Search

Intracranial Langerhans cell histiocytosis commonly presents as skull lesions in children. An intratumoral cyst with fluid level and epidural hematoma occurring with eosinophilic granuloma is very rare. We report a 15-year-old boy who presented with a spontaneous epidural hematoma which was the result of a temporal eosinophilic granuloma. Multiple explanations for epidural hematoma in such cases have been discussed. Intratumoral hemorrhage followed by cyst formation and rupture may explain the pathophysiology of epidural hemorrhage formation.

Background: The authors describe 9 cases of children with eosinophilic granuloma (EG) of the skull and report on their clinical manifestations, treatment, and prognosis. Method: Nine consecutive patients were diagnosed as EG of the skull and confirmed pathologically between 1996 and 2005. In the present study, multi- and single-system Langerhans’ cell histiocytosis without skull involvement were excluded. Patients with EG of the skull were divided into two groups: (1) those with only a single bone lesion and those with (2) multiple bone lesions. Surgical removal was performed between 2 and 10 years of age (mean, 4.2 years). Results: Eight (88.9%) of the study subjects were found to have a single bone lesion at diagnosis, and 1 had multiple bone lesions. Seven patients had a painless skull mass and 2 patients had a painful skull mass. Total removal was performed in all 9 patients. Eight patients received postoperative chemotherapy or indomethacin as adjuvant therapy. Of the 8 patients who received adjuvant therapy, 4 were treated with indomethacin and the remaining 4 received methotrexate-based chemotherapy. Eight patients did not experience EG recurrence, however, 1 patient developed additional lesions 2 years after surgical excision. Conclusions: EG of the skull is a clinicopathological entity with a good outcome. However, therapies and prognoses are dependent on age at diagnosis and the number of bony involvements.

There are several treatment modalities for Langerhans’ cell histiocytosis (LCH) of bone, formerly usually referred to as eosinophilic granuloma, including surgery, radiotherapy and chemotherapy. In spinal lesions, surgery is not recommended generally, because of the potential for reconstitution of vertebral height. We report a case of multifocal monosystemic LCH treated by surgery and chemotherapy for calvarial and spinal lesions. A 7-year-old boy was initially operated for an occipital monostotic LCH of bone. Then, he was treated by surgery for cervical and thoracic lesions, and by chemotherapy with vinblastine. The patient had no complaints at the 24-month follow-up. Although surgery is not recommended for spinal lesions in children with LCH of bone, short segmental fusion and internal fixation may be a suitable choice for selected cases, especially in children who are not likely to comply with activity restrictions and in those who it is difficult to follow up. The treatment must be planned according to the characteristics of the patient.

A 3-month-old female patient presented with a meningomyelocele at the lumber region associated with congenital hydrocephalus. She underwent ventriculoperitoneal (V-P) shunt surgery using the Sophy system. The shunt system was replaced due to a malformation. Following replacement, the patient presented with cerebrospinal fluid (CSF) eosinophilia at the age of 8 months. The eosinophilic granulocytosis of the CSF improved dramatically following systemic prednisolone administration. CSF eosinophilia without accompanying inflammation or pyrexia in the present case may have resulted from an allergic response to a foreign material such as the silicone tube pressure valve of the Sophy system or the sutures rather than bacterial or fungal infection. Based on our results, we believe that some patients may experience CSF eosinophilia following postoperative V-P shunt due to an allergic reaction to the shunt equipment. Prompt steroid treatment can produce spontaneous regression in such cases.

Resorbable plating systems have been adapted into routine use for craniofacial reconstruction in children. After implantation in some patients, the area around the plates can develop palpable and visible fibrous capsules, with underlying bone resorption and a significant foreign-body giant cell reaction. The reaction is usually self-limited. We report a case in which Langerhans cell histiocytosis was resected, and then recurred at the sites of resorbing plate and screw placement in association with a foreign-body giant cell reaction.

Background/Aims: Eosinophilic granuloma (EG) involving the vertebrae in the pediatric population presents a difficult management scenario. Issues of surgical versus nonsurgical intervention, spinal stability and continued skeletal growth must all be considered. Methods: A retrospective review of medical records and radiographs from 1964 to the present yielded 12 patients with age less than 18 at the time of diagnosis of primary spinal EG. Results: Eleven of these 12 patients presented with pain; 2 patients had neurological deficits. Nine patients were managed nonsurgically, including those undergoing tissue diagnosis by needle (2) or extraspinal (3) biopsy; 3 patients underwent gross total resections. Radiographic diagnosis alone was made in 4. With an average follow-up of 8.1 years, survival is 100%. Conclusions: EG in the spine infrequently produces neurological deficits in the pediatric age group, although it may result in spinal instability. As such, nonsurgical management is the preferred strategy to effect symptomatic relief.

