetic concerns, oral incompetence or feeding difficulties, sleep-disordered breathing symptoms, and speech. Nine patients underwent ten reduction glossoplasty surgeries. The mean age at surgery was 22 months. The postoperative course for each case was uneventful, except for one patient who had wound dehiscence. The questionnaires revealed significant improvements in tongue appearance, feeding, drooling, facial appearance, and psychosocial outcomes. There was also a significant reduction in sleep-disordered breathing symptoms after surgery. Keyhole reduction glossoplasty is a safe and effective procedure for the treatment of macroglossia in BWS patients, with excellent functional and aesthetic outcomes and a low complication rate., (Copyright © 2024 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Objective: This review was conducted to define the natural history of unoperated Beckwith-Wiedemann syndrome (BWS) macroglossia and the effect of tongue reduction surgery upon breathing, eating, speaking and dentoskeletal development in individuals having BWS. Design: This is a retrospective study of medical records., Setting: All patients were evaluated and treated in one of two Children's Hospitals by an ACPA approved Craniofacial Team., Patients/participants: Medical records were reviewed of 526 individuals having a diagnosis of BWS and evaluated in-person by a single craniofacial surgeon between 1986 and 2014 in conjunction with a series of multi-disciplinary craniofacial team colleagues. 28 individuals were excluded having had multiple tongue reductions elsewhere. 498 individuals comprise the "pre tongue-reduction group". The "post tongue-reduction group" consists of 391 individuals who underwent surgical tongue reduction by one surgeon using one technique between 1986 and 2014., Main Outcome Measures: The primary outcome measure was change in anterior dental occlusion following tongue reduction surgery. Tongue reduction surgery was performed on the assumption that it would improve dentoskeletal relationships. Secondary outcome measures were: breathing, feeding/swallowing, and speech. Results: A significant difference (p<0.001) over time between the two groups was found with less anterior occlusal abnormality in the tongue reduction group. Tongue reduction surgery had no mortality and minimal morbidity for breathing, feeding/swallowing, and speech and can ameliorate obstructive sleep apnea. Conclusions: Surgical tongue reduction for BWS macroglossia is recommended for the infant or child in primary dentition with a grossly abnormal anterior tooth/jaw relationship and/or obstructive sleep apnea., Competing Interests: Declaration of Conflicting InterestsThe author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Robin sequence, macroglossia, and ankyloglossia are disorders affecting the tongue and mandible in the pediatric population. Each of these can have a significant impact on breathing, feeding, speech, dentition, and craniofacial growth. This review discusses the interdependent and coordinated development of both the tongue and mandible, the functional impacts of these disorders, and appropriate management strategies., Competing Interests: Disclosure The authors have no disclosures or conflicts of interests., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Beckwith-Wiedemann Syndrome (BWS) is a rare genetic disorder that causes developmental defects as well as an elevated risk of malignancies. Macroglossia, or an enlarged tongue, is a common symptom of BWS that may have a negative influence on a person's quality of life. The aim of this systematic review is to look at the present state of knowledge about the repercussions of macroglossia, as well as the influence of the timing of surgical resection, or glossectomy, in the treatment of severe cases of macroglossia (Ref. 35). Keywords: macroglossia, Beckwith-Wiedemann syndrome, glossectomy.

Objectives: The role of tongue reduction surgery (TRS) in preventing excessive mandibular growth and anterior open bite in children with Beckwith-Wiedemann Spectrum (BWSp) is still controversial. This cross-sectional study aimed at comparing craniofacial growth pattern in children affected by BWSp either treated or not treated with early TRS for severe macroglossia. Considering the invasive nature of such surgery, the present study could help in clarifying the need for TRS to reduce or prevent growth disturbances., Materials and Methods: Orthopantomography and lateral skull x-ray images were taken either from surgically treated or non-surgically treated patients, aged 5 to 8 years, to compare dentoskeletal features and craniofacial growth by cephalometric analysis. Molecular testing results were collected from their medical records., Results: Eighteen BWSp patients were consecutively recruited: 8 underwent TRS at 14.9 ± 2.2 months of age, while 10 did not. Anterior open bite and dental class III were more frequently observed in the surgically treated group, but none showed skeletal class III. No statistically significant differences were observed in growth pattern, but children treated with TRS showed a tendency towards both maxillary and mandibular prognathism with protruding lower lip. Growth pattern seemed to be not related to molecular subtypes., Conclusions: These preliminary data suggest that early TSR does not improve craniofacial growth pattern and dentoskeletal features in BWSp children., Clinical Relevance: Reductive glossectomy may not be justified for preventing or avoiding oro-facial deformities in BWSp; therefore, early monitoring of maxillofacial development of each affected child has a great clinical significance., (© 2023. The Author(s).)

