Chiari I Malformation Presenting as Recurrent Spinal Headache R. Michael HuIlander, MD, Terrence D. Bogard, MD, David Leivers, Deborah Moran, MD, and David M. Dewan, MD
FRCAnaes,
Departments of Anesthesiology and Clinical Investigation, Naval Hospital, San Diego, California and the Department of Anesthesiology, Wake Forest University, Bowman Gray School of Medicine, Winston-Salem, North Carolina
P
ostdural puncture headache (PDPH) is a wellrecognized complication after spinal anesthesia (1). When conservative treatment or epidural blood patch (EBP) fail, alternative causes of the headache need to be reconsidered. We present a previously unreported association of recurrent PDPH with a Chiari I malformation.
Case Report A 31-yr-old woman (gravida 1, para 0), with an unremarkable medical history, received analgesia for labor via a lumbar epidural catheter. Subsequently, when she required cesarean section for failure of the labor to progress, satisfactory lumbar epidural anesthesia could not be established. Good surgical anesthesia was provided by subarachnoid block, although spinal needle placement proved technically difficult and required several attempts before successful dural puncture with a 22-gauge needle. The surgery and immediate postoperative period were uneventful. Although she reported a mild positional headache, her surgeon discharged her on the third postoperative day. However, over the following several days, the patient's headache became progressively more severe. The headache was associated with mild neck pain, which worsened when the patient was sitting or standing. Moderate relief was obtained by caffeine ingestion. On the sixth postoperative day, although afebrile, she developed hearing changes (echoing), visual disturbances (blurring and color distortion), and nausea. The diagnosis of PDPH was made. An EBP using 15 mL of autologous blood was administered, with marked improvement of all symptoms. The patient had a recurrence of the headache 4 days The Chief, Navy Bureau of Medicine and Surgery, Washington, D.C., Clinical Investigation Program, sponsored this report No. 84-16-1968-330, as required by HSETCINST 6000.41.The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the U.S.Government. Accepted for publication July 14, 1992. Address correspondence to Dr. Hullander, c/o Clinical Investigation Department, Naval Hospital, San Diego, CA 92134-5000.
0003-2999/92/$5.00
later. The headache was similar in character to the original, but the severity had increased, causing her to awaken at night. In view of these symptoms, neurologic consultation was obtained before consideration was given to a repeat EBP. Although the physical examination was normal, a magnetic resonance imaging scan was ordered, which revealed a Chiari I malformation. During the recommended treatment of a 6-wk course of prednisone, her symptoms progressively improved, and she remains symptom free.
Discussion The Chiari I malformation consists of displacement of the tonsils of the cerebellum through the foramen magnum and, possibly, into the cervical spinal canal (2). Banerji and Millar (3) state that the presenting features of the anomaly in adults are variable and may be deceptive, with the diagnosis only being made after radiologic investigation. The prognosis in such patients is good with conservative therapy, but when objective neurologic deficits develop, surgical decompression to establish free-flow cerebrospinal fluid (CSF) from the fourth ventricle may be required
(4). The prevailing opinion is that the herniation is secondary to congenital hydrocephalus (5).However, on the basis of several patients becoming symptomatic after placement of subarachnoid-peritoneal shunts, Welch et al. (6) postulated that it may be an acquired disorder. The common feature of both theories is that an increased gradient of CSF pressure develops between the brain and the spinal canal. A similar pressure differential, caused by CSF leakage, is also believed to account for the PDPH (7). In this case, the presenting features were typical of PDPH. After conservative measures failed, an EBP was performed with initial success, supporting this diagnosis. Persistent PDPH is effectively treated by an EBP in approximately 95% of cases (8). Persistence or recurrence of a PDPH in obstetric patients necesAnesth Analg 1992;751025-6
1025
1026
CASE REPORTS
ANESTH ANALG 1~2;75:1025-6
sitates reviewing alternative causes for headaches, such as cortical vein thrombosis (9,lO). Prolonged headache with neurologic signs may indicate a subdural hematoma (11,lZ) requiring specific investigation. The finding of a Chiari I malformation on magnetic resonance imaging scan in this case led to the possibility that the headache was secondary to the Chiari I malformation. Whether the spinal puncture unmasked a preexisting malformation or created the anomaly is unknown. In either case, the Chiari I malformation should be considered in the differential diagnosis of persistent PDPH. Although there are no reported cases of the administration of spinal anesthesia in patients with a symptomatic Chiari I malformation, we believe that the choice of anesthesia should take into account the risk of increasing the CSF pressure gradient between the brain and the spinal canal when the diagnosis is known.
