15 Spinal dysraphism

March 13 th 2012

• 4 y F comes in FFHC for well child exam 

• As you are about to leave mom states…she has been 

complaining of leg pain”

• Vitals: 36.5, 100, 24, 90/64, 

• Growth Parameters: Wt 15.2 kg (25%) Ht: 40 in (25%) 

• Gen: alert cooperative, smiling 

• HEENT: NCAT, PERRL, TM’s clear, neck no LAD, full ROM 

• CV: RRR, no murmurs, 2+ femoral pulses 

• Pulm: CTA B 

• Abdomen: soft NDNT, no masses, no hepatosplenomegaly 

• Skin: mild eczematous lesions elbow, knees, sacral dimple 

noted as well, no palpable lymph nodes inguinal/axillary 

• Extremities: symmetric muscle bulk, no joint swelling, no 

palpable point tenderness, full ROM in all joints, 

• Neuro: no focal deficits, 5/5 strength, 2+ dtrs, nl gait

• Any thoughts? 

• What are you going to tell this mom?

• Which ones are benign? 

• When should you be concerned? 

• What can they be associated with? 

• Which ones should be evaluated further? 

• What further evaluation would you do?

Adapted from Pediatrics in Review

Adapted from Pediatrics in Review 

• Solitary 

• gluteal cleft 

• Sacrococcygeal 

• Visualized base 

• < 2.5 cm from anus 

• No other skin 

findings


Adapted from Pediatrics in Review 

Greater than 

2.5 cm from 

anus


Appendage 



Duplicated 

gluteal 

cleft 



Base visualized 

Within 2.5 cm of 

anal verge 



Atretic 

Meningocele 

“cigarette 

burn” 



Hairy tuft 



Eccentric/Not Midline 

Isolated 

Within sacral spine 

Abnormal if: 

**any other lesions 

**outside of sacrum 


• Multiple dimples (look up the spine) 

• Dimple diameter >5 mm 

• Greater than 2.5 cm above anal verge 

• Cutaneous stigmata 

• Hair tufts, hemangioma, appendages, hyper/hypo-pigmentation 

• Gluteal Cleft abnormalities 

• Any midline skin lesions or more than one skin marking 

anywhere along the spine

• Occultus… 

• …clandestine, hidden, secret, 

referring to "knowledge of the 

hidden 

http://audubonoffloridanews.org/?p=3409

1 in 4,000 

Skin Covered NTD 

90% cutaneous stigmata 

Not Spina Bifida Occulta 

s 

Tethered 

Cord 

Lipomeningocele 

http://www.neurosurgery.ufl.edu/patient 

s/pediatric-spina-bifida.shtml 

Dermal 

Sinus 

Tract 

Diastematomelia

• 5 y/o F with bilateral leg pain occuring intermittently for the 

last few months, that is occasionally waking her up from sleep, 

exam wnl with the exception of a sacral dimple


What type of imaging is best?

Ultrasound: infants up to 3-5 mos of age, dynamic 

image, no radiation or sedation 

Cons: difficult to interpret, operator dependent, hard to 

determine if have a fatty filum 

MRI: modality of choice for visualizing level of the 

conus medullaris and for identifying fatty filum 

◦Cons: it may require sedation

L1 

S1 

Conus low 

at mid L2

Spinal cord does not 

move anteriorly 

PRONE

Fatty Filum

• IMPRESSION: 

1. Tethered cord with lower than normal position at the level of 

mid L2 with diffuse 

linear fatty infiltration of the filum terminale 

• 

2. No evidence of bone deformation of the posterior elements 

of the lumbar or sacral 

vertebral bodies

What causes it? 

What are the symptoms?

True incidence unknown 

2:1 (female: male) predominance 

May have associated orthopedic/vertebral abnormalities, 

anorectal, or urogenital malformations 

genetic predisposition? 

MRI: Conus medullaris below L2 and a funky filum

• 1° Abnormal embryologic development 

• Loss of function of the filum terminalis (elastic band) 

• Fatty infiltration /Thickened 

• Cord becomes anchored 

• stress (esp. flexion/extension) 

• ↑stretch 

• ↓blood flow/oxidative metabolism 

• Neurological signs and symptoms 

http://yoursurgery.com/ProcedureDetails.cfm 

BR=2&Proc=82

• Pain 

• Bladder/Bowel 

Function 

• Cutaneous 

Findings 70-90% 

• Delayed 

development 

• Asymmetry 

• Sensorimotor 

• LMN signs

• Ultrasound/MRI: 

• all abnormal US should have MRI done and neurosurgery 

referral. 

• Neurosurgery Referral 

• Urodynamic Studies 

• Can identify deficiencies and also be used as a maker for 

improvement

Surgery: un-tethering 

Indications: 

◦ Symptomatic, low lying conus, abnl filum 

◦ Asymptomatic, low lying conus, abnl filum 

Complications: 

◦ Infection 

◦ CSF leakage 

◦ Meningitis 

◦ Neurologic Damage (

Maximal improvement: 

Usually by 6 months 

Improvement rates 

◦ Pain 90-100% 

◦ Sensorimotor 43% (related to duration) 

◦ Urodynamics improved 50-87% 

Complete urological recovery is rare 

Importance of finding these patients before symptomatic!

• Referred to Neurosurgery 

• Urodynamic Studies: abnl for low capacity, high pressure 

bladder felt to be related to the tethered cord 

• VCUG and Renal US normal 

• Surgery was performed 2 months after initial MRI 

• Currently doing well should be following up soon

• DDx for child with leg pain 

• Cutaneous stigmata associated with OSD 

• What type of imaging should be done to evaluate these lesions 

• Reasons to refer to Neurosurgery 

• What are the signs and symptoms of a tethered cord 

• ALWAYS CHECK YOUR NEW PATIENTS SPINE/SACRUM!!

Zywicke, Holly MD and Curtis Rozzelle MD. “Sacral Dimples.” 

Pediatrics in Review 32 No. 3 (2011):109-113 

Bui, Cuong MD; Tubbs, Shane Ph.D.; and Jerry Oakes MD. “Tethered 

Cord Syndrome in children a review.” Neurosurg Focus 23(2) (2007) 

: E2 p1-9 

Tse, Shirley and Ronald M. Laxer. “Approach to Acute Limb Pain in 

Childhood” Pediatr. Rev. (2006):27;170-180 

Kliegman et al. Nelson Textbook of Pediatrics. Philadelphia: 

Saunders, 2007. 

Your surgery.com.2007. Laminectomy for Tethered Cord. 6 Mar 

2011.

15 Spinal dysraphism

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