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Cleft lip and palate, lower lip pits, and limb deficiency defects.

Cleft lip or palate and lower lip pits are typical features of the autosomal dominantly inherited Van der Woude syndrome. Limb defects have not been reported in this syndrome so far. A girl with a unilateral complete cleft lip and palate, bilateral lower lip pits, and amniotic deformities of all four limbs is reported and the possibility of chance occurrence of cleft lip and palate, lower lip pits, and limb defects is discussed.

Abnormalities, Multiple↗

Effects of examiner expectancy on speech ratings of individuals with cleft lip and/or palate.

The label 'cleft palate,' any facial disfigurement associated with cleft lip and/or palate and the description 'hypernasal' were studied relative to their effects on ratings of speech. Sixty-four listeners rated hypernasality and articulation characteristics of ten males with cleft lip an/or palate. Results indicated that the speech ratings assigned to this group of individuals did not change significantly with this additional information. Severity of speech defectiveness, mode of stimulus presentation and listener reliability were identified as important variables to be considered in the interpretation of these data.

Attitude of Health Personnel↗

Early and one stage repair of bilateral cleft lip and nose.

In bilateral cleft lip repair numerous complicated problems still remain. In our opinion, it is better to begin treatment soon after birth. We start nonsurgical correction of a nasal deformity using a nose retainer and preoperative orthodontics using a palatal plate. Surgical repair of the lip is done within one month of birth, by which time the nose alveolus and projecting prolabium can be adequately changed into the desired form. Primary lip repair is performed in one stage which includes restoration of the muscle union, labial sulcus reconstruction and nasal correction. We use a two straight parallel suture line method for symmetric bilateral cleft lip repair. The distance between the two cupid's peaks is 4 mm. The central tubercle is reconstructed using bilateral cutaneo-mucosal flaps. The tissue volume of both sides is not the same in asymmetric cases so we have refined our method in order to achieve symmetry.

Child, Preschool↗

Psychological issues in cleft lip and palate.

The treatment of cleft lip and palate extends over the infancy, childhood, and adolescence of individuals who are born with this condition. The children and their families contend with multiple surgeries throughout these years. Depending on the severity of the cleft, the families may also need to cope with speech therapy, ear infections, learning disabilities,and various orthodontic treatments. The end result of these treatments and interventions should be a child, teen, or adult who is an appropriately contributing member of society at each stage of development. The psychological support needed to enable a child to meet that goal should be provided by family, school, the surgeon, and other members of the cleft treatment team. These adults should demonstrate a belief in the child's ability to cope with the challenges of cleft lip and palate treatment and should focus the child on the efficacy he will gain from having had the experience of growing up with a cleft lip and palate.

Adaptation, Psychological↗

Congenital heart disease and associated malformations in children with cleft lip and palate in Pakistan.

Children with cleft lip and palate often have other associated malformations. The reported incidence and types of associated malformations vary between different studies. There is a great paucity of literature on the subject from the region in general and none from Pakistan at all. The purpose of this study was to assess the frequency of associated malformations, particularly congenital heart disease, in children with cleft lip and palate presenting to the Aga Khan University (AKU) and Murshid Hospital (MH). From 1st October 1999 to 31st March 2002, all children with cleft lip and palate who presented to AKU and MH were prospectively enrolled in the study group. Socio-demographic characteristics and a number of other variables were documented. All children underwent a thorough clinical examination and an echocardiogram as part of the study protocol. 123 children formed the study group. Thirty-five (29%) of these children were found to have associated malformations. The most common of these was congenital heart disease, which accounted for 51% of all associated malformations. Thirty percent of cleft palate children had associated anomalies while 27% of cleft lip, with or without cleft palate, children had associated anomalies. There was a significant association between children born of a consanguineous marriage and the risk of associated malformations (p-value: 0.001). Consanguinity was present in 74% of children with associated anomalies as compared to 40% of children with no associated anomaly. Dysmorphic features and the presence of associated anomalies were also significantly associated (p-value: 0.009). Dysmorphic features were present in 46% of children with anomalies as compared to 21% of children with no associated anomaly. Fifty percent of children with associated anomalies had a low birth weight compared to 34% of children with no anomalies, but the difference was not statistically significant. The presence of consanguinity in a child with dysmorphic features should raise the suspicion of an associated anomaly. The likelihood of this being a cardiac defect is high and should be ruled out with a thorough clinical examination, supplemented with an echocardiogram in certain cases.

