Frontofacial Monobloc Advancement Research Articles

SYM9.9B Quantitative facial and orbital assessments of fronto-facial monobloc advancement with internal distraction in faciocraniosynostoses

SYM9.9B Quantitative facial and orbital assessments of fronto-facial monobloc advancement with internal distraction in faciocraniosynostoses

Correcting Exorbitism by Monobloc Frontofacial Advancement in Crouzon-Pfeiffer Syndrome: An Age-Specific, Time-Related, Controlled Study.

In FGFR2 craniosynostosis, midfacial hypoplasia features oculo-orbital disproportion and symptomatic exorbitism. Clinical consequences may mandate surgery at a young age to prevent globe subluxation, corneal ulceration, and potential loss of vision. Monobloc osteotomy and distraction osteogenesis (monobloc distraction) seek to correct exorbitism. A report of the age-related impact of monobloc osteotomy and distraction osteogenesis on orbital volume, globe volume, and globe protrusion is presented. Computed tomographic scan data from 28 Crouzon-Pfeiffer patients were assessed at preoperative, early postoperative, and 1-year follow-up time points. Orbital volumes, globe volumes, and globe protrusions were measured by manual and semiautomatic segmentation techniques, and these were compared to 40 age-matched controls. Crouzon-Pfeiffer syndrome orbital volumes are significantly small, and are significantly overexpanded by distraction to endpoints correcting symptomatic exorbitism. Globe volumes are significantly larger than controls under 5 years, do not independently correlate with globe protrusion, and are unaffected by surgery. Correlation between orbital volume expansion and reduction of globe protrusion is not significant. Age-related variations of postoperative growth potential occur to 1 year postoperatively. The Crouzon-Pfeiffer syndrome FGFR2 orbit exhibits early growth acceleration followed by premature growth arrest at 10 to 14 years. Orbital volume expansion by monobloc osteotomy and distraction osteogenesis is not the sole determinant of reduced globe protrusion. Mean volume relapse of the orbit at 1 year is insignificant across the series. Derived Crouzon-Pfeiffer growth curves suggest that "early functional monobloc" in infants occurs on a background of dynamic orbital growth, which remains programmed to a Crouzon-Pfeiffer FGFR2 phenotype and aligns with the incidence of delayed clinical regression and later secondary surgery. Therapeutic, IV.

Dental consequences of pterygomaxillary dysjunction during fronto-facial monobloc advancement with internal distraction for Crouzon syndrome

Crouzon syndrome is a syndromic faciocraniosynostosis that can be associated with severe fronto-facial retrusion leading to major functional impairments: extreme exorbitism may be vision-threatening and severe respiration impairment can be life-threatening. The procedure of choice for the primary correction of this retrusion is fronto-facial monobloc advancement (FFMBA) with internal or external distraction. FFMBA involves pterygomaxillary dysjunction (PMD), using either a superior or an intra-oral approach. This step is at risk of damaging the germs of the decidual and permanent molars. Here we considered a series of 15 patients with Crouzon syndrome who benefited from FFMBA performed by the same surgeon, using a superior approach through the infra-temporal fossa for PMD. Based on pre-operative, early post-operative and late post-operative CT-scans, we recorded missing teeth, morphological dental anomalies and the Nolla stage for the first and second permanent maxillary molars. We showed that early FFMBA has significant dental consequences, and that these dental effects are correlated with an early age at surgery. Although the indications of early FFMBA in Crouzon syndrome with severe functional repercussions are not questionable, our results should be compared to dental outcomes of FFMBA performed with an intra-oral PMD.

Anterior Skull Base and Pericranial Flap Ossification after Frontofacial Monobloc Advancement.

