Anne-Marie Kuijpers-Jagtman

Early secondary osteotomy-stabilization of the premaxilla in bilateral clefts

Using the same arguments as for early bone grafting of the alveolar process in unilateral clefts, the cleft team of the University of Nijmegen started about 10 years ago to apply early osteotomy-stabilization of the premaxilla to bilateral clefts. A series of 13 cases with a minimum follow-up of 15 months is presented. The patients were operated on at the age of 8 2/12 to 12 5/12 years. The results are considerably better than when doing the same operation in the adult. More than 90% are successful. In comparison with adults we additionally register more favourable eruption of the canine, the possibility of closing the dental arch without prosthetic appliances and in some cases also the elimination of a psychological handicap. The inhibition of growth by this operation seems not to be important. If need be, Le Fort I osteotomies are possible after completion of growth. They will be in one piece which is technically easier than the usual three-segment Le Fort I. In conclusion we prefer early secondary osteotomy and stabilization of the premaxilla to the tertiary operation.

Infant Orthopedics and Facial Growth in Complete Unilateral Cleft Lip and Palate Until Six Years of Age (Dutchcleft)

Objective: To evaluate longitudinally the effect of infant orthopedics (IO) on dentofacial cephalometric variables in unilateral cleft lip and palate (UCLP) patients from 4 to 6 years of age. Design: Prospective two-arm randomized controlled clinical trial in three cleft palate centers in The Netherlands (Dutchcleft trial). Patients: Fifty-four children with complete UCLP. Interventions: Patients were divided randomly into two groups. Half of the patients (IO+) had IO until surgical closure of the soft palate at the age of ±52 weeks; the other half (IO−) received no intervention. Mean Outcome Measures: Cephalometric values representing soft tissue, hard tissue, and dental structures, measured on lateral headfilms made at 4 and 6 years of age. Results: In the IO+ group, 21 patients were analyzed; in the IO− group, 20 patients were analyzed at age 4 and 22 at age 6. No differences were found between IO+ and IO−, except for two measurements: The interincisal angle was larger and the mentolabial angle was smaller in the IO+ group. Conclusions: For infants with UCLP whose surgical management included soft palate repair at 12 months and delayed hard palate closure, cephalometric outcomes at ages 4 and 6 provide no indication for the type of IO used in this study.

Histologic evaluation of skin-derived and collagen-based substrates implanted in palatal wounds

Tissue shortage complicates the surgery of cleft lip and palate anomalies and the healing of defects on the palate impairs growth of the dento‐alveolar complex due to scar tissue formation. Implantation of substitutes into the wound area might overcome this adverse effect. The aim of this study was to compare the tissue response to three collagen‐based (collagen type I substrate alone, or collagen coated with elastin or chondroitin‐6‐sulfate) and two skin‐derived substrates (unprocessed dermis and AlloDerm) after implantation into 12 dogs. Histology was performed at 3, 10, and 20 days postsurgery. We showed that all substrates were well tolerated. However, it is unclear whether AlloDerm was rapidly degraded or if it was sequestrated. There was no elastin or collagen present in these wounds. All collagen‐based substrates showed good epithelial regeneration, although heparan sulfate (JM 403) was absent. Wounds treated with the collagen‐based substrates contained fewer myofibroblasts at 20 days postsurgery and the type III collagen fibers in the immature scar tissue were more randomly oriented than in an untreated wound. In conclusion, palatal wounds with a dermal substrate heal with fewer indications of scar tissue formation and evoke only a mild inflammatory reaction, which is preferred over the tissue reaction in an untreated wound.

Presurgical orthopedics and satisfaction in motherhood: a randomized clinical trial (Dutchcleft)

Objective: To study the effect of infant orthopedics on satisfaction in motherhood. Design: Prospective two-arm randomized controlled trial in parallel with three participating academic cleft palate centers. Treatment allocation was concealed and was performed by means of a computerized balanced allocation method. Setting: Cleft palate centers of Amsterdam, Nijmegen, and Rotterdam, the Netherlands. Patients: Two groups of infants with complete unilateral cleft lip and palate and no other malformations. Interventions: Group IO+ (n u200a=u200a 27) wore passive maxillary plates during the first year of life, group IO− (n u200a=u200a 27) did not. Main Outcome Measure(s): Mean satisfaction scores were obtained from completed questionnaires at 6, 24, and 58 weeks of age. A 4-point scale was used (1 u200a=u200a very satisfactory to 4 u200a=u200a very unsatisfactory). Results: The range of the mean scores for the individual items on the questionnaires for both groups ranged between 1.1 and 2.4. No differences were found between groups. Mothers appear to be satisfied in motherhood, least satisfied with the available time for themselves, and very satisfied with hugging and walking their babies. No differences were found between groups. Conclusions: Results from the present study show that infant orthopedics, with a passive plate during the first year of life, in children with a unilateral cleft lip and palate has no influence on the mothers’ satisfaction in motherhood.

Early secondary osteoplastic closure of the residual alveolar cleft in combination with orthodontic treatment

In 84 early secondary bone graftings, rib and chin transplants were used. While good overall results are obtained, comparison of the two types of graft show that the cases with bone from the chin clearly do better.

