1/34. Radial forearm fasciocutaneous free flap as a solution in case of noma.The authors describe a case of noma or Cancrum Oris, an oral gangrenous disease, features more frequently found in children from developing countries. The clinical features, its ethiopathogenesis, and its particular link with different geographic and economic areas of the world, its clinical evolution as well as surgical treatment are all discussed. Underlined is the functional and organic aspect of this disease, in particular the distortion of the face, which commonly involves the full thickness of the cheek skin and bone, mandibular ankylosis and craniofacial dismorphisms, and the modern approach in reconstructive microsurgery. The authors report a case of a patient affected by noma, with a very evident left face dismorphism, where we found a brilliant solution using a left radial forearm fasciocutaneous free flap, appropriately shaped.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
2/34. anesthesia for Freeman-Sheldon syndrome using a laryngeal mask airway.PURPOSE: To present a case of Freeman-Sheldon syndrome (FSS) with a previously unreported technique of anesthetic management, consisting of a malignant hyperthermia free anesthetic and laryngeal mask airway. CLINICAL FEATURES: Freeman-Sheldon syndrome (also known as whistling face syndrome, Windmill-Vane-hand syndrome, cranio-carpo-tarsal dysplasia and distal arthrogryposis type 2) is a rare congenital disorder defined by facial and skeletal abnormalities. The three basic abnormalities are microstomia with pouting lips, camptodactyly with ulnar deviation of the fingers and talipes equinovarus. patients with FSS frequently present for surgical correction of musculoskeletal or facial abnormalities. There are several anesthetic challenges including difficult airway, intravenous cannulation and regional technique. They may be at increased risk for malignant hyperthermia and postoperative pulmonary complications. We present a case of a two-year-old child with FSS undergoing elective unilateral inguinal hernia repair. A non-triggering anesthetic technique was used, consisting of 2 mg x kg(-1) propofol followed by a continuous infusion, nitrous oxide 50%/oxygen, and 3 microg x kg(-1) fentanyl. Intraoperative and postoperative analgesia was provided by an ilioinguinal nerve block with 10 ml bupivacaine 0.25% with epinephrine 1:200,000. The airway was maintained with a #2 laryngeal mask airway. The anesthetic was uneventful and there were no signs or symptoms of malignant hyperthermia. The patient was discharged home later the same day in good health. CONCLUSION: The use of a laryngeal mask airway and non-triggering anesthetic technique should be considered as options for anesthetic management in patients with FSS for short procedures that do not require neuromuscular blockade.- - - - - - - - - - ranking = 51.981349627661keywords = nerve (Clic here for more details about this article) |
3/34. Identification of two de novo partial trisomies by comparative genomic hybridization.We report the use of comparative genomic hybridization (CGH) to define the extra chromosome region present in two de novo partial trisomies 15q25-qter and Xp21-pter, which could not be clarified by conventional G-banding. Investigation with fluorescence in situ hybridization (FISH) revealed that the partial trisomy corresponded to an unbalanced translocation between Y and 15 chromosomes in 1 patient and an unbalanced X/X reorganization in the other patient. The combination of classical karyotyping, CGH, and FISH is useful for the identification and characterization of partial trisomies in clinical diagnostic laboratories, in order to delineate the chromosome regions implicated in specific clinical disorders.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
4/34. Hemifacial myohyperplasia: description of a new syndrome.Hemifacial hypertrophy is a rare condition characterized by unilateral enlargement of all tissues of the face. We describe three patients who exhibit hemifacial hyperplasia of the muscles of facial expression with no other organ system involvement. These three cases, in addition to six other cases identified in the literature, describe a unique constellation of characteristics that place these patients into a distinct syndrome. We suggest that the term "hemifacial myohyperplasia" be used to describe this specific and unique condition.- - - - - - - - - - ranking = 1keywords = organ (Clic here for more details about this article) |
