1. CSF Leak Presented by: Malak Gazzaz

2. History 53 year old, Saudi, female
Known case of HTN, and hypothyroidism
Complaining of watery, colorless, runny nose from the right nostril for the past 9 years, on and off, increasing on bending downwards
Decrease sense of smell
Decrease vision
No fever or neck rigidity
No hx of trauma
She has previous hx of meningitis treated with IV antibiotics ( cefipim and vanco for 14 days)

3. History She was diagnosed as a case of CSF leak 4 years back
She was also diagnosed as a case of empty sella
She refused the repair previously b/c she was only offered a transcranial incision as an option for repair
The following diagnostic procedures can be performed if there is a suspected CSF leak (clinical sign: straw-colored or clear nasal drainage):
Tilt test with positive halo sign (as illustrated)
CT scan with thin coronal cuts (0.5 mm) of the cribriform plate
Comparison of the concentration of glucose between fluid and patient’s serum
Laboratory analysis for beta-transferrin
Application of fluorescent dyes and direct visualization of the leak via transnasal endoscopy. (Note: in many countries the use of intrathecal dyes is not approved and, therefore, special permission from the patient may be necessary.)

4. P/E
Obese
Endoscopy:
Nasal mass in right nostril

5. Investigations
CT cisternography was done by injecting 7ml of intrathecal contrast via LP

6. Investigations
Ant skull base , post table of frontal sinus, difficult area to be reached endoscopically but easy tnascranially, free sphenoid sinus,
CT showed anterior floor skull base defect and confirmed the leakage site at the roof of the right frontal sinus.

7. Investigations
" Bony  defect  seen  in  the  right  side  of  the  cribriform  plate  and  planum  sphenoidal.  The  defect  measured  about  9.7  x  8.6  mm  and  the  injected  intra-thecal  CM  is  seen  passing  through  it  into  the  right  frontal  sinus  and  recess  and  right  ethmoidal  air  cells.
"Opacification  (mostly  by  retained  secretions)  of  the  right  frontal  sinus,  right  frontal  recess,  right  anterior  ethmoidal  air  cells  and  majority  of  the  right  maxillary  sinus  with  upper  part  of  the  right  nasal  cleft. CONCLUSION: Known  case  of  right  sided  CSF  rhinorrhoea  with  right  planum  sphenoidal  &  cribriform  plate  of  ethmoid  bone  defect.

8. Empty Sella Syndrome
Empty sella turcica syndrome

9. Endoscopic transnasal repair of CSF leak
Multidisciplinary approach (ENT/Neurosurgery)
Repaired by 3 layers:
Fascia lata
Septal Cartilage
Nasal mucosa
Right fronto-ethmoid CSF leak on the posterior table of the frontal sinus

10. Endoscopic transnasal repair of CSF leak
Frontal Sinus
Defect

11. Endoscopic transnasal repair of CSF leak
Fascia lata

12. Endoscopic transnasal repair of CSF leak
Methylene Blue on nasal mucosa

13. Endoscopic transnasal repair of CSF leakOR
Nasal endoscopic exam reveling nasal mass in middle meatus
Using the microdebrider or shaver , removal of the mass is achieved
Maluble suction cautery was used to cauterize mass up to its origin at the skull base
Using a 70 scope we can visualize the defect at the skull base and dura pulsating
Elevation of dura is achieved using frontal sinus seeker and freshening of the edges is done
Septal cartilage is used as underlay graft on top of the fascia lata, then the nasal mucosa is put on top
Triple layer repair was done successfully with bio glue on top

14. Endoscopic transnasal repair of CSF leak

15. Hospital course
She was transferred to ICU to be closely monitored

16. Hospital course 1 day later, pt was transferred to ward
Pt was complaining of severe headache ?some fluid oozing, ?CSF
Lumbar drain was inserted and pain control medications were administered
Pt was also taking cefuroxime, nasal saline and fucidine ointment

