|Year : 2019 | Volume
| Issue : 4 | Page : 226-229
Two rare cysts in the same patient: An unusual case of headache in the emergency department
Naveed Syed1, Hasan Qayyum1, Quatullah Rustum2, Ayesha Musabbah Al Memari1
1 Department of Emergency, Mafraq Hospital, Abu Dhabi, UAE
2 Department of Radiology and Clinical Imaging, Al Mafraq Hospital, Abu Dhabi, UAE
|Date of Submission||19-Mar-2019|
|Date of Acceptance||15-Jul-2019|
|Date of Web Publication||11-Nov-2019|
Department of Emergency, Mafraq Hospital, P. O. Box: 2951, Abu Dhabi
Source of Support: None, Conflict of Interest: None
An intracranial colloid cyst is a rare, slow-growing cyst typically found near the foramen of Monro. The mechanisms of its origin are still debated. It has a reported occurrence of 0.2%–2% of all intracranial tumours. A nasopharyngeal/Tornwaldt's cyst is also a rare developmental benign cyst commonly present in the midline of the posterior nasopharynx with a reported incidence of 1.4%–3.3% on autopsy specimens and 0.2%–5% on magnetic resonance imaging. To the best of our knowledge, while a few reports are available where these have been reported separately in different patients, the contemporaneous coexistence of these 2 cysts in the same patient has not been reported before.
Keywords: Colloid cyst, foramen of Monro, headache, hydrocephalus, magnetic resonance imaging, marsupialization, red flags, third ventricle, Tornwaldt's cyst
|How to cite this article:|
Syed N, Qayyum H, Rustum Q, Al Memari AM. Two rare cysts in the same patient: An unusual case of headache in the emergency department. Hamdan Med J 2019;12:226-9
|How to cite this URL:|
Syed N, Qayyum H, Rustum Q, Al Memari AM. Two rare cysts in the same patient: An unusual case of headache in the emergency department. Hamdan Med J [serial online] 2019 [cited 2020 Dec 4];12:226-9. Available from: http://www.hamdanjournal.org/text.asp?2019/12/4/226/270673
| Introduction|| |
Colloid cysts are rare intracranial cystic lesions containing gelatinous material accounting for 0.2%–2% of all intracranial tumours. They mainly rise from the roof of the anterior third ventricle which comprises 15%–20% of all intraventricular space occupying lesions. Extraventricular locations are scarcely reported in the literature. The origin of these cysts is still a debate with most studies showing a neuroepithelium lining on embryological examination and immunohistochemistry. Other studies show endodermal rather than neuroectodermal cells. On histopathological examination, they are benign cysts. Unexpected and serious complications can occur depending on location, size and rapidity of growth.
Tornwaldt's cysts originate from a developmental remnant of the embryonal notochord and can cause clinically troublesome symptoms following an infection like halitosis, persistent nasopharyngeal discharge, Eustachian tube More Details blockage and a sore throat. These are rare and benign midline nasopharyngeal mucosal cysts with a reported incidence of 1.4%–3.3% on autopsy specimens and 0.2%–5% on magnetic resonance imaging (MRI). They are non-gender specific and commonly occur in the second and fourth decades of life as seen in our patient.
| Case Report|| |
A 21-year-old female presented to our emergency department (ED) complaining of an intermittent headache of 1-month duration, worse over the last 3 days. She attributed this headache to a trivial head injury 1 month back. The headache was frontal in location with a tightband-like character around her head, worse in the evenings and on bending forwards, partially alleviated with oral paracetamol and not associated with fever, neck stiffness, motor or sensory loss. After receiving painkillers in our ED, no improvement was noted. Non-contrast computed tomography (CT) imaging of the brain showed a single 3 mm × 5 mm hyperdense well-circumscribed lesion at opening of foramen of Monro without hydrocephalus, consistent with a colloid cyst [Figure 1]. Another lesion in the posterior nasopharynx was noted to be midline, low density and well circumscribed suggestive of a Tornwaldt's cyst or nasopharyngeal cyst [Figure 2].
