A comparison of the craniofacial morphology in 2-month-old unoperated infants with unilateral complete cleft lip and palate, and unilateral incomplete cleft lip.

This paper reports a cephalometric analysis of the craniofacial morphology in infants with unoperated unilateral complete cleft lip and palate (UCCLP) and unoperated unilateral incomplete cleft lip (UICL). The purpose of the study was to determine the nature and extent of the craniofacial deviations in UCCLP as compared to the morphology in UICL, which has previously been shown to be close to normal. The samples comprised 82 infants with UCCLP (58 males and 24 females) and 75 with UICL (48 males and 27 females). The mean age was about 2 months in both groups. The cephalometric analysis of craniofacial morphology included the lateral, frontal, and axial projections. The data were presented as mean plots of the craniofacial region including the calvaria, cranial base, orbits, nasal bone, maxilla, mandible, cervical column, pharynx, and soft-tissue profile. The most pronounced deviations in the UCCLP group were observed in the maxillary complex and the mandible. The most striking findings were: markedly increased width of the maxilla, a short mandible, and bimaxillary retrognathia except for the premaxillary area, which was relatively protruding and asymmetric. The study did not support the hypothesis previously suggested in the literature that cleft lip and palate is a craniofacial anomaly as size and shape of the calvaria and cranial base were found to be normal. The etiology of cleft lip and palate is still incompletely understood. Based on the present study, we suggest that facial type may be a liability factor that could represent a developmental threshold increasing the probability of cleft lip and palate.     

Growth status of children treated for unilateral cleft lip and palate

Cephalometric distances, angles, and proportions were evaluated for 32 children 5 to 8 years of age treated for unilateral cleft lip and palate. The children were age and sex matched with untreated controls with normal skeletal relationships. The unilateral cleft lip and palate sample was treated by the same surgeon and orthodontist using the same techniques and appliances. Measures of overall facial proportions, facial convexity, and prognathism were not significantly different between the two groups. The primary group differences pertain to the posterior aspect of the maxilla, which is vertically short in the unilateral cleft lip and palate sample. Horizontally, the maxilla of the unilateral cleft lip and palate children was significantly longer, producing a steeper palatal plane. In addition, the zygoma and orbits of unilateral cleft lip and palate children were somewhat retruded; the posterior cranial base and total mandibular length also were longer in the unilateral cleft lip and palate children.     

Lip pressure changes following lip repair in infants with unilateral clefts of the lip and palate

The present study was designed to quantitatively assess lip pressure changes following cleft lip repair in infants with unilateral cleft lip, alveolus, and palate. Lip pressure measurements were taken using an electronic transducer system developed especially for this study. Lip pressure was monitored from 3 months (preoperatively) through 2 years of age in cleft and normal control children. Findings from the present study confirm the hypothesis that lip repair in infants with unilateral cleft lip and palate significantly increases lip pressure and that increased lip pressure remains significantly higher than in normal control children for the 2-year duration of the study. Thus increased lip pressure when the palate is unrepaired has to be considered as a factor modulating subsequent craniofacial growth.     

Early palatal changes in complete and incomplete cleft lip and/or palate.

Early palatal development in various complete and incomplete forms of cleft lip and/or palate (CLP) was studied from birth to 3 months of age by means of dental casts. Palatal morphology (shape) and dimensions--based on reproducible reference points--were determined in a group of 128 CLP children and 68 normal children who served as controls. Substantial normal palatal growth during the first 3 months of life was observed. Round arch forms changed into oval arch forms. Growth mainly takes place in the sagittal direction (+4 mm) (transverse: +1 mm). Palates of CLP children differed significantly dependent on the type of cleft and whether the cleft was complete or incomplete. Cleft lip and alveolus children and bilateral cleft lip and palate children had more elongated palatal arches, whereas unilateral cleft lip and palate children and cleft palate children had wider palatal arches than the control group. Incomplete clefts differed from the control group in the same direction as their complete cleft forms, though less distinctly. Preoperative orthopedics used in CLP patients does not stimulate growth. On the contrary, it even restricts growth.     

