Comparison of 2-octyl cyanoacrylate with polyester mesh with standard suture and staples in total knee and hip arthroplasty.

OBJECTIVE: The use of 2-octyl cyanoacrylate with polyester mesh (OCA-M) has become common in total hip and knee arthroplasty (THA, TKA). We aimed to compare the safety and cosmetic outcomes between OCA-M and standard suture techniques and staples, and determine whether OCA-M can safely be used for TKA. METHOD: Inclusion criteria were patients who underwent THA or TKA from January 2010 to October 2011 (Suture group), November 2011 to August 2013 (Staple group), March 2017 to September 2018 (OCA-M group). Exclusion criteria was loss of imaging data. Complications during hospitalisation (early complication) and after discharge (late complication) were compared in groups. Plastic and orthopaedic surgeons performed cosmetic evaluations with the modified Vancouver Scar Scale (VSS) and Likert scale at three and six months postoperatively and compared in groups. RESULTS: A total of 249 arthroplasties (suture group=88 patients; staple group=94 patients; OCA-M group=67 patients) were included in the study. The OCA-M group had a significantly lower early complication rate than the suture group (p=0.015). For THA, the OCA-M group had a significantly lower total complication rate than the suture group (p=0.048). For TKA, there was no significant difference among the three groups. The complication rate in the OCA-M group showed no significant difference between THA/TKA. With regards to the VSS, the OCA-M group was significantly better for cosmetic qualities than the suture group (p=<0.001, p=0.021 at three and six months, respectively). For the Likert scale, the OCA-M group was also significantly better for cosmetic qualities than the suture group and staple group (suture-OCA-M, p=0.003 (three months), p=<0.001 (six months); staple-OCA-M, p=0.027 (three months)). CONCLUSION: In this study, the OCA-M complication rate was low compared to suturing and similar to stapling. Moreover, better cosmetic outcomes were achieved compared to suturing and stapling.

Aqueous Povidone-Iodine Versus Normal Saline For Intraoperative Wound Irrigation on The Incidence of Surgical Site Infection in Clean-Contaminated Wounds After Gastroenterological Surgery: A Single-Institute, Prospective, Blinded-Endpoint, Randomized Controlled Trial.

OBJECTIVE: This trial evaluated the superiority of intraoperative wound irrigation (IOWI) with aqueous povidone-iodine (PVP-I) compared with that with saline for reducing the incidence of surgical site infection (SSI). BACKGROUND: IOWI with aqueous PVP-I is recommended for the prevention of SSI by the World Health Organization and the Centers for Disease Control and Prevention, although the evidence level is low. METHODS: This single institute in Japan, prospective, randomized, blinded-endpoint trial was conducted to assess the superiority of IOWI with aqueous PVP-I in comparison with IOWI with saline for reducing the incidence of SSI in clean-contaminated wounds after gastroenterological surgery. Patients 20 years or older were assessed for eligibility, and the eligible participants were randomized at a 1:1 ratio using a computer-generated block randomization. In the study group, IOWI was performed for 1 minute with 40 mL of aqueous 10% PVP-I before skin closure. In the control group, the procedure was performed with 100 mL of saline. Participants, assessors, and analysts were masked to the treatment allocation. The primary outcome was the incidence of incisional SSI in the intention-to-treat set. RESULTS: Between June 2019 and March 2022, 941 patients were randomized to the study group (473 patients) or the control group (468 patients). The incidence of incisional SSI was 7.6% in the study group and 5.1% in the control group (risk difference 0.025, 95% CI -0.006 to 0.056; risk ratio 1.484, 95% CI 0.9 to 2.448; P =0.154). CONCLUSION: The current recommendation of IOWI with aqueous PVP-I should be reconsidered.

Fragility fractures and delayed wound healing after tooth extraction in Japanese older adults.