References 1. Bridenbaugh PO. Postdural puncture headache. Reg Anesth 1978;3:%3.
2. Chiari H. a e r Veranderungen des Kleinhirns infolge von hydrocephalie des Grosshims. Dtsch Med Wochenschr 1891; 17:1171-5. 3. Banerji NK, Millar JHD. Chiari malformation presenting in adult life, its relationship to syringomyelia. Brain 1974;9715768. 4. Cahan LD, Bentson JR. Considerations on the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg 1982;572&31. 5. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 1965;28:247-59. 6. Welch K, ShiUito J, Strand R, Fischer EG, Winston KR. Chiari I "malformation"-an acquired disorder? J Neurosurg 1981;55: 604-9. 7. Kunkle EC, Ray BS, Wolff HG. Experimental studies on headache. Analysis of headache associated with changes in intracranial pressure. Neurol Psychiatr (Bucur) 1943;49:323-58. 8. Anderson EF. Use of epidural blood patch in the treatment of postdural puncture headache. SD J Med 1984;3759. 9. Hubbert CH. Dural puncture headache suspected, cortical vein thrombosis diagnosed (letter). Anesth Analg 1987;66:285. 10. Younker D, Jones MM, Adenwala T, Citrin A, Joyce TH. Maternal cortical vein thrombosis and the obstetric anesthesiologist. Anesth Analg 1986;651007-12. 11. Newrick P, Read D. Subdural hematoma as a complication of spinal anaesthetic. Br Med J (Clin Res) 1982;285:341-2. 12. Eerola L, Kaukinen L, Kaukinen S. Fatal brain lesion following spinal anaesthesia. Acta Anaesthesiol Scand 1981;25:1156.
FRCAnaes,
Departments of Anesthesiology and Clinical Investigation, Naval Hospital, San Diego, California and the Department of Anesthesiology, Wake Forest University, Bowman Gray School of Medicine, Winston-Salem, North Carolina
P
ostdural puncture headache (PDPH) is a wellrecognized complication after spinal anesthesia (1). When conservative treatment or epidural blood patch (EBP) fail, alternative causes of the headache need to be reconsidered. We present a previously unreported association of recurrent PDPH with a Chiari I malformation.
Case Report A 31-yr-old woman (gravida 1, para 0), with an unremarkable medical history, received analgesia for labor via a lumbar epidural catheter. Subsequently, when she required cesarean section for failure of the labor to progress, satisfactory lumbar epidural anesthesia could not be established. Good surgical anesthesia was provided by subarachnoid block, although spinal needle placement proved technically difficult and required several attempts before successful dural puncture with a 22-gauge needle. The surgery and immediate postoperative period were uneventful. Although she reported a mild positional headache, her surgeon discharged her on the third postoperative day. However, over the following several days, the patient's headache became progressively more severe. The headache was associated with mild neck pain, which worsened when the patient was sitting or standing. Moderate relief was obtained by caffeine ingestion. On the sixth postoperative day, although afebrile, she developed hearing changes (echoing), visual disturbances (blurring and color distortion), and nausea. The diagnosis of PDPH was made. An EBP using 15 mL of autologous blood was administered, with marked improvement of all symptoms. The patient had a recurrence of the headache 4 days The Chief, Navy Bureau of Medicine and Surgery, Washington, D.C., Clinical Investigation Program, sponsored this report No. 84-16-1968-330, as required by HSETCINST 6000.41.The views expressed in this article are those of the authors and do not reflect the official policy or position of the Department of the Navy, Department of Defense, or the U.S.Government. Accepted for publication July 14, 1992. Address correspondence to Dr. Hullander, c/o Clinical Investigation Department, Naval Hospital, San Diego, CA 92134-5000.
