Management of split cord malformation in children: the Lyon experience

Childs Nerv Syst. 2018 May;34(5):883-891. doi: 10.1007/s00381-018-3772-3. Epub 2018 Mar 26.

Abstract

Introduction: Split cord malformation (SCM) is a rare congenital spinal abnormality. Clinical presentation varies. Other congenital defects can be associated. Management is surgical.

Material and methods: We retrospectively reviewed all our SCM cases and reported our experience for its management. From 1990 to 2014, 37 patients were operated. Five situations lead to the diagnosis (orthopedic disorders (n = 8), orthopedic and neurological disorders (n = 16), pure neurological disorders (n = 5), no symptoms except cutaneous signs (n = 7), antenatal diagnosis (n = 1)). Scoliosis was the most common associated condition. The level of the spur was always under T7 except in one case. There were more type I (n = 22) than type II (n = 15) SCM.

Results: Patients with preoperative neurological symptoms (n = 21) were improved in 71.4%. Five out of nine patients that had preoperative bladder dysfunction were improved. Eleven patients needed surgical correction of the scoliosis.

Conclusions: For us, the surgical procedure is mandatory even in case of asymptomatic discovery in order to avoid late clinical deterioration. In any case, the filum terminale need to be cut in order to untether completely the spinal cord. In case a surgical correction of a spinal deformity is needed, we recommend a two-stage surgery, for both SCM type. The SCM surgery can stop the evolution of the scoliosis and it may just need an orthopedic treatment with a brace.

Keywords: Bone spur; Diastematomyelia; Diplomyelia; Split cord malformation; Surgery; Tethered cord syndrome.

MeSH terms

  • Adolescent
  • Child
  • Child, Preschool
  • Disease Management*
  • Female
  • France
  • Humans
  • Infant
  • Infant, Newborn
  • Magnetic Resonance Imaging
  • Male
  • Nervous System Diseases / diagnostic imaging
  • Nervous System Diseases / etiology
  • Nervous System Diseases / therapy
  • Neurosurgical Procedures
  • Retrospective Studies
  • Spinal Cord / abnormalities*
  • Spinal Cord / diagnostic imaging
  • Spinal Cord Diseases / complications
  • Spinal Cord Diseases / diagnostic imaging
  • Spinal Cord Diseases / epidemiology
  • Spinal Cord Diseases / therapy*
  • Tomography Scanners, X-Ray Computed
  • Treatment Outcome