Congenital Dermal Sinus

Infants are frequently referred to the pediatric neurosurgeon with a midline spinal dimple, occasionally with discharge from the tract. This may be the initial presentation of many congenital dermal sinuses but a significant number present with meningitis due to skin or gut organisms. Multiple episodes of meningitis may even occur before the diagnosis is made, since the cutaneous opening of the tract may be minute. Nevertheless, meningitis due to organisms such as Staphylococcus aureus or Escherichia coli in an infant rather than a neonate should arouse suspicion and, particularly after recurrent episodes, a concerted effort to identify a sinus tract should be made. The opening may be anywhere along the midline of the spine and may even occur in the occiput.

Many infants are referred with a dimple in the natal cleft, fixed inferiorly. These are generally innocent and should not be explored surgically because of their benign nature and, if surgery is carried out, they almost always become infected.

The congenital dermal sinus is an epithelial-lined tract that may end in the soft tissues or may extend deeper, to be attached to or penetrate the dura; may end in the subarachnoid space; or, more commonly, be attached to the filum terminale and end at the conus medullaris. There may be inclusion dermoid material, forming a mass anywhere along the tract.

There is rarely any neurological deficit, unless a dermoid cyst has compressed local nerve roots or meningitis has caused a deficit. MRI scanning reveals the extent of the tract and any associated dysraphic abnormality (Fig. 27.5). Contrast media or probes should not be inserted along the tract.

Surgical excision of the tract is indicated both because this may be a tethering lesion and because of the risk of meningitis. This should be carried out without undue delay. If meningitis occurs, this should be treated as appropriate first, and only when the inflammation has resolved should surgery be undertaken. Once meningitis has occurred, the tract and sub-arachnoid spaces may be scarred, making the operation technically more difficult.

The object of surgery is to excise the tract in its entirety. This involves excising its cutaneous orifice and following this to its termination. This may require opening the dura and excision of the attachment to the filum terminale. Any associated dermoid cyst, either intradural or extradural, should also be excised.

Complete excision achieves a cure and neurological outcome is generally very good, with few, if any, long-term problems [24].

Congenital Dermal Sinus
Fig. 27.5. Sagittal T1-weighted MR image of a 14-year-old boy with a congenital dermal sinus entering the theca at the L4 level. He had had two episodes of staphylococcal meningitis prior to referral.
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