A Girl with Lower Back Pain and Rapidly Progressive Atypical Scoliosis

Abstract

Eosinophilic granuloma (EG), which is a bone lesion within the spectrum of conditions termed Langerhans cell histiocytosis (LCH), is nonspecific and variable in clinical presentation and radiographic appearance. Patients with vertebral EG typically present with acute or subacute back or neck pain, but also can develop acute torticollis, kyphosis, and/or scoliosis. The most classic radiographic feature of vertebral EG is vertebra plana or a wedged vertebra due to compression fracture through a vertebral body lytic lesion. However, since the appearance is highly variable, LCH is often referred to as “the great imitator” and requires tissue biopsy for definitive diagnosis, typically with CT-guided needle biopsy. Other conditions can cause vertebra plana, which is not a pathognomonic finding for EG. Systemic involvement of disseminated Langerhans cell histiocytosis must be ruled out with a careful history, physical exam, review of systems, imaging, and referral to hematology-oncology, especially in young children ≤5 years of age with multifocal bony disease. The natural history of a solitary EG is spontaneous resolution with partial vertebral body reconstitution, so children are typically managed symptomatically with nonsteroidal anti-inflammatory medications, spinal orthoses, steroids, and/or CT-guided corticosteroid injections. The long-term prognosis is excellence with low recurrence rate.