John P Dormans

University of Pennsylvania, Philadelphia, Pennsylvania, United States

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Publications (157)382.99 Total impact

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    ABSTRACT: Study Design Consensus-based creation of a checklist and guideline. Objective To develop a consensus-based checklist to guide surgeon responses to intraoperative neuromonitoring (IONM) changes in patients with a stable spine and to develop a consensus-based best practice guideline for IONM practice in the United States. Summary of Background Data Studies show that checklists enhance surgical team responses to crisis situations and improve patient outcomes. Currently, no widely accepted guidelines exist for the response to IONM changes in spine deformity surgery. Methods After a literature review of risk factors and recommendations for responding to IONM changes, 4 surveys were administered to 21 experienced spine surgeons and 1 neurologist experienced in IONM. Areas of equipoise were identified and the nominal group process was used to determine items to be included in the checklist. The authors reevaluated and modified the checklist at 3 face-to-face meetings over 12 months, including a period of clinical validation using a modified Delphi process. The group was also surveyed on current IONM practices at their institutions. This information and existing IONM position statements were used to create the IONM best practice guideline. Results Consensus was reached for the creation of 5 checklist headings containing 26 items to consider in the response to IONM changes. Consensus was reached on 5 statements for inclusion in the best practice guideline; the final guideline promotes a team approach and makes recommendations aimed at decreasing variability in neuromonitoring practices. Conclusions The final products represent the consensus of a group of expert spine surgeons. The checklist includes the most important and high-yield items to consider when responding to IONM changes in patients with a stable spine, whereas the IONM guideline represents the group consensus on items that should be considered best practice among IONM teams with the appropriate resources.
    Spine Deformity. 09/2014; 2(5):333–339.
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    ABSTRACT: BACKGROUND Patients with Ewing sarcoma require local primary tumor control with surgery, radiation, or both. The optimal choice of local control for overall and local disease control remains unclear.METHODS Patients with localized Ewing sarcoma of bone who were treated on 3 consecutive protocols with standard-dose, 5-drug chemotherapy every 3 weeks were included (n=465). Propensity scores were used to control for differences between local control groups by constructing multivariate models to assess the impact of local control type on clinical endpoints (event-free survival [EFS], overall survival, local failure, and distant failure) independent of differences in their propensity to receive each local control type.RESULTSPatients who underwent surgery were younger (P=.02) and had more appendicular tumors (P<.001). Compared with surgery, radiation had higher unadjusted risks of any event (hazard ratio [HR], 1.70; 95% confidence interval [CI], 1.18-2.44), death (HR, 1.84; 95% CI, 1.18-2.85), and local failure (HR, 2.57; 95% CI, 1.37-4.83). On multivariate analysis, compared with surgery, radiation had a higher risk of local failure (HR, 2.41; 95% CI, 1.24-4.68), although there were no significant differences in EFS (HR, 1.42; 95% CI, 0.94-2.14), overall survival (HR, 1.37; 95% CI, 0.83-2.26), or distant failure (HR, 1.13; 95% CI, 0.70-1.84) between local control groups.CONCLUSIONS In this large group of similarly treated patients, choice of the mode of local control was not related significantly to EFS, overall survival, or distant failure, although the risk of local failure was greater for radiation compared with surgery. These data support surgical resection when appropriate, whereas radiotherapy remains a reasonable alternative in selected patients. Cancer 2014. © 2014 American Cancer Society.
