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Maxillary distraction in cleft lip palate patients: a review of six cases.

Cleft lip and palate patients can present with a maxillary retrusion with tendency to Class III malocclusion after cleft repair. Maxillary distraction osteogenesis is a technique that provides simultaneous skeletal advancement and expansion of the soft tissues. Six nonsyndromic cleft lip and palate patients, ages 12 to 16 years (mean, 13.8 years), underwent maxillary distraction; four had a unilateral and two a bilateral cleft lip and palate. After an incomplete LeFort I osteotomy; a latency period of 3 days was respected. On Postoperative Day 4, distraction was initiated through anterior traction on a Delaire facial mask using distraction forces of 900 gm. Photographs and lateral cephalometric radiographs were obtained preoperatively and 4 months after distraction. A cephalometric analysis was performed to compare the sagittal dentocraniofacial morphology before and after distraction. The aesthetic improvement obtained by maxillary distraction osteogenesis during the permanent dentition to correct maxillary retrusion in our cleft lip and palate patients was impressive. Skeletal advancement varying from 1 to 3.5 mm (mean, 1.7 mm) was found. However, significant dentoalveolar compensations occurred in three patients. This was due to the dental anchorage of the distraction device and can be avoided only by the use of skeletal fixation.

Adolescent↗

Bipedicled axial cross-lip flap for correction of major vermilion deficiency after cleft lip repair.

Vermilion irregularities are common secondary deformities after cleft lip repair. Particularly severe in bilateral clefts, vermilion deficiency attracts considerable attention and detracts from an otherwise excellent lip repair. Minor and moderate vermilion deficits can be corrected with upper lip advancement, rotation flaps, tongue flaps, or grafts. Major defects defy correction with local flaps, because of an absolute shortage of upper-lip tissue. A technique is described for correction of large absolute tissue deficits of the upper-lip vermillion using a bipedicled lower to upper cross-lip visor flap. A lower lip wet vermilion/mucosal flap is based bilaterally near the commissures on the coronary arteries and transferred to a releasing incision at the wet/dry vermilion border of the upper lip. The commissural pedicles are divided and inset at a second stage 10 days later to complete the transfer. The procedure provides ample bulk and lining for major upper-lip vermilion augmentation and tubercle reconstruction without disturbing the obicularis oris muscular oral sphincter. In addition it balances the lips and allows for feeding. The technique is illustrated in two patients with major upper-lip vermilion defects after repair of bilateral cleft lips.

Adolescent↗

Detection of cleft lip by early second-trimester transvaginal sonography.

OBJECTIVE: To describe the detection of cleft lip in the early second trimester using transvaginal sonography. METHODS: We analyzed retrospectively 14,988 ultrasound examinations conducted for fetal malformations between 12-16 weeks' gestation. Seventy-five percent of the patients were at low risk for fetal anomalies and 25% were at high risk. RESULTS: Eleven cases of fetal cleft lip with or without cleft palate were detected by transvaginal sonography among 14,988 fetuses (0.07%). In nine cases, no risk factors for cleft lip or palate existed. In six cases, the cleft lip represented an isolated malformation, with no other associated anomaly; in five of these six cases, the pregnancy was terminated. One additional case of a small cleft lip was not detected, for a false-negative rate of 8%. CONCLUSIONS: The fetal lips can be scanned by transvaginal sonography in the early second trimester, and fetal cleft lip can be detected in most cases. Early second-trimester detection of cleft lip by transvaginal sonography allows informed parental choice regarding continuing or terminating the affected pregnancy.

Abnormalities, Multiple↗

Repair of unilateral cleft lip: a comparison of five techniques.

