Open spina bifida: a complete cohort reviewed 25 years after closure

Area of Science:

• Pediatric Surgery
• Developmental Neurology
• Rehabilitation Medicine

Background:

• Open spina bifida is a complex congenital condition requiring early surgical intervention.
• Long-term follow-up is crucial to understand the lifelong impact of the condition and its management.

Purpose of the Study:

• To evaluate the long-term functional outcomes and survival rates in a cohort of patients treated for open spina bifida.
• To identify key determinants of disability and dependency in adulthood following non-selective treatment.

Main Methods:

• A consecutive series of 117 open spina bifida cases were followed for 22 to 28 years post-closure.
• Ascertainment included assessment of survival status and functional independence.
• Neurological deficit and IQ were analyzed as potential predictors of long-term outcomes.

Main Results:

• Mortality was 48% (56 out of 117) by long-term follow-up.
• Among 61 survivors, functional status ranged widely, with only 33 living independently.
• Neurological deficit extent and IQ were the primary factors correlating with disability and dependency levels.

Conclusions:

• Improvements in open spina bifida management have significantly reduced mortality.
• Long-term disability is strongly linked to the inherent severity of the neurological deficit, which current treatments may not fully mitigate.
• Understanding these determinants is vital for patient counseling and long-term care planning.