1/735. 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. ( info) |
2/735. Camouflage in head and neck region--a non-invasive option for skin lesions. The technique of camouflage, a non-invasive procedure to correct flaws in the texture and colour of the facial skin, is presented. The acceptance and use of camouflaging by 52 patients with different diagnoses are presented. The advantages of camouflaging are discussed in comparison to medical tattooing. ( info) |
| 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. ( info) |
4/735. 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. ( info) |
5/735. 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. ( info) |
6/735. 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. ( info) |
7/735. 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. ( info) |
8/735. 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. ( info) |
9/735. 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. ( info) |
10/735. 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. ( info) |