Spontaneous rupture of malignant papillary craniopharyngioma with CSF seeding and metastatic deposits: A case report and literature review.

Introduction: Craniopharyngiomas are benign tumours mainly confined to the cranial cavity in the suprasellar region. Research Question and Case Description: We present a rare case of an aggressive papillary craniopharyngioma with disseminated spinal intradural disease. A 67-year-old woman presented with a 4-month history of headache, visual disturbance, acute confusion and radicular leg pain. Previous history of breast carcinoma (ER â€‹+ â€‹PR â€‹+ â€‹HER2-) was noted. The importance of histological diagnosis prior to treatment of sellar or suprasellar lesions with atypical or aggressive features is explored. Materials and methods: MRI demonstrated a partly solid and partly cystic pituitary mass lesion in the sellar and suprasellar region with chiasmal compression and hypothalamic involvement. The sella was mildly enlarged and there were no calcifications. Whole neuraxis MRI revealed intradural deposits involving the ventricular system, spinal cord and conus. Within a month, the lesion rapidly increased in size. The patient underwent a craniotomy and transventricular resection of the sellar and suprasellar mass. Cranial lesion histology favoured papillary craniopharyngioma, confirmed by BRAF V600 mutation. Lumbar puncture CSF cytology confirmed craniopharyngioma with BRAF mutation and no evidence of metastatic breast cancer. Results: The patient remained confused postoperatively without focal neurological deficit and underwent palliative whole brain radiotherapy. She died 4 months later. A review of the literature identified 29 reports of ruptured craniopharyngioma. Discussion and Conclusion: Ruptured craniopharyngioma presents with a suprasellar mass and drop lesions in the spinal canal, characteristics radiologically indistinguishable from metastatic disease. The importance of histological diagnoses in directing the management of these cases is highlighted.

Do All Notochordal Lesions Require Proton Beam Radiotherapy? A Proposed Reclassification of Ecchordosis Physaliphora as Benign Notochord Cell Tumor.

Objectives Ecchordosis physaliphora (EP) is a benign notochord lesion of the clivus arising from the same cell line as chordoma, its malignant counterpart. Although usually asymptomatic, it can cause spontaneous cerebrospinal fluid (CSF) rhinorrhea. Benign notochordal cell tumor (BNCT) is considered another indolent, benign variant of chordoma. Although aggressive forms of chordoma require maximal safe resection followed by proton beam radiotherapy, BNCT and EP can be managed with close imaging surveillance without resection or radiotherapy. However, while BNCT and EP can be distinguished from more aggressive forms of chordoma, differentiating the two is challenging as they are radiologically and histopathologically identical. This case series aims to characterize the clinicopathological features of EP and to propose classifying EP and BNCT together for the purposes of clinical management. Design Case series. Setting Tertiary referral center, United Kingdom. Participants Patients with suspected EP from 2015 to 2019. Main Outcome Measures Diagnosis of EP. Results Seven patients with radiological suspicion of EP were identified. Five presented with CSF rhinorrhea and two were asymptomatic. Magnetic resonance imaging features consistently showed T1-hypointense, T2-hyperintense nonenhancing lesions. Diagnosis was made on biopsy for patients requiring repair and radiologically where no surgery was indicated. The histological features of EP included physaliphorous cells of notochordal origin (positive epithelial membrane antigen, S100, CD10, and/or MNF116) without mitotic activity. Conclusion EP is indistinguishable from BNCT. Both demonstrate markers of notochord cell lines without malignant features. Their management is also identical. We therefore propose grouping EP with BNCT. Close imaging surveillance is required for both as progression to chordoma remains an unquantified risk.

Atypical Cauda Equina Syndrome with Lower Limb Clonus: A Literature Review and Case Report.

BACKGROUND: Cauda equina syndrome (CES) is a neurosurgical emergency warranting urgent surgical decompression. Treatment delay may precipitate permanent adverse neurological sequelae. CES is a clinical diagnosis, corroborated by radiological findings. Atypical presentations should be acknowledged to avoid inappropriately rejected diagnoses. CASE DESCRIPTION: We report the case of a woman exhibiting bilateral lower limb weakness, perineal numbness, sphincter disturbance, and lower limb clonus. Classically, CES displays lower motor neuron signs in the lower limbs. The presence of clonus, an upper motor neuron sign, brought the diagnosis into doubt. The history included chronic fatigue, difficulty mobilizing, and intermittent blurred vision. A lumbosacral magnetic resonance imaging (MRI) scan demonstrated a large disc prolapse at L5/S1. The cord was not low-lying or tethered. Therefore, the possibility of second diagnoses, including of inflammatory or demyelinating nature, was raised. An urgent MRI scan of the brain and cervicothoracic cord identified no other lesions. On balance, the clinical presentation could overwhelmingly be attributed to the L5/S1 disc prolapse. Given the time-critical nature of cauda equina (CE) compression, an urgent laminectomy and discectomy was offered with continued postoperative investigation of the clonus. Intraoperatively, significant CE compression was found. The operation proceeded uneventfully and the patient recovered fully. In the immediate postoperative period, the clonus persisted yet subsequently resolved completely. CONCLUSIONS: We conclude that the clonus was attributable to CE compression and not a second pathology. The corresponding neuroanatomical correlate remains nondelineated. The presence of clonus does not preclude a diagnosis of CES. If the clinicoradiological information otherwise correlate, surgery should not be delayed while alternative diagnoses are sought. The literature is also reviewed.

