Prospective study of nasal growth in bilateral cleft lip and palate patients after naso-alveolar moulding and primary columella lengthening compared to controls at 5, 10 and 20 years.

INTRODUCTION: A short columella, wide nostrils and a flat nasal tip are common features in patients with bilateral complete cleft lip and palate (BCLP). The objective of this study was to evaluate nasal morphology during growth in patients treated with naso-alveolar moulding (NAM) and primary surgical columella lengthening (PCL) compared with matched non-cleft individuals. STUDY DESIGN: Prospective longitudinal case-control study. PARTICIPANTS AND METHODS: Thirty-four consecutively treated BCLP patients at 5 and 10 years and at the end of growth (19.7 ± 2.0 years) were compared through normalized photogrammetry to a control of 34 age and sex-matched non-cleft individuals. Regression Models for Panel Data assessed how nasal measurements were influenced by surgery, age and gender. RESULTS: Nasal protrusion was equal to non-cleft controls at all ages. Length of the columella was also comparable to controls at 5 and 10 years, but significantly shorter at the end of growth. Inter-alar and nasal tip width and nasolabial angle were significantly wider than controls at all ages: More than 60% of the patients have asked for correction of the nasal width, but no early surgery for columella lengthening was needed. CONCLUSIONS: NAM and PCL have provided a nasal projection close to that of non-cleft individuals until adulthood, while length of the columella was physiological at 5 and 10, but shorter than controls at age 20. Width of the nasal tip and width of the alar bases were significantly wider than the controls and eventually required secondary nasal width correction in over two thirds of the sample.

The First Hybrid International Educational Comprehensive Cleft Care Workshop.

OBJECTIVE: Describe the first hybrid global simulation-based comprehensive cleft care workshop, evaluate impact on participants, and compare experiences based on in-person versus virtual attendance. DESIGN: Cross-sectional survey-based evaluation. SETTING: International comprehensive cleft care workshop. PARTICIPANTS: Total of 489 participants. INTERVENTIONS: Three-day simulation-based hybrid comprehensive cleft care workshop. MAIN OUTCOME MEASURES: Participant demographic data, perceived barriers and interventions needed for global comprehensive cleft care delivery, participant workshop satisfaction, and perceived short-term impact on practice stratified by in-person versus virtual attendance. RESULTS: The workshop included 489 participants from 5 continents. The response rate was 39.9%. Participants perceived financial factors (30.3%) the most significant barrier and improvement in training (39.8%) as the most important intervention to overcome barriers facing cleft care delivery in low to middle-income countries. All participants reported a high level of satisfaction with the workshop and a strong positive perceived short-term impact on their practice. Importantly, while this was true for both in-person and virtual attendees, in-person attendees reported a significantly higher satisfaction with the workshop (28.63 ± 3.08 vs 27.63 ± 3.93; P = .04) and perceived impact on their clinical practice (22.37 ± 3.42 vs 21.02 ± 3.45 P = .01). CONCLUSION: Hybrid simulation-based educational comprehensive cleft care workshops are overall well received by participants and have a positive perceived impact on their clinical practices. In-person attendance is associated with significantly higher satisfaction and perceived impact on practice. Considering that financial and health constraints may limit live meeting attendance, future efforts will focus on making in-person and virtual attendance more comparable.

Core outcomes for orofacial clefts: reconciling traditional and ICHOM minimum datasets.

OBJECTIVE/DESIGN/SETTING: This retrospective study sought voluntary participation from leading cleft centres from Europe and Brazil regarding core outcome measures. The results of this study would inform the debate on core outcome consensus pertaining to the European Reference Network for rare diseases (ERN CRANIO) and achieve a core outcome set for cleft care providers worldwide. INTERVENTION/METHOD: Five orofacial cleft (OFC) disciplines were identified, within which all of the International Consortium of Health Outcomes Measurement (ICHOM) outcomes fall. One questionnaire was designed for each discipline and comprised 1. the relevant ICHOM's outcomes within that discipline, and 2. a series of questions targeted to clinicians. What core outcomes are currently measured and when, did these align with the ICHOM minimum, if not how did they differ, and would they recommend modified or additional outcomes?. RESULTS: For some disciplines participants agreed with the ICHOM minimums but urged for earlier and more frequent intervention. Some clinicians felt that some of the ICHOM standards were compatible but that different ages were preferred and for others the ICHOM standards were acceptable but developmental stages should be preferred to absolute time points. CONCLUSION/IMPLICATIONS: Core outcomes for OFC were supported in principle but there are differences between the ICHOM recommendations and the 2002 WHO global consensus. The latter are established in many centres with historical archives of OFC outcome data, and it was concluded that with some modifications ICHOM could be moulded into useful core outcomes data for inter-centre comparisons worldwide.

