11/47. Acute airway obstruction in an infant with pierre robin syndrome after palatoplasty.This report describes a complication of post-operative oedema of the palate, tongue and pharynx after a Perko-revised cleft palate repair, which resulted in a life-threatening airway obstruction in an infant with pierre robin syndrome. Although infants experiencing airway problems after Wardill-Kilner, von Langenbeck and Furlow palatoplasty have been described, airway complications in a group of Perko-revised repair children have not been previously reported. We speculate that this complication, which occurred in the absence of a history of previous airway problems, is due to prolonged operating time and excessive pressure exerted on the base of the tongue by the Kilner-Doughty retractor. Acknowledgments of this risk permits to identify those patients prior to surgery so that they can be managed appropriately.- - - - - - - - - - ranking = 1keywords = tongue (Clic here for more details about this article) |
12/47. An unusual case of Ankyloglossia Superior.We report the rare finding of a complete cleft of the secondary palate in the presence of a synechial band running from the incisive foramen over the tip of the tongue, terminating in the midline of the floor of the mouth.- - - - - - - - - - ranking = 0.5keywords = tongue (Clic here for more details about this article) |
13/47. Advances in EPG for treatment and research: an illustrative case study.Electropalatography (EPG), a technique which reveals tongue-palate contact patterns over time, is a highly effective tool for speech research. We report here on recent developments by Articulate Instruments Ltd. These include hardware for Windows-based computers, backwardly compatible (with reading EPG3) software systems for clinical intervention and laboratory-based analysis for EPG and acoustic data, and an enhanced clinical interface with client and file management tools. We focus here on a single case study of a child aged 10 /-years who had been diagnosed with an intractable speech disorder possibly resulting ultimately from a complete cleft of hard and soft palate. We illustrate how assessment, diagnosis and treatment of the intractable speech disorder are undertaken using this new generation of instrumental phonetic support. We also look forward to future developments in articulatory phonetics that will link EPG with ultrasound for research and clinical communities.- - - - - - - - - - ranking = 0.5keywords = tongue (Clic here for more details about this article) |
14/47. Prenatal sonographic findings in a case of Varadi-Papp syndrome.OBJECTIVES: We aim to describe a case with oral-facial-digital syndrome type VI (OFDS VI) or Varadi-Papp syndrome where the only prenatal findings were cleft lip and palate and a primum type atrial septal defect (ASD). methods: A 24-year-old pregnant woman, gravida 1 para 0, attended the prenatal clinic for a routine visit at 16 weeks of gestation. Her medical and family history was unremarkable. There was no history of consanguinity, drug or teratogen exposure. Her triple screening test and other routine biochemical and haematological blood tests did not reveal any abnormal results. During routine prenatal sonographic examination at the 16th gestational week, cleft lip and palate including secondary palate on the right side of the face was detected and ASD was suspected. RESULTS: After delivery, prenatally diagnosed cleft lip and palate and primum ASD were confirmed. Postnatally, minimal micrognathia, posteriorly rotated low-set ears, minimal hypertelorism, epicanthal folds, nystagmus, esotropia, broad nasal tip, intraoral frenula and lobed tongue, shawl scrotum and duplicated nail of the index fingers of both hands were also observed. Radiological examination showed partial agenesis of the corpus callosum and partially duplicated terminal phalanx of the index fingers. A diagnosis of Varadi-Papp syndrome was formulated. At one year of age, there was short stature and delayed mental and motor development. CONCLUSION: Understanding the limitations of prenatal ultrasound is very important for the genetic counselling of prospective parents, since major ultrasound findings can be associated with other minor or undetectable features.- - - - - - - - - - ranking = 0.5keywords = tongue (Clic here for more details about this article) |
15/47. 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 = 0.5keywords = tongue (Clic here for more details about this article) |
16/47. Immediate and sustained changes in tongue movement with an experimental palatal "fistula": a case study.OBJECTIVE: To determine the immediate and longer-term effect(s) on tongue movement following the placement of an experimental opening through a palatal obturator (replicate of subject's prosthesis) worn by an adult male with an unrepaired cleft of the hard and soft palate. methods: tongue movements associated with an anterior experimental opening of 20 mm(2) were examined under three conditions: a control condition in which the subject wore the experimental obturator completely occluded, a condition immediately after drilling the experimental openings through the obturator, and a condition after 5 days in which the subject wore the experimental obturator with the experimental opening. An Electromagnetic Articulograph was used for obtaining tongue movements during speech. RESULTS: The findings partly revealed that the immediate introduction of a perturbation to the speech system (experimental fistula) had a temporary effect on tongue movement. After sustained perturbation (for 5 days), the system normalized (going back toward control condition's behavior). Perceptual data were consistent with kinematic tongue movement direction in most of the cases. CONCLUSIONS: Although the immediate response can be interpreted as indicative of the subject's attempts to move the tongue toward the opening to compensate for air loss, the findings following a sustained perturbation indicate that with time, other physiological adjustments (such as respiratory adjustments, for example) may help reestablish the requirements of a pressure-regulating system.- - - - - - - - - - ranking = 4.5keywords = tongue (Clic here for more details about this article) |