    Cancer 09/2014; · 5.20 Impact Factor
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    ABSTRACT: Adolescent idiopathic scoliosis (AIS) is a common rotational deformity of the spine that presents in children worldwide, yet its etiology is poorly understood. Recent genome-wide association studies (GWAS) have identified a few candidate risk loci. One locus near the chromosome 10q24.31 LBX1 gene (OMIM #604255) was originally identified by a GWAS of Japanese subjects and replicated in additional Asian populations. To extend this result, and to create larger AIS cohorts for the purpose of large-scale meta-analyses in multiple ethnicities, we formed a collaborative group called the International Consortium for Scoliosis Genetics (ICSG). Here, we report the first ICSG study, a meta-analysis of the LBX1 locus in six Asian and three non-Asian cohorts. We find significant evidence for association of this locus with AIS susceptibility in all nine cohorts. Results for seven cohorts containing both genders yielded P=1.22×10-43 for rs11190870, and P=2.94×10-48 for females in all nine cohorts. Comparing the regional haplotype structures for three populations, we refined the boundaries of association to a ∼25 kb block encompassing the LBX1 gene. The LBX1 protein, a homeobox transcription factor that is orthologous to the Drosophila ladybird late gene, is involved in proper migration of muscle precursor cells, specification of cardiac neural crest cells, and neuronal determination in developing neural tubes. Our results firmly establish the LBX1 region as the first major susceptibility locus for AIS in Asian and non-Hispanic white groups, and provide a platform for larger studies in additional ancestral groups.
    Journal of Medical Genetics 04/2014; · 5.70 Impact Factor
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    ABSTRACT: Retrospective cohort study using the Pediatric Health Information Systems (PHIS) database. To determine the association between antifibrinolytic use and red cell transfusions in spinal fusion operations performed at 37 US Children's Hospitals. Evidence from randomized clinical trials and systematic reviews suggests that antifibrinolytic therapy can significantly reduce blood loss in children undergoing scoliosis surgery, but the effectiveness of these agents as used in surgeries performed at US children's has not been studied. We included children 0-18 years of age with diagnoses indicating adolescent idiopathic scoliosis (AIS) or neuromuscular scoliosis (NMS) for whom a spinal fusion procedure was performed between 1/1/2006-9/30/2009. Patients with malignancy, trauma, coagulation disorders, or for whom a cell salvage device was employed were excluded. Multilevel logistic regression was used to determine associations between ε-aminocaproic acid (EACA), tranexamic acid (TXA) and aprotinin (APR) use and blood transfusions, controlling for patient and surgery characteristics. Cohorts consisted of 2722 AIS and 1547 NMS procedures. Antifibrinolytic use varied across hospitals (AIS 3.3%, IQR 0%-42%; NMS 12 IQR 0%-46%), and was significantly associated with NMS, posterior fusion, number of vertebrae fused. Overall, 15% of children received EACA, 7% TXA, and 2% APR. The median hospital-specific rate of red cell transfusions was 24% for AIS, and 43% for NMS. In AIS operations, EACA use, but not TXA use, was associated with significantly lower odds of transfusion (OR 0.42, P<0.001 vs. OR 1.0, P=0.8). In NMS operations, neither EACA nor TXA use was associated with a decrease in odds of red cell transfusions. The effectiveness of antifibrinolytics as used outside of clinical trials is unclear and should continue to be explored. Future prospective research is needed to evaluate which administration protocols will most benefit patients, as well as to determine the comparative effectiveness of these drugs in the context of other blood conservation strategies.
    Journal of spinal disorders & techniques 10/2013; · 1.21 Impact Factor
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    ABSTRACT: Interest in developing national health care has been increasing in many fields of medicine, including orthopaedics. One manifestation of this interest has been the development of global health opportunities during residency training. We assessed global health activities and opportunities in orthopaedic residency in terms of resident involvement, program characteristics, sources of funding and support, partner site relationships and geography, and program director opinions on global health participation and the associated barriers. An anonymous 24-question survey was circulated to all US orthopaedic surgery residency program directors (n = 153) by email. Five reminder emails were distributed over the next 7 weeks. A total of 59% (n = 90) program directors responded. Sixty-one percent of responding orthopaedic residencies facilitated clinical experiences in developing countries. Program characteristics varied, but most used clinical rotation or elective time for travel (76%), which most frequently occurred during Postgraduate Year 4 (57%) and was used to provide pediatric (66%) or trauma (60%) care. The majority of programs (59%) provided at least some funding to traveling residents and sent accompanying attendings on all ventures (56%). Travel was most commonly within North America (85%), and 51% of participating programs have established international partner sites although only 11% have hosted surgeons from those partnerships. Sixty-nine percent of residency directors believed global health experiences during residency shape future volunteer efforts, 39% believed such opportunities help attract residents to a training program, and the major perceived challenges were funding (73%), faculty time (53%), and logistical planning (43%). Global health interest and activity are common among orthopaedic residency programs. There is diversity in the characteristics and geographical locations of such activity, although some consensus does exist among program directors around funding and faculty time as the largest challenges.
