PDPH: To Patch or Not to Patch

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PDPH: To Patch or Not to Patch Moderators: 1. Kim Strupp, MD FAAP Assistant Professor of Anesthesiology, Children s Hospital Colorado/University of Colorado, Aurora, CO 2. Debnath Chatterjee, MD Associate Professor of Anesthesiology, Children s Hospital Colorado/University of Colorado, Aurora, CO Learning Objectives: Upon completion of this PBLD, participants will be able to- 1. Discuss the indications and contraindications for an epidural blood patch in the pediatric population. 2. Critically analyze barriers to performing epidural blood patches in the pediatric population. 3. Summarize strategies to prevent post dural puncture headaches. 4. Discuss therapeutic options in the conservative management of a post dural puncture headache. Case Description: A 12-year-old Caucasian male was otherwise healthy until one week ago when he developed symptoms of an upper respiratory infection including cough and rhinorrhea. He presented to the emergency department with fevers to 104 degrees Fahrenheit, headaches and neck stiffness. A lumbar puncture was performed as part of the work up for meningitis and he was started on antibiotics. Two days later he develops a worsening headache and you are consulted on the acute pain service for assistance in management. What additional information (history, physical exam, laboratory/pathology) would you obtain to aide in the diagnosis and treatment of this patient s headache? The patient describes his headache as much worse than it was 2 days ago. The pain involves his entire head. It is a dull ache rated at a 3/10 when laying down, but increases to an 8/10 throbbing headache if he tries to sit or stand. He is sensitive to both light and sound and admits to nausea but no vomiting. What is the differential diagnosis for this patient s headache? What are the initial management strategies for a post dural puncture headache (PDPH)? What could have been done to prevent this patient s PDPH? Would you consider performing an epidural blood patch in this patient? What are the barriers to performing blood patches in the pediatric population?

As you are reviewing the medical record, you discover that the patient s platelet count is 72,000. He denies any history or easy bleeding or bruising and there is no family history of bleeding problems. How does this information change your management plans? You discover that in addition to the above symptoms, the patient also presented with mild bilateral lower extremity weakness. What is your differential diagnosis? How does this new information change your management plan? You speak with the consulting neurologist who has diagnosed the patient with a mild case of guillain-barré syndrome. He insists that the patient will feel much better after an epidural blood patch. What do you tell the neurologist? How would you counsel the patient and his family? What are the potential risks associated with performing an epidural blood patch in a patient with a preexisting neurological condition? What is the role of cosyntropin in the conservative management of PDPH? Discussion Introduction Postdural puncture headaches (PDPH) in pediatrics may present diagnostic and management dilemmas. Many of these patients present after a diagnostic lumbar puncture and admit to having a headache at the time of initial presentation. Often the characteristics of the headache will change and the clinician must determine whether there is development of a postdural puncture headache or worsening of the underlying condition. When suspicion is high for a postdural puncture headache, one must determine whether the patient is an appropriate candidate for an epidural blood patch. Ongoing infection, thrombocytopenia, and pre-existing neurologic injuries should be strongly considered before performing an epidural blood patch. In addition, pediatric patients may or may not be able to cooperate with an awake epidural blood patch. Performing a blood patch may expose the patient to the risk of an anesthetic. Conservative therapies including bed rest, fluid administration, caffeine and potentially cosyntropin should be offered to all patients, but especially those deemed at high risk for an epidural blood patch.

