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11/16. The beckwith-wiedemann syndrome: a longitudinal study of the macroglossia and dentofacial complex.

    case reports provide insights into fundamental mechanisms and also assist clinicians in treatment of similarly affected patients [Pruzansky, 1976]. The present investigation examines the natural history of the macroglossia associated with a case of beckwith-wiedemann syndrome (BWS) and its influence on dentofacial development. Facial skeletal growth and tongue size were assessed by analyzing cephalometric radiographs from age 2 months to 7.5 years. The data were compared with cephalometric norms and new normative data derived from 13 patients with cleft lip. The major influence of the macroglossia was protrusion of dentoalveolar structures, particularly in the lower jaw. This resulted in an anterior cross-bite in the primary dentition. In addition, an abnormally obtuse gonial angle was observed increasing the effective length of the mandible. tongue size in BWS was generally greater than the norm, but the increase with age paralleled the mean growth curve of the tongue in the control. Over time the base of the tongue became longer and the hyoid bone moved posteriorly and inferiorly, allowing for accommodation of the tongue within the oral cavity. The changes in tongue shape and dentofacial morphology support the position that early partial glossectomy should be delayed or abandoned. In cases where tongue reduction is considered necessary, the new cephalometric normative data on tongue size provided herein can be used to establish objective criteria for such surgery.
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12/16. Early tongue resection for Beckwith-Wiedemann macroglossia.

    dentists are aware of the extraordinary influence an active thrusting tongue can have on dental development. In the presence of the profound muscle hypertrophy characteristic of macroglossia in the beckwith-wiedemann syndrome, a compelling need exists for surgical reduction of the tongue as early in life as possible. This report describes our clinical experience with 3 children, 1 referred late and the other 2 treated within the first year of life.
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ranking = 0.85714285714286
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13/16. trisomy 11p15 and beckwith-wiedemann syndrome. A report of two cases.

    Two patients with trisomy 11p15 and features of beckwith-wiedemann syndrome are reported. The first is a female infant with gigantism, macroglossia, abdominal hypotonia with umbilical hernia, moderate mental retardation, malformative uropathy, and atrial septal defect. trisomy 11p15 was due to de novo duplication. The second patient was a stillborn (32-33 weeks pregnancy) with an abnormal tongue, posterior diaphragmatic eventration, inner organ congestion mainly of the adrenals. trisomy 11p15 was due to a t(4;11)(q33;p14)pat. The association of trisomy 11p15 and beckwith-wiedemann syndrome is discussed with regard to cytogenetic data and the gene content of 11p, notably the genes coding for insulin and predisposition to Wilms tumour.
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ranking = 0.14285714285714
keywords = tongue
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14/16. head and neck manifestations of beckwith-wiedemann syndrome.

    beckwith-wiedemann syndrome is a congenital disorder manifested by organomegaly, omphalocele, hypoglycemia, and macroglossia. We have found a significant number of these children to be at risk for upper airway obstruction during infancy or childhood. In this review of 13 children, 2 required tracheotomy during infancy for cor pulmonale caused by macroglossia. Seven of nine children older than 1 year required tonsillectomy and adenoidectomy to relieve upper airway obstruction. Although macroglossia can be a cause of airway obstruction in infants with beckwith-wiedemann syndrome, we have found that airway obstruction during childhood is related to tonsillar and adenoidal hypertrophy and not to macroglossia. Anterior tongue reduction is reserved for the correction of malocclusion, articulation errors, or cosmesis, whereas tonsillectomy and adenoidectomy may be curative of obstructive symptoms.
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ranking = 0.14285714285714
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15/16. Central tongue reduction for macroglossia.

    Unhappy with our postoperative results from standard tongue reduction methods, we have developed a central tongue reduction technique. This technique allows for alteration of the tongue base as well as tongue height and width while minimizing postoperative scarring. Although the technique and its associated surgical complications are in some ways comparable with the standard tongue reduction methods, our postoperative polysomnographic and tongue functioning results are improved. It is noteworthy that none of our patients had worse speech postoperatively and that our one down syndrome patient had improved speech. Our indication for central tongue reduction is macroglossia of any sort in appropriate patients.
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ranking = 1.5714285714286
keywords = tongue
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16/16. The anaesthetic management of a case of Kawasaki's disease (mucocutaneous lymph node syndrome) and Beckwith-Weidemann syndrome presenting with a bleeding tongue.

    An unusual case of a 13-month-old child with Kawasaki's disease and the Beckwith-Weidemann syndrome is presented. The child, while anticoagulated with warfarin and aspirin to prevent extension of a coronary artery thrombus, fell and lacerated the tongue resulting in haemorrhage and significant swelling. The ongoing haemorrhage, combined with difficulty in securing venous access resulted in the child becoming shocked. Surgical intervention was required to stem the haemorrhage. The anaesthetic management of a shocked child with a coronary artery aneurysm and thrombosis, a potentially difficult airway and a full stomach is described.
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ranking = 0.71428571428571
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