Abnormalities, Multiple↗

The importance of accurate repair of the orbicularis oris muscle in the correction of unilateral cleft lip.

Most of the attempts and efforts in cleft lip repair have been directed toward the skin incision. The importance of the orbicularis oris muscle repair has been emphasized in recent years. The well-designed skin incision with simple repair of the orbicularis oris muscle has produced a considerable improvement in the appearance of the upper lip; however, the repaired upper lip seems to change its shape abnormally in motion and has a tendency to be distorted with age if the orbicularis oris muscle is not repaired precisely and accurately. Following the dissection of the normal upper lip and unilateral cleft lip in cadavers, we could find two different components in the orbicularis oris muscle, a superficial and a deep component. One is a retractor and the other is a constrictor of the lip. They have antagonistic actions to each other during lip movement. We also can identify these two different components of the muscle in the cleft lip patient during operation. We thought inaccurate and mixed connection between these two different functional components could make the repaired lip distorted and unbalanced, which would get worse during growth. By identification and separate repair of the two different muscular components of the orbicularis oris muscle (i.e., repair of the superficial and deep components on the lateral side with the corresponding components on the medial side), better results in the dynamic and three-dimensional configuration of the upper lip can be achieved, and unfavorable distortion can be avoided as the patients grow.(ABSTRACT TRUNCATED AT 250 WORDS)

Cleft Lip↗

Cleft lip and palate incidence among the live births in the Republic of Korea.

We present an epidemiologic study of cleft lip and palate in the Republic of Korea from January 1, 1993 through December 31, 1993. In 1993, the number of total live births was 715,817. And from 1993 through 1995, a total of 1,293 new patients with cleft lip and palate who were born in 1993 were identified. The incidence of cleft lip and palate was 1.81 per 1000, that is, 1 per 554 live births. The cleft lip: cleft lip and palate: cleft palate alone ratio was 1.13:1:1.19. The male: female ratio was 2.1:1 in the cleft lip group, and 2.5:1 in the cleft lip and palate group. We could detect a male predominance in both groups. In contrast, the ratio was 0.95:1 in the cleft palate group. The left: right: bilateral ratio was 1.9:1:0.23 in cleft lip group, and the ratio was 2.2:1:1.1 in the cleft lip and palate group. This is the first nation-wide study to provide detailed data on the incidence of cleft lip and palate in the live births in the Republic of Korea.

Cleft Lip↗

[Improvement on the individual operative design of unilateral cleft lip repair].

OBJECTIVE: To study the difference of operative designs in unilateral cleft lip repair according to characteristic deformity of each patient with unilateral cleft lip. METHODS: The distance from both alar bases and midpoint of columella base to both peaks of Cupid's bow were consulted is equal as standard design model. The mark point and incision of individual unilateral cleft lip repair were designed using geometrical analysis. RESULTS: The new operative design technique included that the end point of rotational incision should be inside of the cross point from the line dividing both Cupid's bow peaks and philtrum on the non-cleft side, and the other design was that the end point of alar base on the cleft side located at the cross point from the horizontal line that passed the midpoint of alar base on the non-cleft side to meet the alar base on the cleft side. Depending on the above thesis, the new operative methods were created for unilateral cleft lip repair. One of the new individual operative designs was similar with Millard's method in the shape of operative design, but there was a big difference on the principle of marking the columella base and alar base on the cleft side between individual operative designs and Millard's design. This new operative design only suit for unilateral cleft lip with symmetric distances from alar base to Cupid's bow peaks in both sides. Another individual operative design added a new incision on the cleft side comparing with the former individual operative design, which could be used in unilateral cleft lip when the distance from alar base to Cupid's bow peak on the cleft side was shorter than that on the non-cleft side. CONCLUSION: The new individual operative designs of unilateral cleft lip repair have been created, and the important advantages of these new individual operative designs are that each marking point can been decided clearly according to special mathematical principles. Each operative design only suit for one type of unilateral cleft deformity, so the types of deformity in unilateral cleft lip can been also classified depending on the different distances from alar base to Cupid's bow peak between both sides. This classification of unilateral cleft lip only depends on operative designs, and these new individual operative designs of unilateral cleft lip repair are easy to be understanded and applied, as well as easy to obtain stable result after operation.

