Hip Dysplasia in a Patient in Late Adolescence With Charcot-Marie-Tooth and Multiple Acyl-CoA Dehydrogenase Deficiency.

This case report explores a unique presentation of hip dysplasia in a female patient aged 21 years old diagnosed with Charcot-Marie-Tooth disease (CMT) type 1A and multiple acyl-CoA dehydrogenase deficiency (MADD). The coexistence of these neuromuscular and metabolic disorders in a patient with hip dysplasia provides an opportunity to investigate their potential interactions and impact on diagnosis, treatment, and prognosis. The patient underwent labral repair with shelf osteotomy and later a total hip replacement. This case highlights the need for further research to better understand the relationships between CMT, MADD, neuromuscular dysplasia, and hip dysplasia. A deeper understanding of these interactions may lead to improved diagnostic techniques, earlier intervention, and personalized treatment approaches for patients with co-morbid conditions, ultimately improving patient outcomes and reducing complications later in life.

Osteofibrous dysplasia of the tibia : the importance of deformity in surveillance.

AIMS: Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. METHODS: A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed. RESULTS: Mean age at presentation was 13.5 years (SD 12.4), and mean follow-up was 5.65 years (SD 5.51). At latest review, 62 lesions (61.4%) were in skeletally mature patients. The most common site of the tibial lesion was the anterior (76 lesions, 75.2%) cortex (63 lesions, 62.4%) of the middle third (52 lesions, 51.5%). Pain, swelling, and fracture were common presentations. Overall, 41 lesions (40.6%) presented with radiological deformity (> 10°): apex anterior in 97.6%. A total of 41 lesions (40.6%) were treated conservatively. Anterior bowing < 10° at presentation was found to be related to successful conservative management of OFD (p = 0.013, multivariable logistic regression). Intralesional excision was performed in 43 lesions (42.6%) and a wide excision of the lesion in 19 (18.8%). A high complication rate and surgical burden was found in those that underwent a wide excision regardless of technique employed. There was progression/recurrence in nine lesions (8.9%) but statistical analysis found no predictive factors. No OFD lesion transformed to adamantinoma. CONCLUSION: This study confirms OFD to be a benign bone condition with low rates of local progression and without malignant transformation. It is important to distinguish OFD from adamantinoma by a histological diagnosis. Focus should be on angular deformity, monitored with full-length tibial radiographs. Surgery is indicated in symptomatic patients and predicted by the severity of the initial angular deformity. Surgery should focus more on the deformity rather than the lesion. Cite this article: Bone Joint J 2022;104-B(2):302-308.

Principles of Bracing in the Early Management of Developmental Dysplasia of the Hip.

Bracing is considered a gold standard in treating Developmental Dysplasia of the Hip (DDH) in infants under 6 months of age with reducible hips. A variety of braces are available that work on similar principles of limiting hip adduction and extension. This paper summarises the current evidence regarding bracing in DDH. Most of the literature pertains to the Pavlik harness (PH) and there are few studies for other brace types. Bracing eliminates dislocating forces from the hamstrings, the block to reduction of the psoas and improves the muscle line of pull to stabilise the hip joint. Recent studies have shown no benefit in bracing for stable dysplasia. The rates of PH treatment failure in Ortolani-positive hips have been reported to be high. Barlow positive hips have lower Graf grades and are more amenable to PH treatment. There is consensus that the earlier the diagnosis of DDH and initiation of PH treatment, the better the outcome. Failure rates due to unsuccessful reduction and AVN are higher with treatment initiated after age 4-6 months. Studies have shown no benefits of staged weaning of braces. While there is no maximum time in brace, current consensus suggests a minimum of 6 weeks. The key to successful bracing lies in education and communication with the family.

Musculoskeletal Pathology in Cerebral Palsy: A Classification System and Reliability Study.

This article presents a classification of lower limb musculoskeletal pathology (MSP) for ambulant children with cerebral palsy (CP) to identify key features from infancy to adulthood. The classification aims to improve communication, and to guide referral for interventions, which if timed appropriately, may optimise long-term musculoskeletal health and function. Consensus was achieved by discussion between staff in a Motion Analysis Laboratory (MAL). A four-stage classification system was developed: Stage 1: Hypertonia: Abnormal postures are dynamic. Stage 2: Contracture: Fixed shortening of one or more muscle-tendon units. Stage 3: Bone and joint deformity: Torsional deformities and/or joint instability (e.g., hip displacement or pes valgus), usually accompanied by contractures. Stage 4: Decompensation: Severe pathology where restoration of optimal joint and muscle-tendon function is not possible. Reliability of the classification was tested using the presentation of 16 clinical cases to a group of experienced observers, on two occasions, two weeks apart. Reliability was found to be very good to excellent, with mean Fleiss' kappa ranging from 0.72 to 0.84. Four-stages are proposed to classify lower limb MSP in children with CP. The classification was reliable in a group of clinicians who work together. We emphasise the features of decompensated MSP in the lower limb, which may not always benefit from reconstructive surgery and which can be avoided by timely intervention.

