Fatal intracranial hemorrhage as the presenting sign of acute promyelocytic leukemia: A case report.

Intracranial hemorrhage (ICH) can be a devastating medical event with numerous potential etiologies. In young people under age 40, ruptured vascular malformation is the most common cause of ICH. Without critical review of imaging and laboratory findings and clinical suspicion beyond vascular malformation, alternative etiologies of hemorrhage may be overlooked in the younger age group. Here we present a case of a 22-year-old male presenting with large ICH originally thought secondary to ruptured vascular malformation. After careful review of all imaging and laboratory findings, the patient was found to have hemorrhage secondary to acute promyelocytic leukemia (APL). Though ICH proved fatal in this case, early treatment of acute leukemia with appropriate chemotherapeutic agents and correction of coagulopathy could be life saving for patients with less severe intracranial injury.

Response of arteriovenous malformations to gamma knife therapy evaluated with pulsed arterial spin-labeling MRI perfusion.

OBJECTIVE: The goal of this study was to use pulsed arterial spin-labeling (PASL) MRI to evaluate the effect of gamma knife treatment on arteriovenous malformation (AVM) blood flow by measuring perfusion of the AVM nidus and nearby vascular territories. CONCLUSION: PASL can show and quantify the steal phenomena and the relative flow rates within the AVM nidus and may be used to follow AVM perfusion over time to assess treatment efficacy.

Simplified harvest of autologous pericranium for duraplasty in Chiari malformation Type I. Technical note.

The authors describe a method of harvesting autologous pericranium for duraplasty in patients with Chiari malformation Type I (CM-I) that avoids excessive exposure or a second incision. Nonautologous dural grafts have been associated with numerous complications including hemorrhage, bacteria and virus transmission, fatal Creutzfeldt-Jakob disease transmission, foreign body reaction, systemic immune response, excessive scarring, slower healing, premature graft dissolution, and wound dehiscence. Autogenous tissues have the advantage of being nonimmunogenic, nontoxic, readily available, and inexpensive. Pericranium is a preferred substrate because it is flexible, strong, and easily sutured for a watertight closure. Current literature supports the use of autogenous pericranium for dural grafting in CM-I procedures, but has heretofore failed to provide a method of harvest that avoids the complications associated with a larger exposure or second incision. The authors offer a simple alternative technique for using local pericranium in duraplasty for CM-I or other posterior fossa abnormalities.

Composite pleomorphic xanthoastrocytoma-ganglioglioma presenting as a suprasellar mass: case report.

OBJECTIVE AND IMPORTANCE: Composite pleomorphic xanthoastrocytoma (PXA)-ganglioglioma (GG) is a recently recognized, rare type of neoplasm that most commonly presents as a temporal seizure focus among male patients less than 30 years of age. This case represents the only reported suprasellar presentation, with the youngest reported age at diagnosis. CLINICAL PRESENTATION: We present the case of a 12-year-old boy with new-onset diplopia who was diagnosed as having a large suprasellar mass, which was presumptively designated a craniopharyngioma on the basis of its clinical and radiological appearance. INTERVENTION: Gross total resection of a well-encapsulated tumor with adjacent cysts was achieved, without postoperative neurological deficits. Frozen-section studies suggested pilocytic astrocytoma; however, the final histological diagnosis was composite PXA-GG. CONCLUSION: Composite PXA-GG, although extremely rare, may present as a pediatric suprasellar mass. The histopathological diagnosis is contingent on the demonstration of distinct coincident PXA and GG components. The prognosis after gross subtotal resection is likely favorable; however, long-term follow-up monitoring is indicated for these rare neoplasms.

Treatment of atlantoaxial instability in pediatric patients.

