Facial clefts involving the midline in combination with intracranial anomalies: Case studies illustrating surgical treatment and medical substitution

Abstract Malm? and Uppsala have been regional centres for the treatment of cleft lip and palate since the beginning of the 1950s. We have about 80 new cases every year and most patients have conventional oronasal clefts, either cleft lip and palate or isolated cleft palate. During a 10-year period we have come across four patients who have had varying degrees of midface dysplasia combined with intracranial anomalies. One child died at an early age, but the other three children were given medical substitution of hypopituitarism and have had their clefts reconstructed.     

Speech and satisfaction with outcome of treatment in young adults with unilateral or bilateral complete clefts

Thirty-five young adults (22–32 years old, mean 25) born with complete unilateral or bilateral clefts participated in a follow-up of speech, appearance, and teeth after treatment. They completed a questionnaire about their satisfaction with their speech, nose, lip, and teeth on visual analogue scales, and indicated on two overall questions how often they thought about their cleft, and how often they were asked questions about their speech, nose, or lip. Their speech was recorded and assessed blindly and independently by two speech and language pathologists. Participants’ satisfaction with their speech did not correlate significantly with the speech assessments. Satisfaction with the nose had the highest correlation with the overall questions. No participant indicated more dissatisfaction with speech than the midpoint of the scale, making conclusions about covariance between satisfaction with speech and the overall questions difficult.     

33例颅面裂Tessier分类诊断与治疗体会

目的介绍颅面裂的Tessier分类诊断与手术治疗体会。方法按Tessier分类法对33例颅面裂患者进行分类，并根据畸形类型及轻重程度制定手术方案；共施术29例，手术方法包括单纯眶内侧壁截骨与鼻翼软骨修整、局部皮瓣转移、内眦固定、真皮移植、骨移植、组织代用品置入、吻合血管的游离组织瓣移植等。结果33例中属0号颅面裂1例、0—14号颅面裂3例、1号颅面裂1例、2-12号颅面裂2例、3号颅面裂3例、4号颅面裂1例、5／6号复合颅面裂1例、7号颅面裂18例、6／7／8号复合颅面裂2例、11号颅面裂1例；29例接受手术的患者，术后面部形态明显改善，无并发症发生。结论①Tessier颅面裂分类法有助于发现与主诉体征相伴的其它颅面畸形，对作出全面正确的诊断及制定手术方案具有重要的指导作用。②在Tessier颅面裂中以7号颅面裂较为常见。③Tessier颅面裂有不同的类型，且每一类型的颅面裂又有不同的严重程度，而且不同类型及不同严重程度的颅面裂又可见于同一患者，需根据患者具体情况制定个性化的手术方案。     

干细胞在唇腭裂患者牙槽突裂中应用的研究进展

干细胞具有自我更新能力及多向分化潜能,已被广泛应用于多个领域。相对于唇腭裂患者牙槽突裂的常规手术治疗方法,干细胞具有来源广泛、取材方便、免疫原性低等优点,整形外科医生已逐渐关注唇腭裂患者牙槽突裂干细胞治疗的研究。该文对胚胎干细胞、人间充质干细胞、颌面部来源干细胞在唇腭裂患者牙槽突裂中应用的研究进展进行了综述。     

Synovial sarcoma of the spine: a case involving paraspinal muscle with extensive calcification and the surgical consideration in treatment

Introduction

Synovial sarcoma is a rare malignant tumor of the spine. This tumor may present as a painless mass of the spine or slowly enlarge, causing pain or neurologic deficits. As it is difficult to differentiate this lesion from other soft tissue tumors, synovial sarcoma requires histologic confirmation for definite diagnosis. Thus, the treatment strategy is often planned in the final step depending on the pathologic results. Despite its rare incidence, a few cases of primary or metastatic synovial sarcoma involving the spinal cord, foramen, vertebral body, or paraspinal muscles have been reported in the literature.

Materials and methods

We present the case of a 29-year-old man with a synovial sarcoma in the paraspinal muscle of the cervical spine. The patient was evaluated radiologically and histologically. Plain radiography, computed tomography, and magnetic resonance imaging were performed as part of the preoperative workup, and immunohistochemical and cytogenetic studies were additionally performed to identify the histologic features of the tumor. The patient underwent marginal resection followed by adjuvant radiation therapy. The patient has been followed up for 2 years.

Conclusions

This article highlights the features of synovial sarcoma of the spine via a comprehensive review. Synovial sarcoma of the spine is uncommon, but it is a challenging issue in both diagnostic and therapeutic aspects. The currently available evidence suggests the use of a multidisciplinary approach in the treatment of synovial sarcoma, which includes complete resection and radiation therapy.