Congenital midline cervical cleft.

Congenital midline cervical cleft (CMCC) is a rare disorder of the ventral neck that is clinically evident at birth and must be differentiated from the more common thyroglossal duct cyst. The case of CMCC presented here was associated with chromosomes 13/14 de novo Robertsonian translocations as well as midline deformities including a sacral tuft and a minor tongue-tie. The case is presented as well as discussion of histopathology, embryology, and surgical treatment.     

Management of congenital midline cervical cleft

Congenital midline cervical cleft (CMCC) is a rare developmental defect of the anterior neck normally characterized by an atrophic mucosal plaque with a cranial nipple-like skin tag, a short caudal sinus, and may be attached to a subcutaneous fibrous cord of variable length. Clinically, patients present at an early age with, white females being the most commonly affected population. In addition to aesthetic concerns, CMCC can prevent full extension of the neck, result in micrognathia and torticollis, predispose patients to infection, and can coexist with other clefting defects or cysts. Fewer than 50 cases have been published in the English-language literature. Herein, we report a case of CMCC that also presented with a mild contracture of the right sternohyoid muscle. The embryopathogenesis, histopathology, diagnosis, and treatment of this rare condition are also discussed.     

Atypical midline cleft with duplication of the metopic suture.

INTRODUCTION: In contrast to the common clefts of the lip, alveolus and palate, the atypical clefts of the face may come in myriad patterns of clinical expression and are often not easy to define. PURPOSE: In this report, a case of median craniofacial dysraphia is described. PATIENT: At presentation, the 3-month-old male patient had a bilateral complete cleft of the lip, alveolus and palate. The nose was wide and a horn was present on the nasal dorsum. 3-D CT AND MRI REVEALED: Duplication of the metopic suture ending at the wide anterior fontanel; orbital hypertelorism; midline cranial cleft ending just superior to the nasal dorsum; frontoethmoidal encephalocoele and holoprosencephaly. The presence of two metopic sutures was confirmed during surgery. CONCLUSION: The presented case carries the characteristics of the median cleft face syndrome. However, it differs from similar cases in two respects. First, the patient had two metopic sutures, one on either side of the cranial extension of the median cleft. Second, the patient had a bilateral cleft lip in contrast to the expected median cleft lip deformity.     

The effect of 1-stage versus 2-stage palate repair on facial growth in patients with cleft lip and palate: a review

The purpose of this review was to evaluate the effect of 1-stage versus 2-stage palate repair with delayed hard palate closure on facial growth in patients with cleft lip and palate. A literature survey from the PubMed database from January 1966 to December 2007 used the medical subject headings terms ‘facial growth’, ‘cleft lip and palate’, ‘cephalometry’, and ‘1-stage palate repair’ in combination with ‘2-stage palate repair’ or ‘delayed hard palate closure’. The Cleft Palate-Craniofacial Journal from 1964 to November 2007 was hand searched. Controlled studies written in English were selected. Two reviewers selected and extracted the data independently and assessed the quality of the studies. On the basis of this search only nine studies were included in this review. All studies were retrospective and non-randomized. Six studies were cross-sectional, and three were longitudinal. Heterogeneity and methodological deficiencies in the studies prevented conclusions regarding stage of palate repair and facial growth. Further well-designed controlled studies and long-term studies are needed, and researchers should focus on the variation in hard palate repair timing before 3 years of age and consider patient speech.     

Development of the residual cleft in the hard palate after velar repair in a 2-stage palatal repair regimen

Delayed closure of the hard palate is believed to improve maxillary growth and facial appearance in cleft lip and palate patients. However, the cleft opening in the hard palate after velar closure might impair speech development. The aim of this investigation was to study the development of the residual cleft in the hard palate after 2-stage palatal repair (TSPR) in children born with complete cleft lip and palate (bilateral [BCLP]; n=7 or unilateral [UCLP]; n=22) or isolated cleft palate (CP; n=9). Moreover, we aimed to investigate whether any morphologic factors before surgery might predict development of the residual cleft. Dental casts obtained prior to velar repair (mean age 7 months) and postoperatively at 1 1/2, 3, 4, 5 and 7 years were analyzed with a Reflex Microscope regarding the width, length and area of the cleft in the hard palate.The palatal cleft varied in size both pre- and postoperatively in all 3 types of cleft patients. The width of the cleft in the UCLP subgroup showed a marked reduction immediately after velar repair, but then, on average, remained stable until final surgical closure of the hard palate. In the BCLP subgroup the initially rather narrow width of the clefts remained unchanged postoperatively. Clefts in the CP subgroup, especially in those with a complete cleft, remained large after veloplasty. In 4 of the UCLP and 2 of the BCLP patients, the cleft width increased gradually. In some other subjects, both in the UCLP and BCLP subgroups, the residual cleft closed functionally with time, but this development could not be foreseen.     

Alveolar bone grafting in the treatment of midline alveolar cleft and diastema in incomplete median cleft lip

Median cleft lip is a rare congenital anomaly. The wide diastema with mesial tipping observed in these patients has been largely overlooked. A midline submucosal alveolar cleft prevents adequate treatment. The purpose of this article is to describe an alveolar bone grafting (ABG) technique used in the combined surgical-orthodontic approach to diastema treatment in patients presenting with incomplete median cleft lip. Patients treated for incomplete median cleft lip and diastema were identified in the clinic registry from 1981 to 2007. Six patients were identified; 4 underwent ABG before permanent maxillary incisor eruption, the other 2 were seen later when they were 11 years old. All 6 ABGs were successful. The incisors erupted through the graft or were successfully moved into it with lasting results. Follow-up ranged from 8 to 21 years. The existence of a midline submucosal alveolar cleft and subsequent diastema should be recognized and addressed in all patients who present with incomplete median cleft lip repair. This includes taking maxillary occlusal view X-rays before the age of 5 years to detect the cleft, and proceed to ABG if necessary, generally before permanent maxillary incisor eruption.     

