Sudden death due to a colloid cyst of the third ventricle: Report of three cases with a special sign at autopsy

Sudden death due to a colloid cyst of the third ventricle: Report of three cases with a special sign at autopsy

Forensic Science International 189 (2009) e33–e36 Contents lists available at ScienceDirect Forensic Science International journal homepage: www.els...

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Forensic Science International 189 (2009) e33–e36

Contents lists available at ScienceDirect

Forensic Science International journal homepage: www.elsevier.com/locate/forsciint

Case report

Sudden death due to a colloid cyst of the third ventricle: Report of three cases with a special sign at autopsy Serafettin Demirci a,*, Kamil Hakan Dogan a, Zerrin Erkol b, Mete Korkut Gulmen c a

Selcuk University, Meram Medical School, Department of Forensic Medicine, Akyokus Mevkii, 42080 Meram, Konya, Turkey Abant Izzet Baysal University, Medical Faculty, Department of Forensic Medicine, Bolu Turkey c Cukurova University, Medical Faculty, Department of Forensic Medicine, Adana, Turkey b

A R T I C L E I N F O

A B S T R A C T

Article history: Received 3 December 2008 Received in revised form 2 April 2009 Accepted 14 April 2009 Available online 15 May 2009

Colloid cysts of the third ventricle are rare benign cysts but they may be potentially life-threatening. Three cases of sudden death resulting from colloid cysts of the third ventricle are presented. The first and second cases were treated for migraine headaches. In the first case, the patient was a 24-year-old woman who presented to the hospital with a severe headache and was sent back home after medical treatment. Six hours later, she was found dead in her bed. The second case was a 21-year-old woman who experienced a severe headache, dizziness and vomiting 1 day prior to her death. She was transported to the hospital, where she was pronounced dead upon arrival. The third case was a 25-year-old man who experienced headaches and vomiting and was diagnosed with and medically treated for sinusitis. He lost consciousness and was taken to hospital, where he was pronounced dead on arrival. During the autopsy of all three cases, there was a grey transillumination area observed that occurred due to the stretching of tissue at the base of brain between the corpus mamillare and chiasma opticum. Dissection of the brain revealed a colloid cyst of the third ventricle. To avoid such fatal complications, prompt diagnosis using CT or MRI is essential in patients who have a long-standing history of intermittent headaches. During the autopsy of the sudden deaths of people with medical antecedents of headaches, if a grey color is observed between the chiasma opticum and the corpus mamillare in the base of the brain, a colloid cyst should be considered and this region should be dissected and examined carefully. ß 2009 Elsevier Ireland Ltd. All rights reserved.

Presented at the 18th Triennial Meeting of the International Association of Forensic Sciences, New Orleans, USA, July 21–25, 2008 Keywords: Colloid cyst Headache Sudden death Third ventricle Cerebral edema

1. Introduction A broad spectrum of neuropathological abnormalities may be encountered during medicolegal autopsies, e.g., brain tumors, trauma, intoxication, infectious diseases or vascular diseases [1]. Colloid cysts are considered to be an ectopic endodermal migration in the velum interpositum that occurs during the development of the central nervous system. They typically originate from the rostral aspect of the third ventricle roof and project inferiorly, thus occupying the anterosuperior quadrant adjacent to the foramina of Monro [2,3]. The colloid cyst is attached to the wall of the third ventricle via a stem that provides its partial stability in the lumen of the ventricle [4–6]. Colloid cysts of the third ventricle are rare, accounting for approximately 0.5–2.0% of all intracranial tumors and approximately 10–20% of intraventricular tumors [2]. It is the most

* Corresponding author. Tel.: +90 332 223 6752; fax: +90 332 223 6181. E-mail address: [email protected] (S. Demirci). 0379-0738/$ – see front matter ß 2009 Elsevier Ireland Ltd. All rights reserved. doi:10.1016/j.forsciint.2009.04.016

common third ventricular mass in adults, and typically presents in mid-adulthood [7]. Many colloid cysts are asymptomatic and are discovered coincidentally. In symptomatic patients, headache is the most common initial symptom in 75% of patients. Such headaches may be constant, intermittent or migrainous. Other symptoms include disturbed mentation, vomiting, seizures (20%), vertigo, drop attacks and sudden attacks of leg weakness [8]. Sudden death is the most extreme and challenging feature of the disease. The definitive cause of this lethal phenomenon is still a matter of debate, but acute blockage of cerebrospinal fluid (CSF) with instant an herniation or decompensation in the chronic hydrocephalus have been postulated. Another proposed mechanism is the disturbance of hypothalamus-mediated cardiovascular reflex control [9]. It has been estimated that at least one-third of symptomatic patients are at risk for precipitous decline or death [10]. In this paper, three cases are presented of third ventricle colloid cysts that were discovered only at autopsy, but which upon reflection, displayed some of the classical clinical features.