    Clinical Orthopaedics and Related Research 07/2013; · 2.79 Impact Factor
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    ABSTRACT: Heparan sulfate (HS) is an essential component of cell surface and matrix-associated proteoglycans (HSPGs). Due to their sulfation patterns, the HS chains interact with numerous signaling proteins and regulate their distribution and activity on target cells. Many of these proteins, including bone morphogenetic protein family members, are expressed in the growth plate of developing skeletal elements, and several skeletal phenotypes are caused by mutations in HS-synthesizing and modifying enzymes. The disease we discuss here is Hereditary Multiple Exostoses (HME), a disorder caused by mutations in HS synthesizing enzymes EXT1 and EXT2, leading to HS deficiency. The exostoses are benign cartilaginous-bony outgrowths, form next to growth plates, can cause growth retardation and deformities, chronic pain and impaired motion, and progress to malignancy in 2-5% of patients. We describe recent advancements on HME pathogenesis and exostosis formation deriving from studies that have determined distribution, activities and roles of signaling proteins in wild type and HS-deficient cells and tissues. Aberrant distribution of signaling factors combined with aberrant responsiveness of target cells to those same factors appear to be a major culprit in exostosis formation. Insights from these studies suggest plausible and cogent ideas about how HME could be treated in the future. Developmental Dynamics, 2013. © 2013 Wiley Periodicals, Inc.
    Developmental Dynamics 07/2013; · 2.59 Impact Factor
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    ABSTRACT: Study Design: Therapeutic Level II Cohort Study.Objective: To evaluate the safety of adjunctive local application of vancomycin powder (VP) for infection prophylaxis in posterior instrumented thoracic and lumbar spine wounds in pediatric patients >25kg.Summary of Background Data: Spine surgeons have largely turned to vancomycin prophylaxis in an attempt to decrease the incidence of late surgical site infection (SSI) and acute SSI from methicillin resistant Staphylococcus aureus (MRSA). In adult patients the adjunctive local application of VP with an IV cephalosporin has been shown to significantly decrease postsurgical wound infection rates; however, the safety of VP as an adjunct in pediatric spine surgery has not been reported.Methods: We reviewed data collected under a systematic protocol specifically designed to monitor the safety profile of VP. We measured changes in creatinine and systemic vancomycin levels following intrawound application of 500mg of unreconstituted VP during spine deformity correction surgery in patients >25kg (patients also received routine IV cephalosporin prophylaxis). Lab values were measured preoperatively and on postoperative days (POD) 1 and 4. Any adverse reactions and infections through available follow-up (2-8 months) were recorded.Results: 87 consecutive pediatric spinal deformity patients >25kg who received intraoperative VP over a 9 month period were identified. 63% of the patients in this series had adolescent idiopathic scoliosis, 15% congenital scoliosis, 15% neuromuscular scoliosis, and 5% spondylolisthesis. The average change in creatinine levels between the preoperative and POD 1 draw was -0.03 and between the preoperative and POD 4 draw was -0.075. The postoperative systemic vancomycin levels remained undetectable. None of the patients experienced nephrotoxicity or red man syndrome. 3/87 patients developed a SSI.Conclusion: In this cohort there were no clinically significant changes in creatinine level or systemic vancomycin level caused by use of intraoperative VP.
