Aims

Developmental dysplasia of the hip (DDH) can be managed effectively with non-surgical interventions when diagnosed early. However, the likelihood of surgical intervention increases with a late presentation. Therefore, an effective screening programme is essential to prevent late diagnosis and reduce surgical morbidity in the population.

Muscular torticollis is rarely seen in our population group. There is controversy regarding the surgical procedure of choice, post operative immobilization and the ultimate results. Several procedures have been described to release the sternocleidomastoid. In children over 5 years correction of secondary deformities are less certain, and complications are scarring and loss of contour of the neck. The aim of this paper is to review the results of bipolar release of the sternocleidomastoid muscle in muscular torticollis. Between 2000–2006, 3 girls and 1 boy, aged 6–10 years, were treated surgically for muscular torticollis. Spinal abnormalities and other congenital problems were excluded. Through an incision parallel and superior to the clavicle, the sternal and clavicular attachments of the sternocleidomastoid muscle were divided and 2.5cm of muscle was excised. Through a separate transverse incision inferior to the mastoid process, the insertion of the sternomastoid tendon was divided. Post operative traction was used for 5 days, followed by a moulded collar for 3 months. The children were assessed for function and cosmesis. All scars healed well. The wry neck position improved in all. All children had improvement in movements and cosmesis. There were no visual problems and the lateral band was inconspicuous in the neutral position. Follow up ranged from 1–5 years. Lateral flexion and rotation improved markedly. Two patients reported an increase in height. Our early results showed that patients over 6 years had marked improvement in cosmesis and movement following bipolar release of muscular torticollis. The improvement in facial asymmetry is being observed with growth. Obviously, the follow up is not long enough to provide adequate long term evaluation. No major complications occurred

Introduction: Atlanto-axial rotatory fixation is a rare condition which occurs more. commonly in children than in adults. The terminology can be confusing and the condition is also known as. ‘ atlanto-axial rotatory sub-luxation’ and ‘atlanto-axial rotary dislocation’ . Rotatory fixation is the preferred term however , as in most cases the fixation occurs within the normal range of rotation of the joint and by definition therefore the joint is neither subluxed nor dislocated. Atlanto-axial rotatory fixation is a cause of acquired torticollis. Diagnosis can be difficult and is often delayed. The classification. system proposed by Fielding in 1977 is most frequently used and will be discussed in detail. Given that this classification system was devised in the days before CT, as well as the fact that combined atlanto-axial and atlanto-occipital rotatory subluxation is omitted from the classification, we propose a modification to the classification of this rare but significant disorder. Methods and Results: The radiological findings in six cases of atlanto-axial rotatory fixation will be illustrated, including a case with associated atlanto-occipital sub-luxation. The pertinent literature will be reviewed and a more comprehensive classification system proposed. The imaging approach to diagnosis and the orthopaedic approach to management will be discussed. Conclusion: In general, children who present with a traumatic torticollis should be treated conservatively with cervical collar and anti-inflammatory medication for one week. Those children whose torticollis fails to resolve after one week require aggressive investigation by ‘dynamic’ computed tomography to assess whether the joint is fixed. If however there is a history of significant trauma then immediate radiological assessment is advised. This approach will avoid over-investigation and over-treatment yet will still detect atlanto-axial rotatory fixation early enough to achieve a good outcome

Dupuytren Disease (DD), the most common connective tissue disease in man, presents as a benign fibromatosis of the hands and fingers resulting in the formation of nodules and cords and often leading to flexion contractures in association with keloids or Peyronie disease. Surgical resection of the fibrotic nodules, and more recently intra-lesional collagenase injection are the main therapeutic options for these patients. While the exact cause of DD is still unknown, linkage and Genome Wide Association Studies (GWAS) showed molecular heterogeneity with at least 10 different susceptibility loci 6 of which are close to genes encoding proteins in the Wnt-signaling pathway. We aim to identify the molecular basis of Dupuytren Disease (DD). Twenty patients with Dupuytren disease (including 3 patients with autosomal dominant inheritance, 1 with keloids and congenital torticollis, 2 with Peronie disease), were included in this study. Chromosome Microarray Analysis (CMA), Whole Exome Sequencing (WES) of gDNA and proteomic analysis by LC-Tandem Mass Spectrometry (LC-MSMS) studies were performed. Expression and Network analysis of LCMSMS results was performed using Principal Component Analysis (PCA), ANOVA and Ingenuity Pathway Analysis (IPA). No pathogenic copy number variants (CNVs) were found in CMA (n = 3). WES showed potentially pathogenic variants in POSTN, WNT11, MMP1 and COL3A1. PCA showed three differentially expressed clusters and network-IPA identified ACTB, BAX, COL3A1, FBN1, FN1, MMP1 as potential biomarkers. Comprehensive multi-OMIC analysis of gDNA and tissue proteins in patients with DD identified several connective tissue biomarkers potentially important in the pathogenesis of DD

