Patient demographics
Eighty-eight patients had their shunts removed or ligated, fifty-seven of which (65%) underwent simultaneous ETV. There are 49 males with an average age of 20 years at shunt removal/ligation, and an average duration of shunting prior to removal/ligation of 11.6 years. The reasons for original shunting include: congenital hydrocephalus in 20 (23%), post-hemorrhagic hydrocephalus (PHH) of prematurity in 14 (16%), intracranial cyst in 8 (9%), aqueductal stenosis in 10 (11%), brain tumor in 8 (9%), infantile subdural hematomas in 8 (9%), myelomeningocele in 7 (8%), post-traumatic hydrocephalus in 7 (8%) and post-infectious hydrocephalus in 6 (7%). 44 (50%) patients had no history of shunt revision surgery (Table 1). Clinical and imaging presentations that led to a decision to attempt shunt independence include symptomatic shunt overdrainage in 24 (27%); proximal obstruction in 16 (18%); shunt infection in 14 (16%%); distal obstruction, disconnection, or painful shunt track in 16 (18%); aqueductal stenosis in 10 (11%); and clinical suspicion that the shunt is no longer required, namely subdural shunts in 8 (9%). Indications for ETV was based on etiology of hydrocephalus. We did not think ETV was clinically indicated in patients who had successful surgical treatment of colloid cyst (2), complex intraventricular cyst (3), choroid plexus papilloma (2), posterior fossa cyst (2), or Chiari I malformation (2); and in one case, a patient whose shunt was placed late in life for questionable high ICP. Retrospectively, the median ETVSS in the ETV group was 70 (average 72). Most of the predictor variables of interest were continuous measures: age, shunt duration, number of revisions or infection, and ETVSS. Cox proportional hazards estimates for univariate continuous predictors are shown in Table 2. Indications for surgery to re-activate or re-implant a shunt included symptoms of elevated ICP, CSF leak, high ICPs measured through an external ventricular drain (EVD), shunt tap, and ICP monitoring.
Table 1
Patient demographics. AS: aqueductal stenosis; ETV: Endoscopic Third Ventriculostomy; ETVSS: Endoscopic Third Ventriculostomy Success Score; HC: hydrocephalus; MM: Myelomeningocele; PHH: Post-Hemorrhagic Hydrocephalus.
Demographics
|
No ETV = 32
|
ETV = 56
|
Combined = 88
|
n (%)
|
n (%)
|
n (%)
|
Age at shunt placement (years)
|
|
|
|
0–1
|
18 (56.2%)
|
31 (55.4%)
|
49 (55.7%)
|
1 + − 10
|
7 (21.9%)
|
8 (14.3%)
|
15 17%)
|
>10
|
7 (21.9%)
|
17 (30.4%)
|
24 (27.3%)
|
Age at shunt ligation or removal (years)
|
|
|
|
0–1
|
1 (3.1%)
|
3 (5.4%)
|
4 (4.5%)
|
1–10
|
14 (43.8%)
|
9 (16.1%)
|
23 26.1%)
|
>10
|
17 (53.1%)
|
44 (78.6%)
|
61 (69.3%)
|
Time to shunt ligation or removal (years)
|
|
|
|
0–5
|
14 (43.8%)
|
18 (32.1%)
|
32 (36.4%)
|
5 + − 10
|
8 (25%)
|
6 (10.7%)
|
14 (15.9%)
|
10 + − 20
|
6 (18.8%)
|
19 (33.9%)
|
25 (28.4%)
|
> 20
|
4 (12.5%)
|
13 (23.2%)
|
17 (19.3%)
|
Number of shunt revisions
|
|
|
|
0
|
19 (59.4%)
|
26 (46.4%)
|
45 (51.1%)
|
1
|
7 (21.9%)
|
7 (12.5%)
|
14 (15.9%)
|
2
|
2 (6.2%)
|
9 (16.1%)
|
11 (12.5%)
|
3–7
|
4 (12.5%)
|
14 (25%)
|
18 (20.5%)
|
Number of shunt infections
|
|
|
|
0
|
28 (87.5%)
|
45 (80.4%)
|
73 (83%)
|
1
|
4 (12.5%)
|
9 (16.1%)
|
13 (14.8%)
|
2
|
0 (0%)
|
2 (3.6%)
|
2 (2.3%)
|
Etiology
|
|
|
|
Congenital HC
|
7 (21.9%)
|
13 (23.2%)
|
20 (22.7%)
|
Post-traumatic
|
4 (12.5%)
|
3 (5.4%)
|
7 (8%)
|
AS
|
0 (0%)
|