A model for fetal cleft lip repair in lambs.

Fetal wounds heal without inflammation and scar formation. This phenomenon may, in the future, be applicable to human cleft lip and palate repair. However, extensive experimental work must first be done to document the benefits of in utero repair. We developed a large animal model for creation and repair of a complete cleft lip and alveolus using fetal lambs. The cleft lip and alveolus deformity was created in eight 75-day-gestation fetuses (term = 145 days) and either repaired in three layers or left unrepaired. There were four sham-operated fetuses, and all animals were alive at harvest. Repaired, unrepaired, and control fetuses were harvested at 7, 14, 21, and 70 days following surgery. The unrepaired fetuses demonstrated a complete cleft lip and alveolus with an oronasal fistula. The maxilla was asymmetrical, with the greater segment deviated toward the cleft and with decreased anterior maxillary width. In contrast, repaired cleft lip and alveolus animals showed no scar, normal thickness of the lip, and a symmetrical maxilla. Histologic analysis of the repaired wounds showed evidence of tissue regeneration without scar formation. The results of this preliminary study indicate that the fetal lamb cleft lip and alveolus model is technically feasible with an excellent survival rate. Healing occurs without scar formation. In the repaired animals, the maxilla was symmetrical. This model will be used to document facial growth following in utero repair of a cleft lip and alveolus.     

Binderoid complete cleft lip/palate

A small subset of infants with complete cleft lip/palate look different because they have nasolabiomaxillary hypoplasia and orbital hypotelorism. The authors' purpose was to define the clinical and radiographic features of these patients and to comment on operative management, classification, and terminology. The authors reviewed 695 patients with all forms of incomplete and complete cleft lip/palate and identified 15 patients with nasolabiomaxillary hypoplasia and orbital hypotelorism. All 15 patients had complete labial clefting (5 percent of 320 patients with complete cleft lip/palate), equally divided between bilateral and unilateral forms. The female-to-male ratio was 2:1. Of the seven infants with unilateral complete cleft lip/palate, one had an intact secondary palate and all had a hypoplastic septum, small alar cartilages, narrow basilar columella, underdeveloped contralateral philtral ridge, ill-defined Cupid's bow, thin vermilion-mucosa on both sides of the cleft, and a diminutive premaxilla. Of the eight infants with bilateral complete cleft lip, one had an intact secondary palate. The features were the same as in patients with unilateral cleft, but with a more severely hypoplastic nasal tip, conical columella, tiny prolabium, underdeveloped lateral labial elements, and small/mobile premaxilla. Central midfacial hypoplasia and hypotelorism did not change during childhood and adolescence. Intermedial canthal measurements remained 1.5 SD below normal age-matched controls. Skeletal analysis (mean age, 10 years; range, 4 months to 19 years) documented maxillary retrusion (mean sagittal maxillomandibular discrepancy, 13.7 mm; range, 3 to 17 mm), absent anterior nasal spine, and a class III relationship. The mean sella nasion A point (S-N-A) angle of 74 degrees (range, 65 to 79 degrees) and sella nasion B point (S-N-B) angle of 81 degrees (range, 71 to 90 degrees) were significantly different from age-matched norms ( = 0.0007 and = 0.004, respectively). The ipsilateral central and lateral incisors were absent in all children with unilateral cleft, whereas a single-toothed premaxilla was typically found in the bilateral patients. Several modifications were necessary during primary nasolabial repair because of the diminutive bony and soft-tissue elements. All adolescent patients had Le Fort I maxillary advancement and construction of an adult nasal framework with costochondral or cranial graft. Other often-used procedures were bony augmentation of the anterior maxilla; cartilage grafts to the nasal tip and columella; and dermal grafting to the median tubercle, philtral ridge, and basal columella. Infants with complete unilateral or bilateral cleft lip/palate in association with nasolabiomaxillary hypoplasia and orbital hypotelorism do not belong on the holoprosencephalic spectrum because they have normal head circumference, stature, and intelligence, nor should they be referred to as having Binder anomaly. The authors propose the term cleft lip/palate for these children. Early recognition of this entity is important for counseling parents and because alterations in standard operative methods and orthodontic protocols are necessary.     

