1/60. Maxillary distraction for the management of cleft maxillary hypoplasia with a rigid external distraction system.Maxillary hypoplasia is a common finding in patients with repaired orofacial clefts. Management of this condition has been a challenge to the reconstructive team. The introduction of distraction osteogenesis to treat craniofacial skeletal dysplasias has opened alternative approaches to manage these severe conditions. In this article, the authors present their technique to distract the hypoplastic cleft maxilla using a rigid external distraction device. The clinical assessment, indications, orthodontic procedure, surgical technique, and distraction protocol are reviewed. A case report shows the use of the technique. This technique allows the reconstructive team to treat patients in all age groups with predictable and stable results.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/60. Working with parents to promote health.A cleft lip, with or without cleft palate, is the most common craniofacial malformation. The issue of whether health can be attained if the person has a physical defect is discussed. health education can be considered as a means whereby individuals learn to maintain, restore and promote health. Consideration is given to health promotion and the prevention of ill health in relation to infant feeding. Skill teaching, if successfully completed, may be regarded as a fundamental cornerstone to health promotion.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
3/60. Bilateral congenital choanal atresia and absence of respiratory distress.Bilateral congenital choanal atresia is considered a lethal congenital malformation in an obligatory nasal breathing neonate. Described herein are two cases of bilateral choanal atresia associated with craniofacial anomalies who did not present respiratory distress in the neonatal period. Our first patient had a complete unilateral cleft lip which facilitated oropharyngeal respiration. The second patient presented wory distress in the neonatal period by providing an oropharyngeal airway.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
4/60. 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 = 1keywords = craniofacial (Clic here for more details about this article) |
5/60. 4p- syndrome and 9p tetrasomy mosaicism with cleft lip and palate.Chromosome 4p- syndrome is a multiple malformation syndrome associated with partial deletion of the short arm of chromosome 4 (4p-). It is characterized by dysmorphic features and retarded development. cleft lip and/or palate are the major clinical manifestations. Cases of tetrasomy 9p are extremely rare; the principal clinical manifestations of this condition are characteristic craniofacial abnormalities, generalized hypotonia and severe mental retardation. We present the first case of a female infant with 4p deletion and tetrasomy 9p mosaicism, exhibiting a left-sided cleft lip, alveolus and soft palate. karyotype analysis of lymphocytes cultured from the patient revealed that she was mosaic: 86% of the cells were 46, XX, add (4) (p15.32) and 14% were 47, XX, add (4) (p15.32), idic (9)(q12). The G-banding pattern appeared consistent with either translocation or partial proximal deletion of 4p. In order to make a definitive cytogenetic diagnosis of isodicentric chromosome 9, fluorescence in situ hybridization (FISH) was applied. At 8 months, when the patient weighed 4.3 kg, her cleft lip was repaired. Before and after surgery there were no seizures, and the postoperative course was uneventful.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
6/60. Misclassification risk of patients with bilateral cleft lip and palate and manifestations of median facial dysplasia: A new variant of del(22q11.2) syndrome?The generic term median facial dysplasia (MFD) describes a subgroup of patients with cleft lip and palate exhibiting characteristic craniofacial defects: (1) short prolabium, (2) absence of frenulum labii, (3) hypoplasia of premaxilla, (4) absent upper central and lateral incisors of the cleft side, and (5) deficient septal cartilage and nasal spine. Gross brain malformations are usually absent in MFD. The same craniofacial malformations are also described in patients with holoprosencephaly sequence (HPE-S). We report on two male patients with bilateral cleft lip and palate showing the facial findings of MFD or HPE-S. Additional congenital malformations were anal atresia in one patient and severe cardiac defect in the other. In both, HPE was excluded by brain imaging, although uncommon brain anomalies were detected consisting of multiple white-matter lesions in the one patient and unusual enlargement and tortuosity of intracerebral blood vessels in both patients. In addition to facial anomalies, the patients also had psychiatric problems typically seen in velo-cardio-facial syndrome (VCFS). fluorescence in situ hybridization (FISH) analysis confirmed a 22q11.2 microdeletion in both.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
