Dorsal Dermal Sinus

Dorsal dermal sinus

A dorsal dermal sinus is a very uncommon inborn disfigurement of the spine, which happens when the skin and the neural tube do not part completely in the early development of the fetus. It creates short epithelial tracts that project off the skin surface into the spinal canal and which can lead to the spinal cord or its sheaths. It is a form of closed spinal dysraphism and most usually seen in the lumbosacral area, although it may be detected anywhere in the spine where the spine runs the middle of back.

Dorsal dermal sinus symptoms

The signs of the dorsal dermal sinus (DDS) are very diverse, and may differ depending on the depth of the sinus tract, and in the presence of spinal cord abnormalities and infections. There are those children who may be asymptomatic at birth and those who have the severe complications.

External Skin Signs (Most frequent and first hints)

These can be acquired at birth and are the major red flags:

• On the back is a small midline pit or opening.
• And tuft of hair growing out of the pit.
• Dimple on skin over the cleft of the butt.
• Discharge (clear, whitish or pus-like)
• Localized skin erythema or edema.
• Birthmark or discolouration on the spine.

Neurological Symptoms

Occur when the sinus tract is related to the spinal cord or tethering:

• Weakness in one or both legs
• Delayed walking in children
• Abnormal gait
• Paralysis or numbness of the legs.
• Back pain (young children/adults)
• Gradual progressive neurodegeneration.

Symptoms of Bladder and Bowel Dysfunction 

Here, suggest spinal cord involvement:

• Retention or urinary incontinence.
• Routine lower urinary tract infections.
• Straining to defecate or fecal incontinence. 

Infectious Signs

DDS offers a direct route of infection to the spinal canal:

• Recurrent meningitis
• Fever with no obvious source
• Neck stiffness
• Harporescent or sleepy (infants)
• Spinal abscess
• Sepsis (in severe cases)

Symptoms Because of Related lesions

DDS may be mistaken with dermoid or epidermoid cysts:

• Neurological progressively deteriorating deficits.
• Acute worsening in case of rupture of a cyst.
• Indications of elevated intracranial pressure (rare)

Dorsal dermal sinus treatment

Early surgical excision is the one and the only curative intervention to a dorsal dermal sinu (DDS). To treat it only through medical means is not enough.

First-line therapy: Surgery

• The advice is to have surgery at a young age, even if the child shows no symptoms. 

Goals of surgery:

• Total eradication of the sinus tract.
• Hysterectomy of any related dermoid or epidermoid cyst.
• Untethered spinal cord, in case of its presence.

Prevention of:

• Meningitis
• Spinal abscess
• Long term neuronal injury.

Procedure overview

• Surgery was performed under general anaesthesia.
• Microsurgical excision of the sinus tract of the skin up to the end.
• Neural elements need to be carefully safeguarded.
• Dura and skin closure.

Preoperative Evaluation

Prior to surgery, the following are necessary:

• MRI spine (gold standard) to be defined:
• Extent of sinus tract
• Spinal cord involvement
• Tethered cord or cysts associated.
• Baseline neurological evaluation.
• Evaluation for infection

Treatment In the Presence of an Infection

In the case where the child exhibits meningitis or abscess:

• Emergent antibiotics IV
• Patient stabilization.
• Additional surgery after infection has been limited.
• Even after infection is cured, surgery is still necessary to avoid infection again.

Postoperative Care

• Watching CSF leak or wound infection.
• Pain management
• Mobilization early as recommended.
• Bladder and bowel function observation.
• Follow-up MRI if needed

Prognosis After Treatment

Good result in cases that are operated early.

• The asymptomatic patients normally develop normally.
• Pre-existing neurological impairments might:
• Improve partially
• Or be permanent in case of delay in surgery.

Dorsal dermal sinus surgery

Dorsal dermal sinus (DDS) surgery is a permanent neurosurgical operation that involves the elimination of the sinus tract and elimination of severe complications experienced including meningitis, spinal abscess, and irreversible neurological damage.

Indications for Surgery

Surgery in all confirmed cases; including;

• Asymptomatic posterior dermal sinus.
• Sinus with discharge
• Recurrent meningitis
• Neurological impairments or deficits
• Allied dermoid/epidermoid cyst. 
• Tethered spinal cord

Preoperative Preparation

MRI spine (mandatory) to map:

• Sinuosity and length of sinuosity.
• Involvement of the dural and spinal cord.
• Tethering or associated cysts.
• Complete neuro-examination.
• Infection screening
• Active infection: IV antibiotics, then surgery.

Procedure 

• Prone positioning and general anaesthesia.
• Sinus incision in the elliptical skin.
• Surgical microsystemic dissection of the sinus tract.
• Safe exposure- Laminectomy/laminotomy (when necessary).

Complete excision of:

• Sinus tract
• Dermoid/epidermoid cyst (when present)
• Dural opening and untethering of the spinal cord (assuming present).
• Watertight dural closure
• Layered skin and fascia and muscle.

Duration of Surgery

• Typically 2–4 hours

May be longer if:

• Infection
• Large cyst
• There is considerable tethering.

Postoperative Care

Close monitoring for:

• CSF leak
• Wound infection
• Neurological changes
• Pain control
• Premature feeding and progressive mobilization.
• Monitoring of bladder and bowel functioning.
• Antibiotics if indicated

Recovery Timeline

• Hospital stay: 3–7 days
• Wound healing: 2–3 weeks
• Go back to normal functioning: 4–6 weeks (recommended)
• Extended follow-up, with the periodically scheduled neurologic examinations.

Best hospital for dorsal dermal sinus India

• Artemis Hospital, Gurgaon
• Medanta-The Medicity, Gurgaon
• Fortis Memorial Research Institute, Gurgaon
• Max Hospital, Saket

Conclusion

Dorsal dermal sinus is a spinal abnormality that can be superficial but is accompanied with severe internal risks. Any tiny midline opening in the skin may give a direct route of infection through the spine, resulting in meningitis, spinal abscess, tethered cord and irreversible neurological damage in case of non-treatment. MRI diagnosis and timely removal of the tumors via surgery are crucial even in patients who have no symptoms. It produces a very positive result when detected at an early age by a trained neurosurgeon and the majority of children end up leading a normal life with respect to their neurological growth. The secrets of preventing life-threatening complications and long-term neurological safety are recognition in time, correct imaging, and final surgery.

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FAQ

Is dorsal dermal sinus threatening?

• Yes. Although it looks very benign, the disease can cause repeat bouts of meningitis, spinal infections, tethered cord, and there can be permanent neurological damage if left untreated.

How does this differ from a single sacral dimple? 

• Simple sacral dimple is superficial and non-infectious and is normally found in the gluteal cleft. A dorsal dermal sinus is more profound, usually above the cleft and leads to the spinal canal.

Is surgery always necessary?

• Yes. A surgical resection is required for all the confirmed cases even if the patient is asymptomatic in order to prevent complications later in life.

What is the surgery success rate? 

• When performed early the success rate is very high with long term outcomes and low complication rates being very high.

Will my child experience problems in the long term following surgery?

• In the event of early treatment, most children develop normally in terms of the neurological development. Neurological impairments that are present can be improved or stabilized.

Is dorsal dermal sinuosity inherited?

• No. It is an intermittent hereditary disease that is not typically inherited.