The Occurrence of Occult Acetabular Dysplasia in Relatives of Individuals With Developmental Dysplasia of the Hip.

BACKGROUND: This study sought to determine the hip pathology of family members of patients with developmental dysplasia of the hip (DDH). The authors evaluated 120 people from 19 families known to have at least 1 member with surgically treated DDH. Each individual's functional outcome scores and pelvic radiographs were assessed for hip symptoms or pathology. METHODS: Using a genetic population database and a pediatric hospital patient population, 19 families with high rates of DDH were identified. All family members (n=120) underwent physical examination, radiographic assessment, and completion of outcome instruments [American Academy of Orthopedics (AAOS) Hip and Knee; Harris Hip Score (HHS); and Western Ontario and McMaster Universities Arthritis Index (WOMAC)]. RESULTS: The 120 subjects ranged from 1 to 84 years, 34 had orthopaedically treated DDH. Of the remaining 86 supposedly normal subjects, 23 (27%) had occult acetabular dysplasia (OAD) as defined by center edge angle (CEA) <20 and/or a Severin score of III or greater. Sixty percent of the 86 individuals were less than 30 years old, 74% of the OAD group were less than 30. Outcome scores of the treated DDH patients (AAOS, HHS, and WOMAC) were worse on the involved side regardless of age. Over age 30 individuals with OAD had statistically significant decreases in their AAOS Hip and Knee and WOMAC scores on the dysplastic side, but their HHS scores were not significantly different. CONCLUSIONS: Twenty-seven percent of first-degree and second-degree relatives of patients with DDH had unsuspected radiographic acetabular dysplasia in our study. Most of the subjects with OAD were younger than 30. After age 30, many of these patients developed symptoms. CLINICAL RELEVANCE: In families with a significant history of DDH, radiographic screening of siblings of patients with DDH to define OAD may be prudent. LEVEL OF EVIDENCE: Level I­diagnostic study.

Long-term follow-up of open reduction surgery for developmental dislocation of the hip.

BACKGROUND: We posed 2 questions: what is the long-term result of open reduction surgery in developmental dysplasia of the hip, and is there an age at surgery above which the outcome was too poor to recommend the operation? METHODS: Between 1955 and 1995, 148 patients with 179 dislocated hips had open reduction surgery for developmental dysplasia of the hip (141 anterior and 38 Ludloff medial approaches). We attempted to locate all 148 patients for the follow-up evaluation. RESULTS: Fifty-three patients (36%) with 66 hips (37%) were located and participated in the study. These 66 hips represented 34% of the anterior open reductions and 47% of the Ludloff medial reductions. Twenty-two of the 66 hips had Severin IV or worse outcomes and included 7 with total hip arthroplasties and 2 with hip fusions. Age at surgery was significantly lower for Severin I, II, and III, compared with Severin IV and above (P=0.003, 0.001, 0.003) with outcomes deteriorating substantially after age 3. Approximately half of the hips required further surgery for dysplasia. All hips that sustained osseous necrosis had Severin IV or worse outcomes, and hips that redislocated and required revision surgery only achieved Severin I or II ratings 18% of the time. Nine "normal" hips became dysplastic and 3 had pelvic osteotomies as teenagers. Two other normal hips developed osseous necrosis during treatment of the contralateral hip. CONCLUSIONS: Results deteriorate as the age at surgery increases. Osseous necrosis and redislocation predict a poor functional and radiographic result. The "normal" hip may develop insidious dysplasia and also may be injured during treatment of the involved hip. Above age 3, some patients may not have sufficient acetabular growth to remodel a surgically reduced hip. LEVEL OF EVIDENCE: Level IV--case series.

Measurement of the center edge angle and determination of the Severin classification using digital radiography, computer-assisted measurement tools, and a Severin algorithm: intraobserver and interobserver reliability revisited.

