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Cleft Lip

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2601. Hypogonadotropic hypogonadism and cleft lip and palate caused by a balanced translocation producing haploinsufficiency for FGFR1 Full Text available with Trip Pro

Hypogonadotropic hypogonadism and cleft lip and palate caused by a balanced translocation producing haploinsufficiency for FGFR1 16061567 2006 06 05 2016 11 24 1468-6244 42 8 2005 Aug Journal of medical genetics J. Med. Genet. Hypogonadotropic hypogonadism and cleft lip and palate caused by a balanced translocation producing haploinsufficiency for FGFR1. 666-72 Kim H G HG Herrick S R SR Lemyre E E Kishikawa S S Salisz J A JA Seminara S S MacDonald M E ME Bruns G A P GA Morton C C CC Quade B J (...) Sequence Chromosome Mapping Chromosomes, Human, Pair 7 Chromosomes, Human, Pair 8 Cleft Lip genetics Cleft Palate genetics DNA Mutational Analysis Humans In Situ Hybridization, Fluorescence Kallmann Syndrome genetics Male Microfilament Proteins genetics Molecular Sequence Data Mutant Chimeric Proteins genetics Receptor, Fibroblast Growth Factor, Type 1 genetics Tensins Translocation, Genetic 2005 8 3 9 0 2006 6 6 9 0 2005 8 3 9 0 ppublish 16061567 42/8/666 10.1136/jmg.2004.026989 PMC1736121

2005 Journal of Medical Genetics

2602. Primary unilateral and bilateral cleft lip and nose in an older population Full Text available with Trip Pro

Primary unilateral and bilateral cleft lip and nose in an older population A one-stage procedure to reconstruct complete and incomplete unilateral/bilateral cleft lip and nose deformities is presented. Emphasis was made on closure of the lip muscles, correction of the nostril floor, correction of the alveolar cleft as well as reconstruction of the nose through an intranasal approach, with a supported suture technique for nasal correction. No dental or orthodontic treatment was available (...) or performed in this older population. Emphasis was on primary closure of the muscles, using the rotation advancement principle. The repair that was performed was near anatomical, reconstructing the labial sulcus, the nostril floor, the alveolar cleft and the nasal deformity all in one stage. There was a high level of satisfaction both from the patient's and surgeon's point of view.

2005 The Canadian Journal of Plastic Surgery

2603. Progress toward discerning the genetics of cleft lip Full Text available with Trip Pro

Progress toward discerning the genetics of cleft lip Orofacial clefts are common birth defects with a known genetic component to their etiology. Most orofacial clefts are nonsyndromic, isolated defects, which can be separated into two different phenotypes: (1) cleft lip with or without cleft palate and (2) cleft palate only. Both are genetically complex traits, which has limited the ability to identify disease loci or genes. The purpose of this review is to summarize recent progress of human (...) genetic studies in identifying causal genes for isolated or nonsyndromic cleft lip with or without cleft palate.The results of multiple genome scans and a subsequent meta-analysis have significantly advanced our knowledge by revealing novel loci. Furthermore, candidate gene approaches have identified important roles for IRF6 and MSX1. To date, causal mutations with a known functional effect have not yet been described.With the implementation of genome-wide association studies and inexpensive

2005 Current Opinion in Pediatrics

2604. Hemodynamic differences between propofol-remifentanil and sevoflurane anesthesia for repair of cleft lip and palate in infants. (Abstract)

Hemodynamic differences between propofol-remifentanil and sevoflurane anesthesia for repair of cleft lip and palate in infants. Propofol-remifentanil anesthesia is widely used in adults but few studies are available in infants. We aimed at comparing the hemodynamic effects of propofol-remifentanil vs sevoflurane-fentanyl anesthesia. In addition, we sought to investigate recovery and whether remifentanil induced acute opioid tolerance.In total, 39 infants 4-6 months old were prospectively (...) enrolled and randomized to receive either a combination of remifentanil and propofol (n = 17) or a sevoflurane-fentanyl anesthesia (n = 22) for surgical repair of cleft lip and palate. In both groups, sevoflurane was used for induction of anesthesia and fentanyl was administered before tracheal extubation. Mean arterial blood pressure and heart rate were recorded every 5 min after induction. We also recorded time from termination of surgery to tracheal extubation, postoperative behavior and the need

2007 Paediatric anaesthesia Controlled trial quality: uncertain

2605. A comparison of bilateral infraorbital nerve block with intravenous fentanyl for analgesia following cleft lip repair in children. (Abstract)