10 (17.9%)
|
10 (11.4%)
|
Cyst
|
4 (12.5%)
|
4 (7.1%)
|
8 (9.1%)
|
Subdural
|
8 (25%)
|
0 (0%)
|
8 (9.1%)
|
Post-infectious
|
3 (9.4%)
|
3 (5.4%)
|
6 (6.8%)
|
Tumor
|
2 (6.2%)
|
6 (10.7%)
|
8 (9.1%)
|
PHH
|
3 (9.4%)
|
11 (19.6%)
|
14 (15.9%)
|
MM
|
1 (3.1%)
|
6 (10.7%)
|
7 (8%)
|
ETVSS
|
|
|
|
30–60
|
0 (0%)
|
7 (12.5%)
|
7 (8%)
|
70
|
0 (0%)
|
20 (35.7%)
|
20 (22.7%)
|
80
|
0 (0%)
|
29 (51.8%)
|
29 (33%)
|
NA
|
32 (100%)
|
0 (0%)
|
32 (36.4%)
|
Table 2
Cox Proportional Hazard univariate predictor model. Factors that predict shunt independence include (1) age at shunt placement (p = 0.032), (2) number of shunt revision (p = 0.01), and (3) ETVSS (p = 0.017). ETV: Endoscopic Third Ventriculostomy; ETVSS: Endoscopic Third Ventriculostomy Success Score; HR: hazards ratio; LCL: Lower Confidence Limit; UCL: Upper Confidence Limit; Pr: Probability
|
HR
|
LCL
|
UCL
|
Pr(>|z|)
|
Age at shunt placement (years)
|
0.969
|
0.941
|
1
|
0.032
|
Age at shunt ligation/removal (years)
|
0.991
|
0.971
|
1.01
|
0.37
|
Time to shunt ligation/removal
|
1.023
|
0.997
|
1.05
|
0.086
|
Number of shunt revisions
|
1.21
|
1.05
|
1.41
|
0.01
|
Number of shunt infections
|
1.360
|
0.763
|
2.42
|
0.3
|
ETVSS of ETV patients, n = 58
|
0.973
|
0.951
|
1
|
0.017
|
Success of shunt removal/ligation
Median time-to-event, i.e., when half of patients failed shunt ligation/removal and had their shunts activated/reimplanted, is 4 years (Fig. 1, Table 3). The 6-month and 4-year shunt-independent survival periods were estimated from the Kaplan-Meier curve. 56.8% and 50.1% of patients were shunt independent 6 months and 4 years after shunt removal/ligation, respectively (Table 4). The median shunt-independent duration, i.e., period of time when half of patients at risk had their shunt reactivated/reimplanted, is 4 years (95% CI= [11, NA]). Shunt-independent survival in patients whose shunt was placed before 4 months of age was 66.7% and 64.3% at 6 months and 4 years respectively, and in those whose shunt was placed at or after 4 months of age, it was 38.7% and 25.4% respectively. Shunt-independent survival in patients with zero shunt revisions was 64.4% at 6 months as well as 4 years, and in those with 1 or more shunt revisions, it was 48.8% and 34.7%, respectively (Table 3). Six-month shunt independence was achieved in 47% of the ETV group, and 73% of the No ETV group (Fig. 2). In patients with ETVSS < 70, the shunt-independent survival at 6 months and 4 years was 28.6% and 14.3%, respectively. In patients whose ETVSS was 70, the shunt-independent survival at 6 months and 4 years was 45% and 26.2% respectively. And in patients with an ETVSS 80, the shunt-independent survival at 6 months and 4 years was 58.6% and 54.7%, respectively (Table 3).
Table 3. 6-month and 4-year Percent Survival, [95% CI], Categorized Predictors. Following shunt removal/ligation, 56.8% and 50.1% of patients remained shunt independent at 6 months and 4 years, respectively. Median shunt independence (the time period in which half the patients at risk had a shunt reactivated/reimplanted) was approximately 4 years (95% CI= [11, NA]). Predictors of shunt independence are listed by category. AS: Aqueductal stenosis; CI: Confidence Interval; HC: hydrocephalus; MM: Myelomeningocele; PHH: Post-Hemorrhagic Hydrocephalus.; Number of failures: number of patients who failed to achieve shunt independence throughout the duration of the study.
