Rathke's Cleft Cyst Abscess with a Very Unusual Course.

Infected Rathke's cleft cysts (RCC) are extremely rare with only a few published cases. We report the case of a 31-year-old man who presented with headaches, visual disturbance, and hypopituitarism secondary to an infected RCC with extension of abscesses along the optic tract. Magnetic resonance imaging showed ring enhancing cystic lesions within an expanded sella with suprasellar and intraparenchymal extension. The radiological appearance suggested a high-grade optic glioma, but an endoscopic transsphenoidal biopsy revealed frank pus in the pituitary fossa, which subsequently grew Staphylococcus aureus . Pathological examination of the cyst wall showed an inflamed RCC. Following a prolonged course of intravenous antibiotics, the infection resolved and vision improved. RCC abscesses are rare and the intracranial extension of the infection in our case makes it unique.

Foster Kennedy syndrome secondary to a giant prolactinoma with a remarkable response to cabergoline.

Summary: Pituitary adenomas are intracranial neoplasms, usually demonstrating a benign phenotype. We present the case of 21-year-old male with an 18-month history of reduced visual function (acuity and field) in the left eye. Based on neuroimaging and endocrine profile, a giant prolactinoma causing hypogonadotropic hypogonadism was diagnosed and cabergoline was commenced. After a month of treatment, the tumour size reduced, and visual function improved to normal; however, he developed Foster Kennedy syndrome with a swollen right optic disc. After almost 1 year of follow-up, he regained full visual functioning. Two years since his diagnosis, his prolactin remains normal with no adverse effects or further visual complications. Learning points: Foster Kennedy syndrome is a rare entity but can be a feature of pituitary adenomas. Visual deterioration secondary to a compressive optic neuropathy can be reversible, provided that diagnosis and treatment are prompt. This case highlights the importance of frequent monitoring of visual function during follow-up of these lesions, particularly when there are deficits at diagnosis.

A Rare Case of Fungal Rhinosinusitis Caused by Scedosporium apiopermum.

Fungal rhinosinusitis (FRS) is inflammation of the paranasal sinus mucosa due to fungal infections, which can be invasive or non-invasive. The occurrence of a sphenoid mucocele with a fungal ball is rare. We report a case of sphenoid sinus mucocele with a fungal ball caused by Scedosporium apiopermum in a 32-year-old female who presented to the Emergency Department with persistent headache not relieved on medications. The radiological images showed a mucocele with clival osteomyelitis. Urgent endoscopic examination and debridement was undertaken which demonstrated a mucocele with fungal ball. Microbiological examination confirmed it to be Scedosporium apiopermum.

Endoscopic endonasal surgery for Clival Chordomas - a single institution experience and short term outcomes.

Purpose: Clival Chordomas are locally aggressive tumours which pose a significant treatment challenge. Endoscopic endonasal approach for clival chordomas is correlated with higher resection rates and lower morbidity rates in comparison to open approaches. We present our initial single institution experience and short-term patient outcomes following endoscopic endonasal approach for resection of clival chordomas. Materials and methods: This is a retrospective analysis of ten patients undergoing endoscopic endonasal approach for clival chordomas in our neurosurgical unit over a 6 year period between August 2010 and September 2016. The procedures were performed using two surgeons, four hands, binostril endoscopic endonasal approach with a Karl Storz® endoscope and intraoperative BrainLab® image guidance. Results: Overall 15 endoscopic endonasal approach resections of clival chordoma were performed in 10 patients with median follow up period of 39.5 months (range 9-76). Gross total resection was achieved in 4 cases (40%), near total resection in 4 cases (40%) and subtotal resection in 2 cases (20%). 5 cases (50%) required revision resections. Cerebrospinal fluid leak occurred in 2 patients. 1 case of meningitis occurred in a patient with revision surgery. There were no new neurological deficits post operatively with 3 patients demonstrating resolution of diplopia post operatively. No recurrence occurred following gross total resection. 1 out of 4 cases of near total resection showed evidence of progression during the follow up period. Both cases of subtotal resection demonstrated evidence of progression with one dying of unrelated cause during the follow up period. Conclusion: Endoscopic endonasal approach represents a safe technique for debulking and resection of clival chordomas. Due to the rarity of clival chordomas, it is important that patients with this pathology are managed in high volume skull base centres where a multi-disciplinary team approach is available.