A comparison of bilateral infraorbital nerve block with intravenous fentanyl for analgesia following cleft lip repair in children. The efficacy of analgesia with bilateral infraorbital nerve block and intravenous (i.v.) fentanyl were compared for cleft lip surgery in children.Eighty-two children aged 3 months to 10 years undergoing cleft lip repair were prospectively randomized to one of two groups: bilateral infraorbital nerve block (Group B), or i.v. fentanyl (Group F). Group B (n = 41

2007 Paediatric anaesthesia Controlled trial quality: uncertain

2606. A randomized control trial investigating the effect of presurgical orthopedics on feeding in infants with cleft lip and/or palate. (Abstract)

A randomized control trial investigating the effect of presurgical orthopedics on feeding in infants with cleft lip and/or palate. To investigate the controversial assertion that presurgical orthopedics (PSO) facilitate feeding in infants with cleft lip and palate.Randomized control trial of 34 infants with nonsyndromic complete unilateral cleft lip and palate and 16 with cleft of the soft and at least two thirds of the hard palate. Allocation to receive presurgical orthopedics or not used (...) pattern of anthropometric differences emerged. For both cleft groups, infants randomized to presurgical orthopedics were, on average, shorter. The presurgical orthopedics infants were, on average, lighter in the unilateral cleft and lip palate group, but heavier in the isolated cleft palate group. Infants with complete unilateral cleft and lip palate randomized to presurgical orthopedics had lower average body mass index (mean difference PSO-No PSO: -0.45 (95% confidence interval [-1.78, 0.88

2007 The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Controlled trial quality: predicted high

2607. Bilateral intra-oral, infra-orbital nerve block for postoperative analgesia following cleft lip repair in paediatric patients: comparison of bupivacaine vs bupivacaine-pethidine combination. (Abstract)

Bilateral intra-oral, infra-orbital nerve block for postoperative analgesia following cleft lip repair in paediatric patients: comparison of bupivacaine vs bupivacaine-pethidine combination. This prospective, randomised, double blind study compared the efficacy of pethidine as an adjuvant to bupivacaine with the efficacy of bupivacaine alone for infra-orbital nerve block in alleviating postoperative pain in children undergoing cleft lip repair. Forty paediatric patients aged between 5 and 60 (...) months undergoing cleft lip repair were randomly allocated to two groups of 20. After tracheal intubation, group B received bilateral intra-oral, infra-orbital nerve block with 1 ml of 0.25% bupivacaine on each side and group P received 1 ml of 0.25% bupivacaine along with 0.25 mg.kg(-1) body weight pethidine. Sedation after recovery from anaesthesia was assessed using the University of Michigan Sedation Scale (UMSS) and pain was assessed postoperatively up to 36 h using the FLACC scale (Face, Legs

2007 Anaesthesia Controlled trial quality: uncertain

2608. Results of randomized controlled trial of soft palate first versus hard palate first repair in unilateral complete cleft lip and palate. (Abstract)

Results of randomized controlled trial of soft palate first versus hard palate first repair in unilateral complete cleft lip and palate. To compare the outcomes for primary repair of unilateral cleft lip and palate, operating on the soft palate first versus the hard palate first.Randomized controlled trial.The Regional Cleft Service of West Nepal.Forty-seven consecutive patients with nonsyndromic unilateral cleft lip and palate, of whom 37 were assessed 4 to 6 years after completing primary (...) surgical repair.Primary repair of unilateral cleft lip and palate by two differing sequences: (1) soft palate repair, with hard palate and lip repair 3 months later; and (2) lip and hard palate repair, followed by the soft palate repair 3 months later.Analysis of dental study models, weight gain, and speech recordings.Four to 7 years after completing the cleft closure, there was no significant difference in facial growth between the two types of repair sequencing. Completing posterior repair first had

2006 The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Controlled trial quality: uncertain

2609. Infant orthopedics has no effect on maxillary arch dimensions in the deciduous dentition of children with complete unilateral cleft lip and palate (Dutchcleft). Full Text available with Trip Pro

Infant orthopedics has no effect on maxillary arch dimensions in the deciduous dentition of children with complete unilateral cleft lip and palate (Dutchcleft). Evaluation of the effect of infant orthopedics on maxillary arch dimensions in the deciduous dentition in patients with unilateral cleft lip and palate.Prospective two-arm randomized controlled clinical trial with three participating cleft palate centers.Cleft palate centers of the Radboud University Nijmegen Medical Center, Academic (...) Center of Dentistry Amsterdam, and University Medical Center Rotterdam, the Netherlands.Children with complete unilateral cleft lip and palate (n = 54) were included.Patients were randomly divided into two groups. Half of the patients (IO+) had a presurgical orthopedic plate until surgical closure of the soft palate at the age of 52 weeks; the other half (IO-) did not undergo presurgical orthopedics.Maxillary arch dimensions were assessed on dental casts at 4 and 6 years of age with measurements

