Maria Costanza Meazzini

Prevalence of abnormalities in dental structure, position, and eruption pattern in a population of unilateral and bilateral cleft lip and palate patients.

Objective: To evaluate the dental characteristics of patients subjected to a protocol that included early secondary gingivoalveoloplasty (ESGAP). Design: Panoramic radiographs of 87 patients with unilateral cleft lip and palate (UCLP) and 29 with bilateral cleft lip and palate (BCLP) were evaluated. Missing and supernumerary teeth were also quantified on the cleft and noncleft side and in the maxilla and mandible. Crown and root malformations and tooth rotations were quantified. A subsample in permanent dentition was extrapolated to analyze canine eruption patterns. Results: A total of 48.8% of the UCLP patients presented with missing permanent lateral incisors in the cleft area and 6.1% contralaterally. A total of 4.9% presented with missing second maxillary premolars on the cleft site and 1.2% contralaterally. A total of 7.3% presented with supernumerary lateral incisors, and 45% of the BCLP cleft sites presented with missing lateral incisors, while 25% of the cleft sites presented second maxillary premolars agenesis. Five percent of the cleft sites presented with supernumerary lateral incisors. Evaluation of the subsample in permanent dentition showed that 15.5% had a canine retention and 4.4% of the canines had to be surgically exposed. A significant association was observed between canine inclination and retention but not with absence of the lateral incisor. Conclusions: The frequency of dental anomalies in this sample was similar to other cleft populations. As surgical trauma has been suggested to damage forming teeth, the results of this study indicated that ESGAP has no detrimental influence on subsequent dental development.

Le Fort III advancement osteotomy in the growing child affected by Crouzon's and Apert's syndromes: presurgical and postsurgical growth

This is a longitudinal cephalometric study of patients with craniofacial synostosis (Crouzons and Aperts) syndromes who underwent Le Fort III advancement during early childhood. The objectives of the study were to quantify presurgical growth, surgical results, and surgical stability as well as long-term postsurgical growth. Of the 31 patients operated on during their growth period (less than 10 years of age), 17 had sufficient records to be included in the study: at infancy, before the Le Fort III osteotomy (average age = 7.3 years, range: 4.8-10 years), and immediately after surgery. Ten of the patients were followed radiographically long term for an average of 6.1 ± 2.7 years (range: 4.5-10.8 years). The presurgical abnormal midfacial vertical growth pattern was accurately described with a progressive class III discrepancy and an increasing exophthalmos. Remarkable postoperative stability of the maxillary segment was observed. After surgery, growth of the midface was measured, together with minimal, if any, anterior growth, similar to the presurgical growth pattern and rate. No actual detrimental or beneficial effect of surgery on subsequent growth was seen. This study quantitatively confirms that the standard Le Fort III osteotomy is a stable and effective but generally not definitive procedure in childhood. This study might also serve as a control sample to compare with groups of patients undergoing distraction osteogenesis to verify the actual advantages and shortcomings of this alternative technique.

Comparison of mandibular vertical growth in hemifacial microsomia patients treated with early distraction or not treated: Follow up till the completion of growth

AIM Comparison of the long-term follow-up until the completion of growth of two homogeneous samples of children affected by hemifacial microsomia (HFM), one treated by mandibular distraction osteogenesis (DO) in the deciduous or early mixed dentition, the other not subjected to any treatment until adulthood. MATERIAL Fourteen patients affected by vertically severe type I or II HFM were operated at an average age of 5.9 years with an average follow-up of 11.2 years. They were compared to a sample of eight patients who were never treated until the completion of growth. METHODS Mandibular vertical changes were measured on panoramic radiographs taken at different time points. Ratios between affected and non affected ramal heights were calculated and compared. RESULTS In the DO sample, after correction, mandibular vertical changes showed a gradual return of the asymmetry with growth in all patients. The ratio in the non treated sample was unchanged between the initial and the long term panoramic x-rays. CONCLUSION The facial proportions of HFM patients are maintained, when not treated, throughout growth. The same proportions return to their original asymmetry after DO. Even though short term aesthetic and psychological advantages of distraction osteogenesis are well accepted, early surgery should only be applied after careful patient selection and honest clarification of the long term recurrence by genetically guided craniofacial growth pattern.

Alveolar bone formation in patients with unilateral and bilateral cleft lip and palate after early secondary gingivoalveoloplasty: long-term results.

