Skull Base 2009; 19(4): 279-285
DOI: 10.1055/s-0028-1115326
CASE REPORT

© Thieme Medical Publishers

Refractory Cerebrospinal Fluid Rhinorrhea Secondary to Occult Superior Vena Cava Syndrome and Benign Intracranial Hypertension: Diagnosis and Management

Jonathan M. Bledsoe1 , Eric J. Moore2 , Michael J. Link1 , 2
  • 1Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota
  • 2Department of Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota
Further Information

Publication History

Publication Date:
09 January 2009 (online)

ABSTRACT

Objective: This study is designed to describe the association between benign intracranial hypertension (BIH) and spontaneous cerebrospinal fluid (CSF) rhinorrhea and address the effect of extracranial venous flow dynamics on intracranial pressure (ICP). Methods: We present a 58-year-old woman with refractory spontaneous CSF rhinorrhea who was later found to have superior vena cava syndrome. The patient had undergone two prior transnasal endoscopic repair attempts. In retrospect, a preoperative magnetic resonance venogram (MRV) suggested very prolonged cerebral transit time, despite otherwise normal intracranial venous anatomy. Results: The CSF leak was repaired through a bifrontal craniotomy. The intraoperative and postoperative course was complicated due to the patient's significant comorbidities. She ultimately made a good recovery and has not had any further CSF rhinorrhea in more than 2 years of follow-up. Conclusions: Refractory, spontaneous CSF leak must prompt aggressive investigation for multiple causes of elevated ICP. A cerebral transit time can be obtained from scout imaging when a magnetic resonance angiogram or MRV is performed, and this may disclose elevated ICP if it is prolonged. If endoscopic transnasal repair fails, craniotomy and direct suture repair and autologous tissue reinforcement of the skull base may prove successful and durable, even if BIH persists.