BACKGROUND: Numerous studies underscore the poor intraobserver and interobserver reliability of both the center edge angle (CEA) and the Severin classification using plain film measurements. In this study, experienced observers applied a computer-assisted measurement program to determine the CEA in digital pelvic radiographs of adults who had been previously treated for dysplasia of the hip (DDH). Using a teaching aid/algorithm of the Severin classification, the observers then assigned a Severin rating to these hips. Intraobserver and interobserver errors were then calculated on both the CEA measurements and the Severin classifications. METHODS: Four pediatric orthopaedic surgeons and 1 pediatric radiologist calculated the CEAs using the OrthoView TM planning system and then determined the Severin classification on 41 blinded digital pelvic radiographs. The radiographs were evaluated by each examiner twice, with evaluations separated by 2 months. All examiners reviewed a Severin classification algorithm before making their Severin assignments. The intraobserver and interobserver reliability for both the CEA and the Severin classification were calculated using the interclass correlation coefficients and Cohen and Fleiss κ scores, respectively. RESULTS: The intraobserver and interobserver reliability for CEA measurement was moderate to almost perfect. When we separated the Severin classification into 3 clinically relevant groups of good (Severin I and II), dysplastic (Severin III), and poor (Severin IV and above), our interobserver reliability neared almost perfect. CONCLUSION: The Severin classification is an extremely useful and oft-used radiographic measure for the success of DDH treatment. Our research found digital radiography, computer-aided measurement tools, the use of a Severin algorithm, and separating the Severin classification into 3 clinically relevant groups significantly increased the intraobserver and interobserver reliability of both the CEA and Severin classification. This finding will assist future studies using the CEA and Severin classification in the radiographic assessment of DDH treatment outcomes.

Delta9-tetrahydrocannabivarin testing may not have the sensitivity to detect marijuana use among individuals ingesting dronabinol.

The purpose of this study was to determine whether Delta(9)-tetrahydrocannabivarin (THCV), a plant cannabinoid, is a sensitive measure to detect recent marijuana use in cannabis dependent patients. It has been purported that smoking an illicit plant cannabis product will result in a positive THCV urinalysis, whereas the oral ingestion of therapeutic THC such as dronabinol will result in a negative THCV urinalysis, allowing for discrimination between pharmaceutical THC products and illicit marijuana products. In a double-blind placebo-controlled trial to determine the efficacy of dronabinol in cannabis dependence, all 117 patients produced a positive urine for the marijuana metabolite 11-nor-Delta(9)-THC-9-carboxylic acid; THC-COOH, but 50% had an undetectable (<1 ng/ml) THCV-COOH test. This suggests that THCV may not be a sensitive enough measure to detect recent marijuana use in all heavy marijuana users or that its absence may not discriminate between illicit marijuana use and oral ingestion of THC products such as dronabinol. We propose that the lack of THCV detection may be due to the variability of available cannabis strains smoked by marijuana users in community settings.

Analysis of radiographic characteristics of anterolateral bowing of the leg before fracture in neurofibromatosis type 1.

BACKGROUND: Anterolateral leg bowing is associated with neurofibromatosis type 1 (NF1) frequently leading to fracture and nonunion of the tibia. The objective of the study was to characterize the radiographic findings of tibial dysplasia in NF1. METHODS: This study is a retrospective review of radiographs of tibial dysplasia obtained within 52 years, between 1950 and 2002, from the Shriners Hospitals for Children, Salt Lake City, and of peripheral quantitative computed tomographic images of 3 individuals with anterolateral bowing of the leg without fracture compared with age- and sex-matched controls. RESULTS: Individuals with NF1 with bowing of the leg have the appearance of thicker cortices with medullary narrowing on plain film radiographs. The peripheral quantitative computed tomographic images of individuals with NF1 with anterolateral bowing show an unusual configuration of the tibia. CONCLUSIONS: Anterolateral bowing of the leg in NF1 is associated with the appearance of thicker cortices with medullary narrowing rather than thinning of the long bone cortex on plain film radiographs as currently used as a qualifier in the sixth diagnostic criterion for the clinical diagnosis of NF1. Individuals with NF1 who have anterolateral bowing of the leg have differences in tibial geometry compared with age- and sex-matched controls. CLINICAL RELEVANCE: The characterization of the radiographic findings of long bone bowing in NF1 helps clarify the NF1 clinical diagnostic criteria.

The use of anterolateral bowing of the lower leg in the diagnostic criteria for neurofibromatosis type 1.

