Long-term effects of clefts on craniofacial morphology in patients with unilateral cleft lip and palate.

OBJECTIVE: To identify the long-term effects of clefts (intrinsic and functional) on craniofacial growth and to evaluate the possible association between the sizes of the cleft maxillary segment (intrinsic) and alveolar cleft (functional) and the craniofacial morphology in patients with unilateral cleft lip and palate (UCLP). DESIGN: Retrospective case-control study. SETTING: Sri Lankan Cleft Lip and Palate Project. SUBJECTS: Thirty unoperated adult patients with UCLP and 52 normal controls. MAIN OUTCOME MEASURES: Maxillary dental cast was used to measure the sizes of the cleft maxillary segment and alveolar cleft. Cephalometry was used to determine craniofacial morphology. RESULTS: Patients with UCLP had shorter height of the basal maxilla, shorter posterior length of the basal maxilla, and less protruded basal maxilla at the zygomatic level than did control subjects. In patients with UCLP, the posterior height of the basal maxilla was related to the size of the cleft maxillary segment, and there was a tendency toward significant association between the anterior height of the basal maxilla and the size of the alveolar cleft. CONCLUSION: The adverse effects of clefts on the growth of the maxilla in patients with UCLP are restricted to the basal maxilla in size. This growth inhibition is major in height and minor in length. The reduced posterior height of the basal maxilla in unoperated patients with UCLP might be primarily attributed to intrinsic effects, whereas the reduced anterior height of the basal maxilla might be attributed to functional effects.     

Parental craniofacial morphology in cleft lip with or without cleft palate as determined by cephalometry: a meta-analysis

OBJECTIVE: To integrate findings from previous cephalometric studies comparing the craniofacial complex of unaffected parents with cleft lip with or without cleft palate (CL/P) children to controls with no history of the disease. DESIGN: Meta-analysis of case-control cephalometric data. INCLUSION CRITERIA: Studies were selected if the unaffected parents of children with CL/P were included and were not combined with parents of children with isolated CP; quantitative data were obtained through cephalometry; the cephalometric variables used were not unique to a study; a case-control design was used; and the means and standard deviations for all variables were reported or could be calculated for both the experimental and the control group. OUTCOME MEASURE: Using raw data obtained from nine studies, mean weighted effect sizes with 95% confidence intervals were calculated for 28 cephalometric variables (mothers and fathers combined) or 18 variables (mothers and fathers separately). Heterogeneity statistics for the effect sizes were also calculated. RESULTS: In general, unaffected parents of children with CL/P possessed significantly wider interorbital, nasal cavity and upper facial dimensions, narrower cranial vaults, longer cranial bases, longer and more protrusive mandibles, shorter upper faces and longer lower faces compared with controls. Increased width of the nasal cavity was the most robust finding. Significant effect size heterogeneity was observed in roughly half of the variables examined. CONCLUSION: Unaffected parents of children with CL/P are characterized by a suite of consistent, yet subtle, craniofacial differences, which could indicate an underlying genetic liability.     

Hereditary cleft lip/palate and Wilms tumor: a rare association.

OBJECTIVE: The association of cleft lip/palate (CLP) with other anomalies is not uncommon, but its association with Wilms tumor (WT) is very rare, especially in a familial pattern. In this report, we present a family in which six members in two generations were affected with CLP, WT, or both. PATIENTS AND RESULTS: A male patient presented with right complete CLP. He had a family history of facial cleft and abdominal tumor. Lip repair was performed at 3 months of age. An abdominal mass was noticed at 12 months of age, which proved to be WT. Surgical excision of the tumor and chemotherapy were conducted. He subsequently underwent palate repair. His father had an unrepaired microform cleft lip. Three of his aunts were known to have similar problems: one had both facial cleft and WT, one had WT only, and the other had facial cleft only. One of his cousins also was affected with WT. CONCLUSIONS: This is a unique family affected with a rare association of CLP and WT. Pedigree study revealed an autosomal dominant hereditary pattern.     

