1/183. Stenosis of the nasal vestibule and its treatment.From our own experience and a review of the literature, we present a few techniques which, in our eyes, give the surgeon the possibility to treat most encountered cases of stenosis of the nasal vestibule. During 1991 to 1998 the author in Stuttgart (W.G.) performed simple z-plasty combined with local flaps in 6 patients and composite grafts only in 12 cases, to correct nasal vestibule stenosis. The author in Lausanne (R.M.), who first described the paranasal myocutaneous flap to correct not only nasal vestibule stenosis but also alar base malposition has treated over 50 patients with this technique and with composite grafts during the last 20 years.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
2/183. Multiple systemic and periocular malformations associated with the fetal hydantoin syndrome.anticonvulsants remain necessary during pregnancy and the removal of such drugs is not recommended. However, on the available evidence, the physician may expect an increased risk of malformation including eye abnormalities as has been outlined. The abnormalities include growth deficiencies and delayed motor/mental development together with dysmorphic features, the most common of which seems to be cleft lip/cleft palate. Additionally, many of these children suffer from eye abnormalities including hypertelorism, ptosis, strabismus, epicanthal folds, and in this case abnormalities of the lacrimal apparatus.- - - - - - - - - - ranking = 0.5keywords = ring (Clic here for more details about this article) |
3/183. Computer-aided surgery in distraction osteogenesis of the maxilla and mandible.When using unidirectional intraoral distraction devices, it is desirable to be able to determine the final position of the bone fragment after the distraction procedure. However, additional constraining forces from adjacent tissues render the prediction of the distraction direction difficult. We have utilised computer-aided surgery in three patients for intraoperative control of the distraction direction. In one cleft palate patient, suffering from maxillary hypoplasia and anterior open bite, a modified Le Fort I osteotomy and maxillary distraction was performed. Despite a ventrocaudal position of the distraction device, intraoperative computer visualisation showed an unfavourable caudal vector of distraction without any anterior movement. The final result confirmed the direction indicated by the computer. Maxillary advancement remained insufficient. In two patients suffering from mandibular hypoplasia, intraoperative assessment revealed a favourable direction of distraction. The distraction procedure led to a satisfactory result in both cases. Computer-aided surgery is helpful in assessing the vector of distraction intraoperatively, making the result of the distraction procedure more predictable and allowing instant correction by adequate reapplication of the device.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
4/183. Increased occurrence of cleft lip in glycogen storage disease type ii (GSDII): exclusion of a contiguous gene syndrome in two patients by presence of intragenic mutations including a novel nonsense mutation Gln58Stop.Genetic deficiency of lysosomal acid alpha-glucosidase (acid maltase) results in the autosomal recessive disorder glycogen storage disease type ii (GSDII) in which intralysosomal accumulation of glycogen primarily affects function of skeletal and cardiac muscle. During an earlier review we noted 3 in 100 cases of GSDII with incidental description of cleft lip. In addition, we identified 2 of 35 GSDII patients referred to us for molecular studies with co-occurence of cleft lip, considerably greater than the estimated frequency of nonsyndromic cleft lip with or without cleft palate of 1 in 700 to 1,000. Because several lines of evidence support a minor cleft lip/palate (Cl/P) locus on chromosome 17q close to the locus for GSDII, we defined the molecular basis for the GSDII in these two patients to determine if they represented a contiguous gene syndrome. Patient I (of Dutch descent) was homozygous and the parents heterozygous for an intragenic deletion of exon 18 (deltaex18), common in Dutch patients. Patient II was heterozygous for delta525T, a mutation also common in Dutch patients and a novel nonsense mutation (172 [corrected] C-->T; Gln58Stop) in exon 2, the first coding exon. The mother was heterozygous for the delta525T and the father for the 172 [corrected] C-->T; Gln58Stop. The finding that both patients carried intragenic mutations eliminates a contiguous gene syndrome. Whereas the presence of cleft lip/cleft palate in a patient with GSDII could be coincidental, these co-occurences could represent a modifying action of acid alpha-glucosidase deficiency on unlinked or linked genes that result in increased susceptibility for cleft lip.- - - - - - - - - - ranking = 45.815796867169keywords = chromosome, ring (Clic here for more details about this article) |
