Reconstructive management of a patient with Tessier number 10 cleft.

Tessier number 10 cleft is one of the rarest facial clefts. Surgical treatment of this type of cleft is challenging due to the complexity of periorbital and temporal soft tissue deformities. A 23-year-old male patient presented with typical facial deformities of Tessier number 10 cleft.

Management of Hypertelorism.

Aim: To summarize the experience of the author with the treatment of hypertelorism.

Settings And Design: The author has been heading a high-caseload department of craniofacial surgery for 38 years; the research is based on his experience with this pathology by this time.

Materials And Methods: The charts of 38 patients were used for this research.

Crouzon Syndrome Anatomy, Usefulness of Vestibular Orientation.

Spatial resolution of computerized tomographic scanner has reached a level to which accurate anatomic measurements could be done in. Three-dimensional accurate measurements require a reliable referential system. In craniology landmarks are usually selected in the skull base.

Tessier's Cleft Number 6 Revisited: A Series of 26 new Cases and Literature Review of 44.

Objective: To fix a gray zone left in Tessier's classification of rare clefts with cleft 6 and to give a more comprehensive description of cleft 6 anatomy.

Design: The material used for the research was a series of 26 clinical cases of patients with assessed cleft 6 and 44 cases found out of a literature review with enough data to be useful. The 70 cases were cross-examined by the authors.

Crouzon syndrome: posterior fossa volume studies in vestibular orientation.

Introduction: With the increasing possibilities of surgical treatment addressing the complex spectrum of defects in Crouzon syndrome (CS), it is of utmost importance to obtain accurate assessment of the malformation. Evaluating the volume of the posterior fossa is of great importance since many authors, considering that its volume is always decreased, favor posterior fossa enlargement as a first step in the treatment of CS.

Material And Methods: We studied CT scans of children with CS, which were vestibular-oriented (VO) in order to conform with physiological landmarks, and we measured intracranial volume by manual segmentation; these abnormal CT scans were superposed with VO 3D-CT scanners of age-matched controls.

Three-dimensional study of 31 cases of synostotic anterior plagiocephaly before and after surgical management the Lille protocol.

Synostotic anterior plagiocephaly is a rare pathological cranial malformation. Therapeutic options are rarely studied due to the rarity of the malformation and difficulties in diagnosis and care management. The objective of this study was to analyze the results obtained with the Lille protocol based on 62 CT-scans done before and after surgery in 31 patients.

Prevention of Tartrate Crystallization in Wine by Hydrocolloids: The Mechanism Studied by Dynamic Light Scattering.

Young wines are supersaturated in potassium bitartrate, which induces rather uncontrolled crystallization processes. Delayed crystallization may occur even after bottling of the young wines, which is undesirable because it gives rise to a visual defect in the wine. Colloids such as mannoproteins, metatartaric acid, and carboxymethylcellulose are available on the market and may be added to delay crystallization.

Amniotic constriction band: a report of two cases with unique clinical presentations.

Amniotic constriction band is a rare clinical entity with varied manifestations that range from a combination of congenital malformations to isolated malformations that are unique to each patient. The etiology of this entity remains unknown. Herein, we highlight two cases of amniotic constriction band that presented to our unit with unique clinical characteristics.

Internal and external spheno-orbital meningioma varieties: different outcomes and prognoses.

Background: Internal variation among spheno-orbital meningiomas (SOM) is surgically challenging. Optic canal invasion management is discussed.

Method: This retrospective study includes 70 patients with SOM who underwent surgery between 1995 and 2012.

Frequency and socio-psychological impact of taunting in school-age patients with cleft lip-palate surgical repair.

Objectives: Cleft lip-palate (CLP) is a "social" pathology because of its impact on the child's facial appearance and speech. School is the first place where children are confronted to others and when they start socializing. Taunting and bullying are common and their psychological impact remains hard to assess.

Fifteen years of experience with the midfacial distraction without maxillary osteotomy protocol.

Purpose: Midfacial distraction for facial stenosis is minimizing the communication between cranial fossa and nasal fossa caused by the Le Fort III osteotomy during frontofacial advancement procedures. There are different types of distractors, such as internal and external devices. The aim of our study is to present a series of 22 consecutive distraction cases operated without any Le Fort osteotomy with external distraction frames.

Mutations in endothelin 1 cause recessive auriculocondylar syndrome and dominant isolated question-mark ears.

