Medical Journal of Dr. D.Y. Patil Vidyapeeth

CASE REPORT
Year
: 2017  |  Volume : 10  |  Issue : 3  |  Page : 297--299

Ruptured intracranial dermoid: Case report and literature review


Pravin Tukaram Survashe, Sachin Parshuram Guthe, Vernon Velho, Anuj Bhide 
 Department of Neurosurgery, Grant Government Medical College and Sir J.J. Group of Hospitals, Mumbai, Maharashtra, India

Correspondence Address:
Pravin Tukaram Survashe
Room No. 702, 300 Resident Quarters, J.J. Hospital Campus, Byculla, Mumbai - 400 008, Maharashtra
India

Abstract

Intracranial dermoid cysts are nonneoplastic, ectodermal inclusion cysts of embryological origin. They contain various ectodermal derivatives including apocrine glands, sebaceous glands, hairs, squamous epithelium, and occasionally teeth. Spontaneous rupture of these cysts is uncommon, and so far very few cases have been reported in the literature. Hereby, we report a case of young male presenting with ruptured intracranial dermoid cyst managed surgically.



How to cite this article:
Survashe PT, Guthe SP, Velho V, Bhide A. Ruptured intracranial dermoid: Case report and literature review.Med J DY Patil Univ 2017;10:297-299


How to cite this URL:
Survashe PT, Guthe SP, Velho V, Bhide A. Ruptured intracranial dermoid: Case report and literature review. Med J DY Patil Univ [serial online] 2017 [cited 2024 Mar 29 ];10:297-299
Available from: https://journals.lww.com/mjdy/pages/default.aspx/text.asp?2017/10/3/297/206576


Full Text

 Introduction



Intracranial dermoid cysts comprise 0.04%–0.6% of all intracranial tumors.[1] They are composed of a thick fibrous capsule lined by stratified squamous epithelium enclosing a thick, viscous, greenish-brown fluid, a mix of lipids, cholesterol crystals, hairs, and decomposed epithelial cells. They increase in size with accumulation of glandular secretions and desquamated cells from the lining epithelium. Symptoms associated with unruptured cysts are usually due to mass effect and slowly progressive in nature, while rupture results in spillage of fatty globules into the ventricles and in subarachnoid space. The irritative effect of these lipid metabolites leads to aseptic granulomatous meningitis, vasospasm, and hydrocephalus. Rupture of these cysts is often a spontaneous process; however, closed head injury has been implicated in certain cases. Early diagnosis and appropriate treatment are essential for improving the disease outcome.[1],[2]

 Case Report



A 21-year-old male was admitted with the symptoms of severe headache since 15 days and two episodes of generalized convulsions. On clinical examination, he was neurologically intact. Magnetic resonance image (MRI) scan of the brain was suggestive of a well-defined extra-axial lesion involving right Sylvian fissure compressing on the right caudate nucleus and frontal lobe with multiple discrete and confluent foci of cerebrospinal fluid (CSF) seeding in basal cisterns and ventricular system suggestive of ruptured dermoid cyst. Lesion was heterogeneous in appearance with hyperintense signals in T1 and T2 sequences which were suppressed in FAT-SAT sequence. Gradient recalled echo images showing extensive blooming suggestive of dermoid cyst [Figure 1],[Figure 2],[Figure 3],[Figure 4],[Figure 5].{Figure 1}{Figure 2}{Figure 3}{Figure 4}{Figure 5}

Patient was operated electively after complete preoperative workup. Orbitofrontal craniotomy was done through a supraorbital incision. Lesion was well-defined, yellowish gray with extensive adhesions with surrounding brain parenchyma. On opening the capsule, it had cheesy material inside with numerous hairs [Figure 6]. After internal decompression, the wall of the cyst was excised in toto taking care to prevent spillage of contents. Endoscope was used to confirm complete removal of the cyst. Postoperatively, lumbar drain was kept for 5 days to drain the contaminated CSF so as to prevent chemical meningitis. Postoperative course was uneventful and patient recovered well.{Figure 6}

Histopathological examination revealed a cyst filled with keratinous material and lined by atrophic epithelial cells along with numerous hair shafts. Cyst wall was composed of fibrocollagenous tissue and infiltrated by inflammatory cells and foreign body granulomas [Figure 7].{Figure 7}

 Discussion



Intracranial dermoid cysts derive from the inclusion of ectodermally committed cells at the time of neural groove closure in embryonic life.[1] They are extra-axial lesions, frequently located in basilar region near the midline at parasellar, frontobasal, and posterior fossa areas.

Clinical presentations vary depending on size and location of the lesion. Seizures and headache are the most common symptoms of uncomplicated supratentorial dermoids.[1],[2] Dermoids that rupture acutely either spontaneously or during surgery result in chemical meningitis that may be severe leading to vasospasm and cerebral infarction. Their slow growth rate often leads to a delay in clinical presentation until the third or fourth decades.

Microscopic appearance of the cyst is typical. The outer cyst is composed of a dense fibrous capsule, whereas the interior is lined with squamous epithelium, hair, and dermal appendages (hair follicles, sebaceous, and sweat glands). Desquamated debris containing cholesterol and keratin are common.[3],[4]

The dermoids show high signal intensity on a T1-weighted image and variable signals on a T2-weighted image with no gadolinium contrast enhancement on MRIs. Characteristically, the tumor is often inhomogeneous due to its mixed composition. Ruptured dermoids typically have high-signal fat droplets within the subarachnoid spaces and intraventricular fat/CSF levels.[1],[2]

In our case, the patient had no signs of meningitis or hydrocephalus. Surgical excision of the cyst helped to reduce the mass effect while postoperative lumbar drainage effectively cleared contaminated CSF. As he was operated through a small eyebrow incision, the surgical results were cosmetically appealing.

 Conclusions



Rupture of intracranial dermoid is usually a spontaneous event with clinically benign to fatal presentation. Early diagnosis with complete surgical excision offers highest cure rate.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

References

1Orakcioglu B, Halatsch ME, Fortunati M, Unterberg A, Yonekawa Y. Intracranial dermoid cysts: Variations of radiological and clinical features. Acta Neurochir (Wien) 2008;150:1227-34.
2Liu JK, Gottfried ON, Salzman KL, Schmidt RH, Couldwell WT. Ruptured intracranial dermoid cysts: Clinical, radiographic, and surgical features. Neurosurgery 2008;62:377-84.
3El-Bahy K, Kotb A, Galal A, El-Hakim A. Ruptured intracranial dermoid cysts. Acta Neurochir (Wien) 2006;148:457-62.
4Ray MJ, Barnett DW, Snipes GJ, Layton KF, Opatowsky MJ. Ruptured intracranial dermoid cyst. Proc (Bayl Univ Med Cent) 2012;25:23-5.