Pre-emptive intrathecal vancomycin therapy reduces external ventricular drain infection: a single centre retrospective case-control study.

OBJECTIVES: External ventricular drain (EVD)-related infection is a significant source of morbidity in neurosurgical patients. Recently, there has been a drive to adopt new catheters with bactericidal properties to reduce infection rates. We propose that the use of standard catheters combined with pre-emptive intrathecal vancomycin (ITV) 10 mg daily provides an effective alternative. DESIGN: Retrospective study of all patients with EVDs between 2010 and 2012, comparing infection rates in those who did and did not receive pre-emptive ITV. All EVDs were of the standard silicon catheter type. CSF infection was defined, as per Centre for Disease Control (CDC) guidelines, as clinical suspicion ± positive CSF gram stain/culture or leucocytosis. Infection rates were compared using Pearson's chi-squared test. RESULTS: 262 EVDs were included in the study, of which 111 were managed with pre-emptive ITV. The infection rate was 2.7% in the vancomycin group and 11.9% in the control group (p<.01). There were no cases of vancomycin-resistant infection in either group. CONCLUSION: The use of pre-emptive ITV is associated with a significantly lower EVD infection rate. This compares favourably with those reported in the literature for bactericidal catheters.

Surgical access rather than method of pleurodesis (pleurectomy or pleural abrasion) influences recurrence rates for pneumothorax surgery: systematic review and meta-analysis.

AIM: Surgery for recurrent spontaneous pneumothoraces is one of the most commonly performed procedures in thoracic surgery, but few studies have evaluated the efficacy of the surgical treatment options. We aimed to evaluate the influence of the type of pleurodesis on recurrence whilst adjusting for surgical access by systematic review and meta-regression of randomised and non-randomised trials. METHODS: A systematic literature search undertaken for studies on pneumothorax surgery in MEDLINE, EMBASE, Cochrane Library, Internet trial registers and conference abstracts identified 29 studies (4 randomised and 25 non-randomised) eligible for inclusion. Meta-regression was performed adjusting for access to screen for evidence of a difference in recurrence rates. RESULTS: Access remained the principal determinant of recurrence rates after surgery. The relative risk of recurrence was 4.731 (2.699-8.291; p < 0.001) using video-assisted thoracoscopic surgery compared to open access. After adjusting for access, the relative risk of recurrence of pleural abrasion compared to pleurectomy was observed to be 2.851 (95 % CI 0.478-17.021), but this was not statistically (p = 0.220). CONCLUSION: Surgical access remains the most important factor that influences outcome after surgery for recurrent pneumothoraces. Although the relative risk of recurrence was higher with pleural abrasion compared to pleurectomy, it was not statistically significant, and more work needs to be conducted to address this question.

Psychological distress does not compromise outcome in spinal surgery.

SUMMARY OF BACKGROUND DATA: Physical outcomes following surgery for degenerative spine disease have been well studied whereas the importance of psychological factors has only recently been acknowledged. Previous studies suggest that pre-operative psychological distress predicts poor outcome from spinal surgery. In the drive to identify patients who will not benefit, these patients risk being denied surgery. STUDY DESIGN: This is a prospective series from a spinal surgical register. AIM: The study examines the relationship between the physical symptoms, pre-operative psychological distress and outcome following surgery. METHODS: The Short Form 36 (SF36) Health Survey Questionnaire and the Hospital Anxiety and Depression Scale (HADS) were administered to patients undergoing elective surgery for degenerative spine disease pre-operatively and at 3 and 12 months post-operatively. Levels of physical disability (SF-36 physical functioning (SF36PF) and bodily pain (SF36BP) scores) and psychological distress (HADS-anxiety and HADS-depression scores) before and after surgery were compared. RESULTS: A total of 302 patients were included (169 men, 133 women, mean age 55 years). Pre-operatively patients had worse physical scores than age-matched controls (SF36PF normative mean (S.D.) 80.97 (12.69) vs. pre-op 33.31 (24.7) P < 0.05). Of the 302 patients, 117 (39%) had significant anxiety or depression. Increased levels of anxiety or depression pre-operatively correlated with worse physical (SF-36PF and SF-36BP) scores pre-operatively (Spearman's r P < 0.05). Levels of anxiety and depression were reduced post-operatively and physical outcomes improved post-operatively. Physical function remained worse in those groups who had high levels of anxiety and depression pre-operatively but when matched for pre-operative physical function, psychological distress did not have any additional effect on outcome. CONCLUSIONS: Poor physical function pre-operatively correlates with psychological distress. Both physical and psychological symptoms improve after surgery. Physical outcome after surgery is strongly influenced by pre-operative physical functioning but not independently by psychological distress. Anxious and depressed patients should continue to be offered surgery if clinically indicated.

