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Plastic surgery is facing a major problem: the risk of a splitting, a divorce between the aesthetic and reconstructive parts of our speciality – the public hospitals dealing with the reconstructive cases, the private hospitals and private offices dealing with the lucrative aesthetic surgery.

Trigonocephaly secondary to the premature fusion of the metopic synostosis is associated to a risk of cerebral compression and several craniofacial morphological alterations. Numerous surgical techniques have been proposed. They all carry a risk of secondary temporal hollowingThe aim of this paper is to describe the surgical technique used for trigonocephaly at the craniofacial unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis) focusing on its advantages and limitations. Resorbable osteosynthesis should be part of the current techniques.

Objective The aim of this paper is to describe the surgical technique, originally devised by Dr. Renier which is currently used to treat children with scaphocephaly under 6 months of age at the Craniofacial Unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis), focusing on its advantages and limitations.

BACKGROUND This report documents the authors' experience with 95 hypertelorism corrections performed since 1971. The authors note their findings regarding outcomes, preferred age at surgery, technique, and stability of results with growth. METHODS Patients were classified into three groups: midline clefts (with or without nasal anomalies, Tessier 0 to 14); paramedian clefts (symmetric or asymmetric with or without nasal anomalies); and hypertelorism with craniosynostosis. The authors developed a hypertelorism index to measure longitudinal orbital position. RESULTS A total of 70 box osteotomies were performed. Twelve of 95 patients had a bipartition. Six of 95 patients underwent a unilateral orbital box displacement or a three-wall mobilization, and seven of 95 had a medial wall osteotomy. Eighty patients were graded 1 to 4 using the Whitaker scale. Fifty-nine of 80 patients received a grade of 1, 15 patients received a grade of 2, five patients received a grade of three, four patients initially scored a 4, and three patients underwent reoperation and were rescored as 1. The authors developed a hypertelorism index to rate 28 patients with long-term follow-up. None showed deterioration of results over the long term. The complication rate was 4 percent. CONCLUSION The most interesting finding was that an initially good result in terms of orbital correction, whatever the severity, remains good with time, and facial balance improves after completion of growth. CLINICAL QUESTION/LEVEL OF EVIDENCE Therapeutic, IV.

Background: in April of this past year, Transatlantic Innovations brought a variety of organizations and industry together for an international exchange of ideas, new technology, and current trends in plastic surgery. Methods: The meeting was highly interactive and included audience response devices. The focus was on 10 major areas: (1) new surgical techniques; (2) composite allografts versus conventional techniques, facing the future; (3) interspecialty collaboration versus competition; (4) the business of plastic surgery, best practices; (5) the image of the plastic surgeon, branding yourself; (6) medical tourism; (7) publicity; (8) the regulation of innovation, U.S. Food and Drug Administration and European Medicines Agency perspective; (9) the future of plastic surgery, cutting edge technologies; and (10) applications and controversies in fat grafting. The meeting concluded with the 8th International Committee for Quality Assurance, Medical Technologies and Devices in Plastic Surgery Consensus Conference with the development of a consensus statement. Results: Through an interactive audience response system, additional questions and attitudes were asked of the audience and, in real time, international differences were identified, which led to further discussions from panelists. Responses were identified in three major groups: European Union, North America, and the Rest of the World. Responses and data are included in this article. Conclusions: The meeting brought participants, industry, regulators, and educators from both sides of the Atlantic. The interaction of these groups in these outlined topics brought a unique perspective to the meeting and, in the end, volumes of data. We have more in common than we believe. It is our anticipation that as we as plastic surgeons move forward, we can use these interactions to help our own practices but more specifically the specialty as a whole. (Plast. Reconstr. Surg. 126: 316, 2010.)

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.

There is virtually no literature on the effect of correction of syndromal faciocraniosynostosis with monobloc advancement on the palpebral fissure shape. Using image processing software, we measured the effect of monobloc advancement on the position of the upper and lower eyelids as well as the palpebral fissure slant in a series of 18 patients with syndromal faciocraniosynostosis who had undergone surgery for correction of orbital and midface hypoplasia. For both eyes of each patient, 3 variables were measured on the pre- and postoperative photographs: the linear distances between the upper and lower eyelid margins, the pupil center and the angle between the inner and outer canthi. The globe protrusion was also measured on axial computed tomography scans before and after surgery. The results indicate that the exorbitism reduction induced by monobloc advancement is accompanied by a diminution of the distance between both eyelids and the pupil center. However, the downward slant of the palpebral fissure is increased after surgery. The data suggest that the lower and upper eyelid retraction seen preoperatively in the majority of patients with faciocraniosynostosis tend to be corrected when the frontofacial region is advanced by the monobloc. On the other hand, the surgery tends to lower the outer canthus, increasing the negative slant of the fissure. The postoperative changes induced by the frontofacial monobloc advancement need to be taken into account when the surgery is going to be performed.