Diagnosis Postdural puncture headaches occur as a complication of intentional puncture of the dura for procedures such as diagnostic lumbar puncture, lumbar puncture for intrathecal chemotherapy administration, myelogram and spinal anesthesia/analgesia or from unintentional puncture of the dura related to epidural anesthesia/analgesia. The International Classifications of Headache Disorders includes diagnostic criteria for postdural puncture headaches. A) The headache worsens within 15 minutes after sitting or standing and improves within 15 minutes after lying, with at least one of the following and fulfilling criteria C and D: neck stiffness, tinnitus, hypacusia, photophobia, nausea. B) Dural puncture has been performed. C) Headache develops within 5 days of dural puncture. D) Headache resolves either spontaneously within 1 week or within 48 hours after effective treatment of the spinal fluid leak (usually by epidural blood patch). Most commonly the headache will worsen within 20 seconds of a postural change and reaches its maximum within a minute; the headache subsides within 20 seconds of assuming a recumbent position. The pain is typically severe and described as burning, dull, or throbbing. Location may be variable; however, commonly it is described as frontal or occipital and may radiate to the neck and shoulders. Pain is worsened by Valsalva, coughing, sneezing or straining. In addition to the associated symptoms listed above, back pain, vertigo, cranial nerve palsies, diplopia, and cortical blindness have all been reported. 1 Pathophysiology There are several theories proposed to describe the pathophysiology of PDPH. Relative CSF hypovolemia develops due to a hole in the dura causing persistent leakage. When the patient assumes an upright position, it is theorized that downward pull on pain-sensitive structures causes the headache. Alternatively, a theory involving the Monro-Kellie doctrine states that intracranial vessels vasodilate in an attempt to maintain constant intracranial volume. A third mechanism theorizes that hypersensitivity to substance P is associated with an upregulation of neurokinin 1 receptors (NK1R). 1 Differential Diagnosis Differential diagnosis includes migraine headaches, tension headaches, venous sinus thrombosis, subdural hematoma, ischemic stroke, cervical facet syndrome, and headache associated with postural orthostatic tachycardia. Though clinical features alone are sufficient to diagnose PDPH, there are several diagnostic tests to consider. Firm continuous pressure on the patient s abdomen or Trendelenburg position may relieve the headache by increasing CSF pressure. Magnetic resonance imaging (MRI) may be normal or may show diffuse pachymeningeal enhancement with gadolinium, cerebellar tonsillar descent with crowding of the posterior fossa and obliteration of the basilar cisterns, enlargement of the pituitary gland and/or decreased ventricular size. A diagnostic lumbar puncture may have low or normal opening pressure, slightly elevated CSF protein, and elevated CSF lymphocytes. 1

Risk Factors and Prevention Risk factors for PDPH are divided into nonmodifiable and modifiable. Nonmodifiable risk factors include age, female gender, low body mass index, history of prior PDPH and history of chronic headache. Risk is highest in 20-30-year-olds, and lowest over age 60. The risk in children ranges from 2-15%; the risk in adolescents may approach that of adults. 1 It is imperative to understand modifiable risk factors in order to optimize prevention of PDPH. These risk factors may be categorized as equipment related (size of the spinal needle, needle shape), procedure-related factors (bevel orientation and angle of insertion, stylet replacement), and operator experience. 2 Larger needle size is one of the most important risk factors for development of PDPH. For spinal anesthesia, 25-, 26-, and 27-gauge needles are recommended. The American Academy of Neurology practice guidelines recommend the use of 22-gauge needles with the concern that smaller needles will extend the duration of the procedure due to slow CSF flow. Non-cutting needles are less likely to cause PDPH than cutting needles. The hole created by a non-cutting pencil point needle is more irregular than the hole created by a cutting needle. The irregular hole may cause more local edema or tissue reaction, which may plug the dural tear limiting the leakage of CSF. 3 One study in 414 children aged 2-17 years undergoing surgery with spinal anesthesia showed a reduction in PDPH from 4.5% to 0.4% when comparing a 26-gauge cutting needle to a 27-gauge pencil point needle. There was no difference in the incidence of backache. Only 2 children received an epidural blood patch or epidural saline. 3 Another study evaluated the introduction of a 25-gauge pencil point spinal needle into their oncology practice. 162 oncology patients between the ages of 2-17 years were included. The authors found no significant difference in the incidence of PDPH between the 22-gauge Quincke cutting needle and 25-gauge pencil point needle; however, they did note less back pain reported with the 25- gauge pencil point needle. 4 Bevel orientation is only important when utilizing cutting needles. When the bevel is oriented parallel to the long axis of the spine (rather than perpendicular), the incidence of PDPH is reduced. 5 Stylet reinsertion reduced PDPH incidence from 16.3% to 5% in one study using 21- gauge Sprotte needles. Experienced clinicians are less likely to cause an inadvertent dural puncture during epidural anesthesia/analgesia. Factors that have not been shown to prevent PDPH include bed rest, hydration, patient position during the LP, the volume of CSF removed, and the number of attempts. 2 In cases of inadvertent dural puncture where an epidural is in place, epidural morphine administration has shown a significant risk reduction compared to placebo, though it may increase the risk for nausea and itching. 6,7,8 Intravenous cosyntropin also showed a significant risk reduction compared to placebo. 6,8,9 Adrenocorticotrophic hormone (ACTH) may stimulate the release of aldosterone, thereby enhancing salt and water retention and expanding blood volume. This change may induce dural edema or overlap the edges of the dural tear.

