Category Archives: Articles

Update: Desmopressin


Desmopressin, sold under the trade name DDAVP among others, is a medication used to treat diabetes insipidus, bedwetting, hemophilia A, von Willebrand disease, and high blood urea levels.

In hemophilia A and von Willebrand disease, it should only be used for mild to moderate cases.

It may be given in the nose, by injection into a vein, by mouth, or under the tongue.

More potent and longer acting than vasopressin.

In patients with central diabetes insipidus DI, desmopressin is the drug of choice.

A synthetic analogue of antidiuretic hormone (ADH), desmopressin is available in subcutaneous, IV, intranasal, and oral preparations.

Generally, it can be administered 2-3 times per day. Patients may require hospitalization to establish fluid needs. Frequent electrolyte monitoring is recommended during the initial phase of treatment.

Alternatives to desmopressin as pharmacologic therapy for DI include synthetic vasopressin and the nonhormonal agents chlorpropamide, carbamazepine, clofibrate (no longer on the US market), thiazides, and nonsteroidal anti-inflammatory drugs (NSAIDs). Because of side effects, carbamazepine is rarely used, being employed only when all other measures prove unsatisfactory. NSAIDs (eg, indomethacin) may be used in nephrogenic DI, but only when no better options exist. In central DI, the primary problem is a hormone deficiency; therefore, physiologic replacement with desmopressin is usually effective. Use a nonhormonal drug for central DI if response is incomplete or desmopressin is too expensive.


Desmopressin (DDAVP) is a well-known hemostatic agent, and recent guidelines already suggest its use in individuals exposed to antiplateletdrugs.

Francoeur et al. hypothesized that DDAVP administration in patients with SAH at admission would be associated with lower risks of intracranial aneurysm rebleeding.

They performed an observational cohort study of patients enrolled in the Columbia University SAH Outcome Project between August 1996 and July 2015. They compared the rate of rebleeding between patients who were and those who were not treated with DDAVP. After adjustment for known predictors, logistic regression was used to measure the association between treatment with DDAVP and risks of rebleeding.

Among 1639 patients with SAH, 12% were treated with DDAVP. The main indication for treatment was suspected exposure to an antiplatelet agent. The overall incidence of rebleeding was 9% (1% among patients treated with DDAVP compared with 8% among those not treated). After adjustment for antiplatelet use and known predictors, treatment with DDAVP was associated with a 45% reduction in the risks of rebleeding (adjusted OR 0.55, 95% CI 0.27-0.97). DDAVP was associated with a higher incidence of hyponatremia but not with thrombotic events or delayed cerebral ischemia.

Treatment with DDAVP was associated with a lower risk of rebleeding among patients with SAH. These findings support further study of DDAVP as first-line therapy for medical hemostasis in patients with SAH 1).

Desmopressin seems to be an effective and accepted as well as frequently adopted measure to antagonize the aspirin effect on platelet function during various major surgical procedures 2).

Bilateral inferior petrosal sinus sampling (IPSS) with desmopressin is a sensitive approach in the diagnosis of Cushing’s disease (CD) and has moderate accuracy in tumour lateralization, making it an alternative choice to IPSS with CRH 3).


Francoeur CL, Roh D, Schmidt JM, Mayer SA, Falo MC, Agarwal S, Connolly ES, Claassen J, Elkind MSV, Park S. Desmopressin administration and rebleeding in subarachnoid hemorrhage: analysis of an observational prospective database. J Neurosurg. 2018 Feb 2:1-7. doi: 10.3171/2017.7.JNS17990. [Epub ahead of print] PubMed PMID: 29393750.


Korinth MC, Gilsbach JM, Weinzierl MR. Low-dose aspirin before spinal surgery: results of a survey among neurosurgeons in Germany. Eur Spine J. 2007 Mar;16(3):365-72. Epub 2006 Sep 5. PubMed PMID: 16953446; PubMed Central PMCID: PMC2200713.


Feng M, Liu Z, Liu X, Zhang X, Bao X, Yao Y, Deng K, Xing B, Lian W, Zhu H, Lu L, Wang R. Tumour lateralization in Cushing’s disease by inferior petrosal sinus sampling with desmopressin. Clin Endocrinol (Oxf). 2018 Feb;88(2):251-257. doi: 10.1111/cen.13505. Epub 2017 Nov 27. PubMed PMID: 29080355.

