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1/16. tongue reduction in Beckwith-Weidemann syndrome.

    OBJECTIVE: To review our experience with patients with macroglossia as a component of Beckwith-Weidemann Syndrome (BWS). DESIGN: Chart review of six patients treated with BWS. SETTING: Tertiary care teaching hospital. patients: Six patients diagnosed with BWS and macroglossia. INTERVENTIONS: Four patients underwent at least one surgical procedure to address their macroglossia. The surgical options and potential complications are discussed. RESULTS: Three patients who have undergone tongue reduction have a functioning tongue with normal mobility. Two patients have required tracheotomy as apart of their management and still have significant tongue enlargement. CONCLUSIONS: macroglossia as a part of BWS may present a difficult management problem. Various methods of tongue reductions have been reported with mixed results.
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2/16. Long-term changes in dentoskeletal pattern in a case with beckwith-wiedemann syndrome following tongue reduction and orthodontic treatment.

    Long-term changes in the dentoskeletal pattern in a 6-year-old Japanese girl with beckwith-wiedemann syndrome were demonstrated. The patient showed macroglossia, which is the most common symptom of the syndrome, protruded lower lip, mandibular protrusion and anterior open bite. The jaw base relationship improved to skeletal Class I and the molar relationship to Angle Class I at the early preadolescent period following tongue reduction and phase I orthodontic treatment using a chin cap and tongue crib. Optimum intercuspation of teeth was achieved after edgewise treatment without orthognathic surgery, and a skeletal Class I apical base relationship and good facial profile were maintained after the retention period of 2 years. This case report suggests that early orthodontic treatment with tongue reduction can be effective in a case with beckwith-wiedemann syndrome to improve an abnormal dentoskeletal pattern.
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3/16. Sudden death in an 8-week-old infant with beckwith-wiedemann syndrome.

    The authors report a case of a 2-month-old girl diagnosed with beckwith-wiedemann syndrome (BWS) who was born prematurely and died suddenly in the hospital just before being discharged. BWS is a malformation syndrome associated with an increased risk of childhood tumors. The major features of BWS are macroglossia, abdominal wall defects, and visceromegaly, frequently leading to premature birth. Due to complex inheritance patterns, a predominance of nonfamilial cases, and the variability in expression of the features (termed incomplete penetrance), the risk of delayed diagnosis is evident. Secondary to hyperplastic pancreatic islands, hypoglycemia occurs frequently, and if not anticipated, adequate measures for prevention of hypoglycemic episodes may be delayed, resulting in possible intellectual deficits. The infant presented here died of natural causes: immaturity of the lungs resulting in marginal respiratory function and compounded by increased risk for asphyxia secondary to the enlarged tongue. The clinical history and findings in this infant are discussed in respect to the genetic syndrome with their relevance to medicolegal examination and the causes and manner of death.
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4/16. A less-invasive approach with orthodontic treatment in Beckwith-Wiedemann patients.

    The beckwith-wiedemann syndrome (BWS) is a rare genetic disorder, linked to an alteration on the short arm of chromosome 11 that comprises multiple congenital anomalies. macroglossia is the predominant finding, with subsequent protrusion of dentoalveolar structures, which results in a protruding mandible, anterior open bite, abnormally obtuse gonial angle and increased mandibular length. A less-invasive treatment with orthopaedic appliances in a patient with early tongue reduction is presented. This work summarizes the oral signs linked to macroglossia, and highlights the influence of macroglossia on mandibular growth structures. In our opinion, glossotomy could be carried out in the paediatric patient as a preventive measure in that it curbs the tongue's influence on skeletal growth and dramatically reduces the duration and extensiveness of subsequent treatment.
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5/16. 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.
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6/16. cleft palate and beckwith-wiedemann syndrome.

    OBJECTIVE: patients with beckwith-wiedemann syndrome suffer numerous anomalies, which vary somewhat from case to case. cleft palate in combination with this syndrome has rarely been reported in the literature. Through two cases, this report examines the staging of the surgical repairs and the role of macroglossia in cleft palate and the consequences of the scarred palate on mandibular development. RESULTS: Of four patients with beckwith-wiedemann syndrome, only two had a cleft palate. The timing of the repair in these two children was different. speech development was satisfactory in the first case but mediocre in the second. This result seemed to be related to a poor social environment. Mandibular prognathism persisted in both cases. CONCLUSION: The treatment of patients with cleft palate and beckwith-wiedemann syndrome remains complex. It is preferable not to operate on a cleft palate before performing a tongue reduction plasty, but rather to combine these two surgical interventions. This would reduce the risks of anesthesia and enable the palate to heal more efficiently. Surgical treatment should be performed after the age of 6 months and before problems in speech development occur. An orthognathic surgery at adolescence could be performed if prognathism persists. While the origin of the cleft palate is still being discussed, we cannot claim that macroglossia is related to the development of cleft palate, nor that the scarred palate has an impact on the mandibular development.
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7/16. Assessment of obstructive apnea by using polysomnography and surgical treatment in patients with beckwith-wiedemann syndrome.

    PURPOSE: Obstructive apnea is sometimes seen in patients with Beckwith-Wiedemann syndrome. The cause of apnea is not limited to macroglossia, and the surgical indication for obstructive apnea has not yet been established. The authors performed polysomnography for the assessment of apnea. METHOD: Overnight polysomnograms were obtained in 2 patients who developed obstructive apnea after 1-stage repair for omphalocele. CASE 1: apnea index (AI), defined as apneic events per hour, indicated 17.3, and SpO2 below 95% occupied 80% of the total sleep time. Computed tomography and magnetic resonance imaging indicated obstruction of the airway between macroglossia and the hypopharynx. Central tongue resection and the division of the frenulum linguae for associated ankyloglossia were performed 97 days after birth. One month after surgery, apneic events disappeared and SpO2 below 95% occupied only 1% of the total sleep time. CASE 2: Obstructive AI indicated 28.1. Division of the frenulum linguae and anterior glossopexy were performed 55 days after birth. Postoperative polysomnogram indicated a marked reduction of AI. CONCLUSIONS: These results indicated that polysomnography was useful for evaluating obstructive apnea and that advancement of the tongue by division of the frenulum linguae may be recommended for the treatment of obstructive apnea in patients with beckwith-wiedemann syndrome.
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8/16. 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.
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9/16. loss of heterozygosity in non-tumoral tissue in two children with beckwith-wiedemann syndrome.

    The insulin-like growth factor ii (IGF-II) gene has been analysed by Southern blotting in healthy and tumor tissue in two children with beckwith-wiedemann syndrome, using the enzymes, AvaII and SacI. loss of heterozygosity was determined not only in the dna of a nephroblastoma, but also in the healthy kidney, leukocytes and fragments of tongue removed for obstructive macroglossia. Allele loss therefore seems to be sufficient to provoke tumorigenesis in some tissues, but not in others.
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10/16. macroglossia as a presentation of the beckwith-wiedemann syndrome.

    Plastic surgeons are not infrequently required to reduce the size of a large tongue in a child. macroglossia is one of the main presenting features of the beckwith-wiedemann syndrome. This comprises a spectrum of disorders that includes a high incidence of malignancy. patients with this syndrome should be identified and carefully assessed to exclude serious complications. This paper reports a series of 30 patients presenting to the Royal Children's Hospital over an 8-year period. An incidence of malignancy of 10 percent is recorded.
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