Recurrence rates of video-assisted thoracoscopic versus open surgery in the prevention of recurrent pneumothoraces: a systematic review of randomised and non-randomised trials.

BACKGROUND: Evidence supporting similar recurrence rates between video-assisted and open surgery for the treatment of recurrent pneumothorax is questionable, because the number of randomised trials is sparse and they are underpowered to detect any meaningful difference. Our aim was to do a systematic review of randomised and non-randomised studies to compare recurrence rates between the two forms of surgical access. METHODS: We did a systematic literature search for studies on pneumothorax surgery in Medline, Embase, Cochrane Library, trial registers on the internet, and conference abstracts, and identified 29 studies (four randomised and 25 non-randomised) eligible for inclusion. Meta-analysis was done by combining the results of reported recurrence rates in patients undergoing video-assisted thoracoscopic surgery compared with those having open surgery. Both fixed and random effects models were applied to the results pooled for analysis. RESULTS: In studies that did the same pleurodesis through two different forms of access, the relative risk (RR) of recurrences in patients undergoing video-assisted surgery compared with open surgery was similar between non-randomised and randomised studies (RR 4.880 [95% CI 2.670-8.922] vs 3.951 [0.858-18.193]), yielding an overall RR of 4.731 (2.699-8.291; p<0.0001). There was no evidence to suggest heterogeneity of trial results (p=0.88). The high RR of recurrence for video-assisted surgery remained robust to a random effects model (4.051 [1.996-7.465]; p<0.0001), by including all comparative studies (3.991 [2.584-6.164]; p<0.0001), with only high-quality studies used (4.016 [1.8468.736]; p<0.0001), and on a simulation biased in favour of video-assisted surgery when there were no events in either group (3.559 [2.165-5.852]; p<0.0001). INTERPRETATION: Both randomised and non-randomised trials are consistent in recurrence of pneumothoraces and show a four-fold increase when a similar pleurodesis procedure is done with a video-assisted approach compared with an open approach.

Both short- and long-acting D-1/D-2 dopamine agonists induce less dyskinesia than L-DOPA in the MPTP-lesioned common marmoset (Callithrix jacchus).

The current concept of dyskinesia is that pulsatile stimulation of D-1 or D-2 receptors by L-DOPA or short-acting dopamine agonists is more likely to induce dyskinesia compared to long-acting drugs producing more continuous receptor stimulation. We now investigate the ability of two mixed D-1/D-2 agonists, namely pergolide (long-acting) and apomorphine (short-acting), to induce dyskinesia in drug-nai;ve MPTP-lesioned primates, compared to L-DOPA. Adult common marmosets (Callithrix jacchus) were lesioned with MPTP (2 mg/kg/day sc for 5 days) and subsequently treated with equieffective antiparkinsonian doses of L-DOPA, apomorphine, or pergolide for 28 days. L-DOPA, apomorphine, and pergolide reversed the MPTP-induced motor deficits to the same degree with no difference in peak response. L-DOPA and apomorphine had a rapid onset of action and short duration of effect producing a pulsatile motor response, while pergolide had a slow onset and long-lasting activity producing a continuous profile of motor stimulation. L-DOPA rapidly induced dyskinesia that increased markedly in severity and frequency over the course of the study, impairing normal motor activity by day 20. Dyskinesia in animals treated with pergolide or apomorphine increased steadily, reaching mild to moderate severity but remaining significantly less marked than that produced by L-DOPA. There was no difference in the intensity of dyskinesia produced by apomorphine and pergolide. These data suggest that factors other than duration of drug action may be important in the induction of dyskinesia but support the use of dopamine agonists in early Parkinson's disease, as a means of delaying L-DOPA therapy and reducing the risk of developing dyskinesia.