1/50. Salivary gland aplasia with cleft lip and palate: a case report and review of the literature.We report the case of a patient with lifelong symptoms of xerostomia and a repaired bilateral cleft lip and palate. The clinical evaluation demonstrated aplasia of the major salivary glands. A review of the literature pertaining to salivary gland aplasia is presented, along with a summary of the data regarding patient gender, defect sites, hereditary background, and combined manifestations. The diagnostic methods, possible pathogenesis, and management are also discussed.- - - - - - - - - - ranking = 1keywords = cleft, palate (Clic here for more details about this article) |
2/50. Midface distraction osteogenesis in cleft patients: a case report.We present a case of midface distraction in a bilateral cleft lip and palate patient. The patient was a 10-year-old who underwent a high LeFort I osteotomy followed by placement of the Rigid External Distraction halo. Distraction was commenced on the fifth postoperative day at a rate of 1 to 1.5 mm per day until a total of 17 mm of maxillary advancement had been achieved. There were no complications and follow up was at 9 months post distraction. Results show that the patient had improved facial aesthetics and dental occlusion which was overcorrected to a Class III relationship. Velopharyngeal function was unaffected. Distraction osteogenesis of the midfacial skeleton in cleft patients offers the possibility to remodel not only the underlying bony skeleton but also all the soft tissues of the face and palate.- - - - - - - - - - ranking = 0.83194273959495keywords = cleft, palate (Clic here for more details about this article) |
3/50. Popliteal pterygium syndrome in a Swedish family--clinical findings and genetic analysis with the van der Woude syndrome locus at 1q32-q41.The present study describes a Swedish family in which the mother and her son were affected with signs of popliteal pterygium syndrome (PPS, OMIM 119500). Both individuals had bilateral complete cleft lip and palate, oral synechiae, paramedian pits on the lower lip, toe syndactyly and a piece of triangular skin overgrowth on the great toes. The son also presented with soft tissue syndactyly of the 2nd and 3rd fingers. Although popliteal pterygium was not found, the above clinical features were diagnostic for PPS. Chromosomal abnormalities were not revealed in either case by cytogenetic analyses. A test for microdeletion in the VWS region at 1q32-q41 was performed in the family using 5 polymorphic microsatellite markers from the region. The affected son was found to be heterozygous for all 5 markers, suggesting that microdeletion at the VWS region was unlikely. The VWS locus, however, was not excluded by haplotype analysis of the family.- - - - - - - - - - ranking = 0.2keywords = cleft, palate (Clic here for more details about this article) |
4/50. Double tongue, intraoral anomalies, and cleft palate--case reports and a discussion of developmental pathology.OBJECTIVE: Isolated cleft palate is the most common presentation of the nonsyndromic cleft lip/palate combinations and is multifactorial in etiology. We report two cases of children with clefts of the secondary palate coexistent with double tongue and in either case mandibular epulis or superiorly displaced salivary gland. RESULTS AND DISCUSSION: In each case, the palatal cleft correlated anatomically with the intraoral space-occupying lesion. The ratio of tongue volume to intraoral volume during palatogenesis is discussed with reference to the pathogenesis of cleft palate. These clinical cases propose the model of a unifying sequence of developmental events whereby deformation of palatal shelf elevation results in secondary palatal clefting.- - - - - - - - - - ranking = 4.8189385109776keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
5/50. Midline cleft. Treatment of the bifid nose.BACKGROUND: Midline facial clefts are rare deformities with a wide range of clinical findings from a simple midline vermillion notch to major skeletal malformations, including orbital hypertelorism. The bifid nose is a relatively uncommon malformation that is frequently associated with hypertelorbitism and midline clefts of the lip. The presentation of a bifid nose ranges from a minimally noticeable midline nasal tip central groove to a complete clefting of the osteocartilaginous framework, resulting in 2 complete half noses. We describe our experience with 2 patients with midface clefts who presented with bifid noses and a variety of other congenital abnormalities. The anatomy, extensive treatment, and complications of the bifid nose are discussed. DESIGN: Retrospective case review and literature review. RESULTS: Successful creation of an aesthetic nasal contour and normal nasal function was achieved without complication via extensive skin, bony, and cartilaginous resection. CONCLUSIONS: The bifid nose challenges the rhinoplasty surgeon. A successful outcome is dependent on a thorough understanding of the bifid nasal anatomy, proper patient evaluation, careful preoperative planning, and meticulous surgical technique.- - - - - - - - - - ranking = 0.86388547918989keywords = cleft (Clic here for more details about this article) |