Repair of unilateral cleft lip is a challenging procedure with no single technique satisfactory for all types of unilateral cleft deformity. This study compares retrospectively five techniques of unilateral cleft lip repair in 72 children (45 boys, 27 girls). Twenty-two children had a Millard rotation-advancement repair, 5 children had a Davies Z-plasty, 22 had a modified Z-plasty, 10 had a Tennison-Randall triangular flap repair, and 13 had a Nakajima-Yoshimura straight-line repair. Assessment was performed clinically by a plastic surgeon not involved in the original surgery. Repairs were assessed objectively by measurement of the vertical length of both the repaired and normal sides of the lip with calipers. Subjective criteria used to evaluate the repair were the symmetry of Cupid's bow, the quality of scar, the alignment of white roll, the evenness of the vermilion, and the lip pout. The mean follow-up period was 5.4 years (range, 9 months-29 years). Sixty-five of the 72 repairs measured (90%) were within two standard deviations (SDs) of normal and thus were considered to be of "acceptable" length. Seven repairs were unacceptably short (>2 SDs) on measurement, six of which included a rotation-advancement repair for a complete cleft lip. Subjective results paralleled the objective results. The outcome following repair of unilateral cleft lip was similar for all five surgical methods assessed except for complete cleft lips repaired by the rotation-advancement technique, which tended to result in an unacceptably short lip as measured on the repaired side.

Adolescent↗

Associated congenital abnormalities in Nigeria children with cleft lip and palate.

This is a prospective study of 106 patients with cleft lip and or palate seen over a 5 year period between January 1981 and December, 1985 at the Lagos University Teaching Hospital. Thirty of these patients (28.3 percent) have other associated congenital anomalies which lies within the range of 3 percent to 63.3 percent reported by other authors. The associated anomaly rate was 19 percent in cleft lip only, 35 percent in cleft lip and palate and 28 percent in cleft palate only. All the associated anomalies in the cleft lip cases were single and simple. On the other hand, the multiple and more complex associated anomalies occurred in patients with cleft plate alone and cleft lip and palate combined. Three syndromes were recognised in patients with cleft palate and two in patients with cleft lip and palate.

Abnormalities, Multiple↗

Management of unilateral cleft lip nose deformity, with retracted ala of the noncleft side.

BACKGROUND: Traditionally, the goal of management of unilateral cleft lip-cleft nose deformity has been nasal symmetry, with improved nasolabial and nasofacial relationships and the noncleft side as the reference point. However, symmetry does not always ensure an aesthetically desirable form. The retracted ala is characterized by an alar rim to the long axis of the nostril distance greater than 2 mm on lateral view of the nose. In the management of unilateral cleft lip nose deformity in a patient with a retracted ala of the noncleft side, a simple correction of the deformity symmetric to the noncleft side results in bilateral alar retraction, an aesthetically undesirable result. METHODS: The authors present eight cases of managing unilateral cleft lip deformities with retracted alae of the noncleft side between January of 2003 and October of 2004. RESULTS: Correction of alar retraction of the noncleft side with or without the correction of cleft lip nose deformity yielded better aesthetic results that were maintained throughout the follow-up period. CONCLUSIONS: Aesthetic surgery is the natural evolution of reconstructive surgery; in the reconstruction of selected unilateral deformity, the concept of "as symmetric to the contralateral side as possible" should be changed to the pursuit of an aesthetically pleasing appearance of both sides.

Adolescent↗

A model for the cleft lip nasal deformity.

The underlying pathology of the cleft lip nasal deformity has yet to be fully realized, and cleft lip rhinoplasty continues to challenge the reconstructive surgeon. A new model is proposed, which is composed of elements that represent known anatomical structures of the nose. These structures are considered elemental to the mechanism of the primary cleft lip nasal deformity. The lobule is reduced to four arches. Five points on the skull provide foundations for these arches, which react interdependently to extrinsic forces and positional change. When certain changes are imposed on the model, predictable alterations in the configuration of the model imitate the observed deformities in the spectrum of the cleft lip nasal deformity, unilateral and bilateral, mild through severe. The model is described with illustrations, anatomic dissection, physical models, and selected clinical cases. A better understanding of the mechanisms of the cleft nasal deformities can be obtained through analysis of the model.

Cadaver↗

Location and distribution of epithelial pearls and tooth buds in human fetuses with cleft lip and palate.

