Clinical outcome of pediatric patients with a tethered spinal cord secondary to fatty

filum terminale
Introduction
Tethered spinal cord syndrome is a constellation of neurologic, orthopedic,
urologic, and pain symptoms. Dorsal or caudal tethering elements range in severity
from open spinal dysraphism to simple fatty filum terminale. Appropriate treatment
of an asymptomatic tethered cord is controversial, with advocates for both
conservative management and surgical release. At our institute, the management of
asymptomatic tethered cord patients has evolved over the years and we have at
times offered prophylactic surgical release, and more presently follow patients and
make surgical decisions based on clinical factors. As such, we are well positioned to
discuss the clinical outcomes of patients treated along each of these competing
pathways.
Methods and Materials
Retrospective review of clinical and surgical data from 1995-2013 at Stollery
Children's Hospital in Edmonton. A total of 84 patients with a fatty filum as the
cause of a tethered cord were identified, varying in age of presentation from birth to
sixteen years old. These patients were divided based on clinical presentation and
medical/surgical treatment offered: conservative management, prophylactic
release, and surgical release of patients presenting symptomatically. The majority of
patient data was gathered from surgical records, and therefore there is an
exaggerated percentage of surgical cases compared to nonsurgical cases.
Results
There were eight conservatively managed patients with MRI confirmed fatty
filum, of which four remained asymptomatic while four patients later developed
symptoms requiring surgical release. All four patients showed resolution of
symptoms. Of nineteen prophylactic releases, one required a secondary release due
to retethering. In addition to the case of retethering, five patients suffered transient
symptoms postoperatively and one patient had a surgical wound complication for a
complication rate of 37% (7/19). Of the fifty-seven patients who were surgically
managed because of symptoms at presentation, eleven needed at least one
additional release of their tethered cord for secondary retethering. Symptom
resolution from surgery varied based on the symptom. Urological symptoms (40)
improved in twenty-five cases (63%). Orthopedic symptoms (23) improved in
fourteen cases (61%). Pain symptoms (20) improved in fifteen cases (75%).
Weakness symptoms (23) improved in fifteen cases (65%). Some symptoms
resolved following additional explorations and release.

Conclusions
Our data suggests that the natural history of a tethered cord from a simple fatty
filum is not benign given that 57 patients presented symptomatically, plus an
additional 4 patients who deteriorated during clinical observation. Additionally,
patients presenting with tethered cord syndrome have incomplete resolution of their
symptoms in more than 1/3 of cases. However, it is important to recognize that
many cases of tethered cord syndrome secondary to a fatty filum are comorbid with
other disorders, and therefore persisting disabilities may be related to their
underlying syndrome beyond their fatty filum. As such, many of these symptomatic
patients presented at early age, likely with congenital disability. Our retrospective
data set under-identifies patients followed expectantly. Importantly, all patients who
demonstrated clinical symptoms during observation had complete resolution of
those symptoms with release of their tethered cord. As such we now recommend
continued clinical follow-up including urodynamic studies, spinal cord
electrophysiologic monitoring (SSEPs, and MEPs), and imaging and reserve tethered
cord release for patients with clinical deterioration or pre-clinical changes in
adjuvant studies.

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Appropriate treatment of an asymptomatic tethered cord is controversial particularly when secondary to a fatty filum.
At our institute, the management of these patients has evolved and we have at times offered prophylactic surgical
release, and more presently follow patients and make surgical decisions based on clinical factors. As such, we are
well positioned to discuss the clinical outcomes of patients treated along each of these competing pathways.
Methods:
Retrospective review from 1995-2013 of 84 pediatric patients with fatty filum as the cause of a tethered cord. Patients
were divided based on clinical presentation and treatment offered: conservative management, prophylactic release,
and surgical release of patients presenting symptomatically. The majority of patient data was gathered from surgical
records, therefore there is an exaggerated percentage of surgical cases.
Results:
Of eight conservatively managed patients with MRI confirmed fatty filum, four remained asymptomatic while four
developed symptoms requiring surgical release. All four patients showed resolution of symptoms. Of nineteen
prophylactic releases, one required a secondary release due to retethering. Five patients suffered transient
symptoms postoperatively and one patient had a surgical wound complication for a complication rate of 37% (7/19).
Of fifty-seven patients surgically managed because of symptoms at presentation, symptom resolution was
variable. Orthopedic symptoms (23) improved in fourteen cases (61%). Urological symptoms (40) improved in twentyfive cases (63%). Weakness symptoms (23) improved in fifteen cases (65%). Pain symptoms (20) improved in fifteen
cases (75%).
Conclusions:

The natural history of a tethered cord from a fatty filum is not benign given that 57 patients presented
symptomatically, plus an additional 4 patients deteriorated during clinical observation. Additionally, patients presenting
with tethered cord syndrome have incomplete resolution of their symptoms in more than 1/3 of cases. However, it is
important to recognize that many patients with tethered cord syndrome have comorbidities, and persisting disabilities
may be related to their underlying syndrome. Our retrospective data set under-identifies patients followed expectantly.
Importantly, all patients who demonstrated clinical symptoms during observation had complete resolution of those
symptoms with release of their tethered cord.