Cone Beam-CT-Based Bone Volume Assessments of Alveolar Synthetic Bone Graft GlassBONE™ in Cleft Lip and Palate Patients: A Retrospective Study.

Background: Clefts of the lip and palate (CLP) are facial deformities that require multiple surgical procedures during childhood. One of these steps consists of filling the alveolar space with bone graft, traditionally removed from the iliac crest. However, this procedure could be invasive in children. Aim: Here, we aimed to evaluate the outcomes of GlassBONE™ graft, a bioactive glass used as a bone substitute, as an alternative to the deleterious autologous bone graft in children. Materials & methods: Retrospective monocentric study with 17 children aged 7.5 ± 2.2 yo [3.8-13.3 yo] carrying CLP. This technique has been established at La Timone Children hospital (Assistance Publique - Hôpitaux de Marseille) since 2011. Clinical (scar, graft rejection and periodontal status) and radiological (both panoramic radiographs and cone beam-CT) follow-up was conducted one year after the graft. The primary outcome was the reduction of the cleft volume, and secondary was the eruption of the adjacent tooth through the graft. Results: GlassBONE™ permitted a significant reduction in the cleft volume by 42.4 ± 27.7% [0.6-81.1%] (p < 0.0001), corresponding to a filling of 57.6 ± 27.7% of the alveolar cleft. GlassBONE™ is well tolerated, ensuring satifactory clinical results (improvement in both scar and periodontal coverage), as well as the physiological evolution of the germs through the biomaterial. GlassBONE™ appears particularly suitable for small volumes, and we were able to determine a minimum volume of approximtely 0.259 + / - 0.155 cc required for a successful bone fusion. Conclusion: The bioactive glass GlassBONE™ could be safely used in children with small CLP cases, providing satisfactory clinical and radiological results.

A Comparative In Vitro and In Vivo Study of Osteogenicity by Using Two Biomaterials and Two Human Mesenchymal Stem Cell Subtypes.

Background: Bone repair induced by stem cells and biomaterials may represent an alternative to autologous bone grafting. Mesenchymal stromal/stem cells (MSCs), easily accessible in every human, are prototypical cells that can be tested, alone or with a biomaterial, for creating new osteoblasts. The aim of this study was to compare the efficiency of two biomaterials-biphasic calcium phosphate (BCP) and bioactive glass (BG)-when loaded with either adult bone marrow mesenchymal stem cells (BMMSCs) or newborn nasal ecto-mesenchymal stem cells (NE-MSCs), the latter being collected for further repair of lip cleft-associated bone loss. Materials and Methods: BMMSCs were collected from two adults and NE-MSCs from two newborn infants. An in vitro study was performed in order to determine the best experimental conditions for adhesion, viability, proliferation and osteoblastic differentiation on BCP or BG granules. Bone-associated morphological changes and gene expression modifications were quantified using histological and molecular techniques. The in vivo study was based on the subcutaneous implantation in nude mice of the biomaterials, loaded or not with one of the two cell types. Eight weeks after, bone formation was assessed using histological and electron microscopy techniques. Results: Both cell types-BMMSC and NE-MSC-display the typical stem cell surface markers-CD73+, CD90+, CD105+, nestin - and exhibit the MSC-associated osteogenic, chondrogenic and adipogenic multipotency. NE-MSCs produce less collagen and alkaline phosphatase than BMMSCs. At the transcript level, NE-MSCs express more abundantly three genes coding for bone sialoprotein, osteocalcin and osteopontin while BMMSCs produce extra copies of RunX2. BMMSCs and NE-MSCs adhere and survive on BCP and BG. In vivo experiments reveal that bone formation is only observed with BMMSCs transplanted on BCP biomaterial. Conclusion: Although belonging to the same superfamily of mesenchymal stem cells, BMMSCs and NE-MSCs exhibit striking differences, in vitro and in vivo. For future clinical applications, the association of BMMSCs with BCP biomaterial seems to be the most promising.

A child dural-based occipital cavernoma with an accessory venous sinus.

BACKGROUND: The diagnosis of extra-axial cavernoma and surgical decision-making can be difficult on children. REPORTED CASE: In this report we present the case of a 33-month-old child for whom the work up done for asthenia, anorexia and psychomotor regression reveals a well-defined occipital extra-axial lesion. A follow up MRI was performed two months later, revealing an increase in the size of the lesion associated with hemorrhagic changes. The surgical decision is performed after an arteriography; it allows the total removal of the lesion contained in a duplication of the dura mater, by placing a vascular clip on an accessory venous sinus draining the lesion, respecting the integrity of the lateral sinus. Histology confirms a cavernoma. No complication resulted from the surgical procedure. This atypical case of pediatric dural cavernoma associated with a well-defined accessory venous sinus in arteriography is to our knowledge the first description in the literature. CONCLUSION: Extra-axial cavernomas have a misleading presentation. The management of these lesions is of twofold interests: to avoid a repercussion on the development of these children, and to obtain histological confirmation.

