Posterior thoracolumbar fusion in a patient with Kleefstra Syndrome related scoliosis: The first case reported

Spine deformities could be considered a possible manifestation of the childhood hypotonia, typical feature of Kleefstra Syndrome (KS). There is a paucity of literature describing posterior spinal fusion in the Kleefstra syndrome patient. For patients who develop severe scoliotic curve, bracing is often ineffective and surgery is recommended. We report the first corrective surgery for scoliosis in one patient with KS.We describe a case of 13-year-old female with severe developmental scoliosis in KS. Preoperative examination showed a thoracolumbar scoliosis with left convex thoracic curve (T3-T9, 97°) and right convex thoracolumbar curve (T9-L3, 88°). Posterior correction, pedicle screw fixation and bone graft fusion T3-L5 was performed. Postoperatively, the thoracic curve was corrected to 33° while the thoracolumbar one to 26° and better standing posture was obtained. Six month follow-up images showed no loosening of the hardware. The patient is still in our follow-up program.Scoliosis seems to be a rare evenience of the severe hypotonia of patients with KS. We report the first case of scoliosis in KS treated successfully with surgery. Corrective surgery for spinal deformity, such as scoliosis, could help in posture and improve the quality of life especially in complicated patients such as syndromic ones.     

Cranial nasal bone grafts

Reconstitution of the nasal scaffolding with maintainence of soft tissue proportions either following severe facial trauma or as a sequela to aesthetic rhinoplasty misadventures frequently is best achieved using the stability afforded by bone grafts. Split cranial bone grafts offer many advantages and may be the donor site of choice, and may even allow such surgery to be performed on an outpatient basis in some cases. The use of miniplate or screw osteosynthesis, now commonly accepted as a superior technique in craniomaxillofacial procedures, may simplify fixation of these calvarial nasal bone grafts with an apparent decrease in the risk of resorption.     

43例鼻咽,鼻腔恶性淋巴瘤的临床病理及免疫表型研究

本文对遵义地区４３例鼻咽、鼻腔恶淋巴瘤的临床病理及免疫学表型进行了研究，结果显示：全部病例均为弥漫型，无１例滤泡型，组织学类型以多表细胞性淋巴瘤为主，共３３例（包括小细胞型１３例，中多形１５例，大多形５例），占７６．７％。用ＵＣＨＬ－１、ＣＤ２０和Ｍａｃ３８７等多单克隆抗体进行免疫表型研究。显示Ｔ细胞淋巴瘤３７例（８６％），Ｂ细胞淋巴瘤４例（９．３％），无１例组织细胞性淋巴瘤。鼻咽部Ｔ．Ｂ淋巴瘤比     

鼻腔鼻窦恶性肿瘤83例报告

目的 :评价鼻腔鼻窦恶性肿瘤的治疗方法及其效果。方法 :83例原发于鼻腔、上颌窦、筛窦、蝶窦及额窦的恶性肿瘤 ,分别施行上颌骨次全与全切除术 ,前额开窗 -鼻锥翻转进路额筛蝶眶肿瘤大块切除及颅面联合切除术 ,部分病例实施冷冻切除 ,颌颈联合根治等手术 ,术后常规放疗。结果 :3年生存率为 4 7.5 %(36 76 ) ,5年生存率为 36 .2 %(2 5 6 9)。结论 :鼻腔鼻窦恶性肿瘤通过手术及采用术后放疗等综合治疗 ,可提高疗效 ,但仍需进一步改进治疗方法 ,以提高患者生存率。     

鼻腔、副鼻窦原发性肿瘤70例CT分析

本文回顾分析了70例经手术病理证实的鼻腔、副鼻窦原发性良恶性肿瘤的临床和CT表现。讨论了良恶性肿瘤的鉴别诊断,不典型的CT表现及CT的检查价值,认为CT检查可做为鼻腔、副鼻窦肿瘤的首选方法。     

