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AANS, 2014
B. C. Phillips et al.
In 10% of infants with MMC, hydrocephalus is apparent at birth, with patients having macrocrania and
massive ventriculomegaly.2 While these patients will
likely benefit from CSF shunting, there are no widely
applied criteria for CSF shunting in other patients with
MMC with less profound hydrocephalus. Previously reported indicators of the need for a shunt include level of
the lesion, clinical signs of elevated intracranial pressure
such as a tense or bulging fontanelle, bradycardia, sunsetting eyes, increasing head circumference, and increasing
ventricular size.6,14,16
The Management of Myelomeningocele Study
(MOMS) explored the shunt rate of patients with prenatal
versus postnatal MMC repair as one of its primary end
points. This study demonstrated a statistically significant
difference in CSF diversion, with the rate of shunting in
the fetal closure group (65%) lower than in the postnatal
closure group (92%).1 Another recent publication indicated a shunt rate of 52% from 1 institution using stringent
shunt criteria.6 This institution was not involved in the
MOMS trial and performed exclusively postnatal MMC
repair. Also, while the results of the MOMS trial are
compelling, the rate of shunt placement for the postnatal repair group was high compared with other published
results.6
The goal of this retrospective study was to determine
the shunt rate over 7 consecutive years for patients at our
institution and to determine whether there was a change
in the shunt rate over time. We also sought to identify the
criteria used to determine the requirement for shunting at
our institution and identify which factors were the most
predictive of the need for CSF shunting.
Methods
Study Population
168
Statistical Analyses
The statistical significance of all clinical and radiological variables was determined using the univariate
Cox proportional hazards model. Multivariate Cox proportional hazards analysis was also performed. In this
analysis, 3 models were used. Model 1 included only
clinical characteristics (gestational age, sex, apneic episodes, bradycardic episodes, fontanelle description, head
circumference at birth, and head growth rate). Model 2
included the clinical characteristics from Model 1 in addition to the radiological parameters obtained from head
J Neurosurg: Pediatrics / Volume 14 / August 2014
Results
Fig. 1. Line graph displaying the percentage of patients with MMC who received shunts per year. There was not a statistically
significant change in shunt rate over the period evaluated.
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B. C. Phillips et al.
TABLE 1: Characteristics of hydrocephalus in a cohort with MMC
Variable
36.3 3.3
11 (61.1)
37.5 2.3
29 (53.7)
14 (77.8)
1 (5.6)
2 (11.1)
1 (5.6)
6 (33.3)
5 (27.8)
0
42 (77.8)
5 (9.3)
6 (11.1)
1 (1.9)
11 (20.4)
19 (35.2)
4 (7.4)
14 (77.8)
3 (16.7)
1 (5.6)
32.8 3.8
0.13 0.077
0.446 0.084
1.77 0.36
2.35 0.97
13 (24.1)
33 (61.1)
8 (14.8)
34.8 3.5
0.316 0.264
0.549 0.089
2.33 0.83
4.04 1.61
p Value
0.1850*
0.7848
0.8270
0.3379
0.7737
0.5660
0.0003
0.0611*
0.0126*
0.0004*
0.0004*
<0.0001*
Discussion
No.
Missing
HR (95% CI)
p Value
0
0
0
0
0
0
0
12
1.13 (0.991.28)
0.0673
1.16 (0.681.99)
0.5813
1.14 (0.602.17)
0.6890
1.33 (0.622.57) 0.4068
1.06 (0.611.86)
0.8342
3.23 (1.716.09)
0.0003
1.11 (1.031.20)
0.0042
1.28 (1.161.40) <0.0001
11
6
6
1.17 (1.081.25)
1.77 (1.312.39)
1.48 (1.251.74)
<0.0001
0.0002
<0.0001
HR (95% CI)
3.23 (1.706.12)
1.11 (1.031.20)
2.26 (1.264.56)
1.37 (1.151,63)
1.22 (1.091.37)
1.12 (1.041.22)
p Value
<0.0001
0.0003
0.0042
<0.001
0.0219
0.0005
<0.0001
0.0009
0.0047
* In Model 1, the predictor pool included patient characteristic variables that had no missing values (see Table 2); only fontanelle type and
head circumference remained in the model after a stepwise variable
selection procedure. In Model 2, the predictor pool included the same
set from Model 1 as well as the radiological measures VI and TOD. In
Model 3, all variables were included in the predictor set.
