Infection

Cerebellar Abscesses Secondary to Occipital Dermoid Cyst with Dermal Sinus

CASE REPORT
Ali Akhaddar, M.D.,* Mohamed Jiddane, M.D.,* Noureddine Chakir, M.D.,* Rachid El Hassani, M.D.,* Brahim Moustarchid, M.D., and Fouad Bellakhdar, M.D., *Department of Neuroradiology, Hospital des Spe cialite s and Department of Neurosurgery, Avicenne Hospital, University Hospital Centre of Rabat, Rabat, Morocco

Akhaddar A, Jiddane M, Chakir N, El Hassani R, Moustarchid B, Bellakhdar F. Cerebellar abscesses secondary to occipital dermoid cyst with dermal sinus. Case report. Surg Neurol 2002;58: 266 70. BACKGROUND

KEYS WORDS

Cerebellar abscess, dermoid cyst, cranial dermal sinus, posterior fossa dermoid tumors.

Hydrocephalus and cerebellar abscesses as the principal manifestations of posterior fossa dermoid cyst are rare. In addition, extradural dermoid cyst of the posterior fossa has been described in only 9 cases in the literature. We present an unusual case of obstructive hydrocephalus due to cerebellar abscesses induced by an adjacent extradural dermoid cyst with complete occipital dermal sinus.
CASE DESCRIPTION

A 14-month-old child presented with acute raised intracranially pressure, seizures, and meningitis. Neuroradiological studies revealed cerebellar cysts with ring enhancement associated with a contiguous occipital cyst, with compression of the adjacent cisterns and the fourth ventricle causing hydrocephalus. The diagnosis of cerebellar abscesses with congenital occipital defect was briey entertained. The patient was treated by radical excision of the occipital cyst with hair contents, the dermal sinus, and the abscesses through a suboccipital approach, followed by systemic antibiotic therapy with a good outcome. Pathologic examination revealed a dermoid cyst.
CONCLUSION

ntracranial dermoid cysts are rare congenital benign neoplasms that grow slowly as a result of progressive epithelial desquamation and gland secretion within the cyst. They arise from inclusion of ectodermal elements within the neural tube during its closing between the third and fth week of embryonic development [6,12]. Patients with a posterior fossa dermoid cyst and an associated dermal sinus may develop bacterial meningitis or abscess formation of the dermoid itself [10,18,25]. In contrast, cerebellar abscesses as a complication of posterior fossa dermoid cyst and dermal sinus are exceptional, only 14 cases were reported in the literature [8,25]. We report an original case of intradiploic occipital dermoid cyst with intracranial extension and contiguous dermal sinus, causing multiple cerebellar abscesses and obstructive hydrocephalus.

Posterior fossa dermoid cyst should be considered in all children with occipital skin lesions, especially dermal sinus. CT scan and MRI are the methods of choice for further investigation of suspect congenital dermal lesions. Neurosurgical treatment of these malformations should be planned early to prevent the high incidence of infections such as bacterial meningitis and cerebellar abscess. Clinical presentation, diagnostic evaluation, and treatment of these rare lesions are reviewed. 2002 by Elsevier Science Inc.

Case Report
A 14-month old girl with an unremarkable medical history was admitted to our institution with a 2-month history of psychomotor retardation signs and weight loss. Two days before her admission, she become lethargic and developed generalized tonic-clonic seizure, nausea, vomiting, and irritability. Physical examination revealed mild confusion, a temperature of 39C, meningeal signs, and bilateral papilledema. She had horizontal nystagmus without sensory nor motor decit.
2002 by Elsevier Science Inc. 360 Park Avenue South, New York, NY 10010 1710

Address reprint requests to: Dr Ali Akhaddar, 34 rue Oued Ziz, appt no. 7, Agdal, Rabat, Morocco. Received August 6, 2001; accepted May 17, 2002. 0090-3019/02/$see front matter PII S0090-3019(02)00847-9

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(A-C). Contrast-enhanced CT images of the posterior fossa scan showed 3 cystic masses in both cerebellar hemispheres, with ring enhancement of the cystic walls and some edema. Note the well-dened hypodense, occipital intradiploic cystic lesion in the posterior fossa midline with occipital bone defect and epidural intracranial extension (much more hypodense that previous cystic lesions).

