Synostosis of the lambdoid suture: a spectrum.

PURPOSE: Lambdoid synostosis (LS) is a rare condition, which is either isolated; associated with sagittal synostosis, the "Mercedes-Benz" syndrome (MBS); or with synostosis of the coronal sutures (oxycephalic form). In addition, LS is part of the phenotype of a growing number of genetic diseases. The nosology, pathophysiology, and management are controversial. We decided to review our experience with LS. METHODS: We reviewed retrospectively pediatric cases of LS proved on CT-scanner, isolated or associated with other conditions, followed in our craniofacial center during the last 15 years, regarding clinical presentation, anatomical lesions, syndromic associations, surgical management, and outcome. RESULTS: We reviewed 48 cases: 6 isolated LS, 22 MBS, and 20 oxycephalic. A syndromic context was present in 72% (up to 80% of oxycephalic cases), and faciostenosis was present in 23%, mostly oxycephalic cases (40%). Transverse sinus agenesis was found in 61% of documented patients. A total of 31% of children had a dystocic birth, up to 45% of MBS. Decompressive craniectomy or cranioplasty was needed in a majority of patients, often young infants, while posterior fossa decompression was mostly performed in older children. CONCLUSION: LS is rarely isolated and non syndromic; most cases are found in a wide spectrum of diseases, and LS is often associated with sagittal or coronal synostosis. Genetic evaluation is mandatory for LS; conversely, geneticists may require neurosurgical advice for LS in an increasing number of very rare diseases. The surgical management of LS should be tailored according to clinical presentation, age, and syndromic context.

[Suture simulator - Cleft palate surgery]. / Simulateur de suture ­ chirurgie des fentes vélopalatines.

INTRODUCTION: Cleft palate requires surgery in the first years of life, furthermore repairing anatomically the soft and hard palate is complex on a surgical level because of the fine tissues and the local intraoral configuration. It is valuable to train first on simulators before going to the operating room. However, there is no material dedicated to learning how to perform intraoral sutures in cleft palate surgery. We made one, in an artisanal manner, in order to practice before the real surgical gesture. BUILDING THE SIMULATOR: The simulator was designed based on precise anatomical data. A steel pipe, fixed on a rigid base represented the oral cavity. An adapted split spoon represented the palate. All pieces could be removed in order to apply a hydrocellular dressing before training for sutures. USE OF THE SIMULATOR: Our simulator was tested by 3 senior surgeons in our department in close to real-life conditions in order to evaluate its anatomical accuracy. CONCLUSION: It is valuable to have a simulator to train on cleft palate sutures within teaching university hospitals that manage this pathology. Our simulator has a very low cost, it is easy to make and is anatomically accurate.

[Rational use of distraction osteogenesis in craniofacial surgery]. / Utilisation rationnelle de la distraction osseuse en chirurgie craniofaciale.

Distraction osteogenesis, initially developed by Ilizarov for limb, is the tissular extension caused by the progressive space of the osseous pieces following an osteotomy. Distraction is osteogenesic and histogenic. Twenty-five years ago, at the instigation of McCarthy, this technique was used to handle the craniofacial malformations in the various floors of the face : mandibular, mediofacial and cranial. The most wide-spread protocols respect a latency period from 0 to 7 days, a rhythm of distraction from 1 to 2mm a day in 2 at 4 times and a period of consolidation from 4 to 8 weeks. Distraction is the result of the inventiveness of the pioneers then the work to always adapt to the multiple complex clinical situations. The surgeon has to choose between internal or external materials allowing a mono- or multi-vectorial extension, in osseous and/or dental anchoring. The mandibular distraction is very effective for the treatment of the secondary obstructive syndromes in the unilateral or bilateral severe hypomandibular malformations. She also allows desobstruction of the superior airways within the framework of the mediofacial hypoplasies as well as the secondary treatment of the growth defects in cleft lips and palates. Finally, the distraction osteogenesis enhanced reliability of the fronto-facial advancement in early and secondary treatment of craniofaciosynostosis. This is a real support of the facial growth, which has to be included in a plan of global treatment.

[Non syndromic craniosynostosis]. / Les craniosténoses non syndromiques.

