Introduction

Tethered cord syndrome (TCS) is a disease with neurocutaneous, neuroorthopaedic, neurourologic, and  pure neurologic manifestations  Timely surgical therapy is especially important in pediatric population whereas the growth spurt may cause many disabilitating processes including scoliosis, sphincter incontinence, gait disturbances.

Primary tethered cord syndrome (due to occult spinal dysraphism) includes lipomyelomeningocele, split cord malformation, Thick-tight / fatty filum terminale, dermal sinüs, and neurenteric cyst. Secondary tethered cord syndrome (due to apert spinal dysraphism) involves the cases with repaired meningocele/myelomeningocele and arachnoid adhesions related to trauma or infection.

Laboratory work-up consists X-rays, MRI, CT (axial bone cuts), SEP, and urodinamic investigations. This presentation deals with 79 consecutive pediatric tethered cord cases in the last six years (January 2006 – December 2011) in a single institution, Inonu University Turgut Özal Medical Center, Malatya, Turkey.

Coexisting pathologies are meningocele/meningomyelocele (%35), SCM-II (%21), pes equinovarus / foot deformities (%20), midline hypertrichosis (%20), scoliosis (%15), filum terminale lipoma (%15), syringo/hydromyelia  (%12), midline hyperpigmentation  (%10), dermal sinüs (%8), hydrocephalus requiring shunt (%8), SCM-I  (%8), atrophic leg (%6), lipomeningomyelocele (%4), anal atresia (%4), intracranial infarct                     (%2), congenital hip dislocation (%2), renal anomaly (%2), cervical meningocele (%2), myxopapillary ependymoma of filum terminale (%2).

Main admission reasons are external stigmata (%58), sphincter problems          (%33), gait disturbance  (%23), motor weakness  (%17), lombalgia (%13), and bone deformity  (%10).

Results

Primary TCS                                                        (%65)

Secondary TCS                                                   (%35)

Persistant sphincter problems                          (%70)

Improvement in sphincter control                  (%30)

Persistant motor loss                                         (%75)

Improvement in motor function                     (%25)

Pain relief                                                            (%70)

No improvement in pain                                  (%30)

Conclusion

· Due to effective surgery in tethered cord syndrome

- preoperative pain is usually relieved

- progression in scoliosis is controlled

- fixed motor function loss and neurogenic bladder signs are rarely improved

- gait disturbances related to slight motor loss may improve after early surgery.

- -short follow-up of some patients (4-52 months) may be a drawback for our series.

· Due to disperse range and different morphological characteristics of anomalies that cause TCS and difficulty of sustaining objective criteria for neurological deficits of pediatric patients, the comparison and evaluation of results are difficult.

· In the pediatric age group -whether TCS is symptomatic or not- it is very likely to show clinical signs till adolescence.

· It is strongly advised to operate on those patients as early as possible with adequate radiological and laboratory work-up.

· Lipomyelomeningoceles and intradural lipomas with intact neurological findings may be exceptions for early surgery.

DYSRAPHIA - CRANIO-SPINAL MALFORMATIONS