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Use of applied behavioral techniques and an adaptive device to teach lip closure to severely handicapped children.

The effectiveness of applied behavioral programming in teaching lip closure was investigated with 3 severely retarded male students who had oral motor dysfunction. A multiple-baseline design across subjects was employed. The results revealed dramatic increases in the level of functional lip closure for all 3 students when an appropriate adaptive device was combined with behavioral intervention. Follow-up results indicated that functional lip closure generalized to other feeding tasks. Findings suggest that applied behavioral management may be successfully used to teach specific oral motor skills and facilitate integration of these skills into a normalized feeding pattern.

Adolescent↗

[Sphenoethmoidal meningoencephalocele associated with agenesis of corpus callosum and median cleft lip and palate--report of two cases (author's transl)].

Two cases of sphenoethmoidal meningoencephalocele are reported. Patients are a 1 year 7 month old boy and a 3 year old boy. Although sphenoethmoidal meningoencephalocele is reasonably classified as a type of basal meningoencephalocele, the authors could not find out any reports on cases designated as such. The reason must be in difficulty to differentiate the sphenoethmoidal type from the transsphenoidal type. Authors could differentiate them using CT scan. Two other cases were found in cases reported as transsphenoidal type. Further, it is assumed that these four cases including our two cases were accompanied with agenesis of corpus callosum and median cleft lip and palate. Despite the fact that the respective anomalies are rare, they are completely the same, which signifies the possibility that 1) sphenoethmoidal meningoencephalocele, 2) agenesis of corpus callosum and 3) median cleft lip and palate are a single unit of congenital anomalies. It probably should be considered that the cause for these malformations were present even before the formation of the lips which takes place earliest, that is, sixth week of gestation.

Abnormalities, Multiple↗

Facial features associated with predisposition to cleft lip.

Teratologic studies have led to the hypothesis that, in the mouse, face shape is the underlying basis for the susceptibility to cleft lip. To see if this may hold true in man superficial facial dimensions, face shape and relation of lips have been recorded in a series of parents of children with cleft lip and in a control population. Preliminary results suggest that there are differences between the two populations that support the hypothesis.

Cephalometry↗

Straight-line closure: a preliminary to Millard closure in unilateral cleft lips (with a history of the straight-line closure, including the Mirault misunderstanding).

We have completed 25 SLC-RAC sequences for complete unilateral clefts of the lip, using the straight-line closure as a preliminary step to facilitate the definitive closure, which is done about 6 months later. Fourteen of the straight-line closures were done on newborn children with local anesthesia, and the rest were done at a later time under general anesthesia because of later referral or refuge/immigrant status, using general anesthesia. We have had no major complications such as lip dehiscence, major infection, respiratory problems, or bleeding. Since our original publication, we have increased the scope of the straight-line closure to include wider nasal undermining for better repositioning of the nasal structures, and we have adopted Millard's technique of lining the gap in the pyriform aperture with a vermilion flap (Millard's "L" flap). Our oldest patient is not yet in adolescence, so our study is incomplete, but our impression is that the preliminary straight-line closure has materially improved the results of surgery for complete unilateral cleft lips. The history of the straight-line closure forms a continuum from the 1st century A.D., and includes Malgaigne, Mirault, and Veau repairs as well as those of many others.

Child↗

On the importance of muscle repair in secondary cleft lip deformity.

Generally the evolution of cleft lip repair has included a transition from those methods with primary emphasis on the skin design with intricate flaps, going to further elaborations of carefully designed skin and muscle units but with minimal muscle dissection or intentional reorientation, on to those with greater emphasis on extensive muscle dissection with specific realignment of the orbicularis oris muscle. These latter methods assign to the skin a more complementary role in the plan of correction. This article joins others in which it is now felt that extended dissection and surgical reorientation should be done to include associated involved muscles of the lip, nose, and medial face. In particular, attention to the details of muscle repair as completely as possible, extending to the entire oro-naso-facial complex, seems pertinent in the matter of secondary correction of cleft lip deformities. A review of the anatomy involved, certain surgical philosophy, and examples of illustrated cases are included in this article.

Adolescent↗

Craniofacial morphology in adults with bilateral complete cleft lip and palate.

Cephalometric x-ray studies were carried out on 26 adult males with complete bilateral cleft lip and palate (BCLPc) and 50 non-clefts. The configuration of the neurocranium and cranial base showed no substantial differences. Facial differences were similar to those in unilateral clefts (UCLPc). Protrusion and retroinclination of the premaxilla affected the depth and height of the upper jaw and thus the total height and posterior growth rotation of the face. Thus the depth of the maxillary complex was unchanged, while the depth of the posterior maxilla was reduced, as in UCLPc. The increased length of the face, its posterior growth rotation, the dentoalveolar maxillary retroinclination and the displacement of the maxilla backwards were all more marked in bilateral clefts then in unilateral clefts. Changes of mandibular shape, an enlarged interocular distance, retrusion of the upper jaw and the configuration of the soft tissue profile, including the shorter upper lip were identical in both forms of clefts. However the mandible was more retrognathic and posteriorly rotated in bilateral clefts. The skeletal profile was not flattened, but the palatal plane was retroinclined and the depth of the nose was increased. BCLPc was associated with a widening of the nasal cavity and a thinner upper lip. The dimensions of the nasopharyngeal skeletal framework were reduced. The retrocheilia was conspicuous. Premaxillary setback resulted in a reduction of the depth of the upper jaw, of the height of the upper face and of the dentoalveolar retroinclination of the maxilla.

