<%server.execute "isdev.asp"%> Posterior fossa infected dermoid with congenital heart disease: A novel hypothesis of an unusual association Teegala R - J Pediatr Neurosci
home : about us : ahead of print : current issue : archives search instructions : subscriptionLogin 
Users online: 2366      Small font sizeDefault font sizeIncrease font size Print this page Email this page

  Table of Contents    
Year : 2015  |  Volume : 10  |  Issue : 3  |  Page : 250-253

Posterior fossa infected dermoid with congenital heart disease: A novel hypothesis of an unusual association

Department of Neurosurgery, Alluri Sita Ramaraju Academy of Medical Sciences, West Godavari, Andhra Pradesh, India

Date of Web Publication18-Sep-2015

Correspondence Address:
Ramesh Teegala
Department of Neurosurgery, Alluri Sita Ramaraju Academy of Medical Sciences, Room No: 116, Eluru, West Godavari - 534 004, Andhra Pradesh
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/1817-1745.165681

Rights and Permissions



Intracranial dermoid cysts commonly present as a discharging sinus, local swelling, mass lesion, or abscess formation. These can sometimes be found in association with congenital anomalies. The author presents two original cases of infected posterior fossa dermoid associated with congenital heart diseases (CHDs) that is very rare. The embryologic basis for this unique occurrence is reviewed, and a new hypothesis proposed. Two infants with CHD presented with infected midline posterior fossa dermoid. Excision of the dermoid cyst with the sinus tract was performed. Postoperative period was uneventful. Both the infants had undergone surgery for congenial heart disease a few months prior to the present clinical presentation with uneventful recovery. Infected posterior fossa dermoid cyst without a discharging sinus should prompt a thorough examination to detect CHD. Early diagnosis and timely management results in better outcome.

Keywords: Congenital anomalies, congenital heart disease, dermoid abscess, posterior fossa dermoid

How to cite this article:
Teegala R. Posterior fossa infected dermoid with congenital heart disease: A novel hypothesis of an unusual association. J Pediatr Neurosci 2015;10:250-3

How to cite this URL:
Teegala R. Posterior fossa infected dermoid with congenital heart disease: A novel hypothesis of an unusual association. J Pediatr Neurosci [serial online] 2015 [cited 2023 Jan 30];10:250-3. Available from: https://www.pediatricneurosciences.com/text.asp?2015/10/3/250/165681

   Introduction Top

Dermoid cysts are congenital, nonneoplastic lesions comprising of epidermal and dermal derivatives. [1],[2],[3] These are thought to develop from the totipotent ectodermal cells that remain within the developing neural tube. [4],[5] Dermoid cysts have a predilection to localize along the midline and are often associated with a dermal sinus tract. [4],[6],[7],[8] Patients with discharging dermal sinus may develop bacterial meningitis or abscedation of dermoid itself [9],[10],[11],[12],[13] and thus present early. Noninfected dermoids usually present late with either subtle signs or as part of the syndromic association. [14] Association of cranial dermoid tumors with congenital heart disease (CHD) is not reported in the English literature.

The author reports two original cases of infected posterior fossa dermoid and their rare association with CHD. The diagnosis, management, and the embryological basis of this clinical presentation are discussed in detail.

   Case Reports Top

Case 1

A 1-year-old male infant, a known case of total anomalous pulmonary venous circulation (TAPVC) presented with features of meningitis for one week. He had undergone corrective surgery for TAPVC three weeks prior to his presentation. On examination, he was febrile with altered sensorium. Fundoscopy showed bilateral gross papilledema. A large posterior fossa ring enhancing mass lesion with obstructive hydrocephalus [Figure 1] was seen on emergency contrast enhanced computed tomography (CECT) scan of the brain.
Figure 1: Contrast-enhanced computed tomography brain of case 1 showing (a) large ring enhancing cerebellar mass lesion causing obstructive hydrocephalus. It also reveals a hyper dense subcutaneous dermal sinus tract (b) bone window showing suboccipital midline bony defect

Click here to view

An emergency suboccipital craniectomy and excision of the dermoid abscess and inflamed subcutaneous nodule was done [Figure 2]. Cyst contained abundant hair and thick pus. Culture from the pus revealed coagulase negative Staphylococcus aureus. The infant had an uneventful postoperative recovery. He was given culture-based antibiotics for 6 weeks. Histological examination of the cyst confirmed the diagnosis of the dermoid tumor.
Figure 2: Clinical photograph taken after the hair clipping of case 1 showing the skin dimple (arrow) over the subcutaneous nodule without evidence of discharging sinus

