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.

Comparison of the psychosocial impact on patients affected by cranio facial anomalies between traditional orthodontic brackets and aligners.

OBJECTIVES: Malocclusion affects the psychological wellness and social interaction, impacting negatively on adolescents' quality of life and self-esteem. Facial attractiveness is mostly based on the esthetics of the smile and patients seek orthodontic treatment mainly to improve aesthetics. The aim of this prospective clinical study is to compare the psychosocial impact of aligners (AL) and fixed appliances (FA) as orthodontic treatments in patients affected by craniofacial anomalies (CFA). METHODS: 100 syndromic Caucasian patients with CFA followed in two different hospitals were divided in two groups: 50 patients treated with AL and 50 patients treated with FA. The two groups were matched for gender, age and CFA and filled out a modified psychosocial impact of dental aesthetics questionnaire (mPIDAQ). RESULTS: Patients affected by CFA treated with AL reported a better psychosocial impact than patients treated with FA, showing higher esthetic self-perception and self-esteem, lower social interaction impairments, and lower physical/practical disturbances. CONCLUSIONS: The results of this study suggest that AL could be a valid alternative, especially in complex patients with CFA. Since AL application requires more cooperative patients the orthodontist should dedicate more time to motivate the patient to adhere to the treatment schedule.

International Consensus Statement on the diagnosis, multidisciplinary management and lifelong care of individuals with achondroplasia.

Achondroplasia, the most common skeletal dysplasia, is characterized by a variety of medical, functional and psychosocial challenges across the lifespan. The condition is caused by a common, recurring, gain-of-function mutation in FGFR3, the gene that encodes fibroblast growth factor receptor 3. This mutation leads to impaired endochondral ossification of the human skeleton. The clinical and radiographic hallmarks of achondroplasia make accurate diagnosis possible in most patients. However, marked variability exists in the clinical care pathways and protocols practised by clinicians who manage children and adults with this condition. A group of 55 international experts from 16 countries and 5 continents have developed consensus statements and recommendations that aim to capture the key challenges and optimal management of achondroplasia across each major life stage and sub-specialty area, using a modified Delphi process. The primary purpose of this first International Consensus Statement is to facilitate the improvement and standardization of care for children and adults with achondroplasia worldwide in order to optimize their clinical outcomes and quality of life.

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.

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.

Prioritizing Cleft/Craniofacial Surgical Care after the COVID-19 Pandemic.

BACKGROUND: It is anticipated that in due course the burden of emergency care due to COVID-19 infected patients will reduce sufficiently to permit elective surgical procedures to recommence. Prioritizing cleft/craniofacial surgery in the already overloaded medical system will then become an issue. The European Cleft Palate Craniofacial Association, together with the European Cleft and Craniofacial Initiative for Equality in Care, performed a brief survey to capture a current snapshot during a rapidly evolving pandemic. METHODS: A questionnaire was sent to the 2242 participants who attended 1 of 3 recent international cleft/craniofacial meetings. RESULTS: The respondents indicated that children with Robin sequence who were not responding to nonsurgical options should be treated as emergency cases. Over 70% of the respondents indicated that palate repair should be performed before the age of 15 months, an additional 22% stating the same be performed by 18 months. Placement of middle ear tubes, primary cleft lip surgery, alveolar bone grafting, and velopharyngeal insufficiency surgery also need prioritization. Children with craniofacial conditions such as craniosynostosis and increased intracranial pressure need immediate care, whilst children with craniosynostosis and associated obstructive sleep apnea syndrome or proptosis need surgical care within 3 months of the typical timing. Craniosynostosis without signs of increased intracranial pressure needs correction before the age of 18 months. CONCLUSIONS: This survey indicates several areas of cleft and craniofacial conditions that need prioritization, but also certain areas where intervention is less urgent. We acknowledge that there will be differences in the post COVID-19 response according to circumstances and policies in individual countries.

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.

Infant ossified cephalohematoma: a review of the surgical management and technical update.

