Tethered cord with tandem lipomyelomeningoceles, split cord malformation and thick filum

Hanif, Hamed; Khanbabazadeh, Sajjad; Nejat, Farideh; Khashab, Mostafa El

doi:10.4103/1817-1745.123665

Cited by 6 publications

(1 citation statement)

References 9 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…But literature reveals that the incidence of the double lipomas along with SCM type 2 is as rare as with SCM type 1 [ Table 1 ]. [ 7 8 , 22 23 ] Different unknown genetic pathways for the development of EMT and premature disjunction might be responsible for the phenomenon. It needs further genetic testing such as ARHGAP29 and RADIL genes and embryological studies and hypothesis testing to prove whether the development of double lipomas in association with SCMs are a sequel more than a chance.…”

Section: Discussionmentioning

confidence: 99%

Split cord malformation type 2 with double dorsal lipoma: A sequela or a chance

et al. 2020

View full text Add to dashboard Cite

A BSTRACT An 11-month-old baby girl, with normal perinatal history, presented with a congenital lumbosacral swelling and a sacral dimple. Imaging revealed a split cord malformation (SCM) type 2 at L1–L3 with a lipomeningocoele extending intradurally and tethering both the hemicords, the conus was noted to be at L4. She underwent excision of the lipoma and detethering of the hemicords. Intraoperatively, the split cord was seen from L1 to L3 with an aberrant median vessel passing between the hemicords. Two lipomas were seen separately attached to each of the hemicords, the lipomas were dissected off the hemicords, and the hemicords were neurulated. The case helps revisit the unified theory proposed by Pang for SCM as well as the theory of premature disjunction in the pathogenesis of lipomeningocoele. Formation of the endomesenchymal tract splits the developing cord into two, whereas the premature detachment of neuroectoderm from the cutaneous ectoderm can lead to lipomeningocoeles. The present case is only the fourth case ever reported of an SCM type 2 with double lipoma. In the case report, we discuss the embryological basis of this condition and surgical nuances of management.

show abstract

Section: Discussionmentioning

confidence: 99%