Medpor与游离真皮脂肪移植修复上颌骨及软组织缺损畸形

目的：寻找一种修复上颌骨及软组织缺损的理想方法。方法：采用Medpor与游离真皮脂肪移植，对不同原因所致的上颌骨及软组织缺损畸形进行整复。结果：临床治疗5例，面部畸形均得到良好矫正。结论：这一手术方法创伤小，可塑性强，且大大降低了软组织移植吸收、坏死等并发症的发生，是修复上颌骨及软组织缺损的可取方法。     

利多卡因混合不同剂量肾上腺素局部浸润麻醉对全麻下鼻内窥镜手术患者血液动力学的影响

目的 观察利多卡因混合不同剂量肾上腺素局部浸润麻醉对全麻下鼻内窥镜手术患者血液动力学的影响。方法 69例拟在全身麻醉下行鼻内窥镜手术的患者随机分为3组（n=23），采用含不同剂量肾上腺素的1％利多卡因进行鼻粘膜下多点浸润注射。Ⅰ组：含1：20万肾上腺素的1％利多卡因4ml（20μg）；11组：含1：10万肾上腺素的1％利多卡因4ml（40μg）；Ⅲ组：1％利多卡因4ml（不含肾上腺素）。采用阻抗心动图（ICG）测量并记录浸润麻醉开始前（基础值）、浸润麻醉开始后0．75、1．5、2．25、3、3．75、4．5、5．25、6min平均动脉压（MAP）、心率（HR）、心脏排血指数（CI）、外周血管阻力指数（SVRI）和加速度指数（ACI），同时评价术中出血状况。结果与基础值比较，Ⅰ组和Ⅱ组在浸润麻醉开始后1．5min时MAP下降，HR增快（P〈0．01）；从浸润麻醉开始后1．5minSVRI下降，CI、ACI上升，到浸润麻醉开始后6min时仍未恢复（P〈0．05或0．01）。与Ⅱ组比较，Ⅰ组在浸润麻醉开始后2．25、3、3．75minMAP下降，从2．25min起CI下降（P〈0．01或〈0．05）。与Ⅲ组比较，Ⅰ组、Ⅱ组出血较少（P〈0．01）；Ⅰ组和Ⅱ组之间差异无统计学意义。结论 全身麻醉下鼻内窥镜手术中局部浸润麻醉时，局麻药中肾上腺素的吸收会引起明显的血液动力学变化，推荐在鼻内窥镜手术中使用含有5μg/ml肾上腺素的利多卡因。     

数字图像分析在面部整形中的应用

将数字图像分析、模拟技术应用于隆鼻术及烧伤后口唇畸形的整复中，通过测量分析，在术前模拟出术后的面部图像，并输出手术数据，在医生及患者认可后，指导手术进行，否则，继续图像的修改，在手术允许的范围内，直到医、患双方认可。力求美容手术精确化、定量化及图像化并就如何应用数字图像技术于畸形整复进行了探讨     

The bipartite tarsal navicular bone: Radiographic and computed tomography findings

Two cases of bipartite tarsal navicular bone are presented. The radiographic and computed tomography (CT) findings of this anatomical variant are described. Correct recognition of this entity is important, both because it may be the cause of symptoms perse, and because it may be misdiagnosed as a fracture. When plain films are not diagnostic, CT scanning is helpful in distinguishing between a fracture and this variant.     

Cleft lip-nose repair technique: Sequential repair

The results of repairing cleft lip by aesthetic plastic surgery are now excellent. However, the cleft lipnose deformity is still very difficult to repair with the present techniques. A technique that can repair the cleft lip-nose deformity with good results is presented. The technique is divided into three parts: Part I consists of nasal repair of the primary cleft lip. Part II is nasal reconstruction as a secondary operation with or without lip repair. For example, nasal reconstruction may be secondary to repair of deformities of the sill, rim, limen nasi, septum, or nasal bones. Part III is an aesthetic nasal operation such as rhinoplasty, mentoplasty, or zygomaplasty.