John W. Canady

Structural brain abnormalities in adult males with clefts of the lip and/or palate.

Purpose: To evaluate brain morphology of adult males with nonsyndromic clefts of the lip and/or palate (NSCLP) in comparison to a matched healthy control group.Methods: Brain structure was measured using quantitative analysis of magnetic resonance images.Results: Subjects with NSCLP had significant abnormalities in brain morphology consisting of abnormally enlarged anterior regions of the cerebrum, and decreased volumes of the posterior cerebrum and cerebellum. Overall, the most severely affected region was the left temporal lobe. Furthermore, these structural abnormalities were directly related to cognitive dysfunction.Conclusions: These findings highlight the important relationship and interplay between face and brain development.

Suitability of the Iliac Crest as a Site for Harvest of Autogenous Bone Grafts

Many donor sites have been advocated for obtaining cancellous bone to be used for grafting alveolar defects. Recently, some authors have suggested that the iliac crest site produced an unacceptably high degree of postoperative morbidity. Because of this morbidity, the use of other donor areas, e.g., rib, cranium, and mandible, are advocated. The iliac crest donor site for alveolar bone grafting has been common practice in our institution for years under the assumption that little or no postoperative morbidity occurred. To investigate and document the accuracy of our assumption, the charts of the most recent 50 consecutive patients receiving alveolar bone grafts, all of which used the iliac crest as a donor site, were reviewed. Postoperative pain, numbness, infection, paralysis, or long-term disability were catalogued from the charts. In addition, a questionnaire was sent to the parents and patients requesting their perceptions of these parameters. In the cases reviewed, the average length of skin incision was 4.2 cm. The average quantity of bone harvested was 4.1 cc. The usual hospitalization time was 2-3 days. No patients experienced serious or long-term complications at the surgical donor site. Immediate postoperative complications among the 50 patients studied were limited to one hematoma, one stitch abscess, one case of swelling with pain, one erythema, and one 4-day fever accompanied by slight serosanguineous drainage. Information from the questionnaire suggests that most patients returned to full activity within 4-6 weeks. No patient reported any long-term pain or disability.(ABSTRACT TRUNCATED AT 250 WORDS)

Conductive hearing loss and otopathology in cleft palate patients

OBJECTIVES: Assess incidence of conductive hearing loss, ear pathology, and associated communicative disorders in cleft palate patients. STUDY DESIGN: Retrospective chart review of 101 patients all treated at a tertiary facility since birth. RESULTS: The median patient age was 19 years old (range 8–25) at last follow-up, 35% female. Median age of cleft palate repair was 16 months (range 12–60). Median number of myringotomy tubes was 3 (range 1–7). Conductive hearing loss (CHL) greater than 20 db PTA was found in 25% of patients at last follow-up. Severity of CHL was mild in 75%, moderate in 21%, and severe in 4%. Cholesteatoma was identified in 5.9%. The mean age at resolution of CHL was 5 years (range 3–19). Risk factors associated with CHL at last follow-up included middle ear surgery (P = 0.016), cholesteatoma (P = 0.003), and 4 or more myringotomy tube insertions (P = 0.030). Associations between CHL and age at cleft repair, speech impairment, or learning disabilities were not found. CONCLUSIONS: Children requiring increased number of myringotomy tubes and middle ear surgery and found to have cholesteatoma are at increased risk for long-standing CHL. EBM rating: C-4 SIGNIFICANCE: Cleft palate children requiring multiple tube insertions should be monitored closely for CHL.

Cognitive dysfunction in adult males with non-syndromic clefts of the lip and/or palate

Cognitive deficits have been well-documented in children with non-syndromic clefts of the lip and/or palate. However, no study to date has formally assessed cognition in adults with oral clefts. This study was designed to evaluate neuropsychological functioning in adult males with non-syndromic clefts of the lip and/or palate (n=50) compared to age and sex-matched controls. Subjects with oral clefts were found to have significantly lower full scale IQ, performance IQ and verbal IQ scores compared to their matched controls. After controlling for IQ, patients showed specific deficits in verbal fluency. Adult males with oral clefts manifest a specific pattern of cognitive deficits. As the development of the face is highly interdependent with the development of the brain, it is theorized that the etiology of these cognitive deficits is a primary problem with abnormal brain development.