Spondylodiscitis rarely coexists with endocarditis (around 5% of patients with endocarditis). Furthermore, viridans streptococci are not common pathogens of spondylodiscitis and finally the combination of spondylodiscitis and right – sided endocarditis due to viridans streptococci is rare. We present a case of right-sided native valve endocarditis due to Streptococcus mutans presenting as cervical and lumbar spondylodiscitis in a patient with obstructive cardiomyopathy. A 52 year – old man with a history of hypertrophic obstructive cardiomyopathy was admitted with fever and back pain of ten days duration, followed by torticollis. He had undergone dental therapy some weeks before symptom appearance, due to bad oral hygiene, without receiving any chemoprophylaxis. Magnetic resonance imaging revealed L4-L5 and C4-C5 spondylodiscitides. Four blood cultures drawn were all positive for Streptococcus mutans, while fine needle aspiration of the lumbar lesion was unsuccessful. Transesophageal echocardiogram revealed tricuspid and possible pulmonary valve vegetations. The patient was treated with ceftriaxone plus gentamicin for 2 weeks and then ceftriaxone only, for a total of 3 months. He had an uneventful recovery and was referred for cardiosurgical consultation. Physicians managing cases of spondylodiscitides should bear in mind to rule out endocarditis, especially in cases with underlying cardiopathy. The possibility of coexistence is even greater when there is sustained bacteremia and the pathogen isolated from blood cultures is a common pathogen for endocarditis

Aims

Early detection of developmental dysplasia of the hip (DDH) is associated with improved outcomes of conservative treatment. Therefore, we aimed to evaluate a novel screening programme that included both the primary risk factors of breech presentation and family history, and the secondary risk factors of oligohydramnios and foot deformities.

Malformation and hypoplasia of the clavicle can result in pain, impaired function, restricted shoulder movement, subjective feeling of instability and cosmetic deformity. There are no reports of clavicle lengthening by osteotomy and distraction osteogenesis (DO). This is a retrospective review of 5 patients (7 clavicles) who underwent clavicle lengthening by DO using a monolateral external fixator for clavicular hypoplasia. There were 3 males and 2 females with mean age 15 years (9 to 23) and mean follow-up 21 months (8 to 51). Preoperative diagnoses included Klippel-Feil syndrome, cleidocranial dysplasia with torticollis, congenital myopathy and Noonans syndrome and obstetric brachial plexus injury. Mean length gained was 31 mm (15 to 41) which represens an average of 24.7% of overall bone length. Mean time in fixator was 174 days (161 to 263) and mean external fixation index was 56 days/cm. Two patients required internal fixation following fixator removal to consolidate union and one required additional internal fixation for atrophic regenerate. Mean preoperative oxford shoulder score improved from 28.5 to 41 and all patients were extremely satisfied with their result. Two patients developed pin site infections. Clavicular lengthening by distraction osteogenesis for congenital clavicular hypoplasia is a previously unreported technique that enables gradual correction of deformity without risking brachial plexus traction injury following acute correction. It has the potential to improve shoulder pain, function, range of movement and cosmesis. Distraction ≥25% of overall bone length may require additional plate fixation to consolidate union