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Fig. 1. Appearance of tuber cinereum. (a) The tuber cinereum is grey and transillumination is present (case 1); (b) normal appearance (for comparison).

2. Case reports 2.1. Case 1 A 24-year-old woman had been treated for migraine headaches for 12 months. The patient presented to the hospital with a severe headache and was sent back home after 3 h of medical treatment. Six hours later, she was found dead in her bed in the early morning. An autopsy revealed no relevant findings in organs besides the brain that would contribute to the death of the patient. The brain weighed 1510 g. Upon macroscopic examination, the brain was markedly swollen with gyral flattening, sulcal narrowing and bilateral uncal grooving. The cerebellar tonsils were grooved. It was observed that the tuber cinereum, which is located between the corpus mamillare and the chiasma opticum, was grey and that transillumination was present in this region (Fig. 1a). Brain sections revealed a cyst inside the third ventricle near the foramen of Monro that measured 2 cm in diameter and contained greenish-

brownish mucoid and gelatinous fluid (Fig. 2). The lateral ventricles were extremely dilated. Both lungs showed intrapulmonary hemorrhage associated with congestion and moderate edema. All the other organ systems were normal and showed no abnormalities. 2.2. Case 2 A 21-year-old woman was treated for migraine headaches for a 6-month period, as in the first case. She complained of dysmenorrhea and irregular menstrual cycles. One day prior to her death, she experienced a severe headache, which started during the evening and continued through the night. This headache was combined with dizziness and three instances of vomiting attacks. She lost consciousness in the early morning and was transported to the hospital, where she was pronounced dead upon arrival. The medical history, taken from her family, showed that she had a similar attack 3 months prior, which had resolved

Fig. 2. Colloid cyst measuring 2 cm in diameter and containing greenish-brownish mucoid and gelatinous fluid inside the third ventricle (case 1).

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greenish-brownish mucoid and gelatinous fluid. The lateral ventricles were extremely dilated. The cerebral tissue in this area had become thin and presented only a 1 cm thickness. Both lungs showed subpleural petechial hemorrhages and moderate edema. Microscopic examination of the cysts in all three cases revealed similar findings; a cyst lined by a single layer of non-ciliated columnar epithelial cells with pale eosinophilic cytoplasm, resting on a fibrocollagenous membrane. These observed epithelial cells were partly vacuolated (Fig. 3). The epithelial lining of the cyst was strongly positive for mucicarmine staining (Fig. 4). 3. Discussion

Fig. 3. Cyst wall, HE, 100.

without any medical treatment. During the external examination of the autopsy, hirsutism on the face, abdomen and both legs was observed. The brain weighed 1425 g and was markedly swollen. Gyral flattening, sulcal narrowing and bilateral uncal grooving were noted. The cerebellar tonsils were grooved. The tuber cinereum was grey and transillumination was present. Dissection of this area revealed a cystic, round lesion inside the third ventricle that measured 2 cm in diameter and contained greenish-brownish mucoid and gelatinous fluid. The lateral ventricles were extremely dilated. Polycysts were also noted in both ovaries. 2.3. Case 3 A 25-year-old man had headaches and vomiting and was diagnosed with and medically treated for sinusitis 3 days before his death. He lost consciousness at 6:00 AM on the day of his death and was taken to hospital, where he was pronounced dead on arrival. At autopsy, the brain weighed 1575 g and was markedly edematous. Sulcal narrowing, gyral flattening and bilateral uncal grooving were observed. The cerebellar tonsils were grooved. The tuber cinereum was grey and transillumination was present. Dissection of this area revealed a cystic lesion inside the third ventricle that measured 2.5 cm  2.5 cm  2 cm and contained

Fig. 4. Mucicarmine staining of the cyst wall, 200.

