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Congenital inclusion dermoid cyst of the posterior fontanel--a case report.

Introduction

Congenital inclusion dermoid cysts (CIDC) are rare and benign lesions originating from a failure during normal embryologic development of the neural tube (1,2,3,4). Usually, they are found over the anterior fontanel during childhood. (5,6,7,8) The diagnosis of CIDC is suspected on the basis of clinical and radiologic features of the lesion. However, only after surgical excision and histopathological analysis can it be fully confirmed (2,4,9). The goal of treatment is the complete surgical removal of the tumor, which provides very good outcome for the patients with low recurrence rates and satisfactory aesthetic results (2,5).

The authors report for the first time a case of a CIDC over the posterior fontanel. No other similar cases could be retrieved from the world literature.

Case report

A two-year-old boy presented with a history of a bulging lesion on the posterior aspect of the head. The patient was neurologically intact. The physical examination revealed a painless, not pulsatile lesion, adherent to the posterior surface of the cranium and not associated with any kind of draining secretions (figures 1 and 2). The head computed tomography (CT) illustrated an extracranial tumor over the posterior fontanel without intracranial involvement (fig. 3). The lesion was completely resected and the patient was discharged free of any complications. The histopathological analysis was consistent with a dermoid cyst.

Discussion

Dermoid cysts are rare lesions representing 0.1 to 0.2% of the cranial tumors (1,8,9,10,11). Pathologically, they can be classified in three types: the congenital teratoma type, the acquired implantation type, and the congenital inclusion type (2,4,5,6,9). The last one, unlike the teratoma, is not neoplasic and develops due to inclusion of ectodermic and mesodermic cells during the third to fifth week of gestation (8,10).

[FIGURE 1 OMITTED]

[FIGURE 2 OMITTED]

The CIDC is usually diagnosed in childhood, (5,6,11) being rare in the adult population. (5,9,14) Its main location is over the anterior fontanel. (1,2,5,7,11,13,14). The authors describe herein a case occurring over the posterior fontanel which, to their knowledge, is the only case of CIDC of the posterior fontanel reported in the literature.

[FIGURE 3 OMITTED]

During clinical examination, the CIDC is characterized as a soft and painless not pulsatile lesion, lacking inflammatory signs (2). Recently, it has been reported that it can be associated with spinal dysraphism (14), but normally there are no neurologic or systemic complications associated with CIDC (2). Histological features include sebaceous and sweat glands, hair follicles and a thick stratified squamous epithelium that can undergo dystrophic calcifications (3). The cystic secretion can be rich in lipids, due to the sebaceous glands, and have a high concentration of sodium, chloride, potassium, and glucose (2,3,12).

The presented case is in agreement with the aforementioned characteristics but it is unique in the sense that it is located on the posterior fontanel. In addition to the anterior fontanel (2,3,5,7,8,15) there have been reports in the literature of dermoid cysts occurring in the neck (16), sacrococcygeal region, (17) and spleen (18).

The differential diagnosis related to CIDC includes lipoma, hemangioma, epidermoid cyst, sebaceous cyst, encephalocele, subgaleal hematoma, and sinus pericranii (1,2,4,5,6,13,14,15,19). The CT and magnetic resonance imaging (MRI) are useful tools to help making an accurate diagnosis before intervention. The CT demonstrates a cystic, hypodense, extracranial lesion over the fontanel (14). The MRI, most commonly, reveals a hypointense tumor on T1 and hyperintense on T2-weighted images (8). Histopathological studies confirm the diagnosis (2,5,9).

The treatment of choice for CIDC is surgical removal in order to prevent infection and to provide an acceptable aesthetic result (4,7). Recurrence and malignant degeneration are both very rare (15). In the described case, surgical treatment was opted with total resection of the lesion and good aesthetic outcome.

Recibido: 25.02.12

Aceptado: 20.03.12

References

(1.) Adeloye A, Odeku EL. Congenital subgaleal cysts over the anterior fontanelle in Nigerians. Arch Dis Child 1971;46:95-8.

(2.) Aquino HB, Miranda CC, Filho CA, Carelli EF, Borges G. Congenital dermoid inclusion cyst over the anterior fontanel: report of three cases. Arq NeuroPsiquiatr 2003;61:448-52.

(3.) Mirniotopoulos JG, Chiechi MV. Teratomas, dermoids and epidermoids of the head and neck. Radiographics 1995;15:1437-55.

