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Cranial nerve involvement in Charcot-Marie-Tooth disease (CMT) is rare, though there are a number of CMT syndromes in which vocal cord paralysis is a characteristic feature. CMT disease due to mutations in the ganglioside-induced differentiation-associated protein 1 gene (GDAP1) has been reported to be associated with vocal cord and diaphragmatic palsy. In order to address the prevalence of these complications in patients with GDAP1 mutations we evaluated vocal cord and respiratory function in nine patients from eight unrelated families with this disorder. Hoarseness of the voice and inability to speak loudly were reported by eight patients and one had associated symptoms of respiratory insufficiency. Patients were investigated by means of peripheral and phrenic nerve conduction studies, flexible laryngoscopy, pulmonary function studies and polysomnography. Nerve conduction velocities and pathological studies were compatible with axonal CMT (CMT2). Flexible laryngoscopy showed left vocal cord palsy in four cases, bilateral cord palsies in four cases and was normal in one case. Restrictive respiratory dysfunction was seen in the eight patients with vocal cord paresis who were all chair-bound. These eight had confirmed phrenic nerve dysfunction on neurophysiology evaluation. The patient with normal vocal cord and pulmonary function had a less severe clinical course.This study shows that CMT patients with GDAP1 mutations develop severe disability due to weakness of limb muscles and that laryngeal and respiratory muscle involvement occurs late in the disease process when significant proximal upper limb weakness has developed. The early and predominant involvement of the left vocal cord innervated by the longer left recurrent laryngeal nerve suggests a length dependent pattern of nerve degeneration. In GDAP1 neuropathy, respiratory function should be thoroughly investigated because life expectancy can be compromised due to respiratory failure.

Objectives: Imaging assessment for the clinical management of femoroacetabular impingement syndrome (FAIS) remains controversial because of a paucity of evidencebased guidance and notable variability in clinical practice, ultimately requiring expert consensus. The purpose of this agreement is to establish expert-based statements on FAIS imaging, using formal techniques of consensus building driven by relevant literature review. Methods:The validated Delphi method and peer-reviewed literature were used to formally derive consensus among 30 panel members (21 musculoskeletal radiologists and 9 orthopedic surgeons) from 13 countries.Forty-two questions were agreed on, and recent relevant seminal literature was circulated and classified in five major topics ("General issues", "Parameters and reporting", "Radiographic assessment", "MRI evaluation" and "Ultrasound") in order to produce answering statements.The level of evidence was noted for all produced statements and panel members were asked to score their level of agreement with each statement (0 to 10) during iterative rounds. Either "group consensus", "group agreement" or "no agreement" was achieved.Items near consensus were further queried using 4 moderated group sessions and in 4 Delphi rounds.Results: Forty-five statements were generated and group consensus was reached for 43 (95.7%). Seventeen of these statements were selected as most important for dissemination in advance. There was no agreement for the two statements pertaining to "Ultrasound". Conclusion:The first international Delphi-based consensus for the imaging assessment of FAIS was developed. The resulting consensus can serve as a tool to reduce variability in clinical practices and guide further research for the clinical management of FAIS. Key Points• FAI imaging literature is extensive although often of low level of evidence.• Radiographic evaluation with a reproducible technique is the cornerstone of hip imaging assessment.• MRI with a dedicated protocol is the gold standard imaging technique for FAI assessment.

Bone nonunion in the pediatric population usually occurs in the context of highly unfavorable biological conditions. Recently, the vascularized fibular periosteal flap has been reported as a very effective procedure for treating this condition. Even though a vascularized tibial periosteal graft (VTPG) was described long ago and has been successfully employed in one adult case, there has been no other report published on the use of this technique. We report on the use of VTPG, pedicled in the anterior tibial vessels, for the treatment of two complex pediatric bone nonunion case: a recalcitrant supracondylar femoral pseudarthrosis secondary to an infection in an 11-year-old girl, and a tibial nonunion secondary to a failed bone defect reconstruction in a 12-year-old girl. Rapid healing was obtained in both cases. In the light of the data presented, we consider VTPG as a valuable surgical option for the treatment of complex bone nonunions in children.