|
|
n
|
Number of failures (n)
|
6-month survival
[95% CI]
|
4-year survival
[95% CI]
|
Median survival (years) [95% CI]
|
Logrank p
|
Etiology
|
All
|
88
|
47
|
56.8 [47.4,68.2]
|
50.1 [40.5,62]
|
4.06 [0.11,NA]
|
0.0043
|
Congenital HC
|
20
|
12
|
55 [37,81.8]
|
48.9 [30.9,77.4]
|
1.86 [0.06,NA]
|
Post-traumatic
|
7
|
4
|
42.9 [18.2,100]
|
42.9 [18.2,100]
|
0.22 [0.08,NA]
|
AS
|
10
|
3
|
70 [46.7,100]
|
70 [46.7,100]
|
NA [0.11,NA]
|
Cyst
|
8
|
4
|
62.5 [36.5,100]
|
50 [25,100]
|
0.63 [0.03,NA]
|
Subdural
|
8
|
0
|
100 [100,100]
|
100 [100,100]
|
NA [NA,NA]
|
Post-infectious
|
6
|
3
|
50 [22.5,100]
|
50 [22.5,100]
|
0.04 [0.02,NA]
|
Tumor
|
8
|
3
|
87.5 [67.3,100]
|
70 [42,100]
|
9.35 [3.03,NA]
|
PHH
|
14
|
12
|
28.6 [12.5,65.4]
|
21.4 [7.9,58.4]
|
0.06 [0.02,NA]
|
MM
|
7
|
6
|
28.6 [8.9,92.2]
|
14.3 [2.3,87.7]
|
0.06 [0.01,NA]
|
Age at Shunt Placement (months)
|
0-4
|
31
|
24
|
38.7 [24.9,60.3]
|
25.4 [13.8,46.7]
|
0.07 [0.03,2.98]
|
0.0003
|
>4
|
57
|
23
|
66.7 [55.5,80.1]
|
64.3 [52.8,78.3]
|
NA [8.9,NA]
|
Number of Shunt Revisions
|
0
|
45
|
18
|
64.4 [51.9,80.1]
|
64.4 [51.9,80.1]
|
NA [8.9,NA]
|
0.024
|
1-7
|
43
|
29
|
48.8 [36,66.3]
|
34.7 [22.5,53.5]
|
0.3 [0.07,NA]
|
ETVSS of ETV Patients, n=56
|
30-60
|
7
|
6
|
28.6 [8.9,92.2]
|
14.3 [2.3,87.7]
|
0.04 [0.02,NA]
|
0.015
|
70
|
20
|
16
|
45 [27.7,73.1]
|
26.2 [11.9,57.8]
|
0.06 [0.02,NA]
|
80
|
29
|
14
|
58.6 [43.2,79.6]
|
54.7 [39.2,76.4]
|
12.22 [0.11,NA]
|
Using Cox proportional hazards estimate, we identified factors that correlate with shunt independence after removal/ligation. These include age at shunt placement, number of shunt revisions, etiology, and ETVSS. Factors that did not independently correlate with success of shunt removal/ligation are duration of shunting prior to removal/ligation, number of shunt infections, and age at shunt removal/ligation (Table 2). A cox proportional hazards multiple regression analysis was completed on the continuous predictive variables identified in the univariate model. The number of shunt revisions is protective of shunt independence (P = 0.039) (Table 4).
Table 4
Cox Proportional Hazard multiple regression model. This analysis was completed on the continuous predictive variables identified in the univariate model in Table 2. HR: hazards ratio; LCL: Lower Confidence Limit; UCL: Upper Confidence Limit; Pr: Probability
|
HR
|
LCL
|
UCL
|
Pr(>|z|)
|
Age at shunt placement (years)
|
0.974
|
0.946
|
1.00
|
0.073
|
Number of shunt revisions
|
1.172
|
1.008
|
1.36
|
0.039
|
Table 5. Late ETV failures. Eight patients presented with late failure (beyond 6 months) after shunt ligation or removal, with an average of 6.8 years. Five of these were from the ETV group. Cyst-P: Cyst-Peritoneal shunt; ETV: ETV: Endoscopic Third Ventriculostomy; ETVSS: Endoscopic Third Ventriculostomy Success Score; ICP: intracranial pressure; VP: Ventricular-Peritoneal shunt; IVH: Intra-ventricular hemorrhage.