Counteracting bone fragility with human amniotic mesenchymal stem cells.

The impaired maturation of bone-forming osteoblasts results in reduced bone formation and subsequent bone weakening, which leads to a number of conditions such as osteogenesis imperfecta (OI). Transplantation of human fetal mesenchymal stem cells has been proposed as skeletal anabolic therapy to enhance bone formation, but the mechanisms underlying the contribution of the donor cells to bone health are poorly understood and require further elucidation. Here, we show that intraperitoneal injection of human amniotic mesenchymal stem cells (AFSCs) into a mouse model of OI (oim mice) reduced fracture susceptibility, increased bone strength, improved bone quality and micro-architecture, normalised bone remodelling and reduced TNFα and TGFß sigalling. Donor cells engrafted into bones and differentiated into osteoblasts but importantly, also promoted endogenous osteogenesis and the maturation of resident osteoblasts. Together, these findings identify AFSC transplantation as a countermeasure to bone fragility. These data have wider implications for bone health and fracture reduction.

Multiple abdominal cocoons: an unusual presentation of intestinal obstruction and a diagnostic dilemma.

Sclerosing encapsulating peritonitis (SEP) or abdominal cocoon is a rare acquired condition with an unknown aetiology. It is characterized by encapsulation of the small bowel by a fibrous membrane and can lead to intestinal obstruction. We present the case of a 42-year-old gentleman with a history of hepatitis C, tuberculosis, and previous abdominal surgery, who presented with subacute intestinal obstruction. Surgical exploration of the abdomen revealed that the entire contents were enclosed into three distinct sacs by a dense fibrous membrane. Excision of the sacs was performed followed by adhesiolysis. This is believed to be the first reported case of multiple cocoons within the abdominal cavity. The case is discussed with reference to the literature.

Management of acute anterior shoulder dislocation.

Shoulder dislocation is the most common large joint dislocation in the body. Recent advances in radiological imaging and shoulder surgery have shown the potential dangers of traditional reduction techniques such as the Kocher's and the Hippocratic methods, which are still advocated by many textbooks. Many non-specialists continue to use these techniques, unaware of their potential risks. This article reviews the clinical and radiographic presentation of dislocation; some common reduction techniques; their risks and success rate; analgesia methods to facilitate the reduction; and postreduction management. Many textbooks advocate methods that have been superceded by safer alternatives. Trainees should learn better and safer relocation methods backed up by the current evidence available.

The efficacy of steroid injection in the treatment of trigger finger.

BACKGROUND: Trigger finger is one of the most common reasons for referral to a hand specialist clinic. The purpose of this study is to investigate the efficacy of steroid injections for treating trigger digits. METHODS: Ninety digits were investigated with at least a year follow up. The study mainly focused on the efficacy of the injections, as well as co-morbidities, presence of a nodule, actual digit injected and the severity at presentation using Green's classification. RESULTS: The study found that 66% of trigger digits were effectively treated using steroid injections. There was a difference between the efficacy of the injection in the different digits, with a statistical significance between the thumb and the fingers. The results also showed that there was no statistical relationship between the severity of the condition, the presence of a nodule or co-morbidities and the efficacy of the steroid injections. CONCLUSIONS: The study found that steroid injections are an effective first-line intervention for the treatment of trigger digit. It also found an increased efficacy for treating the thumb compared to other digits. Both the severity of the condition at presentation and the presence of a nodule had no significant impact on the efficacy of the injections.

Posterior femoral neck impingement secondary to excess acetabular anteversion in hip resurfacing arthroplasty.

Component malposition in resurfacing arthroplasty can cause impingement, reduced range of motion, increased metal wear, and early failure. It is therefore important that any component malposition is recognized early and monitored carefully. We present a case of delayed diagnosis of excess acetabular component anteversion causing posterior femoral neck impingement and pain. We correlate the degree of damage found intraoperatively with the degree of component malposition on the radiographs. This is used as a platform to discuss the optimum positioning of hip resurfacing components and the radiographic methods used to determine component position postoperatively.

Meniscal Regeneration: A Cause of Persisting Pain following Total Knee Arthroplasty.

