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Langerhans Cell Histiocytosis of the Pediatric Thoracic Spine with Development of Neurological Compromise

A Case Report

Burkes, Jonathan C. MD, MBA1,2; Anderson, John T. MD2,3

doi: 10.2106/JBJS.CC.18.00159
Case Reports
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Disclosures

Case: This report describes a 7-year-old girl with Langerhans cell histiocytosis (LCH) of the thoracic spine who developed neurological deterioration during nonsurgical management. She was treated with decompression and instrumented fusion followed by chemotherapy, recovered completely after surgery, and was doing well at 6-year follow-up.

Conclusions: The best treatment of LCH of the spine is not clear, but in the setting of neurologic compromise after failing conservative management, surgical decompression with adjuvant chemotherapy should be considered.

1Department of Orthopedic Surgery, Kansas University Medical Center, Kansas City, Kansas

2Department of Orthopedic Surgery, The Children's Mercy Hospital, Kansas City, Missouri

3University of Missouri—Kansas City School of Medicine, Kansas City, Missouri

E-mail address for J.C. Burkes: jburkes@kumc.edu

Investigation performed at Children's Mercy Hospital, Kansas City, Missouri

Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article (http://links.lww.com/JBJSCC/A822).

Langerhans cell histiocytosis (LCH) of the spine is a rare condition in children. LCH of the spine typically presents with back pain. A typical radiographic finding is vertebra plana or flattening of the vertebral body1. Furthermore, the oversleeve-like or dumbbell sign on magnetic resonance imaging (MRI) can be considered a feature of LCH of the spine1. The term “Langerhans cell histiocytosis” now encompasses many clinical entities: Letterer–Siwe disease, Hand–Schuller–Christian disease, eosinophilic granuloma, histiocytosis X, Hashimoto–Pritzker syndrome, self-healing histiocytosis, pure cutaneous histiocytosis, Langerhans cell granulomatosis, Type II histiocytosis, and nonlipid reticuloendotheliosis2. LCH in children is typically of the eosinophilic granuloma type. Spinal involvement is present in 6.5% to 25% of patients3. Historically, the incidence of thoracic spine involvement is 54% compared to lumbar and cervical; however, more recent series have indicated that the incidence of thoracic spine involvement may be lower1,4,5. Furthermore, LCH with a soft tissue mass causing neurological deficit is exceedingly rare3,6. Typically, LCH of the spine can be managed conservatively with the occasional addition of radiation therapy or, more commonly, chemotherapy5. However, this case demonstrates that a patient presenting with isolated back pain and a characteristic radiographic finding can develop neurological compromise while being managed nonsurgically.

The patient and legal guardian were informed that data concerning the case would be submitted for publication, and they provided consent.

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Case Report

A 7-year-old girl was referred to our spine clinic with a chief complaint of thoracic level back pain over the past 3 weeks. Her pain was primarily activity related, but sometimes woke her from sleep. She denied fever, polyuria, ear pain, skin lesions, or abnormal bleeding. Her medical history was significant for asthma, myringotomy tubes, and adenoidectomy. There was no relevant family history. Her physical examination was significant for mild obesity and painful trunk flexion and extension. She had no tenderness and was neurologically normal. She had no upper motor neuron signs, abnormal cutaneous findings, hepatosplenomegaly, or exophthalmos. Radiographs revealed asymmetric loss of vertebral body height at T7 and evidence of osteopenia (Figs. 1-A and 1-B). An MRI of the thoracic spine revealed T7 vertebra plana with uninvolved discs and no discrete soft tissue mass (Figs. 2-A and 2-B). Her imaging was reviewed by other staff spine surgeons and radiologists. Although vertebra plana is not diagnostic of LCH, this was felt to be the most likely diagnosis. She was prescribed a Jewett brace for comfort.

Figs. 1-A and 1-B Initial anterioposterior and lateral radiographs of thoracic spine.