2006 The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Controlled trial quality: uncertain

2610. Distraction or orthognathic surgery for cleft lip and palate patients: which is better? (Abstract)

Distraction or orthognathic surgery for cleft lip and palate patients: which is better? Maxillary deformities of cleft lip and palate (CLP) can be treated by either conventional osteotomies (CO) or distraction osteogenesis (DO). Which one is better for CLP patients suffering from a moderate extent of maxillary hypoplasia? The aim of the study was to evaluate the treatment outcomes of CO and compared with DO in correction of moderate maxillary hypoplasia. The results showed that CLP patients

2008 Annals of the Royal Australasian College of Dental Surgeons Controlled trial quality: uncertain

2611. Effect of infant orthopedics on facial appearance of toddlers with complete unilateral cleft lip and palate (Dutchcleft). Full Text available with Trip Pro

Effect of infant orthopedics on facial appearance of toddlers with complete unilateral cleft lip and palate (Dutchcleft). To evaluate the effect of infant orthopedics (IO) on facial appearance of 54 patients with unilateral cleft lip and palate (UCLP), aged 4 and 6 years.Prospective two-arm randomized controlled clinical trial in three Cleft Palate Centers in the Netherlands (Dutchcleft-trial).Patients were divided randomly into two groups. Half of the patients (IO+) had a plate until surgical (...) a significant difference on nasolabial photographs between IO+ and IO-. Regression analysis showed a minor effect of occlusion, lip revision, or type of nose reconstruction on the esthetic results.IO had a positive effect on full facial appearance of UCLP children at the age of 4 years, but at the age of 6, only professionals saw a positive effect of IO on the nasolabial photographs. This is irrelevant for UCLP patients since they deal with laymen in their daily life.

2008 The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Controlled trial quality: uncertain

2612. Neonatal repair of cleft lip: a decision-making protocol. (Abstract)

Neonatal repair of cleft lip: a decision-making protocol. Treatment of clefts lip during the neonatal period remains a controversial subject. Those who are in favor of delayed closure argue a higher-risk general anesthesia when it was performed in neonatal period. The purpose of this study was to evaluate the complications and the feasibility of this surgery during the neonatal period.This was a retrospective study of 61 children with labial, labioalveolar, labio-alveolo-palatine (...) , and labiopalatine clefts between May 2000 and November 2006. Each patient's medical file and particularly his or her anesthesia file was used to record the principal demographic data, the results of the malformation workup, and preoperative complications.Sixty-one newborns, 20 girls and 41 boys, aged 7.5 +/- 6.7 days were operated on. The mean weight on the day of surgery was 3190 +/- 454 g. Fifty-four children had a malformation workup (abdominal ultrasonography, spinal bone workup, transfontanelle

2008 Journal of Pediatric Surgery

2613. Gastroschisis associated with cleft lip and palate. (Abstract)

Gastroschisis associated with cleft lip and palate. Gastroschisis is a common congenital abdominal wall defect. Rarely, it is associated with extraabdominal midline congenital anomalies. Oral clefts are the commonest craniofacial congenital midline defects. They can be associated with other midline defects like omphalocele. We believe these are the first 3 case reports of gastroschisis and cleft palate occurring in the same patient.

2007 Journal of Pediatric Surgery

2614. Structure and function of the superior temporal plane in adult males with cleft lip and palate: pathologic enlargement with no relationship to childhood hearing deficits. (Abstract)

Structure and function of the superior temporal plane in adult males with cleft lip and palate: pathologic enlargement with no relationship to childhood hearing deficits. In a previous study from our lab, adult males with non-syndromic cleft lip and/or palate (NSCLP) were shown to have significantly lower temporal lobe gray matter volume than matched controls. The current study was designed to begin a regional analysis of specific subregions of the temporal lobe. The superior temporal plane

2006 Journal of Child Psychology and Psychiatry

2615. Postoperative pain control for primary cleft lip repair in infants: is there an advantage in performing peripheral nerve blocks? (Abstract)

Postoperative pain control for primary cleft lip repair in infants: is there an advantage in performing peripheral nerve blocks? Primary cleft lip repair is performed in infants under 1 year of age. The efficacy of an infraorbital nerve block vs intravenous fentanyl was compared in our prospective, randomized controlled trial.Forty-six infants scheduled for primary cleft lip repair were prospectively randomized to one of two groups: group F that received 2 mcg x kg(-1) of fentanyl intravenously (...) differences in feeding volumes between groups in the PACU (P = 0.46), SSU (P = 0.57) and at home (P = 0.15). There were no differences in time to initiate feeding between the two groups (P = 0.81). However, there was difficulty with feeding in the block groups compared with the fentanyl group (P = 0.01).The infraorbital nerve block can be effectively used for pain control in infants undergoing primary cleft lip repair. Although children undergoing a nerve block experienced minor difficulties in feeding