Emergency computed tomography (CT) scan (Figure 1) showed 3 cystic masses in both cerebellar hemispheres, with ring enhancement of the cystic walls and some edema. Also, a well-dened, occipital intradiploic cystic lesion was noticed in the posterior fossa midline with an occipital bone defect. the lesion appeared to communicate with the skin and intracranial space. The cerebrospinal uid spaces in the posterior fossa and fourth ventricle were compressed and dilatation of the lateral ventricles was seen. The relationship of the cranial and intracranial cysts to the dura mater was not clear. A diagnosis of cystic, cerebellar space-occupying lesions with obstructive hydrocephalus was made. Because of the urgency of the case, magnetic resonance imaging (MRI) was not performed. After shaving the operation site, a small subcutaneous nodule which measured 5 mm in diameter was seen with a small skin opening without pus. A suboccipital craniotomy was performed. Subtotal removal of a 3-cm whitish, midline, encapsulated cystic mass with abundant hair was accomplished. The cyst was attached to the dura and was adherent to the occipital venous sinus, so a fragment was left in situ. The three cerebellar abscesses were evacuated, followed by excision of all visible capsule. Bacterial investigation revealed Staphylococcus aureus. The patient was given ceftriaxone and metronidazole for 2 months. Histologic examination of the intradiploic occipital cyst conrmed the diagnosis of dermoid tumor. The patients general physical condition and neurologic symptoms improved rapidly. After 8 weeks, she was discharged without any residual cerebellar

or general neurologic symptoms. There is no evidence of recurrence after a follow-up of 3 years.

Discussion
Intracranial dermoid cysts are rare (0.1 to 0.7% of all intracranial tumors) [6,13,20,29]. They mostly occur in the posterior fossa, particularly in the midline position in the vermis or adjacent meninges, or in the cavity of the fourth ventricle [5,9,12]. In the cranial vault, dermoid cysts are often found at the anterior fontanel and the occipital region [17]; more rarely, dermoid cysts may communicate with the skin through a narrow tract lined by epithelium (dermal sinus), which, therefore, contains the glandular architecture of skin, encouraging colonization by micro-organisms. Through this communication, micro-organisms may travel into the cyst with potential risk of deeper suppurations [1,12]. Logue and Till [12] classied posterior fossa dermoid cysts into four groups depending on whether they were extradural or intradural, and on the degree of development of the dermal sinus, whether absent, partial, or complete: (1) extradural dermoid cyst with a complete sinus, (2) intradural dermoid cyst without a dermal sinus, (3) an intradural dermoid cyst with an incomplete dermal sinus, and (4) intradural dermoid with a complete dermal sinus. A recent literature review by Martinez [17] disclosed only 9 patients with extradural dermoids arising in the posterior fossa [4,12,1518,21,22] (Table 1). Connection between an intracranial dermoid cyst and the skin surface provides direct access for bac-

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Akhaddar et al

Summary of the 10 Cases of Occipital Dermal Sinus With Extra Dural Dermoid Cyst Published in the Literature

AUTHORS
Martin [15] Logue [12] Matson [18] Schijman [22] Rubin [21] Martinez [16] Gofn [4] Martinez [17] Martinez [17] Our case

YEAR
1943 1952 1969 1986 1989 1992 1993 1997 1997 2001

AGE/SEX
2y/M 2y/M 2.5y/F 3y/F 27y/M 2y/F 7y/M 6m/F 2y/F 14m/M

CLINICAL PRESENTATION
RICP Occipital scalp swelling Occipital scalp swelling, meningitis Occipital scalp swelling, meningitis RICP Occipital scalp swelling, local infection Occipital scalp swelling Occipital scalp swelling Occipital scalp swelling Acute RICP, seizure, meningitis

RADIOLOGICAL DIAGNOSIS
Plain Radiographs Plain Radiographs, ventriculography Plain Radiographs CT scan CT scan CT scan CT scan, stulography MRI MRI CT scan

LOCALISATION
Interdural Epidural Epidural Interdural Epidural Epidural Interdural Epidural Interdural Epidural

COMMENTS
Epidermoid cyst Torcular compression Empyema Sinus haemorrhage Sinus haemorrhage Cerebellar abscesses

RICP Raised intracranial pressure; M/F Male/Female; y years; m months.

teria on the skin to reach the interior of the tumor and may result in meningitis, abscedation of the dermoid cyst itself, or, most rarely, formation of daughter abscesses within the cerebellar hemisphere, as in our case. To our knowledge, including ours, a total of 28 cases of posterior fossa dermoid cysts with suppuration have been reported [25,8,1114,1720,22 26]. Only 14 cases had cerebellar abscesses (suppuration within the cerebellar parenchyma) [3,5,8, 11,14,19,2226], while the rest had abscedation of the dermoid cyst alone. A case with an extradural empyema was reported by Martinez [17]. Of these 28 cases reviewed, the median age at presentation was 17 months. There were 13 males and 15 females. Sixty-one percent of patients had been treated for past meningitis, and 5 patients (18%) had undergone a local surgical procedure. In the histories of the 28 cases, intermittent pus drainage through a dermal sinus was noted in 6 and two children had recurrent meningitis. Meningitis is the most common presentation. Rarely, dermoid cysts may present with raised intracranial pressure or seizures. Sometimes there is a painless lump under the scalp or a swelling of the occipital scalp [8,17]. A bone defect may be palpable. Plain skull lms often show an oval or circular defect, small or moderate in size (less than 20 mm in diameter) with a sclerotic margin localized along