INTRODUCTION: Fragility fractures can cause delayed wound healing after tooth extraction, which contributes to an increased risk of osteomyelitis of the jaw. We evaluated whether a history of fragility fracture was associated with increased risk of delayed wound healing after tooth extraction in older adults in Japan. MATERIALS AND METHODS: Of 5352 people aged 50-89 years in the 2014 basic resident registry of the town of Obuse, the present study included 376 subjects (190 men and 186 women) who completed a structured questionnaire and measurement of the bone mineral densities (BMDs) of the bilateral femoral neck. Delayed wound healing after tooth extraction was self-reported. Fragility fractures were confirmed via examination of hospital medical records. Logistic regression analyses adjusted for age and gender were used to evaluate association of clinical variables with delayed would healing after tooth extractions. Odds ratios (ORs) and the 95% confidence intervals (CIs) of all possible associated variables for the presence of delayed wound healing were calculated. RESULTS: Subjects with a history of fragility fractures had a significantly higher risk of delayed wound healing compared with those without previous fragility fractures (OR 2.68; 95% CI 1.11-6.46, p = 0.028). This association still remained after adjusted for all other variables (OR 2.70; 95% CI 1.10-6.60, p = 0.030). Delayed wound healing was not significantly associated with the BMD of the femoral neck. CONCLUSIONS: History of fragility fracture may be associated with increased risk of delayed wound healing after tooth extraction in Japanese men and women aged 50-89 years.

Construction of 3 animal experimental models in the development of honeycomb microporous covered stents for the treatment of large wide-necked cerebral aneurysms.

The treatment of large or wide-necked cerebral aneurysms is extremely difficult, and carries a high risk of rupture, even when surgical or endovascular methods are available. We are developing novel honeycomb microporous covered stents for treating such aneurysms. In this study, 3 experimental animal models were designed and evaluated quantitatively before preclinical study. The stents were prepared using specially designed balloon-expandable stents (diameter 3.5-5.0 mm, length 16-28 mm) by dip-coating to completely cover their struts with polyurethane film (thickness 20 µm) and microprocessing to form the honeycomb pattern after expansion. (1) In an internal carotid artery canine model (n = 4), all stents mounted on the delivery catheter passed smoothly through the tortuous vessel with minimal arterial damage. (2) In an the large, wide-necked, outer-sidewall aneurysm canine model, almost all parts of the aneurysms had embolized immediately after stenting (n = 4), and histological examination at 2 months revealed neointimal formation with complete endothelialization at all stented segments and entirely organized aneurysms. (3) In a perforating artery rabbit model, all lumbar arteries remained patent (n = 3), with minimal change in the vascular flow pattern for over 1 year, even after placement of a second, overlapping stent (n = 3). At 2 months after stenting, the luminal surface was covered with complete thin neointimal formation. Excellent embolization performance of the honeycomb microporous covered stents without disturbing branching flow was confirmed at the aneurysms in this proof-of-concept study.

Effectiveness of dental checkups incorporating tooth brushing instruction.

The purpose of this study was to compare the effectiveness of dental checkups incorporating tooth-brushing instruction (TBI) with that of conventional dental checkups. A team consisting of one dentist and three dental hygienists saw an average of 60 employees per day on-site at an airline company. The patient's teeth were stained with a disclosing tablet and the results recorded on a Plaque Control Record (PCR) chart. The patient was then given TBI. After recording the relevant data, including TBI given and PCR scores, the charts were stored. Checkups were performed in a total of 3,854 patients between 2001 and 2005 and changes in annual scores investigated. In addition, annual shifts in mean score in patients receiving checkups over all five years were compared with those in patients receiving checkups for the first time in each of the five years. The mean score in patients receiving a checkup in 2001 was 35.1%, declining by 2.6 points to 32.5% in 2005. Among patients receiving checkups over all five years, the mean score in 2001 was 34.0%, declining by 11.2 points to 22.8% in 2005. Over the five-year period, the mean score in patients receiving checkups was 34.1%. In patients receiving checkups over all five years, the proportion with PCR scores <30% increased each year. This was because the number of patients with PCR scores ≥60% decreased each year. These findings suggest that TBI is effective in reducing poor plaque control. When compared with in patients who had not received TBI, five consecutive years of checkups was clearly effective. These results indicate that checkups incorporating TBI are more effective than conventional dental checkups that simply check for caries. In future, this type of checkup should contribute to improved preventative dentistry with minimal intervention.

Comparison of fusion versus non-fusion surgery for retro-odontoid pseudotumor with atlanto-axial subluxation.