Alternatively, ACTH may increase production of CSF. When given to parturients in a 1 mg intravenous dose, cosyntropin reduced the incidence of PDPH after accidental dural puncture from 68.9% to 33%. 9 Prophylactic administration of caffeine has shown no benefit in reducing the risk of PDPH; however intravenous aminophylline has shown a significant risk reduction compared to placebo. 6 Though showing some benefit in smaller studies, prophylactic epidural blood patch, epidural saline and intrathecal catheters have not been shown effective in reducing the risk of PDPH in larger trials. 7 Conservative Treatment The majority of PDPH resolve within a week with conservative management. Bed rest and adequate hydration as well as symptomatic treatment with acetaminophen, non-steroidal antiinflammatory drugs and antiemetics are the basis for initial conservative therapy. More aggressive medical therapy involves intravenous administration of methylxanthines including caffeine, aminophylline and theophylline. 2,10 These medications may counteract the cerebral vasodilation that occurs with CSF volume loss. Methylxanthines block adenosine receptors which causes vasoconstriction of cerebral blood vessels. Side effects of methylxanthines include central nervous system stimulation, seizures, gastric irritation and cardiac arrhythmias. 2 Gabapentin, hydrocortisone and theophylline have been shown to decrease pain severity scores. 10 There is lack of conclusive evidence for treatment with sumatriptan, adrenocorticotropic hormone, pregabalin and cosyntropin. 10 Greater occipital nerve blocks (GONB) have also been used to treat PDPH. In a prospective audit of 24 patients with PDPH, 19 were offered GONB or EBP. 18 patients chose GONB and of those, 12 headaches resolved. 6 patients had a partial response and were treated with EBP. The authors concluded that GONB with dexamethasone may have a role in the management of PDPH, with success noted in treating PDPH, photophobia, nausea and neck stiffness. Of note, GONB had minimal effect on tinnitus. 11 Epidural Blood Patch When conservative therapy fails, an epidural blood patch may be effective in treating PDPH. Blood is drawn sterilely from a peripheral vein or artery and injected into the epidural space at a level at or below the level of the previous dural puncture. If the child is awake, the injection should cease if the child describes pressure or discomfort in the back. In the anesthetized child, no more than 0.3 ml/kg or 20 ml of blood should be injected. 12 Theoretically the blood patch creates a clot over the meningeal hole to prevent further CSF leakage. The initial success rate of EBP is quoted as less than 70%; however, repeat blood patch is effective 90% of the time. 8 Contraindications to EBP include patient refusal, coagulopathy, systemic sepsis, fever and infection at the site. Controversy exists with regard to performance of EBP in patients with malignancy, HIV and acute varicella, with concern of seeding the epidural space with malignant cells or infection.

Complications include cranial nerve palsies, elevated intracranial pressure, paraparesis, cauda equina syndrome, bradycardia, aseptic arachnoiditis, chemical or infectious meningitis, subdural hematoma, subarachnoid hematoma, seizures, back pain, neck pain, and radicular pain from nerve root displacement and irritation. 2 Two hospitals in Finland described their experience in performing EBP s in children over a 10- year period. 42 EBP s were performed in 41 patients during this period. 5 patients were aged 3-12 years and the remaining 36 patients were aged 13-18 years. The source of the dural puncture was diagnostic LP in 26, spinal anesthesia/analgesia in 11, chemotherapy in 2, and inadvertent dural puncture with an epidural needle in 4 patients. The initial success rate of EBP was 90%, with permanent relief in 85%. There was no correlation with the volume of blood injected and the efficacy of EBP. 12 Patients with PDPH who underwent diagnostic LP s for the suspicion of meningitis and LP for chemotherapy are challenging to manage. Injection of blood into the epidural space increased the risk of infectious complications and the risk for the spread of malignancy. 12 The recommended volume of blood to inject is 0.25-0.5 ml/kg, to a maximum of 15 ml in a child. The injection should be stopped if increased resistance is encountered. Repeat EBP should be considered if the headache recurs. 13 When epidural blood patch fails, computed tomography (CT)-guided inject of fibrin glue has been utilized in an attempt to seal the dural tear. 2 Guillain-Barré Syndrome Guillain-Barré syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy characterized by progressive motor weakness, areflexia and ascending paralysis. These symptoms are typically preceded by an upper respiratory or gastrointestinal infection by one to three weeks. Weakness starts in the extremities, followed by the trunk, neck, and facial muscles. As the disease progresses, motor paralysis and respiratory failure may develop. Its incidence is approximately 0.75-2 in 100,000 per year. 14 Treatment involves supportive therapy. As the disease progresses, plasmapheresis and intravenous immunoglobulin (IVIG) are utilized. 14,15 Controversy exists in pregnancy complicated by GBS with regard to regional anesthesia. A case report describing a 27-year-old parturient whose neurologic status worsened following epidural labor analgesia was published in Anesthesia and Analgesia in 2004. The patient initially presented at 36 weeks gestation with bilateral facial palsy, progressive numbness and weakness in all limbs and inability to walk. She was treated with IVIG with improvement in walking ability and mouth opening. She went into labor at 41 weeks gestation and epidural analgesia was provided for labor and delivery. She developed a sensory and motor block with incremental dosing of the epidural. After delivery, the motor block did not subside. She was unable to walk and had increased facial and upper extremity weakness. MRI was normal, nerve