    Spine 06/2013; · 2.16 Impact Factor
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    ABSTRACT: Vascular malformations are abnormal proliferations of the endothelial lining of the blood vessels that are most commonly found in skin and can penetrate the local soft tissue and muscle. Only 1% of all vascular malformations are found in the bone. Skeletal vascular malformations of the long bones, including the humerus, are extremely rare entities that are particularly uncommon in children. Only 4 reports involving the humerus have been described in children previously. We present the case of an intraosseous capillary vascular malformation of the right distal humerus in a 3-year-old boy. Definitive treatment was successful with a single operation using curettage and intramedullary decompression with grafting of an osteoconductive calcium sulfate pellet filler and autogenous bone graft. This is, to our knowledge, the youngest reported case of a solitary intraosseous capillary vascular malformation involving the distal humerus.
    Journal of pediatric orthopedics 06/2013; 33(4):e52-7. · 1.23 Impact Factor
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    ABSTRACT: Chondroblastoma and chondromyxoid fibroma are benign but locally aggressive bone tumors. Chondroblastoma, a destructive lesion with a thin radiodense border, is usually seen in the epiphysis of long bones. Chondromyxoid fibroma presents as a bigger, lucent, loculated lesion with a sharp sclerotic margin in the metaphysis of long bones. Although uncommon, these tumors can be challenging to manage. They share similarities in pathology that could be related to their histogenic similarity. Very rarely, chondroblastoma may lead to lung metastases; however, the mechanism is not well understood.
    The Journal of the American Academy of Orthopaedic Surgeons 04/2013; 21(4):225-33. · 2.46 Impact Factor
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    ABSTRACT: EDUCATIONAL OBJECTIVES As a result of reading this article, physicians should be able to:1.Identify the motivation for vancomycin prophylaxis in clean orthopedic surgery.2.Identify the complications and challenges surrounding vancomycin prophylaxis in clean orthopedic surgery.3.Describe the effectiveness of intravenous vancomycin in clean orthopedic surgery.4.Describe the effectiveness of locally delivered vancomycin in clean orthopedic surgery. Community-acquired methicillin-resistant Staphylococcus aureus (MRSA) has been recognized as a public health concern since the mid-1990s. Because of the increase in reports of this pathogen, it has become increasingly tempting for clinicians to provide prophylaxis against this entity using antibiotics known to be effective against MRSA. The goal of this study was to assess the use of MRSA prophylaxis to determine whether it is safe and effective.A systematic search of the literature was performed to identify articles that examined the use of vancomycin in clean orthopedic surgery. Infection rates and adverse events were extracted, and the data were aggregated and analyzed using a DerSimonian and Laird random effects model. Publication bias and study quality were also assessed. No benefit of parenteral administration of vancomycin was identified. Local, vancomycin-impregnated cement and powder are associated with lower infection rates. Few adverse events occurred, and most of those that occurred involved infusion rate.Cost, resistance, and side effects are concerns in using vancomycin therapy in addition to standard antibiotic prophylaxis. Given the lack of efficacy of intravenous vancomycin, the authors do not recommend its routine use in clean orthopedic surgery. However, local administration appears to be safe and effective. The data are most compelling in orthopedic spine surgery in which a patient without prophylaxis is more than 4 times as likely to have a deep postoperative wound infection compared with a patient who received local vancomycin. The authors recommend the use of local antibiotics when possible in clean orthopedic surgery.