Frontofacial monobloc advancement creates a communication between the anterior cranial fossa and nasal cavities. To tackle this issue, transorbital pericranial pedicled flaps are routinely performed in the authors' center. This study aimed to assess the postoperative ossification of the anterior skull base and pedicled flaps following frontofacial monobloc advancement, and to identify factors influencing this ossification. Measurements of the skull base only and of the ossified pedicled flaps together with the skull base were performed on computed tomographic scans at the nasofrontal and the nasoethmoid frontal junctions. The total thickness of the skull vault was measured and a qualitative defect score for the anterior skull base was computed. Twenty-two patients who underwent frontofacial monobloc advancement at a median age of 3.1 years (range, 1.9 to 3.6 years) were included: 14 with Crouzon, five with Pfeiffer, and three with Apert syndrome. One year and 5 years after surgery, the distraction gap was completely ossified in the anterior skull base midline in all patients. Ossified pedicled flaps together with the skull base were thicker in patients than in controls at these two time points (p < 0.005 and p < 0.02). Patients with Pfeiffer syndrome had a significantly thicker skull base only and ossified pedicled flaps together with the skull base thicknesses (p = 0.01 and p = 0.03) and lower defect scores than patients with Crouzon or Apert syndrome (p = 0.03) 1 year postoperatively. As ossification of the pedicled flaps and total reossification of the anterior skull base midline were observed in all patients, the authors indicate that performing pedicled flaps in frontofacial monobloc advancement surgery could promote the reossification of the anterior skull base. Therapeutic, IV.

Stratégie craniofaciale pour les faciocraniosténoses

The complexity of treatment of faciocraniosynostosis justifies the treatment in a reference center for rare diseases. The growth disturbances in the skull and face being variable according to the type of mutation in the FGFr (Crouzon, Pfeiffer, Apert), the strategy is adapted to the phenotype according to the following principles: posterior expansion with or without distraction around 6months to limit the descent of the cerebellum tonsils and to prevent the turricephalic development; fronto-facial monobloc advancement with internal distraction around the age of 18months in case of severe exorbitism or breathing impairment. The dissociated strategy (fronto-orbital advancement first, followed by facial osteotomy of Le Fort 3 type). The growing evolution dictates the sequence of subsequent surgeries according to the monitoring of intracranial pressure by fundus examination and of the respiration by polysomnography. Le Fort 3 and transversal maxillary distraction may be repeated if necessary. Orthognathic surgery is almost always compulsory after the age of 14, before the aesthetic refinements which can be undertaken ultimately (rhinoplasty, genioplasty, canthopexies, fat grafting…).

Apert syndrome: Surgical outcomes and perspectives.

Apert syndrome is a rare congenital malformation with severe craniofacial anomalies. The aim of this study was to review the outcomes of craniofacial and neurosurgical interventions in Apert syndrome patients treated at a single institution. A retrospective review of all patient records with a diagnosis of Apert syndrome assessed and managed in the Australian Craniofacial Unit (ACFU) from 1985 to 2013 was conducted. A total of 94 patients were identified, and 130 transcranial procedures were performed. Of the patients, 83 underwent a fronto-orbital advancement (FOA) as their primary procedure, and 18 patients also underwent a posterior vault procedure. Twenty patients underwent a fronto-facial monobloc advancement. Overall, 70% of patients underwent at least 2 transcranial procedures. Shunts were inserted in 2 patients preoperatively and in 5 patients postoperatively for cerebrospinal fluid (CSF) leaks or acute hydrocephalus. Re-do FOAs were performed in 8 patients. Patients who underwent an FOA at the age of more than 18 months had no recurrence of raised intracranial pressure (ICP). Of 18 patients who also underwent a posterior vault procedure, 1 patient had recurrence of raised ICP. Midfacial surgery was performed early if there was evidence of obstructive sleep apnoea (OSA), but delayed midfacial surgery was preferred. Complications were reported in 18% of procedures. The most common complications were CSF leaks and acute hydrocephalus. Shunting is rarely required in Apert syndrome patients, confirming a predominantly nonprogressive ventriculomegaly. FOA appears to be a more stable procedure when performed at an age of more than 18 months. Undergoing a posterior vault procedure may reduce the risk of recurrent raised ICP and lead to fewer transcranial procedures needed in childhood. Midfacial surgery should be delayed until adolescence where there is no evidence of OSA, psychological disturbance, or complications of exorbitism. Complications are rare when these patients are treated by an experienced craniofacial team.