After a frontofacial monobloc in a 12-year-old Crouzon patient, the frontal bone was lost to infection. The patient did not attend postoperative appointments and was lost to follow-up. Nine years later, he reappeared requesting surgical reconstruction. This was performed satisfactorily with a titanium plate; however, it became apparent later that he had considerable atrophy of the frontal lobes and had developed the unstable behavior of patients who have undergone a frontal lobotomy. The atrophy would appear to be the consequence of the long-term absence of the frontal bones and the resultant repetitive frontal lobe microtrauma. Frontal infection and subsequent loss of the frontal bone is a recognized complication of monobloc advancement for faciocraniosynostosis and is mainly caused by the communication between the oronasal cavities and the cranial base. The frontal bone defect would normally be repaired after a delay of 6 months to a year with no significant sequelae. We present the unusual case of a patient whose frontal defect was not reconstructed for 9 years and who, as a result, developed frontal lobe atrophy and subsequent severe psychologic and behavioral difficulties.

Neurofibromatosis of the orbitopalpebral complex is a debilitating disease. The development of safe craniofacial surgical technique has greatly improved outcome in the surgical palliation of this progressive condition, and various centres have reported their experience. Extensive upper eyelid neurofibroma creates a functional and aesthetic problem, which is inadequately addressed in the literature. The experience of the senior author (DM) representing the treatment of 27 such patients is currently reported. The stigmata of eyelid neurofibromatosis were scored retrospectively by a novel scale applied to standardised photographs at patient presentation. A numeric severity score was given to each of: upper eyelid ptosis, canthal malposition, and oculo-palpebral diastasis. Disease progression and post-operative result were similarly scored at various stages follow-up photography. All patients underwent a full thickness, transverse or transverse-oblique resection of upper eyelid disease, with immediate levator reconstruction and canthopexies as necessary. A mean improvement of 3.33 points was achieved in the majority of patients (median one operation per patient; mean 1.5, range 1-3). Secondary eyelid procedures (n=9 pts) represented tumour debulking, canthopexy, or procedures to deepen the fornix in patients with prostheses. This series demonstrates that a tailored, often radical palpebral remodelling in orbitopalpebral neurofibromatosis may provide functional and aesthetic benefit.

Fibrin glue is increasingly finding use in plastic surgery at the clinical and basic science level. The authors conducted a prospective, nonblinded, randomized, controlled trial in 30 patients undergoing face lifts to examine the efficacy of fibrin glue in reducing postoperative wound drainage, hematomas, and, in particular, the degree of ecchymosis and edema at 24 hours and at 8 days. Patients were their own controls and were randomized to have the glue on one side of their face only to compare the glued and unglued sides. The patients ranged in age from 42 to 72 years (mean age, 60 years). There was one major hematoma requiring surgical evacuation. In the remaining 29 patients, the mean drainage on the glued side was 26 ml, compared with 33.5 ml on the control, unglued side. This difference was statistically significant numerically (p = 0.037) but was not thought to be surgically significant. Comparing scores among grades of hematomas, ecchymosis, and edema, there were minimal differences between the glued and unglued sides. This study suggests that fibrin glue may not be as beneficial as previously thought in reducing ecchymosis and edema in the early postoperative period after face lifts, and its future role is discussed.

Bioabsorbable osteofixation devices were developed to avoid problems associated with metals. Bioabsorbable devices are mostly made of the polymers polylactide, polyglycolide, and their copolymers [polyglycolide-co-polylactide and P(L/DL)LA]. Using the technique of self-reinforcement of bioabsorbable materials, it is possible to manufacture osteofixation devices with ultra high strength. Self-reinforced polyglycolide-co-polylactide 80/20 was selected to make devices (Biosorb PDX) for this study because of its favorable degradation characteristics. The aim of this study was to evaluate the efficacy of using self-reinforced polyglycolide-co-polylactide 80/20 (Biosorb) plates and screws in the fixation of osteotomies in craniomaxillofacial surgery. In a prospective study, 165 patients (161 children and 4 adults) were operated on in four European Union centers (Paris, Innsbruck, London, and Oulu) from May 1, 1998 to January 31, 2002. Indications included correction of dyssynostotic deformities (n = 159), reconstruction of bone defects after trauma (n = 2), tumor removal (n= 2), and treatment of encephalocele (n = 2). Plates used were 0.8, 1, or 1.2 mm thick, and screws had an outer (thread) diameter of 1.5 or 2 mm and a length of 4, 6, or 8 mm. Tacks had an outer diameter of 1.5 or 2 mm and a length of 4 or 6 mm. During surgery, the devices were easy to handle and apply and provided stable fixation apart from 2 cases. Postoperative complications occurred in 12 cases (7.3%), comprising infection (n = 6), bone resorption (n = 4), diabetes insipidus (n = 1), delayed skin wound healing/skin slough (n = 2), and liquorrhea (n = 1). Accordingly, self-reinforced polyglycolide-co-polylactide 80/20 (Biosorb) plates and screws can be used safely and with a favorable outcome in corrective cranioplasties, especially in infants and young children.

