Cleft palate in spondyloepiphyseal dysplasia congenita: case reports

Cleft palate is one of the common features of spondyloepiphyseal dysplasia congenita (SEDC). However, there are few clinical data about cleft palate in SEDC. We report four patients with cleft palate and SEDC including two with overt cleft palate and two with submucous cleft palate. Our results suggested that SEDC associated with cleft palate should be treated in the same way as solitary cleft palate, and submucous cleft palate may be more common in patients with SEDC than previously appreciated.     

单侧唇裂的鼻整形术

目的 探讨单侧唇裂鼻整形的优良手术方法。方法 采用切开患鼻鼻翼软骨与鼻侧软骨间的粘膜，形成软骨粘膜瓣，使鼻翼软骨外侧脚向上方突出、向内移位，以矫正鼻畸形。结果 52例一期鼻畸形矫正及18例二期矫正均达到较好的手术效果。结论 此术式更接近鼻生理解剖特点则一种值得提倡的手术方法。     

唇裂的鼻小柱侧面皮瓣修复法

目的为唇裂患者塑造一个正常唇的美好形态。改进现在常用的手术对人中嵴、人中窝和朱缘弓这三个重要美容结构的塑造中的缺欠,使修复后的上唇丰满、松动。方法在鼻小柱裂侧面形成一个矩形瓣,将其与人中一起垂直下降,补充裂侧唇人中的长度不足和保持朱缘弓在下降的正常位。结果 3年来,共用新法修复单侧唇裂23例,双侧唇裂9例,继发唇裂畸形14例。都没有常用方法的缺点。结论唇裂的鼻小柱侧面皮瓣修复法是较常用手术更符合整形外科从隐蔽处采取组织来修复组织缺损的原则的新方法,可以取得更好的形态效果。     

Rhinoplasty and Nasal Function in Patients with Cleft Lips

The purpose of this study was to find out whether harvesting septal grafts and simultaneously doing a septoplasty improves nasal function in patients with clefts who are having a rhinoplasty to correct the external deformity. We studied 14 patients with a unilateral cleft (UCL(P)) and four with bilateral clefts (BCLP). Nasal airflow resistance was measured with active rhinomanometry preoperatively and postoperatively. In nine of the 14 with UCL(P) and in all those with BCLP nasal resistance was normal or near normal both preoperatively and postoperatively. In five with UCL(P) postoperative nasal resistance was highly increased on the cleft side and in three of these patients nasal resistance decreased significantly but still remained abnormal. Only one patient subjectively complained of poor nasal airflow but all sought improvement of their nasal appearance. Harvesting of the graft and simultaneous straightening of the nasal septum during a rhinoplasty in patients with clefts neither improved, nor had a deleterious effect on, nasal function.     

Cleft hand in Kabuki make-up syndrome: case report

Kabuki make-up syndrome (KMS) is a multiple malformation/mental retardation syndrome that was first described in Japan but is now reported in many other ethnic groups. Kabuki make-up syndrome is characterized by multiple congenital abnormalities: craniofacial, skeletal, and dermatoglyphic abnormalities; mental retardation; and short stature. Common hand anomalies associated with KMS include persistent fingertip pad, brachydactyly, clinodactyly, and lax joints. We report a patient with KMS who presented with cleft hand, a feature that has not yet been described in KMS, and describe the potential genetic cause.     

双侧颊粘膜肌瓣腭裂修复术

目的探索腭裂修复减轻对上颌骨生长发育的影响,又能达到腭咽闭合。方法应用双侧颊粘膜肌瓣对腭裂进行修复手术。结果应用该手术方法修复16例腭裂患者,效果满意。结论该方法有以下优点:①手术不剥离硬腭区粘骨膜,对上颌骨的损伤很小,减轻了上颌骨的发育障碍;②采用两块带蒂颊粘膜且几瓣重建了腭帆提肌功能,促使异位的提肌悬吊再形成;③血运良好的颊粘膜肌瓣插入于软硬腭交界处,能使软腭充分后退,实现腭咽闭合,而且可有效地防止瘘孔的发生。