Lesions of the lateral pterygoid muscle-an overestimated reason for temporomandibular dysfunction: a 3T magnetic resonance imaging study.

The aim of this study was to examine the lateral pterygoid muscle (LPM) parenchyma, myotendinous junction, and tendon in temporomandibular disorder (TMD) patients using 3T magnetic resonance imaging (MRI). Results were compared with findings reported in the literature, in which the LPM has been attributed a major role in triggering TMD. 3T MRI was used for temporomandibular joint (TMJ) imaging. The MRI images of 63 patients were analysed for muscle contracture and atrophy, tendon rupture, signal alterations of the tendon, tendon contrast enhancement, and peritendinous fluid collection. Descriptive statistics and the coefficient estimate method were used for statistical analysis. Focus was placed on the association between LPM tendon pathology and TMJ lesions like osteoarthritis and disc displacement. Severe lesions of the LPM tendon and muscle parenchyma, like rupture or fibrosis, were detected in very few cases. Only moderate signs of tendinosis were found in TMD patients. In contrast, there was a clear correlation between tendon lesions and osteoarthritis or anterior disc displacement. These results indicate the need to discuss and question the role of the LPM and its tendon in TMD. Data suggest that LPM and tendon lesions are part of complex degenerative changes of the TMJ, and it seems less likely that a LPM disorder is causative in TMD.

Growth modulation in idiopathic angular knee deformities: is it predictable?

PURPOSE: To evaluate the temporal and spatial sequence of events following temporal hemiepiphysiodesis in idiopathic knee varus/valgus. METHODS: This is a retrospective multicentre study on 372 physes in 206 patients. The average rate of correction (ROC) was calculated; univariate and multivariate analysis were performed. RESULTS: In all, 92% of the femoral physes were followed for more than one year/reached skeletal maturity. Of those, 93% were corrected to a mechanical lateral distal femoral angle (mLDFA) of 85° to 89°; 2% did not, while 5% were over-corrected. A total of 92% of the tibial physes were followed for more than one year/reached skeletal maturity. Of those, 92% were corrected to a mechanical medial proximal tibial angle (mMPTA) of 85° to 89°; 2% did not, while 6% were over-corrected. Factors significantly influencing success and ROC were age, direction and magnitude of deformity. Femoral ROC was significantly faster than tibial ROC: 0.85° versus 0.78°/month, respectively (p = 0.05). Femoral valgus ROC was significantly faster than varus ROC: 0.90° versus 0.77°/month, respectively (p = 0.04). A constant was derived to calculate the amount of correction. Significant correlation was found between calculated and actual mLDFA in valgus deformity during the first year (r = 0.58 to 0.87, p < 0.01). Calculated mLDFA of varus deformity did not correlate with actual mLDFA. Significant correlation was found when calculating mMPTA correction in all deformities. CONCLUSIONS: Femur corrects faster than tibia; valgus femoral deformities are corrected faster than varus. Valgus correction in the distal femur/proximal tibia as well as varus correction in the tibia in idiopathic patients is highly predictable. The constant derived is the first tool which enables predicting and monitoring amount of correction in hemiepiphysiodesis when correcting angular deformities around the knee. LEVEL OF EVIDENCE: IV.

Guided growth: preliminary results of a multinational study of 967 physes in 537 patients.

BACKGROUND: Guided growth by tension band plating is commonly used to correct coronal plane deformity. The purpose of this study was to measure the effect and further define parameters that influence results in coronal plane deformity around the knee. METHODS: The retrospective multicentre study included data on 967 physes in 537 patients, with an average follow-up of 16 months after plate insertion. Alignment analysis was compared preoperatively and in at least two measurements postoperatively, as well as with parameters that influence the rate and amount of correction. RESULTS: Average age at plate implantation was 11.35 years (SD 3.29).Of those with femoral deformities, 85% of the patients finished the treatment and of those, 70% were corrected to standard alignment, while 14% have not yet achieved correction, and are still growing.Of those with tibial deformities, 75% of the patients finished the treatment and of those 80% were corrected to standard alignment, while 25% have not yet achieved correction and are still growing.The calculated rate of correction was 0.77°/month for the femur and 0.79°/month for the tibia.In terms of complications, the overall rate of infection was 1.48%. In three patients (0.55%) screw breakage was recorded.Factors found to significantly influence the amount of correction were age at plate implantation and direction of deformity. CONCLUSION: Temporary hemiepiphysiodesis takes the advantage of physiological physeal growth to effectively treat angular deformities. Success of treatment is influenced by the age of the patient at plate implantation and direction of deformity. LEVEL OF EVIDENCE: IV.