Photometric Evaluation of Adult Patients With Bilateral Cleft Lip and Palate Treated With Nasoalveolar Molding and Primary Columella Lengthening.

OBJECTIVE: The aim of this study was to assess the nasal shape of young adults with BCLP treated with primary surgical columella lengthening and nasoalveolar molding (NAM). SETTING AND PATIENTS: A group of 28 young adult patients with BCLP (mean age: 19.1±1.4 years) was compared through normalized photogrammetry to a control of 28 age- and sex-matched noncleft young adults. RESULTS: Nasal protrusion and length of the columella were not different from noncleft young adults. On the other hand, nasolabial angle, columellar width, interalar, and nasal tip width were significantly wider than the noncleft controls. Thus, 27% of the patients have requested at this time secondary correction of the excessive nasal width. CONCLUSIONS: Both NAM and primary rhinoplasty in patients with BCLP resulted in a near normal length of the columella and nasal projection until young adulthood. Nevertheless, width of all nasal features was significantly wider than the noncleft population and required secondary nasal correction in one-third of the sample.

Orthodontic Pre Grafting Closure of Large Alveolar Bony and Soft Tissue Gaps: A Novel Nonsurgical Protraction of the Lesser Segments in Growing Patients With Cleft Lip and Palate.

BACKGROUND: Closure of wide alveolar clefts with large soft tissue gaps and reconstruction of the dentoalveolar defect are challenging for the surgeon. Some authors successfully used interdental segmental distraction, which requires an additional surgical procedure. OBJECTIVE: This study evaluates the effectiveness of tooth borne devices utilized to orthopedically advance the lesser segments, with a complete approximation of the soft tissue of the alveolar stumps, allowing traditional simultaneous soft tissue closure and bone grafting, and avoiding the need for supplementary surgery. METHODS: Eight growing patients, 2 with unilateral complete cleft lip and palate (UCLP) and 6 with bilateral complete cleft lip and palate (BCLP), with large soft tissue and bony alveolar defects prior to bone grafting were prospectively selected. A banded rapid palatal expander (RPE) in BCLP and a modified RPE in UCLP combined with protraction face mask in younger patients or a modified Alt-Ramec in patients older than 12 years were applied. Radiographic and photographic records were available at T0, at the end of protraction (T1) and at least 1 year after bone grafting (T2). RESULTS: Patients with large gaps showed a significant reduction in the bony cleft area and approximation of the soft tissues at T1. All patients received bone grafting with good healing and ossification at T2. CONCLUSION: In growing patients with UCLP and BCLP with large gaps, presurgical orthodontic protraction seems to be an efficient method to reduce the cleft defect, minimizing the risk of post grafting fistulas, reducing the need for supplementary surgical procedures.

Circummaxillary Sutures in Patients With Apert, Crouzon, and Pfeiffer Syndromes Compared to Nonsyndromic Children: Growth, Orthodontic, and Surgical Implications.