BACKGROUND: Due to the limited number of reports comparing posterior fusion with posterior decompression alone for retro-odontoid pseudotumor, there remains no consensus on treatment preference, especially in older patients. This study compared posterior fusion (with or without additional decompression) with posterior decompression alone for treating spinal cord pressure from non-inflammatory retro-odontoid pseudotumor with atlanto-axial subluxation (AAS). METHODS: Forty-one patients (27 male and 14 female; mean age, 73.0 ± 11.4 years) who underwent either posterior cervical fusion or decompression alone for the treatment of non-inflammatory retro-odontoid pseudotumor with AAS and were observed for more than 1 year between September 2009 and July 2019 were enrolled. Thirty-two patients (23 male and 9 female; mean age: 71.8 ± 10.9 years) received posterior fusion surgery (fusion group) and 9 patients (4 male and 5 female; mean age: 77.2 ± 12.5 years) underwent decompression alone (non-fusion group). We compared pre- and postoperative Japanese Orthopaedic Association (JOA) scores and preoperative cervical alignment parameters between the groups. RESULTS: In the fusion group, the mean preoperative JOA score was significantly improved from 9.0 ± 3.2 points to 11.7 ± 3.2 points at the final follow-up (p = 0.0002). Similarly in the non-fusion group, the mean preoperative and final follow-up JOA scores were 8.2 ± 3.5 points and 11.7 ± 3.8 points, respectively (p = 0.003). The recovery rate at the final follow-up was 22.6% in the fusion group and 43.4% in the non-fusion group, which were statistically comparable (p = 0.23). We observed no remarkable correlations between cervical sagittal spinal alignment parameters and JOA score recovery rate in the cohort, nor was any significant subluxation progression seen. CONCLUSION: Compared with fusion surgery, surgical decompression alone may be a suitable and less invasive option for the treatment of non-inflammatory retro-odontoid pseudotumor with AAS, especially in elderly patients.

Cold-induced sweating syndrome with neonatal features of Crisponi syndrome: longitudinal observation of a patient homozygous for a CRLF1 mutation.

Cold-induced sweating syndrome (CISS) is a rare autosomal recessive disorder caused by mutations in CRLF1 (cytokine receptor-like factor 1), characterized by profuse sweating in cold environmental temperature and craniofacial and skeletal features. Mutations in CRLF1 also cause Crisponi syndrome (CS), characterized by neonatal-onset paroxysmal muscular contractions as well as craniofacial and skeletal manifestations and abnormal functions of the autonomic nerve system. To date, it is an unresolved problem whether the two conditions are distinct clinical entities or a single clinical entity with variable expressions or with different presentations depending on the patients' age at diagnosis. We report on a 30-year-old Japanese woman with CISS and homozygous out-of-frame 23-base deletion of CRLF1. In infancy, she did not show paroxysmal muscular contractions, but showed feeding difficulty, hyperthermia, and facial characteristics including thick and arched eyebrows, a short nose with anteverted nostrils, full cheeks, an inverted upper lip, and a small mouth, resembling those observed in CS. Profuse sweating was noticed at 3 years of age. Cold-induced sweating was recognized in her elementary school days. In adolescence to adulthood, she showed a Marfanoid habitus with progressive kyphoscoliosis and craniofacial characteristics including dolichocephaly, a slender face with poor expression, a distinctive nose with hypoplastic nares, malar hypoplasia, prognathism, and a small mouth. This is the first report of detailed longitudinal observation of a patient with CRLF1 abnormalities, compatible with the notion that CISS and CS may be a single clinical entity.

A new Ehlers-Danlos syndrome with craniofacial characteristics, multiple congenital contractures, progressive joint and skin laxity, and multisystem fragility-related manifestations.

