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Triangular flap for atypical cleft lip. A modification in design.

Standardized steps or criteria for designing a triangular flap do not always fit for all types of cleft lip repair. Some compromise among the criteria is required, especially for atypical cases, such as those with a long vertical height on the noncleft side and a short lateral lip element. In order to secure symmetry in the horizontal length of vermilion, or in the distance from the peak of the cupid's bow to the mouth corner, E' should be located as high as allowable. The ratio of the length of the vertical incision to that of the triangular base affects the final shape of the repaired lip in many ways. Since the bottom of the cupid's bow is generally gradual, slight horizontal asymmetry in the cupid's bow is usually acceptable, as compared with that of the other components.

Child↗

Metastatic squamous cell carcinoma of the lip.

Squamous cell carcinoma of the lip is a common lesion that is not always acknowledged as a potentially lethal disease. We reviewed the cases of 27 patients with metastatic squamous cell carcinoma of the lip. Our data confirm that patients in an older age group are at greater risk for development of metastases than the general population of patients with squamous cell carcinoma of the lip. We also found a universally grave prognosis for patients who exhibit fixation of tumor to the mandible or erosion of the mandible. We were unable to confirm a greater risk for development of metastases among more advanced (T3 and T4) lesions. A number of our patients had T1 and T2 primary lesions. We also could not establish a relationship between a patient's delay before seeking medical assistance and subsequent development of metastases.

Adult↗

Carcinoma of the lip.

We reviewed 250 patients with carcinoma of the lip who were treated with radiation therapy during a 30-year period. Although radiation techniques underwent continued refinement, the radiation dose delivered to the tumor remained relatively constant. Nearly all tumors were squamous cell carcinoma (247/250) and located on the lower lip (240/250). Only 9% initially manifested lymph node metastases. The median survival was 13.8 years. Treatment failure occurred in 11% of patients. Surgery salvaged half of these patients. Eighteen patients died from carcinoma of the lip, and six others had persistent tumor when last seen. Our experience would support the work of others. Early lesions can be successfully managed by either surgical or radiation treatment. The intermediate tumors and those near the commissure are better handled by radiation. Late lesions and those with lymph node metastases challenge the best surgical and radiation techniques.

Adult↗

Cleft lip and palate surgery in La Ceiba, Honduras.

The volume of experience in the surgical repair of cleft lip and palate deformities is limited in many areas of the United States. This deficit in experience exists not only for those in resident training programs but also for those who practice at universities or are in private practice. Physicians are performing too few operations to maintain their surgical skills in cleft lip and palate surgery. This deficit is also encountered by physicians in the major specialties of plastic surgery, oral surgery, and otolaryngology who perform cleft lip and palate surgery. Physicians from the United States can gain surgical experience in other countries through groups like the Medical Group Mission Christian Medical and Dental Society. Surgical experience is available in Central and South America, India, China, and Africa through similar organizations. The complexity of providing service to under-privileged people through organizations such as the Medical Group Mission Christian Medical and Dental Society is described.

Child↗

Does maternal cigarette smoking during pregnancy cause cleft lip and palate in offspring?

To investigate the relationship between maternal cigarette smoking and the risk of oral clefts in offspring, we examined data from the Atlanta Birth Defects Case-Control Study, which included 238 cases of cleft lip with or without cleft palate and 107 cases of cleft palate ascertained by the Metropolitan Atlanta Congenital Defects Program from 1968 through 1980. In all, 2809 infants who served as controls were frequency matched to cases by race, period of birth, and hospital of birth. Maternal periconceptional exposures to smoking were investigated through use of a structured questionnaire. Smoking exposure was defined as reported maternal smoking during the periconceptional period (from 3 months before conception to 3 months after pregnancy began). Offspring of smoking mothers were 1.6 and 2.0 times more likely than offspring of nonsmoking mothers to have isolated cleft lip with or without cleft palate and cleft palate, respectively. On the other hand, offspring of smoking mothers were not at increased risk of having cleft palate or cleft lip with or without cleft palate that are associated with other defects. Adjustment for potential confounding variables did not alter these results. A relatively modest effect of smoking may be explained by the presence of underlying etiologic heterogeneity in oral clefts and differential susceptibility to smoking. Because of the inconsistencies in the literature on the relationship between smoking and oral clefts, these results suggest the need to refine oral clefts into more homogeneous subgroups in epidemiologic studies of these defects.