Auriculocondylar syndrome (ACS) is a rare craniofacial disorder with mandibular hypoplasia and question-mark ears (QMEs) as major features. QMEs, consisting of a specific defect at the lobe-helix junction, can also occur as an isolated anomaly. Studies in animal models have indicated the essential role of endothelin 1 (EDN1) signaling through the endothelin receptor type A (EDNRA) in patterning the mandibular portion of the first pharyngeal arch.

Trigonocephaly: Lille's surgical technique.

From the early beginning, in 1977, of our experience in the treatment of trigonocephaly and with the follow-up of 263 patients, we have been convinced that it was a condition related to the skull base. It is now proved thank to the comparison between patient's CT scan and normal template (normal skull of the same age) in vestibular orientation. We used to correct this condition by a frontal reshaping and a rotation of the superior and lateral orbital rim along an oblique axis.

Heterogeneity of mutational mechanisms and modes of inheritance in auriculocondylar syndrome.

Background: Auriculocondylar syndrome (ACS) is a rare craniofacial disorder consisting of micrognathia, mandibular condyle hypoplasia and a specific malformation of the ear at the junction between the lobe and helix. Missense heterozygous mutations in the phospholipase C, β 4 (PLCB4) and guanine nucleotide binding protein (G protein), α inhibiting activity polypeptide 3 (GNAI3) genes have recently been identified in ACS patients by exome sequencing. These genes are predicted to function within the G protein-coupled endothelin receptor pathway during craniofacial development.

Aplasia cutis congenita: review of 29 cases and proposal of a therapeutic strategy.

Introduction: Aplasia cutis congenita (ACC) is a rare congenital disorder, which most commonly involves the scalp, and can affect the galea, the pericranium, the bone, and the dura mater. ACC thus is at risk of infection and hemorrhage. There is no consensus over the ideal management and the role for plastic surgery.

Non-syndromic oxycephaly and brachycephaly: a review.

Background: Non-syndromic coronal synostoses oxycephaly and brachycephaly (NSCSOB) are rare. Their natural history, surgical management, and outcome are debated.

Material And Methods: We reviewed the available literature on NSCSOB.

Unicoronal synostotic plagiocephaly: surgical correction: Lille's technique.

Introduction: For 35 years, we have a tight neuro-plastic surgical cooperation for the surgical correction and long-term systematic follow-up of 125 cases of unicoronal synostotic plagiocephaly.

Methods: We have tried to understand why some patients had kept an asymmetrical facial growth pattern in spite of a good fronto orbital correction. Analysis in vestibular orientation which was available from 1993 has demonstrated a discrepancy between the ocular and the vestibular verticality referential system.

Atypical scaphocephaly: a review.

Background And Purpose: Sagittal craniosynostosis (SCS) is common and easily recognized and corrected surgically. However, rare cases of SCS are more complex: these associate closure of the metopic or delayed closure of the coronal suture, uni- or bilaterally.

Material And Methods: We reviewed the available literature on atypical sagittal craniosynostosis (ASCS).

Relational development in children with cleft lip and palate: influence of the waiting period prior to the first surgical intervention and parental psychological perceptions of the abnormality.

Background: The birth of a child with a cleft lip, whether or not in association with a cleft palate, is a traumatic event for parents. This prospective, multidisciplinary and multi-centre study aims to explore the perceptions and feelings of parents in the year following the birth of their child, and to analyse parent-child relationships. Four inclusion centres have been selected, differing as to the date of the first surgical intervention, between birth and six months.

Surgical and prosthetic treatment for microphthalmia syndromes.

Our aim was to evaluate the long-term outcomes of prosthetic treatment and orbital expansion in the management of microphthalmia syndromes. We did a retrospective single-centre study of all cases of microphthalmia treated between 1989 and 2010. The patients were divided into three groups: isolated microphthalmia, microphthalmia associated with micro-orbitism, and complex microphthalmia syndrome.

Midfacial distraction without osteotomy using a transfacial pin and external devices.

Introduction: During the 1970s, frontofacial advancement revolutionized the treatment of severe facial stenosis. Unfortunately, this method was associated with significant morbidity due to the Le Fort III osteotomy, which creates a major communication between the frontocranial dead space and the nasal fossae. Midfacial distraction improves the complication rate by diminishing the size of this gap.