The influence of lip repair with and without soft-tissue undermining on facial growth in beagles

The present study was designed to test the hypothesis that undermining of the soft tissues on the surface of the maxilla at the time of lip repair in unilateral cleft lip, alveolus, and palate results in more severe craniofacial growth aberrations than lip repair alone. Sixty-seven purebred beagles were used in this experiment. The animals were divided into four groups: two control groups (unoperated and unrepaired) and two experimental groups (lip repair without undermining and lip repair with undermining). Lip pressures were monitored in all groups. Significantly higher lip pressures were observed in animals with soft-tissue undermining. Cephalometric measurements were analyzed using univariate and multivariate techniques. The results of this study indicate that lip repair performed with soft-tissue undermining results in more severe craniofacial growth aberrations than lip repair performed alone.     

Simultaneous cleft lip and palate repair: an experimental study in beagles

This study was designed to test the hypothesis that simultaneous lip and palate repair results in more severe craniofacial growth aberrations than lip repair or palate repair performed separately. Seventy-six purebred beagles were divided into five groups. Two of these groups were controls (unoperated and unrepaired animals); the three remaining groups were experimental (in one group only the lip was repaired, in another only the palate was repaired, and in the last the lip and palate were repaired simultaneously). Cephalometric measurements were analyzed using univariate and multivariate statistical techniques. In multivariate analysis, stepwise multiple regression and discrimination were applied to precisely assess the effects of the various surgical procedures. The results of this study indicate that simultaneous lip and palate repair results in more severe craniofacial growth aberrations than lip repair or palate repair performed separately.     

Cleft lip, cleft palate, and velopharyngeal insufficiency

This article provides an introduction to the anatomical and clinical features of the primary deformities associated with unilateral cleft lip-cleft palate, bilateral cleft lip-cleft palate, and cleft palate. The diagnosis and management of secondary velopharyngeal insufficiency are discussed. The accompanying videos demonstrate the features of the cleft lip nasal deformities and reliable surgical techniques for unilateral cleft lip repair, bilateral cleft lip repair, and radical intravelar veloplasty.     

Primary correction of unilateral cleft lip nasal deformity in Asian patients: anthropometric evaluation

Previously it was thought that primary correction of nasal deformity in cleft lip patients would cause developmental impairment of the nose. It is now widely accepted that simultaneous correction of the cleft lip nasal deformity has no adverse effect on nasal growth. Thus, the authors tried to evaluate the results of primary correction of cleft lip in Asian patients. Of 412 cases of cleft lip, 195 cases were corrected by means of the conventional method from June of 1992 to June of 1997, and 217 cases were corrected by simultaneous rhinoplasty from July of 1997 to October of 2001. The average patient age was 3 months. Photographs and anthropometric evaluation were used to evaluate the results. Nasal tip projection, columellar length, and nasal width were measured in 60 randomized normal children, 30 randomized children treated with the conventional method, and 30 randomized children with primary nasal repair. Data were analyzed using t tests, and the level of significance was 5 percent (p < 0.05). In cases of simultaneous repair, nasal tip projection and columellar length were increased 24.8 percent and 28.8 percent, respectively. Nasal width was increased 12.3 percent in the cases of simultaneous repair and 12.6 percent in the cases without primary rhinoplasty. Simultaneous repair of cleft lip and nasal deformity in Asian patients showed that more symmetry of nostril and nasal dome projection and better correction of buckling and alar flaring were achieved. More balanced growth and development of the alar complex was achieved, and no interference with nasal growth was encountered.     

A prospective evaluation of the prevalence of submucous cleft palate in patients with isolated cleft lip versus controls.