Treatment of intracranial aneurysms using the pipeline flex embolization device with shield technology: angiographic and safety outcomes at 1-year follow-up.

PURPOSE: The Pipeline Embolization Device (PED) is a routine first-line treatment option for intracranial aneurysms (IAs). The Pipeline Flex Embolization Device with Shield Technology (Pipeline Shield) is an updated version of the PED which has been modified to include a surface phosphorylcholine biocompatible polymer. Its early technical success and safety have been reported previously. Here, we assessed the long-term safety and efficacy of the Pipeline Shield for the treatment of IAs. MATERIALS AND METHODS: The Pipeline Flex Embolization Device with Shield Technology (PFLEX) study was a prospective, single-arm, multicenter study for the treatment of unruptured IAs using the Pipeline Shield. The primary endpoint was a major stroke in the territory supplied by the treated artery or neurologic death at 1-year post-procedure. Angiographic outcomes were also assessed by an independent radiology laboratory at 6 months and 1 year. RESULTS: Fifty patients (mean age, 53 years; 82% female) with 50 unruptured IAs were treated. Mean aneurysm diameter was 8.82±6.15 mm. Of the target aneurysms, 38/50 (76%) were small (<10 mm), 11/50 (22%) were large (≥10 and<25 mm), and 1/50 (2%) was giant (≥25 mm). Forty-seven (94%) were located in the internal carotid artery and three (6%) in the vertebral artery. At 1-year post-procedure, no major strokes or neurologic deaths were reported, and complete occlusion was achieved in 27/33 (81.8%). There were no instances of aneurysm recurrence or retreatment. CONCLUSIONS: Our 1-year follow-up concerning angiographic and safety outcomes corroborate previous evidence that the Pipeline Shield is a safe and effective treatment for IAs. TRIAL REGISTRATION NUMBER: NCT02390037.

Macroprolactinoma causing VI, X, XII cranial nerve palsies nearly 30 years after initial treatment.

A 48-year-old man was diagnosed with a large macroprolactinoma in 1982 treated with surgery, adjuvant radiotherapy and bromocriptine. Normal prolactin was achieved in 2005 but in 2009 it started rising. Pituitary MRIs in 2009, 2012, 2014 and 2015 were reported as showing empty pituitary fossa. Prolactin continued to increase (despite increasing bromocriptine dose). Trialling cabergoline had no effect (prolactin 191,380 mU/L). In January 2016, he presented with right facial weakness and CT head was reported as showing no acute intracranial abnormality. In late 2016, he was referred to ENT with hoarse voice; left hypoglossal and recurrent laryngeal nerve palsies were found. At this point, prolactin was 534,176 mU/L. Just before further endocrine review, he had a fall and CT head showed a basal skull mass invading the left petrous temporal bone. Pituitary MRI revealed a large enhancing mass within the sella infiltrating the clivus, extending into the left petrous apex and occipital condyle with involvement of the left Meckel's cave, internal acoustic meatus, jugular foramen and hypoglossal canal. At that time, left abducens nerve palsy was also present. CT thorax/abdomen/pelvis excluded malignancy. Review of previous images suggested that this lesion had started becoming evident below the fossa in pituitary MRI of 2015. Temozolomide was initiated. After eight cycles, there is significant tumour reduction with prolactin 1565 mU/L and cranial nerve deficits have remained stable. Prolactinomas can manifest aggressive behaviour even decades after initial treatment highlighting the unpredictable clinical course they can demonstrate and the need for careful imaging review. LEARNING POINTS: Aggressive behaviour of prolactinomas can manifest even decades after first treatment highlighting the unpredictable clinical course these tumours can demonstrate.Escape from control of hyperprolactinaemia in the absence of sellar adenomatous tissue requires careful and systematic search for the anatomical localisation of the lesion responsible for the prolactin excess.Temozolomide is a valuable agent in the therapeutic armamentarium for aggressive/invasive prolactinomas, particularly if they are not amenable to other treatment modalities.

Idiopathic intracranial hypertension: consensus guidelines on management.