OBJECTIVE: The purpose of this study was to establish the location and distribution of epithelial pearls and tooth buds in cleft palate fetuses, relative to the time of palate fusion. DESIGN: The facial skeletal structures, dental laminae, tooth buds, and epithelial pearls were examined in seven spontaneously aborted human fetuses, of which five had unilateral or bilateral cleft lip and palate or cleft palate. The sectioned fetuses were reconstructed by 3D-computer technology. RESULTS: Epithelial pearls were found in four of the investigated cases, of which one was a control specimen. They were located at the margins of the palatal shelves. In the cleft lip and palate cases, the cleft was found in the premaxilla between the first and second incisor tooth. The premaxilla was found to be hypoplastic in both bilateral cleft lip and palate cases and was totally absent in the unilateral cleft lip and palate case. The maxilla was hypoplastic in one case with unilateral cleft lip and palate. In all other specimens, it was developed symmetrically. CONCLUSIONS: The results indicate that cleft lip and palate development may occur after the fusion of the frontonasal prominence with the maxillary prominence and the palatal shelves, as well as a nonfusion of the palatal shelves in the secondary palate.

Cleft Lip↗

Septoplasty for obstructive sleep apnea in infants after cleft lip repair.

A neonate with a unilateral cleft lip and palate usually presents with a deviated nasal septum due to the asymmetric bony base associated with cleft palate. Prior to repair, the facial cleft offers a wide open breathing passage despite the septal deviation. Cleft lips are traditionally repaired in neonates at about 3 months of age. These patients usually do not present with significant symptoms of nasal obstruction following repair, except in unusual cases. Severe septal deviation may cause obstructive sleep apnea. Repair of septal deformities in children is controversial due to the potential alteration of facial growth. We present two patients with documented obstructive sleep apnea that began after cleft lip repair. Conservative surgical correction of the septal deviation resulted in relief of the sleep apnea.

Cleft Lip↗

Self-reports of psychosocial functioning among children and young adults with cleft lip and palate.

OBJECTIVE: A cross-sectional study was employed to determine the psychosocial effects of cleft lip and/or palate among children and young adults, compared with a control group of children and young adults without cleft lip and palate. PARTICIPANTS: The study comprised 160 children and young adults with cleft lip and/or palate and 113 children and young adults without cleft lip and/or palate. All participants were between 8 and 21 years of age. OUTCOME MEASURES: Psychological functioning (anxiety, self-esteem, depression, and behavioral problems) was assessed using validated psychological questionnaires. Happiness with facial appearance was rated using a visual analog scale. Social functioning, including experience of teasing/bullying and satisfaction with speech, was assessed using a semistructured interview. RESULTS: Participants with cleft lip and/or palate reported greater behavioral problems (p < .001) and more symptoms of depression (p < .01); they were teased more often (p < .001) and were less happy with their facial appearance (p < .01) and speech (p < .001), compared with controls. There were no significant difference between subjects with cleft lip and/or palate and subjects without cleft lip and/or palate in terms of anxiety (p > .05) or self-esteem (p > .05). Having been teased was a significant predictor of poor psychological functioning, more so than having a cleft lip and/or palate per se (p < .001). CONCLUSIONS: Teasing was greater among participants who had cleft lip and/ or palate and it was a significant predictor of poorer psychosocial functioning. Children and young adults with cleft lip and/or palate require psychological assessment, specifically focusing on their experience of teasing, as part of their routine cleft care.

Adolescent↗

Lip and nose morphology in patients with unilateral cleft lip and palate from four Scandinavian centres.

Sixty patients with unilateral cleft lip and palate were compared for lip and nose appearance. All patients were photographed from an anteroposterior and a basal view at 7-10 years of age. The photographic registration method was tested for validity and accuracy. Although the four groups of 15 patients each were treated according to different protocols, many similarities were found with shorter lip heights at the cleft side and inclination of the rima oris. Asymmetry of nose and retropositioning at the cleft side naris were generally seen. Significantly straighter noses were demonstrated in the group treated with a two-stage lip nose operation combined with nose plugs, and the two groups where vomer flaps were used showed the greatest deviation of the nose.

Child↗

Cleft lip in the Talmud.