Many patients have persisting knee pain following total knee arthroplasty. We report the unusual case of a patient whose chronic lateral and medial knee pain were caused by entrapped regenerated meniscal tissue. This was diagnosed and successfully treated by arthroscopic debridement.

The uses of the iPhone for surgeons.

Mobile technology is continuously improving and it is important that all physicians are aware of its new advances. Smartphones have the potential to improve diagnostic skills and education of a surgeon. The iPhone is a popular type of smartphone in the market. This article intends to educate surgeons about its uses, functions and medical applications. The phone is an invaluable tool for the modern day surgeon.

A case report of a septic hip secondary to a psoas abscess.

Psoas abscess was first described by Mynter in 1881. Though rare, its prevalence is increasing with advances in radiology and an increasing ability to accurately diagnose the condition. The symptoms of a psoas abscess can be insidious and nonspecific, and patients often present with a limp, fever, weight loss, and flank or abdominal pain.A psoas abscess can be classified as either primary or secondary depending on the presence or absence of an underlying disease. Primary psoas abscess has become more prevalent in the developed world, especially in immuno-compromised patients.We present the case of a 48 year old man who presented with fever, left hip pain and difficulty weight-bearing. He had a past medical history of chronic renal failure secondary to hypertension. Following laboratory, radiological and microbiological analyses the patient was diagnosed as having a Staphylococcus Aureus hip sepsis secondary to a psoas abscess.Psoas abscess should be included as a differential diagnosis in all patients presenting with hip pain and constitutional symptoms. The case is discussed with reference to the literature.

Pseudogout Associated Hip Pain in a Patient with HIV Infection.

HIV infection is a global pandemic, currently affecting approximately 77,000 people in the UK and 33 million people around the world. The infection has widespread effects on the body and can involve the musculoskeletal system. It is therefore important that orthopaedic surgeons are aware of the condition and its sequelae. We present the case of a 46-year-old man with a 10-year history of HIV who presented with acute hip pain, difficulty weight-bearing, and constitutional symptoms. Following radiological, microbiological, and serological tests a diagnosis of pseudogout was established following microscopic analysis of the hip joint aspirate. The patient's symptoms resolved completely following the joint aspiration and NSAID therapy. Studies have shown a relationship between HIV infection and gout. The virus has also been linked to osteonecrosis, osteopenia, bone and joint tuberculosis, and septic arthritis from rare pathogens. However, it is difficult to fully ascertain whether these conditions are related to the HIV infection itself or the HAART (highly active antiretroviral therapy). There are no previously reported cases of HIV-infected patients with pseudogout. The case is discussed with reference to the literature.

Adductor tenotomy: its role in the management of sports-related chronic groin pain.

PATIENTS AND METHODS: Chronic adductor-related groin pain in athletes is debilitating and is often challenging to treat. Little is published on the surgical treatment when conservative measures fail. This single center study reviews the outcomes of 48 patients (68 groins) who underwent percutaneous adductor tenotomy for sports-related chronic groin pain. Questionnaire assessments were made preoperatively and at a minimum follow-up of 25 months. RESULTS: Mean pre-injury Tegner activity scores of 8.8 reduced to 6.1 post-injury and these improved to 7.7 following surgery (p < 0.001). Sixty percent of patients regained or bettered their pre-injury Tegner activity scores after the adductor surgery; however, mean post-surgical Tegner scores still remained lower than pre-injury scores (p < 0.001). No patient had been able to engage in their chosen sport at their full ability pre-operatively, and 40% had been unable to participate in any sporting activity. The mean return to sports was at 18.5 weeks postoperatively, with 54% returning to their pre-injury activity levels, and only 8% still unable to perform athletic activities at latest follow-up. Seventy-three percent patients rated the outcome of their surgery as excellent or very satisfactory, and only three patients would not have wished to undergo the procedure again if symptoms recurred or developed on the opposite side. No patients reported their outcome as worse. A 78.1% mean improvement in function and an 86.5% mean improvement in pain were reported, and these two measures showed statistically significant correlation (p = 0.01). Groin disability scores improved from a mean of 11.8 to 3.9, post-operatively (p < 0.001). Bruising was seen in 37% of procedures, 3 patients developed a scrotal hematoma and 1 patient had a superficial wound infection. One patient developed recurrent symptoms following re-injury 26 months post-surgery, and fully recovered following a further adductor tenotomy. CONCLUSIONS: Adductor tenotomy provides good symptomatic and functional improvement in chronic adductor-related groin pain refractory to conservative treatment.