CSF Rhinorrhea

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CSF Rhinorrhea

Rhinology
Author Credentials 

Amber Luong, MD, PhD
Associate Professor and Director of Research
Dept. of Otorhinolaryngology – Head and Neck Surgery
McGovern Medical School at the University of Texas Health Science Center
6400 Fannin Street, MSB 5.036
Houston, TX

Module Summary

Cerebrospinal fluid (CSF) rhinorrhea typically presents with unilateral anterior dripping of clear fluid. There are a number of possible etiologies. Understanding these etiologies and defining and localizing the skull base defect has implications on the perioperative, intraoperative and postoperative surgical approach and management. Despite the variety of repair options, the success of skull base fistula repairs are reported as over 80%.

Module Learning Objectives 
  1. Recite the health risks of cerebrospinal fluid (CSF) fistulae.
  2. State relevant anatomic sites associated with CSF fistulae.
  3. Perform a comprehensive history and physical (H&P), as well as use appropriate lab and radiologic testing to identify and locate a CSF fistula.
  4. Describe the etiologies of CSF rhinorrhea.
  5. Utilize medical and surgical treatment options for CSF rhinorrhea.
  6. Recognize potential postoperative complications.
  7. Recognize patients who may require postoperative evaluation for benign intracranial hypertension.

Anatomy

Learning Objectives 

Apply the knowledge of anterior and lateral skull base anatomy along with associated anatomic relationships pertinent to the diagnosis and management of CSF rhinorrhea.

  1. Ethmoid skull base
    1. Cribriform plate
    2. Ethmoid roof
      1. Relationship with the frontal recess.
      2. Relationship with the sphenoid planum
    3. Lateral lamella of the cribriform
      1. Relationship with the anterior ethmoidal foramen and artery
      2. Relationship with the origin of the middle turbinate
  2. Sphenoid skull base
    1. Anatomy of the sphenoid sinus
      1. Tuberculum sellae
      2. Sella turcica
      3. Anterior clinoid process (optic recess)
      4. Carotid canal
      5. Lateral recess
    2. Pterygomaxillary space
      1. Vidian canal
      2. Foramen rotundum
      3. Internal maxillary artery branches
  3. Temporal bone anatomy
    1. Patterns of pneumatization
    2. Eustachian tube anatomy
References 
  1. Schuknecht HA, Gulya AJ. Anatomy of the temporal bone with surgical implications. Philadelphia: Lea & Febiger; 1986. 
  2. Stammberger H. Functional endoscopic sinus surgery. Philadelphia: Mosby Year Book; 1991. 

Pathogenesis

Learning Objectives 

Recite the common etiologies and how they lead to CSF rhinorrhea.

  1. Trauma
  2. Surgical complication
  3. Neoplasms
  4. Congenital CSF leaks
  5. Benign intracranial hypertension
References 
  1. Gonen L, Monteiro E, Klironomos G, Alghonaim Y, Vescan A, Zadeh G, Gentili F. Endoscopic Endonasal Repair of Spontaneous and Traumatic Cerebrospinal Fluid Rhinorrhea: A Review and Local Experience. Neurosurg Clin N Am. 2015 Jul;26(3):333-48. [EBM Level 5]
  2. Karnezis TT, Baker AB, Soler ZM, Wise SK, Rereddy SK, Patel ZM, Oyesiku NM, DelGaudio JM, Hadjipanayis CG, Woodworth BA, Riley KO, Lee J, Cusimano MD, Govindaraj S, Psaltis A, Wormald PJ, Santoreneos S, Sindwani R, Trosman S, Stokken JK, Woodard TD, Recinos PF, Vandergrift WA 3rd, Schlosser RJ. Factors impacting cerebrospinal fluid leak rates in endoscopic sellar surgery. Int Forum Allergy Rhinol. 2016 Nov;6(11):1117-1125.

Basic Science

Learning Objectives 
  1. Recognize that the intracranial compartment is a closed space, minimizing ability to accommodate for additional volume.
  2. Discuss how and where CSF is produced and reabsorbed in a steady-state fashion.
References 
  1. Daube JR, Reagan TJ, Sandok BA, et al. The cerebrospinal fluid system. In: Daube JR, Reagan TJ, Sandok BA, et al., eds. Medical Neurosciences: An Approach to Anatomy, Pathology, and Physiology by Systems and Levels, 2nd ed. Boston: Little, Brown and Company 1986:93-111. 