Introduction La strategie de prise en charge des faciocraniostenoses comprend habituellement au moins deux temps operatoires majeurs : un avancement frontal avant un an pour traiter la craniostenose, et un ou plusieurs avancements faciaux pour corriger la retrusion faciale. L’avancement fronto-facial monobloc (AFFM) est une intervention qui permet de corriger simultanement ces deux anomalies crânienne et faciale, mais, dans sa forme classique, sa morbidite en restreignait l’indication. Patients et methode Nous rapportons l’experience preliminaire de l’AFFM chez 20 enfants dont l’âge moyen etait de 3,2 ans (ages de 6 mois a 14 ans). L’intervention a consiste en une osteotomie de type fronto-faciale monobloc totale avec mobilisation peroperatoire sans avancement. Quatre distracteurs (Martin-Medizin), deux frontaux supra-orbitaires et deux faciaux temporo-malaires ont ete positionnes et syntheses avec du materiel d’osteosynthese resorbable ou metallique. Chez un des patients les plus jeunes, une broche transfaciale a ete utilisee entre les deux systemes malaires. La distraction a ete debutee a J7 en moyenne (J5 a J15) avec un rythme de 1 mm par jour sur chaque distracteur. L’avancement a ete mene jusqu’a la butee maximale des dispositifs ce qui a necessite de 15 a 20 jours. Le recul maximal a ete de 30 mois. Resultats La correction de l’exorbitisme superieur et inferieur a ete obtenue dans 18/19 cas permettant de retablir l’occlusion palpebrale dans les cas extremes. Chez un enfant pour lequel la course du distracteur a ete insuffisante (15 mm au lieu de 20), la correction a ete partielle. La correction de la retrusion maxillaire a egalement ete satisfaisante chez 17/19 cas. Deux episodes infectieux sont survenus durant cette phase initiale au niveau frontal et ont necessite le retrait d’un distracteur et l’arret de la distraction (2/19). Une rupture de l’un des distracteurs est survenue chez un des patients a egalement necessite une reintervention precoce. Un deces immediat par hypertension intracrânienne suraigue est survenu chez l’un des patients dans les 12 premieres heures avant meme que la distraction n’ait ete debutee, chez une maladie de Crouzon traitee tardivement. Chez trois des six premiers patients, l’ablation des dispositifs de distraction a 3-4 mois a eu pour consequence un recul secondaire. Pour les suivants, les dispositifs ont ete laisses plus de six mois et la conservation de l’avancement initial semblait obtenue, mais l’osteogenese restait limitee. Discussion L’utilisation de la distraction lors d’un avancement frontofacial monobloc semble en reduire la morbidite. Il serait ainsi possible d’augmenter les indications d’une telle procedure dont le principe est adapte aux faciocraniostenoses severes. L’insuffisance de croissance genetique necessite d’autres interventions ulterieurement. L’evolution a long terme reste a etre evaluee.

Osteolipoma is a very rare condition, and only a few cases affecting the head were found in the literature. None can be compared with the giant lesion of the side of the head developing in the left temporo-parietal region of an 8-year-old boy from Central Africa. Under a huge lipoma, an irregular bony mass was found. Contouring resection was performed with an uneventful recovery. Technique and pathology are discussed.

Bilateral coronal synostosis causes functional and morphological problems that require fronto-orbital advancement in infancy to correct the brachycephalic deformity and to prevent mental impairment caused by the intracranial hypertension. In this study, 99 children with isolated cases of brachycephaly were prospectively followed to study their preoperative and postoperative mental outcome, which was evaluated using developmental or intelligence quotients. Several factors were analyzed: age before treatment, age at the time of surgery, and the correlation between mental assessments before and after surgery. In a subgroup or patients tested for the FGFR3 P250R mutation (n = 48), mental and morphological assessments were analyzed. Before surgery, mental status was better in the patients tested before 1 year of age (p0.001). The preoperative mental assessment always correlated with the postoperative assessment (p0.0001). The postoperative mental outcome was better when surgery was performed before the patient reached 1 year of age (p0.02). Although both the morphological and functional outcomes were better in the subgroup of noncarriers of the mutation, the differences were not statistically significant. Prominent bulging of the temporal fossae was frequently responsible for poor morphological outcome in carriers of the mutation. This study confirms the need for early corrective surgery before 1 year of age in brachycephalic patients to prevent impairment of their mental development. Suboptimal morphological and mental outcomes can be expected in patients with nonsyndromic brachycephaly who carry the FGFR3 P250R mutation. Primary correction of the temporal bulging should be performed in conjunction with fronto-orbital advancement to improve the morphological outcome in patients with the mutation.

Frontofacial monobloc advancement is a surgical procedure which corrects at the same time both frontal and facial retrusions in faciocraniosynostosis. The high risk morbidity limited its use to major exorbitism or severe breathing impairment. We report the results of a preliminary experience in five children with a mean age of 3.8 years. All patients presented with a Crouzon or Pfeiffer syndrome. The procedure performed was a frontofacial monobloc osteotomy with peroperative mobilization. Two pairs of distractors (Martin-Medizin) were implanted. The upper pair of frontal distractors was located behind the supraorbital bar. The pair of temporo-zygomatic distractors (a modification of the MicroFrance prototypes) was implanted behind the zygomatic bone. In one patient a transfacial pin was connected to the anterior part the lower distractors, to avoid facial bipartition. Distraction was performed according to classical protocols with a 1 mm advancement per day, until sufficient advancement was obtained. The correction of exorbitism and facial retrusion was obtained in all cases. An open-bite was created by the advancement in two out of the five patients. Minor infection occurred in one patient. This combination of distractors with frontofacial advancement seemed to increase the efficacy and reduce the morbidity. This might lead to a one stage strategy for faciocraniosynostosis treatment, even if minor additive procedures will be necessary when children get older and that insufficient facial growth still reoccurs.

