Complex Type Split Cord Malformation: A Two-Center Study

Area of Science:

• Neuroscience
• Developmental Biology
• Pediatric Surgery

Background:

• Complex spinal dysraphism presents with multiple pathologies in a single patient.
• Complex spinal muscular atrophy (SCM) is a phenotype involving disruptions across developmental stages, characterized by co-occurring SCM and spina bifida aperta.

Purpose of the Study:

• To differentiate complex SCM from classical SCM cases.
• To analyze embryological aspects, perioperative management, and follow-up in complex SCM.

Main Methods:

• Retrospective analysis of 44 patients with complex SCM who underwent surgery.
• Evaluation of surgical timing, neurological status, SCM type, and co-occurring anomalies.

Main Results:

• 81.8% of patients had neonatal surgery, often combined with spina bifida aperta repair.
• High prevalence of neurological deficits (68.2% plegic/severely paretic) and associated anomalies like hydrocephalus (77.3%) and Chiari malformation (65.9%).
• Type 1 SCM was most common (79.5%), with a higher incidence in the thoracic region when diagnosed younger.

Conclusions:

• Complex SCM requires a multidisciplinary approach, considering associated spina bifida aperta anomalies.
• Early diagnosis influences SCM type and location, with a trend towards Type 1 and thoracic region involvement.
• Complex SCM cases exhibit a higher frequency of additional anomalies compared to classical SCM.