    Orthopedics 02/2013; 36(2):138-46. · 1.05 Impact Factor
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    ABSTRACT: Study Design: Retrospective cohort study using the Pediatric Health Information Systems (PHIS) database.Objective: To describe longitudinal patterns of prophylactic antibiotic use and determinants of antibiotic choice for spinal fusion surgeries performed at US children's hospitals.Summary of Background Data: Surgical Site Infections (SSIs) account for a significant proportion of post-spinal surgery complications, particularly among children with complex conditions such as neuromuscular disease. Antimicrobial prophylaxis with intravenous (IV) cefazolin or cefuroxime has been a standard practice, but postoperative infections caused by organisms resistant to these antibiotics are increasing in prevalence. Studies describing the choice of antibiotic prophylaxis for pediatric spinal surgery are lacking.Methods: We included children 6 months to 18 years of age discharged from 37 US children's hospitals between January 1, 2006 - June 30, 2009 with 1) an ICD9-CM procedure code indicating a spinal fusion and 2) combinations of diagnosis codes indicating adolescent idiopathic scoliosis (AIS, n = 5,617) or neuromuscular scoliosis (NMS, n = 3,633). After identifying antibiotics ordered on the operative day, we described variation in broad-spectrum antibiotic use over time, and measured associations between patient/surgery characteristics and antibiotic choice.Results: Prophylactic antibiotic choice varied across hospitals and over time. Broad-spectrum antibiotics were used in 37% of AIS and 52% of NMS operations. Seven (19%) hospitals used broad spectrum coverage for greater than 80% of all cases. For NMS procedures, broad-spectrum antibiotic use was associated with patient characteristics known to be associated with high SSI risk. Use of vancomycin and broad gram-negative agents increased over time.Conclusion: Broad-spectrum antimicrobial prophylaxis varied across hospitals, and was often associated with known risk factors for SSI. These results highlight the need for future studies comparing the effectiveness of various prophylaxis strategies, particularly in high risk subgroups. This research can inform the development of best practice for SSI prevention in spinal fusion procedures.
    Spine 01/2013; · 2.16 Impact Factor
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    John P Dormans, Sean P Kearney
    European Spine Journal 01/2013; · 2.47 Impact Factor
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    ABSTRACT: Osteochondromas and enchondromas are the most common tumors affecting the skeleton. Osteochondromas can occur as multiple lesions, such as those in patients with hereditary multiple exostoses. Unexpectedly, while studying the role of β-catenin in cartilage development, we found that its conditional deletion induces ectopic chondroma-like cartilage formation in mice. Postnatal ablation of β-catenin in cartilage induced lateral outgrowth of the growth plate within 2 weeks after ablation. The chondroma-like masses were present in the flanking periosteum by 5 weeks and persisted for more than 6 months after β-catenin ablation. These long-lasting ectopic masses rarely contained apoptotic cells. In good correlation, transplants of β-catenin-deficient chondrocytes into athymic mice persisted for a longer period of time and resisted replacement by bone compared to control wild-type chondrocytes. In contrast, a β-catenin signaling stimulator increased cell death in control chondrocytes. Immunohistochemical analysis revealed that the amount of detectable β-catenin in cartilage cells of osteochondromas obtained from hereditary multiple exostoses patients was much lower than that in hypertrophic chondrocytes in normal human growth plates. The findings in our study indicate that loss of β-catenin expression in chondrocytes induces periosteal chondroma-like masses and may be linked to, and cause, the persistence of cartilage caps in osteochondromas.