OBJECTIVE: To evaluate patency of circummaxillary sutures in children with Apert, Crouzon, and Pfeiffer Syndromes and to compare it to a nonsyndromic matched control group. DESIGN: Case-control study. SETTING: Tertiary care public hospital. MATERIALS AND METHODS: Thirty-eight computed tomography (CT) scans of patients affected by syndromic craniofacial synostosis (13 patients with Apert syndrome, 20 patients with Crouzon syndrome, and 5 patients with Pfeiffer syndrome), average age 5 ± 2.8 years, range 1.9 to 12 years, were compared to age- and sex-matched control CTs of 38 nonsyndromic children. Computed tomography scans of the study group had to be performed prior to any midfacial surgery. MAIN OUTCOME MEASURES: Midpalatal suture, zygomaticomaxillary sutures, and pterigomaxillary sutures were evaluated and scored. RESULTS: The syndromic group showed a significant earlier ossification of all sutures compared to the nonsyndromic group. Significant differences were already present in early childhood and continued through adolescence. CONCLUSIONS: Based on the differences in terms of maxillary sutural ossification identified, midfacial hypoplasia does not seem to be only secondary to premature cranial base ossification, but also to primary synostosis of facial sutures, thus providing new insights into the pathogenesis of midface deficiency in children with craniofacial-synostosis. Care should be taken when planning any maxillary orthopedics, such as expansion or maxillary protraction, given the high frequency of early fusion of circummaxillary sutures.

Long-term follow-up of late maxillary orthopedic advancement with the Liou-Alternate rapid maxillary expansion-constriction technique in patients with skeletal Class III malocclusion.

INTRODUCTION: The objective of this study was to evaluate short and long-term results of the application of the alternate rapid maxillary expansion/constriction (Alt-RAMEC) technique in patients with skeletal Class III malocclusion. METHODS: Forty-two white patients were consecutively treated with the Alt-RAMEC technique. The average age of the patients was 12.7 ± 1.6 years (range, 9.4-15.9 years) before protraction. The average age at long-term follow-up was 19.4 ± 2.8 years (range, 17.2-26.9 years). A sample of nontreated patients with Class III malocclusion from the archives of orthognatic surgery in our hospital was used as a control group. The initial records were matched for sex, the severity of Class III malocclusion, and age (mean, 12.1 ± 1.4 years; range, 9.7-14.1 years) with the old records available in the archive. The control sample had records presurgery (mean, 19.8 ± 2.2 years; range, 16.6-21.6 years). RESULTS: The sagittal advancement of A-point, after the application of the technique, was 5.43 ± 2.71 mm. Some mandibular dentoalveolar adaptation was noted. The position of the maxilla was stable in the long term. In contrast, the control group showed limited growth at the maxillary level during the long-term follow-up period. CONCLUSIONS: Our results showed that the Alt-RAMEC technique, performed at the correct time, with a double-hinged expander, followed by Class III spring or elastic traction, 24 h/d, allows for satisfactory maxillary protraction, with stable long-term results. The comparison with a sample of matched nontreated patients with Class III malocclusion allowed to suggest the positive effect of the treatment on the maxillary position vs the natural evolution of the Class III skeletal discrepancy.

Costochondral graft in growing patients with hemifacial microsomia case series: Long-term results compared with non-treated patients.

OBJECTIVE(S): The aim of this study was to evaluate the long-term effectiveness of costochondral graft in hemifacial microsomia (HFM) type III patients. SETTINGS AND SAMPLE POPULATION: A sample of 10 patients affected by HFM type III treated during growth in the same Centre with costochondral graft (CCG patients group) is compared with a control group (CG) sample of 10 non-treated patients affected by the same malformation in order to understand whether surgery during growth provides advantages in terms of bony and facial symmetry after an 8-year follow-up. MATERIALS AND METHODS: The growth of the CCG was assessed on panoramic X-rays. To assess facial symmetry, a photometric evaluation on the frontal view was carried out. RESULTS: In CCG patients group the graft grown in mean less than the healthy ramus, a good facial symmetry was achieved after surgery, but was lost in the majority of the cases at the most recent control. In CG, occlusal canting slightly increased and facial asymmetry was relatively stable during the years. CONCLUSION: In patients with a congenital deformity, restoring the height of the ramus leads to an immediate restitution of facial symmetry, but in the long term, there is a return to the asymmetrical pattern. In CG, the asymmetry is stable during years with no increase of the facial deformity.

Comparison of Pain Perception in Patients Affected by Cleft and Cranio Facial Anomalies Treated With Traditional Fixed Appliances or Invisalign.