[Congenital tibial hemimelia]. / Die kongenitale Tibiahemimelie.

BACKGROUND: The congenital tibia hemimelia, also called tibial deficiency, is a rare disorder with unknown cause, showing many associated abnormalities or varying syndromes. METHODS: The correct diagnosis can be easily established using radiographs and/or magnetic resonance imaging in the postpartum setting. However, treatment may be difficult and needs to take into consideration the given anatomic situation in the knee and ankle joint. CONCLUSION: Prosthetic fitting may be possible in mild cases. Nevertheless, the majority of patients need to undergo surgical reconstruction in order to restore a functional, mobile, and stable knee and ankle joint.

Open surgery versus nonoperative treatments for paediatric trigger thumb: a systematic review.

The purpose of this systematic review was to determine the outcome of interphalangeal (IP) joint motion in children undergoing open surgical release, splinting, and passive exercising therapy for the treatment of paediatric trigger thumb. We conducted an online literature search of seven major databases. Only studies with a mean follow-up of at least 12 months were considered for inclusion. Seventeen retrospective studies and one prospective study met all the inclusion criteria. They reported on the results of surgery (634 children, 759 thumbs), splinting (115 children, 138 thumbs), and passive exercising (89 children, 108 thumbs). The mean follow-up periods were 59 (surgery), 23 (splinting), and 76 months (exercising), respectively. Full IP joint motion without residual triggering was achieved in 95% of all children undergoing surgery, in 67% of children treated with continuous splinting, and 55% after passive exercising. Based on the low level of evidence available, it seems that open surgery resulted in more reliable and rapid outcomes compared with nonoperative treatment.

[Hip dysplasia and spinal osteochondritis (Scheuermann's disease) in a girl with type II manifesting collagenopathy]. / Hüftdysplasie und Morbus Scheuermann bei einem Mädchen mit Typ-II-Kollagenopathie.

OBJECTIVES: This paper describes the natural course of irritable hip pain associated with spinal rigidity and pain in the thoracic region with subsequent development of mild kyphosis in a girl with a mutation in the collagen 2 alpha 1 gene (type II collagenopathy). METHODS: Phenotypic and genotypic characterization was carried out in a 14-year-old girl to identify the underlying pathology of severe irritable hip pain associated with thoracic spinal rigidity and pain. Detailed clinical examination, skeletal survey and genetic testing were performed accordingly. Bernese periacetabular osteotomy was used to alleviate pain and to improve the anatomical correlation of the acetabular and femoral heads. RESULTS: Short stature associated with acetabulo-femoral dysplasia, spinal osteochondritis (Scheuermann's disease) and mild thoracic kyphosis were the most prominent abnormalities. Genetic analysis showed a heterozygous mutation in the collagen type II gene (COL2A1-c.1636G>A, p. G546S). A Bernese periacetabular osteotomy was performed to improve the clinical status of the patient. There was significant improvement in the extrusion index, the acetabular index and the lateral center-edge angle. CONCLUSIONS: Hip dysplasia and Scheuermann's osteochondritis have never been reported in connection with a mutation in COL2A1 (collagenopathy type II). Awareness is needed for careful phenotypic and genotypic characterization in patients with irritable hip pain and spinal stiffness.

[Congenital dislocation of the knee (CDK)]. / Kongenitale Kniegelenkluxation (CDK).

Congenital dislocation of the knee (CDK) is a rare deformity presenting itself either as an isolated idiopathic entity or in the context of syndromes like arthrogryposis, myelodysplasia or Larsen syndrome. Patients can be diagnosed clinically after childbirth based on hyperextension of the knee. Confirmation of the diagnosis is done by X-ray or sonography. Many theories concerning the pathogenesis have been proposed since CDK was described; according to recent literature fibrosis and contracture of the m. quadriceps is the most likely reason. Therapy should start as soon as possible after birth, conservatively using redressing casts or operatively in syndromal conditions aiming for reduction. The prognosis concerning re-dislocation is benign; a good outcome was shown for idiopathic CDK.