Objective: To report the development of acquired atlan-toaxial instability in pediatric patients with spastic cerebral palsy and no previous history of traumatic incident. Study Design-Material: We present three patients with severe spastic quadriplegia who developed C1–C2 instability and cervical myelopathy at mean age 12.6 years. Results: These patients demonstrated similar clinical picture with symptoms attributed to cervical myelopathy in varied severity including apneic episodes, opisthotonus, alteration in muscle tone, torticollis, respiratory problems, hypereflexia and bradycardia. Patient 1 was scheduled for surgery but died due to an apneic episode. Patient 2 denied surgery and has been followed for 3 years while his neurological condition remains unchanged. Patient 3 underwent decompression through a C1 laminectomy and occiput-C4 posterior spinal fusion, recovered neurologically and resumed his previous functional skills. Conclusions: The cases included in our study serve to illustrate the numerous difficulties related to the diagnosis and treatment of severely involved children with spastic cerebral palsy complicated with acquired atlan-toaxial instability and myelopathy. A high index of suspicion in patients who demonstrate a significant functional deterioration or a sudden change in their previous neurological status may lead to the diagnosis of cervical myelopathy at an early stage, when the possibility of recovery is higher. The management of spastic patients with documented cervical instability and myelopathy remains controversial, mainly due to the complexity of the medical problems that affect the general condition of these patients. We believe that even in severely involved pediatric patients appropriate management, including spinal cord decompression and fusion, may alter the natural history of myelopathy if the treatment is performed when the neurologic damage is not long standing. If the etiology of myelopathy is not addressed it may result to fatal complications, although clinical progression is not documented in all patients

Aim: To evaluate the necessity for further radiological investigation in patients with suspicion of rotatory subluxation of the atlanto-axial complex on plain radiography following acute cervical trauma. To outline guidelines for assessment of patients with atlanto-axial asymmetry on plain radiography. Methods: A retrospective review of all patients who had undergone atlanto-axial CT scanning as a result of radiographic C1–C2 asymmetry following cervical spine trauma in the 3 year period from January 1999 to December 2001. The plain X-ray and CT images were reviewed retrospectively and correlated with their clinical presentation and outcome by the senior author. Results: Twenty-eight patients were included in the study. Acute cervical spine trauma had occurred most commonly following a road traffic accident. No patient was found to have acute cervical spine torticollis or severe cervical pain. Patients age ranged from 21–44 years (M:F – 15:13). All patients were found to have atlanto-odontoid asymmetry on initial plain X-ray. No patients were found to have rotatory subluxation on CT images. 3 patients were found to have minor degrees (< 10°) of rotation on the CT scan which is within normal limits. 9 patients (32%) were found to have congenital odontoid lateral mass asymmetry. All patients were treated conservatively and had no further intervention. All plain radiographs were then assessed to determine the underlying reason for asymmetry. In 19 cases the orientation of the radiographic beam in combination with head rotation was found to be at fault. Conclusion: Rotatory subluxation of the cervical spine is a rare but serious condition in the adult. The condition is suspected radiologically in the presence of odontoid lateral mass asymmetry on open mouth view. The application of ATLS principles in the initial assessment of trauma patients has resulted in a significant increase in the number of radiological examinations performed. This has led inevitably to an increase in the number of anomalies identified. An average of 400 c-spine X-rays per year are performed for trauma in our casualty department. In this study, we have identified 9 patients out of a total of 29 with congenital odontoid lateral mass asymmetry over a 3 year period. This represents approximately 0.75% of the cervical spine X-rays and should be considered in the differential diagnosis following acute cervical trauma. We outline guidelines for recognising benign atlanto-axial asymmetry

Traumatic rotatory atlanto-axial dislocation and subluxations are rare injuries. The diagnosis is often missed or delayed because of subtle clinical signs. Head tilt makes the interpretation of plain radiographs difficult. Delayed diagnosis often results in chronic instability necessitating surgical stabilization. A hitherto undescribed clinical sign was evaluated which should lead to increased awareness and avoid delay in the diagnosis. Why a new clinical sign?. Easily missed injury. Uncommon but not that uncommon. Difficult to diagnose. Needs high index of suspicion. Not much emphasis given in training. Radiographs usually inconclusive because of torticollis deformity Prerequisites for test. Patient should be conscious. A Lateral radiograph should not show any facet dislocations or fractures in cervical spine. Explain the patient what you intend to do and he/she should report any paraesthesias, sensory or motor symptoms if felt during the test Clinical sign- Elastic Recoil:. Supine patient. Hold head carefully with hands on either side of the head. Instruct patient to report any neurological deterioration. Try to straighten the head tilt gently. Once it is corrected, release the supporting hand towards tilt of the head taking care not to let the head overshoot the original position. An elastic recoil of the head to previous position indicates a positive test. Methods: This study was carried out between 1997 to 2003. The test was applied to 59 patients presenting in Accident and Emergency. All this patients had head tilt even after the application of a hard cervical collar. All the 59 patients had CT scans to confirm or exclude the diagnosis of Rotatory atlanto axial dislocation/subluxation. Results: The new clinical sign was found to be positive in all the fourteen patients with atlanto- axial rotatory dislocations/subluxations which was confirmed by CT scan. The test was also found to be positive in 5 patients with unilateral facet joint dislocation. The sensivity of the test in our study was 100%. The specificity was 89%, positive predictive value 0.73, negative predictive value 0.9 (90%). Conclusion: This new clinical sign may help in early recognition of the injury and also act as an effective screen to indicate which patient needs a CT scan to confirm the diagnosis. This can also be applied in places where the CT scan facilities are not readily available especially in the developing nations

