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Radioisotope scanning can be helpful while following the progress of the infecClinical Findings tion and helps to determine the length of the course of intravenous therapy that is required. Hyperbaric oxygen the onset of facial palsy in childhood is frequently has also been used as an adjuvant therapy in some cases obscured by the excellent tone of aponeurotic tissues with success, although recent meta-analyses have failed and skin and, therefore, the excellent static suspension to find it significantly adds to the rate of cure. Facial paralysis in children: by trauma, iatrogenic, congenital, Bell/idiopathic, relapsing, differential diagnosis. Facial nerve paralysis secondary to acute otitis media in inreviewed in this classic article, and symptoms and signs assofants and children. Facial electromyography in newborn and young infants demonstrate that facial paralysis is an uncommon complicawith congenital facial weakness. Associated craniofacial malformations, often those involving first and second bran8. Microtia and facial Traumatic Perinatal Facial Palsy clefts are most frequently noted. Palsies isolated to a single branch, particularly the marginal mandibularis, Intrauterine trauma to the facial nerve may occur as a indicate the need for a cardiac evaluation in light of a consequence of compression from the maternal sacrum. The extratemporal facial nerve is at Otologic, electrodiagnostic, and radiologic evaluarisk because the absence of an overlying mastoid tip tions are performed, as necessary, to determine the etiplaces the vertical segment of the nerve at risk for ology. A traumatic cause of the facial nerve dysfunction by a concomitant defect (or defects) involving other is suggested by hemotympanum, periauricular ecchycranial nerves and the absence of evidence of electrical mosis, and the progressive decline of facial nerve responsiveness to evoked and spontaneous electromyoresponsiveness to an applied stimulus. The assessment of perinatal facial nerve dysfunction the Mobius syndrome encompasses a wide specrelies heavily on electrodiagnosis. Electromyographic trum of anomalies due to dysgenesis at the level of evidence of preserved or declining neuromuscular activthe brainstem with resultant neuromuscular deficits ity is most diagnostic. The bilateral absence of facial and abducens muscle biopsy may be required to determine whether a nerve function, as well as other cranial neuropathies, congenital palsy exists. The auditory brainstem response is often A review of the etiologic basis for facial palsy in 95 abnormal and is a helpful adjunct in diagnosis. However, resting tone auricular injury or electrical testing (evoked and spontamay provide adequate eye coverage and oral compeneous electromyography). Occasional procedures and reconstructive procedures to effect betcases of poor recovery, however, suggest the need for a ter symmetry may be indicated later in life. Mobius synsuch cases, surgical exploration and decompression of drome: classification and grading system. The spectrum of congenital facial didrome using a newly developed grading system termed plegia (Mobius syndrome). In the Risks associated with middle ear device implant surgery aging population, 25% of individuals between age 65 and include sensorineural hearing loss, ossicular chain disrup74, and 50% of individuals age 75 and older have hearing tion, facial nerve injury, external canal laceration, and problems. Beyond surgical risks, the United States have moderate to severe sensorineural other considerations associated with implantable hearing hearing loss.

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Thus, in 1978 it was counted that human has 1489 autosome-dominant genes, 1117 autosome recesive and 205 sex linked genes. In human populations the factors increasing inbreeding rate are distance and customs. If the population level in this population is stable for several generations and the numbers of offsprings are different, it increases the probability of close related marriages with all consequences of it. It is more possible in small villages separated from others by distance and natural barriers (mountains, big rivers and so on). The custom to look for a wife among cousins and nieces results in the same effect. The urbanization of last decades rapidly decreases the rate of close related marriages. It was stated that in the small populations with high inbreeding rate, the level of heterozygotes falls down. That can result in accumulation of defect traits and elimination of individuals with these traits from population without natural selection. At the same 209 time, the frequency of these alleles in the Eskimo population in Alaska and Greenland is 30% and 6% respectively; in a small tribe in Tula (Greenland), the frequency of allele la is 9%. Thus, among Cherokee Indians the frequency of la is 80%, whereas among Indians of Utah State it is 2%. As Darwin pointed out, some individuals leave behind more progeny than others, and the rate at which they do so is affected by their inherited characteristics. The phenotype is determined by the interaction of the genotype and the environment, and the linkage between the particular alleles and the particular characteristics of the phenotype is less direct for some features than for others. It is believed that the frequencies ofparticular genes in human population are subject for selection, especially ifthey live in the environment that has been changed not so far ago. It can be stated by evaluation of reasons of spontaneous aborts and perinatal mortality. Kuleshov findings (1978), more than 42% of spontaneous aborts are due to chromosome abnormalities.

