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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.

Abnormalities, Multiple↗

[The study and application of the alar cartilage ring in the treatment of deformities of unilateral cleft lip nose].

OBJECTIVE: To explore the possible pathogenesis and to look for effective reconstructive procedures of unilateral cleft lip nose. METHODS: The author has had a detailed comparative study by comparing the cartilaginous skeleton of 20 normal cadaver specimens with that of one unilateral cleft lip stillborn, as well as intraoperative findings of 24 clinical patients. RESULTS: There are two alar cartilaginous rings, namely alar cartilaginous fibrous ring and alar cartilaginous muscular ring, in external nose. Both rings are hypoplastic in the cleft lip patients. According to the findings, the author designed and applied a new operation procedure to reconstruct alar cartilage ring for 24 patients from 1994 to 1997. An acceptable short-term results were found. CONCLUSION: Alar cartilaginous fibrous ring and alar cartilaginous muscular ring are very important anatomic structures to maintain the shape of the lower part of the nose. Hypoplasia of these rings is an important factor leading to deformities of the nose. It is very important to reconstruct alar cartilage ring for the patients with unilateral cleft lip nose.

Abnormalities, Multiple↗

Are isolated facial cleft lip and palate associated with increased perinatal mortality? A cohort study from the West Midlands Region, 1995-1997.

OBJECTIVE: To investigate the association between cleft lip and/or palate and perinatal mortality. METHODS: A retrospective review was performed of cases of cleft lip/palate born to West Midlands residents from 1995 to 1997. Perinatal mortality for identified cases was compared with all births from 1995 to 1997. RESULTS: 347 cases of cleft lip and/or cleft palate were delivered from 1995 to 1997. Thirty-six pregnancies were terminated due to parental wishes--2 were registerable births. There were 310 spontaneous registerable births (stillbirths/livebirths) with cleft lip and/or palate and 1 further late fetal loss. In 220 (70.5%), the lesion was isolated. Of these, there were 7 perinatal deaths, 5 had post mortems and no additional anomalies were identified. In 92 (29.5%) cases other abnormalities were identified. The overall perinatal mortality rate (PNMR) in the West Midlands, was 10.0/1000 total births. The overall PNMR for babies with facial clefts was 89.7/1000 total births. The PNMR for those with associated anomalies was 228.3/1000 live/still births. The PNMR for isolated facial clefts was 31.8/1000 live/still births, significantly higher than the background population (OR 3.3, 95% CI: 1.5-7.0). CONCLUSION: Consideration should be given to screening the fetus at 20-24 weeks for facial deformity. This has implications for detection both of fetal anomalies and of a population at risk for adverse outcome.

Cleft Lip↗

Variable expression in ankyloblepharon-ectodermal defects-cleft lip and palate syndrome.

The ankyloblepharon-ectodermal defects-cleft lip and palate (Hay-Wells) syndrome is a rare autosomal dominant form of congenital ectodermal dysplasia. It is characterized by coarse, wiry, sparse hair; dystrophic nails; slight hypohidrosis; scalp infections; ankyloblepharon filiforme adnatum; hypodontia; maxillary hypoplasia; and cleft lip and palate. To date, 12 patients have been reported; however, the diagnosis has been questioned in 3 of these patients. We report 2 additional patients, one of whom has nasal speech but not cleft palate, in contrast to all other reported patients. This entity must be distinguished from numerous other forms of ectodermal dysplasia, especially those forms that can be associated with oral clefts and/or ankyloblepharon.

Child↗

Effect of primary surgery for cleft lip and palate on mid-facial growth.

Surgery for cleft lip and palate is known to have an effect on growth and development of the mid-face. This paper studies the outcomes in 34 consecutive 10-year-old patients with unilateral cleft lip and palate. Clinical observations of the importance of both surgical technique and the influence of cranial base morphology on maxillo-mandibular position are discussed.

Cephalometry↗

An intraoral appliance for management of the protrusive premaxilla in bilateral cleft lip.

Management of the protruding premaxilla and prolabium in bilateral cleft lip and palate can be a confounding problem. This report introduces an intraoral traction appliance that has been successfully used to move the premaxilla and prolabium to a more favorable position for surgical repair of the bilateral cleft lip. The appliance consists of a palatal baseplate for anchorage and a latex rubber traction strip looped over the prolabium to retract the premaxillary segment. The results in five cases are presented and compared to two cases where no presurgical management was performed and lip repair had been delayed for medical reasons. Advantages and disadvantages of the appliance are discussed.

Cleft Lip↗

Nasal sill augmentation in adult incomplete cleft lip nose deformity using superiorly based turn over orbicularis oris muscle flap: an anatomic approach.

