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Aaron 2014
Aaron 2014
Aaron 2014
Otolaryngology
Head and Neck Surgery
patient and is either diagnosed incidentally on routine patients. Radiographic imaging assessment included image-
ophthalmologic examination or discovered during the guided surgical navigation CT scans in all cases and MRI
workup of a visual disturbance. In a recent study, papille- scans. Data regarding demographics, nature of presentation,
dema was found in 94.3% of patients with IIH at 1 center.5 location and size of defect, surgical approach, reconstructive
Importantly, it is estimated that anywhere between 10% and technique, and management of ICP were collected.
25% of patients with papilledema have permanent visual The control group was derived from 16 consecutive
loss despite therapeutic interventions. 6,7 patients diagnosed with IIH who had papilledema on fundo-
In the past 10 years, there has been an increased interest scopic exam and lumbar puncture to obtain opening pres-
in the association of IIH with spontaneous CSF leaks. sure measurements during the clinical workup for the
Studies have established a highly suggestive correlation disorder. Demographic data including sex, age, and BMI
between the 2 entities through clinical symptoms, radio- were also collected for comparison.
graphic signs, and direct intracranial pressure measure-
ments. A recent literature review published in 2013 found Ophthalmologic Examination
23 studies that correlated spontaneous CSF leaks with the All patients enrolled in the study took part in a complete
diagnosis of IIH.8 Most recently, Chaaban et al9 revealed ophthalmologic examination at the University of Alabama
that patients with spontaneous CSF leaks undergoing repair at Birminghams Callahan Eye Center. Participants were
had an opening intrathecal/intracranial pressure of 24.3 cm examined by a single neuroophthalmologist (MSV) who
H2O that significantly increased to 32.3 cm H2O once the specializes in the treatment of IIH. Focus was made primar-
CSF leak had been sealed.9 Imaging studies often can direct ily on the fundoscopic examination to determine if there
the source of the CSF leak along with evidence of IIH. MRI was any evidence of papilledema in both the spontaneous
evidence includes partially or totally empty sella, flattening CSF leak group and the control group.
of the posterior globes, stenosis of the transverse sinus, and
optic nerve distension along with encephaloceles that are Surgical Technique
associated with CSF leaks, though not specific to IIH.10,11 The technique for endoscopic management varied depend-
Evidence of changes on CT of patients with IIH with spon- ing on the site and size of the defect, presentation, and other
taneous CSF leaks includes erosions and defects of the skull factors but generally outlines those previously described.13-18
base with arachnoid pits and enlarged foramen ovales.11,12 Lumbar drains (LDs) or ventriculostomies were used in all
Despite the known relationships between IIH and papille- surgical patients.19 Fluorescein was also utilized according
dema and IIH and spontaneous CSF leaks, there are no to previously published protocols to localize defects, iden-
studies examining if there is any correlation between spon- tify multiple CSF leaks, and inspect for a watertight clo-
taneous CSF leaks and papilledema. Patients with sponta- sure at the conclusion of the case.20-22 A mixture of 0.1
neous CSF leaks infrequently complain of any visual mL of 10% fluorescein diluted in 10 mL of the patients
changes and are thus rarely evaluated by an ophthalmolo- CSF is slowly injected over 10 to 15 minutes. Surgical
gist. The objective of the current study is to correlate the exposure is characterized according to transethmoid tech-
presence of preoperative papilledema with opening intracra- nique (sphenoethmoidectomy) with additional exposures
nial pressure (ICP) in patients undergoing endoscopic repair (eg, transpterygoid, Draf IIB frontal sinusotomy) as neces-
of spontaneous CSF leaks and compare to a control group sary (Figures 1, 2). The transpterygoid approach is per-
of patients with IIH and papilledema. formed as previously described for all lateral sphenoid
recess CSF leaks.23
Materials and Methods
Intracranial Pressure Measurements
Subjects Lumbar punctures were performed in the lateral decubitus
Prospective evaluation and data collection of subjects was position with the spine leveled using a manometer to deter-
approved by the institutional review board at the University mine opening pressures. The control group had a lumbar
of Alabama at Birmingham and Callahan Eye Foundation. puncture performed by radiology, while the surgical cohort
All patients were treated by a single otolaryngologist underwent lumbar tap or ventriculostomy under anesthesia
(BAW) for CSF leaks of spontaneous etiology and enrolled just prior to the procedure. Surgical patients also had LDs
during a 1-year period (December 2012 to December 2013). or ventriculostomies placed for postoperative pressure moni-
CSF leaks were considered spontaneous when there was no toring. Although the use of LDs in CSF leak repair has
previous history of skull base fracture/trauma, tumor invol- recently been labeled controversial,17,21,24,25 we utilize
vement, or other obvious etiology. Preoperative evaluation drains when we feel the benefits far outweigh the risks.
of all patients consisted of a thorough history and physical Because spontaneous CSF leaks often have multiple defects,
examination (with nasal endoscopy), including inquiries the administration of intrathecal fluorescein is useful for
about previous history of head trauma, prior sinus or neuro- identifying other leaking sites that may not be readily appar-
logical surgery, congenital abnormalities, prior episodes of ent on preoperative imaging. LDs (or ventriculostomies) are
meningitis (or other intracranial events), and obesity. Body managed according to previously described protocols with
mass index (BMI) calculations were performed in all slight alterations.13 In general, LDs are opened at the time
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Aaron et al 1063
Figure 1. Triplanar CT image guidance and endoscopic view of a spontaneous encephalocele and cerebrospinal fluid leak from the right
planum sphenoidale.