Craniofacial asymmetry is caused by various aetiologies but clinical examination remains the most important criteria since minor asymmetry is always present. The diagnosis can be confirmed by anthropometric measurements and radiological examinations but only severe asymmetries or asymmetries with an associated functional impairment should be treated. The treatment depends on the cause, and on the time of appearance. Congenital asymmetries might be treated early, during the first year of life if a craniosynostosis is present. Hemifacial microsomia are treated later if there is no breathing impairment. Since the pediatricians have recommended the dorsal position for infant sleeping, an increasing number of posterior flattening of the skull has been appearing, and could be prevented by adequate nursing. Other causes of craniofacial asymmetries are rare and should be adapted to the cause (tumors, atrophies, neurological paralysis, hypertrophies) by a specialized multidisciplinar team.

The wall separating the face and the cranium was broken by Paul Tessier and Gérard Guiot in the 1960s, making it possible to perform a combined operation around the orbits and forehead, and opening up close cooperation between plastic surgeons and neurosurgeons, especially for treatment of the major malformations such as hypertelorism of major facial retrusions. The principles of mobilization of the orbits to correct teleorbitism or orbital dystopia are recalled with reference to the different variations and with clinical examples. Facial advancement to correct the retrusions created by faciocraniosynostosis is explained with the many possible variants, combined with a intracranial approach or not, with or without a bipartition. The indications are discussed as is the risk linked to combined advancement of face and forehead. The progressive bone elongation principle introduced by Ilizarov for the limbs has been applied to the face at the mandibular level by McCarthy, with great success. The distraction of bone structures is now also applied at the level of the midface and makes it possible to overcome the retraction of soft tissues and lower the risk of relapse of facial retrusion. Many technical problems have still to be solved, but the results are already very promising. Many other applications of the distraction principle will be developed for the midface level, with other technical improvements such as the use of absorbable mini-plates and screws.

Learning Objectives: The reader is presumed to have a broad understanding of plastic surgical procedures and concepts. After studying the article, the participant should be able to: 1. Review the surgical anatomy of the temporal scalp area. 2. Evaluate a surgical approach to the periauricular area that minimizes visible postoperative scarring. Physicians may earn 1 hour of Category 1 CME credit by successfully completing the examination based on material covered in this article. The examination begins on page 194. Background: Hiding the scars and avoiding hairline displacement are crucial in performing face lift surgery. Objective: We review temporal and retroauricular flap techniques aimed at avoiding hairline displacement. Methods: We introduce a new “high” retroauricular flap, used since 1997, which avoids the usual transverse retroauricular scar. Results: The objective of maintaining the hairline has been achieved with this incision. Conclusions: To diminish hairline displacement and retroauricular scars in face lifts, the temporal rotation flap and the retroauricular flap with and without a “high” skin flap are used.

Gilbert P. Gradinger, MD Madeleine Lejour, MD Eugene H. Courtiss, MD Daniel Marchac, MD Dr. Gradinger: In 1960 Dr. Jan Strombeck1 published his experience with the horizontal bipedicle technique for reduction mammoplasty. In 1972 Dr. Paul McKissock2 published an article on the vertical bipedicle technique. Each of our panelists was selected because of his or her significant contributions to the body of knowledge on the art of reduction mammaplasty. Dr. Eugene Courtiss3 described an inferior pedicle technique in 1977. Dr. Daniel Marchac4 published his work on the superior pedicle and short inframammary scar in 1982. Dr. Madeleine Lejour5, 6 presented her work in 1990 describing the use of a vertical technique in conjunction with liposuction to eliminate the horizontal scar. The first patient is a 46-year-old woman who is 5 ft, 4 in tall and weighs 145 lb. She has one child and is premenopausal. She has had no breast masses, and her mammography results have been normal. Her nipples and areolae are displaced medially, and the bulk of her breast tissue is in the lower outer aspect of the breast (Figure 1). Dr. Marchac, how would you address these problems? Figure 1 A , Front and B , lateral views of a 46-year-old woman whose nipples and areolas are displaced medially, and the bulk of her breast tissue is in the lower outer aspect of the breast. Dr. Marchac: This patient has an unusual breast configuration. I would use a vertical reduction mammaplasty technique with a short horizontal scar (Figures 2–4). In this instance I would excise much more breast tissue on the lateral side than on the medial side to end up with a desirable shape and size with the areola in the proper position. Figure 2 Vertical reduction mammaplasty technique with short horizontal …

