An adolescent girl came with swelling of both thighs with difficulty in walking for a few months. Based on clinical, and radiological evaluation and nerve conduction studies, she was diagnosed to be having bilateral hips Charcot's arthropathy due to hereditary sensory autonomic neuropathy type 4. Other common causes of Charcot arthropathy were ruled out. The patient was conservatively managed and parents were educated about preventive measures. Through this case report, we want to highlight the myriad number of manifestations and clinical presentations of a child presenting with hereditary sensory autonomic neuropathy type 4.

The National Institute of Health now estimates that there are well over 37 million persons with diabetes in the United States alone, a number well over 11 per cent of our population. The associated multiple organ system disease is responsible for more than 327 billion dollars in direct and indirect medical costs and more than 140,000 lower extremity amputations yearly in the United States. Because healthcare professionals have begun to appreciate the economic and pathologic burden that diabetes imparts on our society, there has been a growth in both the understanding and treatment of the responsible pathologic disorders. The goal of this monograph is to provide an evidence-supported foundation to better understand the pathophysiology that leads to the development of neuropathic (Charcot) foot arthropathy and provide insight into developing a treatment plan for addressing this complex disease process that presents in a highly comorbid patient population.

Charcot neuropathic arthropathy is a relatively rare, chronic disease that leads to joint destruction and reduced quality of life of patients. Early diagnosis of Charcot arthropathy is essential for a good outcome. However, the diagnosis is often based on the clinical course and longitudinal follow-up of patients is required. Charcot arthropathy is suspected in patients with suggestive symptoms and an underlying etiology. Failed spinal surgery is not a known cause of Charcot arthropathy. Herein we report a patient with ankle Charcot neuropathic arthropathy that developed after failed spinal surgery. A 58-year-old man presented to the emergency room due to painful swelling of the left ankle for 2 weeks that developed spontaneously. He underwent spinal surgery 8 years ago that was associated with nerve damage, which led to weakness of great toe extension and ankle dorsiflexion, and sensory loss below the knee. CT and T2-weighted sagittal MRI showed a fine erosive lesion, subluxation, sclerosis, fragmentation, and large bone defects. Based on the patient's history and radiological findings, Charcot arthropathy was diagnosed. However, the abnormal blood parameters, positive blood cultures, and severe pain despite the decreased sensation suggested a diagnosis of septic arthritis. Therefore, diagnostic arthroscopy was performed. The ankle joint exhibited continued destruction after the initial surgery. Consequently, several repeat surgeries were performed over the next 2 years. Despite the early diagnosis and treatment of Charcot arthropathy, the destruction of the ankle joint continued. Given the chronic disease course and poor prognosis of Charcot arthropathy, it is essential to consider this diagnosis in patients with neuropathy.

Charcot neuroarthropathy (CN) is a destructive complication of the joints in patients with diabetes and should be managed from the onset of the first symptoms to avoid joint deformity and the risk of amputating the affected joint. Here, we describe the case of a young 24-year-old patient living with type I diabetes who developed active bilateral CN in both tarsal joints. This case of neuroarthropathy was uncommon due to the bilateral presentation at the same level of the joint. Despite the patient consulting from the beginning of the symptoms, none of the physicians suspected or diagnosed CN, leading to a delay in management and the aggravation of bone destruction by CN. This highlights the importance of early management of CN with the need to refer people with suspected CN to specialised diabetic foot care centres.

Bone in diabetes mellitus is characterized by an altered microarchitecture caused by abnormal metabolism of bone cells. Together with diabetic neuropathy, this is associated with serious complications including impaired bone healing culminating in complicated fractures and dislocations, especially in the lower extremities, so-called Charcot neuroarthropathy (CN). The underlying mechanisms are not yet fully understood, and treatment of CN is challenging. Several in vitro and in vivo investigations have suggested positive effects on bone regeneration by modifying biomaterials with sulfated glycosaminoglycans (sGAG). Recent findings described a beneficial effect of sGAG for bone healing in diabetic animal models compared to healthy animals. We therefore aimed at studying the effects of low- and high-sulfated hyaluronan derivatives on osteoclast markers as well as gene expression patterns of osteoclasts and osteoblasts from patients with diabetic CN compared to non-diabetic patients with arthritis at the foot and ankle. Exposure to sulfated hyaluronan (sHA) derivatives reduced the exaggerated calcium phosphate resorption as well as the expression of genes associated with bone resorption in both groups, but more pronounced in patients with CN. Moreover, sHA derivatives reduced the release of pro-inflammatory cytokines in osteoclasts of patients with CN. The effects of sHA on osteoblasts differed only marginally between patients with CN and non-diabetic patients with arthritis. These results suggest balancing effects of sHA on osteoclastic bone resorption parameters in diabetes.

