Child’s Nerv Syst (2002) 18:74–76 DOI 10.1007/s003810100491
Juan F. Martínez-Lage Mar Bermúdez Enrique Martínez-Barba José L. Fuster Máximo Poza
Received: 16 February 2001 Published online: 22 September 2001 © Springer-Verlag 2001 J.F. Martínez-Lage (✉) · M. Poza Unit of Pediatric Neurosurgery, 4-planta, Virgen de la Arrixaca University Hospital, National Institute of Health, 30120 El Palmar, Murcia, Spain e-mail:
[email protected] Tel.: +34-968-369489 Fax: +34-968-369678 M. Bermúdez · J.L. Fuster Section of Pediatric Oncology, Institute of Oncology, Virgen de la Arrixaca University Hospital, 30120 El Palmar, Murcia, Spain
C A S E R E P O RT
Epidural hematoma from a cranial eosinophilic granuloma
Abstract The formation of an epidural hematoma from an eosinophilic granuloma of the skull is an exceptional occurrence. A 9-year-old boy presented with severe headache, somnolence and vomiting following a minor head injury. Cranial computerized tomography scan showed a seemingly depressed skull fracture together with an epidural hematoma in evolution. A neoplasm and an epidural hematoma were removed at operation. Histopathological study of the excised mass confirmed the diagnosis of eosinophilic granuloma.
Keywords Eosinophilic granuloma · Histiocytosis X · Intracranial epidural hematoma · Langerhans’ cell histiocytosis · Skull neoplasm
E. Martínez-Barba Service of Pathology, Virgen de la Arrixaca University Hospital, 30120 El Palmar, Murcia, Spain
Introduction
Case report
Eosinophilic granuloma (EG) represents the focal form of Langerhans’ cell histiocytosis (histiocytosis X) [4]. EG occurs predominantly in children and adolescents. The skull is the bone most frequently involved by EG [4]. Local pain and a subscalp mass are the typical presenting complaints of the disease [4, 5]. It is not uncommon to elicit the antecedent of a mild head injury preceding the appearance of clinical symptoms [4, 5]. The acute presentation of a cranial EG with an epidural hematoma is certainly a most unusual event [2]. The history and the neuroimaging findings in the case we are reporting did, however, suggest an epidural hematoma. A careful interpretation of clinical and radiographic findings indicated a bone neoplasm as the true origin of the patient’s symptoms. A search of the current literature disclosed only two cases with a similar presentation [2, 3].
A 9-year-old boy was transferred to our hospital with the diagnosis of depressed skull fracture and an intracranial epidural hematoma made on the basis of a computerized tomography (CT) scan. The patient had sustained a mild blow to the back of his head 2 weeks earlier. Shortly after, he was noted to have a subcutaneous hematoma in his right occipital region, which was expected to reabsorb spontaneously. On the day of his admission to our hospital, he fell down and struck his head on the floor. The boy did not lose consciousness, but he became drowsy, complained of headaches, and vomited. His past medical history and the family’s were unremarkable. On examination the child looked ill, was oriented but drowsy, and had no focal neurological signs. There was a soft, tender mass 4 cm in diameter in his right occipital region. A head CT performed at his local hospital showed a subcutaneous mass, an epidural image of mixed densities, and a bone defect that had been interpreted as a depressed skull fracture (Fig. 1). Routine blood analyses showed an erythrocyte sedimentation rate of 33 and mild anemia. A postcontrast CT performed 3 days later showed some enhancement of the extra- and intracranial lesions,
75
Fig. 1 Initial unenhanced CT scan showing a subcutaneous swelling, a presumed skull fracture and an epidural hematoma
Fig. 2 Postcontrast CT scan revealing a hypodense epidural collection and a bone defect together with a subcutaneous swelling Fig. 3 Skull radiograph showing a punched-out lytic lesion
Fig. 4 Histopathological study of the mass after removal. Left: cellular proliferation of histiocytic cells, showing nuclear slits, within an inflammatory background with abundant eosinophils. Note zones of hemorrhage at the upper part of the image (H&E, 100×). Right: S-100 protein-positive immunostaining in Langerhans’ cells (PAP, 100×)
while the bone gap was becoming more evident (Fig. 2). A fineneedle biopsy was performed, which confirmed the diagnosis of histiocytosis X. A bone survey ruled out other bone lesions and revealed a “punched-out” lytic skull lesion (Fig. 3). In view of the severity of the patient’s symptoms and the rapid growth of the subscalp lesion, it was decided to operate on the boy. On 12 December 1999, the child underwent surgical removal of the soft tissue mass and of a 1-cm-thick epidural hematoma. The two lesions were connected through a bone defect. The dura mater was intact. The margins of the skull lesion were excised, and we filled-up the osseous gap with pieces of healthy bone obtained from the adjacent skull.
