Anterior Cervical Arachnoid
Cyst
Simulating Syringomyelia A Case With Joel
Preceding
Posterior Arachnoid
Herskowitz, MD; Martin A. Bielawski, MD; Nagagopal Venna, MB, MRCP; Thomas D. Sabin,
\s=b\ An arachnoid
cyst lying anterior to the
cervical cord at level C6-7 was found in a 28-year-old woman believed to have syringomyelia. This diagnosis was based both on previous findings at laminectomy and on computerized tomography. The diagnosis of arachnoid cyst was suspected because of clinical features atypical for classical syringomyelia and a history of arachnoid cysts found during childhood. Air myelography demonstrated an extramedullary intradural mass anteriorly that proved to be an arachnoid cyst. Drainage and subtotal resection resulted in marked clinical improvement. This case illustrates the need for reevaluation when a patient with "known" syringomyelia presents an atypical clinical picture. Anterior cervical arachnoid cyst, which may accompany or succeed posterior arachnoid cysts, should be considered. (Arch Neurol 35:57-58, 1978)
Syringomyelia
characteristically has the presenting clinical picture of dissociated sensory loss in a cape-like distribution, wasting of intrinsic hand muscles, and loss of
upper limb reflexes.1 Atypical cases have been ascribed to unusual location of the intramedullary cyst or sufficient ex¬ pansion of the lesion to cause cord
compression. Extramedullary lesions can produce complicated syndromes that may be mistaken for syringomyelia. We re¬
that demonstrates the diagnostic difficulties of anterior cer¬ vical arachnoid cyst can present despite direct visualization of the cord during laminectomy and the use of computerized tomography. The clin¬ ical examination and history com¬ bined with standard neuroradiologic procedures established the diagnosis
port
Cysts
a
case
Accepted for publication July 18, 1977. From the Neurological Unit, Boston City Hospital, Boston. Reprint requests to the Neurological Unit, Boston City Hospital, 818 Harrison Ave, Boston, MA 02118 (Dr Sabin).
and led to treatment that otherwise would not have been undertaken. REPORT OF A CASE 1958 Hospitalization
9-year-old girl was admitted to Boston City Hospital with a two-week history of pain in her mid-to-upper back. Three years previously, she had been hospitalized because of posterior neck pain after a ß-hemolytic streptococcal pharyngitis treated with intramuscular penicillin. She A
found to have mild weakness of the right arm and leg that gradually resolved. On admission in 1958, she had weakness of the left arm and leg, absent left triceps jerk, bilateral extensor plantar responses, and diminished sensation to pinprick and light touch below T-4. A myelogram demonstrated a complete block at C-7 to T-l. At operation, three small posterior arachnoid cysts were found just above that level. Histologie examination showed a cyst lined by columnar and cuboidal epithelium without teratomatous elements. The senso¬ ry deficit resolved and motor function (
MD
complaints as in 1971. Generalized hyperreflexia was again noted, with a sensory deficit to pinprick in a T-2 distribution. Computerized tomography of the head
showed ventricles of normal size. A neck scan did not disclose an intraspinal cyst. At operation, a widened cord was again noted, and the presumed syrinx was drained of clear, colorless fluid. The catheter, which was patent but not draining spinal fluid, was replaced. The patient was discharged with improved motor function.
was
improved. 1971
Hospitalization
Following
several admissions during adolescence for symptomatic treatment of pain in neck and arm, the patient was admitted to another hospital because of neck pain and gait disability worsening over a six-month period. Examination showed weakness of all extremities, hyperreflexia, and extensor plantar responses. Pinprick sensation was reduced below the midcervical level, with sacral sparing. Vibratory sensation was mildly impaired in the lower extremities. Position sense was intact. Myelogram now showed ob¬ struction at C5-6. At operation, the cord was described as "ballooned posteriorly." Aspiration through the midline yielded 5 ml of clear, slightly viscous fluid, with subsequent collapse of the cord. A catheter was inserted into the presumed syrinx for drainage. At discharge, the patient could ambulate with a walker and regained nearly normal arm strength.