[Complications after femoral lengthening with ring fixators in congenital femoral deficiency]. / Komplikace po prolongaci femuru kruhovým fixátorem pri vrozeném defektu femuru.

PURPOSE OF THE STUDY: Congenital femoral deficiency (CFD) is a rare and complex deformity. The results and complications of surgical correction of this deformity using ring fixators (Ilizarov/Taylor Spatial Frame) have been evaluated. MATERIAL AND METHODS: We retrospectively reviewed a consecutive series of 30 patients with CFD, between the ages of 3.3 and 17 years (mean, 9.3 years), with 35 lengthening procedures. Ten cases were treated with an Ilizarov frame, 25 cases with a Taylor Spatial Frame (TSF). RESULTS: The average lengthening at femoral osteotomy was 44.3 mm. Nine patients underwent an additional tibial lengthening by an average of 24.2 mm. The total lengthening was 50.5 mm (28-85 mm). A fracture after removal of the fixator occurred in 25.7% of the cases, and a pin-site infection with the subsequent need of operative revision was found in 8.5%. A subluxation of the knee joint was observed in 11.4% and a persistent limitation of the knee joint range of motion in 22.8%. CONCLUSIONS: Despite several complications, the use of ring fixators, especially the TSF, is an effective method for treatment of this complex deformity. The complication rate was similar with the use of either ring fixator. Some complications can be decreased with experience. The risk of knee dislocation can be reduced by joint bridging and fractures after frame removal can be avoided by prophylactic rodding.

Maternal attitudes towards prenatal diagnosis of idiopathic clubfoot.

OBJECTIVES: To examine maternal attitudes towards prenatal diagnosis of idiopathic clubfoot and to determine the incidence of false-negative ultrasound examinations. METHODS: Surveys were mailed to mothers of patients with clubfoot born between 2000 and 2007 who were treated at either Sinai Hospital of Baltimore or Orthopaedic Hospital Speising. Exclusion criteria were underlying syndrome, genetic abnormality and multiple pregnancy. The survey asked the mother whether she had had any ultrasound examinations before her child was born, whether any of these had shown clubfoot, and whether she would have preferred to find out about her child's clubfoot before birth or after birth. RESULTS: Mothers completed 220 (USA, 105 surveys; Austria, 115 surveys) of 401 mailed surveys. The prenatal detection rate was 60% in the USA compared with 25% in Austria (P = 0.001). Overall, 74% of mothers indicated a preference for prenatal diagnosis and 24% indicated a preference for postnatal diagnosis of the condition. Of 92 patients diagnosed prenatally, 96% of mothers indicated a preference for a prenatal diagnosis. Of 128 patients diagnosed postnatally, 58% of mothers indicated a preference for prenatal diagnosis, 38% for postnatal diagnosis and 4% were undecided. CONCLUSIONS: The diagnosis of clubfoot is still often missed during routine ultrasound examination. When a prenatal diagnosis is made, most mothers appreciate having this information. However, when prenatal diagnosis is missed, a significant proportion of mothers seem to accept the false-negative diagnosis retrospectively.

Perspectives from Spanish infectious diseases professionals on 2009 A (H1N1) influenza: the third half.

The first influenza pandemic in more than 40 years was declared in 2009. We aimed to evaluate the beliefs of Spanish infectious diseases professionals regarding several aspects of 2009 A (H1N1) influenza once the epidemic waned. An online survey was designed and distributed among members of the Spanish Society of Infectious Diseases and Clinical Microbiology (SEIMC). The survey considered hospital organization and preparedness planning and conduct, as well as the opinion of the infectious diseases professionals regarding several key issues. Between 7 March and 22 March 2010, 303 responses, corresponding to 12.8% of the SEIMC membership, were received. Of the respondents, 48.2% were microbiologists and 42.3% were clinicians dealing with infectious diseases. Forty-one per cent of respondents did not believe that 2009 A (H1N1) influenza had a more severe presentation than other seasonal influenzas. Only 5% fully agreed that 2009 A (H1N1) influenza had a more severe presentation. Influenza planning was available in 69.7% of represented institutions before the arrival of 2009 A (H1N1) influenza, and was considered to be useful, to different extents, by most professionals. In most institutions (88.3%), a multidisciplinary team was created to coordinate local pandemic influenza actions. The most successful protocols were those provided by regional healthcare authorities, followed by those from the CDC. The most problematic issues regarding 2009 A (H1N1) influenza were the management of patients in the emergency room and the vaccination and awareness of healthcare professionals (HCPs) regarding infection control. Microbiological diagnosis and the availability of antivirals were the least problematic areas. Although the majority of surveyed infectious diseases professionals did not believe that 2009 A (H1N1) influenza had an especially severe presentation, most of them agreed with the way that this epidemic was managed in their institutions.