Incidence of cleft lip, cleft palate, and cleft lip and palate among races: a review

A review of the literature pertaining to the incidence of cleft lip, cleft palate, and cleft lip and palate in different races is presented. The studies have been evaluated according to the method used to record the incidence rate. Half of the studies include in their base population livebirths, stillbirths, and abortions, or livebirths and stillbirths to record the incidence rate. In addition, in most of the studies, clefts with associated malformations and possible syndromes are included in the reported incidence. There is evidence, however, to suggest that the risk of developing clefts in stillbirths and abortions is three times as frequent as in livebirths and that clefts with associated malformations behave differently epidemiologically from clefts without associated malformations. It is suggested, therefore, that the incidence of cleft lip, cleft palate, and cleft lip and palate should be studied separately for each group, namely for livebirths, stillbirths, and abortions and should be reported separately for clefts without associated malformations, clefts with associated malformations, and syndromes. More research is needed to study the risk of developing clefts among the various groups that exhibit different epidemiologic behavior for each race.     

Primary alveolar cleft bone grafting in unilateral cleft lip and palate: craniofacial form at age 8.

Counterpart analysis can be advantageous for the clinician interested in the underlying determinants of the craniofacial form for any given person. This analysis was performed for a group of patients who underwent primary alveolar cleft bone grafting (N = 18) and a group of patients who did not undergo grafting (N = 19) who were 8 years of age (+/- 6 months). The primary grafting group more frequently noted maxillary retrusion, but of a nonsignificant magnitude. Also, the primary grafting group had greater mean magnitudes of mandibular opening as a compensatory adjustment in some patients, but this could not be generalized to all patients who had underdone primary grafting. The mean magnitude of craniofacial vertical shortening was also greater for some patients who had undergone primary grafting, but it, too, did not exhibit a generalized pattern for all patients who had undergone primary alveolar cleft bone grafting procedures. This study emphasizes the great diversity of craniofacial skeletal adjustments made within each group of patients with unilateral cleft lip and palate and cautions the clinician against generalizations concerning a particular treatment protocol.     

Median cleft of the upper lip associated with a mass: A rare case

Median cleft lip is a midline vertical cleft through the upper lip. This is a very rare anomaly described in the literature. Median cleft lip is caused by the failure of fusion of the medial nasal prominences. In this case report, a 4-month-old boy with a median cleft associated with a mass of the upper lip is presented. The patient has no other anomalies of the nose or alveolus. The patient has normotelorism. A Z-plasty technique was used on the skin of the base of the columella. A vertical excision of the cleft with muscle approximation was performed on the white roll and the wet-dry border of either side of the defect of the upper lip. Postoperatively, the patient had a satisfactory result. The incisive scars were not visible. Cupid's bow was appropriately aligned, and the height of the upper lip was equal on both sides.     

Repair of bilateral clefts of lip, alveolus and palate. Part 1: A refined method for the lip-adhesion in bilateral cleft lip and palate patients.

The protruding premaxilla represents the most severe problem in the surgical closure of a bilateral cleft lip, alveolus and palate (BCLP). In principle there are two methods to overcome this obstacle: (1) preliminary lip adhesion and (2) presurgical repositioning with intraoral devices. According to the various degrees of premaxillary protrusion, sometimes adhesion alone is sufficient, if the surgical technique is unlikely to break down. In this paper a refined adhesion method is presented, withstanding traction to the wound margins and concomitantly enables lip and nose repairs in a single second operation. For patients with severe premaxillary protrusion, presurgical use of a Latham appliance achieves conditions for safe lip adhesion as above. Both treatment methods are outlined.     

Choanal atresia and rare craniofacial clefts: report of three cases with a review of the literature.

