1/6. Anesthetic considerations of two sisters with beckwith-wiedemann syndrome.Anesthetic considerations of 21-mo-old and 4-yr-old sisters with beckwith-wiedemann syndrome during surgical repair of cleft palate and reduction of macroglossia are presented and discussed. This syndrome is characterized by exomphalos, macroglossia, gigantism, hypoglycemia in infancy, and many other clinical features. This syndrome is also known as exomphalos, macroglossia, and gigantism (EMG) syndrome. Principal problems associated with anesthetic management in this syndrome are hypoglycemia and macroglossia. Careful intraoperative plasma glucose monitoring is particularly important to prevent the neurologic sequelae of unrecognized hypoglycemia. It is expected that airway management would be complicated by the macroglossia, which might cause difficult bag/mask ventilation and endotracheal intubation following the induction of anesthesia and muscle paralysis, so preparations for airway difficulty (e.g., awake vocal cord inspection) should be considered before induction. A nasopharyngeal airway is useful in relieving postoperative airway obstruction.- - - - - - - - - - ranking = 1keywords = cleft palate, palate, cleft (Clic here for more details about this article) |
2/6. 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.- - - - - - - - - - ranking = 6.3820410779399keywords = cleft palate, palate, cleft (Clic here for more details about this article) |
3/6. 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.- - - - - - - - - - ranking = 0.047755134742487keywords = palate (Clic here for more details about this article) |
4/6. macroglossia and ankyloglossia in beckwith-wiedemann syndrome.Since the recognition of the beckwith-wiedemann syndrome was first noted in 1963, the number of associated anomalies has vastly increased. The rate of appearance of this abnormality is 1 in 13,5000 births. This article presents a case that includes macroglossia and ankyloglossia along with a bifid uvula and a submucous cleft of the palate. A discussion of treatment follows.- - - - - - - - - - ranking = 0.071721305378159keywords = palate, cleft (Clic here for more details about this article) |
5/6. 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.- - - - - - - - - - ranking = 0.023966170635672keywords = cleft (Clic here for more details about this article) |
6/6. 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.- - - - - - - - - - ranking = 1keywords = cleft palate, palate, cleft (Clic here for more details about this article) |