1974
Hospitalization
The patient, now 25 years old, was admitted to a third hospital with the same
Downloaded From: http://archneur.jamanetwork.com/ by a University of Pennsylvania User on 05/14/2015
1975
Hospitalization
patient was admitted to the same hospital with neck pain and was found to have a spastic quadriparesis greater on the right, with hypalgesia in a C7-8 distribu¬ tion. Myelography now showed a nearly complete block at C-7 to T-l. A computer¬ ized tomographic scan of the neck demon¬ strated a large cyst at C-6 occupying nearly the entire spinal canal interpreted as a probable syrinx. She did not have another operation and experienced mild improve¬ ment. Several weeks after discharge, she The
fell and struck her head without loss of consciousness, after which her neck pain disappeared and walking improved.
Current Hospitalization The
patient was admitted to the Neuro¬ logical Unit of Boston City Hospital on Dec 18, 1976, with a three-week history of Fig
1.—Routine myelogram block at C-7 level.
demonstrating
exquisite posterior
pain radiating down both arms and progressive weakness of the right lower extremity, rendering her neck
unable to walk without assistance. Exami¬ nation showed spastic quadriparesis with wasting and weakness of intrinsic hand
muscles, generalized hyperreflexia, except for absent triceps jerks, and extensor plantar responses. Pinprick and light touch
perception was
Fig 2.—Air myelography in lateral view showing posterior displacement of cord with cyst lying anteriorly. Arrow indicates inferior margin of cyst. Fig 3.—Pantopaque myelogram through C1-2 puncture defining upper border of cyst at C5-6.
diminished in a C-8 to T-l distribution, and vibration and position senses decreased in both legs, right greater than left. Sensation of pinprick, light touch, and heat was reduced anteriorly in a T2-5 distribution. Pantopaque myelogra¬ phy in the prone position showed a complete block at C-7 (Fig 1). An extramedullary intradural mass at C-7, compress¬ ing and displacing the cord posteriorly, was demonstrated by air myelography (Fig 2). The upper border of the cyst, C5-6, was further delineated by contrast myelog¬ raphy through a Cl-2 puncture (Fig 3). Surgical exploration with a right anterolateral approach on Jan 18,1977, disclosed a 2-cm multiloculated arachnoid cyst con¬ taining 5 ml of clear, colorless fluid. The cyst was adherent to the ventral cord so the procedure was limited to drainage and subtotal resection. Microscopic examina¬ tion showed fibrous tissue only. Postoperatively, the patient suffered a meningitic syndrome, with a negative CSF culture. She had a mild right Horner's syndrome that gradually improved. At discharge on Feb 19, 1977, she was free of neck pain and could walk up stairs unassisted. Strength of hands and sensation in the lower legs were also improved. Further improvement of both motor and sensory functions was found three months later.
COMMENT
need not have the dissociated sensory loss with its characteristic distribu¬ tion. Nevertheless, our patient's sen¬ sory deficit was sufficiently unusual, her neck pain so exquisite and local¬ ized, and her upper limb reflexes sufficiently spared that the diagnosis of syringomyelia was questioned, de¬ spite the findings at two operations apparently confirming that diagnosis. The childhood history suggested the possibility of arachnoid cyst and that aspiration of an anterior cyst through a thinned cord could alternatively account for the surgical findings. Adams2 has previously called atten¬ tion to the difficulty in diagnosis based on widening of the spinal cord noted at laminectomy. In Palmer's case,3 the anterior arachnoid cyst was recognized only at necropsy. Air
Syringomyelia classical sign of
Downloaded From: http://archneur.jamanetwork.com/ by a University of Pennsylvania User on 05/14/2015
myelography was most useful in our patient in outlining the anterior extramedullary cyst. None of the myélographie features of syringomye¬ lia described by Ellertsson4 were seen.
