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Biomedical subjects

Alex A Kane

Publications and source records attributed to Alex A Kane.

18 recordsLinked to original sources
PubMed2006

Phenotypic integration of neurocranium and brain.

Evolutionary history of Mammalia provides strong evidence that the morphology of skull and brain change jointly in evolution. Formation and development of brain and skull co-occur and are dependent upon a series of morphogenetic and patterning processes driven by genes and their regulatory programs. Our current concept of skull and brain as separate tissues results in distinct analyses of these tissues by most researchers. In this study, we use 3D computed tomography and magnetic resonance images of pediatric individuals diagnosed with premature closure of cranial sutures (craniosynostosis) to investigate phenotypic relationships between the brain and skull. It has been demonstrated previously that the skull and brain acquire characteristic dysmorphologies in isolated craniosynostosis, but relatively little is known of the developmental interactions that produce these anomalies. Our comparative analysis of phenotypic integration of brain and skull in premature closure of the sagittal and the right coronal sutures demonstrates that brain and skull are strongly integrated and that the significant differences in patterns of association do not occur local to the prematurely closed suture. We posit that the current focus on the suture as the basis for this condition may identify a proximate, but not the ultimate cause for these conditions. Given that premature suture closure reduces the number of cranial bones, and that a persistent loss of skull bones is demonstrated over the approximately 150 million years of synapsid evolution, craniosynostosis may serve as an informative model for evolution of the mammalian skull.

Brain↗
PubMed2006

Anthropometric precision and accuracy of digital three-dimensional photogrammetry: comparing the Genex and 3dMD imaging systems with one another and with direct anthropometry.

A variety of commercially available three-dimensional (3D) surface imaging systems are currently in use by craniofacial specialists. Little is known, however, about how measurement data generated from alternative 3D systems compare, specifically in terms of accuracy and precision. The purpose of this study was to compare anthropometric measurements obtained by way of two different digital 3D photogrammetry systems (Genex and 3dMD) as well as direct anthropometry and to evaluate intraobserver precision across these three methods. On a sample of 18 mannequin heads, 12 linear distances were measured twice by each method. A two-factor repeated measures analysis of variance was used to test simultaneously for mean differences in precision across methods. Additional descriptive statistics (e.g., technical error of measurement [TEM]) were used to quantify measurement error magnitude. Statistically significant (P < 0.05) mean differences were observed across methods for nine anthropometric variables; however, the magnitude of these differences was consistently at the submillimeter level. No significant differences were noted for precision. Moreover, the magnitude of imprecision was determined to be very small, with TEM scores well under 1 mm, and intraclass correlation coefficients ranging from 0.98 to 1. Results indicate that overall mean differences across these three methods were small enough to be of little practical importance. In terms of intraobserver precision, all methods fared equally well. This study is the first attempt to simultaneously compare 3D surface imaging systems directly with one another and with traditional anthropometry. Results suggest that craniofacial surface data obtained by way of alternative 3D photogrammetric systems can be combined or compared statistically.

Anthropometry↗
PubMed2006

Long-term osseous morphologic outcome of surgically treated unilateral coronal craniosynostosis.

