Sacral extradural arachnoid cyst in association with split cord malformation

Area of Science:

• Pediatric Neurosurgery
• Congenital Spinal Disorders
• Spinal Dysraphism

Background:

• Split cord malformation (SCM) is a congenital anomaly often associated with other spinal malformations.
• Concurrent sacral extradural arachnoid cysts (SEAC) and SCM are exceptionally rare, with few reported cases.
• Understanding the interplay and management of these concomitant anomalies is crucial.

Purpose of the Study:

• To document the experience and outcomes of simultaneous surgical management for patients with coexisting SEAC and SCM.
• To highlight the challenges and efficacy of treating these rare spinal concomitant anomalies.
• To provide insights into the surgical approach for this specific patient cohort.

Main Methods:

• A case series design was employed, analyzing patients with concurrent SCM and SEAC.
• Data were collected from 73 patients operated on for SCM between 2008 and 2014.
• Clinical data, radiological findings, urodynamic tests, and surgical outcomes were prospectively filed and retrospectively evaluated.

Main Results:

• Seven patients (2 male, 5 female) with coexisting SCM and SEAC were identified (age range 18-119 months).
• Six patients had Type I SCM, one had Type II SCM; all had cord tethering, and six developed syrinx.
• All patients presented with neurogenic bladder; surgical intervention involved ligating or repairing the cyst's fistulous orifice.

Conclusions:

• Symptoms of SEAC can be masked by SCM, making diagnosis challenging, especially for urological signs.
• Simultaneous surgical correction of both SCM and SEAC in a single session is recommended.
• This approach minimizes risks associated with repeat anesthesia and surgical interventions in scar tissue.