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A European guideline on Robin Sequence was developed within the European Reference Network for rare and/ or complex craniofacial anomalies and ear, nose, and throat disorders. The guideline provides an overview of optimal care provisions for patients with Robin Sequence and recommendations for the improvement of care.

The impact of non-invasive ventilation (NIV) on pediatric maxillary growth is a subject of ongoing research considering its increased use in the pediatric population due to technological advancements and broader indications. This review examines the existing literature, encompassing original articles, case reports, and reviews, to evaluate the effects of NIV on maxillary development and explore potential treatment options. Although the majority of studies agree on the adverse effects of prolonged NIV on maxillary development, techniques for its correction remain understudied. Introducing a novel treatment protocol, we addressed the challenge of correcting severe midfacial hypoplasia in a child with congenital central hypoventilation syndrome (CCHS) undergoing NIV therapy, thus sidestepping the necessity for osteotomies. This proposed protocol holds promise in correcting the adverse impact of NIV on maxillary growth, emphasizing the need for further exploration into innovative treatment modalities.

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.

Background: The aim of this study was to achieve a consensus on the minimum set of outcome measures and predictors to be used in the neurosurgical practice and on the timing of outcome assessment. Methods: A consensus building approach was employed. All neurosurgical departments in Lombardy (Italy) were invited to participate by the Carlo Besta Neurologic Institute IRCCS Foundation. Three workshops were organized during which a multidisciplinary group called Neurosurgical Outcome Network (NEON) was created and the methodology to select outcome measures, predictors, and timing of outcome assessment was established. Eight working groups were created for the different neurosurgical diseases (neuro-oncological, skull base, vascular, traumatic, spinal, peripheral nervous system, malformation, functional) and 8 workshops were organized to identify the outcome measures and predictors specific for each of the neurosurgical diseases based on the experts' clinical practice and the existing literature. Results: A total of 20 neurosurgical departments participated in this study. Specific outcome measures, predictors and the timing of outcome assessment were identified for each of the 8 neurosurgical diseases. Moreover, a list of variables common to all pathologies were identified by the NEON group as further data to be collected. Conclusions: A consensus on the minimum set of outcome measures and predictors and the timing of outcome assessments for 8 neurosurgical diseases was achieved by a group of neurosurgeons of the Lombardy region, called NEON. These sets could be used in future studies for a more homogeneous data collection and as a starting point to reach further agreement also at national and international level.

The application of surgical navigation in oral and maxillo-facial surgery has been increasing over time. In fact, computer-assisted surgery provides real-time, precise, and accurate position and guidance during surgery. The purpose of our work is to introduce the evolution of surgical navigation in recent decades, describe some technical aspects of this technology, explore new possibilities of application of surgical navigation in oral surgery, and validate the accuracy of computer-assisted surgery. We included four patients in our sample who underwent virtual planning on the cone beam CT data set and surgical navigation using non-invasive fiducial markers. The first patient presented a dislocated orthodontic arch in the soft tissues of the cheek, while the other patients presented supernumerary and impacted dental elements. Among them, two patients were affected by craniofacial synostosis. We evaluated the accuracy of computer-assisted surgery, calculating the discrepancy between the real and virtual target. In all cases, the target registration error was less than or equal to 1 mm. We can affirm that surgical navigation is a valid tool to enhance oral surgery, guaranteeing an undoubted advantage in terms of the reliability and predictability of the results, especially in complex cases.

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.

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.

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.

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.

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.

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 cm2 and 50 cm2 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.

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 11·57day). 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

Le Craniosinostosi,Craniofaciosinostosi ed Ipertelorismo sono deformità del cranio e del cranio e della faccia che necessitano di valutazione e trattamento multidisciplinare. Vengono illustrati i principali quadri clinici ed enunciati e descritti i principi di trattamento.

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 nonsurgical treatments for the specific case.

Objective: Modern bone surgery aim at a rapid and complete restoration of form and function. The conventional system of internal fixation with miniplates, especially in the unfavourable physiopathologic cases, could have potential complications like plates failure or plates loosening. A disadvantage of conventional bone plates system is that the plate must be perfectly adapted to the underlying bone to prevent alterations in the alignment of the segments. Some Authors maintain that the most important reasons of early mobilization are the compression of the plates on cortical bone and the traction of the screws on plate. The use of 2.0 Lock system plates could avoid these complications. Methods: We treated 34 cases with 2.0 Lock plates (Compact Lock 2.0, Synthes). The cases were selected according to fractures type: site, type of fracture, number of fragments, fracture line with or without dislocation, bone quality, malocclusion, patients characteristic and requirements. Results: All patients have a good fracture healing. In any case we use the inter maxillary block in the postoperative period. In the 10 cases with condylar fractures we performed an early physiotherapy. Conclusions: This system gives optimal results avoiding delayed fracture healing or non-union and allows a complete and early recovery of skeletal function. The 2.0 Lock plates are a valid alternative to the conventional mini plates, but they don’t substitute 2.4 lock plates that takes indication in treatment of comminuted fractures, in atrophic mandible and in oncologic reconstruction

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.

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.

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.