BACKGROUND: Unilateral coronal craniosynostosis has characteristic osseous dysmorphology that persists into adulthood if untreated. Knowledge of the long-term in vivo osseous morphologic outcome of surgically treated unilateral coronal craniosynostosis patients is limited. The purpose of this study was to define the osseous morphology of adolescent patients who underwent surgery for unilateral coronal craniosynostosis in infancy, compared with both their 1-year postoperative morphology and the morphology of other individuals with untreated unilateral coronal craniosynostosis. METHODS: Three populations of unilateral coronal craniosynostosis were studied: group 1, patients with surgical treatment of unilateral coronal craniosynostosis in infancy who had reached dentoskeletal maturity, ranging in age from 13.5 to 32.7 years (n= 9); group 2, individuals with untreated unilateral coronal craniosynostosis, ranging in age from 1.1 to 21 years (n= 11); and group 3, a subset of group 1 patients 1 year after surgical correction of unilateral coronal craniosynostosis, ranging in age from 1.2 to 2.6 years (n= 6). Data from high-resolution, thin-slice computed tomographic scans of the head were analyzed. Thirty-five reproducible osseous landmarks were recorded as three-dimensional coordinates using ETDIPS imaging software. Nonmidline landmarks were designated as either ipsilateral or contralateral to the synostosis. One researcher performed all landmarking with high intrarater reliability (average error, <2 mm). Data from the three groups were analyzed for asymmetry using Euclidean distance matrix analysis techniques. RESULTS: Euclidean distance matrix analysis asymmetry analysis demonstrated more statistically significant ipsilateral-contralateral asymmetric pairs in group 1 (68 of 135) than in group 3 (25 of 135), but fewer statistically significant ipsilateral-contralateral asymmetric pairs than in group 2 (93 of 135). CONCLUSIONS: Surgical treatment of unilateral coronal craniosynostosis in infancy results in a less asymmetric craniofacial skeleton in adolescence than nontreatment. However, patients who have been followed to dentoskeletal maturity have a greater degree of asymmetry than those evaluated at 1 year postoperatively. These results support the conclusion that with time there is a partial reversion to the untreated phenotype.

Adolescent↗
PubMed2006

The Pittsburgh Oral-Facial Cleft study: expanding the cleft phenotype. Background and justification.

The Pittsburgh Oral-Facial Cleft study was begun in 1993 with the primary goal of identifying genes involved in nonsyndromic orofacial clefts in a variety of populations worldwide. Based on the results from a number of pilot studies and preliminary genetic analyses, a new research focus was added to the Pittsburgh Oral-Facial Cleft study in 1999: to elucidate the role that associated phenotypic features play in the familial transmission patterns of orofacial clefts in order to expand the definition of the nonsyndromic cleft phenotype. The purpose of this paper is to provide a comprehensive review of phenotypic features associated with nonsyndromic orofacial clefts. These features include fluctuating and directional asymmetry, non-right-handedness, dermatoglyphic patterns, craniofacial morphology, orbicularis oris muscle defects, dental anomalies, structural brain and vertebral anomalies, minor physical anomalies, and velopharyngeal incompetence.

Brain↗
PubMed2005

Brain morphology in nonsyndromic unicoronal craniosynostosis.

Studies of isolated craniosynostosis have shown biomechanical and biochemical influences on the craniofacial phenotype, resulting from both genetic and epigenetic factors. Much less attention has been directed toward the morphology of the brain, despite the interactive nature of the developing skull and developing brain. The aim of this study is to define the morphology of the brain in nonsyndromic unilateral coronal synostosis (UCS) in order to form more complete hypotheses about the cause of craniosynostosis. Landmark coordinate data were collected from 3D magnetic resonance image reconstructions of the brain in a sample of UCS patients and an age-matched morphologically normal cohort. These data were analyzed using Euclidean distance matrix analysis. The results of our study demonstrate that despite the basic similarity of overall shape of the brain and skull in UCS, the effects of craniosynostosis on the brain are not localized to structures immediately adjacent to the fused suture or to the endocranial surface of the skull. Rather, alterations are observed throughout the volume of the brain, with subcortical structures altered in conjunction with cortical changes. These results indicate that the morphological correlates are different for brain and skull and suggest that there is a large degree of independence in the developmental trajectories of the brain and skull.

Age Factors↗
PubMed2005

Os parietale divisum.

