Split Calvarial Bone Graft for Reconstruction of Skull Defect in Langerhans Cell Histiocytosis

Abstract

Langerhans cell histiocytosis (LCH) is a rare disease in which histiocytes proliferates in several tissues. It mainly occurs in children between 1 and 15 years of age, and affects bone especially the skull being the most common site of invasion. It is desirable to reconstruct the skull defect after surgical removal of the lesion with autologous bone considering the fact that this disease affects children who continuously experience the deformation and growth of the frontal head. We introduce a case report who reconstructs the skull defect using split calvarial bone graft in LCH. A 3-year-old male visited the hospital with a painful, palpable protruding mass in the frontal area lasting 1 month. Craniectomy with skull tumor removal was performed by neurosurgeon, after that we harvested a calvarial bone graft and carefully separated the outer cortex from the inner cortex. Then, we covered the donor site with the inner cortex while covered the bone defect with the outer cortex and fixed with absorbable plates. The patient showed palpable mass at 1-year follow-up but it disappeared. After 5 years, there was no recurrence, the lytic lesion of the skull was resolved, and showed cosmetically good result. We conclude that the palpable lesion was more likely to be the result of postoperative swelling of the plate. We propose split calvarial bone graft as a good treatment option for reconstruction of the skull defect in LCH.

Short-Term Resorption of Grafted Bone After Split Calvarial Bone Grafting for an Occipital Skull Defect in a Patient With Multisystem Langerhans Cell Histiocytosis

“…Furthermore, soluble interleukin-2 receptor (sIL-2R), which is a known indicator of disease activity in LCH, was within the normal range at the time of the preoperative examination, and CT, MRI, and bone scintigraphy did not show any osteolytic changes in the skull defect. There has been a case report describing a good outcome after a 1-stage cranioplasty using a split calvarial bone graft for a skull defect that occurred after removal of a skull lesion in a patient with single-system unifocal-type LCH 7 . However, in our patient, bone was grafted onto the remaining bone defect after chemotherapy and not after removal of the lesion.…”

“…There has been a case report describing a good outcome after a 1-stage cranioplasty using a split calvarial bone graft for a skull defect that occurred after removal of a skull lesion in a patient with single-system unifocal-type LCH. 7 However, in our patient, bone was grafted onto the remaining bone defect after chemotherapy and not after removal of the lesion. Therefore, it is possible that this patient still had a locally active lesion.…”

“…This method has been reported to be useful for a skull defect in LCH. 7 However, in this case, the grafted bone was resorbed in the short term. To our knowledge, this is the first report of resorption of an autologous bone graft when used to repair a skull defect in a patient with multisystem LCH in remission.…”

“…We performed cranioplasty using an autologous bone graft that was separated into 2 parts, namely, an inner cortex and an outer cortex. This method has been reported to be useful for a skull defect in LCH 7 . However, in this case, the grafted bone was resorbed in the short term.…”

“…There are no established guidelines for the treatment of a bone defect in a patient with LCH that is in remission. 7 We performed cranioplasty using an autologous bone graft that was separated into 2 parts, namely, an inner cortex and an outer cortex. This method has been reported to be useful for a skull defect in LCH.…”