Figure 2. The encephalocele is removed with bipolar coblation and the surrounding mucosa stripped (A) The defect is measured and (B)
repaired in multiple layers including a bone graft (C) to provide support against elevated intracranial pressure.
of graft placement, and the height of the collection chamber therapy with carbonic anhydrase inhibitors (generally \10
is adjusted to maintain drainage at approximately 10 cc/hr. cm H2O decrease), permanent ventriculoperitoneal (VP)
The drain is clamped on the morning of postoperative day shunting is recommended. If patients refuse the VP shunt,
2, and at least 6 hours is allowed to help equilibrate the they are kept on acetazolamide, and electrolytes are checked
patients CSF volume. A pressure transducer or manometer periodically to ensure no life-threatening abnormalities.
is connected to the lumber drain with the patient in the lat- Data were analyzed for statistical significance with paired
eral decubitus position zeroed at the spinal column. Normal and unpaired t tests.
CSF pressure is between 5 and 15 cm H2O in this position.
If pressure is elevated, oral acetazolamide (500 mg) is admi- Postoperative Management
nistered and read again at 4 to 6 hours to assess the effect Patients are instructed on movement techniques to avoid
of the medication. Acetazolamide is a carbonic anhydrase breath holding and Valsalva maneuvers. An antistaphylo-
inhibitor that decreases CSF production. In patients with coccal antibiotic is prescribed until the packing is removed
significantly elevated ICP (generally .35 cm H2O at base- at the first postoperative visit 9 to 13 days postoperatively.
line), multiple defects, or an inadequate response to medical A stool softener is prescribed for every patient, and light
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1064 OtolaryngologyHead and Neck Surgery 151(6)
activity is continued for 6 weeks after surgery. Patients are pressures were significantly higher than ICP measured in
seen anywhere from 1 to 4 weeks after the first visit. the spontaneous CSF leak cohort (36.2 6 11.7 cm H2O;
P \ .01). However, there was no statistical difference
Results between the 6- hour clamped CSF pressure of the sponta-
neous CSF leak group and the IIH patients with papille-
Over a 1-year period, 16 patients met the criteria of sponta-
dema, which were nearly identical.
neous CSF leaks and were included in the study. Patient
demographics and clinical data are presented in Table 1.
Fourteen (87.5%) of the patients were female (average
age = 52 years old, range, 33-67). Average BMI for patients
with a spontaneous CSF leak was 43 (range, 27-65) with
only 1 patient (BMI = 27) not meeting the definition of obe- Table 2. Idiopathic Intracranial Hypertension Patients.
sity (BMI .30). All patients in the surgical cohort did not Age Sex BMI OP Papilledema
have papilledema during the preoperative assessment. Two
patients had multiple leaks identified during the surgery. 25 F 52 38 Yes
Average defect size was 5.9 3 6.2 mm (length vs width). 32 F 30 49 Yes
The size and location of the individual CSF leaks as well as 23 F 50 60 Yes
specific reconstruction techniques are provided in Table 2. 28 F 21 59 Yes
Two patients required a ventriculostomy when lumbar punc- 25 F 35 35 Yes
tures were unable to be performed secondary to the depth of 30 F 32 21 Yes
adipose tissue in the lower back. Opening pressures were 47 F 35 37 Yes
27.4 6 7.7 cm H2O. The 6-hour postclamp average CSF 29 F 38 34 Yes
pressure taken on postoperative day 2 was 36.0 6 9.6 cm 26 F 41 32 Yes
H2O, which was a statistically significant increase (P \ 34 F 49 22 Yes
.0001). Eleven patients were placed on long-term acetazola- 46 F 22 44 Yes
mide, while 4 individuals received a VP shunt to control 30 F 28 33 Yes
CSF pressure. One patient died from a massive pulmonary 23 F 34 31 Yes
embolism on postoperative day 2. 29 F 35 32 Yes
The control cohort IIH patients with papilledema 43 F 45 25 Yes
included 16 participants. All subjects (100%) were females 54 F 30 27 Yes
with an average age of 33 years old (range, 23-54). Average
Abbreviations: BMI, body mass index; OP, opening pressure.
BMI for this group was 36.0 (range, 21-52). Opening
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