Variability in dentofacial phenotypes in four families with WNT10A mutations

This article describes the inter- and intra-familial phenotypic variability in four families with WNT10A mutations. Clinical characteristics of the patients range from mild to severe isolated tooth agenesis, over mild symptoms of ectodermal dysplasia, to more severe syndromic forms like odonto-onycho-dermal dysplasia (OODD) and Schöpf–Schulz–Passarge syndrome (SSPS). Recurrent WNT10A mutations were identified in all affected family members and the associated symptoms are presented with emphasis on the dentofacial phenotypes obtained with inter alia three-dimensional facial stereophotogrammetry. A comprehensive overview of the literature regarding WNT10A mutations, associated conditions and developmental defects is presented. We conclude that OODD and SSPS should be considered as variable expressions of the same WNT10A genotype. In all affected individuals, a dished-in facial appearance was observed which might be helpful in the clinical setting as a clue to the underlying genetic etiology.

Dental development in hemifacial microsomia

Hemifacial microsomia (HFM) is a congenital disorder marked by facial asymmetry. Whether facial asymmetry accounts for asymmetrical dental development is unknown. There are few data on dental development relative to mandibular development or severity of HFM, or on development over time. We hypothesized that when mandibular development was severely disturbed, local dental development was also affected. We compared dental development scores between affected and non-affected mandibular sides in patients with HFM (n = 84) and compared these data with those collected from Dutch control children (n = 451). Logistic functions were constructed for dental age over time for all four Pruzansky/Kaban types. The results showed a tendency toward delayed dental development in Pruzansky/Kaban types IIb and III at younger ages. The temporary delay of tooth formation in patients with severe forms of HFM and the distribution of agenic teeth suggest an interaction between mandibular and dental development.

Prenatal Ultrasound Diagnosis in 51 Cases of Holoprosencephaly: Craniofacial Anatomy, Associated Malformations, and Genetics

Objective To analyze the prenatal ultrasound findings of the craniofacial and extracephalic anatomy, the postnatal pathological findings, and the genetic anomalies in 51 cases of holoprosencephaly (HPE). Materials and Methods Between 1990 and 2005, a collective of 51 fetuses with tentative ultrasound diagnosis of HPE was recruited at two tertiary referral centers for prenatal ultrasound diagnostics via the Pia Fetal Database (GE Medical Systems, Webling, Germany). Cephalic as well as extracephalic anomalies were investigated, and all cases were subdivided into the subgroups lobar, alobar, and semilobar HPE. In addition to the ultrasound investigation, 45 fetuses were analyzed for genetic anomalies and 21 fetuses underwent an autopsy. Results The average age at diagnosis was 21.9 weeks of gestation. There was a greater number of female fetuses, with an overall ratio of 2.67:1. In 61% of all cases, there was a reduction of growth in comparison with healthy fetuses of the same age. Within the second trimenon, the cephalic anomalies became evident when investigating the diameter of the fetal head (second trimenon: 71% below the fifth percentile; third trimenon: 92% below the fifth percentile). In 82% of the cases, extracephalic anomalies were diagnosed additionally. In 63%, the diagnosis of holoprosencephaly led to a termination of pregnancy. Ten percent of the fetuses were born alive. In 81% of the cases, the diagnosis of HPE was confirmed postnatally. The remaining 19% showed other severe cephalic and extracephalic anomalies. Chromosomal anomalies were detected in 79% of the fetuses, most frequently trisomy 13 (59%). Discussion Because of recent advances in the development and improvement of high-resolution ultrasound, early diagnosis of congenital anomalies such as HPE is now possible. In this study, which represents the largest collection of prenatally diagnosed HPE reported in the literature to date, the average age at diagnosis was earlier than in other studies. The ultrasound devices of today provide excellent images of the fetus that allow an exact diagnosis of craniomaxillofacial anomalies as well as extracephalic anomalies. Apart from a very few cases, the diagnosis of HPE is incompatible with life.

Timing of facial osteotomies: A consensus conference

A concensus conference on the timing of facial osteotomies was held in Jönköping, Sweden. Seven teams consisting of a maxillofacial surgeon and an orthodontist representing five countries discussed treatment planning for young patients with maxillomandibular deformities with special emphasis on timing. Patients with severe syndromic facial deformities were not considered. Consensus was reached that for most deformities it is best advised to wait until growth has ceased, as determined by longitudinal cephalometric data. This rule is less tight for Angle Class II than for Class III cases. For asymmetries often there is still not enough scientific evidence to obtain well-founded uniform agreement.

Radiologic analysis of the effects of subperiosteal palatal soft-tissue expansion in growing cats

Palatal soft-tissue expansion might be appropriate for use in cleft palate surgery. Seventy-five cats were divided into four different experimental groups and one control group. Intraoral tissue expansion was started at the age of 14 weeks in normal or scarred mucoperiosteum. The experiment lasted until 24 weeks of age. Serial standardized lateral cephalograms from each animal were digitized, and the results were statistically analyzed. The results indicate that the effects are independent of the presence of scarred tissue, that sagittal growth is impaired by tissue expansion, and that the tissue expander induced resorption of palatal bone.