Craniosynostosis are rare congenital malformations of the skull resulting from the premature fusion of one or several cranial sutures. Prevalence is considered in approximately 1 on 2000 births. Non syndromic craniosynostosis (NSC) or isolated form are the most frequent forms (85 % of the cases). They are classified most of the time according to the synostotic suture(s) and the engendered cranial deformation: sagittal synostosis or scaphocephaly, metopic synostosis or trigonocephaly, bicoronal synostosis or brachycephaly, coronal synostosis or plagiocephaly and oxycephaly. Although the multifactorial origin is commonly admitted, the precise mechanisms which lead to the premature fusion of a suture, remain incompletely resolute. The main risks are the intracranial high blood pressure and its consequences on the psychomotor development, the visual or respiratory infringement which can require a surgery in emergency. The treatment is realized by multidisciplinary teams allowing to provide a strategy adapted to every situation. The decision-making process depends on patient's age, on the type and severity of the craniosynostosis, and on the patient's health. This surgery is ideally performed before the age of 1 year and indication only in morphological purpose is widely recognized to avoid any social damage to the child. The follow-up is essential and is made throughout the growth in particular to detect a recurrence or the evolution towards a complex form of craniosynostosis.

[Hibernoma: an uncommon adipocytic tumor]. / L'hibernome : une tumeur adipocytaire rare et méconnue.

Hibernomas are uncommon tumors composed of brown fat. Their consistency is multiple but usually harder than a regular lipoma. It is a mobile, slow-growing mass. Clinical symptoms are exceptional (pain or massive weight loss). The case of a 26 years old patient who suffered of a cervical hibernoma is reported to discuss the clinical and paraclinical aspect of the diagnosis.

[History of skin graft]. / Une histoire de peau.

Skin graft is the most common and simple procedure to cover superficial defect. Skin of variable thickness and size is completely detached from its origin (donor site) to cover a defect (recipient site). This simple procedure is the result of a long and eventful technical and theoretical evolvement. The aim of this article is to re-trace the history of skin grafting, from its discovery until today.

[Nipple-areola complex reconstruction with full-thickness skin graft and subcutaneous pedicled island flap]. / Reconstruction de la plaque aréolomamelonnaire par un lambeau à pédicule sous-cutané associé à une greffe de peau totale.

We are reporting on an easy, reliable and reproducible nipple-areola complex reconstruction. The main feature is to perform a full-thickness skin graft to rebuild the areola and a subcutaneous pedicled island flap for the reconstruction of the nipple in the middle of the graft. Each step is described. This technique remains easily practical and allows to keep an appropriate nipple projection. Moreover, it fits to all kind of breast reconstruction.

[Sensory function of FAMM flap: a report of 15 cases]. / Etude de la sensibilité du lambeau de FAMM : à propos de 15 cas.

INTRODUCTION: The facial artery musculomucosal (FAMM) flap described by Pribaz in 1992 is a musculomucosa flap of the cheek plugged into the higher or lower facial pedicle artery. Indications in terms of substance losses in the oral cavity are numerous. PATIENTS AND METHODS: Twenty cases carried out from 2003 to 2006 at the Oscar-Lambret Neck Surgery Cancer Center are reported with a study first on record, then a study on 15 patients (three deaths, two flaps take away for local recurrence). The dimensions of the flap, its epicritic sensitivity, its sensitivity to hot and cold temperatures, the Semmes-Weinstein test and a key test of pique were analyzed. A neck dissection was most often done in the cases studied, and did not advise against the flap. RESULTS: The main indication was represented by tumors of the floor of the mouth. Individual susceptibility testing were broadly similar to the flap and on the opposite side healthy. The results seem to show that, as with skin flaps, there is a complete recovery of sensation. CONCLUSION: This flap is very reliable and most often resensitizes. The low ligation of the artery during neck dissection does not jeopardize the survival (rich regional anastomoses). The FAMM flap makes up for the loss of medium size substance, it represents a very good solution between directed healing and more consistent skin flaps.

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. To evaluate the results a score was computed for each patient by assessing the length of the palpebral fissure, the depth of the conjunctival fornix, and local complications together with an evaluation of the satisfaction of patients and their families. Forty-four children were included (27 boys and 17 girls). Twenty-seven had unilateral microphthalmia (61%) and 17 bilateral microphthalmia (39%). Twelve patients were lost to follow up. The mean duration of follow-up was 12 years (range 4-21). Management involved an ocular conformer in only 31 patients (71%). The treatment was deemed satisfactory in all except 10 children. Surgical treatment with orbital expansion permitted good symmetry of the orbital cavities with a final mean difference of 9% (range 3-17) compared with the initial 16.8% (range 13.6-20.3). The prosthetic treatment gives satisfactory results. Despite limited indications and difficult follow-up, our experience emphasises the value of surgical treatment for severe micro-orbitism.