spinal dysraphism meningomyelocele tethered cord lipoma filum terminale

1. Dr. Shahnawaz Alam
Neuroradiological Case
Discussion

2. Neuropathic ulcer
4x4 cm, Epithelialized midline mass
Brief History & examination:
 6 Yrs./M, Lower back midline swelling since birth; gradually progressive, no
discharge, no tufts of hair, non-tender, soft to firm, partially compressible, mobility
restricted in upper aspect, transillumination and fluctuation +, no bruits; Limping
gait for last 2 years; first noticed by mother, asst. with slippage of footwear and
multiple traumatic ulcer, FT-LSCS, 2 sisters and 1 brother, no similar complaints in
any sibling, No H/o LOC or seizure or no NICU admission.
 O/E: Vitals: stable; HMF-intact; no milestone delay; GCS 15/15; no CNs deficit;
Power: 5/5 in B/L UL ,4/5 in B/L LL; DTR 2+, Tone & Bulk- diminished in B/L
LL; Pain, temp., crude touch, joint position- decrease in B/L LL(Lt.>Rt.); Planter-
down; bowel and bladder intact; No cerebellar/meningeal signs/No neurocutaneous
stigma.

6. • The spinal cord forms as the result of two distinct processes: primary and secondary
neurulation.
 Primary neurulation entails the proliferation and folding of neuroectoderm into a
neural tube that ultimately comprises the spinal cord. This process begins on
postovulatory day (POD) 18.
 Cutaneous ectoderm (which eventually becomes skin) separates from the
neuroectoderm and fuses on the midline during a critical process called
“disjunction.”
 Error at this stage is responsible for myelomeningocele (nondisjunction),
lipomyelomeningocele (premature disjunction), and dermal sinus tract
(incomplete disjunction).
Discussion
• Lew S, Kothbauer K (2007). Tethered cord syndrome: an updated review. Pediatr Neurosurg 43: 236 248.
• Warder DE (2001). Tethered cord syndrome and occult spinal dysraphism. Neurosurg Focus 10: e1.
• Yamada S, Zinke DE, Sanders D (1981). Pathophysiology of ‘tethered cord syndrome’. J Neurosurg 54: 494-503.

8.  Secondary neurulation refers to the formation of distal spinal elements caudal to S2 as
well as the filum. This phase of development occurs between POD 28 and 48.
 Errors that occur during canalization or regression are thought to contribute to the
formation of low lying conus, terminal lipomas/myelocystoceles and fatty filum
pathology.
 Hoffman and colleagues used the phrase ‘‘tethered spinal cord’’ and defined as a
spinal cord ‘‘with a low conus medullaris and a thickened filum terminale measuring
2 mm or more in diameter,’’ with an abnormal attachment of the spinal cord to the
tissues that surround it.
• Lew S, Kothbauer K (2007). Tethered cord syndrome: an updated review. Pediatr Neurosurg 43: 236 248.
• Warder DE (2001). Tethered cord syndrome and occult spinal dysraphism. Neurosurg Focus 10: e1.
• Yamada S, Zinke DE, Sanders D (1981). Pathophysiology of ‘tethered cord syndrome’. J Neurosurg 54: 494-503.

9.  Common presentation of tethered cord syndrome
 Derek C. Samples and Izabela Tarasiewicz (2016). Review and Classification of Occult Spinal Dysraphism and
Tethered Cord Syndrome in Children ; J Spine, an open access journal Volume 5 • Issue 4 • 1000325

11.  Pang et al. provided a unified theory of split-cord malformations (SCMs) that
encompasses both diastematomyelia and diplomyelia.
 The primary anomaly is an adhesion between ectoderm and endoderm during
embryogenesis resulting from formation of the accessory neurenteric canal through
the midline of the embryonic disk.
1. In type I, there are two sagittally separate hemicords in individual dural sacs
separated by a rigid osseocartilaginous septum, and these include
diastematomyelia.
2. Type II SCMs involve two separate hemicords within one dural sac separated by
a flexible fibrous septum.
 Medial nerve roots occur in 75% of cases and are almost always dorsal.
 Type I SCMs account for 60% of cases.
 True diplomyelia is rare and consists of duplicated cords.

12. ThankYou!