Duplication of spine with hemi-lipomyelomeningocele

Abstract

Background Duplication of the spine is very rare, and this malformation is generally considered as a severe form of type I split cord malformations. To the best of our knowledge, this is the first reported case of spine duplication associated with lipomyelomeningocele. Case We report an exceptional case of 14-year-old, asymptomatic and neurologically intact girl with duplication of the spine and marked separation of bony elements at thoraco-lumbar region. One of the split thecal sacs includes a tethered spinal cord whereas other thecal sac has no visible neural content, and there is a neighbor lipomyelomeningocele located in the midline. Conclusion A surgical operation was planned to release the tethered cord and instrumentation and fusion for scoliosis; however, the operation was declined by the patient.

Prenatally diagnosed fetal thoraco-lumbar spine duplication associated with lipomyelomeningocele: An extremely rare case of split cord malformation

“…However, this classification excludes the spine duplication, in which the bone elements are completely and separately copied. Some authors consider spine duplication a type 1 split cord malformation, while others suggest that it is unclassified ( 7 ) .…”

“…To the best of our knowledge, the case we report is the first case diagnosed in the prenatal period, and it demonstrates that the spine duplication can be diagnosed by prenatal ultrasonography. Some of the reported cases were neurologically asymptomatic patients diagnosed between the ages of 1 and 44 years ( 7 , 8 , 9 , 10 , 11 ) . Among the reported cases, the one that was most similar to our case is a neurologically asymptomatic female who did not require surgical treatment and was followed up for 6 years ( 8 ) .…”

“…However, in this study, unlike this case, there was a large lipomyelomeningocele sac that may cause possible neurological symptoms in the early stages of life and may require surgical treatment. In another reported case of spine duplication accompanied by lipomyelomengocele, the patient was diagnosed at the age of 14 and was neurologically asymptomatic ( 7 ) . In that case report, due to concerns about the clinical consequences of the tethered cord, the authors planned fusion for scoliosis and a surgical operation to release the tethered cord and instrumentation; but the operation was refused by the patient.…”

“…Spine duplication may be a component of the caudal duplication syndrome, which includes gastrointestinal, genitourinary, and distal neural tube malformations ( 3 , 4 , 5 , 6 , 7 ) . In this syndrome, unlike split cord malformations, duplication is not limited to the distal spine and clinical manifestations are present in all three germ layers, including the hindgut structures.…”

Caudal Duplication Syndrome: the Vital Role of a Multidisciplinary Approach and Staged Correction

“…Duplication of the spine is a rare anomaly 9 and even more exceptional is the association with lipomyelomeningocele. 10 While most patients with duplication of the spine have an associated neurologic deficit, some can be neurologically intact. 11,12 Our patient had normal sensory and motor function of his lower limbs and normal bladder function on cystometric studies.…”