Beckwith-Wiedemann syndrome (BWS) is a genetic disease with phenotypic variability and the following signs: macroglossia, asymmetry, lateralised overgrowth, overgrowth of the internal organs, abdominal wall defects, neonatal hypoglycemia and increased risk of embryonic tumours. The prevalence is reported as being between 1 in 10,000 and 1 in 21,000 live births. The disease is caused by molecular changes in gene clusters on the short arm of chromosome 11 region P15.5. We present the case of a female, born preterm at 32 0/7 weeks. A UPD(11)pat-mutation was diagnosed postnatally. The particular feature of her case was an early tongue reduction surgery which was necessary because of drinking and breathing difficulties. Long-lasting hypoglycemia was difficult to treat., Competing Interests: Die Autorinnen/Autoren geben an, dass kein Interessenkonflikt besteht., (Thieme. All rights reserved.)

While macroglossia is a newly accepted component of brachycephalic obstructive airway syndrome (BOAS) in dogs, macroglossia with increased tongue fat is a well-known cause for obstructive sleep apnea (OSA) in people, and targeted reduction procedures such as midline glossectomy are used to treat people with OSA. While midline glossectomy has been described in dogs, tissue contributions to macroglossia have not been characterized. The purpose of this retrospective, descriptive, case-control study was to describe and compare volumetric dimensions of the tongue and tongue fat in brachycephalic (BC) and mesaticephalic (MC) dogs using CT images. Data collected included head and neck CT images from 17 BC and 18 control MC dogs. Multiplanar reformatted and 3D reconstructed images were created using image segmentation and specialized visualization software to calculate volumetric dimensions of the total tongue, tongue fat, and tongue muscle. Rostral and caudal topographical distributions of fat were compared. Total tongue and tongue muscle volume (P < 0.0001) and tongue fat volume (P = 0.01) normalized to body weight (BW) were greater in BC dogs. More fat was localized in the caudal tongue in both groups (P < 0.04). In regression analysis, BC conformation and increased weight were significant predictors of increased tongue fat volume. As in people, increased tongue fat may contribute to macroglossia and sleep-disordered breathing in BC dogs. Use of CT volumetry to identify tongue fat deposits may permit targeted surgical reduction of tongue volume in BC dogs and contribute substantially to treatment of BOAS., (© 2023 American College of Veterinary Radiology.)

While traditionally in the realm of otorhinolaryngology or oral maxillofacial surgery, conditions involving the tongue may also be managed by plastic surgeons. The authors present an unusual case of acquired macroglossia resulting from angiotensin-converting enzyme inhibitor-induced angioedema and review the literature to discuss its surgical management from a plastic surgery perspective. A 62-year-old female suffered severe airway obstruction, respiratory arrest, and anoxic brain injury from angioedema-associated macroglossia. After tracheostomy, the patient was managed nonsurgically, with bite wound care and medications to minimize angioedema to marginal effect. Ultimately, a partial glossectomy was planned. The edematous distal tongue was amputated and closed primarily. On postoperative day 2, she was successfully weaned off mechanical ventilation and no longer suffers trauma from tongue biting. The simple anterior tongue resection described in this paper was an appropriate approach for our patient. More research is needed to guide plastic surgeons in an optimal approach for clinical scenarios., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by Mutaz B. Habal, MD.)