conduction velocities were more reduced and distal latencies more delayed than before. She was treated with IVIG again. At her 4 month follow up she still had a gait disturbance and was dependent on a walker. 15 Alternatively, there are multiple case reports of successful epidural anesthesia/analgesia in parturients with GBS who experienced no worsening of neurologic symptoms. 14 The risks of worsening neurologic deficit must be balanced against alternative anesthesia/analgesia options. If the alternative is general anesthesia, caution must be exercised with neuromuscular blockade. Administration of succinylcholine has resulted in hyperkalemic arrests. 15 Conclusion PDPH is a relatively common complication of either intentional or inadvertent puncture of the dura. Using smaller, pencil point needles has been shown to be effective in reducing the risk of PDPH. In cases of inadvertent dural puncture, prophylactic cosyntropin, epidural morphine or intravenous aminophylline should be considered. Initial conservative treatment of a PDPH is warranted and may include bed rest, hydration, caffeine, aminophylline and theophylline. Greater occipital nerve blocks are another option for initial treatment. With failure of conservative therapy, an epidural blood patch should be considered. In pediatric patients, this procedure may require an additional risk of sedation or general anesthesia. In addition, the clinical picture may be complicated by pre-existing neurologic deficits including GBS, coagulopathy, malignancy, or ongoing systemic infection. Though EBP is a highly effective treatment for PDPH, these potential risks should be balanced against the benefit of this treatment. References: 1. Bezov D, Lipton RB and Ashina S. Post-Dural Puncture Headache: Part I Diagnosis, Epidemiology, Etiology, and Pathophysiology. Headache. 2010; 50:1144-1152. 2. Bezov D, Ashina S and Lipton R. Post-Dural Puncture Headache: Part II Prevention, Management, and Prognosis. Headache. 2012; 50:1482-1498. 3. Apiliogullari S, Duman A, Gok F and Akillioglu I. Spinal needle design and size affect the incidence of postdural puncture headache in children. Pediatric Anesthesia. 2010; 20:177-182. 4. Lowery S and Oliver A. Incidence of postdural puncture headache and backache following diagnostic/therapeutic lumbar puncture using a 22G cutting spinal needle, and after introduction of a 25G pencil point spinal needle. Pediatric Anesthesia. 2008; 18:230 234. 5. Richman JM, Joe EM, Cohen SR, et al. Bevel Direction and Postdural Puncture Headache: A Meta-Analysis. The Neurologist. 2006; 12(4):224-228. 6. Basurto Ona X, Uriona Tuma SM, Martinez Garcia L, et al. Drug therapy for preventing post-dural puncture headache (review). The Cochrange Collaboration. 2013; 2: CD001792.

7. Apfel CC, Saxena A, Cakmakkaya OS, et al. Prevention of postdural puncture headache after accidental dural puncture: a quantitative systematic review. British Journal of Anaesthesia. 2010; 105(3):255-263. 8. Bradbury CL, Singh SI, Badder SR, et al. Prevention of post-dural puncture headache in parturients: a systematic review and meta-analysis. Acta Anaesthesiol Scand. 2013; 57(4):417-430. 9. Hakim SM. Cosyntropin for Prophylaxis against Postdural Puncture Headache after Accidental Dural Puncture. Anesthesiology. 2010; 113:413-420. 10. Basurto Ona X, Osorio D, Bonfill Cosp X. Drug Therapy for treating post-dural puncture headache (review). The Cochrane Collaboration. 2015; 7:CD007887. 11. Niraj G, Kelkar A and Girotra V. Greater occipital nerve block for postdural puncture headache (PDPH): A prospective audit of a modified guideline for the management of PDPH and review of the literature. Journal of Clinical Anesthesia. 2014; 26:539-544. 12. Kokki M, Sjovall S, and Kokki H. Epidural blood patches are effective for postdural puncture headch in pediatrics a 10-year experience. Pediatric Anesthesia. 2012; 22:1205-1210. 13. Borges BCR, Wong G, Isaac L and Hayes J. Unusual presentation of postdural puncture headache requiring repeat epidural blood patch in a 4-year-old child. Pediatric Anesthesia. 2014; 24:541-543. 14. Kocabas S, Karaman S, Firat V and Bademkiran F. Anesthetic management of Guillain- Barré syndrome in pregnancy. Journal of Clinical Anesthesia. 2007; 19:299-302. 15. Wiertlewski S, Magot A, Drapier S et al. Worsening of Neurologic Symptoms After Epidural Anesthesia for Labor in a Guillain-Barré Patient. Anesth Analg. 2004; 98:825-827.