INTRODUCTION: The objective of this prospective study was to compare the difference in pain perception between treatment with aligners (AL) and fixed appliances (FA) in patients affected by cleft and craniofacial anomalies (CFA). METHODS: The sample consisted of 100 syndromic caucasian patients affected by various CFA from 2 different hospitals. Fifty patients treated with AL were matched for sex, age, and CFA with a control sample of 50 patients treated with FA. A modification of the Mc Gill Pain Questionnaire was adapted to our needs. RESULTS: Statistical differences were found. Aligners induced more tightness and tension than FA, while FA induced more pain descriptors and patients reported a higher intake of painkillers. CONCLUSIONS: The results of this study documented a higher pain perception with FA than with AL in patients affected by CFA. The higher sensitivity to pain in cleft and craniofacial patients with fixed treatment could be related to their higher prior sensitization, given the past surgeries and orthodontic treatments. Thus, this study might suggest that Invisalign treatment might be a further interesting treatment option for patients with cleft in order to reduce their burden of orthodontic pain.

Ultrastructural analysis of collagen fibril diameter distribution in cleft lip.

OBJECTIVE: A preliminary study to determine collagen fibril diameter (CF-ED) distribution on medial and lateral sides of cleft lip (CL). MATERIAL AND METHODS: Tissue samples from medial and lateral sides of CL were fixed in 2.5% glutaraldehyde and 1% osmium tetroxide and embedded in Araldite CY212 resin for transmission electron microscopy. The analysis of CF-ED was performed using the ImageJ program. To characterize the packaging of collagen fibrils (CFs) in the two tissues, we estimated the collagen number density (CF-ND) and fibril-area-fraction (FAF). Differences in measurements across the two sides were calculated using Wilcoxon signed-rank test. RESULTS: The CF-ED was statistically significantly (p < 0.001) smaller on the medial side (45.69 ± 7.89 nm) than on the lateral side (54.18 ± 7.62 nm). The medial side had a higher CF-ND and a higher percentage of FAF than the lateral side. CONCLUSION: Our finding of a smaller CF-ED and higher CF-ND and FAF for the medial side suggests possible differences in size and distribution of CFs between medial and lateral sides of CL. This finding provides knowledge toward underlying tissue biomechanics that may help reconstruction of perioral tissue scaffolds, ultimately resulting in better treatment of patients with oral clefts.

Short- and Long-Term Effects of Late Maxillary Advancement With the Liou-Alt-RAMEC Protocol in Unilateral Cleft Lip and Palate.

OBJECTIVE: The objective of this retrospective longitudinal study was to evaluate short- and long-term results of the application of the Liou Alt-RAMEC (alternate rapid maxillary expansion and constriction) technique, a late orthopedic maxillary protraction technique, with intraoral anchorage, in patients with cleft. MATERIALS AND METHODS: Twenty-six patients with unilateral cleft lip and palate (UCLP) were consecutively treated with the Alt-RAMEC technique. The average age of the patients was 11.7 years (10.3-13.2 years) before protraction and 18.3 years (17.4-21.1 years) at long-term follow-up. A sample of nontreated patients with UCLP was used as a control group. It was matched for sex, skeletal class III, and age (11.3 years). The control sample had records at the end of growth (18.7 years). RESULTS: The sagittal advancement of A-point, after the application of the technique, was 5.7 (2.17) mm. Some mandibular dentoalveolar and positional adaptation was noted. The position of the maxilla was stable in the long term. On the other hand, the UCLP control group showed hardly any growth at the maxillary level during the long-term follow-up period. CONCLUSION: Our results showed that the Alt-RAMEC technique, performed at the correct time, with a double-hinged expander, followed by class III spring or elastic traction, 24 hours per day, allows for satisfactory maxillary protraction, with, at this stage, apparently stable long-term results. Nevertheless, as only 50% of the patients had long-term follow-up data, we are still unable to predict the percentage of patients which will not eventually need orthognathic surgery.

Evaluation of a Sample of Patients With Unilateral Cleft Lip and Palate Treated With a Two-Stage Protocol.