Cleft Lip↗

[Presurgical orthodontic treatment of complete unilateral cleft lip and palate in 100 infants].

OBJECTIVE: To investigate the application and curative effect of naso-alveolar molding plate in the presurgical orthodontic treatment of complete unilateral cleft lip and palate in infants. METHODS: From January 2003 to March 2004, 100 infants with complete unilateral cleft lip and palate received presurgical orthopedics by using naso-alveolar molding plate. According to the age, 100 infants (aging 10 days to 3 months, including 60 males and 40 females) were divided into the test group (10 days to 1 month) and the control group (1-3 months). The widths of lip cleft and dentoalveolar cleft and the rate of satisfaction for nose wing were compared between 2 groups before treatment and after the treatment. RESULTS: The widths of cleft lip and dentoalveolar cleft before orthodontic (5.0 +/- 1.6 mm, 6.9 +/- 2.6 mm) and after orthodontics (4.1 +/- 2.7 mm, 6.4 +/- 2.9 mm)in the test group were less than those in the control group (7.5 3.1 mm, 12.5 +/- 4.0 mm and 8.3 +/- 3.0 mm, 10.8 +/- 2.6 mm), being statistically different (P < 0.05). The satisfactory rate of nose wing in the test group (86.3%) was significantly higher than that in the control group (62.4%, P < 0.05). CONCLUSION: The advantages of presurgical orthodontic treatment of complete unilateral cleft lip and palate in infants are as follows: repositioning the premaxillary segment, reducing the width of palate cleft, correcting the nasal deformities and facilitating surgical repair of cleft lip and palate.

Cleft Lip↗

Morphogenesis of hypoxia-induced cleft lip in CL/Fr mice.

Cleft lip with or without associated cleft palate [CL(P)], one of the most common human malformations, is in most cases, believed to be caused by a combination of genetic and environmental factors. Previous studies have shown that maternal respiratory hypoxia (10% O2) increases the incidence of CL(P) from the spontaneous level of 36% to 89% in CL/Fr mice. The current investigation was designed to study, morphologically, the developmental alterations of the primary palate primordia in CL/Fr embryos, following a reduction in maternal respiratory oxygen levels. Scanning electron microscopy was utilized to compare the development of 35-43 somite hypoxia and control (normoxia) embryos. Hypoxia increased the incidence of resorptions and increased the incidence of CL(P) in viable embryos, compared to normoxia. Debris, most of which was limited to the deeper aspects of the invaginating nasal placode, was present in hypoxia embryos at stages prior to primary palate fusion and was absent in comparably staged normoxia embryos. It is believed that this debris is cellular in nature and that associated retardation of placodal invagination is primarily responsible for the increased incidence of CL(P). Other effects on morphogenesis and/or growth retardation may also be contributing factors.

Animals↗

Management of an infant with cleft lip and palate with phocomelia in dental practice.

Cleft lip and palate is a severe birth defect occurring approximately one in 800-1000 newborn infants. The incidence varies widely among races. Cleft lip and palate together account for approximately 50% of all cases whereas isolated cleft lip and isolated cleft palate occur in about 25% of cases. Many of these congenital anomalies appear to be genetically determined though the majority are of unknown causes or teratogenic influences. Presented here is a 3 day old infant with bilateral cleft lip and palate and phocomelia for whom a feeding obturator was made and delivered to facilitate feeding.