The atlantoaxial region has been extensively described as a spinal segment especially prone to injury in children. In this clinical review, the authors evaluate and summarize the management of 23 pediatric cases of atlantoaxial instability treated between March 1990 and October 2002. Four broad categories of atlantoaxial problems were observed-atlantoaxial rotatory subluxation in six patients, anterior-posterior atlantoaxial instability caused by ligamentous injury or congenital ligamentous laxity (10 patients), atlantoaxial fracture with or without dislocation (five patients), and atlantooccipital dislocation (two patients). Most cases (60.9%) were treated without surgical intervention, resulting in excellent outcomes; however, 21.7% of cases were treated with a cervical halo (mean patient age 72.6 months) alone for 3 months. Various techniques of surgical stabilization including transarticular screws with sublaminar wiring, transoral decompression with posterior plating, and laminectomy with Steinmann pin occipital-cervical fusion were used with good results. Both patients with atlantooccipital dislocation underwent immediate Locksley occipital-cervical fusion, with marked neurological improvement. Individualized case management must be based on clinical presentation, with internal fixation being the last resort.

Effects of antibiotic-impregnated external ventricular drains on bacterial culture results: an in vitro analysis.

OBJECT: Treatment of ventriculoperitoneal shunt infections frequently requires placement of an external ventricular drain (EVD). Surveillance specimens obtained from antibiotic-impregnated (AI) EVDs may be less likely to demonstrate bacterial growth, potentially resulting in undertreatment of an infection. The purpose of this study was to assess whether AI EVDs had any significant effect on bacterial culture results compared with nonantibiotic-impregnated (NAI) EVDs. METHODS: In vitro assays were performed using AI EVDs containing minocycline and rifampin (VentriClear II, Medtronic) and NAI EVD controls (Bioglide, Medtronic). The presence of antibiotics was evaluated via capillary electrophoresis of sterile saline drawn from AI and NAI EVDs after predefined incubation intervals. Antimicrobial activity was assessed by evaluating zones of inhibition created by the catheter aspirates on plates inoculated with a quality control strain of Staphylococcus epidermidis (American Type Culture Collection strain 12228). To determine the effects of cultures drawn through AI compared with NAI EVDs, the quality control strain was then incubated within 4 new AI and 4 new NAI EVDs for predefined intervals before being plated on culture media. Spread and streak plate culture results from each type of catheter were compared at each time interval. RESULTS: Capillary electrophoresis showed that more minocycline than rifampin was eluted from the AI EVDs. Sterile saline samples incubated within the AI EVDs demonstrated zones of growth inhibition when placed on plates of S. epidermidis at all time intervals tested. No zones of inhibition were noted on NAI EVD control plates. When a standardized inoculum of S. epidermidis was drawn through AI and NAI EVDs, antimicrobial effects were observed after incubation in the AI EVD group only. Colony counting demonstrated that significantly fewer colonies resulted from samples drawn through AI compared with NAI EVDs at the multiple time intervals. Similarly, streak plating yielded a statistically significant number of false-negative results from AI compared with NAI EVDs at 2 time intervals. CONCLUSIONS: The findings in the current study indicate that the risk of a false-negative culture result may be increased when a CSF sample is drawn through an AI catheter. In the management of a known shunt infection, a false-negative result from an EVD culture specimen may lead to an inappropriately short duration of antibiotic therapy. These data have significant clinical implications, particularly given the widespread use of AI drains and the current high rates of shunt reinfection after EVD use worldwide.

A review of surgical intervention in the setting of traumatic central cord syndrome.