Resume A partir d'une serie de 1 833 craniostenoses, les auteurs analysent 278 observations de trigonocephalies. La prevalence de la trigonocephalie est evaluee a 1/15 000 nouveau-nes. Patients et methodes Tous les patients porteurs d'une synostose de la suture metopique ont ete selectionnes, en excluant les cas ou d'autres sutures etaient egalement atteintes. L'âge au moment du diagnostic allait de 15 jours a 15 ans. Le diagnostic de trigonocephalie etait fait sur l'examen clinique et les radiographies du crâne. La recherche de malformations associees etait fondee sur la clinique, la tomodensitometrie crânienne (et/ou l'IRM), les radiographies du squelette et l'echographie abdominale. Autant que possible, une etude du caryotype etait realisee lorsqu'une malformation associee etait decouverte. L'etude familiale etait faite par contact direct ou, plus rarement, par telephone. Les observations ont ete divisees en trois groupes : groupe 1, les trigonocephalies isolees ; groupe 2, les cas avec malformations associees mais sans syndromes repertories ; et groupe 3, les syndromes repertories. Resultats Il y avait 213 garcons et 65 filles, donnant un sex-ratio de 3,3. Une enquete familiale etait disponible dans 216 cas. L'âge maternel moyen etait de 29,6 ans et l'âge paternel moyen etait de 32,2 ans a la naissance des enfants, ce qui correspond aux moyennes nationales. On comptait 18 grossesses multiples (7,9 %). Trois des six paires de jumeaux monozygotes etaient concordantes pour la craniostenose. On denombrait 13 cas de trigonocephalies familiales (6 %). Dans un seul cas, la transmission etait verticale ; tous les autres comportaient des germains atteints avec des parents indemnes. Onze des formes familiales correspondaient a des trigonocephalies isolees ; les deux autres etaient des formes syndromiques. Sur 53 caryotypes obtenus, neuf revelaient des anomalies, qui portaient sur les chromosomes 7, 9, 11 ou 13. Le groupe 1 comportait 208 observations. Le groupe 2 regroupait 53 cas : les malformations associees etaient uniques dans 32 cas et affectaient surtout le cœur (12 cas), les extremites (six cas), l'encephale (cinq cas) et l'appareil genito-urinaire (quatre cas). Elles etaient multiples dans 21 cas, le plus souvent a la fois viscerales et des extremites (15 cas). Certaines de ces associations pourraient correspondre a des syndromes encore non decrits. Dix-sept syndromes, dont neuf syndromes chromosomiques, representaient le groupe 3. Onze de ces syndromes presentaient des malformations multiples. Une exposition in utero a l'acide valproique etait retrouvee dans deux cas du groupe 1, cinq cas du groupe 2 et un cas du groupe 3.

Object. A retrospective study of 1727 cases of craniosynostosis was undertaken to determine the interrelationship between abnormal cerebrospinal fluid (CSF) hydrodynamics and craniosynostosis. Methods. The patients were divided into two groups: nonsyndromic craniosynostosis and syndromic craniosynostosis. Cases of occipital plagiocephaly without suture synostosis and cases of shunt-induced craniosynostosis were excluded from the study. The majority of patients (1297) were treated surgically for their cranial deformity; 95% of these patients had a postoperative follow-up review period lasting 5 years. Clinical and radiographic charts covering the time from presentation through the follow-up period were reviewed. Conclusions. Abnormal intracranial CSF hydrodynamics was found in 8.1% of the patients (3.4% of whom had received shunts and 4.5% of whom had not). Three types of CSF hydrodynamic disturbance were observed: progressive hydrocephalus with ventricular dilation, nonprogressive ventriculomegaly, and dilation of the subarachnoid spaces. Hydrocephalus occurred much more frequently in patients with syndromic craniosynostosis (12.1%) than in those with isolated craniosynostosis (0.3%). In fact, patients with kleeblattschädel exhibited hydrocephalus as a constant feature and patients with Crouzon's syndrome were far more likely to have hydrocephalus than those with other syndromes. In Apert's syndrome, ventricular dilation occurred very frequently, but it was almost always nonprogressive in nature. In most cases of syndromic craniosynostosis, venous sinus obstruction and/or chronic tonsillar herniation were found. Their role in the pathophysiology of hydrocephalus in craniosynostosis is discussed.

From a series of 1,713 patients with craniosynostosis hospitalized between 1976 and 1996, 237 propositi with metopic synostosis were analyzed. The prevalence of metopic synostosis was estimated in the order of 1 in 15,000 children. Family information was obtained from 184 propositi from 179 families. The male-to-female ratio was 3.3:1. There was no maternal or paternal age effect. A family history was obtained in 10 of the 179 families, giving a 5.6% figure of familial cases. The frequency of twinning was 7.8% with two concordances for metopic synostosis in two monozygotic twin pairs. The male-to-female ratio, the twinning frequency, and the proportion of familial cases in trigonocephaly are very similar to those observed in scaphocephaly, which also involves the longitudinal sutural system. Fetal exposure to valproic acid was noticed in eight cases. The series was divided into two groups: nonsyndromal trigonocephaly (n = 184) and trigonocephaly associated with other malformations (n = 53). The second group included 13 cases of well-delineated syndromes and 40 cases of trigonocephaly associated with one or more malformations, but without any known syndrome, that could be undelineated syndromes. These groups differed significantly in their mental prognosis.

Upper airway stenosis in patients with faciocraniosynostosis is very common and often severe. Midface advancement, either with a Le Fort III or concomitantly to a monobloc frontofacial advancement, may prevent a tracheotomy or result in its ablation. The amelioration of respiratory function appears to be much better if the midface advancement is combined with distraction osteogenesis, although large studies with long-term follow-up are rare. In this study, we reviewed the respiratory outcome between Le Fort III with distraction and monobloc advancement with distraction in 54 faciocraniosynostotic patients. Early respiratory results of both procedures were very good and stable at long-term follow-up. The choice between a Le Fort III and a monobloc procedure is made on the basis of presenting morphology, previous surgery, and age. Both can be expected to give a long-lasting improvement of upper airway obstruction.