Frontofacial Monobloc Advancement With Simultaneous Frontal Cranioplasty in Adolescents With Residual Apert Syndrome Deformations.

The treatment of faciocraniosynostosis has steadily evolved since the introduction of craniofacial surgery in the 1950s. The aim of this study is to demonstrate the positive results obtained by frontofacial monobloc advancement with simultaneous frontal cranioplasty in adolescents with adult facial bones and residual Apert syndrome deformations. Three adolescents underwent surgery between September 1, 2010 and March 31, 2011. All had faciocraniosynostosis in the context of Apert syndrome and had undergone brain decompression surgery during the first year of life. However, they presented intracranial hypertension. The authors carried out frontofacial monobloc advancement with internal distraction and frontal cranioplasty. The mean frontal advancement was 13.8 mm. The mean maxillary advancement was 16.3 mm. About exorbitism, 2 patients had grade III and 1 had grade I before surgery. After monobloc advancement, 2 patients had no exorbitism and 1 had grade I. About dental occlusion, 3 patients had class III before surgery and were overcorrected in class II after advancement. Frontofacial monobloc advancement yields satisfactory functional and esthetic results in these cases. In conclusion, simultaneous frontofacial monobloc advancement and cranioplasty appears to be a promising technique for the treatment of adolescents with residual craniofacial deformations.

Aberrant facial flushing following monobloc fronto-facial distraction.

Patients with syndromic forms of craniosynostosis may experience functional problems such as raised intracranial pressure, proptosis, obstructive sleep apnoea and failure to thrive. The monobloc fronto-facial advancement with osteogenic distraction is increasingly used to correct these functional problems in one procedure as well as improve appearance. The authors report the phenomenon of post operative aberrant facial flushing - an unusual and previously unreported complication of the procedure. The case notes of 80 consecutive patients undergoing fronto-facial advancement by distraction using the rigid external distraction device (RED) were reviewed for features of aberrant facial flushing. Four out of eighty individuals developed facial flushing after monobloc fronto-facial distraction using the rigid external distractor (RED) frame. All were female with Crouzon or Pfeiffer syndromes causing the severe functional problems for which they underwent the surgery. They were aged 6-8 years. Following removal of the frame, they developed intermittent but severe facial flushing. The flushing spontaneously settled in three patients after up to four years but persists in the other child seven years after her surgery. Aberrant facial flushing is a rare but significant complication of monobloc fronto-facial surgery. It occurred in 4 of our 80 (5%) patients. The skull base osteotomies essential for the procedure are made anterior to the pterygopalatine ganglion and it is our contention that damage from these was responsible for a neuropraxia of its efferent nerve branches. A review of the autonomic control of the facial vascular system suggests that the phenomenon is due to an unequal process of recovery that leaves the cutaneous vasodilating parasympathetic or beta-adrenergic innervation relatively unopposed - a situation that persists until with time a normal balance of autonomic input is achieved.

Frontofacial surgery in children and adolescents: techniques, indications, outcomes.