OBJECTIVE: This retrospective study reports three cases associating choanal atresia (CA) and rare craniofacial clefts (RCFCs) described in Tessier's classification. Although still discussed, these two malformations could derive from the same embryopathogenic context, with an anomaly of migration of the neural crest cells as a common factor. In two of the three cases presented, the RCFC was diagnosed during the neonatal period, and the third case (case 3) was diagnosed at the age of 12 years. The knowledge of this malformative association must permit the diagnosis of CA as early as the neonatal period or through the thorough and directed examination of the children as part of the follow-up.     

Augmentation by autologous adipose tissue in cleft lip and nose. Final esthetic touches in clefts: part I

Although patients with a cleft lip and palate undergo many surgeries and other therapeutic procedures in the course of their treatment, many are still deeply concerned with their handicap and continue searching for perfection in their appearance. Augmentation using the subject's own fat cells involves minor invasion, is readily available, is an unpretentious method regarding time and cost, and has no contraindications. This method can serve to supplement a hypotrophic scarred upper lip and nasal columella, and by improving the volume, it induces a more natural contour, which reduces the stigmatizing deformity as well as the visibility of externally apparent scars. Using this approach, five patients with a complete cleft have been treated. The median follow-up interval is 22 months (through January 2003). The procedure and postoperative course had a pleasing outcome and were without any complications. The disadvantage was the temporary effect of the outcome, which necessitated repeated application every 7 months on average.     

A rare case of accessory maxilla and bilateral Tessier no. 7 clefts,a 10-year follow-up

A Tessier no. 7 cleft is a lateral facial cleft which originates from the oral cavity and extends towards the tragus, involving both soft-tissue and skeletal components. A male patient presenting with both maxillary jaw duplication and bilateral Tessier no. 7 clefts, which has been reported only twice in the literature, is described. Bilateral facial clefts, macrostomia and chondro-cutaneous remnants were noted, which were repaired and resected. With further growth, facial asymmetry and asymmetric facial nerve dysfunction became apparent. Radiographic examination showed an accessory maxillary jaw and a flattened and hypoplastic right coronoid process. A maxillary alveolar cleft was also present between the left second bicuspid and the second permanent molar. This case may represent an under-recognized phenotype with an unusual combination of maxillary jaw duplication, macrostomia, Tessier no. 7 clefts, and chondro-cutaneous remnants. A long-term follow-up of these patients is recommended as they often develop craniofacial deformities later in life.     

Microbiota associated with residual clefts and neighboring teeth in patients with cleft lip, alveolus, and palate.

Twenty patients with residual clefts or pronounced soft tissue grooves, treated for uni- or bilateral cleft lip, alveolus, and palate were included in this study. Ten patients were recalled for dental prophylaxis at regular intervals, 10 patients were not. One microbiologic sample was obtained from the cleft area and two samples from a tooth adjacent to the cleft (sites adjacent and distant to the cleft). Between the recall and the nonrecall group there were notable differences in the presence of anaerobic Gram-negative organisms. Fusobacterium spp., Prevotella melaninogenica, and P. intermedia were more often found in nonrecall patients. While rarely seen in recall patients, spirochetes and motile rods were a common feature of nonrecall patients. The putative periodontal pathogens Actinobacillus actinomycetemcomitans and Porphyromonas gingivalis were not detected in either group. The differences between the recall and the nonrecall groups were more pronounced when the respective samples from teeth were related to each other than when the samples obtained from the clefts were compared. The cleft flora was less complex irrespective of how good maintenance was and resembled the flora of teeth of well-maintained patients. Samples from clefts were never Wolinella positive, and harbored significantly less Capnocytophaga and Actinomyces viscosus than samples from dental sites.     

Lower lip cleft,bifid tongue and fibrolipoma: a case report of rare congenital anomaly

A non-syndromic bifid tongue with fibrolipoma, ankyloglossia, and clefts of the lower lip and alveolus is rare. We describe a case in which a bifid anterior tongue was separated by a fibrolipomatous mass. We excised the mass and repaired the lip and the tongue with satisfactory results.     