    American Journal Of Pathology 12/2012; · 4.60 Impact Factor
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    ABSTRACT: PURPOSEChemotherapy with alternating vincristine-doxorubicin-cyclophosphamide and ifosfamide-etoposide cycles and primary tumor treatment with surgery and/or radiation therapy constitute the usual approach to localized Ewing sarcoma in North America. We tested whether chemotherapy intensification through interval compression could improve outcome. PATIENTS AND METHODS This was a prospective, randomized controlled trial for patients younger than 50 years old with newly diagnosed localized extradural Ewing sarcoma. Patients assigned to standard and intensified treatment were to begin chemotherapy cycles every 21 and 14 days, respectively, provided an absolute neutrophil count greater than 750 × 10(6)/L and a platelet count greater than 75 × 10(9)/L. Patients received vincristine (2 mg/m(2)), doxorubicin (75 mg/m(2)), and cyclophosphamide (1.2 g/m(2)) alternating with ifosfamide (9 g/m(2)) and etoposide (500 mg/m(2)) for 14 cycles, with filgrastim (5 mg/kg per day; maximum, 300 mg) between cycles. Primary tumor treatment (surgery, radiation, or both) was to begin at week 13 (after four cycles in the standard arm and six cycles in the intensified arm). The primary end point was event-free survival (EFS). The study is registered at (identifier: NCT00006734).ResultsFive hundred eighty-seven patients were enrolled and randomly assigned, and 568 patients were eligible, with 284 patients in each regimen. For all cycles, the median cycle interval for standard treatment was 21 days (mean, 22.45 days); for intensified treatment, the median interval was 15 days (mean, 17.29 days). EFS at a median of 5 years was 65% in the standard arm and 73% in the intensified arm (P = .048). The toxicity of the regimens was similar. CONCLUSION For localized Ewing sarcoma, chemotherapy administered every 2 weeks is more effective than chemotherapy administered every 3 weeks, with no increase in toxicity.
    Journal of Clinical Oncology 10/2012; · 18.04 Impact Factor
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    ABSTRACT: Quantity of citation is often used as a surrogate measurement of an article's importance or relevance in a given field. To date, there has been no study on citation quantity or density in pediatric orthopedics. We present such an analysis. We reviewed all articles in the ISI web of science under the subheading 'orthopedics' to find articles of relevance to pediatric orthopedic surgeons. We subsequently analyzed the characteristics of the most cited articles in terms of the level of evidence, journal of publication, and subcategory of pediatric orthopedics. The majority of the top 50 articles in pediatric orthopedics were from the 1970s and 1980s. All the articles were in English. Uncontrolled case series comprised the largest single level of evidence (level IV). We describe the top 50 articles in pediatric orthopedics by citation and citation density. The level of evidence for highly cited papers was low. Although many of these articles were written by the great thinkers of our field, a need exists for more rigorous methodology.
    Journal of pediatric orthopaedics. Part B / European Paediatric Orthopaedic Society, Pediatric Orthopaedic Society of North America 05/2012; 21(5):463-8. · 0.66 Impact Factor
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    ABSTRACT: Congenital scoliosis, a lateral curvature of the spine caused by vertebral defects, occurs in approximately 1 in 1,000 live births. Here we demonstrate that haploinsufficiency of Notch signaling pathway genes in humans can cause this congenital abnormality. We also show that in a mouse model, the combination of this genetic risk factor with an environmental condition (short-term gestational hypoxia) significantly increases the penetrance and severity of vertebral defects. We demonstrate that hypoxia disrupts FGF signaling, leading to a temporary failure of embryonic somitogenesis. Our results potentially provide a mechanism for the genesis of a host of common sporadic congenital abnormalities through gene-environment interaction.