Click here to view

Case 2

A 1-year-old male infant presented with high-grade fever associated with vomiting. On examination, he was febrile, conscious, and responding to commands. There was an inflamed nondischarging dermal nodule over suboccipital area. He was a known case of CHD with a perimembranous ventricular septal defect (VSD). He underwent closure of the defect 8 months before the presentation. He was one of the twins, born to nonconsanguineous parents. Mother had gestational diabetes mellitus (GDM) and was delivered by elective lower segment caesarean section. CECT of the brain revealed a subcutaneous nodule in the suboccipital area [Figure 3]. Blood culture revealed S. aureus infection. A subsequent magnetic resonance imaging brain revealed a posterior fossa lesion, which was continuing with the subcutaneous nodule through a bony defect [Figure 4].
Figure 3: Computed tomography brain of case 2 showing (a) subcutaneous nodule, (b) cutaneous sinus tract and (c) bone window showing the small midline bony defect

Click here to view
Figure 4: Noncontrast T1-weighted images magnetic resonance imaging brain scan of case 2 showing (a) small hyperintense subdural lesion with sinus tract. (b) Sinus tract continuing with the subcutaneous nodule (red arrow). (c) Sinus tract traversing through the skin (black arrow)

Click here to view

Elective suboccipital craniectomy and excision of the dermoid, subcutaneous nodule, and dermal sinus tract was performed. His postoperative recovery was good.

   Discussion Top

Intracranial dermoid cysts are unusual congenital, slowly progressive tumors accounting for approximately 0.1-0.7% of all intracranial tumors. [1],[15],[16],[17],[18] They occur predominantly in the posterior fossa, particularly in the midline. [3],[19],[20],[21],[22] In about 66%, the dermoid cysts are associated with some form of dermal sinus. [4] Some of these dermoid cysts can get infected with dermal colonizing bacteria and cause deeper suppurations. [13],[15],[23] Hence, congenital dermoid cysts associated with discharging sinuses, present early in childhood due to infection or abscess formation. [13]

About one-third of dermoid cysts are not related to discharging sinus. These cysts may present late in life in association with other developmental defects. [4],[14],[21],[24],[25],[26],[27],[28],[29] Intracranial dermoid cysts can sometimes be associated with chromosomal abnormalities like  Klippel-Feil syndrome More Details. [30] There are a few case reports on dermoid cysts in association with congenital abnormalities [30] like Dandy-Walkers cyst, [4] corpus callosal agenesis, [4] and diastematobulbia. [31] Exact incidence of congenital anomalies associated with dermoid cysts is not known. [4],[14],[19],[24],[28],[31],[32],[33] To the authors knowledge, this is the first case report on dermoid cyst found in association with CHD. Possible embryological basis of this association is reviewed in this paper.

Embryogenesis and hypothesis of dermoid association with congenital heart disease

Dermoid cysts are thought to arise from a defect in the closure of the neural tube during the 3 rd to 5 th weeks of gestation. [5],[14] Simultaneously, development of cardiovascular system occurs during the 3 rd week of intrauterine life. Cardiac progenitor cells that lie in the epiblast, immediate lateral to the primitive streak migrate through the streak to form the endocardial cushion cells. Furthermore, the neural crest cells, migrating from the edges of the neural folds in the midbrain region, contribute to the endocardial cushion formation in the atrioventricular, and conotruncal regions. [34],[35],[36] An abnormality in endocardial cushion formation leads to many cardiac malformations, including ASD and VSDs, and defects involving the great vessels. Since the cells populating the endocardial cushion cells include the neural crest cells, a defect in the neural crest cells can lead to abnormal migration, proliferation or differentiation of endocardial cushion cells. This can often produce both heart and craniofacial defects in the same individual [Figure 5]. Defective neural crest or endocardial cushion cells may be because of teratogenic or genetic causes. [35],[37]
Figure 5: Flow chart showing the proposed embryogenic hypothesis for the association of dermoid cyst with congenital heart diseases

Click here to view

Association of the dermoid cyst with TAPVC in our second case could be an incidental finding. However, there are no such studies showing the teratogenic and genetic causes accounting for common cardiac and craniofacial defects. GDM was shown to be associated with these abnormalities as it happened in one of our cases. [38] Defects in the chromosome 5 and 22 are associated with isolated congenital cardiac diseases. [39],[40] Nevertheless, there is no proven evidence stating that the same chromosomal defects are related to neural tube defects.

To understand this unusual association better, further genetic studied are required.

   Conclusions Top

It is wiser to consider posterior fossa dermoid cysts in all children with midline occipital skin lesions. Apart from therapeutic implications, these two cases may throw some light on the embryogenesis and the possible correlation of congenital cardiac anomalies with intracranial dermoid cysts. The exact incidence and prevalence of this unusual association needs further evaluation.


