先天性胫骨假关节出院后随访管理的研究进展

先天性胫骨假关节曾是儿童骨科领域最难治愈的疾病,表现为胫骨成角畸形、髓腔狭窄或囊肿等,最终形成不愈合的假关节,目前以手术治疗为主。由于该病治疗时间长,患儿术后至骨骼成熟前均有再骨折的风险,严重影响患儿健康与生活质量,因此对患儿进行出院后随访管理很有必要。然而先天性胫骨假关节患儿分散遍布在全国各省市,给出院后随访管理带来了很多不确定性。本文就先天性胫骨假关节随访中存在的问题进行文献回顾,总结目前国内外对先天性胫骨假关节患儿出院后随访管理的特点,为临床医务人员制定科学的出院后随访管理策略提供依据。     

先天性左侧胫骨缺如伴左足九趾畸形1例

胫骨缺如也称为轴旁性胫骨半肢畸形。我院近期收治先天性左侧胫骨缺如伴左足九趾畸形,现报告如下。患儿,男,20d,因生后发现左小腿短小伴左足九趾畸形而就诊。体查:一般情况好,左小腿明显短缩、细     

先天性胫骨假关节病理及发病机制的研究进展

先天性胫骨假关节(congenital pseudarthrosis of the tibia,CPT)是由于发育异常所致的胫骨畸形,表现为胫骨成角畸形、囊肿或髓腔狭窄等,最终形成不能愈合的假关节.CPT是一种罕见的疾病,其发病率约占新生儿的1/140 000～1/250 000,主要特点是进行性胫骨和腓骨向前外侧弯曲...     

儿童先天性胫骨前外侧弯曲畸形治疗研究进展

先天性的胫骨前外侧弯曲畸形(anterolateral bowing of the tibia, ALBT)被称为先天性胫骨假关节(congenital pseudoarthrosis of the tibia, CPT)的骨折前状态, 是一种儿童骨科的罕见疾病之一。临床特点为胫骨向前方及外侧成角畸形, 通常合并Ⅰ型神经纤维瘤病, 自发或轻微外力作用下骨折后骨不愈合, 最终形成假关节。目前, 治疗ALBT的方法各式各样, 其疗效及结果也不尽相同, 本文就ALBT的治疗研究进展作一综述, 以期为临床开展工作提供参考。     

一种新的儿童先天性胫骨假关节的X线分型

目的 探讨一种新的儿童先天性胫骨假关节的X线分型,为临床治疗和判断预后提供依据.方法 回顾性分析本单位2004年至2014年期间收治的171例先天性胫骨假关节患儿的X线片,男125例(73％),女46例(27％),手术时平均年龄42.5个月.胫骨X线表现包括以下四种情况:Ⅰ型,胫骨前弓,在畸形的顶点处骨皮质增厚、但骨髓腔正常;Ⅱ型,胫骨前弓,在畸形的顶点处髓腔狭窄、骨皮质增厚和骨小梁的缺失;Ⅲ型,胫骨囊性病变,可以发生在胫骨的任何部位;Ⅳ型,胫骨假关节.腓骨X线表现包括以下四种情况:a:腓骨发育正常;b:腓骨发育不良(腓骨直径小于健侧腓骨直径);c:腓骨囊性变,多数发生在腓骨下1/3;d:腓骨假关节.根据患儿就诊时胫骨和腓骨的X线表现,提出一种新的X线分型方法.无论患儿是否曾经历手术治疗、是否有内外固定.结果 171例儿童先天性胫骨假关节按新的X线分型方法,不同类型的病例数如下:Ⅰa型9例,Ⅰb型3例,Ⅰc型3例,Ⅰd型4例.Ⅱa型9例,Ⅱb型15例,Ⅱc型3例,Ⅱd型6例.Ⅲa型6例,Ⅲb型12例,Ⅲc型8例,Ⅲd型17例.Ⅳa型13例,Ⅳb型10例,Ⅳc型4例,Ⅳd型49例.结论 结合儿童先天性胫骨假关节的胫骨和腓骨的X线表现、提出的这种新的X线分型方法,能够囊括目前本单位所有的病例.有可能为先天性胫骨假关节的治疗、术后并发症的防治、预后判断提供重要的指导作用,但仍有待进一步研究.     