Aims: Imaging assessment for the clinical management of femoroacetabular impingement (FAI) syndrome is controversial because of a paucity of evidence-based guidance and notable variability among practitioners. Hence, expert consensus is needed, because standardised imaging assessment is critical for clinical practice and research. We aimed to establish expert-based statements on FAI imaging by using the formal methods of consensus-building. This is the second part of a three-part consensus series, and focuses on 'General issues' and 'Parameters and reporting'. Methods:The Delphi method was used to formally derive consensus among 30 panel members from 13 countries. Forty-four questions were agreed upon, and relevant seminal literature was circulated and classified in major topics ('General issues', 'Parameters and reporting', 'Radiographic assessment', 'Magnetic resonance imaging (MRI)' and 'Ultrasound') to produce answering statements. The level of evidence was noted for all statements, and panel members were asked to score their level of agreement (0-10). Either 'group consensus', 'group agreement', or 'no agreement' was achieved.Results: Forty-seven statements were generated and group consensus was reached for 45. Twenty-five statements pertaining to 'General issues' (nine addressing diagnosis, differential diagnosis and postoperative imaging) and 'Parameters and reporting' ( 16addressing femoral/acetabular parameters) were produced. Conclusions:The first international Delphi-based consensus on FAI imaging was developed. The available evidence was reviewed critically, recommended criteria for diagnostic imaging highlighted, and the roles of different imaging parameters assessed.The resulting statements can serve as a tool for practitioners working with hip-related pain to reduce clinical variability and guide further research for FAI management. Key points Radiographic evaluation (anteroposterior radiograph of the pelvis and a lateral view of the hip, preferably a Dunn 45° view) with a reproducible methodology is the cornerstone of hip-imaging assessment and minimum imaging study that should be performed when assessing adult patients for FAI. In selected cases, cross-sectional imaging is warranted because MRI with a dedicated protocol is the 'gold standard' imaging modality for the comprehensive evaluation, differential diagnoses assessment, and surgical planning of FAI. For acetabular morphology, coverage (centre-edge angle of Wiberg and acetabular index) and version (crossover, posterior wall, and ischial spine signs) should be assessed routinely. On the femoral side, the morphology of the head-neck junction (alpha angle and offset), neck morphology (neck-shaft angle) and torsion (antetorsion angle) should be assessed routinely. ClinicalRelevance  Imaging assessment for FAI is unstandardised because of a paucity of evidencebased guidance and lack of consensus among experts on which imaging modalities, diagnostic criteria, and parameters should be used/assessed routinely. This Delphibased consensus, aims t...

Field and laboratory-column studies were undertaken in order to investigate soil contamination derived from past mining activity in the Sierra Almagrera (SA) district in southeast Spain. The tailings, soil and sediment samples that were collected showed high concentrations of Ag, As, Ba, Cu, Pb, Sb and Zn when analyzed. The mean concentrations of these elements in the tailings were 29.8, 285.4, 54000, 57.7, 2687.5, 179.0 and 2269.0 ppm, respectively. In the soil samples these decreased to 14.3, 96.9, 24700, 37.5, 1859.1, 168.5 and 815.7 ppm, respectively. Geochemical analyses demonstrated high levels of As, Pb and Zn which were above the intervention values set forth in the Andalusian Regulations for Contaminated Soils for As (>50 ppm), Pb (>500 ppm) and Zn (>2000 ppm). Column experiments and mineralogical studies suggest that the dissolution of sulfates and other secondary phases, accumulated in soils and waste-sites during the dry season, acts to control the mobility of metals. The elution curves obtained from column experiments showed a mobilization of Ba, Cu, Pb and Zn, while a low mobility was seen for Ag, As and Sb.

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The information is taken from normal hips and may not be directly applicable to the deformed hip. Nevertheless, it is a prerequisite for a surgeon to understand the normal anatomy and use those boundaries to prevent mistakes during intra-articular joint-preserving hip surgical procedures.