We previously described two unrelated patients showing characteristic facial and skeletal features, overlapping with the kyphoscoliosis type Ehlers-Danlos syndrome (EDS) but without lysyl hydroxylase deficiency [Kosho et al. (2005) Am J Med Genet Part A 138A:282-287]. After observations of them over time and encounter with four additional unrelated patients, we have concluded that they represent a new clinically recognizable type of EDS with distinct craniofacial characteristics, multiple congenital contractures, progressive joint and skin laxity, and multisystem fragility-related manifestations. The patients exhibited strikingly similar features according to their age: craniofacial, large fontanelle, hypertelorism, short and downslanting palpebral fissures, blue sclerae, short nose with hypoplastic columella, low-set and rotated ears, high palate, long philtrum, thin vermilion of the upper lip, small mouth, and micro-retrognathia in infancy; slender and asymmetric face with protruding jaw from adolescence; skeletal, congenital contractures of fingers, wrists, and hips, and talipes equinovarus with anomalous insertions of flexor muscles; progressive joint laxity with recurrent dislocations; slender and/or cylindrical fingers and progressive talipes valgus and cavum or planus, with diaphyseal narrowing of phalanges, metacarpals, and metatarsals; pectus deformities; scoliosis or kyphoscoliosis with decreased physiological curvatures of thoracic spines and tall vertebrae; cutaneous, progressive hyperextensibility, bruisability, and fragility with atrophic scars; fine palmar creases in childhood to acrogeria-like prominent wrinkles in adulthood, recurrent subcutaneous infections with fistula formation; cardiovascular, cardiac valve abnormalities, recurrent large subcutaneous hematomas from childhood; gastrointestinal, constipation, diverticula perforation; respiratory, (hemo)pneumothorax; and ophthalmological, strabismus, glaucoma, refractive errors.

The Titanium-coated PEEK Cage Maintains Better Bone Fusion With the Endplate Than the PEEK Cage 6 Months After PLIF Surgery: A Multicenter, Prospective, Randomized Study.

STUDY DESIGN: A multicenter, randomized, open-label, parallel-group trial. OBJECTIVE: To investigate interbody bone fusion rates in titanium-coated polyetheretherketone (TiPEEK) and polyetheretherketone (PEEK) cages after posterior lumbar interbody fusion (PLIF) surgery. SUMMARY OF BACKGROUND DATA: Previous clinical studies have not revealed any significant difference in bone fusion rates between TiPEEK and PEEK cages. METHODS: During one-level PLIF surgery, 149 patients (84 men, 65 women, mean age 67 yr) were randomly allocated to use either a TiPEEK cage (n = 69) or PEEK cage (n = 80). Blinded radiographic evaluations were performed using computed tomography and assessed by modified intention-to-treat analysis in 149 cases and per-protocol analysis in 143 cases who were followed for 12 months. Clinical outcomes were assessed using the Japanese Orthopaedic Association Back Pain Evaluation Questionnaire and the Oswestry Disability Index. RESULTS: The interbody union rate at 12 months after surgery was 45% owing to a very strict definition of bone fusion. The rates of bone fusion were significantly higher at 4 and 6 months after surgery in the TiPEEK group than in the PEEK group in the unadjusted modified intention-to-treat analysis and were significantly higher at 6 months in the unadjusted per-protocol analysis. Binary logistic regression analysis adjusted for sex, age, body mass index, bone mineral density, and surgical level showed that using a TiPEEK cage (odds ratio, 2.27; 95% confidence interval: 1.09-4.74; P = 0.03) was independently associated with bone fusion at 6 months after surgery. Japanese Orthopaedic Association Back Pain Evaluation Questionnaire and Oswestry Disability Index results improved postoperatively in both groups. CONCLUSION: Using the TiPEEK cage for PLIF enabled the maintenance of better bone fusion to the endplate than using the PEEK cage at 6 months after the surgery. Our findings suggest the possibility of an earlier return to rigorous work or sports by the use of TiPEEK cage. LEVEL OF EVIDENCE: 1.

A case of corrosive esophagitis causing extensive cicatricial esophageal stenosis treated by esophageal bypass with supercharged pedicled jejunal pull-up.