Divisions of the parietal bone are extraordinarily rare, and have been described previously only in skull specimens in the anatomical literature. We report a case of os parietale divisum (divided parietal bone) in an infant with plagiocephaly. A 36-weeks gestation male with multiple congenital anomalies presented to the deformational plagiocephaly clinic at 11 weeks of age. CT imaging demonstrated a suture dividing the left parietal bone into two segments. He had a plagiocephalic appearance, with the greater occipital and parietal protuberances ipsilateral to the abnormal left parietal suture, and a correspondingly flatter right occiput. Head circumference was in the normal range, and the anterior fontanelle was patent and of normal size. He had mild right-sided right frontal and malar protuberance. Mandibular asymmetry was noted with a chin-point to the right of midline. He had a left-sided head tilt. He was treated with cranial molding helmet therapy for his head shape and physical therapy for his head tilt. After 16 months of helmet therapy, he had little improvement of his head shape. This case is an in vivo presentation of a rare variant, os parietale divisum.

Abnormalities, Multiple↗
PubMed2005

Speech, cognitive, and behavioral outcomes in nonsyndromic craniosynostosis.

BACKGROUND: The neuropsychological morbidity of nonsyndromic craniosynostosis is incompletely understood. The purpose of this study was to establish the prevalence of speech-language, cognitive, and behavioral abnormalities in this population and to stratify the findings on the basis of the affected suture and age of diagnosis with speech-language or psychological abnormalities. METHODS: Charts of all patients with nonsyndromic craniosynostosis evaluated between 1978 and 2000 were reviewed, noting diagnoses of speech-language, cognitive, or behavioral abnormalities. Findings were statistically analyzed for variance with regard to affected suture and diagnosis of abnormalities. RESULTS: Two hundred fourteen patients with nonsyndromic craniosynostosis had documented follow-up evaluations with an average age of 6 years 4 months at last visit. Speech, cognitive, and/or behavioral abnormalities were manifest in 49 percent of the patients with specific rates for each suture as follows: right unilateral coronal, 61 percent; bilateral coronal, 55 percent; multiple, 47 percent; metopic, 57 percent; left unilateral coronal, 52 percent; lambdoid, 44 percent; and sagittal, 39 percent. This prevalence of abnormalities was a statistically significant increase from the general population. Logistic regression demonstrated that as patient age increased, the percentage of abnormal diagnoses also increased. CONCLUSIONS: Nonsyndromic craniosynostosis is often associated with cognitive, speech, and/or behavioral abnormalities. The etiopathology of this association is unknown. Furthermore, the proportion of children diagnosed with cognitive and behavioral dysfunction increases with age. Therefore, longitudinal cognitive, behavioral, and speech assessment and treatment are integral to the care of these patients.

Child↗
PubMed2005

Relationship of brain and skull in pre- and postoperative sagittal synostosis.

Models of vertebrate skull evolution stress the coordinated developmental relationship between the skull and the brain that it houses. This study investigates the relationship between altered skull morphology and brain morphology in premature fusion of the cranial sagittal suture (isolated sagittal synostosis; ISS), a condition associated with dysmorphology of both neurocranium and brain. Although the skull displays a more normal shape following reconstructive cranial vault surgery, effects of this surgery on the brain have not been investigated. Landmark coordinate data were collected from three-dimensional magnetic resonance imaging reconstructions of the brain in a sample of ISS patients and an age-matched unaffected cohort. These data were analysed using Euclidean distance matrix analysis (EDMA). Results show that the brain in ISS is dysmorphic preoperatively, displaying a posteriorly directed neural expansion that does not 'worsen' with growth. Postoperatively, the brain in ISS displays a more globular shape overall as compared with the preoperative morphology, but differs from normal in its subcortical morphology. These results show that the ISS brain is altered following neurocranial surgery, but does not more closely approximate that of unaffected individuals. This suggests that although the brain is affected by manipulation of the skull, it retains a growth pattern that is, at least in part, independent of the skull.

Brain↗
PubMed2004

The effect of timing of surgery for velopharyngeal dysfunction on speech.