Colloid cysts are endodermal congenital malformations [11]. Such cysts contain mucoid and gelatinous material, which is positive for both Periodic acid Schiff (PAS) and mucicarmen staining [12,13]. This type of cyst was first described by Wallman in 1858, and Dandy, in 1922, depicted these cysts via ventriculography and pneumoencephalography [14]. They are predominantly located in third ventricle, however, other locations may include the frontal lobe [12], leptomeninges [15], cerebellum [16], brainstem [17], brain convexity [18], the region of the optic chiasma [19] and the fourth ventricle [20]. Clinical signs may appear at any age, but they appear most often during childhood and adolescence. The cysts do not usually become symptomatic until the third or fourth decade of life [11]. These cysts are often spherical and histologically benign [21]. The diameter of colloid cysts varies from a few millimeters to several centimeters, with symptomatic cysts typically being 1–2 cm in diameter [7]. Clinical symptoms are not specific and are attributable to increased intracranial pressure. Similar to previously published reports, all of the cases in this study were in their third decade of life and the cysts were approximately 2 cm in diameter. The clinical signs and symptoms of colloid cysts are nonspecific and may be misinterpreted as migraine headaches, transient ischemic attacks or other brain tumor entities [22,23]. The most common clinical symptom is an episodic headache, which is the result of CSF flow obstruction. The headache often occurs in the early morning or shortly after the patient gets up and is triggered and intensified by changes in the position of the head or body [2,24]. This rare type of headache disorder is significant because it is associated with sudden death. Recognition of the unusual features of colloid cyst headaches may result in a decrease in the rate of mortality associated with this disorder. Notably, headache was the main symptom in all the cases and the subjects in the first and second cases were treated for migraine headaches, whereas the third case was treated for sinusitis. The incidence of sudden death caused by brain tumors ranges from 0.16% to 3.2%, and the majority of cases are due to colloid cysts of the third ventricle [25]. Asymptomatic colloid cysts of the third ventricle develop a symptomatic progression related to the cyst in 0%, 0% and 8% of cases at 2, 5, and 10 years, respectively [26]. In contrast, in another study, symptomatic colloid cysts of the third ventricle had a reported risk of acute deterioration in an estimated 25 (34%) of 74 patients, with a rate of mortality of 9 (12%) of 74 patients [10]. In a different study, sudden death was reported to occur in about 10% of patients with colloid cysts of the third ventricle [11]. In two large series of 17,404 and almost 11,000 postmortem examinations, it was found that primary intracranial neoplasm was the cause of sudden, unexpected death in only 0.16– 0.17% of cases and that in the same two series, a colloid cyst was the cause of sudden unexpected death in only 0.001–0.009% of cases [27]. Although many cysts remain clinically silent, symptoms may result from persistent or intermittent obstruction of the foramina

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of Monro. This can lead to acute lateral ventricular hydrocephalus with manifestations of intracranial hypertension and, rarely, sudden death. The latter may also be secondary to the reflex cardiac effects mediated through the compression of the hypothalamus by the cysts [2,8,11,22,28–31]. Bu¨ttner et al. [11] reported two cases of fatal third ventricle colloid cyst and reviewed the literature. They found 98 cases of sudden death due to a colloid cyst of the third ventricle reported in the literature. The age of the patients ranged from 6 to 79 years (mean 29.6 years) in 40 females and 41 males. The age in 18 of the cases and the gender in 17 of the cases were not given. The cyst size ranged from 0.8 cm  0.8 cm to 7.9 cm  6.5 cm in diameter. A circadian variation in human CSF production has been demonstrated using magnetic resonance phase imaging. The average CSF production showed a clear tendency to circadian variation, with a minimum production at 30% of the maximum values (12  7 ml/h) at approximately 18:00 and a nightly peak production, which is about twice the daytime values, at approximately 02:00 of 42  2 ml/h [32,33]. All of the cases died in the early morning. It is believed that this situation may result from the peak production of CSF at 02:00. None of the patients in this study underwent a computerized tomography or magnetic resonance visualization as part of their evaluation. It is thought that the cause of death was a combination of an instant herniation due to the increased intracranial pressure, originating from the lateral ventricular hydrocephalus and the colloid cyst of the third ventricle, and the reflex cardiac effects mediated through the compression of the hypothalamus by the cysts. Upon autopsy of the cases, macroscopic examination of the brain showed that the tuber cinereum was grey and transillumination was present (Fig. 1). It is thought that this color alteration and transillumination originates from the tensioning and slimming of the tuber cinereum because of the expanding colloid cyst located inside the third ventricle. 4. Conclusion Many colloid cysts are found only at autopsy [11]. The present cases are illustrative examples of sudden death due to colloid cysts of the third ventricle. They highlight the difficulty in the diagnosis of the condition and the importance of recognizing colloid cyst of the third ventricle, which should be included in the differential diagnosis of headaches in young adults. These cases also help to remind forensic pathologists that this entity, although rare, should remain in the differential diagnosis of hydrocephalus at autopsy. In the autopsy of people with sudden death and a medical history of headaches, a colloid cyst should be considered if a significant grey color is detected over the tuber cinereum, which is located between the chiasma opticum and the corpus mamillare, and in such cases, this region should be dissected and examined carefully. References [1] P. Betz, W. Eisenmenger, Traumatic origin of a meningioma? Int. J. Legal Med. 107 (1995) 326–328. [2] M.S. Greenberg, Tumor, in: M.S. Greenberg (Ed.), Handbook of Neurosurgery, Georg Thieme, New York, 2001, pp. 437–439. [3] K. Kuchelmeister, M. Bergmann, Colloid cysts of the third ventricle: an immunochistochemical study, Histopathology 21 (1992) 35–42.

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