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Colloid cyst of the third ventricle and sudden death
CASE REPORT colloid cyst; headache
Colloid Cyst of the Third Ventricle and Sudden Death Patients with colloid cysts of the third ventricle m a y complain only of severe headache. The threat of sudden death makes these patients a special diagnostic problem to the emergency physician. There may be only a few intermittent and nonspecific signs and symptoms associated with this tumor. A discussion of two such cases and an abstract of similar cases drawn from the literature is included in this report. [McDonald JA: Colloid cyst of the third ventricle and sudden death. Ann Emerg Med 11:365-367, July 1982.]
INTRODUCTION Colloid cysts, also known as ependymomas or neuroepithelial cysts, occur in the third ventricle of the brain. From a clinical standpoint, the antemortem diagnosis is di_fficult because only a few nonspecffic, intermittent signs and s y m p t o m s signal this tumor. The diagnosis often is made at post mortem. Two cases seen at Tripler Army Medical Center, Hawaii, during the past two years posed just such a diagnostic problem. They are presented with a review of similar cases to alert the emergency physician to the clinical findings and ancillary studies helpful in confirming the diagnosis.
John A. McDonald, MD Honolulu, Hawaii From the Department of Medicine; Tripler Army Medical Center, Honolulu, Hawaii. The assertions or opinions contained herein are the private views of the author and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Address for reprints: John A. McDonald, MD, 1812 Kumakani Place, Honolulu, Hawaii 96821.
CASE REPORTS Case Number One The patient was a 6-year-old white girl who first came to the Pediatric Clinic at Schofield Barracks, Hawaii, with a history of five episodes of emesis during the previous 24 hours. Gastroenteritis was diagnosed, and the patient was discharged on symptomatic treatment of clear liquids and anti-emetic medications. Later that day, the patient suddenly became stiff, unconscious, and then "limp." She was returned to the Schofield Barracks emergency department where her right pupil was noted to be fixed and dilated. She was transferred to the Tripler A r m y Medical Center emergency department where she was unresponsive to all stimuli except deep pain. Bilateral papilledema and retinal hemorrhages were present. Complete blood count, electrolytes, blood urea nitrogen, glucose, skull and chest films, and urinalysis were all within normal limits. She briefly regained consciousness and was oriented, but suddenly experienced two seizures, each lasting 5 to 10 minutes, followed by respiratory arrest. Following resuscitation, which included endotracheal intubation, she was placed on controlled ventilation. Pupils were fixed and dilated biIaterally, and she was unresponsive to painful stimuli. Cerebral angiography demOnstrated no arterial flow at the base of the skull and no peffusion of the brain. Two flat-line electroencephalograms were obtained. Mechanical ventilation was discontinued on the second hospital day and the patient died. Autopsy examination revealed a 1.2 cm, mucous-filled cyst attached to the most rostral portion of the third ventricle, which obstructed spinal egress and caused internal hydrocephalus. The increased intracranial pressure caused brainstem transtentorial hemiation and death. The patient's mother had had a neuroepithelial cyst removed three years prior to this patient's death. 11:7 July 1982
Annals of Emergency Medicine
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COLLOID CYST McDonald
Fig. Autopsy photograph of colloid cyst in tPdrd ventricle in Case Number T~vo. Case Number Two A 21-year-old active duty Navy man presented with a 4-day history of "the worst headache of my life." He had had "minor" antecedent head trauma the day before onset of the headache. The headache diminished while he was supine. Physical examination, including a neurological examination, was normal. There was no papilledema. A lumbar puncture was performed in the outpatient clinic. The cerebrospinal fluid was clear with an opening pressure of 430 m m of CSF; however, after the patient's legs were extended, cerebrospinal fluid pressure dropped to 170 mm. The cerebrospinal fluid examination revealed: protein, 23 mg %; glucose, 64 mg %; and was negative for cells and bacteria. The patient was observed for several hours after the puncture and was discharged on acetaminophen 2 tabs q4h. He returned several hours later in the evening, describing the headache as more severe. The diagnosis of "post-lumbar