Upward vectors for osteogenic distraction treatment in secondary chronic intracranial hypertension in children undergoing scaphocephaly surgery: 2 cases reported.

The occurrence of secondary synostosis of coronal sutures at distance from H-craniectomy surgery for scaphocephaly concerns about 10% of children. Intracranial hypertension in these children remains exceptional but generally requires a surgical reoperation. Two children aged 3 and 5- months- old had been operated for scaphocephaly by H-craniectomy in two different hospital centers. Their clinical follow-up described a partial persistence of dolichocephalic deformity and an impression of parietal stenosis. During their growth, chronic headaches appeared with a complaint expressed at the ages of 4 and 5 years. In both cases, ophthalmic examination revealed significant bilateral papillary edema without loss of visual acuity. The imaging assessment (CT-scan and MRI) showed the absence of Chiari malformation and venous abnormality. For both, there was a compression image of the parietal lobes in relation to the persistence of a temporoparietal synostosis. An osteogenic parietal distraction permitted a volumetric brain expansion consecutive to the skull and meninges remodeling in only 6 months, associated with a leap forward acquisition, a normalization of the ophthalmic examination, and a complete loss of headaches. In conclusion, this new approach could be used in the case of chronic intracranial hypertension consecutive to a secondary parietal synostosis after a scaphocephaly surgery.

C1-C2 type Harms internal fixation for unstable C2 fracture in a 6-year-old boy: Case report.

BACKGROUND: Decision-making is often difficult in odontoid fracture in children. CASE REPORT: We present the case of a 6-year-old boy who sustained cervical trauma on falling out of a tree. Initial cervical X-ray and CT-scan did not find any traumatic lesion. Three-week check-up revealed an unstable C2 fracture in the synchondrosis at the base of the odontoid bone, with anterior displacement (type IC on the classification of Hosalkar et al.), without neurological symptoms except for cervical pain and limitation of head rotation. MRI confirmed the absence of medullary lesion. The Harms technique was used to fix C1 and C2, using adult instrumentation without bone graft. Bone fusion was obtained at 8 months. Hardware was removed at 10 months. No complications were reported. CONCLUSIONS: Posterior internal fixation for unstable C2 fractures in children can be effective and relatively safe.

Management of two cavernous sinus dural arteriovenous fistulae by direct microsurgical approach and catheterization of the superior ophthalmic vein.

BACKGROUND: In case of cavernous sinus dural arteriovenous fistula, transvenous embolization of the cavernous sinus via the inferior petrosal sinus is generally sufficient. However, when inferior petrosal sinus access is challenging, various alternative approaches have been reported, with corresponding difficulties and risks. CASE REPORTS: We report the management of two cases of life-threatening cavernous sinus dural arteriovenous fistula revealed by a typical cavernous sinus syndrome. Conventional approaches were unsuccessful, and a direct microsurgical approach was performed, with catheterization of the superior ophthalmic vein. This combined approach safely accessed the cavernous sinus, and obtained complete occlusion of the fistulae by Onyx® embolization. CONCLUSIONS: This procedure could be an interesting alternative option in the treatment of cavernous sinus dural arteriovenous fistula when conventional approaches are not possible.

Multiple neurosurgical treatments for different members of the same family with Currarino syndrome.

INTRODUCTION: Currarino's syndrome (CS) is an autosomal dominant disorder of embryonic development causing a rare malformating syndrome characterized by a triad of an anorectal malformations, presacral mass (most commonly an anterior sacral meningocele) and sacral bony defects. Mutations of the HLXB9 gene have been identified in most CS cases, but a precise genotype-phenotype correlation has not been described so far. Family screening is obligatory. The diagnosis is usually made during childhood and rarely in adulthood. In this context, imaging, and especially MRI plays a major role in the diagnosis of this syndrome. Surgical management is provided by pediatric surgeons or neurosurgeons. FAMILIAL CASE REPORT: Here, we present a family case report with CS requiring different neurosurgical management. The son, a 3-year-old boy, developed a tethered spinal cord syndrome associated to a lipoma of the filum terminale, a sacro-coccygeal teratoma and an anal adhesion. A combined surgical approach permitted a good evolution on the urinary and digestive functions despite a persistent fecal incontinence. The 2-year-old daughter presented with a cyst of the thyreoglossal tract infected and fistulized to the skin. She was also followed for a very small lipoma of the filum terminale that required a neurosurgical approach. The father, 44-year-old, manifested functional digestive and urinary disorders caused by a giant anterior sacral meningocele. The ligation of the neck of the cyst and aspiration of the liquid inside in full through a posterior partial approach permit a complete collapse of the cyst with an instantly satisfactory clinical outcome. CONCLUSION: In these cases, cooperation between pediatric surgeons and neurosurgeons was crucial. The follow-up of these patients should be done in a spina bifida clinic. A geneticist evaluation must be offered to the patient in the case of a CS as well as a clinical evaluation of the relatives (parents, siblings).