Though anterior arachnoid cyst was the preoperative diagnosis, neurent-
either anterior or posterior to the cord and can produce an intermittent course such as seen in our patient.5 This diagnosis was less likely because vertebral anomalies were not present, and no teratomatous or gastric elements had been noted in previous biopsy tissue.6 The etiology of such arachnoid cysts is unknown. The initial neurologic symptoms followed an upper respira¬ tory infection that had required medi¬ cal attention in both Palmer's case and the present patient. No histological, radiological, or surgical evidence for arachnoiditis could be found in either case. The patient reported by Hoffmann7 showed resistance to neck motion since birth and impaired motor function since earliest child¬ hood, which suggested a congenital basis for the lesion. The persistence and progression in our patient of presenting symptoms and signs into adulthood despite removal of the posterior cysts indicate that the anterior cyst may have been present since childhood. Earlier recog¬ nition of such a lesion might have spared the patient a significant amount of pain and disability and might have obviated the need for several operations. eric cysts
can occur
References JB, Hudgson P: The clinical features of communicating syringomyelia, in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia. London, WB Saunders Co, 1973, pp 16-29. 1. Foster
2. Adams RD: Case records of the Massachusetts General Hospital. N Engl J Med 293:33-38, 1975. 3. Palmer JJ: Cervical intradural arachnoid cyst in a 3-year-old child: Report of a case. Arch Neurol 31:214-215, 1974. 4. Ellertsson AB: Semiologic diagnosis of syringomyelia related to roentgenologic findings. Acta Neurol Scand 45:385-402, 1969. 5. Adams RD, Wegner W: Congenital cyst of the spinal meninges as cause of intermittent compression of the spinal cord. Arch Neurol Psychiatry 58:57-69, 1947. 6. Fallon M, Gordon ARG, Lendrum AC: Mediastinal cysts of foregut origin associated with vertebral abnormalities. Br J Surg 41:520\x=req-\ 533, 1954. 7. Hoffmann GT: Cervical arachnoidal cyst: Report of a 6-year-old Negro male with recovery from quadriplegia. J Neurosurg 17:327-330, 1960.
Cyst
Simulating Syringomyelia A Case With Joel
Preceding
Posterior Arachnoid
Herskowitz, MD; Martin A. Bielawski, MD; Nagagopal Venna, MB, MRCP; Thomas D. Sabin,
\s=b\ An arachnoid
cyst lying anterior to the
cervical cord at level C6-7 was found in a 28-year-old woman believed to have syringomyelia. This diagnosis was based both on previous findings at laminectomy and on computerized tomography. The diagnosis of arachnoid cyst was suspected because of clinical features atypical for classical syringomyelia and a history of arachnoid cysts found during childhood. Air myelography demonstrated an extramedullary intradural mass anteriorly that proved to be an arachnoid cyst. Drainage and subtotal resection resulted in marked clinical improvement. This case illustrates the need for reevaluation when a patient with "known" syringomyelia presents an atypical clinical picture. Anterior cervical arachnoid cyst, which may accompany or succeed posterior arachnoid cysts, should be considered. (Arch Neurol 35:57-58, 1978)
Syringomyelia
characteristically has the presenting clinical picture of dissociated sensory loss in a cape-like distribution, wasting of intrinsic hand muscles, and loss of
upper limb reflexes.1 Atypical cases have been ascribed to unusual location of the intramedullary cyst or sufficient ex¬ pansion of the lesion to cause cord
compression. Extramedullary lesions can produce complicated syndromes that may be mistaken for syringomyelia. We re¬
that demonstrates the diagnostic difficulties of anterior cer¬ vical arachnoid cyst can present despite direct visualization of the cord during laminectomy and the use of computerized tomography. The clin¬ ical examination and history com¬ bined with standard neuroradiologic procedures established the diagnosis
port
Cysts
a
case
Accepted for publication July 18, 1977. From the Neurological Unit, Boston City Hospital, Boston. Reprint requests to the Neurological Unit, Boston City Hospital, 818 Harrison Ave, Boston, MA 02118 (Dr Sabin).