REFERENCES

  • 1 Hegazy H M, Carrau R L, Snyderman C H, Kassam A, Zweig J. Transnasal endoscopic repair of cerebrospinal fluid rhinorrhea: a meta-analysis.  Laryngoscope. 2000;  110(7) 1166-1172
  • 2 Hubbard J L, McDonald T J, Pearson B W, Laws Jr E R. Spontaneous cerebrospinal fluid rhinorrhea: evolving concepts in diagnosis and surgical management based on the Mayo Clinic experience from 1970 through 1981.  Neurosurgery. 1985;  16(3) 314-321
  • 3 Mirza S, Thaper A, McClelland L, Jones N S. Sinonasal cerebrospinal fluid leaks: management of 97 patients over 10 years.  Laryngoscope. 2005;  115(10) 1774-1777
  • 4 Carrau R L, Snyderman C H, Kassam A B. The management of cerebrospinal fluid leaks in patients at risk for high-pressure hydrocephalus.  Laryngoscope. 2005;  115(2) 205-212
  • 5 Kirtane M V, Gautham K, Upadhyaya S R. Endoscopic CSF rhinorrhea closure: our experience in 267 cases.  Otolaryngol Head Neck Surg. 2005;  132(2) 208-212
  • 6 Wise S K, Schlosser R J. Evaluation of spontaneous nasal cerebrospinal fluid leaks.  Curr Opin Otolaryngol Head Neck Surg. 2007;  15(1) 28-34
  • 7 Dunn C J, Alaani A, Johnson A P. Study on spontaneous cerebrospinal fluid rhinorrhoea: its aetiology and management.  J Laryngol Otol. 2005;  119(1) 12-15
  • 8 Ransom E R, Komotar R J, Mocco J, Connolly E S, Mullins K J. Shunt failure in idiopathic intracranial hypertension presenting with spontaneous cerebrospinal fluid leak.  J Clin Neurosci. 2006;  13(5) 598-602
  • 9 Schlosser R J, Wilensky E M, Grady M S, Bolger W E. Elevated intracranial pressures in spontaneous cerebrospinal fluid leaks.  Am J Rhinol. 2003;  17(4) 191-195
  • 10 Schlosser R J, Wilensky E M, Grady M S et al.. Cerebrospinal fluid pressure monitoring after repair of cerebrospinal fluid leaks.  Otolaryngol Head Neck Surg. 2004;  130(4) 443-448
  • 11 Schlosser R J, Woodworth B A, Wilensky E M, Grady M S, Bolger W E. Spontaneous cerebrospinal fluid leaks: a variant of benign intracranial hypertension.  Ann Otol Rhinol Laryngol. 2006;  115(7) 495-500
  • 12 Gassner H G, Ponikau J U, Sherris D A, Kern E B. CSF rhinorrhea: 95 consecutive surgical cases with long term follow-up at the Mayo Clinic.  Am J Rhinol. 1999;  13(6) 439-447
  • 13 Lindstrom D R, Toohill R J, Loehrl T A, Smith T L. Management of cerebrospinal fluid rhinorrhea: the Medical College of Wisconsin experience.  Laryngoscope. 2004;  114(6) 969-974
  • 14 Schick B, Ibing R, Brors D, Draf W. Long-term study of endonasal duraplasty and review of the literature.  Ann Otol Rhinol Laryngol. 2001;  110(2) 142-147
  • 15 Corbett J J, Thompson H S. The rational management of idiopathic intracranial hypertension.  Arch Neurol. 1989;  46(10) 1049-1051
  • 16 Fishman R A, Raichle M E. Pathophysiology of pseudotumor.  Ann Neurol. 1979;  5(5) 496-497
  • 17 Raichle M E, Grubb Jr R L, Phelps M E, Gado M H, Caronna J J. Cerebral hemodynamics and metabolism in pseudotumor cerebri.  Ann Neurol. 1978;  4(2) 104-111
  • 18 Bateman G A. Vascular hydraulics associated with idiopathic and secondary intracranial hypertension.  AJNR Am J Neuroradiol. 2002;  23(7) 1180-1186
  • 19 Bateman G A. Association between arterial inflow and venous outflow in idiopathic and secondary intracranial hypertension.  J Clin Neurosci. 2006;  13(5) 550-556 , discussion 557
  • 20 Donnet A, Metellus P, Levrier O et al.. Endovascular treatment of idiopathic intracranial hypertension: clinical and radiologic outcome of 10 consecutive patients.  Neurology. 2008;  70(8) 641-647
  • 21 Karahalios D G, Rekate H L, Khayata M H, Apostolides P J. Elevated intracranial venous pressure as a universal mechanism in pseudotumor cerebri of varying etiologies.  Neurology. 1996;  46(1) 198-202
  • 22 Owler B K, Parker G, Halmagyi G M et al.. Pseudotumor cerebri syndrome: venous sinus obstruction and its treatment with stent placement.  J Neurosurg. 2003;  98(5) 1045-1055
  • 23 Owler B K, Parker G, Halmagyi G M et al.. Cranial venous outflow obstruction and pseudotumor cerebri syndrome.  Adv Tech Stand Neurosurg. 2005;  30 107-174
  • 24 Alperin N, Lee S H, Mazda M et al.. Evidence for the importance of extracranial venous flow in patients with idiopathic intracranial hypertension (IIH).  Acta Neurochir Suppl. 2005;  95 129-132
  • 25 Coulson J D, Pitlick P T, Miller D C et al.. Severe superior vena cava syndrome and hydrocephalus after the Mustard procedure: findings and a new surgical approach.  Circulation. 1984;  70(3 Pt 2) I47-I53
  • 26 Doepp F, Schreiber S J, Benndorf G et al.. Venous drainage patterns in a case of pseudotumor cerebri following unilateral radical neck dissection.  Acta Otolaryngol. 2003;  123(8) 994-997
  • 27 Haar F L, Miller C A. Hydrocephalus resulting from superior vena cava thrombosis in an infant: case report.  J Neurosurg. 1975;  42(5) 597-601
  • 28 Karmazyn B, Dagan O, Vidne B A, Horev G, Kornreich L. Neuroimaging findings in neonates and infants from superior vena cava obstruction after cardiac operation.  Pediatr Radiol. 2002;  32(11) 806-810
  • 29 Kawamata M, Omote K, Sumita S, Iwasaki H, Namiki A. Epidural pressure in a patient with superior vena cava syndrome.  Can J Anaesth. 1996;  43(12) 1277
  • 30 McLaughlin J F, Loeser J D, Roberts T S. Acquired hydrocephalus associated with superior vena cava syndrome in infants.  Childs Nerv Syst. 1997;  13(2) 59-63
  • 31 Kollar C D, Johnston I H, Sholler G F. Communicating hydrocephalus secondary to a cardiac tumour compressing the superior vena cava.  Childs Nerv Syst. 2001;  17(3) 117-120
  • 32 Davson H, Domer F R, Hollingsworth J R. The mechanism of drainage of the cerebrospinal fluid.  Brain. 1973;  96(2) 329-336
  • 33 Johnston I, Hawke S, Halmagyi M, Teo C. The pseudotumor syndrome: disorders of cerebrospinal fluid circulation causing intracranial hypertension without ventriculomegaly.  Arch Neurol. 1991;  48(7) 740-747
  • 34 Malm J, Kristensen B, Markgren P, Ekstedt J. CSF hydrodynamics in idiopathic intracranial hypertension: a long-term study.  Neurology. 1992;  42(4) 851-858
  • 35 de Vries W A, Balm A J, Tiwari R M. Intracranial hypertension following neck dissection.  J Laryngol Otol. 1986;  100(12) 1427-1431

Michael J Link M.D. 

Department of Neurologic Surgery and Otorhinolaryngology

200 First Street SW, Rochester, MN 55905

Email: link.michael@mayo.edu

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