Although there is an established relationship between cleft lip and overt cleft palate, the relationship between isolated cleft lip and submucous cleft palate has not been investigated. To test the hypothesis that patients with isolated cleft lip have a greater association with submucous cleft palate, a double-armed prospective trial was designed. A study group of 25 consecutive children presenting with an isolated cleft lip, with or without extension through the alveolus but not involving the secondary palate, was compared with a control group of 25 children with no known facial clefts. Eligible patients were examined for the presence of physical criteria associated with classic submucous cleft palate, namely, (1) bifid uvula, (2) absence of the posterior nasal spine, and (3) zona pellucida. Nasoendoscopy was subsequently performed just after induction of general anesthesia, and the findings were correlated with digital palpation of the palatal muscles. Patients who did not satisfy all three physical criteria and in whom nasoendoscopy was distinctly abnormal relative to the control group were classified as having occult submucous cleft palate. Classic submucous cleft palate was found in three study group patients (12 percent), all of whom had flattening or a midline depression of the posterior palate and musculus uvulae on nasoendoscopy and palpable diastasis of the palatal muscles under general anesthesia. An additional six study group patients (24 percent) had similar nasoendoscopic criteria and palpable diastasis of the palatal muscles; they were classified as having occult submucous cleft palate. No submucous cleft palate was identified in the control group. Seventeen patients in the study group had an alveolar cleft with a 53 percent (9 of 17) prevalence of submucous cleft palate. In the present study, classic submucous cleft palate in association with isolated cleft lip was 150 to 600 times the reported prevalence in the general population. All children with an isolated cleft lip should undergo peroral examination and speech/resonance assessment no later than the age of 3 years. Any child with an isolated cleft lip with velopharyngeal inadequacy or before an adenoidectomy should be assessed by flexible nasal endoscopy to avoid missing an occult submucous cleft palate.     

Primary definitive nasal correction in patients presenting for late unilateral cleft lip repair

Almost 25 percent of unilateral cleft lip and palate patients present with their deformity in their teens or later years in the developing world. Because more than 80 percent of the world population lives in the developing world, the established protocol for repair of these deformities is not applicable to these patients. Despite the magnitude, there are no significant reports in the literature that deal with this problem. Several issues need to be addressed, but the author limits himself here to the correction of the nasal deformity. The patients at this age are very much concerned with the aesthetic outcome. Procedures described hitherto for primary nasal correction in infants are not successful in restoring nasal shape and symmetry at this late age of presentation. Our experience with radical correction of secondary nasal deformity in unilateral cleft lip patients presenting late prompted us to extend the concept by undertaking a definitive primary correction of the nasal deformity in cleft patients presenting late. Twenty-two patients with unilateral cleft lip deformity (nine male patients and 13 female patients) with ages ranging from 13 to 22 years, presenting between August of 1997 and December of 2000, are included in this study. Of these, 11 patients had a cleft of the lip alone, eight also had a cleft of the alveolus, and three had a cleft of the palate continuous with the cleft lip. All patients showed some maxillary hypoplasia. An external rhinoplasty with lip repair was carried out in all patients. The corrective procedures on the nose included columellar lengthening; augmentation along the pyriform margin, nasal floor, and alveolus using bone grafts; submucous resection of the nasal septum; repositioning of lower lateral cartilages; and augmentation of nasal dorsum by bone graft. Clinical follow-up ranged from 4 to 24 months, and the median follow-up period was 13 months. Results have been very good, and much better than results seen earlier with other primary rhinoplasty techniques. While repairing unilateral cleft lip in adolescents, the author thinks it would be most appropriate to address the entire gamut of the deformity in a single stage, provide complete vector reorientation, and augment the hypoplastic elements by autologous tissue. It is not just the fear of poor follow-up, but that merely correcting the lip deformity in these patients without attempting definitive rhinoplasty, in the author's opinion, would be insufficient surgical intervention.     