ID
|
Etiology
|
Shunt type
|
Age at shunt Independence (years)
|
Indication for shunt ligation/ removal
|
Surgery
|
Time to shunt reactivation/reimplantation (years)
|
Indication for shunt reactivation or reimplantation
|
1
|
Cyst
|
Cyst-P
|
48.8
|
Overdrainage
|
Shunt ligation
|
6.9
|
Headache recurrence requiring repetitive high-volume reservoir taps
|
2
|
Congenital
|
VP
|
26
|
Disconnection
|
Shunt removal
|
8.8
|
Headaches and progressive deterioration in mental status
|
3
|
Congenital
|
VP
|
17
|
Proximal Obstruction
|
ETV without shunt ligation/removal, as shunt was considered obstructed (dry shunt tap)
|
1.8
|
Shunt tap showing normal pressure and flow in the shunt.
|
4
|
Tumor
|
VP
|
14
|
Disconnection
|
ETV + shunt removal
|
9.3
|
Headaches requiring repetitive high-volume lumbar punctures
|
5
|
Tumor
|
VP
|
30.4
|
Proximal Obstruction
|
ETV + shunt removal
|
3
|
Headaches
|
6
|
IVH
|
VP
|
13.9
|
Proximal Obstruction
|
ETV + shunt removal
|
9
|
Headaches, memory issues requiring high-volume lumbar punctures
|
7
|
Congenital
|
VPleural
|
25.5
|
Shunt infection
|
Shunt removal
|
12.1
|
Headaches and presumed high ICP (shunted at a different institution)
|
|
IVH
|
VP
|
20.5
|
Shunt infection
|
ETV + shunt removal
|
4
|
Progressive headaches and ventricular enlargement
|
Age at shunt placement
The average ages at initial shunt placement are 10.2 years (median 2) and 5.6 years (median 0.25 years) in the groups that succeeded or failed to achieve shunt independence, respectively (p = 0.032). Maximal Log Rank estimated an optimal cut-point of 0.34 years for age at shunt placement (p = 0.0086), below which the success rate is very low (18.75%) (Fig. 3, Table 2).
Number of shunt revisions
While less shunt revisions correlates with success of shunt removal/ligation (HR = 1.2, p = 0.01), a history of zero shunt revisions is a statistically significant predictor of success (p = 0.048) (Fig. 4, Table 2).
ETVSS:
The average ETVSS is 72 (median 70) and 69 (median 70) in the groups that succeeded or failed to achieve shunt independence, respectively (Fig. 5). Higher ETVSS scores (cut-point of 70) are protective (HR = 0.98, p = 0.0103, Table 2). ETVSS was calculated retroactively in 47% of patients who underwent surgery before the ETVSS was first described in the literature.[8] ETV success is similar before (55%) and after (52%) ETVSS publication.
Original etiology/reason for shunt placement
A subdural shunt placed for chronic subdural hematoma in infancy was successfully removed/ligated in all 8 patients (100%). The etiology for shunt placement in chronic subdural collections was post traumatic (6), post-meningitis (1) and secondary to leukemia (1). Success of VP shunt removal/ligation at 6 months and 4 years, respectively, correlates with etiology of hydrocephalus, as follows: tumor (88% and 70%), aqueductal stenosis (70% and 70%), cyst (63% and 50%), infection (50% and 50%), trauma (43% and 43%), and PHH (29% and 21%) myelomeningocele (29% and 14%) (Table 3). Survival curves differ by etiology (p = 0.004 and Fig. 6).
Indication for shunt re-implantation
Failure of shunt ligation/removal was recognized via elevated ICP captured in 26 patients (67%) via a postoperative external ventricular drain EVD in 18, Codman parenchymal ICP monitoring in 2, shunt tap in 5 and lumbar puncture in 1; 4 failures (10%) were diagnosed after an incisional CSF leak, and the remaining 9 (23%) developed clinical symptoms such as headaches, balance problems, macrocephaly, papilledema, and behavioral problems.
Late failures
The average time to shunt reactivation/reimplantation was 18.4 days (0 days to 3 months). Twenty-five patients (64%) had their shunt reactivated/reimplanted in the same hospital admission, while the remaining patients were readmitted for surgery. Eight patients (9%) presented with late failures (> 6 months), with an average time to failure of 6.8 years (range 1.8 years to 12 years) in that group. Five of the 8 were in the ETV group (Table 5).
Complications
Operative complications occurred in 4 patients and include 2 intraventricular hemorrhages requiring temporary external drainage and 2 superficial surgical site infections treated with oral antibiotics. CSF leaks (reported above) occurred in the setting of failure of shunt ligation/removal.