Fig. 1-A

Fig. 1-A

Fig. 1-B

Fig. 1-B

Figs. 2-A and 2-B Initial magnetic resonance imaging T2 axial (TE: 103 ms; TR: 3,440 ms) and sagittal (TE: 3,000 ms; TR: 100 ms) magnetic resonance imaging.

Fig. 2-A

Fig. 2-A

Fig. 2-B

Fig. 2-B

At 4-week follow-up, the patient was still having pain with no change in physical examination. Radiographs of the thoracic spine revealed further loss of T7 vertebral height and 32° of kyphosis at T6-8, which had increased from previous studies. A Tc99m-labeled hydroxydiphosphonate whole body bone scan was also performed that did not reveal other lesions. A chest x-ray did not reveal pulmonary involvement. Because her lesion was isolated, an attempt of nonoperative treatment without chemotherapy was felt to be appropriate.

The following day, the patient presented to our emergency department with new onset of severe pain. She was found to be neurologically normal. She was admitted, and the pain service was consulted. A custom thoracic lumbar sacral orthosis (TLSO) was fabricated. Standing posterioranterior and lateral scoliosis films in the orthosis revealed no change in her sagittal balance. She was discharged home with pain medication per pain service recommendations.

Five days later, the patient presented to the emergency department with acute onset of dysesthesias in the anterior and posterior aspects of bilateral thighs as well as difficulty walking. Her bowel and bladder functions were normal. Examination showed normal lower extremity dermatomal light touch sensation, one beat of clonus bilaterally and hyperreflexic patellar reflexes. She would not participate with a gait examination due to pain. She had 5/5 motor strength in all lower extremity myotomes. Because of the patient's dysesthesias and hyperreflexia, a repeat MRI of the thoracic spine with and without contrast was done, which revealed a soft tissue mass causing significant central, lateral recess, and foraminal stenosis at T7 (Figs. 3-A and 3-B). Fig. 3-B does represent the “dumbbell sign” signifying a soft tissue mass. Because of the rapid growth of the soft tissue mass, Ewing sarcoma was added to our differential diagnosis. Infection was also considered with an erythrocytes sedimentation rate of 69 mm/hr and c-reactive protein of 2.3 mg/dL but deemed less likely. Early in the course of her hospitalization, she developed weakness of left ankle dorsiflexion and great toe extension.

Figs. 3-A and 3-B Repeat magnetic resonance imaging T2 sagittal (TE: 89 ms; TR: 3,170 ms) and axial (TE: 5,030 ms; TR: 67 ms) magnetic resonance imaging.

Fig. 3-A

Fig. 3-A

Fig. 3-B

Fig. 3-B

The patient then underwent a computed tomography–guided biopsy by our interventional radiology service, and the diagnosis of LCH was confirmed by pathology (Fig. 4). The oncology service was consulted who recommended chemotherapy per LCH-III guidelines7. This included 6 months of treatment with vinblastine and prednisone. A single lumen Port-A-Cath placed in anticipation of her chemotherapy needs. Because of the rapid growth of her soft tissue mass, severe worsening of pain, and sudden deterioration of her neurological function, the decision was made to proceed with surgical decompression and instrumented fusion. An instrumented fusion was felt to be necessary because of the destabilizing effect of the laminectomy and facetectomies required for decompression and for kyphosis correction.

Fig. 4

Fig. 4

Before her operation, she had intact bowel and bladder function but 4/5 strength with right ankle dorsiflexion and great toe dorsiflexion. Left knee extension was 4/5, ankle dorsiflexion was 3/5, great toe dorsiflexion was 3/5, and ankle plantar flexion was 4/5. She had no upper motor neuron findings.

