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BACKGROUND: Bilambdoid and sagittal synostosis (BLSS), also called "Mercedes Benz synostosis," is a multisutural craniosynostosis that has been described as a specific entity. However, this synostotic pattern can also be found in syndromic craniostenosis. To better define this entity we reviewed our experience with bilambdoid and sagittal synostosis. METHODS: We searched our prospective database for cases of bilambdoid and sagittal synostosis among all types of craniosynostosis. Two groups were distinguished - patients with isolated BLSS and the group of syndromic craniostenosis for whom BLSS was observed at initial presentation. We reviewed the clinical findings, associated diseases, and their management specifically for isolated BLSS patients. RESULTS: Thirty-nine patients were diagnosed with bilambdoid and sagittal synostosis among 4250 cases of craniosynostosis treated in our department over a period of 42 years. Among them, 8 were finally diagnosed as Crouzon syndrome. Of the 31 patients identified with isolated bilambdoid and sagittal synostosis, 25 (81%) were males and 6 (19%) were females. The average age at diagnosis was 17 months. At diagnosis, 16% of the population presented with papillary edema and 58% posterior digitate impressions. Two types of craniofacial dysmorphy were observed - a pattern with narrow occiput (71% of cases) and a pattern with dolichocephaly (29% of cases). Cerebellar tonsillar herniation was the most frequently associated malformation (61% of the isolated BLSS). Surgical management evolved during the years, and several surgical techniques were used to treat patients with BLSS, including isolated biparietal vault remodeling, posterior vault remodelling, and posterior vault expansion with internal or external distraction. In some cases, a craniocervical junction decompression was also performed. The mean follow-up was 82 months (7 years). The overall mental development was within normal limits in most children, but a mental delay was found in 25%. CONCLUSION: Bilambdoid and sagittal synostosis constitute an isolated entity in almost 80% of the cases, whereas in the remaining 20% it is part of a faciocraniosynostosis syndrome. Two phenotypes may be found. Early surgical management is indicated, and several techniques can be used in this heterogeneous population. A cerebellar tonsillar prolapse is present in a majority of cases.

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BACKGROUND: The coronal incision is a standard surgical approach in craniofacial surgery. It has undergone many modifications during the years in an attempt to optimize the esthetic appearance of the scar, including the sawtooth "stealth incision" and the sinusoidal incision. METHODS: We describe an alternative coronal approach extending posteriorly from the postauricular region over the occiput, resulting in an axial scar. RESULTS AND DISCUSSION: The posterior coronal incision provides equivalent exposure of the craniofacial skeleton while placing the scar in an esthetically optimal location that is much more likely to be camouflaged by hair, especially in patients with thinning hair or male-pattern baldness. It avoids a vertical temporal scar that is prone to widening and also allows the incision to be placed remotely from any neurosurgical hardware in the frontotemporal region. It may be used in craniofacial or neurosurgical procedures requiring access to the posterior or anterior cranial vaults or the upper craniofacial skeleton down to the maxillary alveolar rim.

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TCF12 mutations have been reported very recently in coronal synostosis. We report several cases of familial coronal synostosis among four families harbouring novel TCF12 mutations. We observed a broad interfamilial phenotypic spectrum with features overlapping with the Saethre-Chotzen syndrome. TCF12 molecular testing should be considered in patients with unilateral- or bilateral-coronal synostosis associated or not with syndactyly, after having excluded mutations in the TWIST1 gene and the p.Pro250Arg mutation in FGFR3.

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INTRODUCTION: 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 hollowing PURPOSE: The 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.

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INTRODUCTION: Nonsynostotic posterior plagiocephaly has become the most common skull deformation since pediatricians have suggested the supine position for the newborns to reduce the risk of sudden death. Prevention of such a "positional" deformation or its management once it has occurred is mainly based on physical maneuvers such as physiotherapy and active positional corrective measures. SELECTION CRITERIA: Surgical correction, however, may be suggested in rare cases where deformation of the skull is so severe or the referral of the child is so late that physical corrective treatment cannot be taken into consideration. Surgical management is based on the creation of a posterior bone flap to be repositioned after the opportune contouring and rotation. PURPOSE: The aim of this paper is to describe the surgical technique used for posterior vault remodeling in posterior plagiocephaly at the craniofacial unit of Hopital Necker Enfants Malades (French National Referral Center for Faciocraniosynostosis) focusing on its advantages and limitations.