The techniques of frontofacial surgery are most valuable in the clinical management of complex craniofacial deformity to achieve a range of functional and aesthetic gains in children from infancy to maturity. A variety of complex craniofacial osteotomies that can be used to separate the orbits from the skull base have been described. In addition, the combination of circumorbital release and pterygomaxillary disjunction allows advancement of the orbitomaxillary segment for powerful clinical benefit. For the purpose of this article, the principal frontofacial strategies include the monobloc frontofacial advancement by distraction (MBD), frontofacial bipartition advancement by distraction (BpD), orbital box osteotomy (FFBx), and frontofacial bipartition (FFBp). These techniques are broadly used for two purposes: to allow for the translocation of one or both orbits to correct orbitofacial disproportion (hypertelorism, vertical orbital dystopia, or a combination of both), or to advance the orbitomaxillary segment for orbital volume expansion and protection of the eye in syndromes featuring severe exorbitism (oculo-orbital disproportion). Here we describe aspects of our experience of frontofacial surgery in the Craniofacial Centre at Great Ormond Street Hospital for Children, London, with reference to the principles underpinning frontofacial surgical techniques, their challenges, and their impact on function and aesthetics.

Orbitofrontal monobloc advancement for Crouzon syndrome

IntroductionUsually, patients suffering from Crouzon syndrome have synostosis of coronal sutures, exophthalmia, hypertelorism, and hypoplasia of the middle third of face. Sometimes maxillary retrusion is absent, so these patients have class I or II relationship. In these cases, frontofacial monobloc advancement, which is the gold standard, increases the maxillo-mandibular dysmorphia. Therefore we propose orbitofrontal monobloc advancement minus dental arch, without splits of the pterygoid plates. Case reportA 12-year-old girl with Crouzon syndrome had intracranial hypertension, exophthalmia, a middle third retrusion and a class II occlusion. We achieved orbitofrontal monobloc advancement which is frontofacial monobloc advancement minus maxillary dental arch. Four distractors KLS Martin were used. After 20 days of distraction, the final advancement was 10.2 mm for cranial distractors and 10.5 mm at fronto-zygomatic. Distractors were removed after 8 months. DiscussionWe offer patients suffering from Crouzon syndrome with class I or II relationship a change from the classic frontofacial monobloc advancement leaving the maxillary dental arch in place, thus avoiding the worsening of the maxillo-mandibular dysmorphia related to surgery. The idea of associating Le Fort I osteotomy with a frontofacial monobloc advancement or Le Fort III osteotomy has already been described, mainly by Tessier and Obwegeser, however they probably achieved a complete Le Fort I osteotomy while we don't split the pterygoid plates.The patient's morphology and his surgical history determine the choice between Le Fort III and monobloc advancement. Dental occlusion needs to be taken into account for surgical indication.

Monobloc and Bipartition in Craniofacial Surgery

The frontofacial monobloc advancement with osteogenic distraction is increasingly used as a surgical treatment for children with complex craniosynostosis-associated syndromes. However, the subfrontal osteotomy cuts to free the facial skeleton from the skull base require extradural retraction of the frontal lobes. The purpose of this study was to determine the frequency and degree of radiologically identifiable frontal lobe changes and whether any such changes affected the patients' outcome. The clinical records and preoperative and postoperative computed tomography imaging of all patients undergoing monobloc frontofacial distraction advancement (with or without bipartition) were reviewed. A retrospective medical notes review was undertaken to assess any patient or surgically related factors that might predispose to frontal lobe changes and evaluate outcome from surgery. Where available, magnetic resonance imaging scans were reviewed to compare outcome with that on computed tomography. Fifty cases were identified as suitable for the study. Eighteen patients (36%) had no frontal lobe changes. Thirty-two cases (64%) did have changes that appeared related to the position of maximum retraction during subfrontal osteotomy cuts. There were no changes in the incidence/extent of these changes over time or of any link to the patients' diagnosis, age at surgery, phenotype severity, surgery type, or any surgical or postoperative adverse events. We found no evidence that these changes were responsible for neurologic problems (eg, epilepsy) or reduced cognitive function. This study reveals a high incidence of frontal lobe changes demonstrable on neuroimaging following the frontofacial monobloc procedure reflecting the retraction points during surgery. Although no postoperative disability was reported, it is clearly important to consider more detailed neuropsychologic testing and review current surgical techniques to ensure that such changes are kept to a minimum.