Resume L’oxycephalie est mal definie dans la litterature. L’analyse de la presente serie d’oxycephalies vise a en preciser le cadre nosologique et les risques fonctionnels. Patients et methodes. — Les observations de 129 patients porteurs d’oxycephalie ont ete revues. L’analyse a porte sur les radiographies du crâne, l’examen ophtalmologique, le bilan psycho metrique, l’enregistrement de la pression intracrânienne, la tomodensitometrie crânienne et, pour les cas les plus recents, l’imagerie en resonance magnetique. Operes ou non, les patients ont ete surveilles et leur evolution a ete appreciee notamment sur des tests psychomoteurs, avec un recul moyen de 3 ans 7 mois. Resultats. — Pres des deux tiers des patients provenaient d’Afrique du Nord, ou cette craniostenose parait nettement predominante. L’âge moyen au moment du diagnostic etait de 6 ans. Des antecedents de rachitisme etaient retrouves dans 15 % des cas. Sur les radiographies du crâne, la grande majorite des patients avaient une synostose multisuturaire affectant les deux sutures coronates et la suture sagittale, et des impressions digitiformes diffuses. A la premiere evaluation psychometrique, un tiers des patients avaient un QI inferieur a 80. Un œdeme populaire etait retrouve dans 17% des cas, et pres des deux tiers des patients avaient a l’enregistrement une hypertension intracrânienne. Le fond d’œil etait normal chez 64% des enfants en hypertension intracrânienne. Douze des 16 patients explores par imagerie en resonance magnetique avaient une malformation de Chiari type 1. En postoperatoire, tous les œdemes populaires avaient regresse et la pression intracrânienne s'etait normalisee dans tous les cas ou un enregistrement de controle avait pu etre realise. Le niveau mental semblait se stabiliser, le QI moyen postoperatoire etant fortement correle au niveau preoperatoire. Chez les patients non operes, le niveau intellectuel se degradait de facon significative. Conclusions. — L’oxycephalie apparait comme une craniostenose d’apparition tardive, et elle comporte un risque eleve de complications ophtalmologiques et de retard mental. L’intervention, au vu de la presente serie, parait efficace en ce qui concerne la prevention de ces complications.

We report a case of an extremely rare craniofacial condition, which, to our knowledge, has previously been reported once only. A male infant presented with a giant congenital bone defect of the skull, in the vertex region (10 x 20 cm) and without scalp deficiency. Minimal turricephaly and moderate orbital hypertelorism were associated with slight limb abnormalities, but psychometrical assessments appeared normal. Nonsurgical treatment was initially decided upon, but spontaneous reossification was so moderate that skull reconstruction was carried out at 28 months of age because of the risk of trauma. A full-size resin cephalic skeletal reconstruction was obtained according to three-dimensional computed tomography using stereolithographic techniques. A titanium plate was customized on the resin model for ideal adaptation to the convex skull defect (8 x 16 cm). Surgery was simply performed, consisting of a preliminary undermining between the dura mater and the scalp and screwing of the custom titanium plate. The initial follow-up was uneventful.

Resume Le syndrome d'Apert est repute s'accompagner frequemment d'une debilite mentale, a l'origine de laquelle des malformations cerebrales pourraient jouer un role important. D'autres facteurs, tels l'âge a l'operation, la qualite du millieu sociofamilial pourraient egalement influencer l'avenir psycho-intellectual. Population et methodes. - Spixante enfants (32 garcons et 28 filles) ont ete inclus dans l'etude. Leur QI a eteevalue a l'aide de tests psychometriques adaptes pour l'âge. L'anatomie cerebrale a ete etudiee a l'aide de l'imagerie par resonance magnetique (IRM). Une attention particuliere a ete portee sur l'âge a l'intervention et sur la qualite de l'environnement familial. L'age a la derniere consultation etait superieur a 3 ans (3 a 28 ans, moyenne 10 ans) pour 38 patients. Resultats. - Le QI etait superieur a 70 chez 12 patients (32%), parmi lesquels cinq (13%) avnient un QI superieur a 90. Le QI moyen etait a 62 (10 a 114). Trente pour cent des enfants avaient une malformation du corps calleux, 43 % des ventricules lateraux et 55 % du septum pellucidum; l'IRM ne montrait aucune anomalie dans 28 % des cas. Une ou plusieurs interventions ont ete realisees chez 53 patients, avant l'âge de l an pour 37 d'entre eux. Dix enfants etaient places en institution ou vivaient dans un milieu familial precaire. L'âge au moment de l'intervention apparaissait comme le facteur principal du devenir mental: le QI final etait superieur a 70 chez 50 % des patients operes avant 1 an, contre seulement 8 % chez ceux qui avaient ete operes plus tard (p = 0,01). Parmi les malformations cerebrales observees, seules les anomalies du septum pellucidum paraissaient jouer un role: 50 % des patients sans anomalie du septum avaient un QI superieur a 70, contre 18% dans les cas ou existaient de telles anomalies (p Conclusions. - Une investigation soigneuse comprenant un examen en resonance magnetique nucleaire est necessaire pour rechercher des malformations cerebrales associees. Les patients doivent etre operes tot, idealement avant l'âge de 9 mois. La qualite de l'environnement sociofamilial doit etre optimisee.

