CASE REPORT colloid cyst
Colloid Cyst of the Third Ventricle Colloid cyst of the third ventricle is a rare, histologically benign but potentially lethal tumor. TWo cases reflecting the variability it~ symptoms, physical findings, and outcome seen with these rare tumors are presented. One patient with classic age of onset in the third to fifth decade of life, symptoms of escalating intermittent headaches, and signs of papilledema and altered level of consciousness had successful surgical removal of the cyst. Another patient with nonspecific symptoms well outside the usual age of onset and without papilledema succumbed to sudden, complete impaction and was not diagnosed until autopsy [Read EJ Jr: Colloid cyst of the third ventricle. Ann Emerg Med September 1990;19:1060-1062.] INTRODUCTION A colloid cyst is a rare type of cystic t u m o r that occurs in the cavity of the third ventricle and comprises approximately 0.5% of all intracranial tumors.~ The cyst has the gross appearance of a pearly, small sphere attached by a stalklike appendage to the anterosuperior portion of the third ventricle. Classic s y m p t o m s of these tumors are intermittent headache and vomiting and, occasionally, dizziness, transient diplopia, blurred vision, weakness, and paresthesias of extremities. The symptomatology may be exacerbated or alleviated by positional changes. It is theorized that s y m p t o m s are due to an acute hydrocephalus produced by a shift of the tumor, which blocks the outflow of cerebrospinal fluid through the foramina of Monroe. 2 Two patients with colloid cysts of the third ventricle presented to a small community-hospital emergency department within a three-year period - one without and one with classic symptoms.
Edward J Read, Jr, MD State College, Pennsylvania From the Department of Emergency Medicine, Clearfield Hospital, Clearfield, Pennsylvania. Received for publication August 14, 1989. Accepted for publication January 8, 1990. Address for reprints: Edward J Read, Jr, MD, 1378 Deerfield Drive, State College, Pennsylvania 16803-2208.
CASE REPORTS Case One
A 6-year-old girl presented to the ED after her parents witnessed a convulsion at home. Convulsions had not been noted previously in this child. In addition to a history of one to two minutes of unresponsiveness, stiffness, and shaking in all extremities, her parents described two to three days of headache, neck pain, and recurrent vomiting. There was no history of recent or distant-past head trauma. Except for frequent otitis media and insertion of several pairs of t y m p a n o s t o m y tubes, the child's medical history was unremarkable. She was not taking any medications, and there were no k n o w n allergies. Physical examination revealed a pale, well-nourished, appropriately developed child who was very lethargic but able to respond to verbal questioning. Blood pressure was 120/90 m m Hg; pulse, 80; respirations, 26; and temperature, 36.9 C orally. The skin was w i t h o u t rash or petechiae, and her head was atraumatic. Pupils were equal, round, and symmetrically reactive to light. Extraocular muscle m o v e m e n t was intact bilaterally. Funduscopic examination was without papilledema, hemorrhages, or exudates. Tympanic membranes were erythematous, dull, and retracted bilaterally; a t y m p a n o s t o m y tube was noted in the left tympanic membrane. T h e oral m u c o s a w a s m i l d l y dry, and t h e p h a r y n x w a s m i l d l y erythematous. The neck was without meningismus, but discomfort was noted on flexion. Kernig's and Brudzinski's signs were negative. Lungs
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Annals of Emergency Medicine
COLLOID CYST Read
were clear bilaterally, and heart sounds were regular and w i t h o u t murmur. The abdomen was soft and nontender w i t h o u t mass or organomegaly. Neurologic examination revealed no focal findings. Blood and urine specimens were collected. Lumbar puncture was performed atraumatically, yielding a slow drip of clear, colorless fluid. At the conclusion of the lumbar puncture, the patient had a 30-second generalized tonic-clonic seizure. Repeat funduscopic examination was norreal, and neurologic examination was without focal findings. The child was postictal for five to ten minutes and then returned to her presenting level of consciousness. An IV catheter was inserted into a peripheral vein infusing 5% dextrose in water at keepopen rate, and the patient was admitted to the hospital under the care