Munir Elias 20-12-2013
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The patient came to the clinic 19-June-2006 complaining of difficulty in walking, dragging his right lower limb with loss of sensation below D2 more pronounced in the left lower limb. The patient started to to complain of this at 6 years age with sudden onset of paraplegia. The patient was considered as a case of syringomyelia and shunted twice. The first time it was  2002. Deterioration took place 2004 and another time was shunted. He improved for 2 months, but then start to deteriorate with urine retention.

MRI performed recently, showing a mass extramedullary anterior to the spinal cord, more to the right at the level of D2-4 resembling an ependymoma, but it could be an astrocytoma with cystic degeneration.

On examination: analgesia below D2 with crude sensation preserved in the right lower limb, which was more weak than the left with the quadriceps 4/5, knee abduction and adduction 3/5 with inverted foot and dorsi and planterflexion 3/5 of the left foot. The left foot had power of the muscles 4/5.

The old incision was refreshed and further drilling to the right side was performed to attack the spinal cord from the right. The D4 right dentate ligament was released and bisected. The atrophied right Th4 root was resected to have ample to the latero-anterior parts of the spinal cord. The anteriorly located cystic component was opened and evacuated. The violate soft consistency part of the tumor had no proper cleavage to separated it from the lower pole of the cyst, which was stuck and diffusely infiltrating the spinal cord . Biopsy was taken  and frozen section confirmed the presence of low-grade astrocytoma.

Inspection of the spinal cord at the lower edge of the pathology, showed some firm consistency, than usual, but without mass effect. The sac was dissected and removed in most parts. It was possible to see the previously inserted shunt, which was full of adhesions and has continuity to the spinal cord mass. It was decided not to touch it. The right  Th1-2-3 were seen hanging free with severe atrophy. The spinal cord was stuck to the dura in the left side and it was decided not to release it, to minimize the surgical trauma. Routine closure of the wound.  

The patient showed immediate postoperative considerable deterioration of his neurologic deficit. But sensation of the left foot became better and sensation of the right foot deteriorated with gross weakness both lower limbs.

The final histologic verification was that of neurenteric cyst, which was confirmed by immunohistologic studies.

The patient progressed the fifth postoperative day CSF leak from the wound. Tension sutures were applied at the ooze point. The next day CSF leak took place above the previous level and another tension sutures applied.

The patient during the night round 19-Jly-2006 progressed melena with diarrhea with hypotension and Hb 8.4 mg/dL. Decadron which was in tapering stage stopped and nexium with blood and FFP transfusion were started at the ICU.

Endoscopy of the stomach and the duodenum demonstrated "old ulcer" in the duodenum and conservative measures were undertaken. The patient bled three times at 3 days interval and the last one was the night of 26-July-2006, for what urgent laparatomy was performed and closure of the ulcer and control heamostasis was performed.

During this time the spinal surgery wound healed properly with no CSF leak for 2 days.


1. It is hard to tell which tumor in the MRI before the surgery, as in this case. This tumor was noted in the early MRI , but was not reported and evacuation of the cyst did not arrest the progression of the disease.

2. Syringomeylia usually stay intramedullary. The existence of extramedullary extension of the cyst must hold suspicion about the nature of the lesion, which proved to be neurenteric cyst.

3. Despite the fact, that the left 2/3 of the spinal cord was not exposed to the field of the surgery, deterioration of the motor function took place at that side. It is mostly reactionary in the immediate postoperative period. Time will tel. The patient is covered with dexametasone 16 mg 8h.

4. What is neurenteric cyst?
In summery: Neurenteric cysts are infrequently reported congenital abnormalities believed to be derived from an abnormal connection between the primitive endoderm and ectoderm. Children present more commonly with cutaneous stigmata of occult spinal dysraphism(OSD) whereas adults present primarily with pain. Neurological deficit as a presenting symptom is less common, a finding that reflects the slow growth of these lesions. In most patients some form of vertebral anomaly is associated with the cystic lesions, including Klippel–Feil abnormalities. There is a high incidence of associated forms of OSD including split cord malformation, lipoma, dermal sinus tract, and tethered spinal cord. Neurenteric cysts are more common in the cervical region and in a position ventral to the cord.
These cysts most commonly occur as intradural, extramedullary masses in the thoracolumbar region, situated dorsal to the spinal cord. Complete excision of the neurenteric cyst remains the treatment of choice, as subtotal excision is associated with recurrence. For more details click here!

5. The patient is a young man and he never complain of abdominal problems. The endoscopic finding of chronic ulcer hold the suspension of the presence of another anomaly in the duodenum, which could be related with his primary pathology.

Cytokeratin: positive on the lining cells. EMA: positive on the lining cells. EMA: positive on the lining cells. H&E high power
  H&E low power H&E high power CEA: positive on the lining cells.  H&E high power
Final histologic diagnosis: Spinal cord tumor with extramedullary and intramedullary cystic component:
Enteric cyst ( enterogenous cyst), no malignancy. Ki-67: negative on glial cells.
Dr. Hussam Abu-Farsakh, M.D. Consultant Pathologist and Cytopathologist. American boards of Anatomic, Clinical Pathology and Cytopathology.

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