Sincere thanks to Dr. Madhavi Latha for her invaluable support in editing the manuscript.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Naderi S, Nejat F, Shahjouei S, El Khashab M. Cranial dermal sinus: Presentation, complications and management. Pediatr Neurosurg 2012;48:86-92.  Back to cited text no. 1
Schijman E, Monges J, Cragnaz R. Congenital dermal sinuses, dermoid and epidermoid cysts of the posterior fossa. Childs Nerv Syst 1986;2:83-9.  Back to cited text no. 2
Vlachakis E, Alexiou GA, Stefanaki K, Sfakianos G, Prodromou N. Posterior fossa dermoid cyst. J Pediatr Neurosci 2012;7:79.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
Caldarelli M, Massimi L, Kondageski C, Di Rocco C. Intracranial midline dermoid and epidermoid cysts in children. J Neurosurg 2004;100 5 Suppl Pediatrics: 473-80.  Back to cited text no. 4
Cobbs CS, Pitts LH, Wilson CB. Epidermoid and dermoid cysts of the posterior fossa. Clin Neurosurg 1997;44:511-28.  Back to cited text no. 5
Fornari M, Solero CL, Lasio G, Lodrini S, Balestrini MR, Cimino C, et al. Surgical treatment of intracranial dermoid and epidermoid cysts in children. Childs Nerv Syst 1990;6:66-70.  Back to cited text no. 6
Lindbichler F, Braun H, Raith J, Ranner G, Kugler C, Uggowitzer M. Nasal dermoid cyst with a sinus tract extending to the frontal dura mater: MRI. Neuroradiology 1997;39:529-31.  Back to cited text no. 7
Zerris VA, Annino D, Heilman CB. Nasofrontal dermoid sinus cyst: Report of two cases. Neurosurgery 2002;51:811-4.  Back to cited text no. 8
Erdem G, Topaloglu H. Abscess formation in posterior fossa dermoid cysts. Childs Nerv Syst 1997;13:297.  Back to cited text no. 9
Hayek G, Mercier P, Fournier HD, Menei P, Pouplard F, Guy G. Dermal sinus and dermoid cyst revealed by abscess formation in posterior fossa. Report of 2 pediatric cases and review of the litterature. Neurochirurgie 2001;47 (2-3 Pt 1):123-7.  Back to cited text no. 10
Hsu ST, Yu-Yun Lee J, Chao SC, Hsieh MY, Huang CC. Congenital occipital dermal sinus with intracranial dermoid cyst complicated by recurrent Escherichia coli meningitis. Br J Dermatol 1998;139:922-4.  Back to cited text no. 11
Tekkök IH, Baeesa SS, Higgins MJ, Ventureyra EC. Abscedation of posterior fossa dermoid cysts. Childs Nerv Syst 1996;12:318-22.  Back to cited text no. 12
Cai CQ, Zhang QJ, Hu XL, Wang CX. Dermoid cyst of the posterior fossa associated with congenital dermal sinus in a child. World J Pediatr 2008;4:66-9.  Back to cited text no. 13
Chandra PS, Gupta A, Mishra NK, Mehta VS. Association of craniovertebral and upper cervical anomalies with dermoid and epidermoid cysts: Report of four cases. Neurosurgery 2005;56:E1155.  Back to cited text no. 14
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.  Back to cited text no. 15
Guidetti B, Gagliardi FM. Epidermoid and dermoid cysts. Clinical evaluation and late surgical results. J Neurosurg 1977;47:12-8.  Back to cited text no. 16
Lunardi P, Missori P, Gagliardi FM, Fortuna A. Dermoid cysts of the posterior cranial fossa in children. Report of nine cases and review of the literature. Surg Neurol 1990;34:39-42.  Back to cited text no. 17
Yasargil MG, Abernathey CD, Sarioglu AC. Microneurosurgical treatment of intracranial dermoid and epidermoid tumors. Neurosurgery 1989;24:561-7.  Back to cited text no. 18
Groen RJ, van Ouwerkerk WJ. Cerebellar dermoid tumor and occipital meningocele in a monozygotic twin: Clues to the embryogenesis of craniospinal dysraphism. Childs Nerv Syst 1995;11:414-7.  Back to cited text no. 19
Higashi S, Takinami K, Yamashita J. Occipital dermal sinus associated with dermoid cyst in the fourth ventricle. AJNR Am J Neuroradiol 1995;16 4 Suppl: 945-8.  Back to cited text no. 20
Kuribayashi K, Nakasu S, Matsumura K, Matsuda M, Handa J. Dermoid cyst in the fourth ventricle associated with Klippel-Feil syndrome. No To Shinkei 1993;45:747-51.  Back to cited text no. 21
