Journal reading

1. Journal reading R2 Se-yi Chen 2006. April

2. 9 y/o , Female Presenting with severe neck pain, kyphosis for 1 month DTR: +++/ +++, no muscle weakness, steady gait Case report

4. Management and Result • Whole spine survey with MRI  negative at other level of spine • Skull routine, long bone XR negative • Surgery intervention: anterior corpectomy and Mesh cage interbody fusion, internal fixation with ACP plate. • Biopsy: Langerhans cell histiocytosis. CD-1a (+), S-100 (+)

5. Hospital course • Improved neck pain without neurological deficit after operation. • Keep neck collar after operation. • Began steroid therapy after tissue proof • Discharge and OPD follow-up.

6. Langerhan’s cell histiocytosis • Letterer-Siwe disease (10%)(usually <3 y/o): most malignant • Hand-Schüller-Christian disease(20%)(1-5y/o): typically present with a triad of skull lesion, exophthalmos, and D.I. • Eosinophilic granuloma (70%) (5-15 y/o): benign proliferation of Langerhans cell occuring in unifocal or multifocal skeletal system. Same disease process--- monoclonal proliferation of Langerhans cell.

7. Overall incidence: 1: 1,500,000 • Children incidence 1: 200,000 • Mild male predominence Typical microscopy finding: granulomatous infiltrates with histiocyte . EM: Birbeck granules

8. Imaging finding • Best imaging clue: child with vertebra plana, two apposed intervertebral discs are intact. (adjacent discs may appear enlarged and wider). Homogeneous density of the collapsed vertebral body. • CT image: NECT->destructive lesion CECT->homogeneously enhanced soft tissue mass • MR imaging T1WI: hypointense vertebral body, homogenous enhancement of soft tissue mass after contrast. T2WI: heterogeneously hyperintense Radiography: collapsed vertebral body, rare in posterior elements involvement. Bone Scintigraphy--- poor sensitivity

9. Differential diagnosis • Ewing sarcoma • Osteomyelitis • tuberculosis • Metastses • Neuroblastoma • Hematopoietic malignancy (leukemia, lymphoma) • Giant cell tumor

10. Location of involvement skull > mandible> long bones > ribs > pelvis > spine (anterior >> posterior) Thoracic(54%) > Lumbar (35%) > cervical (11%).

11. Presentation of C spine LCH • Neck pain (76%), limited range of motion (76 %) • About 33 % had neurological deficit.

12. During 1970-2003, 26 children were diagnosed and treated as LCH. The 23 children were followed for more than 2 years.(Mean: 9.4 yrs) • Despite heterogeneous treatment, all patients were alive and well with resolution of all presenting signs and symptoms. No evidence of active disease at the time of the most recent follow-up • Radiation therapy poses a risk to the vertebral endplates. we believe that the risk associated with RT is unacceptable given the overwhelmingly positive natural history of this disorder. • Except for open biopsy of the affected vertebra, surgery is rarely indicated for the spinal lesions. Normally, the only indications for spinal surgery are stabilization of an unstable segment of the spine that cannot be stabilized with an orthosis or neurologic symptoms due to compression of the spinal cord • We recommend radiographs every 3 months for the first year after the diagnosis to confirm the benign nature of the disease and annual radiographs thereafter, through skeletal maturity, to monitor spinal growth and balance.

13. Langerhans cell histiocytosis of the cervical spine: a single institution experience in four patientsTan, Gracea; Samson, Ignaceb; Wever, Ivo Dec; Goffin, Jand; Demaerel, Philipped; Van Gool, Stefaan W.aJournal of Pediatric Orthopaedics B 2004 Vol13(2), March 2004, pp 123-16 • Abstract: When Langerhans cell histiocytosis (LCH) occurs at critical sites, such as in the cervical spine, there is a substantial risk for morbidity. Therefore, reports on clinical experiences with those patients remain important. We summarize the history of four patients with unifocal LCH at the cervical spine. All four patients received a biopsy to prove the histopathological diagnosis of LCH by demonstration ofCD1a+cells. They were treated with oral prednisolone. All patients recovered completely and kept a normal function of the cervical spine. No reactivation of the disease occurred with an observation time of 3.4–7.3 years. This report contributes to the clinical experience for the treatment of LCH at critical sites.

14. Eosinophilic granuloma of the cervical spineSpine, vol 27,(11)1408-13, 2002. • A meta-analysis was performed on 53 cases of C spine Eosinophilic ganuloma(EG) • Conclusion: immobilization is an adequate therapy. If the process continuous to progress, R/T is recommaned. Surgical intervention should be reserved for cases with unstable spine or neurological deficit. • The enchondral plate are not destroyed by tumor, however, R/T may destroy the growth potential of the encondral plates. Therefore, R/T should not be the first-choice treatment.

15. Anterior cervical corpectomy and fusion using miniplate and screw in a 7 year-old child with Eosinophilic Granuloma of the cervical spineSpine vol 26(10), 1193-96, 2001.

16. Simple biopsy with curettage and conservative treatment to relieve symptoms are often sufficient to induce the lesion to regress. Due to preserved endochondral ossification, recovery of vertebral height achieved over time. • Due to there’s no proper anterior fixation device for the pediatric spine, the Miniplate was used.

17. Vertebral Remodeling in Eosinophilic Granuloma of the SpineSpine vol 23(12)1351-54, 1998 • A study of 14 conservatively treated patients (during 1980-1990) were studied for remodeling of the vertebral body in an average follow-up of 5.6 years. • 18.2-97% of vertebral body reconstitution was noted in all 14 patients.

18. Take home massage of LCH Self-limited disease if EG. Typical spine image: vertebra plana (the most common reason for children) T> L > C spine involvement Dx by image (general bone survey), tissue proof (immunological study), r/o other malignancy and visceral organ involvement. Tx: conservative treatement , +/- surgical intervention, steroid, R/T, C/T.

19. Thank you