Repair of bilateral clefts of lip, alveolus and palate. Part 2: Concomitant lip closure and columella lengthening after lip adhesion.

Lip repair and synchronous columella lengthening in bilateral clefts of the lip, alveolus and palate following lip adhesion according to the method outlined in Part 1 is described in this part of the paper. Together with lip and nose repair the gingivo-periosteoplasty can also be performed when the alveolar process is perfectly aligned and the greater and lesser segments abutt onto each other.     

Congenital median cleft of the lower lip, bifid tongue with ankyloglossia, cleft palate, and submental epidermoid cyst: report of a case

An unusual case of median cleft of the lower lip associated with bifid tongue and cleft palate is described. Four years later, the child developed a submental epidermoid cyst.     

The craniofacial complex in cleft lip and palate: an update review of anatomy and function.

This report has been prepared for the purpose of updating the previous State-of-the-Art reports on the status of research on the anatomy and physiology of cleft lip and palate (Dickson, et al,. 1974, 1975; Maude-Dickson, 1977). It covers the literature from August, 1976 to August, 1978, on middle ear musculature, the auditory tube, the velopharyngeal mechanism, the lip, mandible, tongue, nose, and larynx. It also includes a review of the literature from August, 1972, through August, 1978, on the growth and development of the craniofacial skeleton. Significant advances have been made in our understanding of the velopharyngeal mechanism and auditory tube. Further investigation is needed on the innervation and blood supply to the velopharyngeal mechanism and on the effects of age, race, and sex on the development of the craniofacial skeleton in both normal and cleft-palate individuals. There is a continued need for interdisciplinary research.     

Repair of bilateral clefts of lip, alveolus and palate Part 1: A refined method for the lip-adhesion in bilateral cleft lip and palate patients

The protruding premaxilla represents the most severe problem in the surgical closure of a bilateral cleft lip, alveolus and palate (BCLP). In principle there are two methods to overcome this obstacle: (1) preliminary lip adhesion and (2) presurgical repositioning with intraoral devices. According to the various degrees of premaxillary protrusion, sometimes adhesion alone is sufficient, if the surgical technique is unlikely to break down. In this paper a refined adhesion method is presented, withstanding traction to the wound margins and concomitantly enables lip and nose repairs in a single second operation. For patients with severe premaxillary protrusion, presurgical use of a Latham appliance achieves conditions for safe lip adhesion as above. Both treatment methods are outlined. Copyright 2001 European Association for Cranio-Maxillofacial Surgery.     

Fusion of maxillary and mandibular alveolar process together with a median mandibular cleft: a rare congenital anomaly.

Congenital fusion of the maxillary alveolar process and mandible is a very rare entity. A one day old male newborn was referred to our clinic with a diagnosis of a 'absence of oral cavity'. Physical examination revealed that there was no mouth opening bilaterally in the posterior region due to maxillary and mandibular bone fusion; and in the front there was only 1 mm of mouth opening. Examination of the intraoral space could not be achieved. No other congenital anomaly was detected on general examination. The infant, who was fed via a nasogastric tube for 1 month, was operated on. An intra-oral device which separated maxillary and mandibular alveolar arches from each other was designed and used for 8 weeks to avoid recurrence of the fusion. An adequate mouth opening was obtained and the infant could be fed orally.     

Seventeen-year follow-up of a patient with median cleft of the lower lip, mandible, and tongue with flexion contracture: a case report.

PURPOSE: We present a 17-year follow-up of an unusual patient with median cleft of the lower lip, mandible, and tongue with a flexion contracture. CONCLUSION: Timing of the mandibular reconstruction needs to take into account growth of the mandible and masticatory function. In this case, fixation of the mandible performed at the age of 6 years 6 months and reconstruction carried out at 7 years 8 months of age resulted in satisfactory occlusion and masticatory function.