Author Affiliations: Department of Neurosurgery, the Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China (Huang SL, Shi W); Department of Surgery, Xi'an Children's Hospital, Xi'an 710003, China (Zhang LG)

Corresponding Author: Wei Shi, Department of Neurosurgery, the Second Affiliated Hospital, School of Medicine, Xi'an Jiaotong University, Xi'an 710004, China (Tel: 86-29-87679366; Fax: 86-29-87678634; Email: sweins@163.com)

Background: Lipomyelomeningocele (LMM) is a common and severe closed neural tube defect in children. Because of the complex anatomy of LMM and the difficulty in assessing the value of surgery, the management of patients with LMM is controversial. This study was undertaken to evaluate effective techniques and procedures in surgical treatment of LMM and to assess the value of early neurosurgical intervention.

Methods: Twenty-five children with LMM aged from 2 months to 6 years underwent surgery between January 2004 and December 2006. Magnetic resonance imaging (MRI) of the spine and electromyography (EMG) of the lower limbs was conducted in all patients preoperatively. Urodynamic studies were appropriate even in asymptomatic patients. Hoffman's functional grading was used to assess the pre and postoperative status of the patients. The operation was composed of subtotal excision of lipoma, suturing of the spinal pia mater, and section of the filum terminale. Suturing of the spinal pia mater was performed in a wide process of reconstruction of meningeal layers including the dura. Follow-up lasted 1-4 years (mean 2.1 years), in which all children underwent neurological examination, EMG and MRI.

Results: After surgery a temporary neurological deterioration was found in two patients including slight weakness of a leg in one patient and urinary retention in the other, but it recovered completely a few days later. No postoperative complications were encountered. During the follow-up, 20 asymptomatic patients remained symptom-free. Symptoms disappeared totally in 2 of the 5 patients with neurological deficits, improved in one patient and stabilized in the remaining 2. MRI showed no tethered cord in all patients who underwent surgical procedures.

Conclusions: Early operation for LMM patients, even asymptomatic ones, should be performed to prevent the development of neurological deficits. Subtotal excision of lipoma, suturing of the spinal pia mater, and section of the filum terminale are recommended in the surgical treatment of LMM. The longitudinal cut of the filum terminale, a technique we have established in our surgical practice, is a simple and practical way to identify the filum terminale by visual inspection. And suturing the spinal pia mater is of extreme importance in preventing postoperative tethering.

Key words: children; filum terminale; lipomyelomeningocele; spinal pia mater; surgical treatment

                    World J Pediatr 2010;6(4):361-365