|Figure 1: Computed tomography image showing mid-line hyperdense focal lesion 8 mm × 6 mm at level of foramen of Monro|
Click here to view
|Figure 2: Computed tomography scan image show midline well-defined cystic nasopharyngeal lesion consistent with Tornwaldt's cyst|
Click here to view
After a neurosurgical consult, she was admitted for analgesia and neuro-observation. A subsequent MRI brain scan the next day revealed a 7 mm × 6 mm well-defined posterior nasopharyngeal mucosal lesion consistent with a Tornwaldt's cyst [Figure 3] and [Figure 4] and a 3 mm × 5 mm isointense grey matter lesion at the foramen of Monro consistent with a colloid cyst [Figure 5] and [Figure 6]. After consultation with the ear, nose and throat team, the patient was deemed suitable for conservative non-surgical management and clinic follow-up with a plan for interval imaging in the future.
|Figure 3: T2 magnetic resonance imaging images showing hyperintense well-circumscribed lesion in nasopharyngeal area|
Click here to view
|Figure 4: T1 magnetic resonance imaging images show isointense to grey matter well-defined lesion in nasopharyngeal area|
Click here to view
|Figure 5: T2 magnetic resonance imaging image shows mid-line isointense to grey matter tiny lesion at level of foramen of Monro – consistent with colloid cyst|
Click here to view
|Figure 6: Flair magnetic resonance imaging image shows iso intense to grey matter lesion at foramen of Monro – consistent with colloid cyst|
Click here to view
| Discussion|| |
The presentation of non-traumatic headache in ED is a common one, with reported attendances ranging from 0.5% to 4.5% of annual figures.
Non-traumatic headaches can be broadly classified into two groups – primary and secondary headaches. Migraine, tension headaches and trigeminal autonomic cephalalgias are the main causes of primary headache. When a new headache occurs for the first time in close temporal relation to another disorder known to cause headache, or fulfils other criteria for causation by that disorder, the new headache is coded as a secondary headache attributed to the causative disorder.
A detailed history incorporating red flags is mandated when differentiating primary from secondary, i.e., more sinister headaches. Emergency physicians should follow a structured history and examination to establish a need for urgent imaging and safe disposal. As collated in [Table 1], National Institute for Health and Care Excellence guidelines elaborate on a set of criteria in patients presenting with headaches who require further investigation or follow-up. As outlined in [Table 2], Dodick described and subsequently updated the popular 'SNOOP4' mnemonic which summarises headache red flags as an aide memoire.
|Table 1: Assessment of headache (from NICE guideline CG 150 Headache in over 12 s)|
Click here to view
|Table 2: SNOOP4 mnemonic for secondary headache (from Dodick D. Semin Neurol 2010;30:74-81.)|
Click here to view
Tornwaldt's cyst is a developmental cyst which is benign, found in midline of the nasopharynx and formed from a gap developing in the nasopharynx at the junction where the notochord bonds with the pharyngeal ectoderm leading to an outpouching. Epithelium lined in this diverticulum secretes mucus which forms contents of the cyst. Different theories around the formation of this cyst have been suggested. One suggests a swelling of the pharyngeal pouch opening during inflammation leading to accumulation of contents while another theory implies an iatrogenic origin during surgical procedures (like adenoidectomy) or an injury of the pharyngeal duct causing stenosis and scarring. Usually asymptomatic, if symptoms develop, a Tornwaldt's cyst can present with middle ear problems like ear ache, chronic serous otitis media, eustachian tube blockage, leaking sinus tract and chronic pain.
Differential diagnoses of nasopharyngeal cyst include a branchial cleft cyst, Rathke's cleft, meningoceles, encephalocele, meningomyelocele and adenoid cysts. On CT imaging, lesions are well circumscribed, hypodense with fluid intensity but no enhancement on contrast. MRI is the investigation of choice showing imaging characteristics which are variable (commonly high signal intensity images on T2 sequencing and hypo- or hyper-intense on T1 image series). This is attributed to proteinaceous contents of these cysts. Most asymptomatic lesions require no treatment but excision or marsupialization of the cyst is the treatment of choice for refractory symptoms.
Colloid cysts are commonly located in the third ventricle but can be seen in any part of the nervous system. They need to be distinguished from lesions protruding into the third ventricle like an astrocytoma or a pinealoma. The origin of colloid cysts is still a debate, and they can contain various materials such as hemosiderin, mucin and cholesterol, giving them a variable appearance on imaging.
They can overgrow over the foramen of Monro or rupture in the third ventricle and obstruct cerebrospinal fluid outflow, leading to serious complications such as hydrocephalus, elevated intracranial pressure (ICP), herniation of brain and death. Colloid cysts sometimes have a ball valve-like action leading to drop attacks or intermittent intense frontal headaches as seen in our patient. Elevated ICP could lead to herniation of the mesial temporal lobe through the opening in the tentorial falx leading to sudden death. Patients with these cysts may present to physicians with various complaints including headache, dizziness, vomiting, weakness and numbness of lower extremities.