17. Hospital course
Pt developed seizure POD 4, induced by cerebral edema and treated with phenytoin

18. CT post op

19. CT post op

20. CT
Site of repair
" Clinical  Data: " 4  day  post  endoscopic  transnasal  repair  of  csf  leak. " Patient  developed  seizure    with    epitasis.  To  role  out    bleeding.
Compared  to  the  previous  C.  T.  scan    dated  on  21  December  2011,  there  is  interval  development  of    right  frontal  hypodense  lesion  about  3cm  diameter  involving  the  right  frontal  lobe  medial  and  inferior  and  consistent  with    contusion/  infarction.  This  lesion  is  adjacent  to  irregular  hyperdensity  representing    operative  material  /  minimal  hemorrhage.  No  other  significant  intracranial  collection  or  hemorrhage  seen.  Midline  structure  are  in  situ.    "   hyperdensity  at  nasal  fossa  and  left  maxillary    sinus  consistent  with  residual  blood.    Air  fluid  level  noted  also  in  the  sphenoidal  sinus. 

21. Hospital course
Upon serial clinical and radiological evaluations , pt has improved with no CSF leak, no seizures, and afebrile
Lumbar drain was removed 3 days later
Pt started to ambulate without deficit

22. Hospital course
She was discharged 2 weeks post op with nasal irrigation by NS 30cc BID for 2 wks

23. MASNOT Questionnaire 0 = absent 1 = very mild 2 = mild 3 = moderate
4 = severe
5= very severe
Modified Arabic Sino nasal outcome test

24. MASNOT Questionnaire Pre op 2 wks post op 5 wks post op
Need to evacuate nostril from nasal secretions 3 2
sneezing 1
Runny nose 5
Nasal congestion or abstructoin
Loss of taste or smell
Modified Arabic Sino nasal outcome test

25. MASNOT Questionnaire 3 2 1 4 Pre op 2 wks post op 5 wks post op cough
itchiness
Post nasal drip
Ear obstruction
Pain/ facial headache 4
Inability to sleep deeply

26. MASNOT Questionnaire 3 1 5 Pre op 2 wks post op 5 wks post op
Fatigue upon waking up in the morning 4 3
Generalized fatigue
Double vision 2 1
Decrease visual acuty 5
Eye protrusion
modi

27. 5 wks post op

28. Transnasal Endoscopic Repair of Cerebrospinal Fluid Rhinorrhea: A Meta-Analysis
Hassan M. Hegazy MD
Ricardo L. Carrau MD
Carl H. Snyderman MD
Amin Kassam MD
Julie Zweig MD

29. Abstract

30. Objectives/Hypothesis
Trauma and surgery are the most common causes of (CSF) rhinorrhea.
Surgical repair is recommended for patients with:
CSF leaks that do not respond to conservative measures
traumatic CSF leaks that require transcranial surgery for associated brain injuries
iatrogenic defects that are discovered intraoperatively

31. The purpose of the study
To ascertain the outcome after transnasal endoscopic repair of CSF leaks and to identify factors regarding the patient, CSF fistula, and treatment that may influence the results of the repair.

32. Methods
Meta-analysis of all studies published between 1990 and 1999 that reported a minimum of five patients with CSF fistulae that were repaired using an endoscopic approach.
Data analysis included type of graft and technique used during the repair, surgical complications, the use of packing, and the use of lumbar drains and antibiotics.

33. Results
Endoscopic repair of CSF leaks was successful in 90% (259/289) of the cases after a first attempt.
Seventeen of 30 persistent leaks (52%) were closed after a second attempt. Thus ultimately 97% (276/289) of the leaks were repaired using an endoscopic approach.
The success rate of repairs using any of the reported techniques and materials was high and not statistically different.
The incidence of major complications such a meningitis, subdural hematoma, and intracranial abscess was less than 1% for each complication.

34. Surgical Repair of Cerebrospinal Fluid Leaks
The review and meta-analysis suggest that the choice of the surgical approach and the grafting materials used during the endoscopic or endonasal closure of CSF fistulae depends on the availability of the material and on the experience and familiarity of the surgeon with various techniques, and that their use does not seem to alter the outcome.