The authors address the problem of why the cleft lip anomalad is not mentioned even once in the Talmud, which contains detailed reports of numerous other forms of pathology and congenital anomalies. It also attempts to define the Sandal deformity, one of the most common congenital anomalies mentioned in the Talmud. It is assumed that cleft lip, the most common major facial defect, existed at the time of the Talmud. It is proposed that the Sandal deformity was actually made up of a number of entities grouped together because of a theorized common etiology, and that the cleft lip entity is included in the Sandal anomalad. This deformity, in its various forms, included, besides cleft lip, aborted tissue and products of gestation. They are all related to the common etiology of a twin gestation during which one fetus presses against the other causing the deformity. The Sandal was noted to look like the Sandal fish or sole, for which it is named. When photos of the Sandal fish and the cleft lip deformity are compared, a similarity is seen. In addition, this deformity apparently was thought to be fatal, and none of these babies survived. It is theorized that cleft lip babies were allowed to die because they were considered nonviable in the eyes of Jewish law. Supporting evidence for the ideas presented by the authors is derived from the talmudic texts and commentaries. If the Sandal does indeed include the cleft lip entity, the citation in the Mishnah (70-200 AD) makes it the earliest historical reference to the cleft lip deformity.

Bible↗

Increased susceptibility to 6-aminonicotinamide-induced cleft lip of heterozygote Dancer mice.

Dancer heterozygotes (Dc/+) very rarely have cleft lip and show a dancing behaviour due to inner ear defects while homozygotes (Dc/Dc) have cleft lip. Males of the two genotypes Dc/+ and +/+ were mated to C3H strain and R stock females and Dc/+ males to Dc/+ females. On day 10/8 of gestation females were treated with 6-aminonicotinamide (6AN) at either 19 mg/kg or 28.5 mg/kg followed 3 h later by a protective dose of nicotinamide. Controls were untreated. Both 6AN treatments caused a significant increase in cleft lip to between 25% and 29% for crosses of Dc/+ males to C3H and R females whereas crosses with +/+ males gave 0% cleft lip. In the controls the cleft lip frequency was: for Dc/+ X Dc/+ 14%, Dc/+ X C3H 1.4%, and for the other three crosses 0%. The four crosses given the high dose of 6AN and the +/+ X C3H and Dc/+ X R cross at the low dose showed significantly increased resorption rates to between 23% and 47% over the control rates of from 5% to 11%. The presence of the Dc gene increased the susceptibility to cleft lip caused by 6AN.

6-Aminonicotinamide↗

Primary dentition status and treatment needs of children with cleft lip and/or palate.

The aim of this study was to assess the primary dentition status and treatment needs in children with cleft lip, cleft palate and or both. A descriptive, cross-sectional study was done through oral examination on a sample of 83, 2-5 years old cleft lip/palate children (46 boys and 37 girls). A self-designed questionnaire to obtain pertinent data related to level of education of parents and infant-feeding practices was used for recording the data while examining the children. Majority of cleft affected children had dental caries experience and required different types of treatment. The result of this study shows that: (1) all age groups of cleft affected children had dental caries experience, (2) children with primary type of cleft had less dental caries prevalence than children with cleft of secondary or combination type ( P < 0.05), (3) treatment needs due to caries were more in combination type of cleft compared to primary and secondary.

Age Factors↗

Major gene determination of liability to spontaneous cleft lip in the mouse.