17/47. A new case of 2q duplication supports either a locus for orofacial clefting between markers D2S1897 and D2S2023 or a locus for cleft palate only on chromosome 2q13-q21.We report on a pure duplication of the proximal chromosome 2q in a 6.5-year-old boy with V-shaped midline cleft palate and bifid uvula, posteriorly located tongue, and micrognathia (Pierre Robin sequence), celiac disease, failure to thrive, and developmental delay. Cytogenetic and FISH analysis indicated a duplication of chromosome 2q13-q22. In general, pure proximal duplication or triplication of 2q is rare. The clinical features and chromosomal breakpoints of the 10 previously reported patients varied, and no common phenotype or proximal duplication/triplication 2q syndrome could be defined to date. However, based on four previous patients with different orofacial clefts and our case, a locus for orofacial clefting may be located at proximal 2q. The duplication/triplication comprised chromosome 2q13 in all five affected individuals including our patient. Our patient and three previous cases (two with cleft palate only (CPO) and one with cleft lip/palate (CL/P)) showed a cytogenetic breakpoint at 2q13, which could support the presence of a critical dominant gene disrupted by a common breakpoint, however, the fifth case with CPO showed different breakpoints, advocating against the disruption of a critical dominant gene and supporting that the overexpression of a gene(s) on chromosome 2q13-q21 may cause cleft palate only (CPO) and Pierre Robin sequence. Hence, our findings support either the presence of one locus for orofacial clefting (CL/P, CPO, and Pierre Robin sequence) between markers D2S1897 (chromosome 2q12.2) and D2S2023 (chromosome 2q14.2), or alternatively the presence of a locus for CPO and Pierre Robin sequence on chromosome 2q13-q21.- - - - - - - - - - ranking = 0.5keywords = tongue (Clic here for more details about this article) |
18/47. cleft palate fistula closure with a mucosal prelaminated lateral upper arm flap.BACKGROUND: Recurrent cleft palate fistula is a challenge for maxillofacial surgeons. In severe cases, microvascular tissue transfer is necessary to treat these patients successfully. methods: A 23-year-old man with cleft lip and palate, previously treated elsewhere, presented with a 2-cm defect in the posterior hard palate. A tongue flap had already been performed. It was decided to close the persistent fistula with a lateral upper-arm flap. In a first-step operation, a free graft of buccal mucosa was fixed to the lateral upper arm subcutaneously and covered with an alloplastic sheet. After 11 weeks, the prelaminated flap was harvested and transferred to the palate. RESULTS: Integration of the buccal mucosa to the upper arm was uneventful. On harvesting the flap, we found a smooth, thin, and continuous fasciomucosal flap with a mucosa surface of 4 x 4 cm. Healing of the microvascular flap in its recipient site was regular. The fistula was closed successfully. CONCLUSIONS: To the authors' knowledge, this is the first report of a lateral upper-arm flap prelaminated with autologous mucosa. By prelamination, it is possible to circumvent the disadvantage of extraoral skin within the oral cavity and to cover mucosal defects with mucosa, especially in elective procedures, such as cleft cases.- - - - - - - - - - ranking = 0.5keywords = tongue (Clic here for more details about this article) |
19/47. Epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma: report of a case.A case of epignathus combined with cleft palate, lobulated tongue, and lingual hamartoma is reported. A newborn female presented with a large skin-covered mass arising from the palate, cleft palate, and lobulated tongue with a soybean-sized lingual lesion. The palatal and lingual masses were removed on the 9th day after birth. A cystic lesion was also found on the nasal septum after removal of the palatal mass; this lesion was resected at its base. Glossoplasty was performed after excision of the lingual lesion. Histologically, the palatal mass was diagnosed as epignathus. The cystic lesion was suspected to be meningothelial tissues on the basis of hematoxylin-eosin (HE) and immunohistochemical staining: vimentin and epithelial membrane antigen were positive, and factor viii was negative. The mass of the tongue dorsum was diagnosed as hamartoma. No recurrence of epignathus or evidence of meningocele have been observed after 5 years of follow up.- - - - - - - - - - ranking = 3.5keywords = tongue (Clic here for more details about this article) |
20/47. Partial duplication of the face: case report and review.Complete or partial facial duplication is a rare congenital malformation. A spectrum of structural abnormalities varying in degrees of severity has been described in affected individuals. We present discordance for facial duplication between monozygotic twins in which maxillary and mandibular duplication was present in one. The involved twin showed the following findings: ocular hypertelorism, bifidity of the nose, duplication of the maxilla, macrostomia, cleft of the lower lip, hamartoma of the vomer, supernumerary teeth, duplication of the mandibular teeth, bifidity of the tongue, and hamartoma of the floor of the mouth. Surgical management of the facial anomalies is discussed. A review of the literature and discussion of this rare malformation are presented.- - - - - - - - - - ranking = 0.5keywords = tongue (Clic here for more details about this article) |
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