“Bibloc Advancement” With a Combination of Internal and External Distracters

Frontofacial monobloc advancement is one of the most rewarding techniques for correcting aesthetic and functional problems of patients with severe craniofacial synostosis, which can advance the upper and middle third of the face simultaneously. Application of gradual distraction technique has been reported to reduce notorious risks after a frontofacial monobloc advancement. The so-called "bibloc advancement" is a derivative of the frontofacial monobloc advancement. "Facial bloc" is horizontally divided into 2 different components: fronto-orbital component and maxillozygomatic component. From a different angle, it can be described as a combination of fronto-orbital advancement and Le Fort III advancement. Two pairs of distracters (1 internal for the supraorbital area and 1 external for the maxillozygomatic area with a pair of cross-facial pinning) were applied after the so-called bibloc osteotomy. Advancement of the upper and middle third of the face was done individually. This technique can be a good option for treating infants with severe syndromic craniofacial synostosis.

Faciocraniosynostosis: monobloc frontofacial osteotomy replacing the two-stage strategy?

Frontofacial monobloc advancement (FFMBA) is a powerful but high-risk procedure to correct both exorbitism and impaired airways of faciocraniosynostosis. One hundred and five children with faciocraniosynostosis (mean 4.9 years, 7 months-14 years) were evaluated prospectively after FFMBA and quadruple internal distraction. The advancement was started at day 5 (0.5 mm/day). Mean follow-up was 61 months (maximum 10.5 years). Relapse was evaluated by the comparison between the evaluation at the time of removal of distractors and 6 months later. Seventy-six patients (72%) completed their distraction uneventfully in the initial period. - One death at D1 from acute tonsillar herniation before beginning of distraction. - Cerebrospinal fluid leaks managed conservatively (11 patients) and with transient lumbar drainage (eight patients). - Revision surgery (dysfunction/infection) of distraction devices (nine patients, subsequently four completed the distraction). Ninety-nine out of 104 patients finally completed their distraction, resulting in exorbitism correction. Respiratory impairment, when present, was corrected and class I occlusal relationship was obtained in 77% of the cases. Reossification was limited at the orbital level but relapse could be prevented by a retention phase of 6 months. Pfeiffer syndrome, previous surgeries, and surgery before 18 months of age were risk factors. Internal distraction allows early correction of respiratory impairment and exorbitism of faciocraniosynostosis. In order to limit the risks, we advise: - Preliminary craniovertebral junction decompression if needed - Four devices to customize the distraction - Double pericranial flap to seal the anterior cranial fossa - Systematical external transient drainage if CSF leak - Slow rate of distraction (0.5 mm/day) - Long consolidation phase (6 months).

Idade e indicações de osteotomias para avanço frontofacial em pacientes com craniossinostoses sindrômicas

INTRODUÇÃO: Desde o início da Cirurgia Craniofacial, muitos desafios foram ultrapassados. Problemas operatórios técnicos e de infraestrutura básica de atendimento especializado foram solucionados. Agora, 25 anos após as publicações iniciais dos avanços frontofaciais, há ainda algumas dúvidas quanto às indicações precisas da idade e do tipo de cirurgia a ser realizada. O objetivo deste estudo foi avaliar a evolução de pacientes submetidos a tratamento de craniossinostoses sindrômicas operados nos últimos 10 anos em nossa instituição. MÉTODO: Todos os pacientes sindrômicos submetidos a avanço frontofacial em monobloco ou somente facial isolado foram selecionados no período de 2001 a 2011. Foram selecionados 70 pacientes, 56 submetidos a avanço frontofacial em monobloco e 14, a avanço facial após remodelagem frontorbitária prévia. Todos os dados referentes a esses pacientes foram correlacionados, avaliando a idade e o resultado final. Os pacientes foram selecionados de acordo com idade à época da cirurgia, complicações existentes e resultados finais correlacionados com os principais problemas existentes previamente. RESULTADOS: Os pacientes sindrômicos apresentaram graus variados de resultados finais, dependendo da síndrome e da idade de realização do procedimento. Os avanços frontofaciais em monobloco apresentaram baixo índice de complicações pós-operatórias imediatas, porém ficou demonstrada a necessidade de procedimentos futuros ao final do crescimento facial. Nos pacientes submetidos a cirurgias mais tardiamente, o índice de resultados positivos foi maior. CONCLUSÕES: Nos casos de craniossinostoses graves, com problemas funcionais, a indicação de avanço frontofacial em monobloco continua sendo a melhor opção terapêutica.

