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The patterns of late deterioration in patients with transitional lipomyelomeningocele
D D Cochrane1, C Finley, J Kestle
1Division of Pediatric Neurosurgery, Children's and Women's Health Center of British Columbia, Vancouver, Canada. dcochrane@cw.bc.ca
Insights
Untethering surgery for transitional lipomyelomeningocele (LMMC) in children is common, but functional deterioration still occurs frequently after the procedure. Outcomes after surgery are similar to those observed in untreated patients.
Area of Science:
- Pediatric Neurosurgery
- Spinal Dysraphism Research
- Clinical Outcomes Analysis
Background:
- Transitional lipomyelomeningocele (LMMC) is often associated with age-related neurological deterioration.
- Prophylactic untethering is frequently recommended to prevent or stabilize this decline.
Purpose of the Study:
- To assess the frequency and patterns of functional deterioration after successful untethering in pediatric LMMC patients.
- To compare outcomes in surgically treated patients with historical data from untreated LMMC cases.
Main Methods:
- Retrospective review of 50 pediatric patients with transitional LMMC treated at a single institution.
- Analysis of clinical status pre-untethering and development of new symptoms post-untethering.
- Follow-up duration ranged from 2 to 138 months (mean 39 months).
Main Results:
- Most patients (82%) were diagnosed and 78% underwent untethering before age one.
- Late clinical deterioration occurred in the majority of patients post-untethering.
- Orthopedic and neurological decline appeared within 60 months, followed by urological decline.
Conclusions:
- Functional loss is common after LMMC untethering, with patterns potentially reflecting detection limitations in infants.
- Untethering generally avoids permanent acute morbidity but does not prevent long-term functional deterioration.
- The rate of symptomatic patients post-untethering mirrors that of untreated LMMC cohorts.
Introduction:
Tethering is assumed to be the primary cause of deterioration seen in children with transitional lipomyelomeningocele as they age. The inevitability of deterioration has led to recommendations for prophylactic interventions to stabilize or prevent further clinical deterioration.
Objective:
Determine the frequency and patterns of functional deterioration observed after successful untethering in patients with transitional lipomyelomeningocele and compare functional outcomes with what is known regarding untreated patients.
Methods:
Fifty patients having transitional LMMC, treated at a single institution and followed in a multidisciplinary clinic were retrospectively reviewed to determine their clinical status prior to untethering, and the time to development of new symptoms or signs following untethering.
Results:
82% of patients were diagnosed and 78% underwent untethering prior to one year of age. All patients had a cutaneous lumbosacral lipoma, 22 patients were considered normal at presentation and 28 showed abnormalities on clinical examination. Forty-nine patients were untethered successfully and all were available for follow-up ranging from 2 to 138 months (mean 39 months). Acute morbidity was limited to transient neurogenic bladder dysfunction and minor wound complications. Late clinical deterioration occurred in the majority of patients. Orthopedic and neurological deterioration occurred over the first 60 months following untethering and urological deterioration occurred thereafter.
Conclusions:
Functional loss after untethering is common and the pattern of loss is likely a reflection of the ability to detect abnormalities in this infant population. Untethering does not usually result in permanent acute morbidity, and does not prevent longer-term functional deterioration. The ratio of asymptomatic to symptomatic patients at follow-up in this operative series is similar to age-matched historical series of untreated patients.