Adult incomplete cleft lip nose deformity is not uncommon in India. Poverty, ignorance, and parental neglect account for its late presentation. Besides the classical features of cleft lip nose deformity, the constant findings observed in this patient population have been a widened and depressed nasal sill. This is attributable to the sparse, hypoplastic, and abnormally orientated orbicularis oris muscle in the region of the sill. Failure to restore the nasal sill symmetry by suitably augmenting the sill frequently leads to unsatisfactory and asymmetric results. However, in the literature, satisfactory restoration of the nasal sill has not been given the importance it deserves while performing cleft lip rhinoplasty. We present a method of augmenting the depressed nasal sill in cases of adult incomplete nose deformity using a superiorly based orbicularis oris muscle flap, which is harvested from the soft tissues between the apex of the cleft and the nostril sill. Following de-epithelialization of the overlying skin, the exposed muscle is raised as a superiorly based flap after dissecting it from the underlying mucosa. It is folded, turned over, and tucked into the nasal sill base and anchored to the anterior nasal spine to give the desired augmentation. Satisfactory results have been obtained in 18 cases of nasal deformity associated with incomplete cleft lip. In our opinion, this technique offers a simple and effective method of augmenting the depressed sill by utilizing locally available tissues and without the need for procuring autologous tissue from distant sites.

Adolescent↗

The influence of bone grafting age on dental abnormalities and alveolar bone height in patients with unilateral cleft lip and palate.

Individuals with total unilateral cleft lip, alveolus and palate, (UCLAP), bone grafted at three different ages were compared with regard to dental abnormalities and alveolar bone height in the cleft area. The primary groups, bone grafted prior to one year of age had fewer supernumerary teeth in the cleft area and a lower frequency of missing and severely malformed central incisors than the other groups. The early secondary group, bone grafted after eruption of the permanent incisors, showed the highest frequency of normal lateral incisors and the most favourable alveolar bone height in the cleft area. The secondary group, bone grafted after eruption of the canines, showed the highest frequency of missing teeth outside the cleft area. Early secondary bone grafting, after eruption of the permanent incisors and before eruption of the canines, seems to be preferable.

Adolescent↗

Correction of secondary cleft lip deformities.

LEARNING OBJECTIVES: After studying this article, the practitioner should be able to (1) describe the common secondary deformities of the cleft lip, (2) determine the appropriate timing for surgical intervention to correct the deformities, and (3) determine the best method of addressing each of the individual secondary deformities of the cleft lip. Secondary deformities are common in children born with a cleft lip and palate. Patients with cleft lip deformity will undergo multiple surgical procedures early in life, so it is imperative to prioritize treatment of their secondary deformities and minimize the number of interventions needed. Of the many approaches used to correct these problems, surprisingly few work well consistently. As with all plastic surgery, the timing and procedure should be predicated on the severity of the deformity.

Cleft Lip↗

Alveolar bone grafting in unilateral complete cleft lip and palate patients.

Autogenous bone graft of an alveolar cleft area has the following advantages: (1) assistance in the closure of buccoalveolar oronasal fistula; (2) provision of bony support for unerupted teeth and teeth adjacent to the cleft; (3) formation of a continuous alveolar ridge to facilitate orthodontic correction of malocclusion; (4) supporting the nostril floor and alar base to improve nasal aesthetics. It has been well accepted in most craniofacial centers as routine procedure in cleft lip and palate rehabilitation. A new surgical technique for alveolar bone grafting has been introduced to the Chang Gung Craniofacial Center since July 1991. It provided a good exposure of the alveolar cleft, primary closure of the fistula and adequate volume of bone graft. A review of 27 consecutive alveolar bone grafting procedures performed in unilateral cleft lip and palate patients from July 1991 to June 1992 was presented. Patients have been followed up for at least 6 months. The alveolar bone graft was evaluated clinically and radiologically at one week, six months and one year after the surgery. The preliminary results indicated that the new surgical technique produced less chance of recurrent fistula, good postoperative gingival height, and improvement of nasal aesthetics. Based on the results of this new study we strongly advocate the use of this new surgical technique.

Adolescent↗

[Individual repair technique of microform cleft lip: state of art].

OBJECTIVE: To explore an technique to repair the microform cleft lip (MCL). METHODS: The individual method was used to repair the MCL, a contrastive research was carried to analyzed the result. RESULTS: It was found that the form of the cupid's bow was good, and the configure of the upper lip was reconstructed. The deficit of the orbicularis muscle was overcame and the function was satisfying by this procedure. Symmetry was recovered. CONCLUSIONS: Individual technique is an available method to repair the microform cleft lip. Furthermore, this technique is also promising to repair the other kinds of cleft lip.