(4.) Pereira CU, Santos DPS, Machado JC, Machado SC, Araujo ES, Costa MD. Cisto dermoide de inclusao congenita localizada na fontanela anterior. Arq Bras Neurocirur 2000;19:32-5.

(5.) Aslan O, Ozveren F, Kotil K, Ozdemir B, Kuscuoglu U, Bilge T. Congenital dermoid cyst of the anterior fontanelle in Turkish children. Neurol Med Chir (Tokyo) 2004;44:150-2.

(6.) Berkamn MZ, Bek S, Derinkok T. Congenital dermoid cyst of the anterior fontanel. Turk Neurosurg 1998;8:105-9.

(7.) Pannel BW, Hendrick EB, Hoffman HJ, Humphreys RP. Dermoid cyst of the anterior fontanelle. Neurosurgery 1982;10:317-23.

(8.) Castro RA, Filho AF, Junior VV. Dermoid cyst of the anterior fontanelle in adults. Arq NeuroPsiquiatr 2007;65:170- 2.

(9.) Carvalho GTC, Fagundes-Pereyra WJ, Marques JAP, Dantas FLR, Sousa AA. Congenital inclusion cysts of the anterior fontanelle. Surg Neurol 2001;56:400-5.

(10.) Pereira WC, Andrade AF, Lopes PG. Cisto dermoide da regiao bregma: relato de dois casos. Arq NeuroPsiquiatr 1969;27:349-52.

(11.) Ojikutu NA, Mordi VPM. Congenital intradiploic dermoid cysts located over the region of the anterior fontanel in adult Nigerians. J Neurosurg 1980;52:724-7.

(12.) Chaudari AB, Ladapo F, Mordi VPN, Choudry HJ, Nassem A, Obe JA. Congenital inclusion cyst of the subgaleal space. J Neurosurg 1982;56:40-4.

(13.) Martinez-Lage JF. Tumores craneales. In: Martinez-Lage JF, Villarejo F (eds) Neurocirugia Pediatrica. Madrid: Ediciones Ergon. 2001; 334-54.

(14.) Borkar S, Bansal A, Mahapatra A. Congenital dermoid inclusion cyst of anterior fontanelle associated with spinal dysraphism: a case report. Childs Nerv Syst 2011;27:2017-19.

(15.) McAvoy JM, Zuckerbraun L. Dermoid cyst of the head and neck in children. Arch Otolaryngol 1976;102:529-31.

(16.) Turkylmaz Z, Karabulut R, Bayazit YA, Sonmez K, Koybasioglu A, Yilmaz M et at. Congenital neck masses in children and their embryologic and clinical features. B-ENT 2008;4:7-18.

(17.) Yang KC. Cystic sacrococcygeal lesions. Zhonghua Wai Ke Za Zhi 1989;27:96-8, 125-6.

(18.) Zganjer M, Zganger V, Cigit I. Huge dermoid cyst of the spleen. Indian J Pediatr 2010;77:454-5.

(19.) Agrawal A, Pratap A, Sinha AK, Agrawal B, Thapa A, Bajracharya T. Epidermoid cyst of anterior fontanelle with clear contents. Surg Neurol 2007;68:313-5.

Endereco do autor:

Prof. Dr. Carlos Umberto Pereira

Av. Augusto Maynard, 245/404

Bairro Sao Jose

49015-380 Aracaju--Sergipe

email: umberto@infonet.com.br

Carlos Umberto Pereira [1], Andre Fabiano Souza de Carvalho [2], Nathalie Serejo Silveira Costa [1], Debora de Melo Cardoso [1], Georgios K. Matis [3], Danilo O de A Silva [3].

[1.] Department of Neurosurgery. Universidade Federal de Sergipe, Aracaju, Sergipe, Brazil.

[2.] Department of Radiology. Servico de Radiologia da Santa Casa de Belo Horizonte. Belo Horizonte, Minas Gerais, Brazil.

[3.] Department of Neurological Surgery. Weill Cornell Medical College, New York, NY, USA.
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Article Details
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Title Annotation:Reporte de casos
Author:Pereira, Carlos Umberto; Souza de Carvalho, Andre Fabiano; Serejo Silveira Costa, Nathalie; de Melo
Publication:Revista Chilena de Neurocirugia
Date:Jan 1, 2012
Words:1206
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