puncture headache" was made. There was no evidence of papilledema. A brain scan was scheduled for the following morning. He was found dead in his barracks early that m o r n i n g . H i s t o r y was obtained from friends in the barracks that earlier in the evening prior to the patient's death, he was "wandering about aimlessly, bumping into the walls." Autopsy revealed a colloid cyst of the third ventricle completely blocking the right foramen of Monro and partially blocking the left foramen of Monro (Figure). Associated findings were internal hydrocephalus and cerebral edema. DISCUSSION Although colloid cysts are common benign tumors encountered throughout the central nervous system, the diagnosis is rarely made during life.1 The incidence at autopsy is 2% of all intracranial tumors, but only 0.5% are clinically evident. 1 Microscopically, the content is a homogeneous material surrounded by a layer of cuboidal and columnar ciliated cells and covered by a layer of connective tissue. 2 They are frequently found in the choroid plexus of the lateral ventricles where they do not produce 38/366
symptoms. 3 Shuangshati and Netsky ~ reviewed 124 routine autopsy specimens of lateral ventricular choroid plexus taken from individuals ranging in age from prenatal to 90 years. Neuroepithelial cysts were found in 66% of choroid plexus specimens. Cysts arising from the roof of the third ventricle are less frequent, but may give rise to symptoms and signs associated with elevated intracranial pressure. 3 A characteristic presentation is the patient who experiences sudden onset of severe headache possibly aggravated or relieved by a m o v e m e n t of the head, which may be due to a ballvalve action of the tumor that intermittently obstructs the free flow of cerebrospinal fluid through the foramen of Monro. 3 Kelly4 analyzed 29 patients and concluded that episodic and/or positional headaches were not peculiar to intraventricular tumors. However, such symptoms occurring in patients presenting with papilledema and the absence of localizing signs were likely the result of a cyst in the third ventricle. Little and MacCarty, s in a review of 38 cases, found that the "classic hist o r y " of a paroxysmal, positional headache was often not seen. The headaches were similar to those comm o n l y present with increased intracranial pressure. Many of the patients in this series presented with a long history of headache, suggesting a Annals of Emergency Medicine
benign process. Drennan 6 described two patients under 26 years of age who died suddenly. One had a history of intermittent severe headache for two years. Both had an impacted colloid cyst in the third ventricle with resultant internal hydrocephalus affecting the lateral ventricles. A similar patient z had a one-year history of severe intermittent headache associated with nausea, vomiting, and vertigo. Each episode lasted approximately 12 hours, with relief of symptoms obtained by reclining with her head t h r o w n back. A symptom-free period of 9 years was followed by a sudden severe headache, projectile vomiting, and death in a few hours. Postmortem examination confirmed a cystic tumor impacted in the foramen of Monro and completely occluding the third ventricle. Malek and Green 8 reported a patient with sporadic abducens nerve paralysis in conjunction with transient decerebrate rigidity, occurring spontaneously as well as when the patient altered his head posture voluntarily. He was also noted to have bilateral papilledema and constricted visual fields. Eventual craniotomy revealed a large colloid cyst of the third ventricle. The tragedy of third ventricular colloid cyst is the physician's failure to diagnose a potentially curable tumor because of his lack of familiarity with the presentation. 11:7 July 1982
In our first case, headache was not p r o m i n e n t ; rather, elevated cerebrospinal fluid pressure was manifest by vomiting followed by coma. The only hint of the cyst was the brief interval of consciousness w h i c h would be unusual for malignant brain tumor, encephalitis, Reye's syndrome, or other c a u s e s of p a p i l l e d e m a in a c h i l d . Suspicion based on the sudden onset of severe u n e x p e c t e d headache augm e n t e d by a n y n e u r o l o g i c a l deficit calls for emergency CAT scan. T h e second patient had the charact e r i s t i c severe h e a d a c h e w h i c h was nonthrobbing and constant. The elevated cerebrospinal fluid pressure noted initially might have raised suspicions. The initial high CSF pressure of 430 m m of CSF was regarded as spurious, b u t in retrospect was the only clue.