Purpose: Idiopathic macroglossia is a rare entity of true tongue enlargement without an underlying etiology. There are only a few case reports on the diagnosis and management of idiopathic macroglossia. This study's purpose was to present a series of patients with idiopathic macroglossia and suggest a treatment algorithm., Methods: This was a retrospective case series of a cohort of patients with macroglossia who were treated by the Oral and Maxillofacial Surgery service at the University of Texas Health Science Center at Houston (UTHealth)and Emory University. The patient's medical comorbidities, history of present illness, clinical presentation, radiographic findings, and disease management were studied. The outcome variables include normalization of the tongue size, dependence on parenteral nutrition, and tolerating tracheostomy decannulation., Results: Five patients with a mean age of 45 years were included in the study. All of the patients (n = 5, 100%) in our cohort developed macroglossia following prolonged oral intubation, with 3.5 weeks being the average length of intubation. All patients presented with difficulty feeding orally and breathing. The average tongue dimension was 12.20 x 6.25 cm. All tongue enlargements were located in the anterior 2/3 of the tongue, and all patients had displaced anterior dentition. In addition, 60% of the patients (n = 3) experienced altered tongue sensation (pain and/or decreased taste). These patients were surgically managed with tracheostomy and percutaneous endoscopic gastrostomy (PEG) tube placement followed by partial glossectomy (n = 5, 100%). We defined successful outcomes as 1) modifying the tongue to a functional, nonprotruding form, 2) tracheostomy decannulation and 3) PEG tube removal. Tracheostomy decannulation and PEG tube removal were achieved in 80% of the patients (n = 4)., Conclusions: In this patient cohort, we were unable to identify the cause of the pathology based on existing clinical data. When the etiology is unclear or irreversible, management should involve tracheostomy and surgical feeding access for the initial stabilization, followed by modified glossectomy to improve form, function, and cosmesis thereby improving the overall quality of life., (Copyright © 2022 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

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.

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.

Introduction: Macroglossia is a term used to describe a large tongue which protrudes outside of the mouth while in a resting position (Balaji, 2013). It is a cardinal sign in children with Beckwith-Wiedemann syndrome and can also be found in children with Down syndrome and Klippel-Trenaunay-Weber syndrome. Macroglossia can lead to airway and feeding difficulties, as well as problems with speech, drooling, and cosmesis. We present a review of tongue reduction operations performed for macroglossia over a 10-year period in Northern Ireland., Methods: We performed a retrospective review of the medical notes of those children identified to have undergone a tongue reduction procedure in the regional pediatric hospital. We reviewed the presenting symptoms and concerns, the operative technique used, postoperative outcomes, and follow up. Outcomes data included improvements in symptoms, complications, and the need for revision procedures., Results: Six children underwent tongue reduction procedures over a 10-year period. Age range at time of surgery was between 4 months to 10 years 3 months. Five children had an underlying diagnosis of Beckwith-Wiedemann syndrome and 1 child had Down syndrome. One child underwent a second tongue reduction for mild tongue protrusion at the 5-year follow up. There were no complications in relation to tongue reduction surgery for any of the children and importantly, there were no airway complications in our series. All patients were found to have improved feeding, better tongue position in the oral cavity, reduced drooling, and better speech development following surgery., Conclusion: Symptomatic macroglossia requiring a tongue reduction procedure is relatively rare and these procedures are, therefore, uncommonly performed. Despite the rarity of this procedure, when it is required, it can be life saving for some infants and children, and life altering for the remainder. Improvements in airway, feeding, speech, and psychosocial wellbeing are the desired outcomes with this procedure. Throughout our 10-year series we have found it to be a relatively safe procedure but potentially anesthetically challenging. We have demonstrated both good short and long-term outcomes for these children., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Introduction: Postoperative management of patients with the congenital growth disorder Beckwith-Wiedemann syndrome (BWS) can be complicated. The main clinical manifestations of the syndrome are macroglossia - which may hamper airway management -, prematurity, hemihypertrophy, omphalocele, embryonal tumours and episodes of neonatal hypoglycaemia., Objective: Our main objective is to describe the perioperative management and potential anaesthetic complications in paediatric patients with BWS undergoing glossectomy., Methods: Case report and literature review., Results: We describe the case of an 11-month-old patient diagnosed with BWS who underwent reduction glossoplasty. We performed a comprehensive preoperative evaluation, taking into account potential anaesthetic complications derived from both macroglossia and prematurity, and the risk of hypoglycaemia. The procedure was performed under general anaesthesia. Intubation - performed according to difficult airway management algorithms - was uneventful and the patient was successfully extubated in the operating room. The patient remained stable during the postoperative period, with good respiratory dynamics, SatO 2 >96% and good glycaemic control. Oral intake was started 4hours after surgery, and she was discharged to the ward at 24hours., Conclusion: BWS patients require a multimodal approach that includes detailed preoperative planning and knowledge of potential airway-related and systemic complications., (Copyright © 2020 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Publicado por Elsevier España, S.L.U. All rights reserved.)