Cephalohematoma is a subperiosteal collection of blood above the skull. It is associated with operative and prolonged deliveries. In most cases the hematoma spontaneously resolves in a short span of time, usually within one month. In a few cases cephalohematoma can become ossified and require surgical treatment. Traditional belief that ossified cephalohematomas are an exclusively cosmetic issue has been called into question by recent description of EEG anomalies associated to a calcified cephalohematoma. We review relevant surgical literature, and we describe a novel variant of Wong's Radial Cap technique by foregoing healthy bone margin removal, removing the cephalohematoma lamellae in a stepwise fashion and using resorbable plaques for fixation. We furthermore review the rationale, timing and step-by-step execution of the procedure. An exemplary case description is discussed.

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.

The CFI score: Validation of a new comprehensive severity scoring system for facial injuries.

At the time of writing there is no measuring scale for the severity of facial trauma that can effectively summarize its clinical relevance, and can therefore be proposed as a definitive tool in trauma center decision making. This paper introduces a new, simple, comprehensive, and reproducible score for facial trauma, in which its severity is expressed in relation to the surgical duration necessary for definitive treatment. This parameter is identified as the most significant in expressing the commitment of care required. Statistical validation of this comprehensive facial injury (CFI) score involved a sample of 1050 patients, treated by the same team in two highly specialized trauma centers. It demonstrated a linear regression between CFI score and surgical duration, and a high degree of accuracy in forecasting overall surgical time for each type of facial injury. The descriptive capacity of CFI score, and its extremely simple use, make it a perfect tool for widespread application and for facilitating communication inside trauma centers. It also allows the classification of homogeneous groups of patients - a prerequisite for benchmarking and effective analysis of results. The CFI model is definitively proposed for the classification of facial injuries, and therefore for the integration of maxillofacial skills, within the trauma team.

Extensive Dural Ossification After Decompressive Posttraumatic Craniectomy: A Case Report and Review of the Literature.

BACKGROUND: We present a case of almost complete bifrontal dural ossification after decompressive craniectomy for severe traumatic brain injury. CASE DESCRIPTION: A 6-year-old boy was brought to the emergency room after a severe traumatic brain injury (Glasgow Coma Scale/Score 7). Due to rapidly increasing intracranial pressure values (>20 mm Hg) refractory to conservative therapy and in the absence of focal lesions justifying the neurological status, a bicoronal decompressive craniectomy was performed. The patient underwent reconstructive bifrontal cranioplasty with autologous bone 110 days after surgery. Preoperative computed tomography scans showed an abnormal hyperdense layer surrounding both frontal lobes. During the procedure, widespread islets of eggshell-shaped bony tissue were found covering the pericranium and the dura mater. They were laboriously removed from the underlying tissues. Two months later computed tomography scans did not show evidence of calcification at the level of the frontal lobes or signs of bone reabsorption or epidural collections. CONCLUSIONS: We focus on the clinical implications of this phenomenon, in terms of its surgical management and how it may influence the timing of cranioplasty. Furthermore, we discuss the main pathophysiologic models described in the literature.

Homologous Banked Bone Grafts for the Reconstruction of Large Cranial Defects in Pediatric Patients.

PURPOSE: Autogenous bone represents the best material in pediatric reconstructive cranioplasty because of its compatibility with growth; however, its availability is limited. Alternative use of alloplastic materials would have the advantage of unlimited amount, but shows an increased risk of complications and incompatibility with growth. Fresh frozen banked cadaveric bones could be ideal for the reconstruction of large cranial defects in growing patients, because it offers unlimited amount of structural grafts with biomechanics properties quite similar to the autologous bones. RESULTS: The authors report 2 cases of growing patients (13 months female and 9 years old male) undergoing the reconstruction of large (about 70 cm and 50 cm respectively) cranial vault defects, by using structural homologous banked bone grafts.Analytic main reconstruction materials risks/benefits evaluation, literature review of few previously reported pediatric cranioplasty with homologous bone, and both clinical cases satisfactory radiologic long-term results (beyond 4 years of F.U.) are reported. CONCLUSION: Structural homologous banked bone grafts appear as a valid solution for pediatric canioplasty reconstruction of wide defects, entailing the advantages of available without limitations and compatibility with future growth. The risk of transmissible infections seems minimal and is totally counterbalanced by the stability of long-term results.

Negative pressure wound therapy in complex cranio-maxillofacial and cervical wounds.