11:7 July 1982
CONCLUSION A c o m m o n thread in the history of patients presenting w i t h colloid cyst is severe headache, paroxysmal and/or p o s i t i o n a l in nature, in the absence of fever or localizing signs. This, together w i t h t r a n s i e n t neurological signs of increased cerebrospinal pressure, should lead to the definitive diagnostic procedure, computerized axial tomography.9, lo
REFERENCES 1. Shuangshati S, Netsky MG: Neuroepithelial (colloid) cysts of the nervous system. Neurology 12:887-903, 1962. 2. Baker AB: Clinical Neurology, ed 2. New York, Hoeber & Harper, 1962, p 554. 3. Vick N: Grinker's Neurology. Springfield, Thomas, 1976, p 403, 433. 4. Kelly R: Colloid cysts of the third ventricle. Analysis of twenty-nine cases. Brain
Annals of Emergency Medicine
74:23-63, 1951. 5. Little" JR, MacCarry C: Colloid cysts of the third ventricle. J Neurosurg 39:230-235, 1974. 6. Drennan AM: Impacted cyst in third ventricle of brain - - report of two cases. Br Med J 2:47, 1929. 7. Rinder CD, Cannon PR: Impaction of neuroepithelial cyst in the third ventricle. Arch Neurol Psychiatr 30:880-883, 1933. 8. Malek JL, Green D: Episodic abducens paralysis during transient decerehrate rigidity associated with a colloid cyst of the third ventricle. Neurology 13:538-540, 1968. 9. Leicht MJ: Nontraumatic headache in the emergency department. Ann Emerg Med 9:404-408, 1980. 10. Ganti SR, Antunes JL, Louis KM, et al: Computed tomography in the diagnosis of the third ventricle. Radiology 138:385-391, 1981.
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Colloid Cyst of the Third Ventricle and Sudden Death Patients with colloid cysts of the third ventricle m a y complain only of severe headache. The threat of sudden death makes these patients a special diagnostic problem to the emergency physician. There may be only a few intermittent and nonspecific signs and symptoms associated with this tumor. A discussion of two such cases and an abstract of similar cases drawn from the literature is included in this report. [McDonald JA: Colloid cyst of the third ventricle and sudden death. Ann Emerg Med 11:365-367, July 1982.]
INTRODUCTION Colloid cysts, also known as ependymomas or neuroepithelial cysts, occur in the third ventricle of the brain. From a clinical standpoint, the antemortem diagnosis is di_fficult because only a few nonspecffic, intermittent signs and s y m p t o m s signal this tumor. The diagnosis often is made at post mortem. Two cases seen at Tripler Army Medical Center, Hawaii, during the past two years posed just such a diagnostic problem. They are presented with a review of similar cases to alert the emergency physician to the clinical findings and ancillary studies helpful in confirming the diagnosis.
John A. McDonald, MD Honolulu, Hawaii From the Department of Medicine; Tripler Army Medical Center, Honolulu, Hawaii. The assertions or opinions contained herein are the private views of the author and are not to be construed as official or as reflecting the views of the Department of the Army or the Department of Defense. Address for reprints: John A. McDonald, MD, 1812 Kumakani Place, Honolulu, Hawaii 96821.