of her primary-care physician. Laboratory results revealed hemoglobin of 13.8 g/dL; h e m a t o c r i t , 41.2%; WBC, 18,200 with 95% segments, 3% l y m p h o c y t e s , and 2% monocytes; and elevated erythrocyte sedimentation rate, 27 mm/hr (Wintrobe, normal, 0 to 15 mm/hr). Sodium was 144 mmol/L; potassium, 3.3 retool/L; chloride, 102 mmol/L; COx, 24 mmol/L; glucose, 163 rag/ dL; and BUN, 24 mg/dL. The serum ammonia level was 16 ~,mol/L (normal, 11 to 35 t~mol/L), and the AST was 22 IU/L (normal, 10 to 42 IU/L). The cerebrospinal fluid revealed 4 mL of crystal-clear fluid in three tubes - RBC, 15; WBC, 0; glucose, 97 mg/dL; total protein, 20 mg/dL; and Gram-stain, negative. Approximately 90 minutes after presentation, the child had a brief episode of decreased level of consciousness without motor activity. Her primary-care physician instituted empirical antimicrobial therapy with a m p i c i t l i n and c h l o r a m p h e n i c o l , pending culture results. At two hours, 15 minutes after presentation, the child experienced sudden complete respiratory arrest, hnmediate successful airway management was performed. Repeat examination at that time revealed total unresponsiveness, complete flaccidity, and divergent eyes with fixed, dilated pupils but no papilledema. EEG by telephone showed very poor anterior activity and slow waves posteriorly. The child was given 10 mg/ kg IV phenytoin and transferred to a 160/1061
tertiary-care facility with a presumed diagnosis of brain stem herniation. On arrival, emergency computed t o m o g r a p h y of the head revealed massively dilated lateral ventricles with a small third ventricle and absent fourth ventricle, cerebral edema, and no tumor or abscess. Repeat neurologic examination revealed total unresponsiveness; negative deep tendon, gag, corneal, and cold caloric reflexes; complete flaccidity; fixed, dilated pupils; and no papilledema. Noninvasive treatment for increased intracranial pressure was continued. Cerebral perfusion scan was negative for cerebral blood flow. Physical and neurologic examinations per protocol for brain death were performed, and the child was declared brain dead approximately 24 hours after presentation. Multiple organ harvest and postm o r t e m e x a m i n a t i o n of the brain was performed. At autopsy, there was no o b v i o u s evidence of t o n s i l l a r grooving consistent with herniation syndrome. A colloid cyst measuring 2.0 x 1.8 x 1.2 cm was found in the third ventricle, obstructing the foramina of Monroe. The cause of death was sudden enlargement of the third ventricle secondary to impaction of the colloid cyst. Of note, litigation was filed against the emergency physician for performing a lumbar puncture. At trial, a jury found in favor of the physician. Case T w o A 25-year-old woman presented to the ED complaining of intermittent h e a d a c h e and neck pain for one month. She admitted to being treated by a chiropractor for the previous three to four weeks without relief. The headaches were described as occipital, radiating upward, and escalating in severity. The head pain was at t i m e s a s s o c i a t e d w i t h dizziness, blurred vision, n u m b n e s s of the hands, and heaviness in the feet. Her medical history was negative for migraine, hypertension, or any chronic illnesses. She was not currently taking any medications, and there were no known allergies. Physical examination revealed a women in moderate distress secondary to cephalgia. Blood pressure was 144/88 m m Hg; pulse, 116; respirations, 20; and temperature, 37.3 C orally. Pupils were equal and reactive to light. Extraocular muscle moveAnnals of Emergency Medicine
m e n t was intact bilaterally. Funduscopic examination showed bilateral indistinct ocular disk margins with flame hemorrhages temporally. Visual acuity was 20/20 in the left and 20/25 in the right eye. There was no meningismus. Neurologic examination revealed no focal findings. The remainder of the physical examination was normal. N e u r o s u r g i c a l c o n s u l t a t i o n by telephone resulted in urgent transfer of the patient for definitive evaluation and treatment. After uneventful transport, computed tomography of the head revealed hydrocephalus in the lateral ventricles and a mass that appeared to be at the foramina of Monroe. Subsequently performed arteriography confirmed these findings; approximately 40 hours after presenting to the ED, the patient underwent successful surgical removal of the colloid cyst.
