Clinical characteristics
A total of 372 pediatric patients were treated for obstructive hydrocephalus at our department from January 2018 to September 2019, and their conditions were all caused by malignant midline intracranial tumors. There was an obvious preponderance of boys (65.1%; Table 1). The mean age at the first surgery was 7.6±4.1 years. The midline tumors originated from three locations, including suprasellar (9.7%), pineal region (26.1%), and the fourth ventricle (64.2%). Moreover, the most common pathology was medulloblastoma (36.0%), followed by germ cell tumor (18.8%), glioma (18.0%), other types of tumors (17.5%), and anaplastic ependymoma (9.7%).
Furthermore, the sex ratio (0.7, 4.4, 1.6, respectively, p<0.001; Table 2), as well as the mean age at first surgery (7.0±4.3, 9.4±4.1, 7.0±3.8, respectively, p<0.001), was significantly different among the three locations of suprasellar, pineal region and the fourth ventricles. The main pathology in suprasellar was glioma (66.7%), whereas the most common pathology in the pineal region was germ cell tumors (63.9%), and the fourth ventricle showed a predominance for medulloblastoma (56.1%), which led to significant differences in pathology among the three locations (p<0.001).
Among the 372 patients, 117 underwent direct excision after diagnosis (31.4%; Table 3), 40 underwent ETV preoperatively (10.8%), and 215 underwent VPS placement (57.8%). After tumor resection, 3 children underwent ETV (1 tumor in suprasellar (5.6%), 2 in the fourth ventricle (0.8%)), and 31 underwent VPS placement (6 tumors in the suprasellar are (16.7%), 9 in the pineal region (9.3%), 16 in the fourth ventricle (6.7%)) due to the recurrence of hydrocephalus.
Outcomes of initial VPS placement or ETV
VPS placement was performed as the first-line treatment in 215 patients with obstructive hydrocephalus. In our analysis, sex and age at the first surgery were significantly different among the three locations (p = 0.021 and 0.010, respectively; Table 4). The outcomes of these 215 patients were optimistic; only 4 children experienced recurrence of hydrocephalus because of shunt blockage (2 tumors in the suprasellar and 1 in the fourth ventricle underwent VPS placement again after resection, and 1 from the fourth ventricle underwent ETV). The effectiveness of preoperative VPS placement was 98.1% (211/215), and the mean duration of relapse was 63.5 ± 15.7 days.
Forty children underwent ETV before tumor resection; sex and age at the first surgery were not significantly different among the locations (p = 0.069 and 0.078, respectively; Table 5). Hydrocephalus recurred in 3 patients with germ cell tumors and 1 with anaplastic ependymoma, so VPS placement was performed after tumor resection (3 from the pineal region and 1 from the fourth ventricle), so the effectiveness of preoperative ETV was 90.0% (36/40), and the mean duration of relapse was 53.8±44.9 days. Therefore, the effectiveness of preoperative VPS placement and ETV were both over 90%, but there was a significant difference between preoperative VPS placement followed by resection and postoperative VPS placement and preoperative ETV followed by resection and postoperative VPS placement (p=0.013).
Outcomes of direct resection
Furthermore, 117 patients underwent direct resection after diagnosis instead of VPS placement or ETV. The sex ratio was significantly different among the three locations (p = 0.003; Table 6), while age at the first surgery was not different (p = 0.155). According to our statistics, 20.5% (24/117) of patients (seven gliomas, seven medulloblastomas, four germ cell tumors, four anaplastic ependymomas, and two other malignant tumors) underwent VPS placement after tumor resection because of recurrence of hydrocephalus. Interestingly, two (1.7%) of these patients underwent ETV after the hydrocephalus recurred, but the symptoms were not relieved, and they finally underwent VPS placement to relieve their condition. The mean duration of relapse was 125.0 ± 170.8 days, the maximum and minimum durations were 710 and 7 days, respectively, and the median was 42 days. It is worth emphasizing that 22 of the 24 patients suspended their treatment due to sudden hydrocephalus relapse during chemoradiotherapy.
