Gastroschisis associated with cleft lip and palate

Gastroschisis is a common congenital abdominal wall defect. Rarely, it is associated with extraabdominal midline congenital anomalies. Oral clefts are the commonest craniofacial congenital midline defects. They can be associated with other midline defects like omphalocele. We believe these are the first 3 case reports of gastroschisis and cleft palate occurring in the same patient.     

Midline cleft of the lower lip associated with Robinow syndrome

Robinow syndrome (also named “fetal face syndrome”) includes a series of anomalies including mesomelic brachymelia, bifid terminal phalanges of the hands and feet, abnormalities of vertebrae and ribs, and hypoplastic external genitalia. A midline cleft of the lower lip and mandible is an extremely rare maxillofacial deformity. Seventy cases have so far been described to our knowledge. We report a patient with Robinow syndrome and midline cleft of the lower lip and mandible and describe the reconstruction of these anomalies. We propose that this anomaly should be added to the range of malformations associated with the syndrome.     

Secondary rhinoplasty in nasal deformity associated with the unilateral cleft lip

The secondary nasal deformity of the unilateral cleft lip involves a retrodisplaced dome of the ipsilateral nasal tip, hooding of the alar rim, a secondary alar-columellar web, and other deficiencies. The sliding-flap cheilorhinoplasty effectively corrects these deformities using a laterally based chondrocutaneous flap. We modified this technique by using an open rhinoplasty approach with the laterally based chondrocutaneous sliding flap. Columellar struts and shield grafts were some of the techniques combined with this approach to produce optimal results. In a total of 7 patients, we performed both the original sliding-flap cheilorhinoplasty and the modified open rhinoplasty, which are described and discussed herein. The laterally based sliding-flap cheilorhinoplasty is an effective technique for correcting both the secondary nasal deformity and the lip scar associated with the unilateral cleft lip.     

Midline cleft of the lower lip associated with Robinow syndrome.

Robinow syndrome (also named "fetal face syndrome") includes a series of anomalies including mesomelic brachymelia, bifid terminal phalanges of the hands and feet, abnormalities of vertebrae and ribs, and hypoplastic external genitalia. A midline cleft of the lower lip and mandible is an extremely rare maxillofacial deformity. Seventy cases have so far been described to our knowledge. We report a patient with Robinow syndrome and midline cleft of the lower lip and mandible and describe the reconstruction of these anomalies. We propose that this anomaly should be added to the range of malformations associated with the syndrome.     

Lengthening of the short columella associated with bilateral cleft lip.

Operations for lengthening the columella can be classified into three groups on the basis of source of material: lip, nose, or ear. When seen in profile, the middle of the lip provides one-fourth to one-third of the forward projection of the columella as compared with the level of the alar bases. Complete bilateral cleft lips usually benefit from columellar lengthening, but symmetrical, incomplete clefts rarely require it. Ancillary procedures, such as correction of a retruded maxilla by Le Fort I osteotomy or by contour build-up, can enhance the overall result. Our experience with advancement of skin from the floor of the nose and ala [15], skin from the alar margins at the tip [7], prolabial advancement flaps, fork flaps, and composite earlobe grafts is reported.     

Bilateral cleft lip

Directing the course of care for a patient with bilateral cleft lip problems is a great responsibility and challenge for the surgeon, and it requires full commitment, effort, and talent. The goal is to enable the patient to develop anatomically, functionally, and psychologically as normally as possible and to achieve and succeed in life according to natural abilities, unimpeded by the congenital deformity complex. An individualized and organized effort must continue until the child is grown. One important aspect of this effort is the technical cleft lip repair. No matter how well done, perfection is always a step away. Open minds and energetic young students should be reluctant to accept the "status quo"; they should be encouraged to accept this challenge and make contributions. Although many other "big, new" operations may be very dramatic, none will be more satisfying to the surgeon than the well-performed bilateral cleft lip repair. The surgeon must remember that the day of cleft lip repair is one of the single most important days of a person's life. The result will have meaning from that moment on!     