and led to treatment that otherwise would not have been undertaken. REPORT OF A CASE 1958 Hospitalization
9-year-old girl was admitted to Boston City Hospital with a two-week history of pain in her mid-to-upper back. Three years previously, she had been hospitalized because of posterior neck pain after a ß-hemolytic streptococcal pharyngitis treated with intramuscular penicillin. She A
found to have mild weakness of the right arm and leg that gradually resolved. On admission in 1958, she had weakness of the left arm and leg, absent left triceps jerk, bilateral extensor plantar responses, and diminished sensation to pinprick and light touch below T-4. A myelogram demonstrated a complete block at C-7 to T-l. At operation, three small posterior arachnoid cysts were found just above that level. Histologie examination showed a cyst lined by columnar and cuboidal epithelium without teratomatous elements. The senso¬ ry deficit resolved and motor function (
MD
complaints as in 1971. Generalized hyperreflexia was again noted, with a sensory deficit to pinprick in a T-2 distribution. Computerized tomography of the head
showed ventricles of normal size. A neck scan did not disclose an intraspinal cyst. At operation, a widened cord was again noted, and the presumed syrinx was drained of clear, colorless fluid. The catheter, which was patent but not draining spinal fluid, was replaced. The patient was discharged with improved motor function.
was
improved. 1971
Hospitalization
Following
several admissions during adolescence for symptomatic treatment of pain in neck and arm, the patient was admitted to another hospital because of neck pain and gait disability worsening over a six-month period. Examination showed weakness of all extremities, hyperreflexia, and extensor plantar responses. Pinprick sensation was reduced below the midcervical level, with sacral sparing. Vibratory sensation was mildly impaired in the lower extremities. Position sense was intact. Myelogram now showed ob¬ struction at C5-6. At operation, the cord was described as "ballooned posteriorly." Aspiration through the midline yielded 5 ml of clear, slightly viscous fluid, with subsequent collapse of the cord. A catheter was inserted into the presumed syrinx for drainage. At discharge, the patient could ambulate with a walker and regained nearly normal arm strength.
1974
Hospitalization
The patient, now 25 years old, was admitted to a third hospital with the same
Downloaded From: http://archneur.jamanetwork.com/ by a University of Pennsylvania User on 05/14/2015
1975
Hospitalization
patient was admitted to the same hospital with neck pain and was found to have a spastic quadriparesis greater on the right, with hypalgesia in a C7-8 distribu¬ tion. Myelography now showed a nearly complete block at C-7 to T-l. A computer¬ ized tomographic scan of the neck demon¬ strated a large cyst at C-6 occupying nearly the entire spinal canal interpreted as a probable syrinx. She did not have another operation and experienced mild improve¬ ment. Several weeks after discharge, she The
fell and struck her head without loss of consciousness, after which her neck pain disappeared and walking improved.
Current Hospitalization The
patient was admitted to the Neuro¬ logical Unit of Boston City Hospital on Dec 18, 1976, with a three-week history of Fig
1.—Routine myelogram block at C-7 level.
demonstrating
exquisite posterior
pain radiating down both arms and progressive weakness of the right lower extremity, rendering her neck
unable to walk without assistance. Exami¬ nation showed spastic quadriparesis with wasting and weakness of intrinsic hand
muscles, generalized hyperreflexia, except for absent triceps jerks, and extensor plantar responses. Pinprick and light touch
perception was
Fig 2.—Air myelography in lateral view showing posterior displacement of cord with cyst lying anteriorly. Arrow indicates inferior margin of cyst. Fig 3.—Pantopaque myelogram through C1-2 puncture defining upper border of cyst at C5-6.