Aim: This study compares long-term mandibular growth between a group of Beckwith-Wiedemann Syndrome (BWS) patients who underwent glossectomy at an early age and a group of patients not operated., Methods: Cephalometric measurements were performed in BWS patients comparing the data obtained between a group of patients operated at an early age and a group of non-operated patients who declined surgery. Statistics included independent sample T-test., Results: Twenty-four out of 78 BWS patients followed since birth completed longitudinal cephalometric x-rays at age 5, 10 and 15. Eighteen patients needed early surgery. Eleven families accepted glossectomy at 2.3 ± 1.3 years of age; seven declined surgery. No differences in mandibular growth were found between the two groups. Inclination of maxillary incisors results were statistically greater in the non-operated group (operated compared to the non-operated group: 103.58 ± 11.30 Vs 108.98 ± 12.47; p-value 0.0168 at 5; 107.06 ± 7.98 Vs 115.14 ± 7.05; p-value 0.0206 at 10; 109.80 ± 4.68 Vs 116.75 ± 5.28; p-value 0.0233 at 15)., Conclusion: Macroglossia has no role in the post-natal mandibular overgrowth in BWS and mandibular overgrowth is part of the syndrome. Therefore, early glossectomy does not change mandibular growth and does not prevent the development of class III skeletal malocclusion in these patients., (Copyright © 2020 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Background: Macroglossia, a cardinal feature of the (epi)genetic disorder Beckwith-Wiedemann syndrome, is associated with obstructive sleep apnea, speech and/or feeding difficulties, and dental or jaw malalignment. These sequelae may be treated and/or prevented with tongue reduction surgery; the authors sought to determine whether certain Beckwith-Wiedemann syndrome patients may benefit from early surgical intervention before age 12 months., Methods: The authors conducted a retrospective review of patients with Beckwith-Wiedemann syndrome who underwent tongue reduction from 2014 to 2019. The authors assessed primary outcomes of change in obstructive sleep apnea by polysomnography, respiratory support required, and feeding route before and after tongue reduction, and reviewed postoperative complications and the need for repeated tongue reduction., Results: Of the 36 patients included, the median age at tongue reduction was 9.5 months (interquartile range, 3.8 to 22.8 months). For those with severe obstructive sleep apnea, there was a significant reduction in the obstructive apnea hypopnea index from 30.9 ± 21.8 per hour to 10.0 ± 18.3 per hour (p =0.019) and improvement in nadir oxyhemoglobin saturation from 72 ± 10 percent to 83 ± 6 percent (p =0.008). Although there was no significant change in overall supplemental feeding tube or respiratory support, there were specific patients who experienced clinically meaningful improvement. Of note, these positive outcomes applied equally to those who underwent surgery at a younger age (<12 months). To date, only one patient required a repeated tongue reduction., Conclusion: Based on improved polysomnographic findings and rarity of surgical complications or repeated surgery, the authors' data support the safety and efficacy of this early intervention when clinical indications are present and an experienced multidisciplinary team is available for consultation., Clinical Question/level of Evidence: Therapeutic, IV.

Beckwith-Wiedemann syndrome is a rare congenital overgrowth disorder with macroglossia being one of the cardinal symptoms. In pronounced cases, macroglossia can lead to airway obstruction, musculoskeletal alterations and functional deficits. Surgical tongue reduction is performed at varying ages and with different techniques. This study evaluated perioperative complications, as well as long-term aesthetic and functional outcomes, in a large cohort. A total of 68 patients, treated either surgically or conservatively, were included. Depending on the severity of macroglossia, patients were divided into three groups to determine the treatment algorithm. Complications after surgical tongue reduction were prolonged intubation and revision due to dehiscence or haematoma. In the long term, no patient suffered from impaired sense of taste or paresthesia, although the shape of the tongue was disproportional in 85%. With the present treatment algorithm, operative tongue reduction exerts a positive influence on skeletal, dentoalveolar and functional development with sufficient long-term outcome and high grade of satisfaction of the patients. Supportive therapy in an interdisciplinary centre is of fundamental importance for both surgical and conservative treatment., (Copyright © 2018 International Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Objective: We describe here a new surgical technique, which allows for efficient tongue reduction with satisfactory appearance and avoids the complications of tip excision., Study Design: This is a retrospective case review that includes 6 patients who had macroglossia and underwent tongue reduction using our new central V-shaped excision technique. Three patients were operated on at an early age because of significant macroglossia with permanent tongue protrusion, cosmetic disorder, and speech delay. The other 3 patients underwent surgery performed in their teenage years because medical treatment had been unsuccessful. We assessed tongue shape, mobility, taste, and sensitivity., Results: The postoperative follow-up period ranged from 2 months to 16 years (average 7 years). On subjective evaluation, all patients were satisfied with the appearance of the tongue and reported no sensitive or sensory difficulties. The patients reported normal food progression in the oral cavity. Objective evaluation showed that the tongue had normal mobility, taste, and sensitivity. Postoperatively, there was no residual tongue protrusion, cosmetic disorder, or speech delay. None of the patients needed revision surgery., Conclusions: This central V-shaped excision offers good surgical results, with efficient tongue reduction and none of the complications of other tongue reduction techniques, such as tip amputation or anterior wedge resection., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Beckwith-Wiedemann syndrome is a rare congenital condition, characterized by presenting macroglossia, defects of the abdominal wall, hemihypertrophy, omphalocele, neonatal hypoglycemia, umbilical hernia, hepatomegaly, cardiac abnormalities, among others. Macroglossia occurs in 90% of cases, causing a problem in chewing, swallowing, phonation and breathing, resulting in a closure of the upper airway. The therapeutic option of choice is partial glossectomy. We present a 2-month-old pediatric patient with Beckwith-Wiedemann syndrome and area blockage due to severe macroglossia; in the medical history, congenital heart disease, interatrial communication, persistent ductus arteriosus, symptomatic epilepsy, renal failure, hypoglycemia, tracheotomy and gastrostomy, due to airway collapse and dysphagia. It was performed an anterior tongue reduction surgery as a surgical treatment with favorable results., (Sociedad Argentina de Pediatría.)