BACKGROUND CONTEXT: Surgical treatment in the setting of central cord syndrome (CCS) has become safer since Schneider's original description. It is generally accepted that a decompressive surgical intervention is a valid treatment option in a patient with CCS and radiographic evidence of spinal cord compression. The optimal timing of surgical intervention for CCS remains controversial. PURPOSE: To review a single institution's experience managing CCS, with particular emphasis on surgical versus medical management, timing of surgery, neurologic outcomes, hospital length of stay, and complications. STUDY DESIGN: Retrospective review. PATIENT SAMPLE: One hundred twenty-six patients diagnosed with CCS were treated at Wake Forest University Baptist Medical Center between June 1985 and September 2006. OUTCOME MEASURES: Neurological outcomes were measured using the Frankel grading scale. Other outcome measures included hospital and intensive care unit (ICU) length of stay and complication profiles. METHODS: A retrospective chart review was performed on patients admitted to Wake Forest University Baptist Medical Center with the diagnosis of traumatic central cord injury from June 1985 to September 2006 with institutional review board approval. Neurologic status was recorded on presentation and at maximum follow-up using the Frankel classification. The surgical cohort was stratified into three subgroups with regard to the timing of surgical intervention after injury: surgery less than 24 hours after injury, surgery greater than 24 hours after injury but during the initial admission, and delayed operative intervention on a second hospital admission. Other variables collected included ICU and hospital length of stay and complication profiles. Data analyses were performed using SPSS (SPSS, Chicago, IL, USA) and Excel 2002 (Microsoft, Seattle, WA, USA). RESULTS: A total of 126 patients treated for CCS were reviewed. Sixty-seven patients received surgery compared with 59 patients managed nonoperatively. Of those managed operatively, 16 patients received surgery within 24 hours of the time of injury. There were 34 patients who received surgery greater than 24 hours after the time of injury but during their initial admission with a mean time to surgery of 6.4 days (5-52 days). There were 17 patients who received their operation on a second hospital admission with a mean time interval of 137 days between injury and surgery (3-209). Mean follow-up was 32 months (1-210 months). An improvement in Frankel grade was seen in the overall operative cohort compared with those patients who received medical management alone. No statistically significant difference in neurologic outcome using Frankel grades was identified between the surgical subgroups with regard to timing of surgery. A trend toward decreased length of stay was seen in the surgical subgroup that received surgery during their initial admission. No statistically significant difference was identified between complication rates for the operative and nonoperative groups; however, a trend toward fewer complications and deaths was seen in those who received surgery in the first 24 hours or during the initial hospitalization. CONCLUSIONS: Surgical treatment in the setting of CCS has become safer since Schneider's original description. Acknowledging its numerous limitations, this retrospective study supports surgical intervention in the setting of CCS as a safe effective management option. Improved Frankel grades were identified in those patients managed surgically compared with those receiving medical management alone. The data further shed light on the safety and potential benefits of early operative intervention for acute CCS compared with delayed surgical treatment. A prospective randomized controlled trial is needed to definitively compare surgical versus medical management and/or early versus delayed surgical treatment in the setting of traumatic CCS.

Rare intraparenchymal choroid plexus carcinoma resembling atypical teratoid/rhabdoid tumor diagnosed by immunostaining for INI1 protein.

The authors present the case of a rare extraventricular, intraparenchymal choroid plexus carcinoma (CPC). This 6-year-old girl presented to the emergency department with a 1-week history of headaches, nausea, and vomiting. Imaging studies revealed an intraaxial cystic and solid mass located in the right frontal lobe with central nodular enhancement and minimally enhancing cyst walls. Gross-total resection was accomplished via craniotomy without complications. The initial pathological diagnosis was atypical teratoid/rhabdoid tumor (AT/RT); however, immunostaining for INI1 protein (using the BAF47/SNF5 antibody) showed retention of nuclear staining in the tumor cells, resulting in a change in the diagnosis to CPC. There was no evidence of recurrence at the last follow-up 2.5 years after treatment, which supports the diagnosis of CPC over AT/RT. This case emphasizes the importance of immunostaining for INI1 protein for distinguishing CPC from AT/RT in cases with atypical or indeterminate features.

Occult dural arteriovenous fistula causing rapidly progressive conus medullaris syndrome and paraplegia after lumbar microdiscectomy.

