CAUDA EQUINA SYNDOME DUE TO SPINAL SCHWANNOMA WITH INTRATUMORAL HEMORRHAGE

SUMMARY Cauda equina syndrome is defined as the group of symptoms that includes low-back pain, sciatica, saddle anesthesia, decreased rectal tone, perianal areflexia or hiporeflexia, bowel and bladder dysfunction, and lower-extremity weakness. There are several causes of this syndrome including trauma, central disc protrusion, hemorrhage, and the neoplastic invasion was occurred rarely. In this case we report a cauda equina syndrome caused by intratumoral hemorrhage of spinal schwannoma at the level of L4. Surgical resection of the tumor significantly improved the neurological symptoms of the patients.

Key words: Cauda equina syndrome, intratumoral hemorrhage, spinal schwannoma

ÖZET

Kauda ekuina sendromu; bel ve bacak ağrısı, eyer tarzı hipoestezi, 38 anal hiporefleksi, bağırsak ve mesane fonksiyon bozuklukları ile birlikte alt ekstremitelerde kuvvetsizlik semptomlarından oluşan klinik bir tablodur. Bu sendrom sıklıkla travma, santral disk protrüzyonu ve kanamalara bağlı gelişirken daha nadir olarak da tümöral lezyonlar nedeniyle oluşabilmektedir. Bu vaka takdiminde L4 seviyesine yerleşmiş bir intradural schwannoma olgusunun, tümör içine kanaması ile oluşan kauda ekuina sendromu sunulmaktadır..

Anahtar kelimeler:: auda ekuina sendromu, tümör içine kanama, spinal schwannoma

INTRODUCTION:

Cauda Equina Syndrome (CES) is a neurosurgical emergency caused by compression of nerves below the level of conus medullaris. The signs and symptoms are complex with pain (unilateral or bilateral sciatica and low back pain) and findings of sensory (in saddle and perianal region), motor (weakness in lower extremity) and reflex (bulbocavernous, patellar and achilles) dysfunctions (1,9).Schwannomas in Cauda Equina are generally sporadic, but they can also be seen in neurofibromatosis type2. They arise from the transition zone of central and peripheral myelin sheaths called Oberstainer-Redlich zone (1). Peak age at presentation is 4o years with progressive symptoms indicating slowly growing tumor. Spinal Schwannomas commonly affects sensory nerve roots in the cervical and lomber regions (5). Schwannomas reported being 60 associated with cauda equina syndrome because of subarachnoid or subdural hematomas, however association with intratumoral hemorrhage is unique (3, 7). In the following report, a patient with cauda equina syndrome caused by an intratumoral hemorrhage was reported.

CASE REPORT:

24 years-old female was admitted to the neurosurgery clinic with complaint of severe back pain, paraparesis and urinary and fecal incontinence. She was suffering from low back pain since 2 years which was improved 2 days ago. Neurological examination showed severe weakness of her bilateral lower extremities and bilateral sensory loss under the L1 level with absence of rectal tonus. Magnetic resonance imaging (MRI) demonstrated an intradural lesion, hyperintense on T1 weighted She was operated in emergency bases and L4 laminectomy was performed. The hemorrhage tumor was seen in the cauda fibers (Figure-2) and total resection of the tumor was achieved with microsurgical techniques in the expense of an involved nerve root. Histopathological evaluation indicated schwannoma with positive S-100 immunostaining. In the early postoperative period, although her neurological deficits did not improved, there were no pain. She was taken into rehabilitation program after her discharge from the ward at 3rd postoperative day. During the follow up period, total recovery in her neurological status could be achieved 6 months after the surgery..

DISCUSSION:

Cauda equina syndrome is a very specific constellation of symptoms including sciatica, low back pain, saddle and perianal hypoesthesia or analgesia; decreased rectal tonus; lack of bulbocavernous, patellar, and achilles reflexes; bowel and bladder dysfunction and lower extremity weaknesses (1). CES was first reported in 1934 by Mixter and Barr (10). The most common cause of CES is central lumbar disc herniations (9). However intratumoral hemorrhage caused by ecstatic and hyalinized blood vessels in a neoplasm was also reported in the etiology (6). Among all spinal tumors, incidence of intratumoral hemorrhage is highest among epandymomas and followed by schwannomas (6). Tumors around the cauda equine region may apply a traction force rostral to caudal direction along the spinal axis, facilitating an intratumoral or a subarachnoid bleeding (6). Tumors of the region can reach to considerable sizes without any symptoms as the area is large and the nerve roots are mobile. Mean duration for diagnosis was reported to be 4 years by Cervoni et al (2). Spinal schwannomas are generally slow growing tumors causing progressive symptoms (5, 8).

Cauda equina schwannomas presenting with acute paraparesis and urinary incontinence are usually associated with intratumoral hemorrhages. In the literature, trauma or anticoagulant drug was blamed for causing subarachnoidal or subdural hemorrhages in cases with spinal schwannomas, which in turn resulted in CES (4, 6) Our case is unique for presenting CES caused by an intratumoral hemorrhage of spinal schwannoma without any precipitating factors. Literature review also revealed unusual bleeding site for spinal schwannoma, thoracolumbar junction versus L4 level. Intratumoral hemorrhage causing CES is quite rare but still an emergent condition that should not be missed. Therefore, in case of CES, MR evaluation should be performed first as soon as possible for unforeseeable pathologies and early surgical decompression of the nerve roots should be aimed, in this case, with tumor resection in order to improve neurological outcome.