The care and the management of the healing of difficult wounds at the level of the skull-facial face many problems related to patient compliance and the need to perform multiple dressings, with long periods of healing and, occasionally, a very long hospitalisation period. The introduction and evolution of negative pressure wound therapy (NPWT) in the treatment of difficult wounds has resulted in better healing, with a drastic reduction in terms of time and biological costs to the patient and cost to the health care system. The main aim of this study is to describe and discuss, using out our experience, the usefulness of NPWT in the cranial-facial-cervical region. We studied 16 patients with complex wounds of the cranial-facial-cervical region treated with NPWT. We divided clinical cases in four groups: cervicofacial infectious disease, healing complications in oncological-reconstructive surgery, healing complications of injury with exposure of bone and/or internal fixations and healing complications in traumatic injury with loss of substance. We evaluated complete or incomplete wound healing; application time, related also to hospitalisation time; days of intensive care unit (ICU) stay; management of the upper airways; timing of medication renewal; and patient comfort and compliance (on a scale of 1-5). Depression values were always between -75 and -125 mmHg in a continuous aspiration pattern. For every patient, we used the ActiVAC Therapy Unit, derived from the vacuum-assisted closure system (Kinetic Concepts Inc., San Antonio, TX). Medication renewals were performed every 48-72 hours. The NPWT application time ranged from 4 to 22 days (mean of 11·57 day). Therapy was effective to gain a complete restitutio ad integrum in every patient included in the group of cervicofacial infectious disease. Therapy has, however, been well tolerated in our series; this is probably due to the decreased number of applications, the ease of use and the comfort of the system relative to traditional dressing. Results were satisfactory for most of cases treated; faster and more effective wound healing was achieved. The lower number of NPWT applications, relating to standard dressings, led to an increase in patient comfort and compliance and a decrease in the use of medical, and in some cases economic, resources according to international literature.

An atypical case of craniometaphyseal dysplasia. Case report and surgical treatment.

INTRODUCTION: Craniometaphyseal dysplasia is a rare hereditary bone disease presenting metaphyseal widening of the tubular bones, sclerosis of craniofacial bones and bony overgrowth of the facial and skull bones. Craniometaphyseal dysplasia occurs in an autosomal dominant (AD) and an autosomal recessive (AR) form. CASE REPORT: We present a 32-year-old patient arrived at our unit in May 2009. His main discomfort was a major limitation of the mouth opening, in the context of a craniofacial deformity. Relying on patient's medical history and the performed diagnostic tests, the diagnosis of craniometaphyseal dysplasia was made. CONCLUSION: After careful evaluation of the clinical case, in accordance with the requirements of the patient, we opted for a surgical treatment aimed at correction of functional limitation of temporomandibular joint and aesthetic improvement of the facial bones. The stability of the clinical results led us to suggest and to undertake the surgical path, also due to the lack of safe and consolidated non-surgical treatments for the specific case.

Photometric evaluation of cranial and facial symmetry in hemicoronal single suture synostosis treated with surgical fronto-orbital remodeling.

OBJECTIVE: Evaluation of frontal vault symmetry and progressive facial symmetrization in a cohort of patients with hemicoronal single suture synostosis treated with a standardized cranioplasty and rigid fixation. PATIENTS AND METHODS: Fifty-four patients with hemicoronal synostosis operated between 1999 and 2014 were reviewed retrospectively. Pre, immediately postoperative and yearly photographs from the top of the skull and frontal views of the face were taken with the same head position and projection. A photogrammetric method was applied to quantify the pre and postoperative contour changes. The anterior skull hemispheres were traced, divided into two equal parts and the areas were compared. Angular measurements obtained by the intersection of the interpupillary line and the glabella perpendicular vertical line were calculated. The average photographic follow-up was 6.8 years. Range 1-14 years. RESULTS: The average advancement on the affected side was 18 mm (range: 16-23 mm). The pre-surgical cranial area on the affected side was increased on average 14.6 + 2.4% (range: 10-18%). The angular measurements documented the frontal symmetry obtained and the progressive improvement of facial symmetry. CONCLUSION: Cranioplasty with rigid fixation achieved a stable correction of anterior plagiocephaly leading to subsequent symmetrical facial growth. Photogrammetry allowed fora quantitative long-term validation.

Temporomandibular joint ankylosis as part of the clinical spectrum of Carey-Fineman-Ziter syndrome?