Update: Tethered Cord Syndrome in Adulthood

Tethered Cord Syndrome in Adulthood

Symptoms related to a congenital tethered cord occur most commonly in childhood, so it was initially regarded as a pediatric problem; but in many patients, the diagnosis is not established until symptoms manifest in adulthood.

The number of adults in whom congenital TCS is diagnosed continues to grow as a result of better imaging and recognition of this syndrome. Pediatric TCS has been well studied in the literature, but much of the information regarding the adult population is still being defined. Patients who never undergo treatment for TCS likely have an elevated risk of developing symptoms with advancing age 1).


Adult tethered cord syndrome is a rare neurological disorder that classically presents with back or leg pain, weakness, and urinary dysfunction. Spinal cord tethering has been associated with acquired Chiari malformations.


Radiographically: low conus medullaris (below L2) and thickened filum terminale. NB:apparent filum terminale diameter on CT myelogram may vary with concentration of contrast material.

Preoperative cystometrogram is strongly recommended, especially if the patient seems continent (postoperative changes in bladder function are not uncommon, possibly due to stretching of the lower fibers of the cauda equina).

Differential diagnosis

It is difficult to differentiate a tethered cord from a congenitally low lying conus (filum diameter is generally normal in the latter).


Standard treatment for TCS diagnosed in adulthood remains controversial. Surgical intervention is usually indicated based on an expected natural history of disease progression in the absence of treatment.

Some adults with TCS decline surgery despite severe neurologic deficit 2).

Surgical treatment

If the only abnormality is a thickened, shortened filum terminale, then a limited lumbosacral laminectomy may suffice, with division of the filum once identified.

If a lipoma is found, it may be removed with the filum if it separates easily from neural tissues.

The filum is differentiated from nerve roots by presence of characteritics squiggly vessel on surface of filum. Also, under the microscope, the filum has a distinctively whiter appearance than the nerve roots, and ligamentous-like strands can be seen running through it. NB: intra-op electrical stimulation and recording of anal sphincter EMG are more definitive.


In the series of Gao et al. all patients received general anesthesia and took their prone position, neural electrophysiological monitoring electrode were then placed, followed by the acquisition and collection of muscle electromyography signals from the anal sphincter, bilateral musculus vastus lateralis, gastrocnemius and mesothenar. A total of 72 cases applied positive straight incision, 10 cases of lumbosacral lipoma with longitudinal incision. After exposing the dura mater spinalis, it was cut from the normal anatomical structure to the lesion. Cauda equina was managed by sharp releasing adhesion under the nerve electrophysiological monitoring, tumors were removed with the use of medical ultrasonic dissector. After the tumor was removed, the dura mater spinalis with low tonus was closed by water, and the dura mater spinalis with high tonus was formed by the autogenous fascia. For patients combined with subcutaneous giant lipoma in the lumbosacral region, the subcutaneous tumor was removed, and the drainage tube was placed into the left empty cavity, followed by pressurized dressing and vacuum aspiration 3).


Surgical release is usually good for pain relief. However, it is poor for return of bladder function.

Results of clinical studies of surgical intervention in adulthood are encouraging 4)5) 6).

It is safe and effective for improving pain and neurological status in the majority of patients; however, patients who have undergone previous intradural detethering procedures in general fare less well, and considerable judgment is required in their management 7).

In a multivariate regression model, laminectomy, bladder dysfunction when associated to muscular weakness, and long-term (>6 months) symptoms were selected as the independent risk factors associated with poor or minimally improved (almost unchanged) surgical outcomes. When the urodynamic test showed overactive detrusor muscle, no improvement was recorded in postoperative urodynamic test. Laminoplasty (or hemilaminectomy), short-term (<6 months) symptoms, patients without lipomas, and presentation with moderate or mild symptoms seem to be proper predictors for good surgical outcomes. Further prospective studies are necessary to investigate these findings systematically. Urodynamic study can be used as a predictive tool for close follow-up of asymptomatic adult patients involved with TCS 8).