Cleft Lip↗

[Prenatal ultrasonic diagnosis of cleft lips and palates].

The purpose of this communication is to demonstrate the possibility of prenatal diagnosis of cleft lips and palates by routine ultrasound screening. As obstetrical ultrasound improves, anomalies of the fetal face can be diagnosed more and more frequently in utero. From 1991 to 1994 9 cases with cleft face syndrome were detected prenatally. In 8 cases the presence of cleft lip and palate was isolated. In one case the cleft lip and palate was associated with phocomelia. In cases with cleft palate the widening of nasal cavity was observed. In cases with bilateral cleft palate polyhydramnions were observed twice and undulating movements of the tongue were also seen.

Adult↗

Problems and reconstruction of "dish face" deformity due to cleft lip and palate.

This paper deals with the unique aspects and surgical indication of "dish face" deformity, including "flat face" deformity, due to cleft lip and palate. The importance of deformities of the nose and lip caused by cleft lip and palate and the role of their reconstruction in the treatment of "dish face" deformity are described in detail. For the protruding chin and pseudoprognathism, vertical osteotomy of the ramus of the mandible is recommended over advancement osteotomy of the hypoplastic maxilla.

Cleft Lip↗

Distinct craniofacial syndrome of lagophthalmia and bilateral cleft lip and palate.

We report on several individuals with bilateral cleft lip and palate, lagophthalmia, megaloblepharon, distichiasis, and ectropion of the lower eyelids, representing in total a distinct craniofacial syndrome. Eight cases (3 from 1 family) in ages ranging from 1-45 years were identified over a 7-year period in the Cleft Palate and Craniofacial Clinic at our center. All cases (3 male, 5 female) presented with bilateral cleft lip and palate and lagophthalmia. Birth weight, growth, and development were normal in all cases, except for one who probably has familial small stature. Five of the 8 cases have distichiasis, and 5 have ectropion in varying degrees; dental findings consisted of hypodontia in 5 and delayed dentition in 1 patient. Hypoplastic nails and clinodactyly were confined to 3 members of 1 family. Clinical evidence in these cases and a review of the literature regarding distichiasis and lagophthalmia, either alone or in combination with ectropion, suggest that this craniofacial syndrome is most likely an autosomal-dominant trait. The 5 non-familial cases probably represent new mutations.

Abnormalities, Multiple↗

Regional anesthesia for cleft lip repair: a preliminary study.

OBJECTIVE: To assess whether local anesthetic blockade of the infraorbital nerve may allow for cleft lip repair to be performed under regional anesthesia. METHOD: Twenty patients above 12 years of age with cleft lip (7 females and 13 males) were enrolled. Careful preoperative counseling was given. Bilateral infraorbital block was performed (extraoral approach) with 10 mL of equal volumes of 2% lidocaine with 1:200,000 adrenaline and 0.5% bupivacaine. The point of entry was the intersection of a vertical line through the pupil of the eye (in the neutral position) and a horizontal line through the ala of the nose. The needle was directed medially and cephalad until the infraorbital foramina was reached and 2 mL was injected. Through the same point of entry, but directed medially (toward the ala), 1 mL was injected followed by a 2-mL injection caudally and medially (into the lip). RESULTS: The block was successful in all patients without complication. The surgery was uneventful, lasting between 45 and 60 minutes. The postoperative duration of analgesia was between 6 and 24 hours (mean 16.5 +/- 5.10 SD). CONCLUSIONS: The nerve supply of the cleft lip, our modification of the block, and possible problems with this technique are discussed. This study confirms the utility of this block for postoperative analgesia. Further studies are required to apply this innovative, safe, and economical modification of anesthesia for cleft lip surgery.

Adolescent↗

[Mandibular movements in dentulous cleft lip and palate patients with lingualized occlusion].

Most dentulous unilateral cleft lip and palate patients show a tendency toward lingualized occlusion in lateral segment teeth. The purpose of this study was to investigate the effect on the stomatognathic function by changing the occlusal contacts of buccal cusps in lateral segment teeth in dentulous unilateral cleft lip and palate patients with lingualized occlusion. Three patients were selected and two types of experimental upper crowns, L crown (with occlusal contact only at the lingual cusp), B crown (with occlusal contacts both at lingual and buccal cusps), were fabricated. The border jaw movements and mandibular movements during chewing an almond were recorded with MMJI-E (Shofu inc.). The results were as follows: 1. By using B crown, the area of border jaw movements in the frontal plane and the amount of maximum opening on the experimental crowns side were increased. 2. By using B crown, mandibular movements in the occlusal phase were converged and stabilized during chewing. 3. It was suggested that favorable mandibular movements during chewing were due to proper occlusal contacts provided in lateral segment teeth.

Adult↗