Evidence of reduced bone turnover and disturbed mineralization process in a boy with Stickler syndrome.

We describe a tall-statured 14-year-old boy who illustrated the full phenotypic and radiographic features of Stickler syndrome type I. A bone biopsy showed evidence of reduced bone mass and bone turnover, such as reduced BV/TV (-43%), TbTh (-29%), and OS/BS (-48%), Ob.S/BS (-27%), and Oc/BS (-47%) compared to "age-matched" controls. Moreover, there was evidence that the mineralization process was severely disturbed. Quantitative backscattered electron imaging revealed that the bone mineralization density distribution (BMDD) of cancellous (Cn) as well as cortical (Ct) bone was shifted toward lower mineralization compared to a young control reference cohort. BMDD parameters of mean degree of mineralization, Cn Ca (-9.8%) and Ct Ca (-18.0%), were dramatically decreased. To the best of our knowledge this is the first clinical report describing bone biopsy findings in a boy with Stickler syndrome. Such a severe undermineralization of bone matrix might essentially contribute to the compromised mechanical competence of the skeleton found in this patient.

Sirenomelia (symelia apus) with Potter's syndrome in connection with gestational diabetes mellitus: a case report and literature review.

We report one case of a fetus of sirenomelia sequence with Potters syndrome which showed oligohydramnios and symelia apus. The infant showed absent urinary tract and external genitalia, the legs were fused by skin and had separate bones associated with Potter's syndrome. The mother had a history of gestational diabetes mellitus.

[Congenital club foot]. / Der kongenitale Klumpfuss.

Congenital talipes equinovarus is the most common deformity of the lower limb. The adequate treatment of clubfeet is still a challenge for orthopaedic surgeons and requires well-founded knowledge of pathoanatomy and established therapeutical options. This refresher, in addition to pathoanatomical fundamentals, provides an update on diagnostic and therapeutical facilities. By reviewing the results of conservative and surgical treatment concepts and their discussion it shall contribute to optimal individual management of congenital talipes equinovarus.

Two novel mutations in the GDAP1 and PRX genes in early onset Charcot-Marie-Tooth syndrome.

Autosomal recessive Charcot-Marie-Tooth syndrome (AR-CMT) is often characterised by an infantile disease onset and a severe phenotype. Mutations in the ganglioside-induced differentiation-associated protein 1 (GDAP1) gene are thought to be a common cause of AR-CMT. Mutations in the periaxin (PRX) gene are rare. They are associated with severe demyelination of the peripheral nerves and sometimes lead to prominent sensory disturbances. To evaluate the frequency of GDAP1 and PRX mutations in early onset CMT, we examined seven AR-CMT families and 12 sporadic CMT patients, all presenting with progressive distal muscle weakness and wasting. In one family also prominent sensory abnormalities and sensory ataxia were apparent from early childhood. In three families we detected four GDAP1 mutations (L58LfsX4, R191X, L239F and P153L), one of which is novel and is predicted to cause a loss of protein function. In one additional family with prominent sensory abnormalities a novel homozygous PRX mutation was found (A700PfsX17). No mutations were identified in 12 sporadic cases. This study suggests that mutations in the GDAP1 gene are a common cause of early-onset AR-CMT. In patients with early-onset demyelinating AR-CMT and severe sensory loss PRX is one of the genes to be tested.

[When is the optimal time for hip ultrasound screening?]. / Wann ist der optimale Zeitpunkt für ein sonographisches Hüftscreening?