Frontofacial Monobloc Distraction in the Very Young

Frontofacial monobloc advancement by distraction osteogenesis is now accepted as an effective treatment for children with syndromic and other complex forms of craniosynostosis because it combines cosmetic advantages with the ability to treat such complications as raised intracranial pressure, advancing exorbitism, and upper airway obstruction in one procedure. In the severely affected child, these complications may be present at an early age, but because of the scale of monobloc surgery, surgeons have been reluctant to perform it on very young children. The authors report a series of 12 children younger than 30 months with various craniosynostosis-associated syndromes complicated by multiple functional problems--including raised intracranial pressure, exposure of the eyes, airway obstruction, and feeding problems--who underwent frontofacial monobloc surgery by distraction osteogenesis. The mean age at operation was 18 months. Mean advancement achieved was 16.6 mm for the upper face and 17 mm for the midface. Ocular protection and reduction of intracranial pressure (when raised) were achieved in all children. Airway improvement was achieved in all but one child. Although not the primary indication for surgery, there was also a marked improvement in every patient's appearance. Complications included two cerebrospinal fluid leaks, pin-site infections, frame slippage requiring replacement, and enophthalmos in one patient. The frontofacial monobloc advance incorporating distraction osteogenesis can be undertaken with acceptable morbidity even in the very young child and good functional outcomes achieved with one operation where previously more were required. Therapeutic, IV.

Cranium and Midface Distraction Osteogenesis

The adaptation of distraction osteogenesis (DO) to the midface and cranium in the 1990s and the advancements that followed at the turn of the century resulted in a shift of paradigm in craniofacial surgery. Because skeletal advancement was not sudden anymore, but incremental, the monobloc advancement became safer to perform. Because bone was generated in the distraction gap, bone grafts were no longer needed, and younger patients could benefit from craniofacial advancement. Today, DO is the most powerful tool to simultaneously correct both exorbitism and the respiratory impairment of the faciocraniosynostosis, but practices vary greatly between teams. Current practices, controversies, and near-term future applications will be outlined and discussed. Our current treatment strategy for faciocraniosynostosis is based on early intervention (<18 months of age) to prevent irreversible brain damage. In the first 6 months of life, infants with faciocraniosynostosis receive posterior vault decompression. We currently use posterior vault distraction, using 2 internal distractors. Around 18 months of age, a frontofacial monobloc advancement with DO is performed. It further decompresses the brain, improves respiratory function, and corrects exorbitism. Because we operate at such an early age, we favor internal over external distractors. In severe faciocraniosynostosis, when midface hypoplasia causes major exorbitism endangering the eye or causes respiratory distress requiring a tracheotomy, we do not hesitate to perform a frontofacial monobloc advancement with DO before the age of 18 months, reinforcing the frontozygomatic junction with a plate and placing a transzygomatic pin. The pin is then connected to a traction rope. We frequently use the external distractors, which allow precise control over the rotation of the maxilla and are well tolerated after 5 years of age. When midface hypoplasia is very severe, we combine external and internal distractors. The ongoing debate between proponents of internal versus external distractors or 1-stage versus 2-stage approach is based mostly on anecdotal data. Multicenter prospective studies are necessary to bring objective data to answer these questions.