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INTRODUCTION: Surgical procedures which aim at expanding the posterior cranial vault have come to play an increasingly important role in the treatment of syndromic craniosynostosis. The posterior cranial vault may be expanded by formal cranioplasty or by less invasive methods based on gradual posterior cranial vault expansion or distraction. These procedures share the common goals to prevent intracranial hypertension and progression toward turricephaly by effectively increasing the intracranial volume. They also reduce any local brain compression within the posterior cranial fossa. PATIENTS AND METHODS: This review describes the various techniques available for less invasive and gradual posterior cranial vault expansion and their rationale based on our experience on the last 40 cases. Volumetric data for three different surgical techniques have been compared (two cases of posterior cranial vault expansion by free-floating parieto-occipital bone flap, two with springs, and two with internal distractors). The volumetric analysis showed an expansion of 13 and 24 % for the free posterior flap, 18 and 25 % for the translambdoid springs, and 22 and 29 % for the distractors. DISCUSSION: Although these figures are not strictly comparable, our data suggest that these three techniques offer a potential for a significant increase in skull volume. The indications for such procedures must, however, be tailored according to the age and condition of the single patient.

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INTRODUCTION: A posterior flatness of the skull vault can be observed in infants with brachycephaly. Such posterior deformation favours the development of turricephaly which is difficult to correct. To reduce the risk of such deformation, an early posterior skull remodelling has been suggested. Translambdoid springs can be used to allow for a distraction through the patent lambdoid sutures and obtain a progressive increase of the posterior skull volume. SURGICAL TECHNIQUE: The procedure consists in a posterior scalp elevation, the patient being on a prone position. Springs made of stainless steel wire (1.5 mm in diameter) are bent in a U-type fashion, and strategically positioned across both lambdoid sutures. No drilling is usually necessary, as the lambdoid suture can be gently forced with a subperiosteal elevator in its middle and an indentation can be created with a bony rongeur on each side of the open suture to allow for a self-retention of bayonet-shaped extremity of the spring. Careful attention is addressed to the favoured prone position during the post-operative period. After a delay of 3-6 months, the springs can be removed during a second uneventful procedure, with limited incisions, usually as a preliminary step of the subsequent frontal remodelling. CONCLUSION: The concept of spring-assisted expansion across patent sutures under 6 months of age was confirmed in our experience (19 cases). Insertion of the springs allowed for immediate distraction across the suture. A posterior remodelling of the skull could be achieved with minimal morbidity allowing to delay safely a radical anterior surgery.

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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.

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OBJECTIVE: This article describes the clinical aspects for both operated and non-operated patients with a cloverleaf skull deformity treated in our service, focusing on hydrocephalus. METHODS: We describe 13 cases of cloverleaf skull deformity treated in our services between 1977 and 2008. Among them, ten were operated (9 out of 13 for the craniofacial stenosis and 7 out of 13 for hydrocephalus). RESULTS: Hydrocephalus was present in all patients with bilateral lambdoid stenosis. There was no case of hydrocephalus among the patients with unilateral or absent lambdoid stenosis. Associated malformations and severe faciostenosis were associated with higher mortality and morbidity. CONCLUSION: The development of hydrocephalus seems to be closely related to a bilateral lambdoid stenosis. The optimal treatment must be tailored individually considering the degree of the malformation and the presence of complications and comorbidities.

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INTRODUCTION: Deformational plagiocephaly (DP), a benign infantile condition, is often thought to improve spontaneously but can in fact lead to permanent skull asymmetry if left untreated. Deformational plagiocephaly can be completely corrected by nonsurgical treatments if recognized in the first year of life. Nevertheless, efficacy of these treatments drastically diminishes after 9 months as the calvaria thickens. Beyond 15 months, if the asymmetry is severe, surgical remodeling becomes an option. In this study, we analyzed the surgical morbidities and the morphologic outcome of surgical remodeling in DP. METHODS: This study was designed as a retrospective analysis of a craniofacial database combined with an outcome study. Between January 1992 and August 2006, 2363 occipital DP cases were treated by repositioning. Thirty patients were operated on, always after the age of 15 months (1.23%). Mean (SD) age at the time of the first clinic was 19.2 (± 5.8) months (range, 11-34 mo). A switch cranioplasty (A) was first used, progressively replaced since 1995 by a large occipital monobloc graft rotated 180 degrees and fixed with tongue-in-groove pillars (B). Morbidities were extracted from the database. Long-term morphologic outcomes were evaluated during a free clinic visit with a 4-point Likert-type outcome scale. RESULTS: Mean (SD) age at the time of surgery was 20.2 (± 5.6) months. Incidents (dural tears or venous sinus breaches) occurred in 28% (A) and 36% (B) or patients. One complication required repeat operation (extradural hematoma). No cerebrospinal fluid leaks and/or meningitis were recorded. Mean (SD) hospital stay was 5.3 (± 0.95) days. There was no statistical difference in the long-term morphologic outcome between the 2 groups (median of group A being "good but still visible"; median of group B being "excellent, unnoticeable"). CONCLUSIONS: Surgical correction of DP is a potentially life-threatening procedure, which should be indicated only in very severe cases, after exhaustion of all other nonsurgical methods, in a reference craniofacial center combining pediatric neurosurgery and plastic surgery.