Objectives: To examine the frequency of partial glossectomy performed for the indication of macroglossia in children within the United States, assessing for differences in rates of intervention across various demographics. To identify potential morbidities associated with partial glossectomy in this population and determine how such factors may influence length of stay and cost of admission following tongue reduction surgery., Study Design: Retrospective cross-sectional study., Setting: The Kids' Inpatient Database 2003, 2006, 2009, and 2012., Subjects: Patients under age 5 diagnosed with macroglossia who underwent partial glossectomy., Methods: Demographics were analyzed and cross tabulations, linear regression modeling, and multivariate analysis were performed., Results: During the four-years studied, partial glossectomy was performed in 196 children under age 5 with macroglossia. A disproportionately higher rate of intervention was seen in white children (p = 0.001), patients undergoing surgery in the mid-west (p < 0.001) and patients in the highest socioeconomic quartile (p = 0.015). Most patients underwent glossectomy in their second year of life. The average length of stay in patients who underwent partial glossectomy for macroglossia was 9.59 days (Range 1-211 days, median 3.45 days) and the average cost was $56,602 (median $16,330)., Conclusion: Partial glossectomy for macroglossia is typically performed prior to age 2 in the United States. A higher rate of intervention is seen in white children, those who have surgery in the mid-west and affluent children even when controlling for confounding variables., Level of Evidence: III., (Copyright © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.)

Macroglossia is classified as true macroglossia, which exhibits abnormal histology with clinical findings, and relative macroglossia, in which normal histology does not correlate with pathologic enlargement. This report describes an atypical case of morbidity with massive macroglossia secondary to myxedema; the macroglossia enlarged over a 3-month period before being presented to the Department of Oral and Maxillofacial Surgery, University of Texas Health Science Center at Houston (Houston, TX). Substantial enlargement of the tongue (16 cm long × 10 cm wide) was first attributed to angioedema, which was refractory to the discontinuation of lisinopril and a C1 esterase inhibitor. A core tongue biopsy examination was performed to rule out angioedema, amyloidosis, myxedema, and idiopathic muscular hypertrophy. Interstitial tissue was positive for Alcian blue and weakly positive for colloidal iron, which are correlated with hypothyroidism and a diagnosis of myxedema. However, the macroglossia did not resolve after correcting for hypothyroidism. The patient required a wedge glossectomy for definitive treatment. She recovered unremarkably, with excellent cosmesis and preservation of lingual and hypoglossal function. There are some case reports of massive macroglossia but none with myxedema as the primary etiology., (Copyright © 2017 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Beckwith-Wiedemann syndrome (BWS) is a rare paediatric overgrowth disorder. Associated macroglossia is a feature of many children with BWS and is felt to be a risk factor for obstructive sleep apnoea (OSA). Sleep-disordered breathing is highly variable in this population. The relationship between degree of macroglossia or other genotypic or phenotypic factors and OSA severity has not been established. The natural history of OSA in this population is unknown; a variety of conservative and surgical therapies have been used to treat OSA in children with BWS but none have been studied systematically. Tongue reduction is the mainstay of surgical therapy for macroglossia associated with BWS, but limited data are available regarding its efficacy in treating OSA or its effect on speech and swallowing. More research is needed to better identify which children with BWS are at risk for OSA and the most effective treatment for these patients., (Copyright © 2017. Published by Elsevier Ltd.)