0003-2999/92/$5.00
later. The headache was similar in character to the original, but the severity had increased, causing her to awaken at night. In view of these symptoms, neurologic consultation was obtained before consideration was given to a repeat EBP. Although the physical examination was normal, a magnetic resonance imaging scan was ordered, which revealed a Chiari I malformation. During the recommended treatment of a 6-wk course of prednisone, her symptoms progressively improved, and she remains symptom free.
Discussion The Chiari I malformation consists of displacement of the tonsils of the cerebellum through the foramen magnum and, possibly, into the cervical spinal canal (2). Banerji and Millar (3) state that the presenting features of the anomaly in adults are variable and may be deceptive, with the diagnosis only being made after radiologic investigation. The prognosis in such patients is good with conservative therapy, but when objective neurologic deficits develop, surgical decompression to establish free-flow cerebrospinal fluid (CSF) from the fourth ventricle may be required
(4). The prevailing opinion is that the herniation is secondary to congenital hydrocephalus (5).However, on the basis of several patients becoming symptomatic after placement of subarachnoid-peritoneal shunts, Welch et al. (6) postulated that it may be an acquired disorder. The common feature of both theories is that an increased gradient of CSF pressure develops between the brain and the spinal canal. A similar pressure differential, caused by CSF leakage, is also believed to account for the PDPH (7). In this case, the presenting features were typical of PDPH. After conservative measures failed, an EBP was performed with initial success, supporting this diagnosis. Persistent PDPH is effectively treated by an EBP in approximately 95% of cases (8). Persistence or recurrence of a PDPH in obstetric patients necesAnesth Analg 1992;751025-6
1025
1026
CASE REPORTS
ANESTH ANALG 1~2;75:1025-6
sitates reviewing alternative causes for headaches, such as cortical vein thrombosis (9,lO). Prolonged headache with neurologic signs may indicate a subdural hematoma (11,lZ) requiring specific investigation. The finding of a Chiari I malformation on magnetic resonance imaging scan in this case led to the possibility that the headache was secondary to the Chiari I malformation. Whether the spinal puncture unmasked a preexisting malformation or created the anomaly is unknown. In either case, the Chiari I malformation should be considered in the differential diagnosis of persistent PDPH. Although there are no reported cases of the administration of spinal anesthesia in patients with a symptomatic Chiari I malformation, we believe that the choice of anesthesia should take into account the risk of increasing the CSF pressure gradient between the brain and the spinal canal when the diagnosis is known.
References 1. Bridenbaugh PO. Postdural puncture headache. Reg Anesth 1978;3:%3.
2. Chiari H. a e r Veranderungen des Kleinhirns infolge von hydrocephalie des Grosshims. Dtsch Med Wochenschr 1891; 17:1171-5. 3. Banerji NK, Millar JHD. Chiari malformation presenting in adult life, its relationship to syringomyelia. Brain 1974;9715768. 4. Cahan LD, Bentson JR. Considerations on the diagnosis and treatment of syringomyelia and the Chiari malformation. J Neurosurg 1982;572&31. 5. Gardner WJ. Hydrodynamic mechanism of syringomyelia: its relationship to myelocele. J Neurol Neurosurg Psychiatry 1965;28:247-59. 6. Welch K, ShiUito J, Strand R, Fischer EG, Winston KR. Chiari I "malformation"-an acquired disorder? J Neurosurg 1981;55: 604-9. 7. Kunkle EC, Ray BS, Wolff HG. Experimental studies on headache. Analysis of headache associated with changes in intracranial pressure. Neurol Psychiatr (Bucur) 1943;49:323-58. 8. Anderson EF. Use of epidural blood patch in the treatment of postdural puncture headache. SD J Med 1984;3759. 9. Hubbert CH. Dural puncture headache suspected, cortical vein thrombosis diagnosed (letter). Anesth Analg 1987;66:285. 10. Younker D, Jones MM, Adenwala T, Citrin A, Joyce TH. Maternal cortical vein thrombosis and the obstetric anesthesiologist. Anesth Analg 1986;651007-12. 11. Newrick P, Read D. Subdural hematoma as a complication of spinal anaesthetic. Br Med J (Clin Res) 1982;285:341-2. 12. Eerola L, Kaukinen L, Kaukinen S. Fatal brain lesion following spinal anaesthesia. Acta Anaesthesiol Scand 1981;25:1156.