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BACKGROUND: Crouzon syndrome with acanthosis nigricans also named Crouzono-dermo-skeletal is a clinically and genetically distinct entity. It associates a craniofacial phenotype to anomalies of the skin and long bones. This syndrome is due to a specific mutation in FGFR3 gene that can be identified by genetic testing. ILLUSTRATIVE CASES: As in our two patients, not all these features might be present and some will be patent only in the infancy or early childhood. Moreover, other organs such as the kidneys might be affected. DISCUSSION: Because several organs might be affected the recognition of such syndrome is important for a correct management of the patient as well as a proper information and genetic counseling of the families.

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OBJECT: The aim of this study was to assess the efficacy of endoscopic third ventriculostomy (ETV) in the treatment of hydrocephalus in children with complex craniosynostosis. METHODS: Medical charts from 2194 children treated at the Craniofacial Unit of Necker Enfants Malades between 1995 and 2008 were reviewed. Among them, 293 were affected by a syndromic faciocraniosynostosis. The management of cases with hydrocephalus treated using ETV was examined and analyzed with regard to successes and failures. RESULTS: Forty-six children with associated progressive hydrocephalus were identified, and 11 (8 boys, 3 girls) had undergone an ETV. All were affected by a faciocraniosynostosis (Crouzon syndrome in 5, Pfeiffer syndrome in 3, and unclassifiable in 3). A descent of the cerebellar tonsils was found in 5 children. In 7 children the hydrocephalus was controlled by an ETV. In the remaining 4 cases a ventriculoperitoneal shunt was also needed. The delay between the 2 surgeries ranged from 1 week to 6 months in these 4 patients. The follow-up period ranged from 11 months to 13 years. CONCLUSIONS: Performing an ETV may facilitate control of hydrocephalus associated with faciocraniosynostosis in select cases. However, because of the relatively high failure rate of this procedure, a close clinical and radiological monitoring of these children is mandatory.

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The treatment of orbital fractures aims at the restoration of orbital anatomy and prevention of posttraumatic sequels. The treatment of facial fractures in patients with traumatic brain injury may necessitate a postponement of fracture surgery to allow for brain recovery. However, such delay of reconstruction in complex orbital fractures may lead to inferior results. Fourteen pediatric patients with complex orbital fractures were retrospectively reviewed. Eleven patients (79%) had brain injury, with a mean Glasgow Coma Scale score of 8.6 at initial evaluation. Fracture patterns were highly complex with many bilateral and multiple orbital wall fractures. Six patients (43%) had injuries to the medial canthal tendon, as part of a naso-orbito-ethmoid fracture. Mean follow-up time was 4.7 years (range, 1.2-13.1 years). Eleven patients (79%) had reconstructive surgery early (within 3 weeks), and 3 (29%) patients had the surgery postponed. Four patients had secondary surgeries for enophthalmos and medial canthal dislocation. Eighty-two percent of patients with brain injury had a good overall neurologic outcome (Glasgow Outcome Scale score of 1 or 2). Sixty-seven percent of patients had a good aesthetic outcome without any need for revisional surgery. Medial canthal injury and late surgery both correlated with an inferior aesthetic result. In conclusion, good results with respect to mental outcome and aesthetics can be achieved in highly complex orbital fractures combined with brain injury. Delayed surgery and medial canthal tendon injury predispose for posttraumatic sequels and secondary surgery.

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OBJECTIVE: To examine the impact of malignancy and location of the cerebellar tumor on motor, cognitive, and psychologic outcome. BACKGROUND: Although many studies focus on long-term outcome after cerebellar tumor treatment in childhood, the impact of its precise location remains unclear. PATIENTS AND METHODS: Children, aged from 6 to 13 years, with a cerebellar malignant tumor (MT; MT group, n=20) or a cerebellar benign tumor (BT; BT group, n=19) were examined at least 6 months after the end of treatment using the international cooperative ataxia rating scale, the Purdue pegboard for manual skill assessment and the age-adapted Weschler scale. Structural changes in brain anatomy were evaluated and parents and teachers answered 2 independent questionnaires. RESULTS: Parents and teachers reported high rate of learning and academic difficulties, but without any difference with respect to the type of tumor. However, children with cerebellar MT showed increased cognitive and motor difficulties compared with children with cerebellar BT. Cerebellar signs at clinical examination and manual skill impairment were strongly associated with cognitive difficulties. Both motor and cognitive impairments were found to be associated with extension of the lesion to the dentate nuclei. CONCLUSIONS: Dentate nuclei lesions are major risk factors of motor and cognitive impairments in both cerebellar BT and MT.