Case reports

A 68-year-old man with a history of distant T12-level spinal cord injury who presented with two weeks of progressive bilateral lower extremity weakness. The patient underwent a T12-L1 laminectomy in 1977, complicated by arachnoiditis and syringomyelia, with eventual placement of a syringo-pleural shunt. He remained neurologically stable until 2012, when he underwent a suboccipital craniectomy for Chiari decompression for new-onset headache and dysphagia. Ten days later, the patient noted progressive leg weakness and radiographic evidence of spinal cord tethering at the T11-T12 level. A T10-L1 laminectomy and medical facetectomy was undertaken for detethering with postoperative recovery of ambulatory function with assistance.

The patient presented with an unusual acquisition of tethered cord syndrome. The tethering of the spinal cord may have been triggered by arachnoid adhesions from initial lumbar surgery 35 years prior to presentation and subsequently exacerbated by alterations of CSF dynamics following Chiari decompression. Given the potentially devastating sequelae of tethered cord syndrome, investigation of CSF flow dynamics may be beneficial prior to operative intervention in patients with risk factors for a tethered cord who present with adult-onset Chiari malformation 9).

1) , 4)

Rajpal S, Tubbs RS, George T, Oakes WJ, Fuchs HE, Hadley MN, Iskandar BJ. Tethered cord due to spina bifida occulta presenting in adulthood: a tricenter review of 61 patients. J Neurosurg Spine. 2007 Mar;6(3):210-5. PubMed PMID: 17355019.

Düz B, Gocmen S, Secer HI, Basal S, Gönül E. Tethered cord syndrome in adulthood. J Spinal Cord Med. 2008;31(3):272-8. PubMed PMID: 18795476; PubMed Central PMCID: PMC2565560.

Gao J, Kong X, Li Z, Wang T, Li Y. Surgical treatments on adult tethered cord syndrome: A retrospective study. Medicine (Baltimore). 2016 Nov;95(46):e5454. PubMed PMID: 27861396; PubMed Central PMCID: PMC5120953.
5) , 7)

Lee GY, Paradiso G, Tator CH, Gentili F, Massicotte EM, Fehlings MG. Surgical management of tethered cord syndrome in adults: indications, techniques, and long-term outcomes in 60 patients. J Neurosurg Spine. 2006 Feb;4(2):123-31. PubMed PMID: 16506479.

van Leeuwen R, Notermans NC, Vandertop WP. Surgery in adults with tethered cord syndrome: outcome study with independent clinical review. J Neurosurg. 2001 Apr;94(2 Suppl):205-9. PubMed PMID: 11302621.

Abdallah A, Emel E, Abdallah BG, Asiltürk M, Sofuoğlu ÖE. Factors affecting the surgical outcomes of tethered cord syndrome in adults: a retrospective study. Neurosurg Rev. 2018 Jan;41(1):229-239. doi: 10.1007/s10143-017-0842-z. Epub 2017 Mar 14. PubMed PMID: 28293750.

Jackson C, Yang BW, Bi WL, Chiocca EA, Groff MW. Adult tethered cord syndrome following Chiari decompression. World Neurosurg. 2018 Jan 31. pii: S1878-8750(18)30208-0. doi: 10.1016/j.wneu.2018.01.165. [Epub ahead of print] PubMed PMID: 29409774.

Update: L-Carnitine


Forty patients with severe traumatic brain injury were randomized into 2 groups. The l-carnitine (LCA-) group received standard treatment with placebo while the (LCA+) group received l-Carnitine 2g/day for one week. Neuron specific enolase (NSE) was measured on days 1, 3 and 7 after the initiation of the study. Neurocognitive and neurobehavioral disorders were recorded on the first and third months.

Neurocognitive function and NSE significantly improved within one week in both groups. Patient mortality was similar in LCA+ and LCA- groups (P value: 0.76). Brain edema was present in 7 patients in LCA+ group and 13 patients in LCA-group (P value: 0.044). While there was no difference in NSE levels between the two groups. Neurological function was preserved in the LCA+ group with an exception of attention deficit, which was frequent in the LCA+ group.

Mahmoodpoor et al. concluded that despite improvements in neurobehavioral function and the degree of cerebral edema, 7-days of treatment with l-Carnitine failed to reduce serum NSE levels or improve mortality rate at 90days in patients with TBI 1).

