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Society for Pediatric Radiology – Poster Archive


Richard Towbin

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Showing 2 Abstracts.

Acroosteolysis (AO) is a rare manifestation of bone resorption that can occur in both genders and is not limited to a specific population. AO can be familial, idiopathic, occupational or secondary. It is marked by distal phalangeal bone resorption and often arises as a secondary manifestation of underlying musculoskeletal, dermatological, or endocrine conditions. Tuft resorption is often associated with conditions like systemic sclerosis, ischemia, hyperparathyroidism, and neurologic disorders, while the destruction of the distal interphalangeal joint is more frequently observed in inflammatory conditions like psoriatic arthritis. Familial AO affects only the feet and is associated with plantar ulcerations. AO due to vinyl chloride exposure is associated with papules and nodules on the hands and forearms and thickening of the skin of the hands. Clinically, AO is marked by a gradual tapering/thinning of the bones in the distal phalanges, resulting in a narrower appearance compared to normal digits. This process is accompanied by reduced bone density in the affected areas, which can be readily observed on radiographic imaging. It is often associated with distal digital ischemia, digital calcinosis, or severe sensory neuropathy. Nail changes in AO include brachyonychia, anonychia, atrophy, transverse ridges, discoloration, thickening of the nail plate, hyperkeratosis of the cuticles, pincer nail, pitted onycholysis, longitudinal ridging, and pterygium. X-ray images typically reveal distinctive bone resorption at the fingertips and toes, while CT scans may show areas of decreased bone density or erosions in the distal phalanges. MRI can detect soft tissue alterations, joint effusion, and inflammation. Beyond the physical changes, patients may experience pain, joint deformities, and reduced functionality in the affected extremities. Treatment mainly involved managing the primary disease causing AO. Learning Objectives: 1) To elucidate the clinical and radiological features of AO like the resorption seen in the distal phalanges. 2) To provide insight into the diverse underlying causes of AO, including associated medical conditions. 3) Understand cutaneous changes in AO to assist in the early recognition and prevention of disease progression. Conclusion: A comprehensive understanding of acroosteolysis, including its clinical presentation, radiological findings, and associated etiological factors, is crucial for accurate diagnosis and appropriate management. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Daggumati Lasya, Malavia Mira, Randhawa Hari, Towbin Richard

Keywords: Phalanges, bone resorption

Primary Hypertrophic Osteoarthropathy (PHO) is a self-limiting, rare condition that emerges in the pediatric or adolescent years with a predilection for males, especially of African descent. It is often accompanied with symptoms of arthralgias, hyperhidrosis, and acne. PHO manifests in various forms, with the complete presentation being the most severe, characterized by facial coarsening, periostosis, and digital clubbing. In contrast, incomplete PHO spares the scalp, and forme fruste PHO exhibits clubbing and facial coarsening with minimal periostitis, duly setting it apart from secondary hypertrophic osteoarthropathy that is commonly associated with cardiac or pulmonary conditions. Genetic mutations in the 15-hydroxyprostaglandin dehydrogenase (HPDG) and solute carrier organic anion transporter family 2A1 (SLCO2A1) genes introduce variability in the inheritance patterns of PHO. HPDG-related PHO is typically autosomal recessive, while SLCO2A1-related PHO can be either autosomal recessive or dominant. These mutations elevate prostaglandin E2 levels, intensifying osteoclast activity, and causing bone resorption, which, in turn, triggers VEGF release and leads to vascular hyperplasia, bone disturbances, and edema. A common radiographic finding is acroosteolysis, and key features to differentiate PHO from other causes of acroosteolysis include terminal tuft resorption and periostitis. Other distinctive radiographic findings in PHO include terminal tuft resorption in fingers and toes and shaggy periostosis in long bone diaphyses. Treatment options include nonsteroidal anti-inflammatory drugs (NSAIDs) and bisphosphonates for pain relief and cosmetic surgery if desired. We will present a few cases from our institution to review classic imaging findings, diagnostic workup, and management. Teaching Points: 1. Understand the clinical variability in presentations of Primary Hypertrophic Osteoarthropathy (PHO). 2. Recognize the genetic basis of PHO and appreciate the variable inheritance patterns associated with the HPDG and SLCO2A1 genes. 3. Identify the distinct radiological features of PHO, including terminal tuft resorption and shaggy periostosis, enabling accurate diagnosis and differentiation from other conditions. 4. Emphasize the importance of early diagnosis, highlighting how prompt recognition of clinical and radiographic features can enhance patient outcomes and mitigate complications. Read More

Meeting name: SPR 2024 Annual Meeting & Postgraduate Course , 2024

Authors: Malavia Mira, Randhawa Hari, Dagumati Lasya, Towbin Richard

Keywords: digital clubbing, peristosis