Unusual presentation of split cord malformation type I associated with myelomeningocela

Abstract

Objective – We report an unusual case of split cord malformation (SCM) associated with open spinal dysraphism and other anomalies of the central nervous system.

Case report – A male newborn was admitted to the Pediatric Intensive Care Unit immediately after birth by Caesarean delivery. Clinical examination and diagnostics (MRI) showed open spinal dysraphism in the distal part of the spine (level L5 and S1), split cord malformation type I which separated two hemicords, tethered cord and syringomyelia. Two days after birth microneurosurgery was performed. Cranial and cervical MRI showed Chiari I and corpus callosum hypoplasia. There was no hydrocephalus. Preoperatively the patient had paraparesis and discretely moved his left foot. MRI showed a dilated bladder so he may have had urinary retention. The postoperative recovery was satisfactory. The patient did not have any additional neurological deficit. The patient was followed up by a neurosurgeon, pediatric surgeon, pediatrician and physiatrist. Control MRI scans showed significant regression of the syrinx and some ascensus of the medulla. About 18 months after operation the patient had discrete paresis of the left foot.

Conclusion – Complex spina bifida is an extremely rare condition. In this paper we describe this interesting case of complex spina bifida: a split cord malformation characterized by atypical bony morphology with a dural fold into the bone septum.