CASE REPORTS Case Number One The patient was a 6-year-old white girl who first came to the Pediatric Clinic at Schofield Barracks, Hawaii, with a history of five episodes of emesis during the previous 24 hours. Gastroenteritis was diagnosed, and the patient was discharged on symptomatic treatment of clear liquids and anti-emetic medications. Later that day, the patient suddenly became stiff, unconscious, and then "limp." She was returned to the Schofield Barracks emergency department where her right pupil was noted to be fixed and dilated. She was transferred to the Tripler A r m y Medical Center emergency department where she was unresponsive to all stimuli except deep pain. Bilateral papilledema and retinal hemorrhages were present. Complete blood count, electrolytes, blood urea nitrogen, glucose, skull and chest films, and urinalysis were all within normal limits. She briefly regained consciousness and was oriented, but suddenly experienced two seizures, each lasting 5 to 10 minutes, followed by respiratory arrest. Following resuscitation, which included endotracheal intubation, she was placed on controlled ventilation. Pupils were fixed and dilated biIaterally, and she was unresponsive to painful stimuli. Cerebral angiography demOnstrated no arterial flow at the base of the skull and no peffusion of the brain. Two flat-line electroencephalograms were obtained. Mechanical ventilation was discontinued on the second hospital day and the patient died. Autopsy examination revealed a 1.2 cm, mucous-filled cyst attached to the most rostral portion of the third ventricle, which obstructed spinal egress and caused internal hydrocephalus. The increased intracranial pressure caused brainstem transtentorial hemiation and death. The patient's mother had had a neuroepithelial cyst removed three years prior to this patient's death. 11:7 July 1982
Annals of Emergency Medicine
365/37
COLLOID CYST McDonald
Fig. Autopsy photograph of colloid cyst in tPdrd ventricle in Case Number T~vo. Case Number Two A 21-year-old active duty Navy man presented with a 4-day history of "the worst headache of my life." He had had "minor" antecedent head trauma the day before onset of the headache. The headache diminished while he was supine. Physical examination, including a neurological examination, was normal. There was no papilledema. A lumbar puncture was performed in the outpatient clinic. The cerebrospinal fluid was clear with an opening pressure of 430 m m of CSF; however, after the patient's legs were extended, cerebrospinal fluid pressure dropped to 170 mm. The cerebrospinal fluid examination revealed: protein, 23 mg %; glucose, 64 mg %; and was negative for cells and bacteria. The patient was observed for several hours after the puncture and was discharged on acetaminophen 2 tabs q4h. He returned several hours later in the evening, describing the headache as more severe. The diagnosis of "post-lumbar puncture headache" was made. There was no evidence of papilledema. A brain scan was scheduled for the following morning. He was found dead in his barracks early that m o r n i n g . H i s t o r y was obtained from friends in the barracks that earlier in the evening prior to the patient's death, he was "wandering about aimlessly, bumping into the walls." Autopsy revealed a colloid cyst of the third ventricle completely blocking the right foramen of Monro and partially blocking the left foramen of Monro (Figure). Associated findings were internal hydrocephalus and cerebral edema. DISCUSSION Although colloid cysts are common benign tumors encountered throughout the central nervous system, the diagnosis is rarely made during life.1 The incidence at autopsy is 2% of all intracranial tumors, but only 0.5% are clinically evident. 1 Microscopically, the content is a homogeneous material surrounded by a layer of cuboidal and columnar ciliated cells and covered by a layer of connective tissue. 2 They are frequently found in the choroid plexus of the lateral ventricles where they do not produce 38/366
