Klippel–Feil Syndrome Associated with Sacral Agenesis, Low Lying Cord, Lipomyelomeningocele and Split Cord Malformation Presenting with Tethered Cord Syndrome: Pentads Neural Tube Defects Spread along Whole Spinal Axis

Neural tube defects are congenital development anomaly of the central nervous system and usually have relatively more predilection to affect at anterior and posterior neuropore embryological development sites, so usually one or two defects are commonly encountered. However, occurrence of simultaneous multiple neural tube defects is very rare, presence of constellation of five neural defects is extremely rare, and all defects add up together to produce gross neurological deficit. We present an interesting case of a 23-year-old male who presented with history of lower backache and noticed wasting and weakness of lower limbs associated with difficulty in walking for the last 2 years but had no associated sphincter disturbances. He was operated for lumbosacral lipomeningocoele repair at the age of 1 year. He was asymptomatic, following the first surgical intervention. At the current admission, he underwent re-exploration of surgical wound with surgical repair although suffered mild-temporary neurological worsening in the immediate postoperative period. Imaging feature and management of such rare constellation of five embryological anomalies and its significance and brief literature are discussed.