Sinus floor augmentation technique and simultaneous implant placement, part II: the 2-stage approach

The 1-stage osteotome sinus floor augmentation and simultaneous implant placement technique has been previously reported as a simple solution to heighten the maxillary posterior region to nearly 5- to 6-mm bone height. In this report, a patient with initial bone height of 2 to 3 mm in the extracted site of #3 was treated with the 2-stage osteotome technique. In the first stage, the sinus floor was grafted and augmented up to 5 to 6 mm. In the second stage, 9 months later, the same area was regrafted and the implant was placed. Eight months after the second stage, radiographs showed the entire 10-mm length of the implant was covered with new bone and the implant was solid and functional.     

Cases of true and false median cleft with polypoid masses in the facial midline.

We describe cases of true and false median cleft combined with polyps. The polyp in the true median cleft was microscopically a skin polyp consisting of fatty tissue and was considered to be subsequent compensatory partial excess caused by impediment at the embryonic stage. The polyp in the false median cleft was considered to be a failure of the nasal septum derived from the nasal (ectethmoid) capsule. The features of these cases were considered of interest because they exhibited transient forms of the cleft. The histological findings of each polyp reflected the distinctive features of each median cleft.     

Facial morphology and growth following surgery for congenital midline cervical cleft patients

Congenital midline cervical cleft (CMCC) is a rare condition that consists of a cutaneous midline neck lesion with a sinus extending inferiorly towards the sternum. A fibrous band that extends superiorly to the mandible is a consistent feature of the condition. Restriction of growth of the mandible, possibly due to incomplete removal of the band, is the most significant long-term problem. It remains unclear whether early removal of the fibrous band might allow catch-up growth of the mandible. This study utilized non-invasive three-dimensional photographs to objectively evaluate the facial growth of six CMCC patients. The growth of these CMCC patients was compared to the average growth of age- and sex-matched controls from a database of three-dimensional facial photographs of clinically normal subjects. After surgical removal of the fibrous cord, CMCC patients experience growth in the chin at the same rate as in the normal population; no evidence was found for catch-up growth. As a result, individuals with CMCC are likely to require further surgical intervention to correct the residual retrognathia on completion of facial growth. Early excision of the lesion including aggressive resection of the fibrous band is still recommended, as this should optimize the early growth of the mandible in infancy.     

A 2-stage procedure combining maxillary advancement by distraction technique with mandibular setback surgery in patients with cleft lip and palate

A 2-stage procedure combining maxillary advancement by distraction technique with mandibular setback surgery was used to correct jaw deformities in 5 patients with severe maxillary retrusion secondary to cleft lip and palate. First, a Le Fort I maxillary osteotomy was performed. Immediately after maxillary distraction, the distraction device was removed. The advanced maxilla was fixed with miniplates after adjusting the length and direction of advancement, and mandibular setback surgery was performed simultaneously to obtain a normal occlusal relationship. This 2-stage procedure resulted in stable occlusion and a markedly improved facial profile.     

Treatment of the unilateral cleft lip nasal deformity

The lack of tip projection on the affected side of a unilateral cleft lip nasal deformity can be difficult to correct due to lack of adequate structural support. A new technique for the correction of the unilateral cleft lip nasal deformity is described. The key components of the technique involve the use of a dorsally angulated unilateral spreader graft on the cleft side and the use of an L-shaped septal graft to provide support to the cleft nasal tip.     

唇腭裂患者牙槽突裂的正畸治疗程序探讨

目的:唇腭裂伴牙槽突裂的上颌骨牙列矫正,常存在牙槽突裂隙区牙槽骨塌陷、牙龈高位、植入骨吸收等不理想状态,本研究回顾性分析8例患者,探讨上颌骨牙列矫治的治疗程序.方法:8例单侧牙槽突裂患者,在牙槽突裂植骨术同期行尖牙骨皮质切开,术后以正畸方法快速将尖牙向颌方、唇向移动,牙根移入植骨区.术前、术后1周、尖牙正轴完成后拍摄曲面体层片、尖牙根尖片、上颌咬合片和尖牙区照片,比较治疗前后尖牙区植骨量、尖牙牙根吸收程度和松动度变化;测量裂隙侧尖牙和邻牙之间的龈缘高度差和牙根之间的夹角,采用SPSS 17.0软件包对治疗前后各测量项目进行配对t检验,比较治疗前后结果有无统计学差异,评价植骨效果以及尖牙移动的有效性和安全性.结果:治疗后牙槽突裂植入骨高度均位于相邻牙牙根长度的1/2以上;裂隙侧尖牙牙根与邻牙牙根基本平行,牙根吸收均<2mm,龈缘高度较治疗前改建良好.结论:该治疗程序具有较强优势,在一次手术的基础上,使尖牙安全、有效进入裂隙区,保证了植入骨的丰满度和高度,重建了正常龈缘.     

面裂患者的口腔颌面矫治:Latham方法

术前婴儿口腔颌面矫治术(PSIOT)有助于在行唇部手术修复之前移动上颌骨和软组织裂。PSIOT的方法之一是使用Latham装置进行固定PSIOT。作者概述了这种方法以及将这些器具置入口腔内的步骤。前瞻性随机临床研究对于明确回答有关PSIOT长期效应的担忧是必要的。