Two genetic analyses suggested that the spontaneous and low frequency trait of cleft lip (primary palate) in the mouse is determined either by a recessive gene at one autosomal locus or by two loci with duplicate epistasis. The low frequency trait of open eyelids, which is characteristic of the A/J strain and sometimes reported to be associated with cleft lip as part of a "syndrome," has not been analyzed genetically. A backcross and test-mating study between A/J and C57BL/6J, done originally to define the genetic control of embryonic tolerance to cortisone-induced cleft palate (secondary palate), was reanalyzed for the cleft lip and open eyelids traits. Cleft lip frequencies in A/J did not change in dose-response studies of either cortisone- or 6-aminonicotinamide-induced cleft palate; for both teratogens the frequencies of open eyelids differed between doses but did not exhibit any obvious dose response. It appeared that the frequency of 7.6% cleft lip and 17.6% open eyelids in A/J in the genetic study, in which all pregnant A/J test-females were treated with a single dose of cortisone (100 mg/kg, day 12), did reflect the occurrence of the spontaneous traits. Within the A/J strain, the traits were not associated and, as expected on outcrossing to C57BL/6J (A.B6 F1 fetuses), both traits are recessive. Significant bimodality of the cleft lip scores of the BC1 sires (BC2 fetuses), test-mated with A/J, suggested that liability to cleft lip is determined by a single autosomal recessive gene. The distribution of open eyelids scores of the BC1 sires did not differ from one normal distribution, and this trait is, therefore, controlled by more than one genetic locus with additivity between loci. Cleft lip and open eyelids segregated independently and do not form a syndrome, in A/J, with one underlying genetic cause. There was no association between cleft lip and three autosomal marker genes, brown (b, chromosome 4), albinism (c, chromosome 7) and H-2 (chromosome 17), or the genetically independent tolerance traits of cortisone- and 6-aminonicotinamide-induced cleft palate. There was significant association between open eyelids and albinism (c) that is in a direction suggesting linkage or pleiotropy. Whether liability of the embryo to cleft lip is determined by one or two genes may be solved by a concerted effort to map the trait; a marker gene will be the key to further analysis of its cause.

Animals↗

[A clinical study of presurgical nasoalveolar molding in infants with complete cleft lip and palate].

OBJECTIVE: To observe the clinical effect of presurgical nasoalveolar molding in infants with complete cleft lip and palate. METHODS: Presurgical nasoalveolar molding was performed in 38 infants with cleft lip and palate (26 patients with unilateral cleft lip and palate, 12 patients with bilateral cleft lip and palate), aged between 5 and 30 days. The width of alveolar cleft was measured before and after the operation and the effect of treatment was assessed. RESULTS: After 108 - 152 days of therapy, the average width of alveolar cleft decreased by 5.3 mm in 26 patients with unilateral cleft lip and palate. Nasal profile was improved in 76 percent of cases. In 12 patients with bilateral cleft lip and palate, the average width of left cleft decreased by 4.7 mm and that of the right decreased by 4.2 mm. The distance between right and left cleft increased by 5.1 mm. Nasal profile was improved in 66 percent of cases. CONCLUSION: Presurgical nasoalveolar molding in complete cleft lip and palate can improve nasal profile and decrease the width of alveolar cleft.

Alveolar Process↗

Influence of surgery on maxillary growth in cleft lip and/or palate patients.

Using a sample of 204 caucasian adults of both sexes, with complete unilateral cleft lip and alveolus, isolated cleft palate and complete unilateral cleft lip, alveolus and palate, the influence of surgery on maxillary morphology and spatial position for the three main types of cleft lip and/or palate were evaluated. 113 cleft patients operated on at conventional timings were compared to 91 cleft patients who had never been operated on. Findings permit the conclusion that cheiloplasty in cleft lip and alveolus patients is associated with osseous remodelling in the anterior alveolar region, without significant changes in anterior nasal spine or other areas of the maxilla. In isolated cleft palate subjects palate repair does not promote significant changes in maxillary morphology and spatial position. The most evident effects were seen in the cleft lip, alveolus and palate group: reconstructive surgery in these patients leads to a severe maxillary retroposition associated with a downward rotation.

Adolescent↗

Predisposing factors to dental caries in children with cleft lip and palate: a review and strategies for early prevention.

OBJECTIVE: To review predisposing factors contributing to dental caries in patients with cleft lip, cleft palate, or both (CL/P). DESIGN: Patients with CL/P have a higher susceptibility to caries compared with the population group without clefts. A review of the literature was undertaken to determine factors that could account for the compromised oral hygiene and the high caries prevalence in patients with CL/P. RESULTS: After a literature review, modern strategies for the prevention of early childhood caries were developed and should be considered for integration into the overall management protocol of patients with CL/P. CONCLUSIONS: Preventive dental care is ever more so important in patients with CL/P compared with patients without clefts.

Cleft Lip↗