The spectrum of median craniofacial dysplasia

Given the multiple permutations in craniofacial malformations, classification of median craniofacial dysplasia or midline Tessier no. 0 to 14 clefts has been difficult and disjointed. In this review, the authors present a summary of normal embryology, prior terminology, and their proposed new classification system. Median craniofacial dysplasia has tissue agenesis and holoprosencephaly at one end (the hypoplasias), frontonasal hyperplasia and excessive tissue (the hyperplasias) at the other end, and abnormal splitting or clefting and normal tissue volume (dysraphia) occupying the middle portion of the spectrum. These three distinct subclassifications have different forms of anomalies within their groups.     

Pathophysiology and medical treatment of pain in fibrous dysplasia of bone

One of the most common complications of fibrous dysplasia of bone (FD) is bone pain. Usual pain killers are often of inadequate efficacy to control this bone pain. The mechanism of bone pain in FD remains uncertain, but by analogy with bone tumors one may consider that ectopic sprouting and formation of neuroma-like structures by sensory and sympathetic nerve fibers also occur in the dysplastic skeleton. Bone pain has been reported in up to 81% of adults and 49% of children. It affects predominantly the lower limbs and the spine. The degree of pain is highly variable and adults reports more pain than children. Bisphosphonates have been shown to reduce bone pain in uncontrolled studies. Their influence on bone strength remains unknown. In a randomized trial testing alendronate, bone pain was not significantly improved. Another trial assessing the effect of risedronate is ongoing. Possible future therapies include tocilizumab, denosumab and drugs targeting nerve growth factor and its receptor TrkA.     

Surgical treatment for secondary retracted nose

This surgical technique is presented to correct nasal tip retraction, which is frequently associated with other surgical sequelae that can be corrected simultaneously. It is based on the use of a shield, an anchor, or half an anchor of otocartilage, with one or two posterior supports that are sutured together forming a small L and are fixed to the bed to project the nasal tip and, if necessary, to correct the unilateral or bilateral alar collapse.     

急性梗阻性大肠癌的外科治疗探讨

目的探讨急性肠梗阻性大肠癌的外科治疗方法。方法回顾性分析我院收治的1994年至2008年85例急性梗阻性大肠癌的外科治疗资料。在36例右半结肠癌中,35例行一期右半结肠切除术,1例行捷径手术,在49例左半结肠及直肠癌中,36例行一期切除术,5例单纯造瘘术,5例行麦氏术,3例行Har-tm ann术。结果一期右半结肠切除35例顺利康复,1例重度感染休克死亡;一期左半结肠切除34例,1例肠麻痹,MoF死亡,6例切口感染,2例吻合口漏,再次行横结肠造瘘术。结论对于急性梗阻性大肠癌,应遵循个体化原则,合理选择手术方法,右半结肠癌性肠梗阻,可行一期手术,对于左半结肠癌性急性肠梗阻,行一期手术是可行的,手术并发症的发生率与分期手术的发生率相仿。     

Inferior turbinate surgery: an adjunct to successful treatment of nasal obstruction in 408 patients

Septal deviation is often associated with hypertrophy of the contralateral inferior turbinate. Failure to reduce the size of the turbinate at the time of septal reconstruction may result in persistent nasal obstruction. The authors present their experience with 408 patients who underwent one of four turbinate procedures over a 6-year period. Most patients underwent unilateral turbinate surgery, although bilateral procedures were undertaken in 7 percent of patients. A graduated surgical approach was taken that varied according to the amount of turbinate enlargement and the degree to which mucosa and bone were involved. Full-thickness excision of the anterior third to half of the inferior turbinate (turbinectomy) became a favored procedure. Relief of nasal obstruction was obtained in greater than 90 percent of patients. Healing was satisfactory regardless of the method, and complications, including hemorrhage and infection, were few. Long-term follow-up revealed no untoward sequelae, and no patient developed atrophic rhinitis. The authors conclude that turbinate surgery, particularly when unilateral, in the carefully selected patient with nasal obstruction is a useful adjunct to septal surgery.     

The surgical management of fibrous dysplasia of bone

The surgical management of Polyostotic Fibrous Dysplasia (FD) of bone is technically demanding. The most effective methods to manage the associated bone deformity remain unclear. The marked variation in the degree and pattern of bone involvement has made it difficult to acquire data to guide the surgeon's approach to these patients. In light of the paucity of data, but need for guidance, recognized experts in the management of these patients came together at the National Institutes of Health in Bethesda, Maryland as part of an International meeting to address issues related to fibrous dysplasia of bone to discuss and refine their recommendations regarding the surgical indications and preferred methods for the management of these challenging patients. The specific challenges, recommended approaches, and "lessons learned" are presented in hopes that surgeons faced with typical deformities can be guided in the surgical reconstruction of both children and adults with FD.     