TABLE 1. Summary of the clinical characteristics of 372 pediatric patients with an initial presentation of hydrocephalus caused by malignant midline intracranial tumors
Clinical characteristics
|
Patients n (%)
|
Gender
|
|
Male
|
242 (65.1)
|
Female
|
130 (34.9)
|
Age (years)
|
7.6 ± 4.1
|
Locations of tumor
|
|
Suprasellar
|
36 (9.7)
|
Pineal region
|
97 (26.1)
|
Fourth ventricle
|
239 (64.2)
|
Pathology
|
|
Glioma
|
67 (18.0)
|
Germ cell tumor
|
70 (18.8)
|
Medulloblastoma
|
134 (36.0)
|
Anaplastic ependymoma
|
39 (9.7)
|
Others
|
65 (17.5)
|
TABLE 2. Clinical characteristics of 372 pediatric patients with hydrocephalus caused by malignant midline intracranial tumors in 3 different locations
|
Location of tumors (n)
|
|
Clinical characteristics
|
Suprasellar (36)
|
Pineal region (97)
|
Fourth ventricle (239)
|
p
|
Gender
|
|
|
|
<0.001
|
Male
|
15 (41.7)
|
79 (81.4)
|
148 (61.9)
|
|
Female
|
21 (58.3)
|
18 (18.6)
|
91 (38.1)
|
|
Age (years)
|
7.0 ± 4.3
|
9.4 ± 4.1
|
7.0 ± 3.8
|
<0.001
|
Pathology
|
|
|
|
<0.001
|
Glioma
|
24 (66.7)
|
6 (6.2)
|
37 (15.5)
|
|
Germ cell tumor
|
7 (19.4)
|
62 (63.9)
|
1(0.4)
|
|
Medulloblastoma
|
0(0.0)
|
0(0.0)
|
134 (56.1)
|
|
Anaplastic ependymoma
|
0(0.0)
|
2 (2.1)
|
34 (14.2)
|
|
Others
|
5 (13.9)
|
27 (27.8)
|
33 (13.8)
|
|
TABLE 3. Treatment for 372 pediatric patients with hydrocephalus caused by tumors located in 3 different regions
Treatments
|
Location of tumors (n)
|
Suprasellar
(36)
|
Pineal region
(97)
|
Fourth ventricle
(239)
|
Direct resection(117)
|
14(36.1)
|
24 (24.7)
|
79 (33.1)
|
Preop. ETV(40)
|
1 (2.8)
|
32 (33.0)
|
7 (2.9)
|
Preop. VPS(215)
|
21(61.1)
|
41 (42.3)
|
153 (64.0)
|
Postop. ETV(3)
|
1(5.6)
|
0 (0.0)
|
2 (0.8)
|
Postop. VPS(31)
|
6 (16.7)
|
9 (9.3)
|
16 (6.7)
|
TABLE 4. Outcomes of 215 pediatric patients with hydrocephalus who underwent VPS placement preoperatively
Clinical characteristics
& Outcomes
|
Location of tumors (n)
|
p
|
Suprasellar (21)
|
Pineal region (41)
|
Fourth ventricle (153)
|
Gender (M : F)
|
11:10
|
34:7
|
94:59
|
0.021
|
Ages (years)
|
7.5 ± 4.5
|
9.2 ± 4.2
|
7.1 ± 3.9
|
0.010
|
Postop. VPS
|
2 (9.5)
|
0 (0.0)
|
1 (0.7)
|
0.042
|
Postop. ETV
|
0 (0.0)
|
0 (0.0)
|
1 (0.7)
|
-
|
TABLE 5. Outcomes of 40 pediatric patients with hydrocephalus who underwent ETV preoperatively
Clinical characteristics
& Outcomes
|
Location of tumors (n)
|
p
|
Suprasellar
(1)
|
Pineal region
(32)
|
Fourth ventricle
(7)
|
Gender (M : F)
|
1:0
|
27:5
|
3:4
|
0.069
|
Ages (years)
|
15.0
|
10.3 ± 4.2
|
7.0± 3.4
|
0.078
|
Postop. VPS
|
0 (0.0)
|
3(9.4)
|
1 (14.3)
|
0.838
|
Postop. ETV
|
0 (0.0)
|
0 (0.0)
|
0 (0.0)
|
-
|
TABLE 6. Outcomes of 117 pediatric patients with hydrocephalus who did not undergo with VPS placement or ETV preoperatively
Clinical characteristics
& Outcomes
|
Location of tumors (n)
|
p
|
Suprasellar
(14)
|
Pineal region
(24)
|
Fourth ventricle
(79)
|
Gender (M : F)
|
3:11
|
18:6
|
51:28
|
0.003
|
Ages (years)
|
6.3 ± 3.2
|
8.4 ± 3.8
|
6.9 ± 3.8
|
0.155
|
Postop. VPS
|
4 (28.6)
|
6 (25.0)
|
14 (17.7)
|
0.540
|
Postop. ETV
|
1 (7.1)
|
0 (0.0)
|
1 (1.3)
|
-
|