There is evidence in the literature for mitochondrial dysfunction in Parkinson’s disease as well as fatty acid beta-oxidation, involving l-carnitine.

Gill et al. investigated l-carnitine in the context of microglial activation, suggesting a potential new strategy of supplementation for PD patients. Preliminary results from this studies suggest that the treatment of activated microglia with the endogenous antioxidant l-carnitine can reverse the effects of detrimental neuroinflammation in vitro 2).


Mahmoodpoor A, Shokouhi G, Hamishehkar H, Soleimanpour H, Sanaie S, Porhomayon J, Rasouli F, Nader ND. A pilot trial of l-carnitine in patients with traumatic brain injury: Effects on biomarkers of injury. J Crit Care. 2018 Feb 9;45:128-132. doi: 10.1016/j.jcrc.2018.01.029. [Epub ahead of print] PubMed PMID: 29454227.

Gill EL, Raman S, Yost RA, Garrett TJ, Vedam-Mai V. l-Carnitine Inhibits Lipopolysaccharide-Induced Nitric Oxide Production of SIM-A9 Microglia Cells. ACS Chem Neurosci. 2018 Jan 31. doi: 10.1021/acschemneuro.7b00468. [Epub ahead of print] PubMed PMID: 29370524.

Update: White cord syndrome

White cord syndrome

Presence of intramedullary MRI hyperintensity signal on T2 weighted image in a patient with unexplained neurological deficits following a spinal cord decompression.


“White cord syndrome” is a very rare condition.


It is thought to be the result of acute reperfusion of chronically areas of spinal cord ischemia.


Its hallmark is the presence of intramedullary MRI hyperintensity signal on T2 weighted image in a patient with unexplained neurologic deficits following a spinal cord decompression.


In previous reports patients have improved following steroid therapy and acute rehabilitation 1).

Case reports


Antwi et al. report an additional case of this complication in a 68-year-old man who developed acute left-sided hemiparesis after posterior cervical fusion for cervical spondylotic myelopathy. The patient improved with high dose steroid therapy 2).


A 64-years old male patient with severe neck pain irradiated to both arms, gait disorder and urinary incontinence. He showed spastic tetraparesis, grip weakness and positive bilateral Hoffman sign, with a Nurick scale score of 3 and a Japanese Orthopaedic Association scale (JOA) of 13, Grade I. MRI imaging documented multiple cervical stenosis with voluminous C3–C4 and C5–C6 disc herniations associated to T2-hyperintense myelomalacic area at C3–C4 level.

Patient underwent double-level ACDF with microsurgical discectomy according to Smith Robinson technique and following anterior arthrodesis, first in C5–C6 with the placement of a titanium cage with intrabody screws (Zero P®, Depuy Synthes – Johnson & Johnson – US), then in C3–C4 level with a stand-alone titanium cage (Cervios®, Depuy Synthes – Johnson & Johnson – US). A diamond drill was used to remove osteophythes in both interbody spaces so to increase spinal cord decompression. An autologous fibrin glue was used to ameliorate haemostasis and fusion.

No surgical, nor anaesthesiological complications were observed, all neural structures were respected and intra-operative x-ray showed the correct placement of both cages. During the closure time of the superficial planes, somatosensory and motor evoked potentials suddenly decreased in voltage. When awakened, the patient showed a severe tetraparesis with complete paraplegia and severe motor weakness to upper limbs with diffuse spastic hypertonia.

A neck collar was then placed and an immediate cervical-spine CT imaging confirmed the correct execution of ACDF.

A following cervical MRI showed an enlarged T2-hyperintense area in C5–C6 level

This ischemic-edematous lesion was supposed to be a case of “white cord syndrome” imputable to a mechanism of improper cord reperfusion. A two-days NASCIS III protocol was then performed.

Three days after, a partial recovery in prehensile strength on the right hand (3/5 Medical Research Council Scale, MRC), a partial recovery in flexion of right arm (2/5 MRC), and in flexion of both legs on thighs (2/5 MRC) were observed.

Seven days after the procedure the patient was transferred to a high specialized Rehabilitation Unit with a Nurick score of 4 and a JOA of 6 3).