From a series of 1,408 patients with craniosynostosis hospitalized between 1976 and 1994, 561 probands with non-syndromal isolated sagittal synostosis were analyzed. The prevalence of sagittal synostosis was estimated in the order of 1 in 5,000 children. Family information was obtained from 373 probands distributed among 366 families. The male:female ratio was 3.5:1. There was no maternal or paternal age effect. In 22 of the 366 pedigrees, a high degree of familial aggregation was observed, giving a 6% figure of familial cases. Segregation analysis of 253 families indicates that sagittal synostosis is transmitted as a dominant disorder with 38% penetrance and 72% of sporadic cases. The frequency of twinning was 4.8% with only 1 concordance for sagittal synostosis in a monozygotic twin pair. The possibility of a mechanical pathogenesis in sporadic cases is discussed.

Resume Le pronostic mental des scaphocephalies est encore controverse. La question de savoir si les indications chirurgicales sont d'ordre fonctionnel ou seulement esthetique reste discutee. Le but de ce travail est de tenter de clarifier ce probleme. Patients et methodes. — L'evolution intellectuelle de 396 enfants atteints de scaphocephalie a ete analysee et correlee a l'âge, la pression intracrânienne (PIC) et le devenir mental. Avant tout traitement, la PIC etait enregistree (systematiquement durant la premiere partie de l'etude); le niveau mental etait evalue initialement, et apres 5 ans d'evolution en moyenne. Les evolutions intellectuelles etaient comparees selon qu'une intervention etait pratiquee ou non. Resultats. — L'evolution intellectuelle des enfants etait normale dans la grande majorite des cas, avec ou sans intervention. Le niveau mental des enfants de moins de 1 an etait significativement plus souvent normal que celui des enfants de plus de 1 an (p Conclusions. — Dans cette serie, le niveau mental initial apparait comme le facteur pronostique principal du devenir mental. Dans la mesure ou le niveau mental final est le reflet du niveau initial, et ou enfants les plus âges ont statistiquement un niveau plus faible que les plus jeunes, on peut envisager l'indication de cette intervention a visee esthetique sous un angle preventif.

✓ Three hundred ninety-six children with scaphocephalies were prospectively studied to analyze the correlation between age, intracranial pressure (ICP), and mental function outcome. The ICP measurements and the early and late psychometric assessments were compared. The influence of surgery, when performed, was analyzed. In most cases, the mental function outcome of the patients was good whether or not they had undergone surgery. The mental level and the frequency of increased ICP both correlated with patient age. A correlation was found between the early and late psychometric assessments in all patients. Thus, the main predictive factor of mental function outcome appears to be the initial developmental level.

Solid coral blocks have been used as a bone graft substitute in clinical orthopedics for more than 12 years. In this study, 36 patients with 54 craniofacial osseous contour defects received subperiosteal augmentations with natural coral granules made chiefly of calcium carbonate. The patients were followed for 12 to 36 months. The postoperative results were satisfactory; there were only 5 sites of clinically evident resorption. There were 2 incidences of wound irritation and 1 instance of overt infection. Technical aspects of the procedure are discussed.

Summary The use of aerosolised fibrin glue has been evaluated in a group of 100 consecutive patients undergoing face lift procedures and compared to a similar group of 100 consecutive patients who had face lifts but received no fibrin glue. There was a statistically significant decrease in the rate of major haematoma formation and presentation of ecchymosis associated with the use of fibrin glue. The incidence of total complications was unchanged between the two groups, despite the fact that there were no drains or postoperative dressings used in the fibrin glue treated group. Patients found the omission of drains and dressings to be most convenient.

Intracranial volume and intracranial pressure have been measured in 66 children with craniosynostosis, 48 boys and 18 girls. The premature fusion of skull sutures is assumed to restrict skull growth and predispose to elevated intracranial pressure. Thirteen children (20 percent) had raised intracranial pressure and demonstrated a significant restriction of skull growth. In this series, volume measurement alone, however, did not serve as a reliable predictor that the intracranial pressure was raised.

UNLABELLED Scaphocephaly is the most frequent craniosynostosis. Many correction techniques have been proposed. Their relevance is not completely appreciated by means of the Cranial Index, which cannot differentiate specifically the anterior and posterior modifications.The aim of this study was to propose a new fronto-occipital morphologic evaluation technique and its validation in normal and scaphocephalic patients, operated or nonoperated. PATIENTS AND METHODS Two evaluation measures (indices) were developed for frontal and occipital deformities, traced over a standard skull radiograph. It was initially applied in 16 normal skull radiographs. Forty-two scaphocephalies were evaluated before and after linear perisutural craniectomy technique. Short- and long-term evaluations were performed in the 42 operated cases, with a follow-up period of 10 or more years.The created indices were named Frontal Sagittal Index (FSI) and Occipital Sagittal Index (OSI). They are proportional measures between 2 cranial lengths. RESULTS A significant difference was found for the FSI and the OSI between scaphocephalies and normal skulls. A significant difference was also found between indices before and after surgery, whether it was short- or long-term follow-up. No significant differences were found between the indices for the short- and long-term follow-up. CONCLUSIONS The FSI and the OSI were more precise measuring methods than the Cranial Index for the scaphocephaly anterior posterior morphologic evaluation and follow-up. There was also a stability of the results in the long-term follow-up with the technique used. We recommend these indices for the presurgical and result evaluation rather than the Cephalic Index.

