Influence of scaffold composition over in vitro osteogenic differentiation of hBMSCs and in vivo inflammatory response.

To understand the role of chitosan in chitosan-poly(butylene succinate) scaffolds (50% wt), 50%, 25%, and 0% of chitosan were used to produce different scaffolds. These scaffolds were in vitro seeded and cultured with human bone marrow stromal cells in osteogenic conditions, revealing that higher percentage of chitosan showed enhanced cell viability over time, adhesion, proliferation, and osteogenic differentiation. Scaffolds were also implanted in cranial defects and iliac submuscular region in Wistar rats, and the results evidenced that chitosan-containing scaffolds displayed mild inflammatory response and good integration with surrounding tissues, showed by connective tissue colonization and the presence of new blood vessels. Scaffolds without chitosan-evidenced necrotic tissue in scaffolds' interior, proving that chitosan exerts a positive effect over cell behavior and displays a milder host inflammatory response in vivo.

Auricular cartilage repair using cryogel scaffolds loaded with BMP-7-expressing primary chondrocytes.

The loss of cartilage tissue due to trauma, tumour surgery or congenital defects, such as microtia and anotia, is one of the major concerns in head and neck surgery. Recently tissue-engineering approaches, including gene delivery, have been proposed for the regeneration of cartilage tissue. In this study, primary chondrocytes were genetically modified with plasmid-encoding bone morphogenetic protein-7 (BMP-7) via the commercially available non-viral Turbofect vector, with the aim of bringing ex vivo transfected chondrocytes to resynthesize BMP-7 in vitro as they would in vivo. Genetically modified cells were implanted into gelatin-oxidized dextran scaffolds and cartilage tissue formation was investigated in 15 × 15 mm auricular cartilage defects in vivo in 48 New Zealand (NZ) white rabbits for 4 months. The results were evaluated via histology and early gene expression. Early gene expression results indicated a strong effect of exogenous BMP-7 on matrix synthesis and chondrocyte growth. In addition, histological analysis results exhibited significantly better cartilage healing with BMP-7-modified (transfected) cells than in the non-modified (non-transfected) group and as well as the control.

A newborn with infantile fibrosarcoma of foot: treatment with chemotherapy and extremity-sparing surgery.

Infantile fibrosarcoma represents less than 1% of all childhood cancers, but it is the most common soft-tissue sarcoma in those under 1 year of age. We report an infant with congenital infantile fibrosarcoma diagnosed as hemangiopericytoma. He was treated with chemotherapy and extremity-sparing surgery. Amputation was avoided.

Venous vascular malformations of the craniofacial region: pre-operative embolisation with direct percutaneous puncture and N-butyl cyanoacrylate.

Craniofacial venous vascular malformations cause severe cosmetic problems and yet these lesions are not candidates for transcatheter embolisation owing to the lack of arterial feeders. The purpose of this study was to evaluate the effectiveness of pre-operative embolisation of these lesions with N-butyl 2-cyanoacrylate (NBCA) via direct puncture. Between September 2003 and April 2006, 13 patients (7 female; age range, 6-64 years; mean, 16.7 years) were embolised with direct puncture and injection of NBCA. All of the patients were referred from plastic surgery with an operational plan. Angiography performed in all patients showed no or little arterial staining. NBCA diluted with iodized oil at a ratio of 1:6 (18%) was injected via a percutaneously placed 21 gauge needle. Complete embolisation was achieved in 8 patients and partial embolisation in the remaining 5. A total of 18 sessions of embolisation were performed on 13 patients. Nine patients underwent only one embolisation session, three patients underwent two sessions and only one patient underwent three sessions. The mean volume of NBCA used per session was 5.8 ml, ranging from 1-12 ml. All patients underwent a successful surgical resection to improve cosmetic disfigurement within 10-15 days after the embolisation procedure. Mean follow-up time was 22 months. One patient experienced skin necrosis on her nose after embolisation. No other complications related to the procedure were observed. In conclusion, pre-operative NBCA embolisation with direct puncture is a safe and easy procedure. It can increase the success of the surgical treatment of these lesions.

Skewed X inactivation in an X linked nystagmus family resulted from a novel, p.R229G, missense mutation in the FRMD7 gene.

AIMS: This study aimed to identify the underlying genetic defect of a large Turkish X linked nystagmus (NYS) family. METHODS: Both Xp11 and Xq26 loci were tested by linkage analysis. The 12 exons and intron-exon junctions of the FRMD7 gene were screened by direct sequencing. X chromosome inactivation analysis was performed by enzymatic predigestion of DNA with a methylation-sensitive enzyme, followed by PCR of the polymorphic CAG repeat of the androgen receptor gene. RESULTS: The family contained 162 individuals, among whom 28 had NYS. Linkage analysis confirmed the Xq26 locus. A novel missense c.686C>G mutation, which causes the substitution of a conserved arginine at amino acid position 229 by glycine (p.R229G) in exon 8 of the FRMD7 gene, was observed. This change was not documented in 120 control individuals. The clinical findings in a female who was homozygous for the mutation were not different from those of affected heterozygous females. Skewed X inactivation was remarkable in the affected females of the family. CONCLUSIONS: A novel p.R229G mutation in the FRMD7 gene causes the NYS phenotype, and skewed X inactivation influences the manifestation of the disease in X linked NYS females.

An intraoperative unexpected respiratory problem in a patient with Apert syndrome.