5/183. Biomechanical considerations in distraction of the osteotomized dentomaxillary complex.The completely osteotomized dentomaxillary complex is essentially a free body constrained only by its soft tissue attachments. Therefore the line of action and point of application of any protractive force(s) used during distraction osteogenesis must be considered relative to its center of mass. This is in contrast to the nonsurgically separated dentomaxillary complex, which is a constrained body, and therefore the application of protractive force(s) must be considered relative to its center of resistance. These two centers are not coincident. With knowledge of the location of the center of mass, predictable protraction of the dentomaxillary complex can be achieved. In this study, the center of mass of an adult maxillary specimen osteotomized to emulate a Le Fort I osteotomy was determined. Protractive force(s) through the center of mass will produce linear advancement along its line of action. Protractive movement of the dentomaxillary complex can be adjusted downward and forward or upward and forward by locating the protractive force(s) line of action superior or inferior to the center of mass. A cleft patient is described wherein the surgically separated dentomaxillary complex is protracted downward and forward with a force vector superior to its approximate center of mass. This results in a predictable increase in overbite and overjet with negligible mandibular rotation.- - - - - - - - - - ranking = 0.5keywords = ring (Clic here for more details about this article) |
6/183. Two sibs with Malpuech syndrome.We report on two Italian brothers with facial clefting, hypertelorism, urogenital anomalies including micropenis, shawl scrotum, hearing loss, caudal appendage, and umbilical hernia. We have evaluated the two cases as Malpuech syndrome. This is an extremely rare autosomal recessive syndrome.- - - - - - - - - - ranking = 0.5keywords = ring (Clic here for more details about this article) |
7/183. Disorganisation: a case with popliteal pterygia and placental-skin appendages.We report a girl with congenital anomalies which include amniotic rings and scars, cleft lip and palate, thumb abnormalities, hexadactyly of feet, severe flexion deformities of legs and unusual finger-like appendages which were attached to the placenta. We suggest this patient represents another example of human homologue for the mouse mutant disorganisation (Ds).- - - - - - - - - - ranking = 0.5keywords = ring (Clic here for more details about this article) |
8/183. Nasal abnormalities in facial clefts.After a short review of their development rare clefts of the nose occurring together with facial clefts are described in a number of clinical cases. These may be divided into 6 groups: 1. clefts of the nose; 2. oblique facial clefts; 3. nasal abnormalities in cleft lip and palate cases; 4. nasal abnormalities in median cleft palate cases; 5. aplasia of the premaxilla; 6. syndromes associated with nasal abnormalities and facial clefts. The discussion deals with the literature, aetiology, prognosis and therapeutic aspects. The publication of individual rare cases is suggested.- - - - - - - - - - ranking = 0.5keywords = ring (Clic here for more details about this article) |
9/183. trisomy iop. A report of two cases due to a familial translocation rcp (10;21) (pII;pII).trisomy for the short arm of chromosome number 10 was diagnosed (by a G-banding method) in two sisters with multiple congenital defects. Their mother and two other sisters showed a balanced translocation 46,XX rcp(10;21)(p11;p11), so the affected girls were the result of a maternal adjacent-1 meiotic segregation with a karyotype 46,XX, der(21), rcp(10;21)(p11;p11)mat. The concordant features in the abnormal patients constitute the following syndrome: severe psychomotor retardation, congenital microsomatia, mild hydrocephalus with cranium-face disproportion, low set ears with hypoplastic helix, ocular colobomata, pulmonary stenosis,flexion deformity of wrists and elbows, bilateral fifth finger clinodactyly and simian creases, hypoplastic dermal ridges, bilateral talipes, persistent icterus and delayed bone age. The phenotypical and cytogenetic findings permit the individualization of the 10p trisomy.- - - - - - - - - - ranking = 45.315796867169keywords = chromosome (Clic here for more details about this article) |
10/183. A possible otological complication due to maxillary expansion in a cleft lip and palate patient.OBJECTIVE: We present a possible negative side effect of a sudden onset of secretory otitis media in a 12-year-old boy with unilateral cleft lip and palate who underwent maxillary expansion. The secretory otitis media caused a temporary hearing loss developed during the activation of the expander appliance. The possible causes for this complication are discussed. CONCLUSIONS: Clinicians should be aware of the possible association between maxillary expansion and secretory otitis media.- - - - - - - - - - ranking = 1keywords = ring (Clic here for more details about this article) |
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