Background: The Milan surgical protocol includes the use of an early secondary gingivoalveoloplasty together with hard palate closure at 18 to 36 months, to avoid later bone grafting. The goal of this study was to evaluate the long-term quality of ossification in patients who have undergone early secondary gingivoalveoloplasty. Methods: The samples consisted of panoramic radiographs of 87 unilateral cleft lip–cleft palate and 29 bilateral cleft lip–cleft palate patients. The records available allowed for a longitudinal and a cross-sectional evaluation of the ossification in the cleft area. Alveolar bridging was assessed using a modified Bergland’s scoring system. Nasal area ossification and canine inclination were each given three different qualitative scores. Results: The alveolar bridging noted was type I (71.7 percent), type II (23.5 percent), and type III (4.8 percent) in the whole sample of unilateral and bilateral cleft lip–cleft palate patients. No type IV ossification was found. Longitudinal analysis showed that approximately one-fourth of the cleft sites improved after permanent tooth eruption, and very few worsened. An evaluation of permanent dentition in a group of 27 unilateral and nine bilateral cleft lip–cleft palate patients (mean age, 14.8 ± 2.0 years) showed that 15.5 percent of the whole sample had canine retention and 4.4 percent of the whole sample had to be surgically exposed. Conclusions: Early secondary gingivoalveoloplasty seems to allow for adequate ossification in both the alveolar and the nasal regions. Permanent tooth eruption occurs at a normal rate. None of the patients has required a secondary alveolar bone graft.

Recommendations of the Scientific Committee of the Italian Beckwith–Wiedemann Syndrome Association on the diagnosis, management and follow-up of the syndrome

UNLABELLED Beckwith-Wiedemann syndrome (BWS) is the most common (epi)genetic overgrowth-cancer predisposition disorder. Given the absence of consensual recommendations or international guidelines, the Scientific Committee of the Italian BWS Association (www.aibws.org) proposed these recommendations for the diagnosis, molecular testing, clinical management, follow-up and tumor surveillance of patients with BWS. The recommendations are intended to allow a timely and appropriate diagnosis of the disorder, to assist patients and their families, to provide clinicians and caregivers optimal strategies for an adequate and satisfactory care, aiming also at standardizing clinical practice as a national uniform approach. They also highlight the direction of future research studies in this setting. With recent advances in understanding the disease (epi)genetic mechanisms and in describing large cohorts of BWS patients, the natural history of the disease will be dissected. In the era of personalized medicine, the emergence of specific (epi)genotype-phenotype correlations in BWS will likely lead to differentiated follow-up approaches for the molecular subgroups, to the development of novel tools to evaluate the likelihood of cancer development and to the refinement and optimization of current tumor screening strategies. CONCLUSIONS In this article, we provide the first comprehensive recommendations on the complex management of patients with Beckwith-Wiedemann syndrome.

A cephalometric intercenter comparison of patients with unilateral cleft lip and palate: analysis at 5 and 10 years of age and long term.

Objective: To compare the short- and long-term craniofacial growth of patients operated with the Milan protocol to those operated with the Oslo protocol. Design: The Milan sample included 88 patients with unilateral cleft lip and palate (UCLP) at 5 years, 26 at 10 years, and 23 at the end of growth. The Oslo samples included 48 UCLP patients at 5 years, 29 at 10, and 23 at growth completion. Lateral cephalograms were used for comparison. An unpaired t test was run for the 5- and 10-year-old samples. The samples long term were matched for age and sex, and a paired t test was run. Results: There was no significant cephalometric difference in the maxillary prominence at 5 years, a mild but significant difference at 10 years, and again no difference at the end of growth. Nevertheless, at an older age, the need for orthognathic surgery was larger in the Milan sample (26%) than in the Oslo sample (13%). Conclusion: Although no statistically significant differences in the cephalometric measurements were found long term, the need for orthognathic surgery was clinically judged to be larger in the Milan sample. On the other hand, although the Milan protocol seemed to require more final jaw surgery, only the cases that needed an additional orthognathic procedure in the Milan group will undertake a third surgical step, while the Oslo protocol included three surgical steps for all the patients.

