Rapid-onset paraparesis and quadriparesis in patients with intramedullary spinal dermoid cysts: report of 10 cases

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Intramedullary dermoid cysts are rare tumors of the spinal cord. Presentation with rapid onset of paraparesis or quadriparesis (onset within 2 weeks) is rarer still. The authors present their experience in the management and outcome of patients with such a presentation.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>Patient records between 2000 and 2014 were retrospectively reviewed to identify those with intraspinal dermoid cysts who presented with rapid-onset paraparesis or quadriparesis. Their clinical, radiological, operative, and follow-up data were analyzed.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Of a total of 50 patients with intraspinal dermoid cysts managed during the study period, 10 (20%) presented with rapid-onset paraparesis or quadriparesis; 9 patients ranged in age from 8 months to 2 years, and 1 patient was 25 years old. A dermal sinus was seen in the lumbar region of 4 patients, the sacral region of 3, and the thoracic region of 1, and in 1 patient no sinus was found. All except 1 patient presented with rapid-onset paraparesis secondary to infection of the intramedullary dermoid cyst. One patient presented with rupture of a dermoid cyst with extension into the central canal up to the medulla. Early surgery was done soon after presentation in all except 2 patients. Among the 9 patients who underwent surgery (1 patient did not undergo surgery), total excision of the intramedullary dermoid cyst was done in 3 patients, near-total excision in 4 patients, and partial excision in 2 patients. Of the 9 patients who underwent surgery, 8 showed significant improvement in their neurological status, and 1 patient remained stable. The 1 patient who did not undergo surgery died as a result of an uncontrolled infection after being discharged to a local facility for management of wound infection.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Early recognition of a dermal sinus and the associated intraspinal dermoid cyst and timely surgical intervention can eliminate the chances of acute deterioration of neurological function. Even after an acute onset of paraparesis or quadriparesis, appropriate antibiotic therapy and prompt surgery can provide reasonably good outcomes in these patients.</jats:p></jats:sec>

Acute presentations of intradural lipomas: case reports and a review of the literature

“…casesAgeType of lesionDeteriorationTreatmentLate outcomeVadivelu et al, 2014 [12]217 mts, 26 mtsUndiagnosed dermal sinus + dermoid cyst + syrinxUndiagnosed dermal sinus + dermoid cystIntramedullary abscess with motor/sphincter deficitIntramedullary abscess with recurrent meningitis and hydrocephalusSurgery + antibiotic therapyAssistive device for walkingDevelopmental delay + VP shuntBhanage et al, 2015 [8]14 mtsDermal sinus and tethered filum terminaleLeg weakness and infection (dorsal and lumbosacral intramedullary abscess (D11-S3)Surgery + antibiotic therapy ( Mycobacterium tuberculosis) Left foot deformity with limping gait and a neurogenic bladderSingh et al, 2015 [11]3 (14.2%) out of a series of 21 cases9 mts to 15 yrs. (mean: 8.2 yrs)Dermal sinusMeningitis, intraspinal abscess and acute paraplegiaSurgery + antibiotic therapyPersistent neurological deficit in one case, persistent sphincter deficit in all 3 casesGirishan et al, 2016 [9]108 to 24 mts (one case: 25 yrs)Dermal sinus + intramedullary dermoid cystRapid onset paraparesis secondary to infection (9 cases) or rupture of dermoid cyst in one case (quadriparesis)Surgery + medical treatment (9 cases), only medical treatment (1 case)Significant neurological improvement (8 cases), stable deficits (1 case), death (1 case, no surgery)Rashid et al, 2016 [10]12 yrs.Dermal sinusInfection (myelitis/polyradiculitis) with full motor/sensitive/sphincter deficitSurgery + antibiotic therapyPermanent neurological deficits…”

A 2-year-old child with dorsal dermal sinus presenting with sudden onset of inability to stand

“…On postcontrast MRI, there is rim enhancement with features of solid component and septations. 10 Treatment of choice is excision of dermal sinus with drainage of abscess through a dorsal midline myelotomy at the site of maximum swelling and fluctuation followed by excision of lesion. 1 11 Non-infected dermoid has excellent prognosis, and in case of infected dermoid cyst, only 20% of case could recover completely.…”

Ruptured Spinal Dermoid Cyst

“…Spillage of dermoid contents into the subarachnoid space may result in aseptic chemical meningitis with profound irritative effects presumably secondary to fatty acids. 3 The goal of surgery is evacuation of the cyst contents with special care to avoid release of fatty elements in the subarachnoid space. The spinal dermoid cyst wall is usually tightly adherent to neural structures, and attempts at complete resection may lead to permanent neurologic damage.…”

“…The spinal dermoid cyst wall is usually tightly adherent to neural structures, and attempts at complete resection may lead to permanent neurologic damage. 2,3 DISCLOSURES…”

“…Rupture of a spinal dermoid is an emergency and early recognition and surgical intervention can decrease morbidity and mortality. Spillage of dermoid contents into the subarachnoid space may result in aseptic chemical meningitis with profound irritative effects presumably secondary to fatty acids 3 . The goal of surgery is evacuation of the cyst contents with special care to avoid release of fatty elements in the subarachnoid space.…”