5/34. Craniofacial and dental manifestations of proteus syndrome: a case report.The proteus syndrome is a rare congenital hamartomatous condition that is characterized by a wide range of malformations, sometimes involving the face. Common manifestations include partial gigantism, congenital lipomas, and plantar hyperplasia. In this report we describe the craniofacial clinicopathological development in a girl with proteus syndrome from age 6 to 20 years. The patient had pronounced hemifacial hypertrophy, exostoses in the left parietal region, and enlargement of the inferior alveolar nerve and mandibular canal in the affected region. The dental development of the affected left mandible and maxilla was characterized by extremely premature development and eruption of the primary and permanent teeth and by pronounced idiopathic root resorptions. The multidisciplinary management of the patient and the treatment outcome is reported. A review of the Proteus patients in the literature who exhibited manifestation in the craniofacial region is presented.- - - - - - - - - - ranking = 51.981349627661keywords = nerve (Clic here for more details about this article) |
6/34. Primary post-traumatic mandibular reconstruction in infancy: a 10-year follow-up.Ballistic trauma to the craniofacial skeleton combines the challenges of complex bone injury and loss with severe soft tissue injury and violation of the naso-orbital or oropharyngeal cavities. The authors report a patient who experienced a unique ballistic injury at 28 months of age that resulted in loss of the mandibular ramus and condyle. A segmental injury to the facial nerve was also identified. Primary costochondral grafting and delayed interpositional nerve grafting was undertaken. After 10 years, the patient has nearly 40 mm of opening, with only slight deviation to the injured side. Her facial nerve regeneration provides complete orbicularis oculi function, oral competence, and only slight facial asymmetry. This traumatic reconstruction differs from that of patients with hemifacial microsomia or post-traumatic/arthritic ankylosis in that the joint space itself was spared. Thus, the costochondral graft benefits from the remaining articular disk and upper disk space and is able to rotate and translate. Function and growth are adequately re-established, even in this young pediatric patient.- - - - - - - - - - ranking = 155.94404888298keywords = nerve (Clic here for more details about this article) |
7/34. An optic nerve hypoplasia and coloboma in a patient with hemifacial microsomia.Posterior segment anomalies are rare features of the oculoauriculovertebral complex. Our patient had hemifacial microsomia with an optic nerve hypoplasia in one eye and an optic nerve coloboma in the other eye. The optic nerve hypoplasia was ipsilateral to the side with hemifacial microsomia and microtia.- - - - - - - - - - ranking = 363.86944739363keywords = nerve (Clic here for more details about this article) |
8/34. Contralateral botulinum injections improve drinking ability and facial symmetry in patients with facial paralysis.PURPOSE: To report two patients who experienced improved drinking ability as well as facial appearance with contralateral injection of botulinum toxin. DESIGN: Retrospective case reports. methods: Two patients were treated with botulinum toxin contralateral to the VIIth nerve palsy to improve drinking ability as well as facial asymmetry. RESULTS: Botulinum toxin injections improved facial asymmetry as well as drinking ability in two patients with facial nerve palsies. CONCLUSIONS: Contralateral botulinum toxin injections improved drinking ability in two patients with facial nerve palsy.- - - - - - - - - - ranking = 155.94404888298keywords = nerve (Clic here for more details about this article) |
9/34. Basicranium malformation with anterior dislocation of right styloid process causing stylalgia.Differential diagnosis of stylalgia is broad, and many causes should be taken into account, such as temporomandibular joint disorders, carotidynia, compression of nerve fibers by neoplasms, cranial nerve neuralgias, chronic pharyngotonsillitis, pharyngeal or base of tongue tumors and elongation of styloid process, or Eagle syndrome. Basicranium malformation can determine a rare form of stylalgia that is caused by dislocation of styloid process. This can produce a reduction of maxillo-vertebropharyngeal space and irritation of nerve fibers together with stylopharyngeal muscular chronic contraction. This case report illustrates the diagnostic dilemma and surgical strategy experienced in a rare case of stylalgia caused by a dislocation of styloid process produced by a basicranium malformation. Basicranium malformation should be considered in the pathogenesis of stylalgia when a styloid process with normal dimension is encountered.- - - - - - - - - - ranking = 155.94404888298keywords = nerve (Clic here for more details about this article) |
10/34. Deformation of the mandible due to neonatal moulding: a 10-year follow-up.A 3-day-old infant with paralysis of the marginal mandibular branch of the facial nerve and asymmetry of the mandible due to neonatal moulding is presented. Ten years after the initial presentation, the asymmetry and paralysis was still present. Orthodontic treatment has been instituted because of malocclusion. Surgical correction of the mandibular asymmetry after adulthood seems not to be necessary.- - - - - - - - - - ranking = 51.981349627661keywords = nerve (Clic here for more details about this article) |
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