Comparison of growth results in patients with unilateral cleft lip and palate after early secondary gingivoalveoloplasty and secondary bone grafting: 20 years follow up

The Milan surgical protocol from 1988 has included repair of lip, nose, and soft palate at 6–9 months of age, and closure of the hard palate and alveolus with an early secondary gingivoalveoloplasty at 18–36 months. The goal of this study was to evaluate the long-term maxillary growth in patients with unilateral cleft lip and palate (UCLP) who had had the early secondary gingivoalveoloplasty, compared with the growth in a sample treated before 1988, by the same surgeon, with a surgical protocol that differed only by the method and the timing of alveolar closure. In the second group they were repaired by secondary bone grafting. The samples consisted of lateral cephalograms of the UCLP early secondary gingivoalveoloplasty sample (15 patients with a mean age of 18 (1.2) years) and of the UCLP bone graft sample (10 patients with a mean age of 19 (1.1) years). The early secondary gingivoalveoloplasty patients showed that maxillary growth was inhibited compared with the secondary bone graft group. Although the early secondary gingivoalveoloplasty allowed for early repair of the alveolus together with palatal repair, eliminating the need for secondary bone grafting, it seemed to have an inhibiting influence on maxillary growth that increased the need for Le Fort I osteotomies. Even with a Le Fort I osteotomy, the early secondary gingivoalveoloplasty allows the total number of operations to be kept down to three, as in most European protocols.

Photometric evaluation of bilateral cleft lip and palate patients after primary columella lengthening.

Objective To evaluate the results in terms of nasal esthetics of children with bilateral cleft lip and palate, operated with the Cutting primary columella lengthening technique, associated with Grayson orthopedic nasoalveolar molding, and to compare them with the nasal aspects of children with bilateral cleft lip and palate operated with a traditional approach and to an age-matched sample of normal Caucasian children. Design Normalized photogrammetry. Setting Regional Center for CLP, Department of Maxillo-Facial Surgery, San Paolo Hospital, Milan. Patients Three groups of patients 5 years of age. Cutting group: 18 patients treated with the Grayson-Cutting technique. Delaire group: 18 patients treated with the traditional Delaire technique. Normal children: 40 normal preschool children. Results With the Cutting-Grayson technique, the columella length, nasal tip angle, and protrusion are greatly improved compared with the previous protocol and are close to normal. On the other hand, the nasolabial angle and interalar distances are still excessively wide in both samples. Conclusions Although this is not a long-term study, at this time none of the patients operated with this technique have needed secondary columella lengthening. On the other hand, although certainly improved, the nasal anatomy obtained is far from normal.

Factors that affect variability in impairment of maxillary growth in patients with cleft lip and palate treated using the same surgical protocol

Abstract No consensus exists about the causes of restriction of maxillary growth in patients with cleft lip and palate (CLP). The aim of this study was to try to identify causes of this impairment other than the influence of surgical technique and skill. We analysed a sample of 129 consecutively treated 5-year-old children with unilateral cleft lip and palate (UCLP), who were operated on by the same surgeon with the same protocol. Multiple cephalometric measurements of the sample showed a wide distribution of values for maxillary growth. We selected SNA as a value describing maxillary position. Variables investigated were: initial cast measurements; timing of lip and of gingivoalveoloplasty (GAP)/palatal surgery; and presence of permanent lateral incisors. The significance of differences was investigated with Pearsons correlation and analysis of variance (ANOVA). The factor most significantly linked with maxillary protrusion was the presence or absence of the permanent lateral incisor, even when peg laterals and supernumerary laterals were considered. Initial width of the palate measured on infant casts correlated with maxillary growth, but the timing of GAP did not. Although surgical skill and technique may be the most important factors responsible for impairment of maxillary growth, inherent tissue hypoplasia, possibly the lack of lateral incisors, seems to be the most important non-iatrogenic factor.

Does functional appliance treatment truly improve stability of mandibular vertical distraction osteogenesis in hemifacial microsomia

AIM After mandibular unilateral distraction osteogenesis (DO) a gradual reappearance of the vertical asymmetry during growth is observed. A pre- and post-surgical functional-orthodontic treatment was added to our distraction protocol in the attempt to increase long-term stability. In order to evaluate the actual efficacy of such a combined treatment, two samples of children affected by hemifacial microsomia were compared long-term. MATERIAL Ten children were treated by a combined orthodontic-distraction treatment, seven by distraction only. METHOD Only the vertical changes in the mandible and maxilla in the panoramic and postero-anterior cephalometric X-rays were measured. RESULTS All of the patients showed a gradual return of the asymmetry with growth. Occlusal plane correction and, to a much lesser extent, mandibular vertical ramus height correction were better maintained over 5 years post-DO in the orthopaedic group. CONCLUSION Although orthopaedic treatment allows for a more stable occlusal plane and for a slower return of the mandibular vertical asymmetry, it has mainly a dento-alveolar effect. Therefore, the decision of applying an orthopaedic treatment associated with distraction, should be taken by surgeon and orthodontist together, considering both the advantages and the disadvantages of this treatment.