Characteristics of the hearing loss in unilateral cleft lip and palate-influence on communication

Etiology of otitis media with effusion (OME) is still unclear and often described as multi-factorial. It is very usual finding in cleft palate population. We tested relationship between the hearing level, audiometric frequencies, aging and ear side in unilateral cleft lip and palate 101 children (UCLP) and subgroups of left (UCLP)(L) and right cleft side (UCLP)(R). Group of left ears is prone to higher frequency and more severe hearing disturbances than groups of right ears, with less chance of normalizing hearing level with aging. Characteristics of hearing loss level and its improvement, in UCLP children depend of cleft type, ear side and age group.     

Orthopedic intrusion of premaxilla with distraction devices before alveolar bone grafting in patients with bilateral cleft lip and palate

Surgical repositioning of the downward displaced premaxilla in bilateral cleft lip and palate patients remains a controversial and perplexing issue because of its detrimental effects on the growth of the premaxilla. The purpose of this prospective clinical study was to introduce and evaluate the treatment results of an innovative technique for nonsurgically intruding the downward displaced premaxilla. Eight consecutive cases of bilateral cleft lip and palate at the age of mixed dentition were included for the correction of their premaxillary deformities. A pair of intraoral tooth-borne distraction devices was used for the orthopedic intrusion. Serial lateral and posteroanterior cephalometric radiographs were taken periodically for evaluating the growth of the premaxilla 1 year before the intrusion, changes during the intrusion, and growth/relapse up to 1 year after the intrusion. There was no overgrowth of the premaxilla or overeruption of the maxillary incisors during the 1-year observing period before the orthopedic intrusion. The treatment results revealed that the downward displaced premaxillae were all corrected within 1 month. Cephalometrically, 46 percent of the correction resulted from a true orthopedic intrusion and another 54 percent from a dentoalveolar effect in which the maxillary incisors were intruded and the premaxillary dentoalveolus was shortened. The cephalometric evaluations also implied that what occurred during the orthopedic intrusion was mostly the sutural contraction osteogenesis/osteolysis in the vomeropremaxillary suture combined with slightly mechanical upward displacement of the vomeronasal septum complex and nasal bones. The orthopedic intrusion of the premaxilla with distraction devices is an effective nonsurgical method for correcting the downward displaced premaxilla before alveolar bone grafting in patients with bilateral cleft lip and palate, and the results remained stable after 1 year.     

Lip height and lip width after extended Mohler unilateral cleft lip repair

The purpose of this study was to evaluate the symmetry in lip height and lip width after extended Mohler unilateral cleft lip repair, with long-term follow-up monitoring. In the Mohler repair procedure, Millard's C-flap is used to fill the entire defect created by the downward rotation of the medial lip element. Because a lateral advancement flap is not transposed into this defect, Mohler repair is often expected to produce a short lip. In a retrospective study of 120 patients, anthropometric measurements were made on black-and-white photographs. Of those patients, 49 met the study criterion of having a set of photographs taken 13 months or less postoperatively and another set taken at least 2 years postoperatively. The distance from the Cupid's bow peak to a line tangent to the base of the columella (lip height) and the distance from the Cupid's bow peak to the ipsilateral commissure (lip width) were measured with a Vernier caliper. The medial intercanthal distance was also measured, for standardization of all measurements. All values were normalized to the mean intercanthal distance at age 6, as reported by Farkas. Matched-pair test analyses were used to assess the statistical significance of differences in cleft-side versus non-cleft-side measurements for each group, as well as changes with time. No statistically significant difference in cleft side versus non-cleft-side lip height for the two groups or with time was observed (< or =13 months, p = 0.28; >2 years, p = 0.08; change with time, p = 0.69). Statistically significant differences in lip width between the cleft side and the non-cleft side were observed for both time groups. The average difference in lip width at 1 to 13 months was 8.6 percent (p < 0.001). The average difference in lip width at 2 years or more postoperatively was 5.8 percent (p < 0.001). In comparisons of early versus late measurements, it was noted that lip width significantly increased with time (mean, 0.91 mm; p = 0.035). The findings suggest that extended Mohler repair does not produce a short lip. Interestingly, lip width was observed to be significantly smaller on the cleft side in the immediate postoperative period. However, this deficiency was observed to decrease significantly during long-term follow-up monitoring.     