Lymphatic malformations are rare slow-flow vascular malformations, with high tendency to appear in the head and neck region. The treatment of these lesions ranges from follow-up to sclerosing agent injection to surgical excision. The authors present a case of a new born with large extensive lingual and submandibular lymphatic malformation, for which the patient underwent tracheostomy and gastrostomy insertion. He was then treated successfully with sclerosing agent injections followed by surgical excision, with 7 years follow-up. The second case presented is a two and a half baby with large lingual lymphatic malformation, treated successfully with doxycycline injections followed by intraoral excision of the lesion. Pathology of the excised lesion is then demonstrated, which shows for the first time the different layers affected by the sclerosing agent.

The macroglossia is a rare condition, congenital or acquired, characterized by hypertrophy of the lingual muscles that can cause both aesthetic and functional changes such as mandibular prognathism and malocclusion. Diagnosis is through clinical examination. Treatment consists of excision of part of the tongue and different surgical techniques have been described in the literature. The keyhole lingual resection technique has shown satisfactory results in reducing the volume and preservation of the neurovascular bundles of the tongue. This work aims to present a clinical report of true macroglossia associated with dental-skeletal discrepancies, submitted to partial glossectomy previously to orthognathic surgery.

Unlabelled: Lipomas are the most common benign soft tissue mesenchymal tumours composed of mature adipose tissue. They are uncommon in the oral and maxillofacial regions, with 15-20 % of cases involving the head and neck region and less than 5% of all benign oral lesions. Multiple symmetric lipomatosis is rare and characterized by diffuse growth and nonencapsulated lipomas. It is usually found in the posterior neck and upper trunk and they are relatively infrequent on the oral and maxillofacial regions like Madelung disease. In the report, we describe a rare case of symmetrical lipomatosis of tongue with OSAS and Dysartria. This lesions were resected under general anesthesia. Intraoperative findings revealed only adipose tissues with replacement of lingual muscles and no capsulation. The lesion was finally diagnosed as symmetric lipomatosis of the tongue based on clinical radiological and histologic examination. SLT (Symmetrical lipomatosis of the tongue) is an extremely rare case that appears like a macroglossia. Partial glossectomy is the treatment of choice because of the improvement of symptoms and the low rate of recurrence., Key Words: Macroglossia, Oral lipoma, Tongue lipomatosis.

Beckwith-Wiedemann syndrome (BWS) is an overgrowth disorder in which macroglossia is one of the main signs. We investigated the long-term outcome of tongue surgery reduction (TRS) on taste and speech in patients with BWS who were more than 5 years of age and had undergone surgical anterior wedge resection of the tongue. A questionnaire was used to assess medical history and to determine some aspects of speech, taste, psychological well-being, and degree of satisfaction with regard to TRS and tongue mobility. Speech sound error pattern and degree of intelligibility were measured by a speech therapist, and taste was assessed using a validated test. The degree of both intelligibility and satisfaction with the surgery was high. There were some speech errors; especially the interdental 's', addental 't', and addental 'd' were more noticed. We conclude that anterior wedge resection is an effective technique to treat macroglossia in children with BWS, and that it has no long-term consequences for intelligibility and taste perception and only limited consequences for speech., (Copyright © 2016. Published by Elsevier Ltd.)

The tongue is a vital human organ that needs to be checked before beginning a dental treatment, especially one in orthodontics or sleep apnea. The size of the tongue is an issue because there is not yet a clear definition of what a normal-sized tongue is. This leads to further problems in defining macroglossia. The current article aims to define macroglossia and bringforth more information about the two different types: pseudo macroglossia and true macroglossia. It further discusses treatment solutions available, such as partial glossectomies, coblation technique, and radiofrequency waves treatment.

The authors report a case of amyloidosis-induced macroglossia treated with surgical reduction of the tongue using a keyhole to inverted T method with particular emphasis on the postoperative sequelae. Significant tongue swelling persisted for longer than anticipated requiring tracheostomy to remain in situ for 14 days., (2016 BMJ Publishing Group Ltd.)

Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder characterised by abdominal wall defects, macroglossia and somatic gigantism. A number of associated features, including gastrointestinal and urinary tract polyps, have been described, but there are no previous reports of oral polyps occurring in this syndrome. We describe the first case of BWS presenting with an oral polyp. Clinicians should be alert to the possibility of BWS if other features of the syndrome are present, in children with oral polyps., (2015 BMJ Publishing Group Ltd.)

Purpose of Review: To discuss the current surgical management of macroglossia., Recent Findings: Traditional surgical management of severe macroglossia has been with anterior wedge or keyhole resection. Long-term follow-up has been limited, and only recently have assessments been done regarding functional and aesthetic outcomes. New methods including double stellate and combination approaches have shown promise, though with limited case size reports. Addressing macroglossia in three dimensions may be the most effective way of achieving positive positional, speech and aesthetic outcomes, but comparative studies are lacking. Other causes of macroglossia, such as vascular malformations, can be managed with less aggressive measures such as laser and radio-frequency ablation., Summary: The aggressiveness of the management should match the severity of the symptoms. The anterior wedge resection and modified keyhole incisions are the most well studied operative strategies. Short and long-term outcome data are limited, and neither method is definitively superior. Less aggressive measures are options for less severe macroglossia. Surgical management of macroglossia should be tailored to each individual patient and in accordance to surgeon experience and expertise.

Macroglossia is a rare condition, but can severely affect the oral and maxillofacial region. Angioedema is a condition resulting from multiple mechanisms, all of which can result in macroglossia. This report describes an unusual case of acquired macroglossia in an adult resulting from chronic edema secondary to angioedema in the setting of stroke. The patient had a morbidly enlarged tongue and presented with clinical signs and symptoms consistent with massive macroglossia. She required surgical intervention for acute management of her symptoms and definitive treatment of her macroglossia., (Copyright © 2015 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Introduction: A reduction glossectomy may be complicated by tongue and mouth floor edema and extend the recovery time for a normal tongue function. We performed reduction glossectomy using Ultracision(®), an ultrasonic vibrating device, so as to limit these complications., Technical Note: We performed a keyhole glossoplasty under general anesthesia. The initial tongue incision was performed with a cold scalpel, then the glossectomy was continued with Ultracision(®) only. We also used CURVED SHEARS(®). We evaluated our preliminary results with 3 patients presenting with Wiedemann-Beckwith syndrome., Conclusion: Ultracision(®) is a useful tool for intra-oral surgery, and should soon be more frequently used. It is an alternative to electrocautery for this type of surgery., (Copyright © 2014 Elsevier Masson SAS. All rights reserved.)

Beckwith-Wiedemann syndrome (BWS) is a congenital growth disorder. Children born with BWS develop enlarged organs, including the tongue, a large body, and other signs. A woman with BWS was treated and followed for 30 years. Treatment consisted of tongue reduction, orthopedic and orthodontic treatment, orthognathic surgery, and retention. The patient was first treated when she was 5 years old. Her original orthodontic problems included macroglossia, anterior open bite, anterior crossbite, and a skeletal Class III jaw relationship caused by significant mandibular protrusion. The jaw-base relationships did not improve in the early preadolescent period after phase 1 of orthodontic treatment with a vertical chincap. With the growth spurt accompanying puberty, she developed a severe skeletal Class III jaw relationship and a constricted maxillary arch. Surgically assisted rapid maxillary expansion was performed at 23 years of age to correct the severe discrepancy between the maxillary and mandibular dental arch widths. Then, at 26 years, a LeFort I osteotomy, a horseshoe osteotomy, a bilateral sagittal split ramus osteotomy, and genioplasty were performed after presurgical orthodontic treatment with extraction of the mandibular first molars. Both the facial profile and the occlusion were stable after 6 years of retention. This case report discusses the result of long-term observation of a patient with BWS who underwent tongue reduction, early orthodontic treatment, and surgical-orthodontic treatment., (Copyright © 2014 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.)

Lymphangiomas are rare congenital malformations which are commonly seen in the head and neck region. The disease can be histologically differentiated from other vascular disorders such as cavernous or capillary hemangioma with the lymphatic endothelium-lined cystic spaces. The onset of lymphangiomas are either at birth (60 to 70%) or up to two years of age (90%). It is rare in adults. The therapeutic strategy is mainly based on the surgical removal of the lesion. The risk of recurrence is high in incomplete removal of the lesion. In this article, we discuss the major clinical manifestations, disease-related impairments and dental problems which patients may face as well as treatment options for lymphangioma of the tongue. Within this context, we present four cases of lymphangioma of tongue, including two with a giant macroglossia leading problems in dentition and related dental problems.

