• History and Examination 
     
    History
    A 5 year old boy, previously healthy, toilet trained for 2 years and with no past medical history, presents with 3 months of progressive toe walking and tight hamstrings.  In addition, he now has worsening urinary incontinence, bedwetting several times weekly and also with episodes of incontinence when playing during the day.
     
    Exam
    On physical examination, he has a scoliosis of the thoracic spine (20 degrees), in turning of his feet and a small dimple with a tuft of dark hair above his gluteal cleft at midline.
     

    Figure 1

    Figure 2

    1. What is the most likely diagnosis?

    2. What test might be useful for establishing a baseline to compare pre- and post-treatment?

    3 .What is the best treatment?

    4. Which of the following describes you?

    5. I practice in one of the following locations.

    6. Comments

    • Excellent case for a fast analysis
    • Associated dorsal dermai sinus should be ruled out by heavily T1 weighted images and eventually CT myelography. Was it the case at surgery?
    • Thank you for that information
    • Nice case
    • Wonderful
    • Figure 1 unseeable
    • Nice case, thank you
    • Excellent case for pediatrics
    • a way to identify that blood vessel on the filum?
    • This case should be easily diagnosed
    • I appreciate sharing this case.  I have seen a lot of similar cases during my training. And the treatment works well for them 
    • Crummy images. Discussion erroneously relates lumbar dermal sinus with fatty film.  Embyrologically distinct entitles
    • Tethered cord syndrome with S1-S4 lesioning bilateral
    • Crummy images
    • Tethered Cord Syndrome is the optional Diagnosis. Release of the Tethered Neural tissue is the best treatment. Prognosis is guarded.
    • Tethered cord
    • Interesting case. We have quite a few of thes cases at the National Institute of Peditrics in Mexico City.

     

    Case Explanation:

    Fatty infiltration of the filum terminale - “fatty filum” – is a congenital condition that can lead to traction on the spinal cord, clinically manifest as tethered cord syndrome.  While general autopsy studies have discovered fatty infiltration of the filum in approximately 5% of the population, the vast majority remain undetected throughout life and only a very small percentage of these end up requiring surgery.  Typically, fatty fila are discovered secondary to a dedicated evaluation triggered by characteristic physical findings (midline spine dermal sinus tract or dimple, focal spinal hirsutism or hemangioma) or the development of symptoms suggestive of spinal cord tethering (incontinence, constipation, scoliosis, foot/leg deformity, hamstring tightness/gait or toe walking problems).  Once suspected, MRI is useful to identify the presence or absence of a fatty filum.  Urodynamics – looking for hyperreflexia, areflexia or loss of synergistic sphincter function – can be helpful as a functional test to help in the diagnosis or exclusion of tethered cord syndrome, and can also serve as a baseline to compare pre- and post- surgery.  Indications for surgery remain controversial in asymptomatic cases, but when clear correlation with symptoms are present, then untethering is often warranted.  

     

    References:

    Features of the lumbar spine on magnetic resonance images following sectioning of filum terminale.

    Kim AH, Kasliwal MK, McNeish B, Silvera VM, Proctor MR, Smith ER.

    J Neurosurg Pediatr. 2011 Oct;8(4):384-9.

     

    Tethered cord syndrome.

    Agarwalla PK, Dunn IF, Scott RM, Smith ER.

    Neurosurg Clin N Am. 2007 Jul;18(3):531-47

     

    Pediatric tethered cord syndrome: response of scoliosis to untethering procedures. Clinical article.

    McGirt MJ, Mehta V, Garces-Ambrossi G, Gottfried O, Solakoglu C, Gokaslan ZL, Samdani A, Jallo GI.

    J Neurosurg Pediatr. 2009 Sep;4(3):270-4

     

    Outcome, reoperation, and complications in 99 consecutive children operated for tight or fatty filum.

    Ostling LR, Bierbrauer KS, Kuntz C 4th.

    World Neurosurg. 2012 Jan;77(1):187-91.

     

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