A case of a rare first branchial arch anomaly with severe hypoplasia of the mandible and fusion between the mandible and adjacent bones is presented. The patient also had intracranial, cardiac, and acral deformities. The craniofacial malformations may reflect incomplete separation of the first branchial arch into its maxillary and mandibular processes. The association between the craniofacial and other corporal anomalies is unknown.

The limited literature on the lateral pharyngeal recess of Rosenmüller and the various presentations of pharyngeal webbing are reviewed. Webbing across the lateral pharyngeal recess is described in 28 of 31 adults with cleft palate examined. The phenomenon has not been reported before and does not appear to be a feature of the nasopharyngeal wall in noncleft individuals.

A tongue flap is frequently used for closure of fistulae following cleft palate repair. Early tongue flap dehiscence is a troublesome complication. While the tongue flap is a very effective means of functionally obliterating the transpalatal oronasal opening, the final appearance of the repair leaves much to be desired. More often than not, the tongue tissue appears bulky and unnatural in the roof of the mouth. A method is described that overcomes the problem of flap detachment during the early postoperative period by suspending and supporting the tongue pedicle with a palatal sling. On peroral view, the repaired area has a better appearance when the tongue flap lines the nasal side rather than the oral side as in conventional procedures.

Pressure-flow data were obtained on 20 noncleft adults with normal speech and 26 adults with repaired cleft palate. All subjects had adequate velopharyngeal function as determined by preliminary aerodynamic assessment. Subjects were considered to have adequate closure if they had velopharyngeal areas no greater than 0.049 cm2 during production of /p/ in the word "hamper." Although the subjects in both groups demonstrated velopharyngeal adequacy, the subjects with cleft palate produced speech with significantly less nasal airflow. In addition, their intra-oral pressure curve was shifted forward in time. These differences suggest that speakers with cleft palate and adequate velopharyngeal function make certain compensatory adjustments that may be necessary because of differences in velopharyngeal movement capabilities. The potential significance of this for treatment planning is discussed.

Glucocorticoid-induced cleft palate (CP) has been used as an animal model for hormonal teratogenesis. In mice, the susceptibility to glucocorticoid-induced CP varies with the strain, A/J being very sensitive and C57/BL6J relatively resistant. Studies in adult and embryonic murine tissues have attempted to correlate the number of glucocorticoid receptors and CP susceptibility, with conflicting results. The relative quantities of dexamethasone receptors were now studied in established palatal and lung fibroblast cell cultures obtained from adult C57 and A/J mice. A rapidly saturable, stable binding system was demonstrated. Scatchard plots were linear indicating a single class of high affinity receptors. The glucocorticoid receptor number ranged from 6.2 x 10(-16) mole/microgram prot to 8.6 x 10(-16) mole/microgram prot, while the KD varied from 1.0 x 10(-8) M to 2.8 x 10(-8) M. The differences in receptor characteristics between murine strains were not significant (p greater than 0.05). The absence of a difference in receptor number between the two strains may reflect the limitation of fibroblast cell culture in assessing glucocorticoid binding in vivo. Alternatively, if a difference in palatal dexamethasone receptor levels between mice strains exists, it may occur only in the embryo.

The purpose of this report is to describe a milling procedure for the fabrication of skull models from 3-D CT data. Data from computer tomographic cuts are transferred to a computer and stored. Model fabrication takes place using high quality styrodur that is shaped with a high speed milling device. This allows the modeling of an individual life size skull in its skeletal as well as in its soft tissue contours. Smaller bone pieces and areas can be enlarged separately up to eight times normal size for better visualization. Planning and simulation of craniofacial surgery in three dimensions is possible, and bone implants or alloplastic biomaterials can be modeled for implantation.

An accurate description of the functioning Eustachian tube (ET) requires a thorough knowledge of the anatomic relationships of its components. To this end, 15 "normal" adult ETs were obtained, sectioned, and stained with hemotoxylin-eosin. Descriptive and quantitative data of ET structures and their relationships were obtained. Eustachian tube length was normalized and comparisons between specimens made. This analysis suggests (1) in its midsection the cartilage is loosely attached to the cranial base; (2) the deep portion of the tensor veli palatini (TVP) originates from the lateral lamina and the fibrous portion of the lateral membraneous wall; and (3) the levator veli palatini (LVP) can interact with the ET primarily via the elongated medial lamina in the anterior portion of the ET. These observations suggest the ET is opened by a medial rotation of cartilage effected primarily by the TVP, but aided anteriorly by the LVP.

The hearing impairment associated with congenital external auditory canal atresia has been managed with early bone conduction hearing aid placement and surgical reconstruction in selected patients. However, many patients do not wear a bone conduction hearing aid because of physical or social considerations and surgical reconstruction of the external auditory canal and middle ear may be difficult or contraindicated. This report details the use of implantable bone conducting hearing devices in five children with bilateral external auditory canal atresia. Each patient had bilateral conductive hearing impairment with normal bone conduction thresholds. Four of the five patients had associated craniofacial anomalies including three cases of microtia. The average preoperative sound field speech reception threshold improved from 63 dB to 13 dB with the implant. Patients experienced a definite preference for the implanted hearing device over the bone conduction hearing aid.

Craniofacial parameters were studied clinically and by cephalometry in 11 trigonocephalic patients from infancy to 4 years of age. Six of the most severe patients had surgery between 6 and 18 months of age. Analysis of morphology indicated that bony interorbital distance was reduced in patients selected for surgery and hypotelorism persisted at final examination. All patients demonstrated orbital width measurements above the mean for the norm, while orbital height was essentially normal. All but one of the patients had a variably prominent forehead bony ridge that was eliminated or reduced as a result of surgery and/or growth. However, the forehead of most patients, whether operated or not, was too narrow when compared to normal skulls. Thus, although some of the striking features of trigonocephaly are eliminated, minor characteristics of the anomaly still persevere at 4 years of age.

Fifty-seven consecutive complete unilateral cleft lip and palate patients were followed longitudinally from birth to 21 years of age. All patients were operated with the same primary surgical procedures. Reevaluation at age 21 with respect to speech, dental condition, need for secondary surgery, and skeletal and soft tissue facial growth was completed. Speech results indicated that 13 patients (23 percent) had required a pharyngeal flap, and that at age 21 only one patient still had unacceptable speech. The majority of patients had slight speech disturbances related to articulation and voice quality. Fifty-one patients had acceptable occlusion following orthodontic treatment, twenty-two patients (39 percent) without need for prosthodontic treatment of the cleft area. Secondary surgery for correction of nasal deformities was required for fifty-two patients. Growth analysis demonstrated deficiency in growth; however, normal and acceptable profiles were obtained in 50/57 of the patients.

The purpose of this study was to compare the influence of two different ages at the time of palatal closure and two surgical methods on the size of the dental arches at the age of 3 years in children, with CP, UCLP, and BCLP. In 88 children the one-stage palatal closure had been performed at the age of 1.8 years and in 92 children at the age of 1.1 years. In both groups palatal surgery had been performed with a V to Y push back procedure (Veau-Wardill-Killner) or with the Cronin modification. Dimensions of the maxillary arch in both groups at age 3 were smaller than in noncleft children, but the timing of palatal closure did not affect arch dimensions in children with CP, UCLP, or BCLP. Arch dimensions at 3 years of age were not related to the method used in palatal closure.

A multidisciplinary International Working Group of scientists was assembled to address the question of standardizing reporting techniques for multiview videofluoroscopy and nasopharyngoscopy, the generally accepted standards for direct observation of the velopharyngeal valve. This report is a first attempt to propose standards while seeking feedback from the readership in order to further develop a common methodology.