Chin et al. report a case of complete loss of somatosensory evoked potentials (SSEPs) during elective ACDF at C4-5 and C5-6 followed by postoperative C6 incomplete tetraplegia without any discernible technical cause. A postoperative MRI demonstrated a large area of high signal changes on T2-weighted MRI intrinsic to the cord “white cord syndrome” but no residual compression. This was considered consistent with spinal cord gliosis with possible acute edema. The acute decompression of the herniated disc resulted in cord expansion and rush-in reperfusion. We postulate that this may have led to disruption in the blood brain barrier (BBB) and triggered a cascade of reperfusion injuries resulting in acute neurologic dysfunction. At 16 months postoperatively our patient is recovering slowly and is now a Nurick Grade 4 4).


1) , 2)

Antwi P, Grant R, Kuzmik G, Abbed K. “White Cord Syndrome” of Acute Hemiparesis after Posterior Cervical Decompression and Fusion for Chronic Cervical Stenosis. World Neurosurg. 2018 Feb 13. pii: S1878-8750(18)30296-1. doi: 10.1016/j.wneu.2018.02.026. [Epub ahead of print] PubMed PMID: 29452319.

Chin KR, Seale J, Cumming V. “White cord syndrome” of acute tetraplegia after anterior cervical decompression and fusion for chronic spinal cord compression: a case report. Case Rep Orthop. 2013;2013:697918. doi: 10.1155/2013/697918. Epub 2013 Mar 4. PubMed PMID: 23533882; PubMed Central PMCID: PMC3603640.

Ophthalmic artery aneurysm surgery

Ophthalmic artery aneurysm surgery

The ophthalmic artery aneurysms can treated safe and effective through a frontolateral approach 1).

The most important risk associated with clipping ophthalmic artery aneurysms is a new visual deficit. Meticulous microsurgical technique is necessary during anterior clinoidectomy, aneurysm dissection, and clip application to optimize visual outcomes, and aggressive medical management postoperatively might potentially decrease the incidence of delayed visual deficits. As the results of endovascular therapy and specifically flow diverters become known, they warrant comparison with these surgical benchmarks to determine best practices 2).

For ophthalmic artery aneurysm treatment if necessary, the ophthalmic artery may be sacrificed without worsening of vision in the vast majority.

Surgery is technically demanding because these aneurysms are often large and may extend into the cavernous sinus 3) 4) 5) 6) 7) 8).

Care must be taken to avoid optic nerve injury caused by the retraction and/or the heat of the drill 9).

For unruptured intracranial aneurysm, drill off anterior clinoid process via an extradural approach before opening dura to approach aneurysm neck maybe safe. Not for ruptured.

Cutting the falciform ligament early decompresses the optic nerve, and helps minimize worsening of visual impairment from surgical manipulation.

In most cases, a side angled clip can be placed paralell to the parent artery along the neck of the aneurysm 10).

Contralateral approach

Case series


Kamide et al. retrospectively reviewed results from microsurgical clipping of 208 OphA aneurysms in 198 patients.

Patient demographics, aneurysm morphology, clinical characteristics, and patient outcomes were recorded and analyzed.

Despite 20% of these aneurysms being large or giant in size, complete aneurysm occlusion was accomplished in 91% of 208 cases, with OphA patency preserved in 99.5%. The aneurysm recurrence rate was 3.1% and the retreatment rate was 0%.

Good outcomes (modified Rankin Scale score 0-2) were observed in 96.2% of patients overall and in all 156 patients with unruptured aneurysms. New visual field defects (hemianopsia or quadrantanopsia) were observed in 8 patients (3.8%), decreased visual acuity in 5 (2.4%), and monocular blindness in 9 (4.3%). Vision improved in 9 (52.9%) of the 17 patients with preoperative visual deficits.

The most important risk associated with clipping OphA aneurysms is a new visual deficit. Meticulous microsurgical technique is necessary during anterior clinoidectomy, aneurysm dissection, and clip application to optimize visual outcomes, and aggressive medical management postoperatively might potentially decrease the incidence of delayed visual deficits. As the results of endovascular therapy and specifically flow diverters become known, they warrant comparison with these surgical benchmarks to determine best practices 11).