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Resorbable osteosynthesis is an important tool in pediatric craniofacial surgery. A prospective clinical study was carried out to evaluate the Sonic Welding resorbable osteosynthesis system. Twenty pediatric patients with craniosynostosis were operated on for craniofacial reconstruction. The techniques used were the same than usual (fronto-orbital remodeling or advancement). During the process of osteosynthesis, similar resorbable miniplates were used, but for fixation, only 2 steps were necessary (drilling and welding), tapping being unnecessary. Clinically, the hold of the pins in the bone seemed stronger, and less-than-usual osteosynthesis materials were necessary. Clearly, the pins were able to hold in a very thin bone in which no screws could hold. Subjectively, the satisfaction of the surgeon was greater owing to the avoidance of the tapping step. On follow-up, resorption took place with an initial swelling effect, like with another pure polylactic acid material. The good resistance of pins suggests that, in such a system, the resorbable plate becomes the weak point.

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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.

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AIM: Occurrence of secondary coronal synostosis (SCS) and its functional consequences were retrospectively analyzed in sagittal synostosis. PATIENTS AND METHODS: Occurrence of SCS and/or fingerprinting and clinical signs of raised intracranial pressure were investigated in children with scaphocephaly with a minimum follow-up of 3 years. Children were divided in 4 groups according to surgery (group 1: "H" craniectomy [193 patients]; group 2: craniectomies with removal of the coronal sutures [24 children]; group 3:"H" craniectomies and flap transpositions without total removal of the coronal suture [36 patients]; and group 4: 253 nonsurgical patients with scaphocephaly). RESULTS: Among group 1, 20 (10.4%) developed SCS (3 mo to 6 y postoperatively). Fingerprinting was generalized in 13 patients, localized in 5, and absent in 2. Headaches were present in 8 patients. Papilledema appeared during follow-up in 2 of them. Intracranial pressure that was first recorded as normal became elevated, and they both required a surgical decompression. In group 2, none developed an SCS. In group 3, 11% developed SCS. In group 4, 1.2% nonsurgical patients with scaphocephaly presented with an SCS. However, group 4 corresponds to less severe morphologic deformation and/or parents refusing surgery. CONCLUSIONS: Secondary coronal synostosis appears to occur with an approximate 10% incidence after craniectomies not involving the coronal sutures, with 1% requiring surgical decompression. Patients with milder forms of scaphocephaly who were not operated on (1.2%) rarely developed an SCS. Long-term follow-up should be undertaken even in the milder forms for possible recurrence of secondary synostosis.

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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.

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OBJECT: The prevalence of the different subtypes of craniosynostosis varies greatly. The aim of this study was to analyze the prevalences of the different subtypes of craniosynostosis at a single major craniofacial center and their changes during a 20-year period. METHODS: The medical charts of 2808 children hospitalized between 1988 and 2007 for a true craniosynostosis were retrospectively reviewed. Patients were divided according to their subtype of craniosynostosis (sagittal, plagiocephaly, brachycephaly, metopic, oxycephaly, syndromic cases, or unclassifiable). RESULTS: The total number of patients according to their craniosynostosis subtype were as follows: 1224 cases of scaphocephaly, 315 cases of plagiocephaly, 598 cases of trigonocephaly, 105 cases of brachycephaly, 69 cases of oxycephaly, 396 syndromic cases, and 101 unclassifiable cases. The prevalences of these craniosynostoses evolved differently over time. The number of children hospitalized each year increased over time from 106 to 181 cases, but this increase varied according to the subtype of craniosynostosis. Cases of scaphocephaly, plagiocephaly, brachycephaly, and syndromic cases increased similarly (170% increase on average), whereas trigonocephalies dramatically increased (420% increase). CONCLUSIONS: The evolution observed in the prevalence of craniosynostosis might result from several factors. An increased recruitment of patients at the center and an improvement in the diagnosis of craniosynostosis might explain the overall increase. However, other mechanisms should be examined to explain the great increase in the number of cases of trigonocephaly (environmental or pharmacological).

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