The timing of surgery for velopharyngeal dysfunction has been based on assumptions about the relation between age, speech development, and velopharyngeal dysfunction. Cleft palate teams often counsel parents to have an intervention for velopharyngeal dysfunction performed earlier rather than later, believing that earlier interventions result in more rapid or better normalization of speech. The objective of this retrospective chart review study is to determine whether the age at surgical intervention for velopharyngeal dysfunction has an effect on the subsequent length of speech therapy. Of 174 patients included in the study database, 36 had velopharyngeal dysfunction for which further velopharyngeal management was required. Of the 36 patients who received surgical velopharyngeal dysfunction management, 27 had verifiable speech therapy records. These 27 patients represent the study population. The outcome measure was the total length of subsequent speech therapy until speech normalization. The data suggest that there is no relation between the age at velopharyngeal dysfunction surgical management and the amount of speech therapy needed to achieve normalization of the speech impairments secondary to velopharyngeal dysfunction after that management. In conclusion, 1) the age at surgical velopharyngeal dysfunction management (pharyngeal flap or sphincter pharyngoplasty) does not have an effect on subsequent normalization of speech as measured by the duration of speech therapy necessary to achieve normalization of the speech impairments secondary to velopharyngeal dysfunction after that management, and 2) the age at surgical velopharyngeal dysfunction management does not affect the likelihood of subsequent surgical velopharyngeal dysfunction management procedures.

Age Factors↗
PubMed2004

Accuracy in identification of patients with 22q11.2 deletion by likely care providers using facial photographs.

Numerous facial characteristics are associated with velocardiofacial syndrome. Care providers may use these facial characteristics to identify patients who may benefit from fluorescence in situ hybridization genetic testing to determine the presence of the 22q11.2 deletion. The purpose of this study was to test the hypothesis that experienced care providers were able to correctly diagnose the 22q11.2 deletion on the basis of studying frontal facial photographs. After approval was obtained from the human studies committee, patients who had undergone fluorescence in situ hybridization genetics testing for the presence of a 22q11.2 deletion were asked to submit two frontal photographs: one at infancy and one beyond the second birthday. These photographs were randomized, made anonymous, and then placed on a secure Web site. Specialists in the fields of plastic surgery, otolaryngology, genetics, and speech pathology were asked to evaluate their experience and confidence levels in diagnosing a 22q11.2 deletion and were then asked to rate the photographs by likelihood of deletion using a five-point Likert scale. Thirty-two specialists (10 surgeons, nine geneticists, and 13 speech pathologists) participated in the study. On the basis of clear responses, respondents predicted the presence (sensitivity) and absence (specificity) of the 22q11.2 deletion at chance levels. Of the remaining responses, 20 to 25 percent were unsure and 20 to 25 percent were clearly wrong. When an unsure response was treated as a weak positive, the results favored sensitivity slightly, with a sensitivity of 70 percent and a specificity of 50 percent. Sensitivity improved somewhat with experience, as measured by the number of patients seen per year. The prediction of the presence or absence of the 22q11.2 deletion at chance levels suggests that the ability to diagnose on the basis of appearance alone is not a sufficient diagnostic tool. Although the ability does increase with experience, it is of statistical but not clinical significance.

Abnormalities, Multiple↗
PubMed2004

Coronal ring involvement in patients treated for unilateral coronal craniosynostosis.