The Carey-Finema-Ziter syndrome (CFZS, MIM 254940) is an apparently autosomal recessively inherited disorder consisting of the combination of non-progressive congenital myopathy with Moebius and Pierre Robin sequence, facial anomalies and growth delay. Mental development has been described as normal or delayed. Temporomandibular joint (TMJ) ankylosis is the immobility of the joint caused by ankylotic fusion of the mandible to the cranial base or zygoma. It is a serious and disabling condition that may cause problems in mastication, digestion, speech, appearance, and oral hygiene. Most often is a true ankylosis of the TMJ but other pathological mechanisms are described (i.e., the fusion of the coronoid process to temporal bone or with the zygoma, or a variety of soft tissues disorders like Fibrodysplasia Ossificans Progressiva). Here we report a 2-year-old girl fitting with a clinical diagnosis of CFZS associated with a limited mouth opening in which temporomandibular joint ankylosis was suspected. Because it has been postulated that many clinical features in CFZS may only be secondary effects of brainstem anomalies and muscle weakness during development, the limited opening of the mouth observed in our patient could represent a rare clinical feature of CFZS itself. © 2016 Wiley Periodicals, Inc.

Long-term follow-up of large maxillary advancements with distraction osteogenesis in growing and non-growing cleft lip and palate patients.

BACKGROUND: Maxillary distraction osteogenesis (DO) in cleft lip and palate patients has been described by several authors, but most studies have a relatively short follow-up and do not clearly separate growing patients from non-growing patients. METHOD: The records of 22 consecutive patients affected by cleft lip and palate, who underwent Le Fort I osteotomy and maxillary distraction with a rigid external distractor (RED), were reviewed. The sample was subdivided into a growing and a non-growing group. All patients had pre-DO cephalometric records, immediately post DO, 12 months post DO and long-term records with a long-term follow-up of >5 years (range 5-13 years). As a control sample for the growing group, cleft children with a negative overjet not subjected to distraction or any protraction treatment during growth were followed up until the completion of growth. RESULTS: The average maxillary advancement in the growing group was 22.2 ± 5.5 mm (range: 15-32 mm); in the non-growing group, it was 17.7 ± 6.6 mm (range: 6-25 mm). Excellent post-surgical stability was recorded in the adult sample. On the other hand, growing children had an average 16% relapse in the first year post DO and an additional 26% relapse in the long-term follow-up. CONCLUSIONS: This study seems to point out that early Le Fort I DO allows for the correction of very severe deformities. It is followed by a relatively high amount of true skeletal relapse in children with cleft lip and palate. Prognosis should be discussed in depth with the family and true aesthetic and psychological needs assessed.

Virtual surgery simulation in orbital wall reconstruction: integration of surgical navigation and stereolithographic models.

PURPOSE: Correction of post traumatic orbital and zygomatic deformity is a challenge for maxillofacial surgeons. Integration of different technologies, such as software planning, surgical navigation and stereolithographic models, opens new horizons in terms of the surgeons' ability to tailor reconstruction to individual patients. The purpose of this study was to analyze surgical results, in order to verify the suitability, effectiveness and reproducibility of this new protocol. METHODS: Eleven patients were included in the study. Inclusion criteria were: unilateral orbital pathology; associated diplopia and enophthalmos or exophthalmos, and zygomatic deformities. Syndromic patients were excluded. Pre-surgical planning was performed with iPlan 3.0 CMF software and we used Vector Vision II (BrainLab, Feldkirchen, Germany) for surgical navigation. We used 1:1 skull stereolithographic models for all the patients. Orbital reconstructions were performed with a titanium orbital mesh. The results refer to: correction of the deformities, exophthalmos, enophthalmos and diplopia; correspondence between reconstruction mesh positioning and preoperative planning mirroring; and the difference between the reconstructed orbital volume and the healthy orbital volume. RESULTS: Correspondence between the post-operative reconstruction mesh position and the presurgical virtual planning has an average margin of error of less than 1.3 mm. In terms of en- and exophthalmos corrections, we have always had an adequate clinical outcome with a significant change in the projection of the eyeball. In all cases treated, there was a complete resolution of diplopia. The calculation of orbital volume highlighted that the volume of the reconstructed orbit, in most cases, was equal to the healthy orbital volume, with a positive or negative variation of less than 1 cm(3). CONCLUSION: The proposed protocol incorporates all the latest technologies to plan the virtual reconstruction surgery in detail. The results obtained from our experience are very encouraging and lead us to pursue this path.