1/27. Tessier type VI-VII cleft combination associated with congenital bimaxillary fusion and anophthalmia.Congenital intermaxillary fusion is a rare anomaly. Combination of the anomaly with any type of facial cleft is extremely rare. death in a majority of these patients as a result of feeding and aspiration problems in early life may have caused the reports to be limited. In this article a 5-year-old patient, probably the first in the literature having Tessier type VI-VII facial cleft combination associated with bimaxillary fusion and anophthalmia on the right side, is presented. The patient has survived on fluid meal through a very small opening for 5 years. The features of the case are presented, and the time and method of the management of such a rare anomaly are discussed with a review of the literature.- - - - - - - - - - ranking = 1keywords = cleft (Clic here for more details about this article) |
2/27. New syndrome of growth and mental retardation, structural anomalies of the central nervous system, and first branchial arch, anophthalmia, heminasal a/hypoplasia, and atypical clefting: report on four Brazilian patients.We report on four unrelated Brazilian patients with growth and mental retardation, structural anomalies of the central nervous system (CNS), mainly callosal agenesis, prominent forehead, facial asymmetry, anophthalmia, heminasal a/hypoplasia, preauricular skin tags, structural anomalies of the external ears, and atypical clefting. This combination of anomalies is unique and, to our knowledge, is a previously undescribed syndrome of unknown etiology, although one of the patients was born to a consanguineous couple, suggesting the possibility of autosomal recessive inheritance. Clinical, genetic, and differential diagnosis aspects are discussed.- - - - - - - - - - ranking = 0.83333333333333keywords = cleft (Clic here for more details about this article) |
3/27. Diencephalic neuronal hamartoma associated with congenital obstructive hydrocephalus, anophthalmia, cleft lip and palate and severe mental retardation: a possible new syndrome.A male infant was born with severe hydrocephalus, bilateral cleft lip/palate, left anophthalmos and right microphthalmos, and an equino-varus foot deformity. Imaging studies showed enlarged lateral ventricles, apparent absence of the corpus callosum and a midline density in the third ventricular region. He had a normal male karyotype. He was severely mentally retarded and died suddenly at 7 years of age. Neuropathological examination of the brain revealed enlarged and polygyric cerebral hemispheres, due to congenital obstructive hydrocephalus, and secondary thinning of the corpus callosum. An unusually large neuronal hamartoma filled the interpeduncular fossa and third ventricle. It was continuous posteriorly with the left thalamus and so was classified as diencephalic rather than as hypothalamic. The right optic nerve merged with the hamartoma, whereas the left nerve was absent. Microscopically the hamartoma consisted of mature grey matter interspersed with narrow bands of white matter. No immature or non-neural elements were identified. This combination of diencephalic neuronal hamartoma, hydrocephalus, ocular and craniofacial abnormalities has not, to our knowledge, previously been described.- - - - - - - - - - ranking = 1.0581934345346keywords = cleft, palate (Clic here for more details about this article) |
4/27. A rare craniofacial cleft: bilateral Tessier no. 5 cleft accompanied by no. 1 and no. 6 clefts.A case of facial cleft that fits the anatomic description of the rare bilateral Tessier no. 5 cleft accompanied by unilateral no. 1 and no. 6 clefts is presented, and brings the total number of reported cases to 21. This case and a review of the literature help to define the soft-tissue and bony course of these clefts, and also emphasize the role of three-dimensional computed tomography to show the bony cleft route. The diagnosis and treatment plan of the no. 5 cleft as well as other rare clefts such as no. 1 and no. 6 clefts are discussed.- - - - - - - - - - ranking = 3.3333333333333keywords = cleft (Clic here for more details about this article) |