The etiopathology of the clinical entity normally referred to as unilateral coronal synostosis is commonly used to connote unilateral fusion of the frontoparietal suture. However, other sutures in the coronal ring may exhibit synostosis concomitant with or independent from frontoparietal synostosis and give rise to similar clinical phenotypes. This study retrospectively analyzes high-resolution computed tomographic data sets to determine patency of sutures within the coronal ring. Computed tomographic scan digital data from 33 infants who subsequently underwent surgical correction of unilateral coronal synostosis were assessed for sutural patency using Analyze imaging software. The frontosphenoidal suture was subdivided into intraorbital frontosphenoidal and extraorbital frontosphenoidal portions, and the patency of the frontoethmoidal suture was also assessed. Patients were sorted into two groups on the basis of the status of their frontosphenoidal sutures: group 1 had patent frontosphenoidal but synostotic frontoparietal sutures (n = 21) and group 2 had both frontosphenoidal and frontoparietal synostoses. Observer reproducibility was tested. The vertical and horizontal dimensions of the bony orbit and the endocranial base deflection angle were measured with the observer blinded with regard to sutural status group. Frontoethmoidal synostosis was not noted in any patients in either group. Two patients had no frontoparietal suture synostosis with isolated intraorbital frontosphenoidal and extraorbital frontosphenoidal suture closures. Suture diagnosis reproducibility was 99 percent. In group 1, the ipsilateral-to-contralateral vertical orbit dimension ratio averaged 1.11, whereas in group 2 it averaged 1.04 (p < 0.05). The ratio of horizontal orbit measurements was not significantly different between groups. In both groups, the endocranial base was deflected ipsilateral to the synostotic frontoparietal suture, with an average angle of 12 degrees in group 1 and 17 degrees in group 2 (p < 0.005). The extent of synostosis along the coronal sutural ring contributes to the dysmorphology of the orbit and the endocranial base deflection in patients whose clinical phenotypic diagnosis is unilateral coronal synostosis.

Child, Preschool↗
PubMed2003

Simultaneous reconstruction of the secondary bilateral cleft lip and nasal deformity: Abbé flap revisited.

The purpose of this retrospective study was to review the method of using the Abbé flap for correction of secondary bilateral cleft lip deformity in selected patients with tight upper lip, short prolabium, lack of acceptable philtral column and Cupid's bow definition, central vermilion deficiency, irregular lip scars, and associated nasal deformity. A total of 39 patients with the bilateral cleft lip nasal deformity received Abbé flap and simultaneous nasal reconstruction during a period of 6 years. Mean patient age at the time of the operation was 19.1 years, and ranged from 6.6 to 38.5 years. The average follow-up period was 1.8 years. Fourteen patients had prior orthognathic operations. The Abbé flap was designed 13 to 14 mm in length and 8 to 9 mm in width and contained full-thickness tissue from the central lower lip, with a slightly narrow reverse-V caudal end. The prolabium, including the scars and central vermilion, was excised. Lengthening procedures of the upper lip segments were performed if vertical deficiency existed. Part of the prolabial skin was preserved and mobilized for columellar elongation, if indicated. Open rhinoplasty was carried out with or without cartilage graft for columella and nasal tip reconstruction. Reduction of the alar width and nostrils was achieved by a Z-plasty or excision of scar tissue at the nostril floor. The Abbé flap was then transposed cephalad, insetting into the median defect and sutured in layers. The results demonstrated no flap problems or perioperative complications. Seven patients needed further minor revisions on the nose and/or lip. Laser treatment was used to improve the lip scars in three patients. The patients were satisfied with the final outcome and found the lower lip scars acceptable. In conclusion, the described technique of Abbé flap and simultaneous rhinoplasty is an effective reconstructive method for select patients with bilateral cleft lip and nasal deformity.

Adolescent↗
PubMed2003

BMP4 promotes chondrocyte proliferation and hypertrophy in the endochondral cranial base.

Defects in the growth and development of the endochondral bones that comprise the cranial base contribute to several craniofacial dysmorphic syndromes. Since Bone Morphogenetic Protein (BMP) signaling regulates chondrocyte differentiation and endochondral ossification in developing long bones, we have tested the hypothesis that BMP signaling also participates in regulating development of the cranial base. During in vivo developmental progression of the cranial base in mice, a burst of skeletal growth and chondrocyte maturation was identified in the perinatal period. Using a novel serum-free organ culture system, cranial base structures were cultured as explants in the presence of BMP4 or noggin, and analyzed for morphological and molecular changes. Growth of perinatal cranial base explants was inhibited by treatment with noggin, a BMP inhibitor. Exogenous BMP4 promoted cartilage growth, matrix deposition and chondrocyte proliferation in a dose dependent manner. Correspondingly, expression level of the cartilage markers Sox9 and collagen type II were also increased. Alkaline phosphatase and collagen type X expression were up-regulated and expressed in ectopic hypertrophic chondrocytes after treatment of the cultures with 100 ng/ml BMP4 for seven days. This increase in chondrocyte hypertrophy was accompanied by increased indian hedgehog (Ihh) and parathyroid hormone/parathyroid hormone related peptide (PTH/PTHrP) receptor (PPR) expression, but not increased PTHrP expression. We conclude that endogenous BMPs are required to maintain cartilage growth, and exogenous BMP4 can enhance cartilage maturation and induce ectopic chondrocyte hypertrophy in the cranial base. Therefore, appropriate levels of BMP signaling are important for normal cranial base development.