symptoms. 3 Shuangshati and Netsky ~ reviewed 124 routine autopsy specimens of lateral ventricular choroid plexus taken from individuals ranging in age from prenatal to 90 years. Neuroepithelial cysts were found in 66% of choroid plexus specimens. Cysts arising from the roof of the third ventricle are less frequent, but may give rise to symptoms and signs associated with elevated intracranial pressure. 3 A characteristic presentation is the patient who experiences sudden onset of severe headache possibly aggravated or relieved by a m o v e m e n t of the head, which may be due to a ballvalve action of the tumor that intermittently obstructs the free flow of cerebrospinal fluid through the foramen of Monro. 3 Kelly4 analyzed 29 patients and concluded that episodic and/or positional headaches were not peculiar to intraventricular tumors. However, such symptoms occurring in patients presenting with papilledema and the absence of localizing signs were likely the result of a cyst in the third ventricle. Little and MacCarty, s in a review of 38 cases, found that the "classic hist o r y " of a paroxysmal, positional headache was often not seen. The headaches were similar to those comm o n l y present with increased intracranial pressure. Many of the patients in this series presented with a long history of headache, suggesting a Annals of Emergency Medicine
benign process. Drennan 6 described two patients under 26 years of age who died suddenly. One had a history of intermittent severe headache for two years. Both had an impacted colloid cyst in the third ventricle with resultant internal hydrocephalus affecting the lateral ventricles. A similar patient z had a one-year history of severe intermittent headache associated with nausea, vomiting, and vertigo. Each episode lasted approximately 12 hours, with relief of symptoms obtained by reclining with her head t h r o w n back. A symptom-free period of 9 years was followed by a sudden severe headache, projectile vomiting, and death in a few hours. Postmortem examination confirmed a cystic tumor impacted in the foramen of Monro and completely occluding the third ventricle. Malek and Green 8 reported a patient with sporadic abducens nerve paralysis in conjunction with transient decerebrate rigidity, occurring spontaneously as well as when the patient altered his head posture voluntarily. He was also noted to have bilateral papilledema and constricted visual fields. Eventual craniotomy revealed a large colloid cyst of the third ventricle. The tragedy of third ventricular colloid cyst is the physician's failure to diagnose a potentially curable tumor because of his lack of familiarity with the presentation. 11:7 July 1982
In our first case, headache was not p r o m i n e n t ; rather, elevated cerebrospinal fluid pressure was manifest by vomiting followed by coma. The only hint of the cyst was the brief interval of consciousness w h i c h would be unusual for malignant brain tumor, encephalitis, Reye's syndrome, or other c a u s e s of p a p i l l e d e m a in a c h i l d . Suspicion based on the sudden onset of severe u n e x p e c t e d headache augm e n t e d by a n y n e u r o l o g i c a l deficit calls for emergency CAT scan. T h e second patient had the charact e r i s t i c severe h e a d a c h e w h i c h was nonthrobbing and constant. The elevated cerebrospinal fluid pressure noted initially might have raised suspicions. The initial high CSF pressure of 430 m m of CSF was regarded as spurious, b u t in retrospect was the only clue.
11:7 July 1982
CONCLUSION A c o m m o n thread in the history of patients presenting w i t h colloid cyst is severe headache, paroxysmal and/or p o s i t i o n a l in nature, in the absence of fever or localizing signs. This, together w i t h t r a n s i e n t neurological signs of increased cerebrospinal pressure, should lead to the definitive diagnostic procedure, computerized axial tomography.9, lo
REFERENCES 1. Shuangshati S, Netsky MG: Neuroepithelial (colloid) cysts of the nervous system. Neurology 12:887-903, 1962. 2. Baker AB: Clinical Neurology, ed 2. New York, Hoeber & Harper, 1962, p 554. 3. Vick N: Grinker's Neurology. Springfield, Thomas, 1976, p 403, 433. 4. Kelly R: Colloid cysts of the third ventricle. Analysis of twenty-nine cases. Brain
Annals of Emergency Medicine
74:23-63, 1951. 5. Little" JR, MacCarry C: Colloid cysts of the third ventricle. J Neurosurg 39:230-235, 1974. 6. Drennan AM: Impacted cyst in third ventricle of brain - - report of two cases. Br Med J 2:47, 1929. 7. Rinder CD, Cannon PR: Impaction of neuroepithelial cyst in the third ventricle. Arch Neurol Psychiatr 30:880-883, 1933. 8. Malek JL, Green D: Episodic abducens paralysis during transient decerehrate rigidity associated with a colloid cyst of the third ventricle. Neurology 13:538-540, 1968. 9. Leicht MJ: Nontraumatic headache in the emergency department. Ann Emerg Med 9:404-408, 1980. 10. Ganti SR, Antunes JL, Louis KM, et al: Computed tomography in the diagnosis of the third ventricle. Radiology 138:385-391, 1981.
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