Bilateral cleft lip

The surgeon's objectives are normal nasolabial appearance and normal speech. The principles for synchronous repair of bilateral cleft lip have been established, and the techniques continue to evolve. Primary repair impairs maxillary growth, but little can be done at this time except to practice gentle craftsmanship and to minimize tension on the lower labial closure. The cutaneous lip should never be reopened for revision, and the number of secondary procedures involving the nasal cartilages should be kept to a minimum. Many adolescents with repaired bilateral cleft lip need maxillary advancement to improve projection of the nasal tip, to protrude the upper lip, and to attain normal sagittal skeletal harmony. With expected improvements in the technology of distraction osteogenesis, maxillary advancement may someday become as acceptable as orthodontic treatment.     

Early repair for infants with cleft lip. Retrospective study of 263 cleft lip repairs

This study attempts to define the effect of early repair on the surgical procedure and immediate outcome of cleft lip surgery. The first part deals with 263 consecutive cleft lip repairs (218 infants) categorized retrospectively by age at operation. 123 patients (150 cleft lips) were operated on during the first 4 weeks of life; a subgroup of 40 infants was operated on at a week or less of age; 95 patients (113 cleft lips) were operated at an older age (1 to 12 months). There were no significant difference in immediate surgical result between the groups and there was no apparent difference in the operative results as defined by whether or not the child needed subsequent revision. We are currently encouraging early repair in the full-term baby as the optimum method of management of newborns with cleft lip.     

A rare patient with a false median cleft lip associated with multiple congenital anomalies

A girl manifesting a false median cleft lip associated with hypertelorism, anophthalmos, hydranencephaly with holoprosencephaly, and osteogenesis imperfecta, among other disorders, survived and was fed mainly milk products up to the age of 11. Then, surgical closure of the false median cleft lip was undertaken to improve the face aesthetically and to make the child more acceptable to society at large. The patient's median cerebrofacial dysgenesis with other anomalies was unique, compared with disorders reported in the literature. The embryological distinction between a false and true median cleft lip is also discussed.     

双侧唇裂或唇腭裂修复后口哨畸形的分级与修复

目的 对双侧唇裂或唇腭裂修复后口哨畸形的严重程度进行分级。方法 将我院136例双侧唇裂或双侧唇腭裂术后上唇口哨畸形患者作为研究对象，观察上颌前门齿牙冠及相应牙龈暴露的程度将口哨畸形分为Ⅳ级。Ⅰ级： 牙冠显露1／2；Ⅱ级： 牙冠全显露或 牙冠全显露伴 显露近中纵行牙冠1／2—2／3；Ⅲ级： 牙冠全显露和附丽龈显露1／2或 牙冠全显露伴附丽龈显露1／2及 牙冠显露2／3以上；Ⅳ级： 牙冠及相应牙龈全显露伴 近中牙冠纵显露2／3以上。口哨畸形严重程度与双侧唇裂或唇腭裂修复术式的关系一并进行分析。结果 口哨畸形Ⅰ级者60例，占44．2％；Ⅱ级者47例，占34．5％；Ⅲ级者16例，占11．8％；Ⅳ级者13例，占9．5％。结果 显示Ⅰ级和Ⅱ级口哨畸形明显多于Ⅲ级与Ⅳ级，前者为后者的3．7倍。结论 对双侧唇裂或唇腭裂修复后口哨畸形的分级具有良好的临床参考价值，为修复上唇口哨畸形选择修复方法提供了客观依据。     

One-stage correction of severe nasal deformity associated with a unilateral cleft lip.

Correction of one of the abnormal features is never enough to alleviate the entire deformity in a patient with a unilateral cleft lip and deformed nose, because the factors that led to the nasal deformity have already produced changes in the lower lateral cartilage, nostril, columella, septum, and alar base. These changes should be treated together with the contributory factors. By restoring the balance of the muscles by primary repair of the lip, orthodontic treatment, and orthognathic surgery, a more symmetric skeletal base can be established. The method of repair of the lip will also influence the deformity. Bone grafting can augment the skeletal base, but it will not improve the position of the lower lateral cartilage or the deviated septum. In this paper we describe a single stage correction of all the irregularities of the nose in patients with unilateral clefts which alleviates the asymmetrical nasal deformity.     

Unilateral cleft lip repair

An accounting is made of twenty years' experience with a triangular flap method of cleft lip repair using a 60 degree tip angle. The inherent difficulty in standardizing the method to ensure a perfect length has been overcome by reducing the problem to a mathematical one--with a mathematical solution. A cleft lip pattern has been designed to facilitate and ensure an accurate end result.