Adult↗

A comparison of results after radiotherapy and surgery for stage I squamous cell carcinoma of the lower lip.

BACKGROUND: Controversy still exists as to whether radiotherapy or surgery is the preferable therapeutic modality for stage I squamous cell carcinoma of the lower lip. Therefore, a retrospective study was undertaken to compare the results of both treatment modalities. METHODS: The results of 90 patients who received radiotherapy and 166 patients who underwent surgery as the primary form of treatment for their stage I primary squamous cell carcinoma of the lower lip were evaluated. Tumor size and histological grade of differentiation were assessed. RESULTS: Local control rates were the same with radiotherapy or surgery. Overall survival rates for both groups of patients were similar as well. Disease-free survival rates in the patients who underwent radiotherapy were significantly lower compared with the surgically treated group. This was due to a higher occurrence of regional metastases in the patients who received radiotherapy. Univariate analysis showed that irradiated patients had a statistically significant greater tumor size. The difference of histological differentiation between the groups was also statistically significant, the poorly differentiated being more common in the irradiated group. Multivariate analysis showed that only tumor size carried significant independent prognostic information. CONCLUSIONS: The cure rates of stage I squamous cell carcinoma of the lower lip are favorable whether treated by radiotherapy or surgery, and local control rates are similar. The radiotherapeutic treated group showed an increased incidence of cervical metastases, which was due to the more advanced tumor size in these patients.

Adult↗

Carcinoma of the lower lip: treatment results at Indiana University Hospitals.

Sixty-six consecutive cases of carcinoma of the lower lip were seen at the Indiana University Hospitals from 1960 to 1972. Each patient's records were reviewed as to age, sex, race, stage, stage by treatment modality, pathology, treatment methods used and cause of failure. Early lip cancer represents a highly curative lesion whether treated by surgery or radiation therapy. Close followup is mandatory on all patients as the development of neck nodes, if discovered late, carries a grave prognosis. Advanced carcinoma of the lip can be successfully treated with current radiation therapy techniques using fractionated doses providing excellent cosmetic results.

Adult↗

Unusual manifestations of multiple cranial nerve palsies and mandibular metastasis in a patient with squamous cell carcinoma of the lip.

A 70-year-old man who had squamous cell carcinoma of the lip later presented with right mental neuropathy, ipsilateral progressive involvement of all three divisions of the 5th, the 7th and 8th cranial nerves, and complete ophthalmoplegia. Biopsy proven metastasis to the mandible was demonstrated. Although repeated studies of CT scan of the head and cerebral angiography were negative, CSF examination revealed positive cytology, elevated protein and low sugar content. Although vascular dissemination from the primary lip lesion to the mandible and brain stem cannot be ruled out, extension through perineurial space via the 5th cranial nerve have been speculated. This case demonstrates two unusual features of squamous cell carcinoma of the lip, namely metastasis to the mandible and meningeal spread with multiple cranial neuropathies.

Aged↗

Case-control study of cleft lip or palate after maternal use of topical corticosteroids during pregnancy.

A case-control survey of 48 children with nonsyndromic cleft lip or palate showed a significant increase in prevalence of maternal use of topical corticosteroid preparations in the first trimester of pregnancy, compared to 58 controls born in the same hospital; the odds ratio was 13.154, 95% confidence interval 1.67-586, P = 0.0049 on Fisher's exact two-tail test. The results were statistically significant although the wide confidence interval reflected the small sample size. Although older epidemiological studies have not detected any association between systemic corticosteroid treatment and the combined incidence of all congenital malformations, experimental studies over several decades have shown that maternal corticosteroid exposure in several species of animals is specifically associated with oral clefts. This association has been confirmed by more specific case-control surveys where the cases were children with cleft lip or palate and the exposure was maternal systemic corticosteroid treatment in the first trimester. Only one previous survey also analyzed topical corticosteroids, and it demonstrated an increased odds ratio for cleft lip or palate. A national survey of children with cleft palate will be necessary to evaluate the results of this pilot study.

