Lipomyelomeningocele

A type of Lipomyeloschisis.

Lipomyelomeningocele, is a closed neural tube defect, taht usually occurs in the lumbosacral area as a single lesion but can be associated with other spinal dysraphism 1) and Caudal regression syndrome.

Represent a unique population within the spectrum of spinal dysraphism.Edit

Pathology

A subcutaneous lipoma that passes through a midline defect in the lumbodorsal fascia, vertebral neural arch, and dura, and merges with an abnormally low tethered cord 2).Edit

Diagnosis

New dynamic MRI-based parameters to establish the presence and magnitude of tethered cord syndrome (TCS) have been defined. oscillatory frequency (OF) measured the extent of loss of translational cord displacement in supine and prone positions; delta bending angle (ΔBA) defined the relative angulation of conus with lower spinal cord, and sagittal and axial root angles represented ventral nerve root stretching. The difference in OF or ΔBA was minimum in the group with thick filum terminale and progressively increased in the groups with lipomyelomeningocele and meningomyelocele 3)Edit

Natural history

The natural history of LMMC remains poorly defined. The description and prevalence of the presenting orthopaedic clinical signs and symptoms for LMMC have been infrequent and often documented only in general terms.Edit

Treatment

Untethering surgery.

An expansile dural graft should be incorporated in cases of lipomyelomeningocele in which primary dural closure does not permit free flow of CSF4).Edit

Case series

In 32 patients with LMMC (21 female and 11 male patients). The majority of patients had their primary tethered cord release (TCR) by ≤1 year of age (59 %), with 22 and 19 % having primary TCR at ages 1-15 and >15 years, respectively. Fifteen patients had at least one repeat TCR, with ten of these having more than one repeat TCR. A significant relationship was noted between low back/radicular pain and repeat TCR (p < 0.001). Ten patients (31%) had a limb length discrepancy of >2.5 cm, and 53 % of patients had asymmetric involvement. Nine patients (28 %) had scoliosis of whom only one required operative treatment. Fifteen patients had foot deformities. Thirteen patients (41 %) had two or more orthopaedic procedures in addition

The presenting musculoskeletal clinical signs and symptoms in patients with LMMC are uniquely different in terms of both pattern and frequency compared to myelomeningocele and other forms of spinal dysraphism.

Its a high prevalence of asymmetrical involvement, a high operative burden, and a high rate of repeat symptomatic tethered cord syndrome requiring TCR. As previously noted by others, TCR in LMMC does not prevent long-term functional deterioration. These findings may be important to our colleagues providing counsel to their patients with LMMC and to their families 5).Edit

Case reports

Fetal lipomyelomeningocele was suspected during the second-trimester ultrasound and confirmed by magnetic resonance imaging. The pregnancy took its course and a term neonate was delivered. At 2 years of age lipomyelomeningocele surgical removal was performed. The patient is now 4 years old and, despite neurogenic bladder, is a healthy boy with normal psychomotor development for his age. This case illustrates the favorable prognosis of this entity and the importance of prompt diagnosis and multidisciplinary counseling 6).Edit

References

Edit1) Hanif H, Khanbabazadeh S, Nejat F, El Khashab M. Tethered cord with tandem lipomyelomeningoceles, split cord malformation and thick filum. J Pediatr Neurosci. 2013 Sep;8(3):204-6. doi: 10.4103/1817-1745.123665. PubMed PMID: 24470813.2) Emery JL, Lendon RG. Lipomas of the Cauda Equina and Other Fatty Tumors Related to Neurospinal Dysraphism.DevMedChildNeurol.1969;11:62–703) Singh S, Behari S, Singh V, Bhaisora KS, Haldar R, Krishna Kumar G, Mishra P, Phadke RV. Dynamic magnetic resonance imaging parameters for objective assessment of the magnitude of tethered cord syndrome in patients with spinal dysraphism. Acta Neurochir (Wien). 2018 Nov 20. doi: 10.1007/s00701-018-3721-7. [Epub ahead of print] PubMed PMID: 30456429.4) Alexiades NG, Ahn ES, Blount JP, Brockmeyer DL, Browd SR, Grant GA, Heuer GG, Hankinson TC, Iskandar BJ, Jea A, Krieger MD, Leonard JR, Limbrick DD Jr, Maher CO, Proctor MR, Sandberg DI, Wellons JC 3rd, Shao B, Feldstein NA, Anderson RCE. Development of best practices to minimize wound complications after complex tethered spinal cord surgery: a modified Delphi study. J Neurosurg Pediatr. 2018 Sep 14:1-9. doi: 10.3171/2018.6.PEDS18243. [Epub ahead of print] PubMed PMID: 30215584.5) Segal LS, Czoch W, Hennrikus WL, Wade Shrader M, Kanev PM. The spectrum of musculoskeletal problems in lipomyelomeningocele. J Child Orthop. 2013 Dec;7(6):513-9. doi: 10.1007/s11832-013-0532-5. Epub 2013 Oct 8. PubMed PMID: 24432115.6) Sarmento-Gonçalves I, Cunha M, Loureiro T, Pinto PS, Ramalho C. Fetal lipomyelomeningocele: A closed neural tube defect diagnosed at second trimester ultrasound examination. J Clin Ultrasound. 2018 Nov 8. doi: 10.1002/jcu.22662. [Epub ahead of print] PubMed PMID: 30411358.

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