Bilambdoid and sagittal synostosis: Report of 39 cases.

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.

The posterior coronal incision.

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.

Clinical spectrum and outcomes in families with coronal synostosis and TCF12 mutations.

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.

Spring-assisted posterior skull expansion without osteotomies.

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.

Posterior cranial vault expansion in the treatment of craniosynostosis. Comparison of current techniques.

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.

Posterior remodeling flap for posterior plagiocephaly.

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.

Anterior fronto-orbital remodeling for trigonocephay.

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.

Hypertelorism correction: what happens with growth? Evaluation of a series of 95 surgical cases.

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.

Cloverleaf skull deformity and hydrocephalus.

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).

Severe deformational plagiocephaly: long-term results of surgical treatment.

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.

Crouzon syndrome with acanthosis nigricans: a case-based update.

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.

Complex pediatric orbital fractures combined with traumatic brain injury: treatment and follow-up.

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.

The role of endoscopic third ventriculostomy in the treatment of hydrocephalus associated with faciocraniosynostosis.

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.

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.

Scaphocephaly part II: Secondary coronal synostosis after scaphocephalic surgical correction.

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).

Scaphocephaly: Part I: indices for scaphocephalic frontal and occipital morphology evaluation: long-term results.

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.

Cognitive and academic outcome after benign or malignant cerebellar tumor in children.

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.

Pediatric craniofacial osteosynthesis and distraction using an ultrasonic-assisted pinned resorbable system: a prospective report with a minimum 30 months' follow-up.

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.

Evolution in the frequency of nonsyndromic craniosynostosis.

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.

Focus session on the changing "epidemiology" of craniosynostosis (comparing two quinquennia: 1985-1989 and 2003-2007) and its impact on the daily clinical practice: a review from Necker Enfants Malades.

Introduction: The aim of this study was to evaluate the evolution in craniosynostosis in terms of incidence and management in the last 20 years and to discuss the clinical implications and future perspectives with regards to the practical organization of daily practice.

Methods: The relative incidence and management modalities of craniosynostosis hospitalized at the Craniofacial Unit of Necker, French National Referral Center for Faciocraniosynostosis, in two different quinquennia: 1985-1989 (group A) and 2003-2007 (group B) were reviewed.

Results: A total of 1,286 children were included in our study; group A 472, group B 814, that is an overall increase of 1.

The increase of metopic synostosis: a pan-European observation.

Metopic synostosis is thought to have an incidence of about 1 in 15,000 births. Traditionally, this makes it the third most frequent single-suture craniosynostosis, after scaphocephaly (1 in 4200-8500) and plagiocephaly (1 in 11,000). Our units have, independently from each other, noted a marked increase in the number of metopic synostosis over the recent years.

Unoperated craniosynostosis patients: correction in adulthood.

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.

Palpebral fissure changes after monobloc frontofacial advancement in faciocraniosynostosis.

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.