    Cell 04/2012; 149(2):295-306. · 31.96 Impact Factor
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    ABSTRACT: Intrathecal morphine (ITM) provides effective analgesia after posterior spinal fusion (PSF). Although most anesthetic drugs have well-characterized effects on evoked potentials, there is little data on the effects of ITM on transcranial electric motor-evoked potentials (tceMEPs). We performed this study to assess the effects of ITM on tceMEPs in the first 30 minutes after administration. We hypothesized that administration of ITM in doses currently used at our institution would not significantly affect mean tceMEP amplitudes and latencies of an ITM study group relative to control patients who did not receive the drug. tceMEPs were recorded before ITM injection and 5, 10, 20, and 30 minutes after injection in 14 subjects ages 11 through 18 years undergoing PSF. These recordings were compared to an age-matched control group undergoing PSF in which ITM was not injected. The effects of ITM on tceMEP amplitude and latency were compared between the 2 groups. Fourteen subjects were enrolled in the ITM group and 16 served as controls. There were no significant differences in the baseline mean response amplitudes of the 2 groups for any of the 8 muscles studied. Mean response amplitudes over the 30-minute posttreatment period in the ITM group did not differ significantly from those of the control subjects. Average response amplitudes collapsed across all muscles for each subject were not significantly different during the baseline period (95% CI = -38% to 45%; P = 0.783), nor were they significantly different between the 2 groups during the posttreatment period (95% CI = -30% to 78%; P = 0.640). There also were no significant differences in the mean response latencies of the 2 groups in either the baseline or posttreatment periods. Average response latencies collapsed across all muscles for each subject were 4% larger for the ITM group than for controls during the baseline period (95% CI = -5% to 13%; P = 0.377), and 3% larger for the ITM group than for controls during the posttreatment period (95% CI = -4% to 12%; P = 0.359). Administration of ITM in doses currently used at our institution did not cause more than a 70% attenuation of mean tceMEP amplitudes or latency changes of an ITM study group relative to control subjects during the 30-minute period after injection. Further studies are required to determine if there are delayed effects after this initial time period.
    Anesthesia and analgesia 03/2012; 115(1):160-9. · 3.08 Impact Factor
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    ABSTRACT: Focused review of the literature. Assist spine specialists in diagnosis and treatment of cervical spine anomalies found in selected genetic syndromes. Cervical spine instability and/or stenosis are potentially debilitating problems in many genetic syndromes. These problems can be overlooked among the other systemic issues more familiar to clinicians and radiologists evaluating these syndromes. It is imperative that spine specialists understand the relevant issues associated with these particular syndromes. The literature was reviewed for cervical spine issues in 10 specific syndromes. The information is presented in the following order: First, the identification and treatment of midcervical kyphosis in Larsen syndrome and diastrophic dysplasia (DD). Next, the upper cervical abnormalities seen in Down syndrome, 22q11.2 Deletion syndrome, pseudoachondroplasia, Morquio syndrome, Goldenhar syndrome, spondyloepiphyseal dysplasia congenita, and Kniest dysplasia. Finally, the chin-on-chest deformity of fibrodysplasia ossificans progressiva. Midcervical kyphosis in patients with Larsen syndrome and DD needs to be evaluated and imaged often to track deformity progression. Upper cervical spine instability in Down syndrome is most commonly caused by ligamentous laxity at C1 to C2 and occiput-C1 levels. Nearly 100% of patients with 22q11.2 deletion syndrome have cervical spine abnormalities, but few are symptomatic. Patients with pseudoachondroplasia and Morquio syndrome have C1 to C2 instability related to odontoid dysplasia (hypoplasia and os odontoideum). Morquio patients also have soft tissue glycosaminoglycan deposits, which cause stenosis and lead to myelopathy. Severely affected patients with spondyloepiphyseal dysplasia congenita are at high risk of myelopathy because of atlantoaxial instability in addition to underlying stenosis. Kniest syndrome is associated with atlantoaxial instability. Cervical spine anomalies in Goldenhar syndrome are varied and can be severe. Fibrodysplasia ossificans progressiva features severe, deforming heterotopic ossification that can become life-threatening. It is important to be vigilant in the diagnosis and treatment of cervical spine anomalies in patients with genetic syndromes.