BACKGROUND CONTEXT: A spinal dural arteriovenous fistula (DAVF) is a known cause of venous congestive myelopathy. These lesions are thought to be acquired. There exist two prior reports that describe DAVF presenting with slowly progressive myelopathy years after lumbar disc surgery. PURPOSE: We report the first case of a preexistent, asymptomatic DAVF that became acutely symptomatic after lumbar microdiscectomy causing rapidly progressive conus medullaris syndrome and paraplegia. STUDY DESIGN: Case report. METHODS: A 53-year-old Caucasian male presented with urinary retention, fecal incontinence, and progressive loss of function in bilateral lower extremities less than 3 months after a lumbar discectomy at an outside facility. The patient underwent microdiscectomy at L4/L5 and L5/S1 for left-sided radicular symptoms and evidence of herniated discs on magnetic resonance imaging (MRI). The patient's preoperative pain improved after the discectomy, but his bowel, bladder, and lower extremity function deteriorated steadily after discharge. Postoperative MRI showed salient serpentine vessels in the region of L1 and L2 with an enlarged T2 bright conus medullaris. Retrospective review of preoperative MRI revealed the presence of similar but less pronounced findings. A spinal arteriogram confirmed concerns of an arteriovenous malformation. The patient was transferred to our facility for definitive management of his DAVF via catheter embolization. RESULTS: Complete fistula obliteration was confirmed at the time of embolization and at 8 weeks follow-up with selective spinal angiography. The appearance of the conus medullaris normalized on follow-up MRI. The patient made a slow but significant recovery in rehabilitation. He regained the ability to independently ambulate but remains significantly disabled secondary to residual lower extremity weakness and spasticity. He continues self-catheterization for persistent neurogenic bladder dysfunction. CONCLUSIONS: This represents the first case of an occult spinal DAVF becoming acutely symptomatic after lumbar disc surgery. Although the etiology of the lesion and its symptomatic progression remains unknown, an alteration of blood flow through the DAVF as a result of surgery may have resulted in progressive venous congestive myelopathy. The present case highlights the importance of considering a spinal DAVF in the differential diagnosis of any patient with signs of myelopathy or conus medullaris syndrome after lumbar discectomy. In addition, this case underscores the gravity of recognizing subtle features that suggest the presence of an occult vascular malformation on preoperative imaging, as symptomatic progression of these lesions carries significant potential morbidity.

Ruptured anterior spinal artery aneurysm: a case report.

We present a case of a subarachnoid hemorrhage and vasospasm secondary to a ruptured anterior spinal artery aneurysm associated with a Chiari 1 malformation. To our knowledge this is the first reported spinal artery aneurysm with this association.

Angiofollicular lymph node hyperplasia resembling a spinal nerve sheath tumor: a rare case of Castleman's disease.

BACKGROUND CONTEXT: Angiofollicular lymph node hyperplasia (Castleman's disease) is a lymphoproliferative disorder of unknown etiology. Although uncommon, the localized form of this disease can manifest in the central nervous system, typically as a meningeal-based intracranial lesion. Castleman's disease involving the spine is exceedingly rare. This represents only the second reported case of a patient with Castleman's disease whose presentation mimicked that of a spinal nerve sheath tumor. PURPOSE: We report a rare case of angiofollicular lymph node hyperplasia that mimicked a spinal nerve sheath tumor and was treated with gross total resection. STUDY DESIGN: Case report. PATIENT SAMPLE: A 31-year-old female with angiofollicular lymph node hyperplasia presenting with a paraspinal mass. OUTCOME MEASURES: The patient's outcome was based on clinical history, physical and radiographic examinations. METHODS: A 31-year-old woman with a mediastinal mass previously diagnosed as sarcoid via biopsy presented with the new onset of radicular pain and radiographic enlargement of her mass. Magnetic resonance imaging demonstrated a 4.3x5.7x6.0 cm homogenously enhancing soft-tissue lesion that emanated from and widened the T3/T4 neural foramen on the left. The differential diagnosis based on the location of the lesion and imaging characteristics included schwannoma, neurofibroma, paraganglioma, sarcoid, and lymphoma. Gross total resection was performed via thoracotomy. Histological examination identified angiofollicular lymph node hyperplasia (Castleman's disease) of the hyaline-vascular subtype. The patient did not receive adjuvant chemotherapy or radiation therapy. RESULTS: The patient had resolution of her symptoms without any clinical or radiographic evidence of recurrence at 1-year follow-up using magnetic resonance imaging with and without contrast. CONCLUSIONS: Castleman's disease is a rare pathologic entity that should be considered in the differential diagnosis of a patient with a paraspinal mass. Spine surgeons should be aware of this diagnosis as it has treatment and follow-up implications that differ from the neoplasms it can mimic. Complete surgical excision is an effective treatment for solitary lesions. Screening for concurrent and future malignancies in these patients is prudent.