The clinical data of 95 patients with carotid ophthalmic artery aneurysms treated via frontolateral approach in the last 1.5 years in Beijing Tiantan Hospital and Beijing Anzhen Hospital were analyzed retrospectively.Before the operation, digital subtraction angiogram (DSA) was performed among all patients.The patients were divided into two groups by the lateral approach.According to preoperative classification, surgical characteristics and prognosis were summarized.

Ninety-five cases of ophthalmic aneurysms were divided into type Ⅰ of 44 cases (46.3%), type Ⅱ of 34 cases (35.7%) and type Ⅲ of 17cases (17.9%), according to the results of DSA.The diameter of aneurysm was <10 mm (35 cases), 10-25 mm (34 cases), and >25 mm (26 cases). In the 17 cases of subarachnoid hemorrhage (SAH), 8 cases were ruptured carotid-ophthalmic artery aneurysms.Among those 95 patients, 93 were clipped successfully, 2 was trapped.Multiple aneurysms in 5 cases were treated in one surgical session through the same approach.No aneurysm residual was found after postoperative CTA review.Ipsilateral vision of 3 cases were decline.Cerebral infarction was appeared in 9 cases.All the others had a good recovery.

The carotid-ophthalmic artery aneurysms could be well exposed. Microsurgery through frontolateral approach has the advantages such as minimal invasion, less effect on the patients’ look and simple procedure.The frontolateral approach is safe and effective in surgery for ophthalmic segment of the internal carotid artery aneurysms 12).

Case reports

Rustemi et al. illustrated the first case of indocyanine green videoangiography (ICG-VA) application in an optic penetrating ophthalmic artery aneurysm treatment. A 57-year-old woman presented with temporal hemianopsia, slight right visual acuity deficit, and new onset of headache. The cerebral angiography detected a right ophthalmic artery aneurysm medially and superiorly projecting. The A1 tract of the ipsilateral anterior cerebral artery was elevated and curved, being suspicious for an under optic aneurysm growth. Surgery was performed. Initially the aneurysm was not visible. ICG-VA permitted the transoptic aneurysm visualization. After optic canal opening, the aneurysm was clipped and transoptic ICG-VA confirmed the aneurysm occlusion. ICG-VA showed also the slight improvement of the optic nerve pial vascularization. Postoperatively, the visual acuity was 10/10 and the hemianopsia did not worsen.

The elevation and curve of the A1 tract in medially and superiorly projecting ophthalmic aneurysms may be an indirect sign of under optic growth, or optic splitting aneurysms. ICG-VA transoptic aneurysm detection and occlusion confirmation reduces the surgical maneuvers on the optic nerve, contributing to function preservation 13).

1) , 12)

Wang JT, Kan ZS, Wang S. [Surgical management of ophthalmic artery aneurysms via minimally invasive frontolateral approach]. Zhonghua Yi Xue Za Zhi. 2017 Apr 18;97(15):1179-1183. doi: 10.3760/cma.j.issn.0376-2491.2017.15.014. Chinese. PubMed PMID: 28427127.
2) , 11)

Kamide T, Tabani H, Safaee MM, Burkhardt JK, Lawton MT. Microsurgical clipping of ophthalmic artery aneurysms: surgical results and visual outcomes with 208 aneurysms. J Neurosurg. 2018 Jan 26:1-11. doi: 10.3171/2017.7.JNS17673. [Epub ahead of print] PubMed PMID: 29372879.

Hosobuchi Y. Direct surgical treatment of giant intracranial aneurysms. J Neurosurg. 1979;51(6):743–756.

Sundt T M Jr, Piepgras D G. Surgical approach to giant intracranial aneurysms. Operative experience with 80 cases. J Neurosurg. 1979;51(6):731–742.

Almeida G M, Shibata M K, Bianco E. Carotid-ophthalmic aneurysms. Surg Neurol. 1976;5(1):41–45.

Kattner K A, Bailes J, Fukushima T. Direct surgical management of large bulbous and giant aneurysms involving the paraclinoid segment of the internal carotid artery: report of 29 cases. Surg Neurol. 1998;49(5):471–480.

Nutik S L. Ventral paraclinoid carotid aneurysms. J Neurosurg. 1988;69(3):340–344.