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Direct extension into contiguous areas does not the lesions (primary and/or metastatic) exhibit less miqualify for a malignant designation. Malignant lesions croscopic diferentiation, showing cytologic atypia and have been divided into two subtypes: malignant amelomitotic fgures. Malignant variants of ameloblastomas blastoma ure 11-13), in which primary and metaare difcult to control locally. The primary intraosseous carcinoma is an epithelial All subtypes mimic enamel organ Peripheral palisades and budding odontogenic malignancy of the mandible and maxilla that No hard tissue formation is believed to arise from odontogenic rests. This lesion does No clinical signifcance to subtypes not have histologic features of ameloblastoma and is reMicroscopic: desmoplastic, follicular, plexiform, granular cell, garded as a primary jaw carcinoma. This rare lesion of adults affects men more than women, and it is seen in the mandible more than the maxilla. Microscopically, about half of these lesions exhibit keratin formation, and about half show peripheral palisading of epithelial cell nests. This lesion must be diferentiated microscopically from acanthomatous ameloblastoma and squamous odontogenic tumor. Another ameloblastoma that might be considered a subtype has been designated as sinonasal ameloblastoma, occurring mostly in men with a mean age of 61. Histopathology The numerous histologic patterns described for ameloblastoma are of no clinical relevance (Box 11-7). Central to these cells are loosely arranged occasionally develop into a network of epithelium, promptcells that mimic the stellate reticulum of the enamel organ ing the term plexiform ameloblastoma ure 11-18). Another typical feature is the budding of the stroma is desmoplastic and the tumor islands become tumor cells from neoplastic foci in a pattern reminiscent of squamous appearing (squamoid) or elongated, the term destooth development. Some tuThe microscopic subtype most commonly seen in solid mors are microscopically similar to basal cell carcinoma and ameloblastoma is the follicular type ure 11-16). A type of posed of islands of tumor cells that mimic the normal dental solid ameloblastoma in which the central neoplastic cells exfollicle. Central cystic degeneration of follicular islands leads hibit prominent cytoplasmic granularity (and swelling) is to a microcystic pattern ure 11-17). Epithelial invagination into supporting connective tissue often occurs, and occasionally, mural islands may be seen. A characteristic spongiotic change is seen in the epithelial lining, and frequent subepithelial hyalinization (so-called Vickers-Gorlin efect) represents odontogenic ectomesenchyme induction. Diagnosis is often retrospective after enucleation for what was thought to be an odontogenic cyst.

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Failed epidural anaesthesia has been and in a series of 24 patients, whole-lung lavage reported (Itoh et al 1993). The diagnosis is often made late, and if it is delayed until hypoxia ensues, the prognosis Management is poor. Infection may supervene and the situation is worsened by inappropriate treatment 1. Occasionally, lung transplantation has been performed, but recurrence of the disease can Bibliography Itoh K,Itimata M,Matsumoto K et al 1993 Anesthesia occur in the transplanted lung, and an for a patient with alkaptonuria. Kusakabe N,Tsuzuki N,Sonada M 1995 Compression Preoperative abnormalities of the cervical cord due to alcaptonuric arthropathy 1. Although it is generally an adult 1998 Aortic stenosis and coronary artery disease caused by alkaptonuria,a rare genetic metabolic disease, children may be affected (Mahut et al syndrome. Chest X-ray shows an ill-defined nodular pattern, worse in the bases and the perihilar Alveolar proteinosis regions of the lung field. A lung condition in which protein, phospholipid, cholesterol and free fatty acids 4. Arterial blood difficult to distinguish from interstitial lung gases often showed hypoxia (in one patient as disease, therefore alveolar proteinosis must be low as 3. Opportunistic infections, such as overinfiation of the cuff such that it is difficult to nocardiosis, may be superimposed (Pascual et al drain the fiuid. The diagnosis may be delayed because the had received two bolus doses of etomidate condition is mistaken for interstitial lung disease within 18 h developed cardiovascular instability or sarcoidosis. In a third of the patients in one series, arterial oxygen saturation may be a problem alveolar proteinosis did not recur during a 28 (Bradfield & Maynard 1989). Since there are Vigorous manual or mechanical chest percussion increases in intrathoracic pressure and large during lavage increases the efficiency of the fiuctuations in cardiac output, the bronchial technique (McKenzie et al 1989). Bronchial lavage may be stressful to a patient whose suction and manual ventilation are performed, myocardium is compromised.

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