Cases reported "Cleft Lip"

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1/642. Congenital fistula of the palate.

    Four cases of congenital fistula of the palate are presented. All four patients had a fistula which was situated in the vault with a bifid uvula, submucous separation of the palatal muscles, deformities of the palatal plates and unilateral cleft lip. Velopharyngeal incompetence appeared in primarily treated children. The aetiology and surgical treatment of the congenital defect are discussed.
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ranking = 1
keywords = cleft, palate
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2/642. Nasal deformity and microform cleft lip in parents of patients with cleft lip.

    OBJECTIVE: To describe subtle nasal deformities and microform cleft lips in parents whose children have complete cleft lip deformities. DESIGN: Clinical analyses of three parents whose children had complete cleft lips. Subtle nasal deformities and microform cleft lips were identified. SETTING: An institutional general hospital: Manuel Gea Gonzalez cleft lip and Palate Clinic in mexico City, mexico. patients: The study first examined the parents of all complete and incomplete cleft lip-palate patients who were seen from March 1994 to February 1997 by the authors (n = 1000). We identified three patients, each of whom had one parent who showed signs of subtle nasal deformity and microform cleft lip. INTERVENTIONS: None for the parents. Interventions in the children with cleft lips and palates were known. MAIN OUTCOME MEASURES: Qualitative photographic analyses were performed. Nostril symmetry, septal deviation, nasal floor position, and orbicularis oris malinsertions were carefully examined. RESULTS: Three of the evaluated parents had one alar cartilage caudally displaced and a deviated septum. One parent's nasal floor was depressed. Two parents also had evidence of a minimal orbicularis oris muscle fissure located in the upper lip. CONCLUSION: Genetic evaluations of children with complete and incomplete cleft lips might also include thorough evaluation of their parents. Although small in size, this study of three case histories identified nasal and lip deformities in the patients' parents.
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ranking = 13.543021332293
keywords = cleft, palate
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3/642. Augmentation rhinoplasty using an L-shaped auricular cartilage framework combined with dermal fat graft for cleft lip nose.

    We prepared an L-shaped framework using autogenous auricular cartilage and combined this with dermal fat, according to each patient, to graft it in 12 patients with cleft lip nose. Although auricular cartilage is weak on its own, by our method we obtained a strong columella strut and nasal dorsum augmentation at the same time. Dermal fat graft provided camouflage for cartilage irregularities and was useful for increasing the graft volume. Although absorption caused a decreased volume to a certain extent, there were no other complications such as cyst formation, and a natural nasal contour was achieved in all patients.
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ranking = 4.2125068372734
keywords = cleft
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4/642. New case of the Richieri-Costa/Guion-Almeida syndrome.

    We describe a boy with multiple congenital anomalies/mental retardation (MCA/MR) syndrome. He has growth retardation, microbrachycephaly, coloboma of the iris, and typical facial anomalies including cleft lip/palate. This phenotype overlaps with that described by Richieri-Costa and Guion-Almeida in three Brazilian brothers. The new patient provides further evidence of the existence of this rare clinical entity.
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ranking = 0.87400109396374
keywords = cleft, palate
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5/642. Congenital facial neuropathy in oculoauriculovertebral dysplasia-hemifacial microsomia (Goldenhar-Gorlin syndrome).

    Four patients with clinical features of Goldenhar-Gorlin syndrome who showed facial paralysis on clinical examination are presented. The fourth case died following surgery for cleft lip. autopsy revealed hypoplasia of the right facial nerve in its intracranial segment, with small right facial nucleus in the brain stem. Nosological aspects of the Goldenhar-Gorlin syndrome are discussed. Peripheral facial paralysis, as a part of this syndrome, is reviewed in the light of clinical and pathological findings and in its relationship to cardiac anomalies. It is suggested that Goldenhar-Gorlin syndrome is a part of a so-called cardiofacial syndrome.
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ranking = 0.84250136745468
keywords = cleft
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6/642. Confirmation of Kapur-Toriello syndrome in an Italian patient.

    We report a patient showing the phenotype described by Kapur and Toriello (Am J Med Genet 41:423-425, 1991) in two sibs. Clinical manifestations include severe mental retardation, cleft lip/palate, and distinctive nose, eye, and intestinal defects. This additional case contributes to a better definition of the apparently new MCA/MR syndrome proposed by Kapur and Toriello.
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ranking = 0.87400109396374
keywords = cleft, palate
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7/642. Evidence that AEC syndrome and Bowen--Armstrong syndrome are variable expressions of the same disease.

    Several clinical disorders combine ectodermal dysplasia (ED) and cleft lip and/or palate (CL/P). These conditions have been recognized as a group of diseases with a narrow phenotypic spectrum and multiple points of overlap. We report a patient with a clinical diagnosis of AEC syndrome (ankyloblepharon, ectodermal defects, and CL/P) who additionally has some features observed in a different ED-CL/P disorder, Bowen-Armstrong syndrome. Because of this clinical overlap, we suggest that AEC syndrome and Bowen-Armstrong syndrome may be variable manifestations of the same pathologic entity.
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ranking = 0.87400109396374
keywords = cleft, palate
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8/642. 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.
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ranking = 1.7484073116164
keywords = cleft, cleft palate, palate
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9/642. 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.
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ranking = 4.3700054698187
keywords = cleft, palate
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10/642. 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.
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ranking = 0.90590594416177
keywords = cleft, cleft palate, palate
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