5/27. Bilateral Tessier no. 4 facial cleft with left eye anophthalmos: a case report.Craniofacial clefts are very rare and manifest in a variety of patterns. Tessier classified these clefts in 1973 and numbered them 0 to 14. Tessier No. 4 Facial cleft is a rare variant of craniofacial cleft. Not more than 50 cases are reported in world literature, amongst which only 5 cases are true bilateral in nature. However, combinations of two different variants are not very uncommon. A case of male Indian child aged 4 years with a true bilateral Tessier No. 4 Facial cleft is reported. He also had anophthalmos of the left eye. This is probably the first case in which true bilateral Tessier No. 4 Facial cleft with anophthalmos of one eye is noticed.- - - - - - - - - - ranking = 1.6666666666667keywords = cleft (Clic here for more details about this article) |
6/27. Fryns "Anophthalmia-Plus" syndrome associated with developmental regression.We report a boy with a nasal deformity, choanal atresia, bifid uvula, severe bilateral microphthalmia and a facial cleft who showed regression of development at the age of 2 years with subsequent improvement. We suggest he represents a further case of the rare Fryns "Anophthalmia-Plus" syndrome.- - - - - - - - - - ranking = 0.16666666666667keywords = cleft (Clic here for more details about this article) |
7/27. Is it a new syndrome or a clinical variability in cerebro-oculo-nasal syndrome?We present a male infant 2.5-months old with asymmetric skull, anophthalmia, apparent hypertelorism, abnormal nares, unilateral cleft lip and palate, and structural abnormalities of the central nervous system. These findings are similar to cerebro-oculo-nasal syndrome except for the appearance of nose. This case is either a clinical variability in cerebro-oculo-nasal syndrome or a new entity.- - - - - - - - - - ranking = 0.21163868690692keywords = cleft, palate (Clic here for more details about this article) |
8/27. A case study of amnion rupture sequence with acalvaria, blindness, and clefting: clinical and psychological profiles.The purpose of this article is to report the case of a 10-year-old girl born with anophthalmia, bilateral oblique facial clefts, and missing scalp and bones over the temporal and parietal areas of the cranial vault bilaterally. Early amnion rupture seems to be the most probable cause of this rare combination of anomalies. Because no similar case has been reported in the literature so far, we describe here the clinical and psychosocial history of this unusual patient, who has been able to live the intellectually and socially normal life of a blind child in spite of the major craniofacial deformities. The already completed and possible future therapeutic strategies are discussed.- - - - - - - - - - ranking = 0.83333333333333keywords = cleft (Clic here for more details about this article) |
9/27. Newly recognized syndrome with heminasal aplasia and ocular anomalies or wider spectrum of heminasal aplasia/atypical clefting syndrome?We report on five unrelated Brazilian patients with heminasal aplasia associated with diverses anomalies, including lateral proboscis, and anomalies of the eye and first branchial arch. We suggest that these patients represent different conditions within the spectrum of the heminasal aplasia malformation. Clinical, genetic, and differential diagnosis are discussed. copyright 2004 Wiley-Liss, Inc.- - - - - - - - - - ranking = 0.66666666666667keywords = cleft (Clic here for more details about this article) |
10/27. Two cases of Tessier no. 4 cleft with anophthalmia.OBJECTIVE: Tessier no. 4 cleft is a very rare craniofacial anomaly, and the primary surgical procedure has not been definitely standardized. The cheek advancement flap, technique, which was first reported by Van der Meulen (1985), has produced cosmetically favorable results. In this report, two cases with Tessier no. 4 cleft, which were treated with the cheek advancement flap technique, are presented. DESIGN AND patients: The first case was unilateral and the second was bilateral, and both were accompanied with anophthalmia on the cleft side. At the primary operation, dissection of the nasal soft tissue, medial canthopexy, and reconstruction of the lower eyelid and conjunctival fornix were performed. cleft lip was repaired according to the rotation-advancement procedure. RESULTS AND CONCLUSIONS: Postoperative appearances were acceptable in both cases, because the resulting scars were made along the esthetic facial units. However, the soft tissue deficiency of the lower eyelid was prominent. Furthermore, in the unilateral case, caudal displacement of the palpebral fissure and cephalic deviation of the nasal ala was recognized.- - - - - - - - - - ranking = 1.1666666666667keywords = cleft (Clic here for more details about this article) |
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