gms | German Medical Science

Objective: Tethered cord syndrome caused by tight or fatty filum belongs to neural tube defects and is part of spina bifida occulta. Most children have cutaneous stigmata like dimples, some may develop palsies of the lower extremities, gait disturbances, back and leg pain, pes equinus, club foot, scoliosis, bladder and bowl disturbances. If the symptoms already exist at first presentation the underlying myelopathy often does not resolve after surgery. Nowadays pediatric neurosurgeons tend to operate these patients in early childhood. Despite this fact the early intervention in such patients is not standard of care in many hospitals.

Methods: In this study we included 35 children, who received microsurgical division of tight or fatty filum between 1999 and 2017. The diagnose based on suspicious cutaneous stigmata or clinical symptoms. Cutaneous stigmata included sacral dimples, angiomas or an asymmetrical gluteal fold. Typical symptoms were club feet, leg size differences, scoliosis or muscle atrophy. When diagnosed, MRI scans of the entire neuro axis and a multidisciplinary evaluation were performed. During the treatment, the children were taken care of in a neuropediatric ward.

Results: The mean age of the operated children (16 boys and 19 girls) was 3.2 years, 5 children were preterm infants. 15 children were younger than 2 years, 7 were over 10 years old at the time of surgery. The average follow-up period was 5.2 years. All patients with neurological symptoms improved, mostly the back and leg pain. Gait disturbances, stumbling, falls and pare disappeared in descending frequency. Existing bladder and bowel disturbances improved less frequently, scoliosis showed signs of improvement or were treated further by pediatric orthopedics. In a number of children, the tight filum was associated with an overlapping disease, spinal dysgenesis, hip dysplasia, and malformations of the pelvic organs. None of the operations resulted in a worsening of symptoms. Reoperations were not required and no secondary tethering was observed. CSF fistula did not occur. In two cases a wound infection was treated surgically. Children operated within the first 2 years of life presented with best outcome.

Conclusion: We interpret these results as an argument for early surgery of asymptomatic tethered cord due to a tight or fatty filum. Surgical morbidity is low and early intervention reliably prevents the development, persistence or deterioration of neurological symptoms.