Background: The purpose of this project was to determine whether adult patients with frontal deformities caused by untreated craniosynostosis can undergo successful surgical correction. Methods: Thirteen unoperated adult craniosynostosis patients are reported: seven women and six men, with a mean age of 24 years. There were four plagiocephalies, four scaphocephalies, three oxycephalies, and two brachycephalies. Computed tomographic scans with cerebral contrast were obtained, and the projected computerized correction was discussed with the patients. Frontocranial remodeling with an intracranial approach was used in 11 cases, and a frontal implant plus nasal en bloc realignment was used in two. Results: No significant perioperative complications occurred in this series. There were two late revisions for bony irregularities, through a limited approach. The morphologic correction was very satisfactory, emphasizing how important correct anatomy of the forehead and upper cranial vault is for the equilibrium of the face. Conclusions: Unoperated craniosynostosis creates a significant to severe disfigurement because of the frontal deformation. Therefore, only in mild cases can one propose a limited operation or an implant, avoiding an intracranial approach. In most cases, a radical intracranial approach is the only effective operation. Risks must be discussed with the patient, but the psychological impact of the disfigurement is so great that these young adults are usually willing to undergo a radical approach. Early operation in infancy is of course preferable, but if this was not performed for various reasons, it is still possible to correct the deformity, despite it being a much more complicated operation.

Both the safety and efficacy of the treatment of isolated craniofacial dysostosis (plagiocephaly) in infancy have been demonstrated. Opinions remain divided, however, as to the optimal type of procedure to be undertaken. In an attempt to answer this question, we have retrospectively evaluated a study population of 48 children operated on in infancy by either a unilateral or bilateral approach. Results of treatment at a minimal follow-up of 3 years were assessed based on preoperative and postoperative photographs and direct patient examination. Based on this retrospective comparison of the unilateral and bilateral approaches to the treatment of isolated craniofacial dysostosis, we conclude that (1) either approach as specifically outlined will give excellent results in the majority of patients, (2) there is no statistically significant difference in the results obtained by using either procedure, (3) in the majority of instances, less than ideal correction was manifested by contour irregularities evident in the temporal and/or lateral forehead region, and strict attention should be given to these areas in an attempt to further improve results, and (4) in those cases where significant protrusion is observed on the "normal side," a bilateral approach is preferable.

Background: Surgical treatment of faciocraniosynostosis is a complex problem that includes both function and aesthetics. Treatment goals are to prevent further neurologic disorders and to correct the morphologic impairment. Methods: Thirty-six patients with faciocraniosynostosis (mean age, 5.2 years) were evaluated prospectively after frontofacial monobloc advancement and quadruple internal distraction. Four distractors were used in combination with a frontofacial monobloc advancement osteotomy. Complications and advancement were evaluated clinically and radiographically. Respiratory status was evaluated by polysomnography. Relapse was evaluated by comparing results at the time of distractor removal to 6-month values. The mean follow-up was 30 months. Results: Distraction was completed in 35 patients. Twenty-eight patients (80 percent) completed their distraction uneventfully in the initial period. In seven patients, a problem related to the distraction devices required revision surgery, and subsequently six of them completed the distraction. One patient died the day after surgery from acute brain tonsillar herniation before distraction was begun. The exorbitism was corrected clinically in all patients in whom distraction was completed (n = 34). A class I occlusal relationship was obtained in 28 of 35 patients (80 percent). When respiratory impairment was present, it was corrected in all but two cases (14 of 16). A septic frontal osteonecrosis occurred in one patient 2 months after distraction was completed (frontal bone loss in one of 35 patients). Reossification at time of distractor removal was limited. Relapse has been observed predominantly at the occlusal level (six of 35) and more frequently in Pfeiffer syndrome. Conclusions: Internal distraction allows for early correction of respiratory impairment and exorbitism of faciocraniosynostosis, and limits the major risks of frontofacial monobloc advancement. Previous operations performed before the frontofacial monobloc advancement increased its morbidity.

According to the author, the short scar face lift seeks to eliminate the retroauricular scar by exerting a vertical pull on the cheek, but residual excess skin frequently remains below the ear lobe. More importantly, this vertical elevation of the cheek creates significant skin excess at the temporal level. To eliminate this excess, a precapillary temporal skin excision is usually performed. A potentially obvious temporal scar is thus created in a very visible part of the face. In this article, the author outlines his views on the drawbacks of the short scar face lift and advocates a different technique, using a vertical retroauricular incision, with the goal of avoiding the disadvantages of the short scar approach.

Monobloc frontofacial advancement with internal distraction osteogenesis is still a relatively new technique, and the literature on exorbitism correction of syndromal faciocraniosynostosis with monobloc advancement is scarce. Using a image processing software, we measured on axial computed tomographic scans the effect of monobloc advancement on the correction of exorbitism on a large series of patients (n=38) with syndromal faciocraniosynostosis who underwent surgery for correction of cranial and facial hypoplasia. A population reference of 23 patients without faciocraniosynostoses was also measured. For each orbit, globe protrusion was expressed as the ratio between the length of the globe that is above the interzygomatic line and eye size. The results indicate that monobloc has a symmetrical effect on both eyes that is correlated to the preoperative degree of exorbitism. Longitudinal data indicate that exorbitism correction changes with time. A mean preoperative globe protrusion of 0.86 dropped sharply to 0.54 at 6 months after distraction and increased to 0.61 at 16 months after distraction removal, a value that is not significantly different from the ratio of the control group (0.60). Overall, the data suggest that a certain degree of enophthalmos in the early postoperative period does not necessarily imply a sustained overcorrection.