Maxillofacial growth in children with unilateral cleft lip and palate following secondary alveolar bone grafting: an interim evaluation

This study evaluates the effect of alveolar bone grafting on the maxillofacial growth in children of mixed dentition with unilateral complete cleft lip-cleft palate. Twenty patients received an iliac crest alveolar bone graft between the ages of 6 years 10 months and 10 years 10 months, whereas 20 matched controls between the ages of 6 years 11 months and 10 years 6 months did not. Geometric morphometric assessments were used to localize alterations between the initial and final cephalographs in the two groups of cleft children, using Procrustes analysis and thin-plate spline analysis, in addition to conventional cephalometric techniques. It is concluded that no statistically significant difference in maxillofacial growth was found between the cleft children having received secondary alveolar bone grafting and the nongraft controls in general during the first to third postoperative years. Further investigation will be undertaken to determine the long-term effects after the age of skeletal maturity.     

唇瘘、腭裂及唇裂综合征两个家系的遗传分析

我们在此报道北京地区没有亲缘关系的两个家庭中属于van der woude综合征的常染色体显性遗传病,其临床症状包括下唇近中线处唇瘘(下唇有先天性向下的瘘管)、腭裂、唇裂(具有或不具有腭裂)。这三个临床症状可以同时发生在一个受累个体身上,或任何两个症状出现在一个个体身上。这种人携带了这种致病基因。在我们所报道的家庭中,先证者每人还具有舌系带过短的体征。     

Upper lip measurements at the time of surgery and follow-up after modified rotation-advancement flap repair in unilateral cleft lip patients

The purpose of this prospective study was to determine whether unilateral cleft lip repaired by the rotation-advancement flap will grow short on the repaired side. This study involved 56 patients with nonsyndromic unilateral cleft lip (31 with complete and 25 with incomplete cleft lip) who underwent a rotation-advancement flap repair by a single surgeon between 1989 and 1997. Eleven patients were lost to follow-up. Forty-five patients have been followed for a varying period of between 8 and 84 months (mean = 37 months). The upper lip was measured immediately after the lip repair and follow-up using calipers. The growth ratios of vertical, horizontal, and nostril sill dimensions were compared between the cleft side and the noncleft side of the same face. Statistical analysis was performed to compare the growths between the cleft and noncleft sides. There was not a significant difference in the growth ratios of vertical (Wilcoxon signed rank test, p = 0.85) and horizontal dimensions (Student's t test, p = 0.18) between the cleft and noncleft sides. There was, however, a statistically significant difference in the growth ratios of nostril sill width between the cleft and noncleft sides (Student's t test, p = 0.02). Our findings indicated that a repaired unilateral cleft retained the vertical and horizontal dimensions determined at the time of the initial repair.     

Modified technique of presurgical infant maxillary orthopedics for complete unilateral cleft lip and palate

This article introduces a modified device fabrication and facial taping method that increases the efficiency and efficacy of presurgical infant maxillary orthopedic therapy for babies born with complete cleft lip and palate. Interarch and intra-arch relationships of the maxillary and mandibular dental arches were evaluated on mounted stone models before and after treatment. The palatal plate device was custom-fabricated in a manner that bypassed the need for periodic acrylic addition and removal, thereby eliminating the risk of natural maxillary growth restriction during therapy. Elastic labial tapes were fabricated and applied in a configuration that mimicked normal function of the orbicularis oris muscle. A nasal stent wire was utilized from the initiation of therapy to enhance intraoral retention and stability of the device. Examples of infants undergoing a unilateral complete cleft lip and palate treatment protocol are presented. Treatment objectives were achieved within 7 to 8 weeks of therapy for patients who had an initial alveolar cleft size more than 10 to 12 mm. The modified protocol of presurgical infant maxillary orthopedic therapy is an effective and efficient treatment modality in reversing the pre-existing orofacial dysmorphism by redirecting the infant's natural growth.