Animals↗
PubMed2002

A new method for the study of velopharyngeal function using gated magnetic resonance imaging.

The purpose of this project was to assess the feasibility of imaging the velopharynx of adult volunteers during repetitive speech, using gated magnetic resonance imaging (MRI). Although a number of investigators have used conventional MRI in the study of the human vocal tract, the mismatch between the lengthy time necessary to acquire sufficiently detailed images and the rapidity of movement of the vocal tract during speech has forced investigators to acquire images either while the subject is at rest or during sustained utterances. The technique used here acquired a portion of each image during repetitive utterances, building the full image over multiple utterance cycles. The velopharyngeal portal was imaged on a 1.5-Tesla GE Signa LX 8.2 platform with gated fast spoiled gradient echo protocol. An external 1-Hertz trigger was fed to the cardiac gate. Subjects synchronized utterance of consonant-vowel syllables to a flashing light synchronized with the external trigger. Each acquisition of 30 phases per second at a single-slice location took 22 to 29 seconds. Four consonant-vowel syllables (/pa/, /ma/, /sa/, and /ka/) were evaluated. Subjects vocalized throughout the acquisition, beginning 5 to 6 seconds beforehand to establish a regular rhythm. Imaging of the velopharyngeal portal was performed for sagittal, velopharyngeal axial (aligned perpendicular to the "knee" of the velum), axial, and coronal planes. Volumes were obtained by sequential acquisition of six to 10 slices (each with 30 phases) in the axial or sagittal planes during repetition of the /pa/ syllable. Spatiotemporal volumes of the single-slice data were sectioned to provide time-motion images (analogous to M-mode echocardiograms). Three-dimensional dynamic volume renderings of palate motion were displayed interactively (Vortex; CieMed, Singapore). A method suitable for the collection and visualization of four-dimensional information regarding monosyllabic speech using gated MRI was developed. These techniques were applied to a population of adult volunteer subjects with no history of speech problems and two patients with a history of cleft lip and palate. The techniques allowed good real-time visualization of velopharyngeal anatomy during its entire range of motion and was also able to image pathology-specific anatomic differences in the subjects with cleft lip and cleft palate. These methods may be applicable to a wide spectrum of problems in speech physiology research and for clinical decision-making regarding surgery for speech and outcomes analysis.

Adult↗
PubMed2002

Effect of distraction osteogenesis of the mandible on upper airway volume and resistance in children with micrognathia.