Syringomyelia is uncommonly revealed by a neuropathic arthropathy (Charcot joint) and characterised by its slow progression with late neurological symptoms. In this particular field, neuropathic arthropathies mainly affect the shoulders and the elbows.We report a new case of neuropathic arthropathy of the wrist caused by syringomyelia, because of the rarity of this condition as well as its uncommon location.Neuropathic arthropathy caused by syringomyelia is poorly published in the literature. This atypical entity should be considered in case of Charcot arthropathy associated with neurological loss, especially when it affects the upper limbs.

Charcot neuroarthropathy (CN) is a systemic disease that causes fractures, dislocations, and deformities involving the foot and ankle, resulting in substantial risk of ulceration, infection, and function loss. Early recognition and prevention of collapsing foot and ankle are still the best options for the management of patients with diabetic CN. For a successful arthrodesis procedure, the principles of adequate joint preparation, deformity correction, and soft tissue protection and care are essentials, associated with robust fixation (internal and/or external), use of different biological graft options in segmental losses, and prolonged off-loading.

Neuropathy is a painful and potentially devastating complication of diabetes mellitus affecting many patients. Neuropathy can lead to foot ulcers, infections, and subsequent amputations. Nerve damage from peripheral neuropathy may lead to Charcot neuropathic osteoarthropathy, commonly known as Charcot foot. Flesh wounds and weakened bones causing microfractures of the foot and ankle may lead to foot malformations. Early recognition and care are essential for the treatment of Charcot foot and prevention of further injury. This article discusses the use of monofilament testing for diabetic neuropathy, increasing awareness of Charcot foot, and introducing a screening algorithm for Charcot foot.

Musculoskeletal conditions are common in patients with diabetes. Several musculoskeletal disorders are viewed as chronic complications of diabetes because epidemiological studies have revealed high correlations between such complications and diabetes, but the pathophysiological links with diabetes remains unclear. Genetic predispositions, shared risk factors, microvascular impairments, progressive accumulation of advanced glycation end-products, and diabetic neuropathy may underlie the development of musculoskeletal disorders. Musculoskeletal complications of diabetics have received less attention than life-threatening microvascular or macrovascular complications. Here, we review several diabetic musculoskeletal complications with a focus on the clinical importance of early recognition and management, which would improve quality of life and physical function.

Surgical reconstruction of Charcot arthropathy in the foot and ankle is extremely difficult. The fundamentals of reconstruction are necessary to provide adequate outcome. Removing and resecting the diseased bone is needed along with good anatomic alignment and rigid fixation. This reconstructive surgery is not only difficult from medical management point of view but also involves patient compliance and good technical components of the surgery from the surgeon. The surgeon must have skills with internal and external fixation, a good understanding of lower extremity vascular disease, and a good understanding of infectious disease and plastic surgical techniques of the lower extremity.

Static circular fixation is a valuable tool for patients with Charcot foot and ankle deformities. The versatility of circular fixators allows for dynamic adjustments over time and can allow for off-loading of flaps or ulcers. The circular fixator can be used as a primary fixation device in cases of osteomyelitis or to facilitate lengthening when a segmental bone defect exists such as loss of the talus. As a secondary fixation device it can protect the internal fixation or be used when there is a compromised soft tissue envelope.

Charcot neuroarthropathy (CN) and its sequela is a disabling pathology in the foot and ankle. The 2-stage computer hexapod-assisted technique is an effective tool to address midfoot Charcot and ankle-hindfoot deformities to restore function and decrease the risk of amputation secondary to ulceration and infection. Although this is not the only technique available, it is an excellent option in cases with significant angular deformity or subluxation, need to reduce shortening of the foot, and in the presence of soft tissue defects, with or without concurrent soft tissue or bone infection.

The Charcot diabetic foot presents unique challenges to the podiatric surgeon in the quest to salvage the limb. This disorder is an intersection of prototypical metabolic diseases and neurodegenerative disorder. Furthermore, it can be considered a disease of bone and ligaments that is often complicated by peripheral vascular disease and serious deep infection. Presently, simplistic ablative surgical procedures and the brace-makers art, still have a valid place in treating this disorder. Newer methods of surgical reconstruction are rapidly evolving to address distorted and nonfunctional limb. This article seeks to evidence the principles and practice of beaming the Charcot midfoot. As will be presented, the beam is a load-sharing device, which can be surgically introduced in an intramedullary method to restore architecture and strength to the Charcot foot. Problems with beam failure and migration have resulted in unsatisfactory outcomes as will be discussed. New Charcot-specific beams are currently reaching the podiatric surgeon with hopes of improving durability. In this article, we aim to address the surgical art of the beam, the engineering principles of beaming, and the novel introduction of a truss/tie rod configuration of beaming.

Reconstruction of the Charcot foot and ankle demonstrates significant challenges to the foot and ankle surgeon. At present, there is limited clear consensus on the best approach for preoperative optimization. The primary aim of Charcot reconstructions is to limit the risk of ulceration by providing a stable plantigrade foot allowing ambulation. The focus of this article is the discussion of modifiable risk factors associated with Charcot reconstruction for preoperative optimization.