Reossification of Cranium and Zygomatic Arch After Monobloc Frontofacial Distraction Advancement for Syndromic Craniosynostosis

Monobloc frontofacial advancement with distraction is becoming more routinely used within craniofacial surgery for faciocraniosynostosis, because of the simultaneous correction obtained on the exorbitism and of the respiratory impairment. Reossification of the cranium and zygomatic bone in monobloc frontofacial advancement with distraction has not been assessed previously on long series. In this study, 40 patients, 22 Crouzon, 11 Apert, and 7 Pfeiffer syndrome who underwent a frontofacial monobloc advancement by distraction osteogenesis, were retrospectively reviewed, after a mean of 2.1 years of follow-up. The bone linkage between both margin of the coronal and zygomatic osteotomy gap was evaluated on three-dimensional computed tomographic scan postoperatively. The correlations between reossification and some clinical situations (diagnosis, existence of previous anterior craniofacial procedure, use of bone paste, and the age at operation) were studied to determine factors that influence on reossification. "Good" or "fair" reossification on coronal gap was demonstrated by 68.2% of those with Crouzon and 54.5% of those with Apert syndrome. In contrast, 85.7% of those with Pfeiffer syndrome had "poor" or "absent" reossification. Rebridging of the zygomatic arch in Pfeiffer was also the poorest among 3 syndromes. Previous operations performed before the frontofacial monobloc advancement decreased reossification of distraction gap. In the group of patients in whom autologous bone paste on coronal osteotomy gap was applied, the bone formation was improved in all syndromes significantly. The reossification of the coronal osteotomy gap in patients with Pfeiffer syndrome is poor compared with those with Crouzon and Apert syndromes. Bone paste is extremely effective on increasing osteogenesis even in patients with Pfeiffer syndrome or the patients with previous surgery.

Frontofacial Monobloc Distraction Using the StealthStation Intraoperative Navigation System

The frontofacial monobloc advancement is a safe, effective, and cosmetically acceptable treatment of frontal and midface retrusions present in craniofacial syndromes. Although a number of modifications have been put forward to supplement this procedure, the basic steps of cleaving the facial skeleton from the skull base remain the same. We present a case where intraoperative navigation was used to visualize the craniofacial anatomy while the bone cuts were made for this cleaving process. This technique visually demonstrates parts of this procedure that were hitherto performed solely using tactile and auditory inputs superimposed on a 3-dimensional model of the craniofacial anatomy in the surgeon's mind. This technique offers a significant advantage for teaching purposes, in performing cases with complex anatomy and for accurate positioning of the distractor devices.

Frontofacial monobloc advancement using gradual bone distraction method

For children with craniosynostosis presenting intracranial hypertension and facial hypoplasia, different techniques have been used. Commonly, an initial frontoorbital advancement to expand a tight cranium volume was followed by Le Fort III osteotomy to repair midface abnormalities several months later. The 2-stage surgeries were unified into a 1-step procedure to optimize treatment. We here report results of frontofacial monobloc advancement, applying gradual distraction by a rigid external distraction device. Three patients were treated with excellent functional and cosmetic outcome with high rate of patient satisfaction. There was no remarkable complication.

The Value of the Maxillo-Malar Osteotomy in the Treatment of Crouzon Syndrome With Exorbitism

Premature fusion of the cranial sutures along with midface hypoplasia, shallow orbits, and ocular proptosis are the principal features of Crouzon syndrome. Treatment varies according to the variable expressivity of the disease and usually begins during a child's first year with fronto-orbital advancement with cranial decompression. Subsequent development of midfacial hypoplasia needs correction. Procedures for this purpose include the Le Fort III osteotomy or its segmental variants, monobloc frontofacial advancement, or bipartition osteotomy. Adult Crouzon often presents with marked midface hypoplasia and exorbitism. We report an adult-diagnosed Crouzon syndrome case with typical facial features and exorbitism corrected by orbital decompression and zygomaticomaxillary advancement.