Objective: To investigate the efficacy of Coblation-channelling for the tongue(CCT) treatment of tongue hypertrophy., Method: The 31 patients with severe obstructive sleep apnea hypopnea syndrome (OSAHS) combining tongue hypertrophy staged as Friedman ll or N were performed CCT after nasal septum surgery, coblation channelling of bilateral inferior turbinate and coblation-assisted UPPP. While the vertical channelling in the base and the body of the tongue, the tilted one parallel side edge and posterior to the tongue were combined. The intraoperative and postoperative complications such as bleeding were observed. The 12 months postoperative follow-up were done by MRI of the tongue., Result: The retrolingual space were expanded postoperatively. The 2 bleedings occurred immediately after channellings in the tongue body, and they were stopped by local compression. No postoperative tongue paralysis, no tongue hematoma and abscess happened. The tongue sizes changed from preoperative III or IV degree to postoperative I to II degrees. One case received three CCT sessions., Conclusion: CCT treatment tongue hypertrophy is a individually safe, effective and minimally invasive treatment.

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.

We report sensory changes after tongue reduction by the Harada-Enomoto method for macroglossia in a 20-year-old woman with Beckwith-Wiedemann syndrome. Sensory tests were performed before surgery and 1 week and 2 months after surgery. We assessed the static tactile threshold, vibration sense, static 2-point discrimination, pain threshold, and taste. No sensory loss of any category tested was observed after tongue reduction., (Copyright © 2014 Elsevier Inc. All rights reserved.)

This article describes the complex orthodontic treatment of a 22-year-old patient with Duchenne muscular dystrophy and macroglossia. His orthodontic treatment hinged on providing proper informed consent and management of the malocclusion with glossectomy, extractions, fixed appliances, and elastics. Challenges to traditional treatment are outlined, and compromises to both process and outcome are discussed from an informed consent point of view because of the serious risks involved. The treatment objectives were met, and the outcome was considered a success., (Copyright © 2013 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.)

A young man, 19 years of age, with the chief complaint of an anterior open bite, came for orthodontic treatment with a skeletal Class I relationship, anterior open bite, shortened maxillary incisor roots, and relative macroglossia. The malocclusion was treated by extracting the maxillary first premolars and using a fixed edgewise appliance. A partial glossectomy was performed before the orthognathic surgery with a 3-piece segmental LeFort I mandibular setback, and advancement was achieved with a reduction genioplasty. A functional and esthetic occlusion with an improved facial profile was established, and the apex of the maxillary left central incisor became slightly rounded after prolonged and significant tooth movement. Four years after treatment, there was occlusal stability of the results, and no further root shortening was observed., (Copyright © 2013 American Association of Orthodontists. Published by Mosby, Inc. All rights reserved.)

Amyloidosis is a disease characterised by the deposition in body tissues of amyloid: abnormal protein in a beta pleated sheet formation. It is a systemic disorder and macroglossia may be seen in all forms. Changes to the normal architecture of the tissues and systemic features of the disease and its underlying cause can complicate the surgical management of the enlarged tongue., (Copyright © 2012 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Background: Macroglossia is the commonest symptom of Beckwith-Wiedemann syndrome (BWS) and sometimes requires surgical tongue reduction for cosmetic, feeding, drooling and speech problems., Methods: We retrospectively reviewed the perioperative course of 14 BWS patients. The subjects were children who underwent tongue reduction surgery or glossopexy between 1994 and 2008 at Osaka Medical Center for Maternal & Child Health., Results: The median age was 18 months, and the median weight was 12.2 kg at the time of surgery. One patient had the trachea intubated and another had tracheostomy to keep airway patency. Other 12 patients had no artificial airway and were premedicated with midazolam or diazepam and had the trachea intubated after induction with sevoflurane and nitrous oxide in oxygen. Only one patient developed difficult mask ventilation for which a nasal airway was applied. No patients demonstrated difficult intubation. All without preoperative artificial airway were extubated in the OR after the surgery. One patient demonstrated hypoglycemia. Airway compromise in the two patients who was intubated or had tracheostomy prior to surgery was not alleviated by the surgery., Conclusions: Airway disorder was not alleviated by tongue reduction surgery or glossopexy.