The histopathological study revealed a polymorphous nuclear cell infiltrate, eosinophils, and Langerhans’ histiocytes (Fig. 4). Hemorrhages within the mass were also particularly noted. At the 10-month follow-up the boy was asymptomatic and his cranial defect was healing nicely.
Discussion Langerhans’ cell histiocytosis, also named histiocytosis X, is a condition characterized by a proliferation of his-
76
tiocytes, which are probably reactive in nature. The disease usually involves the bones, while it is rare for it to involve the viscera [1]. Its etiology is unknown, but some authors suggest that it is a neoplastic process in some instances. The focal form of histiocytosis X is frequently referred to as EG [1, 4, 5]. The disease accounts for 1% of all tumor-like bone lesions and is more frequent in children [1, 2, 3, 4, 5]. The skull is the region most commonly involved by EG [1, 2, 3, 4, 5]. Several authors have associated cases of focal skull lesions with previous head injury [4, 5]. However, it is not clear from the literature whether trauma has a role in the development of EG or whether the injury is the event that attracts attention to a previously unnoticed lesion [5]. In our patient, the symptoms appeared after a fall and worsened following a second, seemingly trivial, head injury. Initial CT findings were interpreted as a depressed skull fracture with an underlying epidural hematoma. The hypodense CT appearance of the extradural lesion was first attributed to the well-known changes in density of nonoperated epidural hematomas. On the other hand, it is not uncommon for an EG to include zones of hemorrhage that are seen during the histopathological study [5]. However, the acute (or subacute) presentation of EG with an epidural hematoma does seem to be exceptional. Manaka et al. reported a 4-year-old girl who showed a collapsible subscalp lesion following a head injury sus-
tained 1 month previously [3]. At operation the superficial lesion was found to end in an intracranial epidural hematoma. In this case the differential diagnosis was established against sinus pericranii [3]. Lee et al. have recently documented an 8-year-old boy who developed an epidural hematoma following a minor head injury [2]. The histopathological study in their patient showed an intracranial epidural hematoma, which was associated with a focal lesion of histiocytosis X. Differential diagnoses in cases of cranial EG manifesting after a head injury have included subcutaneous hematoma, osteomyelitis, depressed cranial fracture, sinus pericranii, and growing skull fracture [3, 4]. With regard to management, we believe that surgery is the option of choice in most cranial lesions of EG that are accessible [4]. The operation in this case permitted confirmation of the diagnosis, evacuation of the epidural hematoma, and removal of the involved bone. A cranioplasty with autologous bone can be performed in the same session to repair the cranial defect [4]. Steroid treatment seems to be ineffective in the presence of large masses such as the one encountered in our patient. Radiotherapy and chemotherapy are best reserved for the treatment of recurrences or of remnants of lesions that have not been totally removed [4, 5]. Acknowledgements The authors wish to express their gratitude to Mr. Saturnino Espin for preparing the illustrations and to Mr. Lee Boyes for reviewing the English manuscript.
References 1. Borrego O, Gilbert-Barness E (1998) Pathological case of the month: eosinophilic granuloma of bone (Langerhans histiocytosis). Arch Pediatr Adolesc Med 52:91–92
2. Lee KW, McLeary MS, Zuppan CW, Won DJ (2000) Langerhans’ cell histiocytosis presenting with an intracranial epidural hematoma. Pediatr Radiol 30:326–328 3. Manaka S, Izawa M, Namata H (1977) Skull tumor simulating sinus pericranii. Case report. J Neurosurg 46:671– 673
4. Martínez-Lage JF, Poza M, Cartagena J, Vicente JP, Biec F, de las Heras M (1991) Solitary eosinophilic granuloma of the pediatric skull and spine. The role of surgery. Child’s Nerv Syst 7:448–451 5. Rawlings CE III, Wilkins RH (1984) Solitary eosinophilic granuloma of the skull. Neurosurgery 15:155–161