Adult↗

Population and family studies of HLA in Japanese with cleft lip and cleft palate.

Population and family studies of HLA were performed in Japanese patients with cleft lip and/or cleft palate (CL/P). Frequency of HLA-Cw 7 was significantly increased in cleft lip (CL) patients (37.5%) and cleft palate (CP) patients (37.8%) but was not increased in cleft lip and palate (CLP) patients (17.5%), compared with control subjects (13.3%). However, the intensities of the associations were not great (relative risk = 4.0). The affected sib pairs method was studied in 13 families with CL- or CLP-affected sib pairs and 10 families with CP-affected sib pairs. However, in both groups of families the distributions of HLA haplotypes in affected sib pairs did not significantly differ from random Mendelian expectation. Thus, HLA-linked major genes (loci) which determine the development of CL/P were not found. These results seem indirectly to support the multifactorial theory of CL/P, but does not exclude other possible genetic mechanisms.

Cleft Lip↗

Functional cleft lip repair: a sequential, layered closure with orbicularis muscle realignment.

A lip repair based on anatomic and electric stimulation studies of the orbicularis muscle in unilateral cleft lip has previously been reported by one of the authors. Following some early modifications, this technique has been used on 125 primary lip repairs. The details of the present technique are described here. The advantages of the procedure, in addition to its functional reconstruction of the orbicularis muscle, are in its applicability to clefts of all widths and superior scar formation. The sequential nature of the procedure and freedom from commitment to a fixed, measured pattern at the outset makes the teaching of the method easier and the achievement of a pleasing result more predictable.

Cleft Lip↗

Dental fragments in the lips.

Fifteen successively presenting cases of dental fragments in the lips were analyzed as to cause, site of injury and relationship to groups of anterior teeth. In each case a lip laceration extended from mucosa to skin. This finding supported the hypothesis that a tooth, having transfixed the affected lip, fractured on emergence from the skin surface on contact with a hard object. Skin wounds were often repaired in general hospitals without detecting the foreign body and prior to seeking a dental opinion.

Adolescent↗

Comparison of craniofacial morphology in infants with incomplete cleft lip and infants with isolated cleft palate.

The craniofacial morphology was compared in 30 infants with incomplete left-sided cleft lip and 30 infants with isolated cleft palate. Cephalometric radiographs were obtained prior to any surgical management at 2-3 months of age in the lateral, anteroposterior and axial projections. Compared to the cleft lip group, the infants with isolated cleft palate showed the following characteristics: short anterior cranial base, short maxilla, reduced posterior maxillary height, reduced dimensions of the mandible--especially mandibular length--, narrow naso- and oropharyngeal airway. The cleft lip group had larger interorbital width and symmetry deviations in the anterior art of the maxilla. It was concluded that already at two months of age and prior to any surgical interference significant differences in craniofacial morphology distinguished the group with clefts of the secondary palate from the group with clefts of the primary palate.

Cephalometry↗

Factitious lip crusting.

Four cases of factitious crusting of the lips in women are reported. Two of the women had hemorrhagic crusts, and two had keratotic yellow crusts. All four patients had personality disturbances. Biting, picking, or unconscious licking of the lips may be the underlying mechanism for trauma and crust formation. This entity should be distinguished from contact cheilitis, actinic cheilitis, infectious cheilitis glandularis, and cheilitis granulomatosa. Some cases of exfoliative cheilitis may also be factitious. The presence of bizarre hemorrhagic or keratotic crusts on the lips should alert the clinician to a possible factitious origin, and a psychiatric evaluation should be done.

Adolescent↗

Lips and labial regions in old age.

The author records the morphology and functions of 80 old (66 to 92 years) subjects lips and labial regions. She mentions the following types: 1. no wrinkles, normal lips and surroundings; 2. marked sulcus nasolabialis; 3. marked sulcus nasolabialis and sulcus tristitiae; 4. marked sulcus nasolabialis and sulcus tristitiae and many small wrinkles instead or in place of the latter; 5. sagging, floppy lower lips. Those types were often found to coincide with the look, the gait, the psycho-somatic status. According to our experiments, the simultaneous marked presence of sulcus nasolabialis and sulcus tristitiae is a real sign of old age. Lonely old people often have marked sulci tristitiae and many small wrinkles. The morphologic and functional changes described in the foregoing are related primarily with the psychovegetative disposition; their emergence is not due to the normal or abnormal clinical laboratory parameters. The above recording is an effort to provide guidance on geriatric therapy, prognosis, rehabilitation, possibilities of prevention.

Aged↗

Induction of cleft lip in cultured rat embryos by localized administration of tunicamycin.