Cleft Lip↗

Cleft lip and palate in Tehran.

Seventy-nine cleft lip and/or palate births were isolated from 21,138 live births between January 1, 1983 and December 31, 1988 in one hospital in Tehran. Among these, 21 (26.58 percent) were cleft lip (CL), 45 (56.96 percent) were cleft lip and palate (CLP), and 13(16.45 percent) were cleft palate (CP). Chemical sulfur mustard gas was indicated as a major factor in 30 (37.97 percent) of the bilateral cleft lip and palate infants.

Cleft Lip↗

Maxillofacial morphology in children with complete unilateral cleft lip and palate treated by one-stage simultaneous repair.

BACKGROUND: There is a common belief among the majority of surgeons occupied with cleft lip-cleft palate repair that early one-stage simultaneous repair of hard and soft palates affects maxillofacial development adversely. This proposition has not been proven with long-term clinical studies. In this study, the effects of one-stage repair on the maxillofacial development of children with complete unilateral cleft lip-cleft palate were investigated, and the results were compared with those of the cleft children treated with conventional two-stage repair. METHODS: The study was designed as follows. Group 1 consisted of 19 children (mean age, 85.4 +/- 12.8 months) treated with a one-stage procedure. In this group, cleft lip, palate, and alveolus were repaired simultaneously at a single surgical session in the first 10 months of life (mean age at time of surgery, 6.8 +/- 1.2 months). Group 2 consisted of 22 children (mean age, 90.1 +/- 13.0 months) treated in two stages as follows: lip repair was performed at a mean age of 4.8 +/- 1.0 months and palate repair was performed at a mean age of 14.6 +/- 2.0 months. The follow-up period was approximately 6.3 years for both study groups. Group 3 (control) consisted of 27 children (mean age, 87.1 +/- 11.7 months) without cleft who were matched for age. RESULTS: Compared with the control group, the children in both cleft groups revealed a greater maxillomandibular retrognathism, a more open palatal plane, larger anterior facial heights, and decreased posterior vertical maxillofacial heights. No significant difference was determined between study groups 1 and 2. CONCLUSIONS: Because both of the surgical treatment procedures give rise to similar maxillofacial development outcomes, regardless of the timing of surgery, the one-stage procedure offers several important advantages, such as less psychosocial trauma, low cost, and possibly an improvement in speech results because of less scarred palatal fields and the low rate of palatal fistula.

Cephalometry↗

Facial balance in cleft lip and palate. I. Normal development and cleft palate.

A full understanding of balanced facial growth, based on a complete knowledge of the anatomy and physiology of the region, is essential if cleft lip and palate is to be treated successfully. The cleft abnormality is the cause of underdevelopment and subsequent loss of function. Cleft surgery must aim to restore normal anatomy and physiology with emphasis on muscle reconstruction of the lip and soft palate if normal facial development is to be achieved.

Adult↗

Cleft lips and palates: a multidisciplinary approach.

Families of infants born with congenital defects such as cleft lip, cleft palate, and combinations of these often grasp with accepting the condition. Nurses can help facilitate acceptance by parents and families and play a key role in the management and habilitation of these children.

Cleft Lip↗

[Surgical treatment of congenital bilateral cleft lip].

Operations were performed on 23 patients with bilateral cleft lips by the method of Mirault--Limberg--Kardozo. Five patients had bilateral symmetrical total cleft lips, eight patients had bilateral nonsymmetrical total cleft lips, ten patients had bilateral incomplete cleft lips. One-stage operations were used in 17 patients, two-stage operations--in six patients. The method consists in the angle-shaped refreshing and uniting the margins of the cleft which provides the possibility to restore the correct form of the Cupid arch. The method is most expedient in children with bilateral non-symmetrical cleft lips with the well-developed median portion. Long-term results were studied in 19 children. Good and excellent results were noted in 12 patients, satisfactory--in 7 patients.

Child↗