先天性胫骨假关节的病因学研究进展

先天性胫骨假关节（congenital pseudarthrosis of the tibia，CPT）是由于发育异常导致的胫骨畸形，表现为胫骨成角畸形、髓腔狭窄或囊肿等，最终形成不愈合的假关节。其发生率占新生儿的1／140000～1／250000。先天性胫骨假关节是骨科领域了解较少、治疗极其困难的疾病。从1709年Hatzoecher首先报告本病以来，已有300余年历史，病因至今不完全清楚。曾有多种学说，如宫内压迫、产伤、血运障碍、全身代谢性疾病等，但都缺乏充足的证据。该病与1型神经纤维瘤病（NF1）存在复杂关系。本文从该病的病理学特点、骨发育相关细胞因子的表达、遗传学与基因学研究、及与1型神经纤维瘤病的关系等方面综述其病因学研究进展。     

趾动脉皮瓣在足部多趾并趾畸形修复中的应用

目的 设计以同趾或邻趾的趾动脉为蒂的岛状皮瓣来修复分趾后趾蹼区的皮肤缺损.方法 2010年1月至2013年1月间共收治并趾多趾畸形49例,28例获得随访.其中,男19例,女9例;年龄13～44个月,平均27.1个月.其中踇多趾畸形7例;并趾畸形21例,包括:第1、2趾并趾畸形5例,第2、3趾并趾畸形3例,第3、4趾并趾畸形4例,第4、5趾并趾畸形9例.所有患儿分趾后均采用趾动脉皮瓣修复趾蹼部皮肤缺损.供区趾体损伤面积较小,可直接缝合,无需植皮.结果 术后所有并趾畸形均得到了矫正,趾间获得很深的趾蹼和自然张开的弧度.出院后28例获得随访,平均随访时间8个月.随访期间发现皮瓣质地柔软,外形满意,无色差,伤指无疼痛,两点分辨率在4～10mm.结论 使用趾动脉皮瓣可完全修复并趾分趾后的软组织缺损而无需植皮,且供区可直接缝合,同时可有效地改善趾蹼外形,是修复足趾并趾分趾畸形的一种安全、有效的新方法.     

顽固性小儿先天性胫骨假关节手术治疗的改良

目的探讨顽固性小儿先天性胫骨假关节改良术式的临床治疗效果。方法1999年1月～2005年8月对顽固性小儿先天性胫骨假关节7例患儿，男5例，女2例，年龄2～11岁，采用带监测皮岛的游离腓骨移植，胫骨假关节切除及胫骨部分骨膜环剥，血管束植入的综合方法治疗。结果经1～4年的随访，平均1．5年，7例患儿假关节均已骨性愈合，其中4例已在支具保护下负重，3例完全不需支具保护负重。随访期间未出现再骨折现象。结论以最大限度的切除病变组织，改善局部血循环为中心的改良术式对于顽固性小儿先天性胫骨假关节的治疗有较好的疗效。     

三合一骨融合术在儿童先天性胫骨假关节联合手术中的应用

目的 探讨采取联合手术同时行三合一骨融合术治疗儿童腓骨完整型先天性胫骨假关节的短期疗效.方法 回顾性分析2014年3月至2015年8月采取联合手术(切除胫骨假关节及病变组织、经足踝髓内棒固定、伊氏架外固定装置加压固定)的同时,行三合一骨融合术(腓骨近端截骨、胫骨假关节远近二个骨端与局部完整的腓骨三者融合、取自体髂骨包裹式植骨)治疗17例腓骨完整型先天性胫骨假关节患儿的临床资料.17例均为单侧,术时平均年龄为3岁(1.1～7.7岁).其中,男12例,女5例;左侧7例,右侧10例.3岁以下者10例(占59％),2例既往有1次胫骨假关节手术史;均伴腓骨完整.伴有胫骨近端发育不良4例,1型神经纤维瘤病10例;11例患儿存在胫骨短缩,胫骨平均短缩1.6 cm (0.3～3.4 cm).术中行腓骨近端截骨、切除胫骨假关节及病变组织、经足踝髓内棒固定、伊氏架外固定装置加压固定、胫骨假关节二个骨端与腓骨融合、取自体髂骨包裹式植骨,2例同时行胫腓骨截骨延长.初步评价胫骨假关节患儿的早期愈合率,再骨折发生率,踝外翻、胫骨不等长和胫骨外翻等后遗畸形的发生情况.结果 本组平均手术时间4.1 h(3.3～4.2 h).所有患儿均获得完整随访,平均随访时间19.5个月(9～26个月).17例患儿均实现初期愈合,平均愈合时间4.9个月(4.1～7.8个月),初期愈合率为100％.13例(76％)患儿存在胫骨不等长,平均胫骨不等长1.2 cm(0.5～2 cm);6例(35％)患儿发生胫骨近端外翻,平均外翻7.8°(5～16°),均行胫骨近端内侧“8”字形钢板螺钉半侧骺板阻滞术予以矫正;2例患儿发生踝外翻,分别为12°、17°.术后胫骨假关节愈合区域的横断面积平均增大至胫骨远近端骨干处横断面积的1.74倍(1.14～2.60倍).17例患儿均未发生再骨折.5例患儿恢复踝关节活动,平均背伸22°(20～30°),平均跖屈41°(40～50°);另外12例踝关节仍固定于中立位.结论 在联合手术同时行三合一骨融合术治疗伴腓骨完整的先天性胫骨假关节初期愈合率高,胫骨假关节愈合处横断面积增加,短期疗效良好.     