    Spine 10/2011; 37(5):E269-77. · 2.16 Impact Factor
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    ABSTRACT: A multicenter retrospective analysis. To evaluate outcomes of closed-suction wound drainage after posterior spinal fusion with instrumentation for adolescent idiopathic scoliosis and to identify surgeon patterns of drain use in this cohort. There is little evidence on the use of drains in spinal surgery, particularly for repair of adolescent idiopathic scoliosis. Studies on hip and knee arthroplasty suggest no advantage to draining. There are few published reports on surgeon technique and rationale for drain use in spinal surgery. Patients were divided into drain and no drain cohorts and followed for 2 years. Primary outcome was complication rate. A separate survey was conducted from surgeons in the Spinal Deformity Study Group to evaluate drain practice patterns. There were 324 drained and 176 undrained patients. Complication rate did not differ between the drain and no drain cohorts in any of the 4 categories (wound infection, neural injury, other infection, and other complication) at any time (all P > 0.1). More drained patients received postoperative transfusions compared with those without a drain (43% vs. 22%, P < 0.001). Of the 50 surgeons in the group, 36 used drains. Half of these did so out of habit. Surgeons tended to place deep drains with bulb suction, without drain manipulation. Half removed drains on the basis of output, whereas half removed them after 1 to 3 days. More patients tended to receive wound drains than not receive wound drains. Drains did not impact complication rate and drained patients received more blood product. There are no universal criteria for draining and practice patterns vary widely.
    Spine 10/2011; 37(11):966-73. · 2.16 Impact Factor
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    ABSTRACT: Lenz microphthalmia syndrome is an X-linked recessive disorder characterized by microphthalmia and dental, urogenital, and skeletal anomalies. This case report represents the first detailed account of congenital kyphoscoliosis in the Lenz microphthalmia literature. We present a case of Lenz microphthalmia syndrome with progressive kyphosis, spinal stenosis, and late-onset tibia vara along with many of the typical features of the disorder. In addition, we provide insight into the syndrome by reviewing the existing Lenz microphthalmia literature. Congenital kyphoscoliosis that is prone to reoccurrence after posterior spinal fusion is an unusual entity that may be associated with Lenz microphthalmia. We recommend close monitoring and early surgical intervention with posterior spinal fusion for congenital kyphosis in patients diagnosed with Lenz microphthalmia syndrome. However, more data on similar patients are necessary to define the optimal treatment strategy. LEVEL OF EVIDENCE (FOR CLINICAL ARTICLES): Level V.
    Journal of pediatric orthopedics 09/2011; 31(6):e64-9. · 1.23 Impact Factor

Publication Stats

1k Citations
382.99 Total Impact Points


  • 2004–2014
    • University of Pennsylvania
      • • Department of Orthopaedic Surgery
      • • Department of Medicine
      Philadelphia, Pennsylvania, United States
    • Temple University
      Philadelphia, Pennsylvania, United States
  • 2013
    • University of North Carolina at Chapel Hill
      • Department of Orthopaedics
      Chapel Hill, NC, United States
  • 2002–2013
    • The Children's Hospital of Philadelphia
      • Department of Orthopaedic Surgery
      Philadelphia, PA, United States
    • Marmara University
      İstanbul, Istanbul, Turkey
  • 2011
    • University of Rochester
      • Department of Orthopaedics and Rehabilitation
      Rochester, NY, United States
  • 2002–2011
    • Hospital of the University of Pennsylvania
      • Department of Orthopaedic Surgery
      Philadelphia, Pennsylvania, United States
  • 2010
    • University of California, San Diego
      San Diego, California, United States
  • 2009–2010
    • Rush University Medical Center
      • Department of Orthopaedic Surgery
      Chicago, Illinois, United States
    • Children's Hospital Los Angeles
      Los Angeles, California, United States
    • Istanbul Surgery Hospital
      İstanbul, Istanbul, Turkey
  • 2005–2007
    • Washington University in St. Louis
      • Department of Orthopaedic Surgery
      Saint Louis, MO, United States
  • 2006
    • University of Wisconsin–Madison
      Madison, Wisconsin, United States
    • Concordia University–Ann Arbor
      Ann Arbor, Michigan, United States
  • 2003
    • University of Pittsburgh
      • Department of Orthopaedic Surgery
      Pittsburgh, PA, United States
    • Harvard Medical School
      Boston, Massachusetts, United States