Whole-embryo culture techniques have advanced to the point where the study of normal and abnormal primary palate development in vitro is possible. The purpose of this study was to ascertain whether localized administration of tunicamycin (TM) an inhibitor of protein glycosylation, into the region of the developing primary palate would induce cleft lip in culture. Rat embryos were explanted on late day 11 of gestation and cultured with open yolk sacs for 40 h. TM was administered by implanting a sterile eyelash soaked in TM into the nasal placode region. The contralateral placode was used as the control by implanting an eyelash lacking TM. Under these conditions, TM-treated placodes were found to develop cleft lip in 14 out of 15 embryos compared to 0 for the controls. These experiments demonstrate that localized administration of TM results in cleft lip formation in whole embryo culture. The technique of localized administration of drugs and teratogens in whole embryo culture should prove useful for similar studies on embryonic development.

Animals↗

Late results of the surgical treatment of complete unilateral cleft lip and palate: soft tissue characteristics.

Sixty-one UCLP patients at 17-20 years of age treated in childhood by the same surgeon (Krauss) were invited for check-up examination to evaluate the late results of the surgical treatment. In the patients, the cleft lip was closed by a modified Le Mesurier procedure at 6-9 months of age and the cleft palate by a modified Veau procedure at 3-4 years of age. The "normal face" appearance showed 69% of the patients. More than one third of the population had successfully corrected nose deformities after the primary operation. In the most cases, the lip was also restored satisfactorily during the first treatment. Regular border between the lip and vermilion was observed in 62% of cases. The correct shape of hard palate was also found in 62% of the patients. The soft palate was well reconstructed in 92% of cases. In the most cases (95%), the speech was correct although the cleft palate was closed relatively late. The late results of the treatment can be considered satisfactory.

Adolescent↗

[Total reconstruction of the lower lip].

In cases the entire lower lip was extirpated because of lip cancer and in 13 of these patients immediate restitution was performed by the slightly modified method of Grimm. The experience gained with this method is analysed. The fact that beside an acceptable function of the lip the oral orifice has not narrowed is considered the main advantage of the method.

Humans↗

[Interstitial brachytherapy in carcinoma of the lip. Case histories and results].

Squamous cell carcinomas of the lip can be treated by surgery or radiation therapy. Since local control rates exceed 90% with both treatment modalities, therapy is chosen according to functional and cosmetic results, to the patient's wishes and to the physician's medical policy. This study was aimed at retrospectively reviewing our local control results and patterns of failure relative to survival as well as evaluating the results according to technical surgical variables. From 1982 to 1991, sixty-nine patients with squamous cell carcinoma of the lip received a course of interstitial brachytherapy with 192Ir. The disease stage was T1 in 36 cases, T2 in 12 cases and T3 in 2 cases and 19 recurrences; 3 patients only had clinically detectable lymph nodes at diagnosis. Interstitial brachytherapy was used as postoperative treatment, at a dose of 60 Gy, in 47 cases. As an exclusive curative approach brachytherapy was given at a reference dose of 65 Gy, with a medium dose rate of 62.88 cGy; a single plane was used in 55 cases, a triangular plane in 11 and a double plane in 3 cases. In one patient only the disease persisted and no local failures were observed. Overall actuarial survival at 5 years is 76.81% (91.3% when corrected for disease). In fact, 6 patients died of disease progression: 3 with lung metastases, 1 with bone metastases and 3 with lymph node metastases. Five patients died of cancer in other sites--i.e., ovary, lung, prostate, hypopharynx, stomach--and 4 of non-neoplastic diseases. Tolerance rates were excellent, with only one mucosal necrosis which resolved spontaneously; no sequelae were observed in gums, teeth and jaws. The cosmetic result was also excellent in most of the cases with a worsening trend for multiplanar disposition. In conclusion, interstitial brachytherapy can be considered the treatment of choice for early lip cancer.

Adult↗

[The surgical treatment of lower-lip carcinoma].

After a brief presentation of methods for the treatment of carcinomas of the lower lip, the author describes a new surgical technique which is a judicious modification to the procedure indicated by Webster and Bernard. Its principle consists in repairing the tissue defect resulting from the excision of the lower lip by the additional surgical removal of one or two soft tissue triangles from the region of the nasolabial fold and in achieving primarily, by means of horizontal relieving incisions at the base of the lip defect, an extension of the mouth aperture. Since 1973, this technique has been mainly used at the Clinic and Policlinic of Maxillofacial Surgery of the Warsaw Medical Academy. The results obtained were satisfying from the functional and the aesthetical point of view. Up to now, no recurrences have been seen.

Humans↗

Actinic cheilitis and carcinoma of the lip.

During dental examinations, the lips are readily overlooked. Dentists should routinely examine the lips visually and by palpation. Actinic cheilitis is a common condition caused by damage to the lips through exposure to sunlight, and is readily diagnosed clinically. Its progress can be minimized by the use of an appropriate sun screen when outdoors. Actinic cheilitis can undergo malignant transformation into squamous cell carcinoma. The clinical features and management of these conditions are described.

Adult↗