The aim was to capture interdisciplinary expertise from a large group of clinicians, reflecting practice from across the UK and further, to inform subsequent development of a national consensus guidance for optimal management of idiopathic intracranial hypertension (IIH). METHODS: Between September 2015 and October 2017, a specialist interest group including neurology, neurosurgery, neuroradiology, ophthalmology, nursing, primary care doctors and patient representatives met. An initial UK survey of attitudes and practice in IIH was sent to a wide group of physicians and surgeons who investigate and manage IIH regularly. A comprehensive systematic literature review was performed to assemble the foundations of the statements. An international panel along with four national professional bodies, namely the Association of British Neurologists, British Association for the Study of Headache, the Society of British Neurological Surgeons and the Royal College of Ophthalmologists critically reviewed the statements. RESULTS: Over 20 questions were constructed: one based on the diagnostic principles for optimal investigation of papilloedema and 21 for the management of IIH. Three main principles were identified: (1) to treat the underlying disease; (2) to protect the vision; and (3) to minimise the headache morbidity. Statements presented provide insight to uncertainties in IIH where research opportunities exist. CONCLUSIONS: In collaboration with many different specialists, professions and patient representatives, we have developed guidance statements for the investigation and management of adult IIH.

A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer.

Co-existence of craniopharyngioma and acromegaly has been very rarely reported. A 65-year-old man presented with visual deterioration, fatigue and frontal headaches. Magnetic resonance imaging revealed a suprasellar heterogeneous, mainly cystic, 1.9 × 2 × 1.9 cm mass compressing the optic chiasm and expanding to the third ventricle; the findings were consistent with a craniopharyngioma. Pituitary hormone profile showed hypogonadotropic hypogonadism, mildly elevated prolactin, increased insulin-like growth factor 1 (IGF-1) and normal thyroid function and cortisol reserve. The patient had transsphenoidal surgery and pathology of the specimen was diagnostic of adamantinomatous craniopharyngioma. Post-operatively, he had diabetes insipidus, hypogonadotropic hypogonadism and adrenocorticotropic hormone and thyroid-stimulating hormone deficiency. Despite the hypopituitarism, his IGF-1 levels remained elevated and subsequent oral glucose tolerance test did not show complete growth hormone (GH) suppression. Further review of the pre-operative imaging revealed a 12 × 4 mm pituitary adenoma close to the right carotid artery and no signs of pituitary hyperplasia. At that time, he was also diagnosed with squamous cell carcinoma of the left upper lung lobe finally managed with radical radiotherapy. Treatment with long-acting somatostatin analogue was initiated leading to biochemical control of the acromegaly. Latest imaging has shown no evidence of craniopharyngioma regrowth and stable adenoma. This is a unique case report of co-existence of craniopharyngioma, acromegaly and squamous lung cell carcinoma that highlights diagnostic and management challenges. Potential effects of the GH hypersecretion on the co-existent tumours of this patient are also briefly discussed. LEARNING POINTS: Although an extremely rare clinical scenario, craniopharyngioma and acromegaly can co-exist; aetiopathogenic link between these two conditions is unlikely.Meticulous review of unexpected biochemical findings is vital for correct diagnosis of dual pituitary pathology.The potential adverse impact of GH excess due to acromegaly in a patient with craniopharyngioma (and other neoplasm) mandates adequate biochemical control of the GH hypersecretion.

Outcome of Nonfunctioning Pituitary Adenomas That Regrow After Primary Treatment: A Study From Two Large UK Centers.

Context: Despite the major risk of regrowth of clinically nonfunctioning pituitary adenomas (CNFAs) after primary treatment, systematic data on the probability of further tumor progression and the effectiveness of management approaches are lacking. Objective: To assess the probability of further regrowth(s), predictive factors, and outcomes of management approaches in patients with CNFA diagnosed with adenoma regrowth after primary treatment. Patients, Design, and Setting: Retrospective cohort study of 237 patients with regrown CNFA managed in two UK centers. Results: Median follow-up was 5.9 years (range, 0.4 to 37.7 years). The 5-year second regrowth rate was 35.3% (36.2% after surgery; 12.5% after radiotherapy; 12.7% after surgery combined with radiotherapy; 63.4% with monitoring). Of those managed with monitoring, 34.8% eventually were offered intervention. Type of management and sex were risk factors for second regrowth. Among those with second adenoma regrowth, the 5-year third regrowth rate was 26.4% (24.4% after surgery; 0% after radiotherapy; 0% after surgery combined with radiotherapy; 48.3% with monitoring). Overall, patients with a CNFA regrowth had a 4.4% probability of a third regrowth at 5 years and a 10.0% probability at 10 years; type of management of the first regrowth was the only risk factor. Malignant transformation was diagnosed in two patients. Conclusions: Patients with regrown CNFA after primary treatment continue to carry considerable risk of tumor progression, necessitating long-term follow-up. Management approach to the regrowth was the major factor determining this risk; monitoring had >60% risk of progression at 5 years, and a substantial number of patients ultimately required intervention.