The perfect time for hip screening is when every pathologically deformed hip can be diagnosed by sonography and after spontaneous resolution of immature, dysplastic hips. In addition, the beginning of therapy should be early enough to provide the best possible outcome concerning the anatomically correct healing of the patient's hip. Even though every child should be screened by sonography in the first few days of life, a reasonable way could be ultrasound screening in the first week for clinically unstable hips or newborns with risk factors such as breech position combined with ultrasound screening of every newborn between the fourth and sixth week.

[Peromelia]. / Peromelie.

Peromelia is a rare but mutilating congenital deformity representing failure of formation of parts of the upper limb. The deformity is presented unilaterally in the vast majority of the cases. Usually, these children are totally healthy beside from the deformity and are not seriously handicapped by the deformity. Correction of the deformity by hand- or forearm transplantation is not yet possible. Treatment consists of support with prosthesis in case of missing upper- or forearm and construction of fingers by toe-to-hand transplantation in case of missing fingers and hand. Treatment is not mandatory as the children use the stump in combination with their unimpaired upper limb for bimanual daily life activities. The mutilating aspect of the deformity frequently does impair especially parents but also the children to a higher extent than the actual functional disability. Psychological aid is recommended to support children and parents in managing their life with this severe deformity.

Clinical variables associated with the isolation of Klebsiella pneumoniae expressing different extended-spectrum beta-lactamases.

Clinical variables associated with the isolation of Klebsiella pneumoniae expressing different extended-spectrum beta-lactamases (ESBLs) were studied. Clinical records of patients with ESBL-positive K. pneumoniae isolates between 1989 and 2003 (n = 80) were reviewed retrospectively. Patients with SHV- and TEM-type ESBLs were identified more frequently in the intensive care units (67% and 78%, respectively), whereas those with CTX-M ESBLs were found in medical wards (52.2%) or were outpatients (17.4%) (p <0.01). The absence of urinary or central catheters was associated with CTX-M-10 (p 0.013 and p <0.01, respectively). Central catheter-related infections and secondary bacteraemia were associated more frequently with SHV- and TEM-type ESBLs, whereas urinary tract infections were associated with CTX-M-10. Previous aminoglycoside use was associated particularly with SHV-type ESBLs (p <0.01), whereas amoxycillin-clavulanate and oral cephalosporins were associated with CTX-M-10 (p <0.01 and p 0.050, respectively). The frequency of adequate empirical treatment was low (22%), and 61% of patients were treated according to the susceptibility testing results. Mortality (22%) and related mortality (14%) did not differ statistically according to the type of ESBL. Different ESBL types in K. pneumoniae were associated with different clinical variables, and this should be taken into account in current and future epidemiological scenarios.

The role of triple pelvic osteotomy in therapy of residual hip dysplasia and sequel of AVN: long-term experience.

Triple pelvic osteotomy was performed for sequel of DDH including AVN between 1981 and 2002 for 329 patients (351 hips, 280 females, 49 males, average age at surgery 16.5 years, range 9-41 years, follow-up 4-25 years). A small modification of Steel's technique consisting of strictly subperiostal resection of segment from both pubic and ischial bone was used. Average gain of lengthening extremity was 1.8 cm. The average CE angle was improved from 7.8 to 35.5 degrees. Clinical results were evaluated according to Merle d'Aubigne and reflected to the preoperative clinical and radiological findings. There were 146 hip joints in 128 patients (76%) with excellent results in the group of congruent hips without arthrosis. In 182 hips in 178 patients with hip joints with some deformity, limited ROM and decentration, 40% were excellent, 32% good, 23% fair and 5% unsatisfactory results were achieved. The group of decentrated hip joints in young adults in incongruency, limited ROM and sometimes severe arthrosis consisted of 23 monolateral surgeries with 39% unsatisfactory, 39% fair and 22% good results, respectively. No major neurovascular complications were seen. Non-unions were recorded in 19 patients (5.4%), including 2 triple and 2 double non-unions. Based on our long-term experience, we can conclude that triple pelvic osteotomy according to Steel in our modification is a safe method and gives regularly excellent or good results for correction of clinical and radiographic appearance of acetabular dysplasia when there is a proper indication.