6/50. New syndrome characterized by sparse hair, prominent nose, small mouth, micrognathia, cleft palate, crumpled upper helices, digit anomaly, and mild developmental delay.A brother and a sister show very similar clinical features, including sparse hair in the first year of life, prominent nose, small mouth, micrognathia, high arched palate or cleft palate, crumpled upper helices, flexion limitation of the distal interphalangeal joint of the fingers, and mild developmental delay. Their clinical appearance suggests a premature aging phenotype, but is not really compatible with the hitherto known syndromes of that group. The mode of inheritance is likely autosomal recessive.- - - - - - - - - - ranking = 3.5944869327514keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
7/50. The Tessier number 5 cleft with associated extremity anomalies.OBJECTIVE: Oblique facial clefts are rare congenital deformities. They constitute 0.22% of all facial malformations. In this case report, an oblique facial cleft defined as Tessier number 5 is presented. The number 5 cleft of Tessier is one of the most rare congenital facial clefts; review of the literature revealed 20 patients. This case was also demonstrated with associated limb anomalies, suggesting that the patient may have been affected by the amnion rupture sequence. The teratology of these malformations is discussed, and attention is drawn to the amniotic rupture sequence as a possible cause.- - - - - - - - - - ranking = 0.86388547918989keywords = cleft (Clic here for more details about this article) |
8/50. Familial partial trisomy of the long arm of chromosome 10 (q24-26).Two fourth cousins with a strikingly similar pattern of malformation and who have an unbalanced translocation (46, XY, -17, t (17p; 10q) are described. From an analysis of the phenotypes of these patients and others reported with 10q trisomy, we propose that the trisomy 10q 24-26 syndrome includes: growth and mental retardation, a characteristic facies (microcephaly, flat face with spacious forehead, small nose, depressed nasal bridge, arched wide-spaced eyebrows, blepharophimosis, microphthalmia, low-set ears, bow-shaped mouth with prominent upper lip, micrognathia), palate anomalies (high-arched cleft or agenesis), congenital heart disease, and anomalies of the hands and feet. Anomalies common to the cousins, but not described in other patients with trisomy 10q, are believed to be expressions of a partial monosomy of 17p.- - - - - - - - - - ranking = 0.2keywords = cleft, palate (Clic here for more details about this article) |
9/50. cleft palate congenital alveolar synechiae syndrome: case reports and review.Five cases of cleft palate with interalveolar synechiae have been previously reported. This is the first report of siblings with this condition, and suggests a genetic aetiology. These patients present with an inability to open the mouth adequately. Initial management is directed towards dividing the intraoral adhesions, which may cause significant problems of airway management. Significantly, the intraoral adhesions resolved spontaneously in one of our patients. This, to our knowledge, has not been reported before.- - - - - - - - - - ranking = 1.0685517839351keywords = cleft palate, cleft, palate (Clic here for more details about this article) |
10/50. The Kabuki syndrome: four patients with oral abnormalities.The aim of this paper is to report the oral signs and symptoms of four patients with Kabuki syndrome. All had oral abnormalities affecting the palate, teeth, and/or lips, including wide spacing between the teeth, screwdriver-shaped upper incisors, hypodontia, delayed tooth eruption, narrow spacing in the upper canine area, large pulp chambers of the upper incisors and permanent molars, external root resorption of the upper central incisors and permanent molars, a division of the lower third of the root canal in normally one-rooted teeth, tooth retention, retrognathia of the upper jaw, a high palate or cleft lip/palate, and microforms of lower lip fistula.- - - - - - - - - - ranking = 0.38402863020253keywords = cleft, palate (Clic here for more details about this article) |
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