Administration, Topical↗

A newly recognized syndrome with double upper and lower lip, hypertelorism, eyelid ptosis, blepharophimosis, and third finger clinodactyly.

Congenital double lip is an uncommon developmental abnormality usually affecting the upper lip. We report a 21-year-old male with double upper and lower lip, hypertelorism, unilateral ptosis, blepharophimosis, and broad nose with broad nasal tip, highly arched palate, and bilateral third finger clinodactyly. The disorder differs from Ascher syndrome and appears to represent a newly recognized syndrome.

Abnormalities, Multiple↗

Test of genetic heterogeneity of cleft lip with or without cleft palate as related to race and severity.

The question of possible heterogeneity among population groups and phenotypic groups on the role of major gene in the etiology of cleft lip with or without cleft palate [CL(P)] was examined using the uniformly collected data in Hawaii. Complex segregation analysis was used to analyze patterns of family resemblance under the mixed model incorporating the effects of major gene and multifactorial inheritance. Analysis of the entire data showed superior fit of the mixed model including the effects of both major gene and multifactorial inheritance over the model of major gene alone or multifactorial inheritance alone. No significant heterogeneity could be detected between the high-incidence group (Oriental or Japanese) and the low-incidence group (non-Oriental) in the underlying general model, although higher heritability was observed in general. When families were classified into "severe" and "mild" phenotypes based on cleft lip vs. cleft lip and palate or unilateral vs. bilateral cleft in the proband, no significant differences could be detected between the two types in the underlying genetic model.

Cleft Lip↗

Desmoplastic melanoma of the lip.

BACKGROUND: This retrospective study looks at the prognosis of desmoplastic melanoma of the lip, correlating it with the clinical course, treatment, and patterns of failure. METHOD: Twenty-two patients with desmoplastic melanoma of the lip were seen at the University of Texas M. D. Anderson Cancer Center from 1965 to 1998. RESULTS: Three disease groups: (I) untreated tumor (3 patients), (II) excisional scar (10 patients), and (III) locoregional recurrence (9 patients). Group I had two cures and one failure. In group II six had no recurrences, and there were four failures. In group III, all patients failed. Ten patients (45%) had no evidence of disease, of which three (30%) had an initial misdiagnosis. Twelve patients (55%) died of disease or were living with disease, of which eight (67%) had an initial misdiagnosis. CONCLUSIONS: Desmoplastic melanoma of the lip is often misdiagnosed and, therefore, inappropriately treated with multiple recurrences and poor prognosis. Accurate diagnosis and combined treatment may improve local control and survival.

Adult↗

Supraomohyoid neck dissection in the management of cervical lymph node metastases of squamous cell carcinoma of the lower lip.

BACKGROUND: Supraomohyoid neck dissection (SOHND) is generally considered an adequate staging procedure in selected patients with squamous cell carcinoma (SCC) of the lip and oral cavity, with clinically negative nodes in the neck that are at increased risk for occult metastatic disease. The potential role of SOHND as a therapeutic surgical procedure for cervical metastasis limited to level I is controversial. METHODS: A series of 44 patients with clinical cervical lymph node metastases at level I from SCC of the lower lip is reported to evaluate the results of a treatment protocol consisting of therapeutic SOHND on indication followed by radiotherapy. RESULTS: Regional recurrences were observed in four (9%) patients. All recurrences developed within the SOHND dissected area only. CONCLUSIONS: A therapeutic SOHND, on indication followed by radiotherapy, can be an oncologically sound and effective procedure in the management of regional lymph node metastases at level I from SCC of the lower lip.

