Terminal Syringomyelia: Is It As Innocent As It Seems?-Case Report-

Abstract

A 5-day-old newborn girl presented with cutaneous signs of hypertrichosis, skin dimple, and subcutaneous lipoma in the lumbar region. Magnetic resonance (MR) imaging of the thoracolumbar spine demonstrated spinal dermal sinus, type II split cord malformation, and tethered cord. A small terminal syringeal cavity was also present. Five months after the diagnosis, weakness in dorsal flexion of the bilateral feet and toes was detected. Surgery to untether the cord was performed. Her deficits resolved completely. Nearly 4 years later, she was admitted with weakness in dorsal flexion of the right foot and toe. MR imaging of the spine showed holocord syringomyelia, postoperative changes, and tethered cord. No Chiari malformation was seen. Syringosubarachnoid shunt placement was performed. Terminal syringomyelia is common in cases of occult spinal dysraphism, but the natural history and management remain unclear. Progression, regression, or persistence of terminal syringeal cavity can occur whether untethering is performed or not. Progression of the terminal syrinx occurred after tethered cord release and the enlargement of the cavity involved the entire spinal cord in the present case. Terminal syringomyelia requires close follow up for a long time even in asymptomatic cases. Surgical treatment should be considered in symptomatic cases.

Intraoperative Spinal Sonography

“…2 If symptoms do not improve or the syrinx enlarges, placement of a syringosubarachnoid shunt is an effective treatment. 3,6,8 Intraoperative sonography can be used to guide for shunt placement. 2,3,6,8 Even when optimally placed, subarachnoid shunts are still subject to malfunction, with the most common being shunt obstruction, followed by injury to or tethering of the spinal cord, infection, and low-pressure CSF state.…”

“…3,6,8 Intraoperative sonography can be used to guide for shunt placement. 2,3,6,8 Even when optimally placed, subarachnoid shunts are still subject to malfunction, with the most common being shunt obstruction, followed by injury to or tethering of the spinal cord, infection, and low-pressure CSF state. 8 Most patients treated with shunting procedures will experience neurological improvement of symptoms with complete or mild regression of the syrinx.…”

An Effective Surgical Method for Terminal Syringomyelia: Terminal Ventriculostomy-Associated “V”-Type Ostomy

“…4 Previous studies have suggested that terminal ventriculostomy causes TCS relapse, which may be due to reclosure of the fistula and preclude further drainage. [4][5][6] Conversely, Beaumont et al confirmed the advantages of this approach. 7 Thus, the effectiveness of terminal ventriculostomy remains controversial.…”

Clinical Significance of Terminal Syringomyelia and Accompanying Congenital Anomalies of Neurosurgical Interest in Adult and Pediatric Patients with Tethered Cord Syndrome

“…Although many theories have been proposed regarding the formation and progression of syringomyelia, the underlying pathogenesis remains unknown. 5,7 Syringomyelia can occur posttrauma and may be associated with Chiari's malformations, intramedullary tumors, meningomyelocele, meningocele, or TCS. It typically occurs in the cervical and/or thoracic segments.…”