DISCUSSION Colloid cyst of the third ventricle was first documented in the medical literature in 1858 by Wallmann. 3 In 1933, Dandy 4 reported that these tumors could be diagnosed, localized, and successfully removed by surgery. The age range of cases reported is from 2 m o n t h s 5 to 79 years. 6 The two sexes are equally represented. The age at onset of symptoms is usually within the third to fifth decade. The m o s t c o n s i s t e n t presenting symptom is headache. The clinical presentation of these tumors may at times be suggestive of the underlying pathophysiology of intermittent ball valve obstruction leading to episodes of increased intracranial pressure. Direct pressure on the wall of the third ventricle also may produce pyramidal tract symptomatology. However, s y m p t o m s p r o d u c e d are so nonspecific and of such a wide spectrum that they are also consistent with lesions located elsewhere in the brain as well as with any process that leads to increased intracranial pressure. The most consistent physical findings are papilledema and an altered state of consciousness. Nevertheless, physical findings, like symptoms, are nonspecific. The findings on examination may be consistent with the degree and duration of increased intracranial pressure secondary to partial or complete obstruction at the foramina of Monroe. Our two cases reflect the vari19:9 September 1990
a b i l i t y i n s y m p t o m s , physical findings, and o u t c o m e seen w i t h these rare tumors. Case 1, with nonspecific s y m p t o m s well outside the usual age of onset, w i t h o u t papilledema, and w i t h apparent sudden c o m p l e t e impaction, was n o t diagnosed u n t i l autopsy. Case 2, classic for age at onset, intermittent escalating headache, and papilledema, resulted in successful surgical removal of the cyst.
SUMMARY Colloid cyst of the third ventricle is a rare, h i s t o l o g i c a l l y b e n i g n b u t potentially lethal tumor. It most oft e n causes s y m p t o m s in the third to fifth decade of life. T h e s y m p t o m s are varied b u t consistent with their
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u n d e r l y i n g source, i n c r e a s e d i n t r a cranial pressure. Two patients presented to a comm u n i t y hospital ED over a three-year period. The first, who presented w i t h nonspecific s y m p t o m s well outside the usual age of onset w i t h o u t papilledema, s u c c u m b e d to sudden complete i m p a c t i o n , and was n o t diagn o s e d u n t i l a u t o p s y . T h e second, with classic age of onset, s y m p t o m s of escalating i n t e r m i t t e n t headaches, and signs of papilledema and altered level of consciousness, had successful surgical removal of the cyst. O n c e suspected, e v a l u a t i o n i n a medical center with neurosurgical capabilities is urgent because sudden c o m p l e t e i m p a c t i o n of the cyst i n
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the third ventricle may lead to irreversible brain injury or death w i t h i n minutes.
REFERENCES 1. FerryDJ, KempeLG: Colloidcyst of the third ventricle. Milit Med 1968;33:734-737. 2. Merritt HH: A Textbook of Neurology, ed 6. Philadelphia, Lea & Febiger, 1979, p 279-281. 3. Wallmann H: Eine colloidcyste im dritten Hirnventrikel und ein Lipom im Plexus Choriodes. Virchows Arch Path Anat 1858~14:385. 4. DandyWE: Benign Tumors of the Third Ventricle of the Brain: Diagnosis and Treatment.
Springfield, Illinois, Charles C Thomas, 1933. 5. GemperleinJ: Paraphyseal cysts of the third ventricle: Report of two cases in infants.J Neuropath Exp Neurol 1960;19:133-134. 6. Bamitzky S, Sarwar M, Leeds N, et al: Colloid cysts of the third ventricle. Radiology 1974;112:327-341.