We present a case of a 5-year-old child who underwent four operations (three for syndactyly of the hands and one for craniofacial corrections). At the third hour of his craniofacial operation, his EtCO2 started to increase and airway resistance was encountered during manual ventilation. The position of the head and neck was checked. An increase in secretion with oral and endotracheal aspiration and a decrease in saturation were observed. When breath sounds disappeared, the patient was reintubated orally. The nasal tube was obstructed with a mucolytic plug. There was no problem during the other operations. This case is presented since anaesthesiologists should be aware of the high incidence of respiratory complications in Apert syndrome.

Embolization of high-flow craniofacial vascular malformations with onyx.

BACKGROUND AND PURPOSE: Various techniques and materials have been used for the endovascular treatment of craniofacial high-flow arteriovenous vascular malformations, because their rarity precludes standardization of their treatment. The aim of this retrospective review is to assess Onyx as the primary embolic agent in the treatment of these vascular malformations. MATERIALS AND METHODS: Six patients with arteriovenous fistulas and 3 with arteriovenous malformations (AVMs) of the head and neck region were treated with intra-arterial (IA)/direct percutaneous injections of Onyx. Adjunctive maneuvers used during embolization included external compression of the arterial feeders or venous outflow (6 patients), balloon assist (4 patients), and direct embolization of the draining vein remote to the fistula site (1 patient). n-butyl-2-cyanoacrylate (n-BCA) was used in addition to Onyx for rapid induction of thrombosis in a large venous pouch (1 patient) and for cost containment purposes (1 patient). Four patients were treated surgically after the embolization. RESULTS: There were no neurologic complications secondary to the embolization procedure. The arteriovenous shunt was eliminated in all of the fistulous lesions and 2 of the 3 AVMs. The embolization was incomplete in 1 patient with a large AVM who declined further endovascular or surgical procedures. Untoward events included 2 instances of catheter entrapment (of 9 IA injections), blackish skin discoloration necessitating surgical revision in 1 patient, and difficulty of balloon deflation/wire withdrawal during a balloon-assisted embolization. CONCLUSION: Onyx appears to be a safe and effective liquid embolic agent for use in the treatment of craniofacial high-flow vascular malformations with distinct advantages and disadvantages compared with n-BCA.

In vivo performance of antibiotic embedded electrospun PCL membranes for prevention of abdominal adhesions.

The aim of this study was to prepare nonwoven materials from poly(epsilon-caprolactone) (PCL) and their antibiotic containing forms by electrospinning, so as to prevent postsurgery induced abdominal adhesions in rats. epsilon-Caprolactone was first polymerized by ring-opening polymerization, and then it was processed into matrices composed of nanofibers by electrospinning. A model antibiotic (Biteral) was embedded within a group of PCL membranes. In the rat model, defects on the abdominal walls in the peritoneum were made to induce adhesion. The plain or antibiotic embedded PCL membranes were implanted on the right side of the abdominal wall. No membrane implantation was made on the left side of the abdominal wall that served as control. Macroscopical and histological evaluations showed that using these barriers reduces the extent, type, and tenacity of adhesion. The antibiotic embedded membranes significantly eliminated postsurgery abdominal adhesions, and also improved healing.

In vitro and in vivo degradation of non-woven materials made of poly(epsilon-caprolactone) nanofibers prepared by electrospinning under different conditions.

The aim of this study was to prepare non-woven materials from a biodegradable polymer, poly(epsilon-caprolactone) (PCL) by electrospinning. PCL was synthesized by ring-opening polymerization of epsilon-caprolactone in bulk using stannous octoate as the catalyst under nitrogen atmosphere. PCL was then processed into non-woven matrices composed of nanofibers by electrospinning of the polymer from its solution using a high voltage power supply. The effects of PCL concentration, composition of the solvent (a mixture of chloroform and DMF with different DMF content), applied voltage and tip-collector distance on fiber diameter and morphology were investigated. The diameter of fibers increased with the increase in the polymer concentration and decrease in the DMF content significantly. Applied voltage and tip-collector distance were found critical to control 'bead' formation. Elongation-at-break, ultimate strength and Young's modulus were obtained from the mechanical tests, which were all increased by increasing fiber diameter. The fiber diameter significantly influenced both in vitro degradation (performed in Ringer solution) and in vivo biodegradation (conducted in rats) rates. In vivo degradation was found to be faster than in vitro. Electrospun membranes were more hydrophobic than PCL solvent-casted ones; therefore, their degradation was a much slower process.

Hereditary intraosseous vascular malformation of the craniofacial region: imaging findings.

Benign vascular lesions can be classified into two categories depending on clinical behaviour and endothelial cell characteristics: neoplasms (haemangiomas) and vascular malformations. However, intraosseous vascular anomaly, previously called intraosseous haemangioma, is a very rare malformation. In our previous study, we described the first hereditary form of intraosseous vascular malformation of the craniofacial region, vascular malformation osseous (VMOS). Characteristic findings are autosomal recessive inheritance, severe and diffuse intraosseous vascular malformation in all craniofacial bones without soft tissue involvement and associated mid-line abnormalities such as umbilical hernia and supra-umbilical raphe. In this paper, we discuss the imaging findings of this new disorder in detail.

Bartsocas-Papas syndrome with fusion of the lips and posterior fusion defects of the thoracic vertebrae.

Bartsocas-Papas syndrome is a rare popliteal pterygial syndrome with multiple anomalies including microcephaly, facial clefts, filiform bands, ankyloblepharon, syndactyly, and other ectodermal anomalies. Affected infants usually die perinatally. The authors present an 8-month-old female infant with manifestations of this syndrome and some additional traits including fusion of the lips, intraoral filiform bands, alopecia totalis, and posterior fusion failure of the vertebrae. The fused lips were opened by incising the fibrotic bands closing her mouth. Details of this patient and a brief review of the literature is presented.