If no imaging is done, most of the colloid cystic lesions are left undiagnosed during life or detected incidentally. Plain CT imaging shows high-density masses in the third ventricle with or without hydrocephalus, which are difficult to differentiate from other high-density masses. MRI signal characteristics are also variable. They can appear hypo-, iso- or hyperintense on T1- or T2-weighted sequences. No significant enhancement on post-contrast studies is noted. Radiological diagnosis is usually straightforward due to their characteristic location and appearances. Many other imaging modalities such as ventriculography, angiography and air contrast studies demonstrate lateral ventricular dilatation and show the outline of the cyst. Colloid cysts are treated surgically if they are symptomatic and require treatment. Placement of a ventriculoperitoneal shunt in case of hydrocephalus without removing slow-growing cystic lesions has shown satisfactory results. Decompression of cysts by aspiration under stereotaxic control has also been effective in some cases.
It can be hypothesised that both of the aforementioned cysts have a common embryological association considering their coexistence in the same patient.
| Conclusion|| |
To the best of our knowledge, a colloid cyst in association with a Tornwaldt's cyst has not yet been reported contemporaneously in the same patient in scientific literature, possibly suggesting a common embryological origin of both cysts. Colloid cysts are known to cause hydrocephalus and sudden death. Tornwaldt's cysts are commonly asymptomatic but if enlarged can give rise to halitosis, eustachian tube blockage and prevertebral spasm.
Emergency physicians should be aware of atypical presentations of headache and its red flag signs. Knowledge of international consensus criteria for evaluating non-traumatic headache would help us differentiate benign primary headaches from the more sinister secondary ones.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Laidlaw J, Kaye AH. Kaye AH, Laws ER. Colloid cysts. In: Brain Tumours: An Encyclopaedic Approach. New York, London: Churchill Livingstone; 2012. p. 849-63.
Lach B, Scheithauer BW, Gregor A, Wick MR. Colloid cyst of the third ventricle. A comparative immunohistochemical study of neuraxis cysts and choroid plexus epithelium. J Neurosurg 1993;78:101-11.
Jyotirmay H, Kumar SA, Preetam P, Manjunath D, Bijiraj VV. Recent trends in the management of thornwaldts cyst: A case report. J Clin Diagn Res 2014;8:KD03-4.
Grimaldi D, Cevoli S, Cortelli P. Headache in the emergency department. How to handle the problem? Neurol Sci 2008;29 Suppl 1:S103-6.
Headache Classification Committee of the International Headache Society (IHS) the international classification of headache disorders, 3rd
edition. Cephalalgia 2018;38:1-211.
Dodick DW. Pearls: Headache. Semin Neurol 2010;30:74-81.
Mukherji SK. Pharynx. In: Som PM, Curtin HD, editors. Head and Neck Imaging. 4th
ed. St. Louis: Mosby; 2003. p. 1507-9.
Yanagisawa E, Yanagisawa K. Endoscopic view of thornwaldt cyst of the nasopharynx. Ear Nose Throat J 1994;73:884-5.
Yousem DM. Case Review: Head and Neck Imaging. St. Louis: Mosby; 1998.
Peyton WT, French LA, Baker AB. Intracranial neoplasms. In: Baker AB, editor. Clinical Neurology. 2nd
ed. New York: Hoeber – Harper International Edition; 1965. p. 482-554.
Thomas DG, Barnard RO, Kendall BE, Mckeran RO, Darling JL, Godlee JN. Cerebral tumours. In: Swash M, Oxbury J, editors. Clinical Neurology. 1st
ed. Edinburgh: Churchill Livingstone; 1991. p. 1077-8.
Brain WR. Brain's Diseases of the Nervous System. 8th
ed. Oxford: Oxford University Press; 1977. p. 246-74.
Cobb CD, Youmans JR. Brain tumours of disordered embryognesis in adults. In: Youmans JR, editor. Neurological Surgery. 2nd
ed., Vol. 5. Philadelphia: WB Saunders Company; 1982. p. 2923-8.
Adams RD, Victor M. Principles of Neurology. 4th
ed. New York: McGraw Hill; 1989. p. 537.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]
[Table 1], [Table 2]