35. Adjunctive techniques
Nasal packing
Gel foam or Gel film
Fibrin glue
Perioperative antibiotic prophylaxis
Lumbar spinal drain
Nasal packing is commonly advocated to support the graft in place
Gelfoam or Gelfilm are frequently used to separate the graft from the packing material, to prevent avulsion of the graft or flap during its removal. Whether to use and how long to keep the postoperative packing are based on the surgeon's experience, although most authors recommend removing the packing 3 to 5 days after the surgery.
. Fibrin glue may enhance the adhesion of the graft
any of these techniques was not significantly different from each other.
Perioperative prophylactic antibiotics contribute to the low incidence of meningitis following skull base surgery 23 and their use during the repair of CSF leak, regardless of the approach,
A lumbar spinal drain is advocated by many authors to reduce the CSF pressure and the flow of leakage in cases of large fistulae. Most of the authors who use lumbar spinal drains after surgery recommend keeping the drain for 24 to 120 hours, to reduce the CSF pressure, preserve the position of the graft, and facilitate the process of adhesion.

36. Recommendation
The use of lumbar spinal drain for pts presenting with idiopathic and post traumatic fistulae that are highly associated with hydrochephalus for recurrent or persistent leaks and for those associated with meningoceles or large skull base defects is recommended

37. Complications of repair
Meningitis
Chronic headache
Pneumocephalus
Intracranial hematoma
Frontal lobe abscess
Anosmia6

38. Conclusion
The endoscopic approach is highly effective and is associated with low morbidity.
The literature supports the endoscopic approach using a variety of techniques and materials for the repair of CSF leaks.

39. Spontaneous cerebrospinal fluid leaks
Woodworth
Bradford Aa
Palmer
James Nb

40. Abstract 

41. Purpose of review
CSF leaks that occur spontaneously are challenging to manage clinically owing to frequent recurrences following attempted surgical closure.
Understanding the underlying pathophysiology allowed the recognition that the vast majority of these patients demonstrate clinical symptoms and radiographic signs of elevated ICP.
Individuals with this disorder also arise from a distinct demographic group. Increased knowledge of the characteristics of this patient population will provide increased success rates in the management of this clinical entity.

42. Recent findings
Current literature indicates that control of intracranial hypertension, coupled with endoscopic repair, will improve success rates comparable with other etiologies.
Improvement in preoperative identification of radiographic signs of intracranial hypertension (i.e. empty sella), operative technique, and postoperative management of elevated intracranial pressure are also reviewed. 

43. Benign Intracranial Hypertension
Elevated ICP frequently manifests itself in the syndrome of benign intracranial hypertension (BIH), aka pseudotumor cerebri.
Symptoms include:
pulsatile tinnitus
balance problems
Headache
visual disturbances.
Because this has recently been identified as the underlying cause in the majority of individuals in this category, the term spontaneous, rather than idiopathic, should be used in the presence of intracranial hypertension
on MRI scanning that has resulted from mechanical compression of the pituitary gland in the area of the sella turcica. the sella has the radiographic appearance of an absent pituitary gland due to filling with CSF (Fig. 1).
Normal CSF pressure is 5–15 cmH2O,

44. Benign Intracranial Hypertension
Many of these patients have total or partial empty sella syndrome (ESS)
Other radiological findings associated with elevated ICP include:
abnormalities of the optic nerve sheath complex,
encephaloceles,
arachnoid pits
dural ectasia 7,8,9,10,11

45. Benign Intracranial Hypertension
Pts with BIH have elevated readings (typically over 25 cmH2O) on lumbar tap opening pressures
In terms of demographics, the majority of patients who develop the diagnosis of BIH are young to middle-aged obese women12. The association of obesity with BIH has been reported in many studies10,13,14.

46. Preoperative Evaluation
Consists of:
History
physical examination
nasal endoscopic examination
radiographic imaging
At the least, coronal and axial CT scans of the sinuses should be obtained before surgery;

47. Recommendation
Computer-aided or image-guided surgical navigation CT scans and MRI studies are recommended.
MRI can enhance the diagnosis of elevated ICP, as it shows evidence of totally or partially empty sella up to 85% of the time15.