The aim of this paper was to assess growth, speech, and aesthetic results at the completion of growth in patients with unilateral cleft lip and palate treated with the 2 stages Milan surgical protocol.Craniofacial growth was evaluated with cephalometric analysis and a theoretical need for orthognathic surgery.Nasolabial appearance was qualitatively assessed using the Asher McDade Aesthetic Index.Speech was assessed using the Gos.Sp.Ass '98 modified for Italian language scoring system.Burden of care was recorded in terms of number of secondary surgical procedures. All of the patients were treated and evaluated at San Paolo Hospital, Smile House, Milan.Fifty-two consecutive patients treated by the same surgeon were recalled, 12 patients did not come for assessment.The first surgical step (average age of 6 months) was cheilorhinoplasty (Millard modified Delaire technique) and soft palate rapair (Pigott). The second step (average age of 35 months) was hard palate and alveolar repair performed simultaneously with an early secondary gengivo alveolo plasty. Fifty-six percent of the patients did not need further surgery after the 2-stage surgery protocol.The 2-stage surgical protocol of Milano, Smile House, seems to be effective for treatment of unilateral cleft lip and palate, with good results in terms of speech, labial appearance, and alveolar cleft management. Nevertheless, maxillary growth was moderately impaired by the protocol.

Photometric Evaluation in Adolescence of Patients With Bilateral Cleft Lip and Palate Treated With Nasoalveolar Molding and Primary Columella Lengthening.

OBJECTIVE: Nasal stigma in patients with bilateral cleft lip and palate (BCLP) are a short columella and a flattened nasal tip. DESIGN: The aim of this study was to evaluate the aesthetics of adolescents with BCLP, operated with a modified Cutting primary columella lengthening technique, associated to a modified Grayson orthopedic nasoalveolar molding (NAM). SETTING AND PATIENTS: 72 BCLP patients were operated with this approach. Standardized photographic records were taken every 2 years. A group of 23 patients between 12 and 13 years of age was compared through normalized photogrammetry to a matched control of 23 noncleft adolescents. RESULTS: Nasal protrusion and length of the columella were very close to normal. On the other hand, nasolabial angle and interalar width were still excessively wide compared to the noncleft sample. CONCLUSIONS: NAM and primary columella lengthening in BCLP has allowed to avoid traditional secondary columella lengthening at 5 to 6 years of age and given the patients a more pleasing, near-normal nasolabial appearance until adolescence. Some of the patients will require correction of the nasal width at a later stage.

Measurements of orbital protrusion from childhood to young adulthood.

BACKGROUND: Ocular protrusion is usually measured as the deepest part of the lateral orbital rim to the apex of the cornea. Several studies have been carried out to propose normative data for ocular protrusion according to different age ranges. The protrusion of the eyeball seemed to increase during childhood and adolescence and become stable after the late teens during adulthood. Relationships of ocular protrusion with age are still not completely clarified. The purpose of the current retrospective study was to investigate ocular protrusion on lateral x-rays at different growth stages. METHODS: Ninety-five patients were retrospectively selected and divided into 5 different groups according to ages. Subjects were of both sexes with an age range from 5 to 20 years (mean 12.3 years). The cephalometric analysis proposed in the current study allowed to successfully evaluate sagittal relationships of the orbital-globe and the orbital-cavity in growing patients. All other sagittal and vertical measurements were successfully computed by one trained operator. RESULTS: Analysis of variance results with post hoc corrections showed for the orbital measurements indicating a significant increase of the orbital protrusion from childhood to pubertal stage. CONCLUSIONS: Ocular protrusion measurements showed significant increase from childhood (5-8 years) to teen age (13-15 years) and remained stable after the pubertal growth spurt (17-20 years) in the examined sample of patients.

Long-Term Follow-Up of UCLP Patients: Surgical and Orthodontic Burden of Care During Growth and Final Orthognathic Surgery Need.

OBJECTIVE: The goal of this study was to evaluate the craniofacial morphology at 5 and 10 years of age and at the completion of growth, the need for final orthognathic surgery, and the orthodontic burden in a sample of patients with unilateral cleft lip and palate consecutively treated by the same surgeon with the same two-step protocol. DESIGN: A sample of 62 adult patients with unilateral cleft lip and palate was retrospectively collected (mean age, 17.5 years). Lateral cephalograms at three time points were traced. The need for orthognathic surgery was assessed, subdividing the sample into an orthognathic surgery group and nonorthognathic surgery group. Time and modality of orthodontic treatment were recorded. RESULTS: Cephalometric values related to maxillary growth (SNA, SNAns) and maxillomandibular relation (ANB, NAPg) were significantly different between the two groups already at 5 and 10 years of age. All patients presenting an ANB smaller than 2° at 5 years needed a Le Fort I osteotomy. Mandibular protrusion (SNB, SNPg) was not different at 5 and 10 years, but was different at the completion of growth. Patients with the same initial maxillomandibular relation did not show better growth when subjected to earlier or longer orthodontic treatment. CONCLUSION: Patients needing final jaw surgery had a more severe skeletal discrepancy during early childhood. The ANB angle at 5 years allowed doctors to identify 45% of the need for orthognathic surgery. The final craniofacial pattern does not seem to change significantly with early or prolonged orthodontic treatment.