Periprocedural outcomes and early safety with the use of the Pipeline Flex Embolization Device with Shield Technology for unruptured intracranial aneurysms: preliminary results from a prospective clinical study.

BACKGROUND AND PURPOSE: The Pipeline Embolization Device (PED) has become a routine first-line option for treatment of intracranial aneurysms (IAs). We assessed the early safety and technical success of a new version of PED, Pipeline Flex Embolization Device with Shield Technology (Pipeline Shield), which has the same design and configuration but has been modified to include a surface synthetic biocompatible polymer. MATERIALS AND METHODS: The Pipeline Flex Embolization Device with Shield Technology (PFLEX) study is a prospective, single-arm, multicenter study for the treatment of unruptured IAs using Pipeline Shield. The primary study endpoints included the occurrence of major stroke in the territory supplied by the treated artery or neurologic death at 1 year post-procedure. Secondary endpoints included the rate of Pipeline Shield-related or procedure-related serious or non-serious adverse events. Analyses were conducted to evaluate early safety findings in the 30-day post-procedure period as well as technical procedural success outcomes. RESULTS: Fifty patients with 50 unruptured target IAs were enrolled. Mean aneurysm diameter was 8.82±6.15 mm. Thirty-eight aneurysms (76%) were small (<10 mm). Device deployment was technically successful with 98% of devices. Complete wall apposition was achieved immediately post-procedure in 48 cases (96%). No major strokes or neurologic deaths were reported in the 30-day post-procedure period. CONCLUSIONS: The results of this first experience with the new Pipeline Flex corroborate the early safety of the device. Mid-term and long-term follow-up examinations will provide data on safety outcomes at the 6-month and 1-year follow-up periods. CLINICAL TRIAL REGISTRATION: NCT02390037.

Corking the WEB and coiling through a jailed microcatheter: WEB assisted coiling, a useful technique avoiding the use of stents in treating wide-necked large intracranial aneurysms.

The WEB is an endovascular flow-disrupting device used in treating wide-necked intracranial aneurysms. Although the device is available in varying sizes, large aneurysms pose a challenge with the need for custom-made devices. We describe the use of coils as an adjunct to the WEB device in successfully treating large aneurysms in two patients, one with an acutely ruptured aneurysm. This novel technique of jailing a microcatheter, deploying the WEB and then coiling the aneurysm saves the need for intracranial stenting, thereby avoiding the need for antiplatelet therapy, which is of benefit in the setting of acute aneurysm rupture.

Preliminary experience with the liquid embolic material agent PHIL (Precipitating Hydrophobic Injectable Liquid) in treating cranial and spinal dural arteriovenous fistulas: technical note.

BACKGROUND: Liquid embolic agents are the preferred embolic material in endovascular treatment of pial and brain arteriovenous malformations and dural arteriovenous fistulas (DAVFs). There is little choice available in interventional neuroradiology practice other than two of the most commonly used liquid embolic agents-n-butyl cyanoacrylate and the Onyx liquid embolic system (ev3 Neurovascular, Irvine, California, USA). PHIL (Precipitating Hydrophobic Injectable Liquid) (Microvention, Inc California, USA) is a new liquid embolic agent, CE marked and available for clinical use in Europe. OBJECTIVE: To present our preliminary experience using PHIL in treating cranial and spinal DAVFs. METHODS: Between September 2014 and January 2015, eight patients, with five cranial DAVFs and three spinal DAVFs were treated with PHIL as the sole embolic agent used with intent to cure. Clinical presentation, location of DAVF, Borden type, fluoroscopic time, radiation dose, procedural time, injecting microcatheter used, volume of PHIL injected, complications, immediate angiographic data, premorbid and discharge modified Rankin Scale score, and any neurologic deficits were included in the analysis. RESULTS: Seven patients were successfully treated with complete angiographic exclusion of the fistula in a single sitting. Treatment failed in one patient where only suboptimal microcatheter positioning could be achieved and PHIL failed to penetrate the fistula's nidus. Venous penetration was achieved in all other patients except one with a small fistula, but with adequate fistula penetration by the embolic material. No other technical complication or neurologic deterioration occurred in any of the patients. CONCLUSIONS: PHIL liquid embolic agent appears to be an excellent alternative embolic material with certain advantages compared with other available liquid embolic agents. Further studies are required to fully evaluate its safety and efficacy.