ing. The initial OFC was not predictive of the need for
CSF diversion, consistent with the widely held belief that
hydrocephalus worsens after MMC closure. Rather, we
found that a more valuable use for OFC is to follow the
measurement of individual patients over an extended period of time. In children who cross percentiles for OFC
measurements, this may be indicative of the progression of hydrocephalus and the subsequent need for shunt
placement. Additionally, in this study we found that patients whose head circumference increased by more than
3 mm per day were more likely to require CSF diversion.
The FOHR makes an accurate estimation of the ventricle size using CT. The FOHR does not change with
age in normal patients.10 In this study, we found a statistically significant difference in this parameter between
patients with and without shunts. As an indirect assessment of ventricular volume, this finding may indicate that
the degree of ventriculomegaly in patients with MMC is
a significant factor in the requirement for CSF shunting
regardless of other signs and symptoms that a patient may
have. Although bias may exist from selective imaging of
patients with suspected hydrocephalus, the FOHR can be
a useful factor as an objective estimate of ventricular size
when deciding if a patient requires a shunt. Almost all of
the MMC patients in this study underwent head ultrasonography as part of our admission protocol. It was also
interesting to find that nearly all of the patients also had
at least 1 CT scan. While there was statistical significance
for both imaging modalities, it appears that head ultrasonography for VI and TOD measurements was as good if
not better than CT for predicting the need for CSF diversion. Therefore, because of the radiation exposure associated with CT, it may be advisable to reserve CT for those
patients with complex intracranial anatomy prior to shunt
placement who cannot undergo MRI.
Many of the variables collected in this study are subjective in nature. Thus, there may be bias when collecting
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B. C. Phillips et al.
this information. Additionally, the measured head circumference was performed by an array of neurosurgical
staff, and while all use the same method of measurement,
it may be subject to some nuances. Experienced neurosurgical caregivers recognize that there can be significant
differences of OFC measurements between examiners.15
In the end, determining which patients will benefit from
a shunt is based on the surgeons gestalt and is somewhat subjective. Although we use placement of a shunt
as a marker for need of a shunt, the two are likely not the
same. Future studies may include a blinded prospective
analysis of CSF diversion to help eliminate the previously
described biases.
Conclusions
Creating a synopsis of variables that facilitate targeting patients with MMC needing CSF diversion will
aid both the consulting physician and treating specialist.
Eliminating unnecessary CSF diversion and treating only
those who require CSF diversion is the ultimate goal.
While there are a number of signs and symptoms that
have long been associated with hydrocephalus in the neonate, this study has shown that several of them were not
significantly associated with the need for CSF diversion,
particularly apneic and bradycardic episodes. Fontanelle
description and the rate of change of the OFC appear to
be the most important clinical signs when determining
the need for shunt. Additional information collected by
head ultrasonography and CT, including the VI, TOD, and
FOHR, are statistically significant measures to evaluate
prior to placement of a ventriculoperitoneal shunt. The
optimal patient with MMC for CSF diversion will have
a soft/full to tense fontanelle, increasing head circumference of more than 3 mm/day, and radiological evidence of
elevated VI, TOD, and/or FOHR as described.
Disclosure
The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this
paper.
Author contributions to the study and manuscript preparation include the following. Conception and design: Ocal, OBrien,
Albert. Acquisition of data: Phillips, Gelsomino, Pownall, Albert.
Anal
ysis and interpretation of data: Phillips, Pownall, Spencer,
Albert. Drafting the article: Phillips, Gelsomino. Critically revising
the article: all authors. Reviewed submitted version of manuscript: all
authors. Approved the final version of the manuscript on behalf of all
authors: Phillips. Statistical analysis: Spencer. Administrative/technical/material support: OBrien, Albert. Study supervision: Albert.
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