Presentation of Intramedullary Spinal Cord Tumors in Children

Symptoms

Knowledge of the typical pattern of symptom evolution will warn the treating physician to prepare for an unusual or aggressive IMSCT.  This in turn will lessen the risk of morbidity due to treatments being rendered to the child.

• Motor regression: Motor regression is the most common symptom at presentation with 65% of the 164 children reviewed by Constantini et al. exhibiting it (10). In younger children motor regression is manifested by slowing, then cessation in motor development. Toddlers frequently revert to crawling. Older children commonly have a history of frequent falling and loss of balance.  Nearly half will have associated gait abnormalities.
• Pain: Pain is another common complaint, occurring in 48% (10). The pain is typically mid-back at the level of the tumor, but pain referred out into the limb is not uncommon. It is not, however, in the crisp radicular pattern that one would expect when a nerve root is compressed. The pain can be particularly intrusive at night.
• Dysesthesia: Dyesthesia is the other common sensory complaint occurring in 32% (10). It can be unilateral or bilateral, depending on the location of the tumor.
• Progressive kyphoscoliosis: Kyphoscoliosis is present in 32% of children with IMSCTs (10).

Kyphoscoliosis:

• Bowel and bladder dysfunction: Bowel and bladder dysfunction has been reported with an incidence of 32% (35).  Typically it is manifest by symptoms of urinary retention (repeated UTIs, overflow incontinence).  Isolated bowel dysfunction is extremely rare and bowel dysfunction in general occurs only late.
• Recurrent URIs: Recurring uupper respiratory tract infections, nausea and vomiting, and change in voice are common symptoms in children with cervicomedullary tumors.

Patterns of evolution

• Slow evolution: Slow evolution of symptoms is the expected history to be obtained when evaluating a child with a benign IMSCT. Constantini found an average 11.6-month delay in diagnosis except for children under the age of 3 years (5.4-month delay), where regression in motor development was more evident (10).
• Rapid evolution: Symptoms evolve more rapidly with a malignant tumor (8).
• Lateralized: Lateralized symptoms are expected since nearly 90% of these tumors are either astrocytomas or gangliogliomas, and these tumors arise eccentrically.

Evaluation at Presentation

• See EVALUATION

Intervention

As long-term survival is expected, the preparation of a child harboring an IMSCT should be done with as much care as the situation allows so that the child is in the best clinical state possible and the surgeon has a complete set of information from which he or she can make knowledgeable treatment decisions.

Preparation for definitive intervention, non-emergent

Children who present with a slowly evolving symptom complex and without threatening signs of decompensation can be assessed systematically and prepared for treatment.

• Steroids: Steroids are started. It is expected that steroids will stabilize or improve the clinical status of the child for 10 days to 2 weeks; thus there should be adequate time to prepare properly for the chlld’s surgery.
• MRI: A scan of good quality, with and without gadolinium, is required.
• Consultations: Consultations are obtained from a pediatric oncologist and are considered for urology (history suggesting urinary tract dysfunction), pulmonology (history raises concern over respiratory status), otolaryngology (history or examination raises concern over vocal cord function) if indicated.
• Surgical team: The surgical team is assembled and communication of the unique needs of the surgery occurs (e.g., compatibility of anesthesia with monitoring needs; availability of monitoring personnel; availability of specialized equipment, including contact laser, ultrasonic aspirator, microscope, and intraoperative ultrasound.

Preparation for definitive intervention, emergent

Rarely a child will present with rapidly evolving paresis that mandates immediate treatment. The response is to arrange that the child’s surgery be done as soon as the needed information can be assembled. The goal of reversing the child’s neurological status must be tempered by the reality of the surgical challenge. Surgery on these tumors can only be done in a controlled setting with adequate information.

• IV steroid: A IV steroid is administered.
• MRI scan:  A scan with and without gadolinium is obtained as soon as possible. If the child is young and unable to tolerate an awake MRI, then the optimal solution is to arrange for anesthesia and operating room availability so that the child can be scanned and then moved immediately to the operating room for surgery.
• Surgery: Surgery within 18 hours is a reasonable goal. If this is not possible and the level of the tumor is known, then an option is a laminectomy and duraplasty with or without a myelotomy.

Tumor herniating out of a myelotomy: