Open AccessThoracic spine Langerhans cell histiocytosis in a child with achondroplasia
Author(s) -
Zoe Wing-Yan Chan,
Lesley A. Simpson,
Pasquale Gallo
Publication year - 2019
Publication title -
bmj case reports
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.231
H-Index - 26
ISSN - 1757-790X
DOI - 10.1136/bcr-2018-228801
Subject(s) - medicine , langerhans cell histiocytosis , spinal cord compression , surgery , thoracic vertebrae , radiology , mccune–albright syndrome , histiocytosis , fibrous dysplasia , spinal cord , lumbar vertebrae , pathology , disease , psychiatry , lumbar , precocious puberty , hormone
Multifocal bone Langerhans cell histiocytosis (LCH) is usually treated with prednisolone and vinblastine. We present a case conservatively treated with indomethacin with good clinical and radiological response. A 7-year-old achondroplastic boy presented with worsening thoracic back pain and leg weakness. An admission MRI spine showed a pathological T1 vertebrae fracture with posterior soft tissue extension compressing and distorting the spinal cord. A CT guided biopsy revealed an LCH. Steroids were avoided to reduce osteopenia risk and further vertebral fragility. Considering the risk of a thoracic surgical approach in a child with this background, he was managed conservatively with indomethacin and a Sternal Occipital Mandibular Immobilizer (SOMI) Brace. Pain resolved completely within 6 months and the brace was discontinued. Serial follow-up scans showed progressive resolution of the pathological T1 fracture and complete resolution of the spinal cord compression.