Raghunath A, Indira Devi B, Bhat DI, Somanna S. Dermoid cysts of the posterior fossa - Morbid associations of a benign lesion. Br J Neurosurg 2013;27:765-71.  Back to cited text no. 22
Logue V, Till K. Posterior fossa dermoid cysts with special reference to intracranial infection. J Neurol Neurosurg Psychiatry 1952;15:1-12.  Back to cited text no. 23
Diekmann-Guiroy B, Huang PS. Klippel-Feil syndrome in association with a craniocervical dermoid cyst presenting as aseptic meningitis in an adult: Case report. Neurosurgery 1989;25:652-5.  Back to cited text no. 24
Drolshagen LF, Standefer M. Dense dermoid cyst of the posterior fossa. AJNR Am J Neuroradiol 1991;12:317.  Back to cited text no. 25
González-Darder JM, Feliu-Tatay R, Pesudo-Martínez JV, Vera-Román JM. Klippel-Feil syndrome associated with posterior fossa dermoid cyst. Case report. Neurol Res 2002;24:501-4.  Back to cited text no. 26
Kennedy PT, McAuley DJ. Association of posterior fossa dermoid cyst and Klippel-Feil syndrome. AJNR Am J Neuroradiol 1998;19:195.  Back to cited text no. 27
Muzumdar D, Goel A. Posterior cranial fossa dermoid in association with craniovertebral and cervical spinal anomaly: Report of two cases. Pediatr Neurosurg 2001;35:158-61.  Back to cited text no. 28
Whittle IR, Besser M. Congenital neural abnormalities presenting with mirror movements in a patient with Klippel-Feil syndrome. Case report. J Neurosurg 1983;59:891-4.  Back to cited text no. 29
McLaughlin N, Weil AG, Demers J, Shedid D. Klippel-Feil syndrome associated with a craniocervico-thoracic dermoid cyst. Surg Neurol Int 2013;4 Suppl 2:S61-6.  Back to cited text no. 30
Ciurea AV, Coman T, Tascu A, Ionescu V. Intradural dermoid tumor of the posterior fossa in a child with diastematobulbia. Surg Neurol 2005;63:571-5.  Back to cited text no. 31
Douvoyiannis M, Goldman DL, Abbott IR 3 rd , Litman N. Posterior fossa dermoid cyst with sinus tract and meningitis in a toddler. Pediatr Neurol 2008;39:63-6.  Back to cited text no. 32
Soto-Ares G, Vinchon M, Delmaire C, Pruvo JP, Vallee L, Dhellemes P. Report of eight cases of occipital dermal sinus: An update, and MRI findings. Neuropediatrics 2001;32:153-8.  Back to cited text no. 33
Sadler TW. Susceptible periods during embryogenesis of the heart and endocrine glands. Environ Health Perspect 2000;108 Suppl 3:555-61.  Back to cited text no. 34
Sadler TW. Embryology of neural tube development. Am J Med Genet C Semin Med Genet 2005;135C: 2-8.  Back to cited text no. 35
Sadler TW, Rasmussen SA. Examining the evidence for vascular pathogenesis of selected birth defects. Am J Med Genet A 2010;152A: 2426-36.  Back to cited text no. 36
Sulik KK, Sadler TW. Postulated mechanisms underlying the development of neural tube defects. Insights from in vitro and in vivo studies. Ann N Y Acad Sci 1993;678:8-21.  Back to cited text no. 37
Schaefer-Graf UM, Buchanan TA, Xiang A, Songster G, Montoro M, Kjos SL. Patterns of congenital anomalies and relationship to initial maternal fasting glucose levels in pregnancies complicated by type 2 and gestational diabetes. Am J Obstet Gynecol 2000;182:313-20.  Back to cited text no. 38
Gawde H, Patel ZM, Khatkhatey MI, D′Souza A, Babu S, Adhia R, et al. Chromosome 22 microdeletion by F.I.S.H. in isolated congenital heart disease. Indian J Pediatr 2006;73:885-8.  Back to cited text no. 39
Sarri C, Gyftodimou Y, Grigoriadou M, Pandelia E, Kalogirou S, Kokotas H, et al. Supernumerary marker chromosome 5 diagnosed by M-FISH in a child with congenital heart defect and unusual face. Cytogenet Genome Res 2006;114:330-7.  Back to cited text no. 40


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]


Print this article  Email this article
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
    Article in PDF (850 KB)
    Citation Manager
    Access Statistics
    Reader Comments
    Email Alert *
    Add to My List *
* Registration required (free)  

   Case Reports
    Article Figures

 Article Access Statistics
    PDF Downloaded64    
    Comments [Add]    

Recommend this journal