Incidence

Learning Objectives 
  1. Recognize that due to the rare nature of CSF fistulae, as well as the variety of causes, a reliable overall incidence of this problem is not available.
  2. Classify the relative incidence of possible etiologies for CSF rhinorrhea.
  3. Classify the incidence of CSF rhinorrhea associated with different surgeries adjacent or involving skull base (endoscopic sinus surgery, transphenoidal hypophysectomy, lateral skull base surgery, translabyrinthine resection of acoustic neuroma).
References 
  1. Black PM, Zervas NT, Candia GL. Incidence and management of complications of transphenoidal operation for pituitary adenomas. Neurosurgery. 1987;20:920-24. [EBM Level 4]
  2. Glasscock ME, Kveton JF, Jackson CG, et al. A systematic approach to the surgical management of acoustic neuroma. Laryngoscope. 1986;34:785-90. [EBM Level 4]
  3. House JL, Hitselberger WE, House WF. Wound closure and cerebrospinal fluid leak after translabyrinthine surgery. Am J Otol. 1982;4:126-28. [EBM Level 4]
  4. Keerl R, Stankiewicz J, Weber R, Hosemann W, Draf W. Surgical experience and complications during endoscopic sinus surgery. Laryngoscope. 1999;109:546-50. [EBM Level 4]
  5. Limskey ME, Lunsford LD, Flickinger JC. Radiosurgery for acoustic neuromas: the early experience. Neurosurgery. 1990;26:736-45. [EBM Level 4]

Patient Evaluation

Learning Objectives 
  1. Interpret aspects of history that help differentiate among the differentiate diagnoses of rhinorrhea from CSF rhinorrhea. 
  2. Recite pertinent aspects of the patient’s history to differentiate between possible etiologies of rhinorrhea from suspected CSF rhinorrhea. 
  3. Employ physical examination maneuver to elicit rhinorrhea consistent with CSF rhinorrhea.
  4. Identify nasal endoscopy findings supportive of CSF rhinorrhea.
  5. Recognize when consultations may assist in patient’s diagnosis and treatment of suspected benign intracranial hypertension.
  6. State the appropriate available testing of collected clear rhinorrhea for biochemical markers of CSF and their sensitivity and specificity. 
References 
  1. Mantur M, Łukaszewicz-Zając M, Mroczko B, Kułakowska A, Ganslandt O, Kemona H, Szmitkowski M, Drozdowski W, Zimmermann R, Kornhuber J, Lewczuk P. Cerebrospinal fluid leakage--reliable diagnostic methods. Clin Chim Acta. 2011 May 12;412(11-12):837-40. [EBM Level N/A]
  2. Oakley GM, Alt JA, Schlosser RJ, Harvey RJ, Orlandi RR. Diagnosis of cerebrospinal fluid rhinorrhea: an evidence-based review with recommendations. Int Forum Allergy Rhinol. 2016 Jan;6(1):8-16. [EBM Level 4]
  3. Wang EW, Vandergrift WA 3rd, Schlosser RJ. Spontaneous CSF Leaks. Otolaryngol Clin North Am. 2011 Aug;44(4):845-56. [EBM Level 4]

Imaging

Learning Objectives 
  1. Describe the imaging that are recommended for identifying the location of the fistula and possible soft tissue masses that may be transferring the fistula.
  2. Appraise additional imaging options when site of leak not easily identified with initial imaging.
  3. Apply the use and indications of intraoperative use of intrathecal fluorescein along with associated risk of its off-label use. Know the typical low concentration and protocol for delivery via lumbar drain of intrathecal fluorescein.
References 
  1. Oakley GM, Alt JA, Schlosser RJ, Harvey RJ, Orlandi RR. Diagnosis of cerebrospinal fluid rhinorrhea: an evidence-based review with recommendations. Int Forum Allergy Rhinol. 2016 Jan;6(1):8-16. [EBM Level 4]
  2. DelGaudio JM, Baugnon KL, Wise SK, Patel ZM, Aiken AH, Hudgins PA. Magnetic resonance cisternogram with intrathecal gadolinium with delayed imaging for difficult to diagnose cerebrospinal fluid leaks of anterior skull base. Int Forum Allergy Rhinol. 2015 Apr;5(4):333-8.
  3. Moseley JI, Carton CA, Stern, WE. Spectrum of complications in the use of intrathecal fluorescein. J Neurosurg. 1978;48:765-67. [EBM Level N/A]

Pathology

Learning Objectives 
  1. Describe the current understanding of how elevated intracranial hypertension can lead to CSF rhinorrhea.
References 
  1. Stevens SM, Rizk HG, Golnik K, Andaluz N, Samy RN, Meyer TA, Lambert PR. Idiopathic intracranial hypertension: Contemporary review and implications for the otolaryngologist. Laryngoscope. 2017 Mar 27. [EBM Level 4]
  2. Wang EW, Vandergrift WA 3rd, Schlosser RJ. Spontaneous CSF Leaks. Otolaryngol Clin North Am. 2011 Aug;44(4):845-56. [EBM Level 4]