diminished in a C-8 to T-l distribution, and vibration and position senses decreased in both legs, right greater than left. Sensation of pinprick, light touch, and heat was reduced anteriorly in a T2-5 distribution. Pantopaque myelogra¬ phy in the prone position showed a complete block at C-7 (Fig 1). An extramedullary intradural mass at C-7, compress¬ ing and displacing the cord posteriorly, was demonstrated by air myelography (Fig 2). The upper border of the cyst, C5-6, was further delineated by contrast myelog¬ raphy through a Cl-2 puncture (Fig 3). Surgical exploration with a right anterolateral approach on Jan 18,1977, disclosed a 2-cm multiloculated arachnoid cyst con¬ taining 5 ml of clear, colorless fluid. The cyst was adherent to the ventral cord so the procedure was limited to drainage and subtotal resection. Microscopic examina¬ tion showed fibrous tissue only. Postoperatively, the patient suffered a meningitic syndrome, with a negative CSF culture. She had a mild right Horner's syndrome that gradually improved. At discharge on Feb 19, 1977, she was free of neck pain and could walk up stairs unassisted. Strength of hands and sensation in the lower legs were also improved. Further improvement of both motor and sensory functions was found three months later.
COMMENT
need not have the dissociated sensory loss with its characteristic distribu¬ tion. Nevertheless, our patient's sen¬ sory deficit was sufficiently unusual, her neck pain so exquisite and local¬ ized, and her upper limb reflexes sufficiently spared that the diagnosis of syringomyelia was questioned, de¬ spite the findings at two operations apparently confirming that diagnosis. The childhood history suggested the possibility of arachnoid cyst and that aspiration of an anterior cyst through a thinned cord could alternatively account for the surgical findings. Adams2 has previously called atten¬ tion to the difficulty in diagnosis based on widening of the spinal cord noted at laminectomy. In Palmer's case,3 the anterior arachnoid cyst was recognized only at necropsy. Air
Syringomyelia classical sign of
Downloaded From: http://archneur.jamanetwork.com/ by a University of Pennsylvania User on 05/14/2015
myelography was most useful in our patient in outlining the anterior extramedullary cyst. None of the myélographie features of syringomye¬ lia described by Ellertsson4 were seen.
Though anterior arachnoid cyst was the preoperative diagnosis, neurent-
either anterior or posterior to the cord and can produce an intermittent course such as seen in our patient.5 This diagnosis was less likely because vertebral anomalies were not present, and no teratomatous or gastric elements had been noted in previous biopsy tissue.6 The etiology of such arachnoid cysts is unknown. The initial neurologic symptoms followed an upper respira¬ tory infection that had required medi¬ cal attention in both Palmer's case and the present patient. No histological, radiological, or surgical evidence for arachnoiditis could be found in either case. The patient reported by Hoffmann7 showed resistance to neck motion since birth and impaired motor function since earliest child¬ hood, which suggested a congenital basis for the lesion. The persistence and progression in our patient of presenting symptoms and signs into adulthood despite removal of the posterior cysts indicate that the anterior cyst may have been present since childhood. Earlier recog¬ nition of such a lesion might have spared the patient a significant amount of pain and disability and might have obviated the need for several operations. eric cysts
can occur
References JB, Hudgson P: The clinical features of communicating syringomyelia, in Barnett HJM, Foster JB, Hudgson P (eds): Syringomyelia. London, WB Saunders Co, 1973, pp 16-29. 1. Foster
2. Adams RD: Case records of the Massachusetts General Hospital. N Engl J Med 293:33-38, 1975. 3. Palmer JJ: Cervical intradural arachnoid cyst in a 3-year-old child: Report of a case. Arch Neurol 31:214-215, 1974. 4. Ellertsson AB: Semiologic diagnosis of syringomyelia related to roentgenologic findings. Acta Neurol Scand 45:385-402, 1969. 5. Adams RD, Wegner W: Congenital cyst of the spinal meninges as cause of intermittent compression of the spinal cord. Arch Neurol Psychiatry 58:57-69, 1947. 6. Fallon M, Gordon ARG, Lendrum AC: Mediastinal cysts of foregut origin associated with vertebral abnormalities. Br J Surg 41:520\x=req-\ 533, 1954. 7. Hoffmann GT: Cervical arachnoidal cyst: Report of a 6-year-old Negro male with recovery from quadriplegia. J Neurosurg 17:327-330, 1960.