2008 Paediatric anaesthesia Controlled trial quality: uncertain

2616. Vertical changes in patients with complete unilateral and bilateral cleft lip, alveolus and palate. (Abstract)

Vertical changes in patients with complete unilateral and bilateral cleft lip, alveolus and palate. Twelve UCLP and eleven BCLP patients who had been treated according to the Hanover treatment protocol were evaluated by means of cephalometric analysis and cast measurements. The aim of this investigation was to demonstrate changes in the vertical plane and in incisor position in the course of craniofacial growth compared to age-matched non-cleft controls. Lateral cephalograms and casts were (...) analyzed for each cleft patient at age 10 and 15 and compared with corresponding data of a non-cleft control group (n = 20).A vertical growth impairment of the maxilla was registered in patients with cleft lip, alveolus and palate. The maxilla of the cleft patients showed a significant clockwise rotation while the inclination of the mandible remained virtually unchanged. Both upper and lower incisors, which were significantly retruded at the beginning of the late mixed dentition, could be protruded

2004 Journal of orofacial orthopedics = Fortschritte der Kieferorthopädie : Organ/official journal Deutsche Gesellschaft für Kieferorthopädie Controlled trial quality: uncertain

2617. The effect of infant orthopedics on the occlusion of the deciduous dentition in children with complete unilateral cleft lip and palate (Dutchcleft). Full Text available with Trip Pro

The effect of infant orthopedics on the occlusion of the deciduous dentition in children with complete unilateral cleft lip and palate (Dutchcleft). Evaluation of the effect of infant orthopedics (IO) on the occlusion of the deciduous dentition in patients with unilateral cleft lip and palate (UCLP).Prospective, two-arm, randomized, controlled clinical trial with three participating cleft palate centers (Dutchcleft).Cleft Palate Centers of the University Medical Center Nijmegen, Academic Center

2004 The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Controlled trial quality: uncertain

2618. Cost-effectiveness of infant orthopedic treatment regarding speech in patients with complete unilateral cleft lip and palate: a randomized three-center trial in the Netherlands (Dutchcleft). Full Text available with Trip Pro

Cost-effectiveness of infant orthopedic treatment regarding speech in patients with complete unilateral cleft lip and palate: a randomized three-center trial in the Netherlands (Dutchcleft). To investigate the cost-effectiveness of infant orthopedic treatment (IO), compared with no such treatment in children with complete unilateral cleft lip and palate (UCLP) focusing on the effects on speech development at age 2.5 years.In a three-center prospective, randomized clinical trial (Dutchcleft

2004 The Cleft palate-craniofacial journal : official publication of the American Cleft Palate-Craniofacial Association Controlled trial quality: uncertain

2619. Monosomy and trisomy of 15q24-qter with cleft lip and palate. (Abstract)

Monosomy and trisomy of 15q24-qter with cleft lip and palate. Chromosome 15 aberrations clinically present as facial dysmorphisms such as a prominent nose, low-set ears, micrognathia and a short neck; a cleft lip and palate have not been reported. This is the first reported case of de-novo terminal deletion at 15q24 with a cleft lip and palate and low-set ears. The baby boy had a complete cleft lip and palate on the left side and incomplete cleft lip and palate on the right. A chromosomal study (...) revealed partial monosomy and trisomy of the long arm of chromosome 15, with a karyotype of 46,XY,add(15)(24q) de novo. Surgery for lip plasty was performed at 6 months old and for palate plasty at 1 year and 9 months. Both operations were uneventful, although preoperative and postoperative examinations showed high creatinine phosphokinase values. At 3 years old, mild mental retardation was observed, but his physical development was normal.

2008 International Journal of Oral and Maxillofacial Surgery

2620. Morbidity of iliac crest donor site following open bone harvesting in cleft lip and palate patients. (Abstract)

Morbidity of iliac crest donor site following open bone harvesting in cleft lip and palate patients. The aim of this prospective study was to assess donor site morbidity associated with open iliac crest bone harvesting to graft secondary alveolar clefts. At a university teaching hospital, 64 Jordanian patients underwent secondary alveolar bone grafting with iliac crest bone harvest. The same standardized surgical technique was used over a 6-year period by a single maxillofacial surgeon

2008 International Journal of Oral and Maxillofacial Surgery

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