Charcot can be a difficult clinical entity to diagnose in the acute phase, and clinicians should have a high clinical suspicion in neuropathic patients who present with erythema, edema, and warmth of the foot or ankle. Immobilization and nonweight-bearing should be immediately initiated when the diagnosis of Charcot has been made and patients should remain nonweight-bearing until the affected bones/joints have coalesced. Educating patients and managing expectations is crucial to improve compliance with the conservative treatment of Charcot and avoid the long-term sequelae including severe deformity, ulceration and infection, and amputation.

There are many similarities between nondiabetic and diabetic Charcot neuroarthropathy (CN) but many of the underlying causes causing nondiabetic neuropathy and CN are associated with poor bone quality. Patient workup for nondiabetic CN should include the underlying cause of the neuropathy and optimization of bony healing, such as vitamin D supplementation and bisphosphonate or calcitonin administration. Surgical reconstruction should include the most robust fixation possible, as nondiabetic patients with CN are more prone to delayed union.

Diabetic neuroarthropathy is a complication of diabetes mellitus that results in instability of the foot, structural deformity, and soft-tissue breakdown. Commonly, midfoot collapse of the medial, lateral, or both longitudinal arches may result in increased plantar pressures and subsequent midfoot ulceration. Many of these wounds can be successfully managed with local wound care and off-loading; however, surgical intervention becomes necessary in cases of osteomyelitis or when the wound fails to heal despite conservative efforts. In cases where surgical reconstruction may not be indicated, nonreconstructive surgical efforts have shown effectiveness in resolving wounds and allowing patients to return to ambulatory lifestyles. This article serves as an update to current treatment recommendations for the nonreconstructive surgical management of Charcot neuroarthropathy.

Bone metabolism in the healthy, young adult is identified as a relatively stable process. Normal bone turnover is a dynamic state, which is conferred through intracellular signaling and complex cellular pathways. It has been well described in the literature that Charcot neuro-osteoarthropathy is a disease state, which is marked by intense bone turnover leading to structural collapse and dissolution of skeletal features of the foot and ankle. Within the last two decades, extensive interest has been placed in characterizing the metabolic pathogenesis of Charcot bone metabolism. Despite this work, there remains an incomplete understanding of this devastating disorder. In this article, we review bone histology, physiologic bone metabolism, biomarkers of bone metabolism, pathologic bone metabolism in Charcot diabetics, and potential avenues for intervention.

Diabetes mellitus with the lack of glycemic control increases risks for developing comorbidities affecting organ systems responsible for critical function. The development of diabetic neuropathy predisposes patients to the onset of Charcot neuroarthropathy (CN). There is significant complexity with treatment of diabetic-induced CN, which can have an often delayed or missed diagnosis. Supervision and treatment from trained specialists are required to provide care for this multifaceted disease process. It is essential for patients to partner with glucose control, comorbidity prevention and care, as well as lower extremity management. Ultimately, CN can result in significant lower extremity deformity placing patients at risk of limb and life.

Few is investigated about the management of acute fore- and midfoot injuries in diabetics. With well controlled diabetes, indications and techniques are similar to non-diabetics. With poorly controlled diabetes, medical optimization should be exercised. Stable internal fixation in case of surgical treatment and prolonged offloading independent of the choice of treatment are advised. With manifest Charcot neuroarthropathy, the goal is to achieve a plantigrade, stable foot that is infection- and ulcer-free and ambulant with orthopaedic shoes. If operative treatment is chosen, the concept of superconstructs in combination with prolonged protection in a well-padded total contact cast is applied.

Conservative treatment of Charcot neuro-osteoarthropathy (CN) aims to retain a stable, plantigrade, and ulcer-free foot, or to prevent progression of an already existing deformity. CN is treated with offloading in a total contact cast as long as CN activity is present. Transition to inactive CN is monitored by the resolution of clinical activity signs and by resolution of bony edema in MRI. Fitting of orthopedic depth insoles, orthopedic shoes, or ankle-foot orthosis should follow immediately after offloading has ended to prevent CN reactivation or ulcer development.

Surgical intervention for Charcot arthropathy is becoming more common; this is driven by an increased prevalence, better understanding of the cause, identifying patient risk factors that influence outcomes, and how to best optimize these. This article aims to summarize the cause of Charcot, look at the factors that influence the outcomes, and the financial cost of managing what is a very challenging condition.

The last decade has seen a significant development in early surgical intervention for patients with or at risk of ulceration owing to deformity resulting from the sequalae of diabetic foot disease. Midfoot Charcot neuroarthropathy is the most common deformity; its correction is enabled by specialized surgical implants designed to maintain surgical corrections. There has also been an increasing number of orthopedic foot and ankle surgeons, with a specific interest in diabetic foot disease who provide early surgical correction in patients identified as high risk. Minimally invasive surgery using percutaneous incisions completes the triumvirate, facilitating earlier surgical intervention to decrease reulcerations.