Neurofibromatosis type 1 is diagnosed clinically based on the presence of two of seven criteria developed by a panel of experts in 1987. The sixth criterion focuses on skeletal findings and is as follows: "A distinctive osseous lesion such as sphenoid dysplasia or thinning of long bone cortex, with or without pseudarthrosis." The wording for this criterion is misleading. In particular, "thinning of long bone cortex" is not the characteristic radiographic presentation, and no mention of long bone bowing is included. The distinctive clinical feature of long bone dysplasia in neurofibromatosis type 1 is anterolateral bowing of the lower leg (portion of the body delimited by the knee and ankle). The usual radiographic findings of long bone dysplasia in neurofibromatosis type 1 at first presentation, prior to fracture, are anterolateral bowing with medullary canal narrowing and cortical thickening at the apex of the bowing. We suggest that anterolateral bowing of the lower leg, with or without fracture or pseudarthrosis, is a more appropriate description of the primary finding that a clinician will use to fulfill the sixth diagnostic criterion for neurofibromatosis type 1. Clarification of this diagnostic criterion is important for the clinician and for research protocols. Appropriate interpretation will improve understanding of the natural history and pathophysiology of neurofibromatosis type 1.

Preparation of animals for research--issues to consider for rodents and rabbits.

This article provides details to consider when preparing to use animals in biomedical research. The stress of transport and receipt of animals into a new environment mandate the need for a period of stabilization and acclimation. This allotment of time often occurs in conjunction with the quarantine period and permits a stress "recovery" period. Discussions in the article include specific effects of the environment on the animal, such as housing and environmental enrichment. Suggestions are offered regarding how to minimize the effects of procedures and equipment through the use of preconditioning techniques. Guidelines for these techniques and for acclimation should be instituted by the institutional animal care and use committee. Stress and distress are placed in perspective as they relate to the preparation of laboratory animals for research.

Phenotyping of genetically engineered mice: humane, ethical, environmental, and husbandry issues.

The growing use of genetically engineered (GE) mice in scientific research has raised many concerns about the animal welfare of such mice. The types of welfare concerns may differ within the three stages that comprise the establishment of GE animal models: development, production, and research use. The role and impact of the members of the research team on these concerns may also vary with each stage. To make both scientific and animal welfare decisions at each stage, it is necessary to have a thorough knowledge of the animal model-in this case, the phenotypic expression of the GE animal. Phenotype screening is the analysis of visible or measurable characteristics of an animal that result from the genotype and its interaction with the environment. Phenotypes expressed that are relevant to the research program are usually carefully investigated; however, those that may have an impact on the animal's welfare but have little or no impact on the disease process under study are often less carefully studied. Thorough analysis and documentation of the animal welfare aspects of phenotype provide the research team with the information they need to control the environment to minimize negative animal welfare effects. Such information is also essential to allow members of the institutional animal care and use committee to perform necessary cost:benefit ethical review of proposed GE animal studies. Investigators who submit information about models for publication should document all aspects of a phenotype, including the area of scientific interest as well as those areas that affect animal welfare, for clarity and for subsequent research with the respective models.

Breeding genetically modified rodents: tips for tracking and troubleshooting reproductive performance.

Infertility or reduced fertility in genetically modified mouse strains represents a serious bottleneck for planned research projects. The authors describe simple methods for troubleshooting poor reproductive performance in breeding colonies and introduce various means of assisted reproduction that might be used to 'rescue' infertile lines.

Radiographic approach to multifocal consolidation.

Consolidation in the lung is seen on radiographs or computed tomography (CT) as increased areas of attenuation that obscure the underlying pulmonary vasculature. There are numerous causes of multifocal consolidative opacities. If the symptoms are acute (days to weeks), the most common causes include edema, pneumonia, and hemorrhage. Depending on the patient's history, signs, and symptoms, the less common causes such as radiation pneumonitis or acute eosinophilic syndrome may be considered. If the symptoms are more chronic (weeks to months), the differential may include alveolar proteinosis, neoplasms such as lymphoma or bronchoalveolar cell carcinoma, granulomatous or inflammatory conditions, and lipoid pneumonia. In this article, we review and discuss characteristic radiographic and clinical findings that can aid the radiologist in prioritizing the differential considerations when faced with multifocal parenchymal consolidative disease.