Nutik S. Carotid paraclinoid aneurysms with intradural origin and intracavernous location. J Neurosurg. 1978;48(4):526–533

Kumon Y, Sakaki S, Kohno K, Ohta S, Ohue S, Oka Y. Asymptomatic, unruptured carotid-ophthalmic artery aneurysms: angiographical differentiation of each type, operative results, and indications. Surg Neurol. 1997 Nov;48(5):465-72. PubMed PMID: 9352810.

Day AL. Clinicoanatomic features of supraclinoid aneurysms. Clin Neurosurg. 1990;36:256-74. Review. PubMed PMID: 2403885.

Rustemi O, Cester G, Causin F, Scienza R, Della Puppa A. Indocyanine Green Videoangiography Transoptic Visualization and Clipping Confirmation of an Optic Splitting Ophthalmic Artery Aneurysm. World Neurosurg. 2016 Jun;90:705.e5-705.e8. doi: 10.1016/j.wneu.2016.03.010. Epub 2016 Mar 12. PubMed PMID: 26979923.

Encephaloclastic cyst

Poorly circumscribed areas of parenchymal destruction associated with cystic components.

Encephaloclastic cysts provoked by intraventricular chemotherapy are very uncommon.

Rare complication of a malfunctioning methotrexate Ommaya reservoir 1) 2).


The pathogenesis may result from alterations in CSF pulsations with retrograde flow of intraventricular chemotherapy into the brain parenchyma and subsequent development of a local chemical encephalopathy.

Mella et al. report two rare cases of encephaloclastic cyst with intraventricular topotecan use. The patients were diagnosed and treated at The University of Texas MD Anderson Cancer Center. They consented to the publication of their laboratory results and imaging studies for educational purposes.

The patients presented with metastatic cancers (breast/lung) complicated by leptomeningeal disease. Ommaya reservoirs were placed in both cases and patients were initiated on intraventricular topotecan at 0.4 mg twice weekly. After approximately 12 intraventricular treatments, both patients developed confusion, seizures and headaches. MRI of the brain demonstrated cystic dilatation of the brain parenchyma around the catheter that connects to the reservoir dome and delivers the drug to the intraventricular space. The catheter was surrounded by vasogenic edema. Catheters were removed and analyzed and were found to be intact. CSF analyses showed no evidence of infection or malignancy. Intraventricular topotecan was discontinued and both patients demonstrated sustained clinical and radiological responses.

These cases highlight an atypical complication of intraventricular use of topotecan with successful management 3).


Chowdhary S, Chalmers LM, Chamberlain PA. Methotrexate-induced encephaloclastic cyst: a complication of intraventricular chemotherapy. Neurology. 2006 Jul 25;67(2):319. PubMed PMID: 16864827.

Lubomski M, Pell M, Lochhead A, Jude M. Encephaloclastic cyst: a rare complication of a malfunctioning methotrexate Ommaya reservoir. Intern Med J. 2018 Feb;48(2):224-226. doi: 10.1111/imj.13704. PubMed PMID: 29415363.

Mella DB, Kamiya-Matsuoka C, Liao B, Tummala S, de Groot J. Recurrent encephaloclastic cyst induced by intraventricular topotecan. J Neurol Sci. 2015 Feb 15;349(1-2):52-3. doi: 10.1016/j.jns.2014.12.024. Epub 2014 Dec 24. PubMed PMID: 25598491.

Update: Unruptured intracranial aneurysm treatment score

Unruptured intracranial aneurysm treatment score

see also PHASES score.

The unruptured intracranial aneurysm treatment score (UIATS) was published in April 2015 as a multidisciplinary consensus regarding treatment of unruptured intracranial aneurysms (UIA).

Etminan et al. endeavored to develop an unruptured intracranial aneurysm treatment score (UIATS) model that includes and quantifies key factors involved in clinical decision-making in the management of UIAs and to assess agreement for this model among specialists in Unruptured intracranial aneurysm (UIA) management and research.

An international multidisciplinary (neurosurgery, neuroradiology, neurology, clinical epidemiology) group of 69 specialists was convened to develop and validate the UIATS model using a Delphi consensus. For internal (39 panel members involved in identification of relevant features) and external validation (30 independent external reviewers), 30 selected UIA cases were used to analyze agreement with UIATS management recommendations based on a 5-point Likert scale (5 indicating strong agreement). Interrater agreement (IRA) was assessed with standardized coefficients of dispersion (vr*) (vr* = 0 indicating excellent agreement and vr* = 1 indicating poor agreement).