Ilizarov技术治疗儿童下肢畸形31例

儿童下肢畸形虽有很多治疗方法可供选择 ,但往往面临很多困难 ,效果也不理想 ,本文介绍我院采用Ｉｌｉｚａｒｏｖ技术治疗儿童下肢畸形的临床经验 ,并就有关问题进行讨论。资料和方法一、临床资料1991～ 1998年我院共收治 31例下肢畸形患儿 ,详细情况见表 1。其中 2例先天性胫骨假关节及 4例骨髓炎后骨不连均接受过一次以上的植骨内固定手术 ,8例先天性马蹄内翻足中 5例均接受过常规的手术治疗。本组 31例下肢畸形采用Ｉｌｉｚａｒｏｖ技术治疗 ,均获预期目的。二、Ｉｌｉｚａｒｏｖ技术治疗Ｉｌｉｚａｒｏｖ技术治疗先天性胫骨假关节采取交…     

Successful Retreatment of a Child with a Refractory Brainstem Ganglioglioma with Vemurafenib

A child with brainstem ganglioglioma underwent subtotal resection and focal radiation. Magnetic resonance imaging confirmed tumor progression 6 months later. Another partial resection revealed viable BRAF V600E‐positive residual tumor. Vemurafenib (660 mg/m²/dose) was administered twice daily, resulting in >70% tumor reduction with sustained clinical improvement for 1 year. Vemurafenib was then terminated, but significant tumor progression occurred 3 months later. Vemurafenib was restarted, resulting in partial response. Toxicities included Grade I pruritus and Grade II rash. Vemurafenib was effectively crushed and administered in solution via nasogastric tube. We demonstrate benefit from restarting vemurafenib therapy.     

Leukemia in a Child with a History of Medulloblastoma

Leukemia of mixed lineage, was diagnosed in a 6.5-year-old boy with a history of medulloblastoma, 38 months after his initial cancer diagnosis. Therapy had included craniospinal radiation and nitrosourea-based chemotherapy. In addition, onset of leukemia was preceded by therapy with recombinant growth hormone for short stature. Although rare, leukemia is a treatment-related complication for patients with past brain tumors whose follow-up should therefore include surveillance with complete blood counts.     

Psychosocial needs of families with a child with cancer

PURPOSE: The aims of this study were to map the problems and needs of children with cancer and their families with regard to possible psychosocial interventions, and to do an acceptability study of different ways to provide support. METHODS: The authors performed a cross-sectional structured telephone interview with 56 parents of children with cancer and 13 adolescents from these families. On 0 to 10 analog scales, parents and adolescents rated the importance of different needs, how these needs had been met, the acceptability of different ways of providing supportive interventions, how often these ways had been used, and comfort using them. RESULTS: Parents' mean rating of importance of information needs was 9.42, peer social support 7.84, and self-management therapy 9.21. The ratings of how well these needs had been met were 8.05, 5.30, and 7.13, respectively. Both parents and adolescents ranked getting information written on paper highest, preferred to communicate in a face-to-face support group for peer social support, and preferred a therapist for self-management therapy. The comfort ratings for using different ways to provide the interventions were all high, as was access; 89% of families had computers in their homes, 76% had Internet access. CONCLUSIONS: The needs for information, peer social support, and self-management therapy are all high. There is still room to meet these needs better. Using paper-based, telephone, computer CD, or an interactive Web-based intervention package all seem to be acceptable and accessible ways to meet the needs and might reduce the risk of families developing psychosocial problems.     

Peritoneal metastasis of a pineoblastoma in a patient with a ventriculo-peritoneal shunt

A 12 years old child had an increase of intra cranial pressure secondary to a large pinealoblastoma. A ventriculo peritoneal shunt procedure was performed followed several days later by a partial resection of the pinealoblastoma. One year later, ultrasound and computed tomography examinations discovered a solid mass in the pelvis. At surgery it appeared to be a metastasis of the pinealoblastoma. It is a very seldom complication of the shunt and various mechanisms are discussed.     

Hypnotherapy of a child with warts

Hypnosis was used to treat a 7-year-old female with 82 common warts. The lesions had been present for 12-18 months and were refractory to routine dermatologic treatment. Hypnotic suggestions were given for the facial warts to disappear before warts from the rest of the body. After 2 weeks, eight of 16 facial warts were gone, with no other changes. After three additional biweekly sessions, all 82 warts were gone. To the author's knowledge, this is the first reported case of systematic wart removal in children. Discussion focuses on the relationship between psychological mechanisms and the immune system.