INTRODUCTION: Swallowing a corrosive substance causes delayed gastrointestinal stenosis due to scar formation. Here, we report on our use of esophageal bypass using a supercharged pedicled jejunal flap to treat cicatricial esophageal stenosis caused by corrosive esophagitis. PRESENTATION OF CASE: Nineteen years before presentation, a 57-year-old man had swallowed a chemical cleaning agent, which caused extensive corrosive cicatricial stenosis from the thoracic upper esophagus to the gastric fornix. An enterostomy had been created, and the patient had since been subsisting on enteral nutrition. However, he wanted to be able to eat through his mouth again and was referred to our department for treatment. With the exception of the cervical esophagus, circumferential cicatricial stenosis was present throughout the esophagus and gastric fornix, with severe adhesions to the surrounding tissue. It was decided not to perform esophagectomy but to perform esophageal bypass surgery using a supercharged pedicled jejunal flap. DISCUSSION: Despite the extremely high risk of cancer in the stenotic esophagus due by corrosive esophagitis, indicating that esophagectomy should be performed if possible, we chose to perform bypass surgery because the severe adhesions posed a high risk of early injury to the surrounding organs. CONCLUSION: We suggest that esophageal bypass using pedicled jejunal pull-up "supercharging" by creating anastomoses between the jejunal and internal thoracic vessels is the optimal procedure for patients with extensive cicatricial esophageal stenosis caused by corrosive esophagitis.

Rigid Occipitocervical Instrumented Fusion for Atlantoaxial Instability in an 18-Month-Old Toddler With Brachytelephalangic Chondrodysplasia Punctata: A Case Report.

STUDY DESIGN: Case report. OBJECTIVE: We report here on an 18-month-old boy with brachytelephalangic chondrodysplasia punctata (BCDP), whose atlantoaxial instability was successfully managed with occipitocervical instrumented fusion (OCF) using screw and rod instrumentations. SUMMARY OF BACKGROUND DATA: Recently, there have been a number of reports on BCDP with early onset of cervical myelopathy. Surgical OCF is a vital intervention to salvage affected individuals from the life-threatening morbidity. Despite recent advancement of instrumentation techniques, however, rigid OCF is technically demanding in very young children with small and fragile osseous elements. To our best knowledge, this is the first report on application of the instrumentation technique to a toddler patient with BCDP. METHODS: A 16-month-old boy with BCDP presented with tetraplegia and swallow obstacle. Hypoplasia of the odontoid process and atlantoaxial instability were present in lateral radiographs. T2-weighted magnetic resonance (MR) images revealed a high signal region in the spinal cord at the C1-2 and C7-T1 levels. Cervical computed tomography (CT) showed that the pedicles and lateral masses in the cervical spine were small and immature, but the laminae were comparatively thick. RESULTS: One week before surgery, the patient was fitted with a Halo-body jacket. We performed plate-rod placement with occipital cortical screws and C2/C3 interlaminar screws, and added an autogenous bone graft using the right 8 and 9 ribs. Rigid fixation of the occipito-cervical spine was completed successfully without major complications. Postoperative halo-body jacket immobilization was continued for 3 months, after which Aspen collar was fitted. CT confirmed occipitocervical bone fusion at 6 months after surgery. Mild clinical improvements in motor power of the affected muscles and swallowing were witnessed at 1 year postoperatively. CONCLUSION: Rigid fixation using screw, rod, and occipital plate instrumentation was successful in an 18-month-old toddler with BCDP and atlantoaxial instability. Bone fusion was achieved at postoperative 6 months. LEVEL OF EVIDENCE: 5.

A reliable aptamer array prepared by repeating inkjet-spotting toward on-site measurement.

A preparation protocol is proposed for a reliable aptamer array utilizing an ink-jet spotter. We accumulated streptavidin and biotinylated-aptamer in this order on a biotinylated-polyethylene glycol-coated gold substrate to prepare an aptamer array. The aptamer array was prepared with an alternate spotting structure where each aptamer spot was placed between reference spots formed with blocking solution thus suppressing contamination from neighboring spots during the blocking and washing processes. Four aptamer spots were prepared in a small area of 1×4.8mm(2) with five reference spots made of blocking solution. We evaluated the thrombin binding ability of the spotted aptamer array using a multi-analysis surface plasmon resonance sensor. We prepared a disposable capillary-driven flow chip designed for on-site measurement (Miura et al., 2010) with our aptamer array and detected thrombin from phosphate-buffered saline at concentrations of 50ngmL(-1) and 1µgmL(-1) (equivalent to 1.35 and 27nM, respectively). A correlation was observed between the refractive index shift and thrombin concentration. This implies that our array preparation protocol meets the requirement for the preparation of a one-time-use chip for on-site measurement.