48. Management of elevated ICP
Acetazolamide
Ventriculoperitoneal shunting
If CSF pressure is elevated over 15cmH2O, acetazolamide is recommended to decrease ICP. Acetazolamide is a carbonic anhydrase inhibitor diuretic that decreases CSF production.
 In patients with significantly elevated ICP (generally >35 cmH2O at baseline) or an inadequate response to medical therapy with diuretics (generally <10 cmH2O decrease), we recommend neurosurgical consultation for permanent ventriculoperitoneal shunting.

49. Recommendation
Because the underlying cause of elevated CSF pressure (either obesity or decreased arachnoid granulations) is likely to remain unchanged over time, we generally recommend lifelong use of the diuretic.

50. Outcomes
Treatment of the underlying intracranial hypertension, whether through medical or surgical means, was critical for success in the repair of these defects
Significant weight loss appears to be required for this to become an effective treatment16.

51. Conclusion
Evidence indicates that treatment of underlying intracranial hypertension in spontaneous CSF leaks coupled with endoscopic repair can provide success rates (95%) approaching those of other etiologies

52. Thank you!

53. References
1 Stankiewicz JA. Cerebrospinal fluid fistula and endoscopic sinus surgery. Laryngoscope 1991; 101:250–256.
2 Zweig JL, Carrau RL, Celin SE, et al. Endoscopic repair of CSF leaks to the sinonasal tract: predictors of success. Otolaryngol Head Neck Surg 2000 (in press).
3 Casiano RR, Jassir D. Endoscopic cerebrospinal fluid rhinorrhea repair: is a lumbar drain necessary?. Otolaryngol Head Neck Surg 1999; 121:745–750.

54. References
4 Lanza DC, O'Brien DA, Kennedy DW. Endoscopic repair of cerebrospinal fluid fistulae and encephaloceles. Laryngoscope 1996; 106:1119–1125.
5 Marks SC. Middle turbinate graft for repair of cerebral spinal fluid leaks. Am J Rhinol 1998; 12:417–419.

55. References
6 Burns JA, Dodson EE, Gross CW. Transnasal endoscopic repair of cranionasal fistulae: a refined technique with long-term follow-up. Laryngoscope 1996; 106:1080–1083.

56. References
7 Mattox DE, Kennedy DW. Endoscopic management of cerebrospinal fluid leaks and cephaloceles. Laryngoscope 1990; 100:857–862
8 Hubbard JL, McDonald TJ, Pearson BW, et al. Spontaneous cerebrospinal fluid rhinorrhea: evolving concepts in diagnosis and surgical management based on the Mayo Clinic experience from 1970 through Neurosurgery 1985; 16:314–321.
9 Schlosser RJ, Bolger WE. Significance of empty sella in cerebrospinal fluid leaks. Otolaryngol Head Neck Surg 2003; 128:32–38.

57. References
10 Corbett JJ, Thompson HS. The rational management of idiopathic intracranial hypertension. Arch Neurol 1989; 46:1049–1051.
11 Silver RI, Moonis G, Schlosser RJ, et al. Radiographic signs of elevated intracranial pressure in idiopathic cerebrospinal fluid leaks: a possible presentation of idiopathic intracranial hypertension. Am J Rhinol 2007; 21:257–261

58. References
12 Gass A, Barker GJ, Riordan-Eva P, et al. MRI of the optic nerve in benign intracranial hypertension. Neuroradiology 1996; 38:769–773. 
13 Radhakrishnan K, Ahlskog JE, Garrity JA, et al. Idiopathic intracranial hypertension. Mayo Clin Proc 1994; 69:169–180

59. References
14 Wilson DH, Gardner WJ. Benign intracranial hypertension with particular reference to its occurrence in fat young women. Can Med Assoc J 1966; 95:102–105.
15 Woodworth BA, Prince A, Chiu AG, et al. Spontaneous CSF leaks: a paradigm for definitive repair and management of intracranial hypertension. Otolaryngol Head Neck Surg 2008; 138:715–720

60. References
16 Radhakrishnan K, Thacker AK, Bohlaga NH, et al. Epidemiology of idiopathic intracranial hypertension: a prospective and case–control study. J Neurol Sci 1993; 116:18–28