Pharyngeal flap surgery: long-term outcomes at the University of Iowa.

The pharyngeal flap is the most often used surgical approach to treat the problem of velopharyngeal insufficiency, a common challenge encountered in cleft palate and craniofacial clinics. The authors retrospectively reviewed short-term and long-term measures of children treated with the pharyngeal flap at the University of Iowa Cleft and Craniofacial Center. All patients who underwent pharyngeal flap surgery between January of 1970 and December of 2000, with at least one postoperative speech assessment between 2 and 5 years after the operation, were identified. Both hypernasality and hyponasality were evaluated on a scale from 1 to 6, with 1 indicating no involvement and 6 indicating severe effect on resonance. Velopharyngeal competence was also rated on a scale of 1 to 3, with 1 indicating competence and 3 indicating incompetence. These short-term data were then compared. The results showed that overall resonance performance continues to be adequate and may even improve as the patient continues to grow and mature. These findings support the use of the pharyngeal flap in the treatment of children with velopharyngeal insufficiency.

Health-Related Quality of Life Among Preadolescent Children With Oral Clefts: The Mother's Perspective

OBJECTIVE. The purpose of this study was to evaluate the factors that affect the health-related quality of life of preadolescent children with nonsyndromic oral clefts using the Pediatric Quality of Life Inventory instrument and to evaluate whether there were any differences related to the type of cleft or other factors. METHODS. Data for this study were derived from telephone interviews with the parents of a statewide population of children who were in the Iowa Registry for Congenital and Inherited Disorders, were aged 2 to 12 years, had nonsyndromic oral clefts, and were born in Iowa between January 1, 1990, and December 31, 2000. Twenty-minute interviews were conducted with mothers of 104 children in the spring and summer of 2003; respondents then completed and mailed back Pediatric Quality of Life Inventory surveys 2 to 3 weeks after the interviews (69% participation rate). RESULTS. After controlling for demographic characteristics, children with less severe speech problems had higher total Pediatric Quality of Life Inventory scores as well as higher physical and psychosocial health domain scores. Age and cleft type interacted, with younger children (aged 2–7 years) with a cleft lip or cleft lip and palate having higher health-related quality of life scores than children with an isolated cleft palate; however, this pattern was reversed for older children (aged 8–12 years). CONCLUSIONS. Speech and aesthetic concerns seem to have been important factors affecting the health-related quality of life for children with oral clefts. These factors seem to be more important as children get closer to adolescence (ages 8–12 years) when acceptance by peers becomes more critical.

Increased incidence of a midline brain anomaly in patients with nonsyndromic clefts of the lip and/or palate.

Background and Purpose. Nonsyndromic clefts of the lip and palate (CLP) are developmental craniofacial abnormalities that are often associated with cognitive dysfunction. This study was designed to evaluate, in patients with CLP, the presence of a specific midline brain anomaly (enlarged cavum septi pellucidi [CSP]) that has been shown in other developmental syndromes to be related to poor cognitive function. Methods. Brain images were obtained using magnetic resonance imaging on 49 adult men with CLP and 75 healthy controls. Size of CSP was measured using consecutive coronal images. Results. The incidence of large CSP in the CLP group was 8% (4 of 49), significantly higher than that found in the control group. In 2 of these 4 subjects, the anomaly was complete nonfusion of the septal leaflets, known as a combined CSP and cavum vergae. Furthermore, there was a significant inverse relationship of IQ and CSP in CLP patients that was not present in controls. That is, in individuals with CLP, the larger the CSP, the lower the IQ. Conclusions. Adult men with CLP have an increased prevalence of enlarged CSP. Moreover, this anomaly is directly related to cognitive deficits. This study provides further evidence that the development of the face and the development of the brain are intimately related and that defects in craniofacial development are most likely associated with defects in brain development.