Children with craniofacial anomalies often have compromise of the upper airway, a condition with potential for morbidity and mortality. In children with microretrognathia, the diminutive size and retruded position of the mandible reduces the size of the oropharynx, thereby predisposing to glossoptosis and airway obstruction. Although several authors have reported successful use of mandibular distraction osteogenesis to alleviate this type of upper airway obstruction, the physiologic relationship between changes in mandibular shape, size, and position and upper airway dynamics remains undefined. The purpose of this study was to develop methodologies to quantitatively evaluate upper airway dynamics in children with micrognathia both before and after mandibular distraction osteogenesis. The patient population consisted of four children with micrognathia who had successfully undergone upper airway stabilization by bilateral mandibular distraction osteogenesis. The data used were digitally archived computed tomographic scan data from high-resolution, thin-slice head computed tomographic scans obtained before and after mandibular distraction. Upper airway evaluation was performed in two ways: static and dynamic. Static analysis consisted of computer quantification of predistraction and postdistraction mandibular and upper airway volumes using Analyze imaging software. Dynamic analysis consisted of fabrication of rigid stereolithographic hollow cast models of the upper airway produced from computed tomographic scan data. Models were used for characterization of upper airway resistance and flow patterns as related to respiration. After distraction osteogenesis, mandibular total volume increased 32, 32, 18, and 25 percent (mean, 27 percent) and upper airway volume increased by 20, 31, 23, and 71 percent (mean, 37 percent). A significant decrease in flow resistance, both inspiratory and expiratory, was observed in the patient with the greatest upper airway volume increase (71 percent) after distraction. After distraction, the inspiratory resistance was diminished by 51 percent and the expiratory resistance diminished by 85 percent. However, the three patients with more modest upper airway volume increases of 20 to 31 percent demonstrated no statistically significant change in flow resistance after distraction. Results of this study support the conclusion that distraction osteogenesis of the micrognathic mandible increases the volume of the upper airway, roughly paralleling the increase in mandibular volume. In the biomechanical airway model studied, upper airway volume expansion has been shown to be able to decrease the flow resistance over the length of the airway, presumably secondary to an increase in the average cross-sectional area. The artificial rigidity of the stereolithographic "airway" compared with the elasticity of the human upper airway may account for the insensitivity of this model to smaller but clinically significant airway changes.

Airway Resistance↗
PubMed2002

A cephalometric study of facial growth in van der Woude syndrome.

OBJECTIVE: To test the hypothesis that maxillary growth and lower lip form in patients with van der Woude syndrome (VDW) is different from patients with non-syndromic cleft lip and palate. DESIGN AND SETTING: Retrospective, case-control study at a tertiary cleft center, Chang Gung Memorial Hospital, Taipei, Taiwan. PATIENTS AND PARTICIPANTS: Records of 53 patients with VDW, who presented for treatment during the years 1968 through 1998 were obtained. Twenty-three of 53 patients had received at least one lateral cephalogram during the course of their treatment. Of these 23, in 17 it was possible to find non-syndromic case controls with identical cleft type, sex, and method of cleft palate repair, with year of birth matching within 1 year of the corresponding VDW patient. For these 17 pairs of VDW and non-syndromic cleft controls, cephalogram acquisition dates were checked to see how well the corresponding pairs matched. A total of 43 pairs of cephalograms were deemed to be acceptably matched because the ages at acquisition differed by less than 15% of the VDW patient's age. MAIN OUTCOME MEASURES: Thirteen measurements were derived from the 11 standard lateral cephalometric landmarks recorded on each cephalogram. The data were classified into five groups according to age at time of cephalogram, and sets of paired non-syndromic cleft and VDW measurements were tested for differences using a Wilcoxon signed rank sum test in two ways, first including all cleft types and then including only those patients with complete bilateral cleft lip and palate. A longitudinal growth analysis considering the movement of the skeletal A and B points was performed on the patients with complete bilateral cleft lip and palate. RESULTS: For the osseous measurements, anteroposterior maxillary length as described by the anterior nasal spine (ANS)-posterior maxillary point distance was statistically significantly shorter in the VDW patients of age 13 years and older, by 5.3 mm. Maxillary height, as described by the nasion-ANS distance was shorter in the VDW patients, closely approaching statistical significance in the age range 7 through 11 years. The lip soft tissue measurements showed significantly greater protrusion over several age ranges in the VDW patients. The longitudinal growth analysis showed a significantly more inferior vertical position of the B point in the controls. CONCLUSIONS: This study demonstrates a few statistically significant differences in maxillary growth and lip conformation between VDW and matching controls. Small sample sizes for each age group hamper the ability to fully interpret or generalize the pattern of these differences.

Adolescent↗