There is a very long way from diagnosis to treatment of the developmental dysplasia of the hip. Everything is complex: clinical examination is subtle and requires a long training. Treatment is not as simple as could be thought. The risks include approximate examinations and of standard, ready-made ones. The discussion has not been settled as to whether all children with hip instability can be clinically detected at birth. The complexity of the problem arises from the fact that only 10% of children who have instability at birth develop long-term problems if they are left untreated. It is well known, affirmed by several different studies that at birth the incidence of hip instability in approximately 1–4% of patients, with figure being higher in girls. There is also a consensus that a large majority of these unstable hips will become stable during the first few days of life, even without treatment. What is not known, however, is how many of those hip that become stable will become completely normal as the child grows. These two fundamental issues are of the utmost importance. They emphasize the significance of early examination (first 48 hours) in order to detect instability and employ careful follow-up of any newborn in whom hip instability has been identified. Some children are at particular risk of hip instability. Those infants are labeled as “high risk”. They include children born in families with hip instability, those presented by breech, first born children or products of oligohydramniotic pregnancies, particularly girls, those with the generalized joint laxity, those with torticollis and scoliosis, those with foot deformities and increased birth-weight over 4000g. Whilst all children should be screened at birth by a doctor experienced in clinical examination with particular attention directed to those children, who are considered high risk. It must be emphasized that clinical examination is the most important for the detection of hip instability in newborns. Clinical examination should be very delicate, gentle, based on feelings rather than signs. Clinically, hip instability can be divided into: 1. irreducible dislocation, 2. reducible dislocation, 3. dislocatability, 4. subluxability. The clinical tests of instability were described by Ortolani and Barlow. Fully dislocated irreducible hip is a very rare condition and may be associated with neuromuscular abnormality. It represents dislocation well before delivery. In this form acetabulum is vacant, femoral head palpable posteriorly. There are no singns of Ortolani and Barlow tests. We can only find the sings of “pump”, which means there exists a movement along the long axis of the leg. This is the most severe pathology of the hip in DDH.The reducible dislocation is characterized by the Ortolani maneuver. With the hip flexed 90 degrees, we abduct the hip and than we feel and hear the click which is the sign of the reduction of the hip. Dislocatable and subluxatable are the most common types of pathology of the hip in DDH. This deformity arises at the end of pregnancy. This is characterized by positive Barlow test, which is the provoked-dislocation test. Using it we can dislocate or subluxate the hip. It is very rare to find restricted hip movements in newborns. The limited abduction of the affected hip is the sign typical for older children, more the 3 months of age. It is crucial to repeat the clinical examination even during the same office visit. Ultrasonography has changed our diagnostic ability for DDH. These direct examinations help us in hip evaluation in the first days of the newborn period. The exact Graf classification and methodology makes possible to classify all types of hip pathology irrespective of the examiner. Another important feature of ultrasonography is the ability to monitor the treatment not only in newborn period. How to start with the prevention of DDH in newborn period? There is no any really good screening without collaboration of orthopedics surgeons and pediatricians. To achieve this, we must control our own environment, especially maternity hospitals and constantly keep pediatricians, pediatric nurses and obstetricians informed. There is no consensus in screening programs – general or limited. I personally recommend limited program. All newborns should be examined clinically after the delivery. The infants with positive or doubtful clinical signs and those with signs of high risk should be examined ultrasonographically immediately after delivery within first 2 weeks. The other children should be screened ultrasonographically at the time when most of the hips are mature enough. It is important, that if we live in the region with high percentage of late detected DDH (more than 3 months of age) the general clinical and ultasonographical screening program should be used