Objective: The recommended treatment for solitary calvarial eosinophilic granuloma (EG) is surgical resection by curettage, craniectomy or craniotomy. The purpose of this study was to describe the spontaneous resolution of calvarial EG and discuss ‘observation only’ as an option in the management of this condition. Methods: A retrospective review was performed of children with calvarial EG seen at a tertiary care children’s hospital. Of a total of 17 such patients, four were managed without any intervention and formed the basis of this report. Results: In all four patients treated by ‘observation only’, there was a tender calvarial mass which enlarged rapidly over a few days and then resolved slowly over many weeks. The diagnosis of EG was based on the findings of plain radiographs and/or computed tomography. All masses had resolved by 8 weeks, and the underlying cranial defect filled in spontaneously in all cases. There was no recurrence at follow-up, which ranged from 2 months to 7 years. Conclusions: ‘Observation only’ should be considered as a viable option in the management of children with solitary calvarial masses that have the radiographic features of EG. Consideration should be given to delaying surgical intervention to allow time to see if spontaneous resolution occurs.

We present an overview of the treatment and clinical outcome of 30 pediatric patients with orbital and optic pathway tumors cared for in our center from 1991 to 2002. Follow-ups were available for 48 months on average. Eight of 14 gliomas of the optic nerve or pathway were subtotally resected via a pterional approach. Two were totally resected with transection of the optic nerve, 2 were only biopsied and 2 were observed. One of 4 optic nerve sheath meningiomas in patients with good visual acuity was subtotally resected with decompression of the optic canal. Two were totally resected with transection of the prechiasmal optic nerve and 1 was only biopsied. Four infectious intra-/extraconal lesions were biopsied (transconjunctival approach) and treated with antibiotics. Two vascular intra-/extraconal tumors (hemangiomas) were removed via a transconjunctival approach and a lateral orbitotomy. The following histologies only occurred once: dermoid cyst, aneurysmal bone cyst, eosinophilic granuloma, fibrous dysplasia, osteopetrosis and rhabdomyosarcoma. The spectrum of pathology in pediatric orbital tumors is wide and requires multidisciplinary treatment.

The most common symptoms of eosinophilic granuloma are local tenderness and an enlarged skull mass. The presence of epidural hematoma is a very rare symptom of eosinophilic granuloma. To our knowledge, this is only the second reported case of eosinophilic granuloma with epidural hematoma. A 2-year-old boy with a soft tumor on the occipital scalp, palpable at the age of 3 months, yet with no obvious history of trauma, was admitted due to a sudden onset of loss of consciousness. A brain computed tomography scan showed a lytic lesion on the occipital skull with a large epidural and subcutaneous hematoma, causing brain compression. He underwent an emergency craniectomy with removal of both the tumor and hematoma. The patient regained consciousness and had no residual neurological damage. Pathological reports showed abnormal proliferation of Langerhans' cells, eosinophilic cells and multinucleated cells. A whole-body bone nuclide scan revealed no other bone lesions. The patient was discharged uneventfully. The causes of hematomas are not very clear. They may be due to tumor necrosis or minor trauma. In our presented case, the cause of the epidural hematoma may have been tumor bleeding which ruptured into the epidural space. A solitary eosinophilic granuloma of the skull with acute epidural hematoma and loss of consciousness is extremely uncommon. Craniectomy with removal of the tumor and hematoma decompression may produce good results.

We describe the case of a 2-year-old male patient with an aneurysmal bone cyst (ABC) of the occiput secondary to unifocal eosinophilic granuloma (EG). The lesion presented as a painless mass of the scalp which had grown rapidly over the 2 weeks prior to admission. Radiologically, the lesion was osteolytic and multicystic with fluid-fluid levels. On histology, the cyst wall contained hemosiderin-laden histiocytes, spindle cells, multinucleated giant cells, Langerhans’ cells and eosinophils. Surgical treatment consisted of en bloc resection. Fourteen months after surgery, the patient was well with no local recurrence. Association between EG and ABC is rare. To our knowledge, this is the first case involving the skull.

We report a case of eosinophilic granuloma involving the vertebral bodies of the cervical spine in a 33-month-old girl. This lesion was diagnosed by needle biopsy and treated with prednisone and vinblastine therapy along with immobilization in a Minerva brace. The child has done well over a 9-month follow-up and has shown MRI evidence of resolution of the lesion, reestablishment of structural integrity within the cervical spine and potential reconstitution of the involved vertebral bodies.