Challenges of Transarticular Screw Fixation in Young Children: Report of Surgical Treatment of a 5-Year-Old Patient's Unstable Os-Odontoideum.

Surgical procedures for atlantoaxial (C1-C2) fusion in young children are relatively uncommon. The purpose of this study was to report on a surgical treatment for a case of atlantoaxial instability caused by os-odontoideum in association with quadriparesis and respiratory paralysis in a 5-year-old girl. We present the patient's history, physical examination, and radiographic findings, describe the surgical treatment and a five year follow-up, and provide a literature review. The instability was treated by halo immobilization, followed by C1-C2 transarticular screw fixation using a computed tomography-based navigation system. At the five year follow-up, the patient had made a complete recovery with solid union. The authors conclude that C1-2 transarticular screw fixation is technically possible as in a case of atlantoaxial instability in a five-year-old child.

Beneficial Effects of a Novel Bioabsorbable Polymer Coating on Enhanced Coronary Vasoconstricting Responses After Drug-Eluting Stent Implantation in Pigs in Vivo.

OBJECTIVES: The aim of this study was to examine which component of drug-eluting stents (DES) plays a major role in enhanced coronary vasoconstricting responses after DES implantation in pigs. BACKGROUND: Recent studies have reported unremitting angina due to vasomotion abnormalities even after successful DES implantation. However, it remains to be elucidated which component of DES (metal stent, polymer coating, or antiproliferative drug) is responsible for DES-induced coronary hyperconstricting responses. METHODS: We developed poly-dl-lactic acid and polycaprolactone (PDLLA-PCL) copolymer technology with higher biocompatibility that is resorbed within 3 months. Four types of coronary stents were made: 1) a stent with polylactic acid (PLA) polymer coating containing antiproliferative drug (P1+D+); 2) a stent with PLA polymer coating alone without any drug (P1+D-); 3) a stent with novel PDLLA-PCL polymer coating alone (P2+D-); and 4) a bare metal stent (P-D-). The 4 stents were randomly deployed in the left anterior descending and left circumflex coronary arteries in 12 pigs. RESULTS: After 1 month, coronary vasoconstriction by intracoronary serotonin was enhanced at P1+D+ and P1+D- stent edges compared with P2+D- and P-D- stent edges and was prevented by a specific Rho-kinase (a central molecule of coronary spasm) inhibitor, hydroxyfasudil. Immunostainings showed that inflammatory changes and Rho-kinase activation were significantly enhanced at P1+D+ and P1+D- sites compared with P2+D- and P-D- sites. There were significant positive correlations between the extent of inflammation or Rho-kinase expression/activation and that of coronary vasoconstriction. CONCLUSIONS: These results indicate the important roles of PLA polymer coating in DES-induced coronary vasoconstricting responses through inflammatory changes and Rho-kinase activation in pigs in vivo, which are ameliorated by PDLLA-PCL copolymers.

Repair of a proximal femoral bone defect in dogs using a porous surfaced prosthesis in combination with recombinant BMP-2 and a synthetic polymer carrier.

Total hip arthroplasty (THA) has become an almost standard procedure for the treatment of various hip lesions. However, one of the limitations has been the mechanical loosening of the prosthesis, a condition termed peri-prosthetic osteolysis. Consequently, at revision surgery, various grades of bone defect are often noted. Alternative approaches aimed at overcoming this problem have included a special design of the revision prosthesis and allo- or autogeneic bone grafting in combination with or without biomaterials. In a further attempt to address the loosening of the prosthesis, we have combined human bone morphogenetic protein-2, produced by DNA recombination (rhBMP-2) with a new synthetic biodegradable polymer (poly-D,L-lactic-acid-para-dioxanone-polyethyleneglycol block co-polymer; PLA-DX-PEG). We present data on the efficacy of the rhBMP-2 laden prosthesis to reconstruct a bone defect in a canine model. In this model, medial half of the proximal femur was surgically resected to create a bone defect that was repaired with the rhBMP-2/PLA-DX-PEG composite. Twelve weeks after implantation, the original bone defects in the rhBMP-2 treatment groups had been repaired. Thus, this type of 'hybrid' prosthesis may provide a new modality to repair bone defects or restore lost bone mass encountered in revision arthroplasty.