McCune-Albright syndrome and the extraskeletal manifestations of fibrous dysplasia

Fibrous dysplasia (FD) is sometimes accompanied by extraskeletal manifestations that can include any combination of café-au-lait macules, hyperfunctioning endocrinopathies, such as gonadotropin-independent precocious puberty, hyperthyroidism, growth hormone excess, FGF23-mediated renal phosphate wasting, and/or Cushing syndrome, as well as other less common features. The combination of any of these findings, with or without FD, is known as McCune-Albright syndrome (MAS). The broad spectrum of involved tissues and the unpredictable combination of findings owe to the fact that molecular defect is due to dominant activating mutations in the widely expressed signaling protein, Gsα, and the fact these mutations arises sporadically, often times early in development, prior to gastrulation, and can distribute across many or few tissues.The complexity can be mastered by a systematic screening of potentially involved tissues and cognizance that the pattern of involved tissues is established, to some degree, in utero. Thorough testing allows the clinician to establish, often times at presentation, the full extent of the disease, and importantly as well what tissues are unaffected. Treatment and follow-up can then be focused on affected systems and a meaningful prognosis can be offered to the patient and family. The authors outline screening and treatment strategies that allow for effective management of the extraskeletal manifestations of FD.     

The effect of nasal polyposis related nasal obstruction on cognitive functions

Chronic rhinosinusitis with nasal polyposis is a chronic inflammatory disease of the respiratory mucosa of the nasal cavity and paranasal sinuses. The aim of this study was investigate the effect of nasal obstruction related to chronic rhinosinusitis with nasal polyposis on cognitive functions. Patients with chronic rhinosinusitis with nasal polyposis causing bilateral total or near total nasal obstruction were enrolled in the study. Symptoms of nasal congestion, loss of smell, postnasal drip, headaches, snoring, concentration difficulties and blunted affect were evaluated by Visual Analog Scale. Brief symptom inventory test, Stroop test, visual aural digit span, serial digit learning test and P300 test were used to evaluate cognitive functions. Three months after treatment, the tests done before surgery were repeated and the results were compared. A total of 30 patients were included in the study. On the Visual Analog Scale, all symptoms showed significant postoperative improvement in all patients (p?<?0.001 for all symptoms). Preoperative nasal congestion accompanied with impaired concentration were detected in 27 patients (90%), and these symptoms recovered in all these patients after treatment (p?=?0.035) (correlation coefficient 0.4). Only 22 patients completed the neuropsychological tests. The mean preoperative Stroop test (23.16?±?5.30), visual aural digit span test (24.68?±?3.52), and serial digit learning test (16.18?±?5.35) scores were showed significant improvement compared with mean postoperative Stroop test (21.12?±?5.69), visual aural digit span test (26.45?±?2.98), and serial digit learning test (19.31?±?4.47) scores (p?=?0.047, p?=?0.022, p?=?0.005 respectively). The postoperative P300 latency values improved in 19 (63%) patients. The preoperative and postoperative latency values for P300 showed a significant difference (p?=?0.029), whereas the preoperative and postoperative amplitude values for P300 did not differ (p?=?0.096). In conclusion, the results of this study indicate that chronic rhinosinusitis with nasal polyposis (CRSwNP) has negative effects on cognitive functions, such as the ability to focus and maintain concentration. These cognitive functions improve after the patients undergo endoscopic sinus surgery to treat their CRSwNP.     

Surgical correction of exophthalmos secondary to Graves' disease

Graves' disease has been recorded in the medical literature for more than 150 years. Despite introduction of iodine into the diet, Graves' disease still remains the most important disorder of the thyroid gland. Clinically, Graves' disease is a multisystem disorder of unknown etiology characterized by the clinical triad of infiltrative pretibial dermopathy, thyroid glandular hyperplasia, and ophthalmopathy. Expansion of the bony orbital volume is an effective method of treating moderate to severe exophthalmos. Our experience with a simplified version of a three-wall orbital decompression to correct exophthalmos secondary to Graves' disease is presented.     

X-ray manifestations of fibrous dysplasia of the facial bones]

The paper is concerned with roentgenological characterization of mono- and poly-osseous fibrous dysplasia in cranial facial bones of 60 persons of both sexes and at a different age, of them 14 persons were under 14. Mono-osseous lesions after radical operations were shown to be capable of continued growth of rearranged osseous tissue in those cases when the periosteum was not completely removed.     

Valvular obstruction of the nasal airway.

Valvular nasal obstruction may occur in the postoperative rhinoplasty patient. One may anticipate a dropping of the tip, from residual redundant or inelastic skin, in some older patients with long noses. Measures to correct (or avoid) this may be undertaken at the time of the primary rhinoplasty. However, an overcorrection may be necessary if there is much redundant skin. Discretion may indicate the need for a secondary procedure. Lateral wall valving is unusual-but it may occur in the long, high, thin nose (where a suggestion of this action may be observed preoperatively). Maintenance of continuous cartilage along the alar rim, at the time of alar cartilage resection, appears to be important in prevention of postoperative valvular obstruction in these few patients.