She underwent a T5-10 posterior spinal fusion and instrumentation with a subtotal T7 vertebrectomy through a T7 costotransversectomy approach (Figs. 5-A and 5-B). The tumor was removed without difficulty and a frozen section confirmed the diagnosis of LCH. The right pedicle was completely absent as was a majority of the vertebral body on the right. A T6-8 anterior interbody fusion was performed with cage placement for anterior column support. Initially, she had no cortical evoked motor potentials in her legs and her somatosensory evoked potentials were diminished. We obtained cortical evoked potentials in her feet after elevating her mean arterial pressure above 80 mm Hg with ephedrine. This was stable throughout the procedure. Her estimated blood loss was 700 mL. She was admitted to the pediatric intensive care unit for continued hemodynamic support. In the immediate postoperative period, she was noted to have right knee extension of 5/5, left knee extension of 2/5, right ankle dorsiflexion of 4/5, left ankle dorsiflexion of 1/5, right great toe dorsiflexion of 4/5, left great toe dorsiflexion of 2/5, right ankle plantar flexion of 5/5, and left ankle plantar flexion of 3/5. Rectal tone was normal. She was discharged home 2 weeks postoperatively and underwent outpatient rehabilitation. Her chemotherapy was started 6 weeks postoperatively directed by the LCH III guidelines7. She wore a TLSO for 2 months after her procedure. At her 2-month follow-up appointment, her neurological examination was normal, and she was ambulating without assistance. During 6 years of follow-up, the patient did not have locally recurrent tumor or new sites of distant disease. Her Adolescent Self-Reported Pediatric Outcomes Data Collection Instrument Global Functioning standardized and normative scores at 6 years were 73 and 20, respectively. The standardized scores range from 0 to 100 and the normative from −78 to 57, with greater numbers correlating with better function.

Figs. 5-A and 5-B posterioranterior and lateral radiographs 6 years postoperatively.

Fig. 5-A

Fig. 5-A

Fig. 5-B

Fig. 5-B

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Discussion

LCH affecting the spine is a rare disease and is commonly managed nonoperatively5. However, neurological compromise can develop during nonsurgical treatment when patients present with isolated back pain associated with a characteristic radiographic finding. When neurological compromise is present, or the patient's neurological status is worsening, surgical intervention should be strongly considered. Based on available evidence, it is controversial whether isolated chemotherapy or surgical intervention is appropriate care. Lee et al. reported successful nonsurgical treatment in 22 patients with spinal LCH with vinblastine based chemotherapy. No recurrence was noted at an average of 6-year follow-up8. Furthermore, Peng et al. suggested that LCH of the spine, even with neurological symptoms, may be treated with chemotherapy and brace alone3. In contrast, Lu et al. suggested a single-stage posterior approach with decompression and fusion with instrumentation without the need of chemotherapy in cases of neurological compromise. They reported no recurrences or implant failures at an average 43-month follow-up on their cohort of 12 patients. All patients made full neurological recovery with no deformity noted at last follow-up. One patient had symptoms of intercostal neuralgia successfully treated with NSAIDs and 2 other patients had pleural effusions that were also successfully treated6. Fortunately, prognosis for LCH in skeletally immature patients is good with a low likelihood of recurrence9. In our case, due to the rapid progression of neurological symptoms and the presence of a large soft tissue component, we performed a surgical decompression with fusion followed by chemotherapy.

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References

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3. Peng XS, Pan T, Chen LY, Huang G, Wang J. Langerhans' cell histiocytosis of the spine in children with soft tissue extension and chemotherapy. Int Orthop. 2009;33(3):731-6.
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7. Allen CE, McClain KL. Langerhans cell histiocytosis: a review of past, current and future therapies. Drugs Today (Barc). 2007;43(9):627-43.
8. Lee SW, Kim H, Suh JK, Koh KN, Im HJ, Yoon HM, Seo JJ. Long-term clinical outcome of spinal Langerhans cell histiocytosis in children. Int J Hematol. 2017;106(3):441-9.
9. Plasschaert F, Craig C, Bell R, Cole WG, Wunder JS, Alman BA. Eosinophilic granuloma: a different behaviour in children than in adults. J Bone Joint Surg Br. 2002;84(6):870-2.

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