[Vertebroplasty].

Vertebroplasty for pseudoartrosis of vertebral fracture using PMMA (polymethylmetacrylate) or CPC (calcium phosphate cement) is a new less invasive surgical method providing sufficient pain relief and correction of kyphosis.

Mandibuloacral dysplasia and a novel LMNA mutation in a woman with severe progressive skeletal changes.

A 56-year-old Japanese woman with mandibuloacral dysplasia and type A lipodystrophy is described. Mutation analysis identified a homozygous missense mutation (1585G > A) in exon 9 of the LMNA gene that replaces well-conserved residue alanine at position 529 to threonine (A529T). The woman showed, in addition to the usual clinical manifestations of the disorder, severe progressive skeletal changes: osteoporotic changes with multiple fractures; osteolysis of the right radius; and destructive changes of the vertebrae, leading to compression of the cervical spinal cord and paraplegia. Laboratory findings included markedly reduced bone mineral density; significantly increased urine N-telopeptide of collagen type I, an osteoclast marker; and normal serum bone specific alkaline phosphatase, an osteoblast marker. Regular follow up of adult patients with the disorder is desirable, including skeletal radiography, estimates of bone mineral density, and biochemical markers of bone turnover. Treatment with bisphosphonates to inhibit osteoclast activity is likely to be beneficial.

Ehlers-Danlos syndrome type VIB with characteristic facies, decreased curvatures of the spinal column, and joint contractures in two unrelated girls.

Two unrelated girls, aged 11 and 14 years, with clinical manifestations of Ehlers-Danlos syndrome (EDS) type VIB, characteristic facies, skeletal abnormalities, and other features are described. They had Marfanoid habitus with pectus excavatum; fragile, hyperextensible, and readily bruisable skin with widened, atrophic scars; recurrent hematomas; generalized joint laxity; hypotonia; scoliosis; and mild delay of gross motor development. Lysyl hydroxylase deficiency was ruled out in Patient 1. Parental consanguinity was present in Patient 2. They both had, in early childhood, down-slanting palpebral fissures, drooping lower eyelids, short nose, small mouth, and long philtrum. Facial features that persisted included thick eyebrows, hypertelorism, strabismus, blue sclerae, low-set, and slanted ears, hypoplastic columella, high-arched palate, and thin upper lip. They had tubular stenosis of the phalanges, metacarpals, and metatarsals; decreased physiological curvatures of the spinal column with tall vertebrae; and joint contractures including talipes equinovarus and progressive talipes valgus. Their hearing of high-pitched sounds was impaired. They had constipation and recurrent cystitis with an enlarged bladder. In view of these findings, we propose that these two girls represent a clinically recognizable subgroup of EDS type VIB.

Anterior thoracic spinal fusion in dogs by injection of recombinant human bone morphogenetic protein-2 and a synthetic polymer.

The purpose of this study was to achieve spinal fusion in the absence of bone graft material using a new, injectable, and semi-liquid synthetic polymer (polylactic acid polyethylene glycol [PLA-PEG] block copolymer) containing recombinant human bone morphogenetic protein-2 (rhBMP-2). Twenty-seven skeletally mature beagles underwent anterior thoracic spinal fusion at T9-T10. Group I (n = 9) was injected with 1 mL of PLA-PEG block copolymer carrier alone into space under the vertebral pleura and the anterior longitudinal ligament. Group II (n = 9) was injected with 1 mL of PLA-PEG carrier containing 500 microgram of rhBMP-2. Group III (n = 9) was injected with 1 mL of PLA-PEG carrier containing 1000 microgram of rhBMP-2. In the Group I animals, no evidence of new bone formation was noted at the implanted sites both radiographically and histologically. In contrast, all of the nine animals in Group III showed new bone formation in 12 weeks, and four of the nine animals in Group II showed bony mass at the injected sites. However, vertebral bony fusion was incomplete despite the significant amount of new bone formation in both groups that showed new bone formation. In addition to resulting in improvements in the surgical procedure, injection of rhBMP-2 and a synthetic polymer is useful for bone formation for spinal fusion.