Cephalometric evaluation of ocular protrusion at stages of growth.

The sagittal relationships between the orbital globe and the ocular orbit have great importance in aesthetic and functional evaluations. These relationships are useful in diagnosis and treatment of some malformations in which the orbital volume is reduced such as craniostenosis. The aim of the present study was the cephalometric evaluation of the ocular protrusion at different growth stages. Ninety-two patients with negative history of craniosynostosis, severe myopia, and skeletal class I were selected for the present study. Lateral teleradiography x-ray was performed for all the patients, and 5 groups were carried out based on the dentition phase. Cephalometric analyses were performed on the lateral teleradiography x-ray. Mean values of ocular protrusion (B-Or post) for females and males were found and digitized for the 5 groups tested. All other sagittal and vertical measurements were successfully computed by the trained operator. B-Or post showed values between -14.93 mm and -16.90 mm among the groups. The cephalometric analysis proposed in the present study allowed to successfully evaluate sagittal and vertical relationship of the orbital globe and the orbital cavity in growing patients. Moreover, the B-Or post maintained its value during growth in the present study.

The Use of Clear Aligners in Multi-Segmental Maxillary Surgery: A Case-Control Study in Cleft Lip and Palate and Skeletal Class III Patients.

Background: Maxillary hypoplasia and mandibular asymmetry may be corrected with orthognathic surgery after growth completion. For most stable results, some cases may require segmental Le Fort I osteotomies. Unfortunately, Invisalign's software (6.0 version) still has some inherent limitations in predicting outcomes for complex surgeries. This study explores the potential of aligners, particularly in multiple-piece maxillary osteotomies in both cleft and non-cleft patients. Method: Thirteen patients who underwent pre-surgical treatment with Invisalign were retrospectively matched in terms of diagnosis, surgical procedure, and orthodontic complexity with thirteen patients treated using fixed appliances. Virtual curves following the lower arch were employed to guide the correct pre-surgical positions of the upper teeth with a simple superimposition technique. The amount of impressions required in both groups to achieve satisfactory pre-surgical alignment of the segmented arches was compared. Results: one or no refinement phases were needed in the Invisalign group to reach an acceptable pre-surgical occlusion, while the amount of pre-surgical impressions needed to reach adequate coordination with fixed appliance treatment was slightly higher (p > 0.05). Conclusions: it appears that clear aligner could serve as an effective treatment for individuals necessitating segmental Le Fort I osteotomies when aided by the suggested simple superimposition approach.

Long-term mandibular growth in patients with airway obstruction treated with mandibular distraction.

Congenital craniofacial malformations play an important role in upper airway obstruction. One of the main causes is mandibular hypoplasia which is present in the pierre robin sequence. Mandibular distraction osteogenesis (MDO) is one of the most commonly used treatments for the resolution of upper airway obstruction in patients that do not respond to a conservative treatment. We performed a long term follow up of syndromic and non-syndromic patients with pierre robin sequence where the lateral xrays were studied before surgery (T1), after MDO (T2) and long-term follow-up (T3, at least 5 years). Possible complications of the surgery were also studied through panoramic x-rays and clinical controls. The results evidenced an increase of mandibular length comparing T1-T2 and a good stability during the long-term follow-up. The Sd patients presented smaller mandible dimensions. We had not found any complications during the follow-up. The scars outcomes are modest, but none of the patients requested a surgical correction. Mandibular distraction osteogenesis is a treatment to be considered in patients with upper airway obstruction that do not respond to conservative treatments. The results of the surgery are stable and the complications in experienced hands are low.