the midline just below the inion. If it is very small, it may not be visible on plain radiographs. Some cases are associated with vertebral deformities such as hemivertebrae, spina bida, or Klippel-Feil deformity [20,23,27]. The CT and MR appearances of dermoid cysts principally depend upon the amount of fat present in these tumors (these cysts contain lipid metabolites formed from the breakdown products of hair follicles combined with secretions from the sweat and sebaceous glands) [1,22,27]. CT scan shows the size and exact location of the cyst, detects thinning or interruption of the skull tables, and shows the presence of calcication. It also reveals the homogeneous hypodensity of the cyst contents (with values ranging from 0 to 150 HU). The cysts do not enhance with contrast medium. If the dermoid cyst is infected, the density values in the central area of the lesion are higher than the negative values usually found with lipids [3,4,13,17]. One of the characteristic features of extradural dermoid cysts is a well-dened bony destruction of the diploe of the cranial vault [12,21]. Among 28 cases, CT scan examination was performed in 9 patients, 8 of whom presented with obstructive hydrocephalus. Of those patients in the literature, only 4 cases had an MRI [35]. On both T1- and T2-weighted MR images, dermoid cysts exhibit increased signal intensity; on STIR images they show low signal intensity [9,27,29]. MR imaging is helpful in displaying

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Surg Neurol 269 2002;58:266 70

the full anatomic extent of the lesion and its relationship with other structures. Sagittal images demonstrate the typically oblique stalk or tract that links cyst and skin. But the relationship to meningeal structures, dural sinuses, and cerebellar parenchyma have not been reported previously [17,25]. The exact origin of the tumor (intradural or extradural) is established only at operation [17]. The absence of peritumoral edema, related to the slow growth of these lesions, is easily detected on MR imaging and further assists in the differential diagnosis. Because of the emergency situation, MR imaging was not performed for our patient. MR angiography determines the relation of the dermal sinus with the venous sinuses, given the proximity of the torcula. Cerebral angiography usually shows an avascular mass [29]. Fistulography through the dermal sinus should be avoided because of the potential risks of introducing additional micro-organisms intracranial and iatrogenic distension or rupture of the infected cyst [4]. Treatment of dermoid cysts consists of microsurgical excision [6,8,13,29]. Total removal was not always performed because of the rm adhesion of the tumor capsule [1]. Extreme caution is advised when a sinus is found to penetrate the occipital region. Connection between the dermal sinus, dermoid cyst, and the cranial venous conuence is possible, and unanticipated penetration is associated with rapid and fatal exsanguination [22,28]. Concerning cerebellar abscesses, the choice of treatment depends on the clinical status of the patient, as well as on the size, location, and the presence or absence of the capsule. Treatment may consist of antibiotics alone or antibiotics with abscess aspiration or total excision [3,5,7,8,12,25]. When hydrocephalus is present, external ventricular drainage promotes more favorable operative conditions and perhaps decreases the likelihood of permanent CSF diversion [8,23,25]. Our patient remained well after surgical excision without previous external ventricular drainage. Postoperatively, severe neurologic decits were seen in 2 children (7%) and postsurgical mortality was 11% (3 deaths). Mortality and morbidity increase if meningitis (chemical or bacterial) develops. Best results were obtained in cases of early diagnosis and treatment [25]. No recurrence of a posterior fossa dermoid cyst after surgery has been reported in the literature. Recently, Hashmi and Jones [7] reported the reappearance of a cerebellar abscess 20 years after excision of a dermoid cyst with bilateral abscesses.

Conclusion
Posterior fossa dermoid cyst should be considered in all children with an occipital skin lesion, especially a dermal sinus. Preoperative neuroradiological investigations are necessary to determine the location of the dermoid cyst and to detect associated disorders, but they appear to be unable to show the extension of the stula to the dura. Early neurosurgical treatment of these benign malformations should be performed to prevent the development of severe intracranial infection such as bacterial meningitis and cerebellar abscess.
We are indebted to Fatiha-Hiba Chayeb for her secretarial assistance in the preparation of this manuscript.

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