Treatment

Learning Objectives 
  1. State the supportive treatment of nonsurgical, posttraumatic CSF fistulae which will frequently close spontaneously, and can be treated expectantly.
References 
  1. Prosser JD, Vender JR, Solares CA. Traumatic cerebrospinal fluid leaks. Otolaryngol Clin North Am. 2011 Aug;44(4):857-73. [EBM Level 4]

Medical Therapies

Learning Objectives 
  1. Know the postoperative adjunctive measures in patients with elevated intracranial pressure.

Surgical Therapies

Learning Objectives 
  1. Distinguish the options of surgical approaches for skull base fistula repair based on location of defect.
  2. Review the advantages and possible complications associated with intraoperative lumbar drain.
  3. Evaluate the variety of layers and materials available for repair, their possible indications and their relative effectiveness. 
    1. Graft materials – acellular dermis, free mucosal grafts, pedicled flaps, bone, cartilage, and fat. 
    2. Biologic adhesives – fibrinogen, fibrinogen/thrombin.
  4. Review the indications, blood supply and how to elevate a nasoseptal flap.
  5. Predict possible operative complications, although relatively uncommon.
References 
  1. Oakley GM, Orlandi RR, Woodworth BA, Batra PS, Alt JA. Management of cerebrospinal fluid rhinorrhea: an evidence-based review with recommendations. Int Forum Allergy Rhinol. 2016 Jan;6(1):17-24. [EBM Level 4]
  2. DeConde AS, Suh JD, Ramakrishnan VR. Treatment of cerebrospinal fluid rhinorrhea. Curr Opin Otolaryngol Head Neck Surg. 2015 Feb;23(1):59-64. [EBM Level 4]
  3. Eloy JA, Marchiano E, Vázquez A, Pfisterer MJ, Mady LJ, Baredes S, Liu JK. Management of Skull Base Defects After Surgical Resection of Sinonasal and Ventral Skull Base Malignancies. Otolaryngol Clin North Am. 2017 Apr;50(2):397-417. [EBM Level 4]
  4. Tien DA, Stokken JK, Recinos PF, Woodard TD, Sindwani R. Cerebrospinal Fluid Diversion in Endoscopic Skull Base Reconstruction: An Evidence-Based Approach to the Use of Lumbar Drains. Otolaryngol Clin North Am. 2016 Feb;49(1):119-29. [EBM Level 4]
  5. Clavenna MJ, Turner JH, Chandra RK. Pedicled flaps in endoscopic skull base reconstruction: review of current techniques. Curr Opin Otolaryngol Head Neck Surg. 2015 Feb;23(1):71-7. [EBM Level 5]
  6. Senior BA, Jafri K, Benninger M. Safety and efficacy of endoscopic repair of CSF leaks and encephaloceles: a survey of the members of the American Rhinologic Society. Am J Rhinology 2001;15:21-25. [EBM Level 5]
  7. Gonen L, Monteiro E, Klironomos G, Alghonaim Y, Vescan A, Zadeh G, Gentili F. Endoscopic Endonasal Repair of Spontaneous and Traumatic Cerebrospinal Fluid Rhinorrhea: A Review and Local Experience. Neurosurg Clin N Am. 2015 Jul;26(3):333-48. [EBM Level 4]
  8. http://www.american-rhinologic.org/videos (Surgical dissection videos on the ARS website, for members. ARS membership is FREE for residents.)

Complications

Learning Objectives 
  1. Describe the possible CSF rhinorrhea related sequelae.
References 
  1. Gonen L, Monteiro E, Klironomos G, Alghonaim Y, Vescan A, Zadeh G, Gentili F. Endoscopic Endonasal Repair of Spontaneous and Traumatic Cerebrospinal Fluid Rhinorrhea: A Review and Local Experience. Neurosurg Clin N Am. 2015 Jul;26(3):333-48. [EBM Level 4]

Review

Review Questions 
  1. Define a CSF fistula.
  2. What are potential etiologies of CSF rhinorrhea?
  3. What are possible medical sequelae of active CSF fistulae?
  4. What are risks factors associated with the development of CSF rhinorrhea?
  5. Develop an algorighm for the confirmation and location of suspected CSF rhinorrhea.
Resources 

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  1. Annual Meeting Webcasts (AMW):