Adult↗

Primary malignant melanoma of the lip.

Melanoma of the lip is a very rare clinical entity. As such, no large studies are available to help in diagnosis and treatment. We report our results on 11 patients with lip melanoma. Patients with lip melanoma should be approached in a similar fashion to patients with other cutaneous melanomas.

Adult↗

The effects of urea treatment in combination with curettage in extensive lip cancers.

Thirty cases of lip cancer in 28 patients, 23 of which were extensive or very extensive, were treated with local urea injections in combination with curettage. Out of the 30 cases there were 26 squamous cell carcinomas, three basal cell carcinomas extending to the mucosa from the skin of the lips, and one adenocarcinoma of the mucosa of the interior surface of the upper lip. This treatment was effective in all our patients. Recurrences appeared in four patients. These were easily treated in three out of the four patients who returned for treatment, by the same method. In three cases metastases appeared in the lymphnodes of the neck, which were surgically treated.

Adenocarcinoma↗

Prenatal diagnosis of Freeman-Sheldon syndrome and usefulness of an ultrasound fetal lip width normogram.

OBJECTIVES: Freeman-Sheldon syndrome (FSS) is a rare genetic condition with facial and joint abnormalities. Here we report the first case of prenatal diagnosis in a fetus with negative familial history for this syndrome. A chart of the fetal mouth length was generated and its usefulness verified. METHODS: A cross-sectional study was carried out to derive standard charts for fetal lip lengths. The study population was made up of 371 fetuses between 15 and 38 weeks of gestation examined only once for this purpose. A statistical analysis previously recommended to derive normal ranges in fetal growth charts was employed. RESULTS: A 34-year-old woman was referred at 28 weeks of gestation for polyhydramnios and clubfeet. A detailed scan confirmed those findings and showed joint contractures of hands, a severe microstomia with a 'whistling' mouth, and an H-shaped mark over the chin. FSS was suspected, though there was no family history for this genetic syndrome. After emergency delivery at 32 weeks, the syndrome was confirmed. The infant died two months later from pneumonia. Upon construction of a standard mouth length chart, it was found that the fetal lip width in this case of FSS was well below the 5th centile. CONCLUSION: Here we present a standard chart for fetal lip width. We demonstrated its usefulness in cases with fetal face anomalies. Specific charts are fundamental for an objective assessment of the fetal parameters.

Adult↗

Test of the hypothesis that embryonic face shape is a causal factor in genetic predisposition to cleft lip in mice.

It was proposed that embryonic face shape is one of the quantitative variables underlying the threshold trait, cleft lip. To test the hypothesis several facial dimensions were measured in photographs of embryos at an early stage of formation of the face in 3 lines of mice. The lines had been selected from one original population using frequency of cleft lip induced by 6-aminonicotinamide as the selection criterion. Line L was susceptible to spontaneous cleft lip; lines M and C were not. Line L had a significantly smaller distance between the nasal pits than the other 2 lines, but did not differ in overall head size. A trend towards reduced angle between the medial nasal processes in the L line was noted, though this was not statistically significant. These results were predicted by the face-shape hypothesis.

Animals↗

Differences in frequency of cleft lip among the A strains of mice.

The frequency of spontaneous cleft lip on Day 14 of gestation was examined in the A/J, A/HeJ, A/WySn, A.BY/Sn, A.SW/Sn, B10.A/SgSn, and B10.A(2R)/SgSn inbred strains of mice. A/WySn was found to have a higher frequency of cleft lip (20-30%) than A/J or A/HeJ (10%). Differences among strains in laterality of clefting and in rates of differential mortality of affected embryos were also found. Results of crosses between A/WySn and A/HeJ suggested that the difference in rate of clefting may be due to a genetic maternal effect. The results for A.BY/Sn, A.SW/Sn, B10.A/SgSn and B10.A(2R)/SgSn indicated that no association of cleft lip liability and the H-2 complex was detectable.

Animals↗