The UIATS accounts for 29 key factors in UIA management. Agreement with UIATS (mean Likert scores) was 4.2 (95% confidence interval [CI] 4.1-4.3) per reviewer for both reviewer cohorts; agreement per case was 4.3 (95% CI 4.1-4.4) for panel members and 4.5 (95% CI 4.3-4.6) for external reviewers (p = 0.017). Mean Likert scores were 4.2 (95% CI 4.1-4.3) for interventional reviewers (n = 56) and 4.1 (95% CI 3.9-4.4) for noninterventional reviewers (n = 12) (p = 0.290). Overall IRA (vr*) for both cohorts was 0.026 (95% CI 0.019-0.033).

This novel UIA decision guidance study captures an excellent consensus among highly informed individuals on UIA management, irrespective of their underlying specialty. Clinicians can use the UIATS as a comprehensive mechanism for indicating how a large group of specialists might manage an individual patient with a UIA 1)

A tertiary center with focus on vascular neurosurgery, aimed to investigate whether there treatment decision-making in patients with UIA has been in accordance with the published UIATS. A retrospective analysis of patients admitted to the center with UIA was performed. UIATS was applied to all identified UIA. Three decision groups were defined: (a) UIATS favoring treatment, (b) UIATS favoring observation, and © UIATS inconclusive. These results were then compared to our clinical decisions. Spearman’s rank-order correlation (ρ) was run to determine the relationship between the UIATS and our clinical decisions. Cases of discrepancies between UIATS and our clinical decisions were then examined for complications, defined as periprocedural adverse events in treated aneurysms, or aneurysm rupture in untreated aneurysms. Ninety-three patients with 147 UIA were included. A total of 118/147 (80.3%) UIA were treated. In 70/118 (59.3%), UIATS favored treatment, in 18/118 (15.3%), it was inconclusive, and in 30/118 (25.4%), it favored observation. A total of 29/147 (19.7%) UIA were not treated. In 15/29 (51.7%), UIATS favored observation, in 9/29 (31%), it favored treatment, and in 5/29 (17.2%), it was inconclusive (ρ = 0.366, p < 0.01). Discrepancies between UIATS and our clinical decisions did not correlate with complications (ρ = 0.034, p = 0.714). Our analysis shows that our more intuitive clinical decision-making has been in line with UIATS. Our treatment decisions did not correlate with an increased rate of complications 2).

The purpose of the study of Ravindra et al. was to compare the unruptured intracranial aneurysm treatment score (UIATS) recommendations with the real-world experience in a quaternary academic medical center with a high volume of patients with unruptured intracranial aneurysms (UIAs).

All patients with UIAs evaluated during a 3-year period were included. All factors included in the UIATS were abstracted, and patients were scored using the UIATS. Patients were categorized in a contingency table assessing UIATS recommendation versus real-world treatment decision. The authors calculated the percentage of misclassification, sensitivity, specificity, and area under the receiver operating characteristic (ROC) curve. RESULTS A total of 221 consecutive patients with UIAs met the inclusion criteria: 69 (31%) patients underwent treatment and 152 (69%) did not. Fifty-nine (27%) patients had a UIATS between -2 and 2, which does not offer a treatment recommendation, leaving 162 (73%) patients with a UIATS treatment recommendation. The UIATS was significantly associated with treatment (p < 0.001); however, the sensitivity, specificity, and percentage of misclassification were 49%, 80%, and 28%, respectively. Notably, 51% of patients for whom treatment would be recommended by the UIATS did not undergo treatment in the real-world cohort and 20% of patients for whom conservative management would be recommended by UIATS had intervention. The area under the ROC curve was 0.646.

Compared with the authors’ experience, the UIATS recommended overtreatment of UIAs. Although the UIATS could be used as a screening tool, individualized treatment recommendations based on consultation with a cerebrovascular specialist are necessary. Further validation with longitudinal data on rupture rates of UIAs is needed before widespread use 3).


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