An Anatomic Study of the Tensor Veli Palatini and Dilatator Tubae Muscles in Relation to Eustachian Tube and Velar Function

In a gross anatomic study of 20 sides in 16 human head specimens, the tensor veli palatini, the dilatator tubae, and the tensor tympani muscles were studied. The tensor veli palatini was observed to insert onto the anterior one-third of the pterygoid hamulus, whereas the dilatator tubae rounded the middle one-third of the pterygoid hamulus without an insertion. Thus, the dilatator tubae, not the tensor veli palatini, could serve to tense the anterior velum. An insertion from the superior pharyngeal constrictor muscle onto the posterior one-third of the hamulus could provide a curbing function for the dilatator tubae muscle. Adipose tissue, located at the hamulus, could provide lubrication for the tendinous fibers of the dilatator tubae as they round the hamulus. The dilatator tubae was observed to attach to the hook of the eustachian tube and is accepted as the tubal dilator. Observed on 13 of 20 sides in 11 specimens, the bulk of the dilatator tubae remained distinct from the tensor veli palatini despite a connective tissue alliance and intermingling of some muscle fibers. On 5 of 20 sides in 5 specimens, fibers of the dilatator tubae intermingled extensively with the tensor veli palatini. Of the 20 dilatator tubae muscles dissected, 2 were observed to be deficient. The tensor veli palatini was observed to be continuous with the tensor tympani. Full color versions of the figures are available at the following website: http://www.shc.uiowa.edu/papers/tensor/.

Cleft palate and craniofacial teams in the United States and Canada : A national survey of team organization and standards of care

Objective This study is the first comprehensive national survey of the organization, function, and composition of cleft palate and craniofacial teams in the U.S. and Canada. Complete descriptions of cleft and craniofacial teams are not currently provided in the literature, and this study will provide an overview for health services research and policy use. Conducted by a national organization, this study examines teams in detail using a pretested and standardized methodology. Design All known (n = 296) North American cleft palate and craniofacial teams were contacted for team listing purposes using a self-assessment method developed by an interdisciplinary committee of national stature. Team clinical leaders classified their teams into several possible categories and provided data on team care. The response rate was 83.4% (n = 247). Results The distribution of listed teams was: 105 (42.5%) cleft palate teams, 102 (41.3%) craniofacial teams (including craniofacial teams that are both cleft palate and craniofacial teams), 12 (4.9%) geographically listed teams, and 28 (11.3%) other teams (including interim cleft palate teams, low-density cleft palate teams, and evaluation and treatment review cleft palate teams). Eighty-five percent of all teams systematically collected and stored clinical data on their teams patient population in the past year. Furthermore, 50% of all teams had a quality assurance program in place to measure treatment outcomes. Other findings presented include the annual number of face-to-face team meetings; new and follow-up patient censuses; and surgical rates for initial repair of cleft lip/palate, orthognathic/osteotomy procedures, and intracranial/craniofacial procedures. Conclusions Two of five North American teams classify themselves as having the capacity to provide both cleft palate and craniofacial care. An additional two of five teams limit their primary role to cleft palate care. Issues are raised regarding the distribution of teams, the regionalization of craniofacial services, health policy, and resource allocation.

Abnormal brain morphology in patients with isolated cleft lip, cleft palate, or both: a preliminary analysis.

OBJECTIVE The aim of this study was to determine whether adult men with cleft lip and palate (CLP) have aberrant cerebral morphology. DESIGN Brain morphology of 14 adult men with isolated CLP were analyzed and compared with 14 healthy controls matched for sex, age, and parental socioeconomic status. SETTING The research took place at a large, tertiary care hospital, with participation on an outpatient basis. PARTICIPANTS The 14 males with CLP were recruited from a large cleft lip and palate registry, while their 14 matched controls were selected from a registry of healthy volunteers collected via the Mental Health Clinical Research Center. RESULTS The males with CLP have significantly smaller cerebellar size (p = .04), significantly larger frontal lobes (p = .02), and significantly smaller temporal and occipital lobes (p = .02; p = .009, respectively). No significant difference in gray/white matter ratios or laterality were found. CONCLUSIONS Adult males with CLP have a significantly different pattern of brain morphology, compared with healthy controls, which is most likely due to aberrant cerebral development. This study highlights the complex interaction and interdependence of craniofacial and cerebral development.