Gas Embolic Stroke Secondary to Bowel Infarction.

A 69-year-old gentleman with metastatic esophageal adenocarcinoma presented with acute abdominal pain to the emergency medicine department and subsequently developed an acute left hemiplegia while in the resuscitation unit. An unenhanced computed tomography (CT) scan of the head showed right frontal cerebral gas emboli while an unenhanced CT scan of the abdomen and pelvis showed extensive portal venous gas and pneumatosis intestinalis, presumed secondary to bowel infarction.

Head-Jolting Nystagmus: Occlusion of the Horizontal Semicircular Canal Induced by Vigorous Head Shaking.

IMPORTANCE: We report a new syndrome, which we are calling head-jolting nystagmus, that expands the differential diagnosis of head movement-induced paroxysmal vertigo. OBSERVATIONS: Two male patients (65 and 58 years old) described rotational vertigo after violent and brief (1- to 2-second) oscillations of the head (head jolting) that triggered intense horizontal nystagmus lasting 45 seconds. Accelerations of the head required to induce these episodes could only be achieved by the patients themselves. In case 1, the episodes gradually disappeared over a 6-year period. In case 2, magnetic resonance imaging (3-T) suggested a filling defect within the left horizontal semicircular canal. He underwent surgical canal plugging in March 2013 that resolved the symptoms. CONCLUSIONS AND RELEVANCE: We attribute head-jolting nystagmus to dislodged material within the horizontal semicircular canal and provide a mechanistic model to explain its origin.

Radiologic differentiation of intracranial epidermoids from arachnoid cysts.

OBJECTIVE: Intracranial epidermoids (cholesteatomas) mimic arachnoid cysts in their radiologic characteristics, especially in the cerebellopontine angle. It is essential to differentiate the two conditions because they warrant different therapeutic interventions. The objective of this study is to elucidate the different radiologic characteristics of the conditions. STUDY DESIGN AND SETTING: This was a retrospective study of 4 patients referred for a differential diagnosis and management of intracranial cystic lesions to the Departments of Neurotology/Neurosurgery and Neuroradiology in a tertiary referral university hospital. PATIENTS: Four patients of different age groups with cystic intracranial lesions, diagnosed epidermoid or arachnoid cysts, were chosen. A retrospective analysis of their case charts, radiologic and surgical interventions, and follow-up records was undertaken. METHODS: The imaging techniques used included computerized tomographic scans, magnetic resonance imaging (MRI) with T1, T2, proton-density, and gadolinium-enhanced T1 images. In addition, special MRI sequences were used that included fluid-attenuated inversion recovery and echo planar diffusion scanning. All the patients underwent an audiovestibular evaluation. RESULTS: Both lesions are characteristically well demarcated and have a homogeneous low density, similar to cerebrospinal fluid on computerized tomographic scan, showing no contrast enhancement. On MRI, epidermoids and arachnoid cysts usually appear hypointense on T1-weighted images and hyperintense on T2-weighted images. On fluid-attenuated inversion recovery, an arachnoid cyst tends to follow cerebrospinal fluid intensity, whereas an epidermoid becomes hyperintense. There are occasions when an epidermoid may appear as a low-intensity lesion on fluid-attenuated inversion recovery. This dilemma is resolved with the use of echo planar diffusion scanning, on which an epidermoid remains bright. CONCLUSION: The authors recommend the use of fluid-attenuated inversion recovery and diffusion sequence MRI when definitive radiologic diagnosis of cystic intracranial lesions becomes difficult with routine computerized tomographic scanning and MRI.