7/60. blindness as a complication of Le Fort I osteotomy for maxillary distraction.High Le Fort I osteotomy and maxillary distraction has become an accepted method for the treatment of maxillary retrusion in children and teenagers with cleft lip and palate or craniofacial anomalies. This procedure effectively corrects the dentofacial deformity in these patients. No major surgical morbidity has been reported. During the past 4 years, 94 cleft patients with maxillary hypoplasia received Le Fort I osteotomy and distraction osteogenesis at the authors' center. Two of them developed blindness after this operation. The first case was a girl with bilateral cleft lip and palate with median facial dysplasia. She received high Le Fort I osteotomy at age 12 years 4 months to correct maxillary retrusion. Right eye swelling and ecchymosis was found after surgery. The patient complained of vision loss in that eye 2 days later. Computed tomography showed subarachnoid hemorrhage and skull base hematoma. There were no atypical fractures in the orbit, pterygoid plates, sphenoid bone, and skull base. Angiogram revealed left ophthalmic and basilar artery aneurysm. The second case was a 12-year-old boy with left cleft lip and palate. He received Le Fort I osteotomy to correct maxillary retrusion. During surgery, abnormal pupil dilatation was found after the osteotomy and down-fracture of maxilla. Emergent computed tomography found no hemorrhage or atypical fractures. Examination revealed complete left optic neuropathy and partial right abducens nerve palsy with mydriasis. magnetic resonance imaging, magnetic resonance angiography, and repeated computed tomography revealed no sign of orbital injury, vascular problem, or abnormal fractures. The cause of blindness was unknown. In both cases, a steroid was used. Maxillary distraction was continued. Recovery of meaningful visual sense did not occur after 3 and 2 years' follow-up, respectively. A review of the literature revealed five other patients who suffered from visual loss after Le Fort I osteotomy. Inadvertent skull base fractures were identified in two cases, but a cause for the blindness was not known in the others. Induced hypotension and indirect trauma may be responsible for the optic nerve injury. In none of the cases was meaningful visual sense recovered, although high-dose steroids were given. In conclusion, a total of seven cases developed blindness after Le Fort I osteotomy. Once blindness develops, the prognosis is poor. High Le Fort I osteotomy should be performed with extreme care, and perhaps the informed consent should include visual loss as a complication of the procedure.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
8/60. Proboscis lateralis: radiological evaluation.Proboscis lateralis is a rare craniofacial anomaly. It consists of a soft, trunk-like process that originates from the medial portion of the orbital roof and it may be accompanied by a number of facial and cerebral anomalies. The characteristics and relationship of the lesion to adjacent structures should be known, and associated anomalies should be detected before surgical correction. The patient should undergo neuroimaging studies besides clinical evaluation to obtain this information in detail. The neuroimaging findings of only one previous case are available in the literature. We report two cases of proboscis lateralis evaluated by CT and MRI before corrective surgery.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
9/60. Median cleft of the lower lip: report of two new cases and review of the literature.Median clefts of the lower lip and mandible are rare craniofacial clefts. Couronne in 1819 was the first to describe the condition. The midline cleft of the lower lip was classified by Tessier as a type 30 craniofacial cleft. Recently, the total number of the reported cases had increased to about 66 in the world literature. In addition, 2 more patients are presented here. The first case involves only a small notch in the vermilion; the deformity was treated by Z-plasty. In the second case, a midline incomplete cleft of the lower lip, a sublingual abnormal frenulum, a complete cleft in the mandible, a bifid sternum, a presternal skin tag, and a ventricular septal defect in the heart were present. At operation, Z-plasty of the lingual frenulum released the normal-sized tongue. The lip cleft was corrected by a simple V excision followed by closure in 3 layers. The mandibular segments were "vitalized" with a bone rongeur and immobilized in the predetermined position with an interosseous stainless steel wire. Because the bone fusion was complete, the stainless steel wire was taken out after 3 months so that it would not prevent mandibular development. Our treatment methods and others are discussed.- - - - - - - - - - ranking = 2keywords = craniofacial (Clic here for more details about this article) |
10/60. Van der Woude syndrome with sensorineural hearing loss, large craniofacial sinuses, dental pulp stones, and minor limb anomalies: report of a four-generation Thai family.A four-generation Thai family affected with Van der Woude syndrome is reported. The disorder appeared to be originally inherited from a person who was half Thai and half Pakistani. The lip lesions found in this family were varied and did not appear to be related to other phenotypes. There were some clinical manifestations possibly specific for the condition in this family. They included sensorineural hearing loss, prominent frontal bone, large frontal/sphenoidal/maxillary sinuses with increased mastoid air cells, long tooth roots, dental pulp stones, ankyloglossia, brachydactyly of hands, brachyphalangy, and hyperphalangy of toes, and single flexion crease of the fifth fingers. fluorescence in situ hybridization analysis revealed no visible deletion at a 1q32-41 region.- - - - - - - - - - ranking = 4keywords = craniofacial (Clic here for more details about this article) |
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