Waldenström's Macroglobulinemia and Cryoglobulinemic Glomerulonephritis: An Unusual Case of Monoclonal Gammopathy of Renal Significance.

Cryoglobulins are immunoglobulins that precipitate at temperatures below 37 °C and dissolve upon reheating. They can induce small-vessel vasculitis with renal involvement. Cryoglobulinemic glomerulonephritis is a rare manifestation that occurs in patients with monoclonal gammopathy, specifically Waldenström's macroglobulinemia. We present the case of a 52-year-old patient with a history of cutaneous vasculitis and hypothyroidism, who presented with generalized edema, moderate anemia, hypercholesterolemia, nephrotic range proteinuria of 12.69 g/day, microhematuria, arterial hypertension, and hypocomplementemia via the classical pathway, without acute kidney injury and with negative serological studies and positive cryoglobulins in the second determination. Serum and urine protein electrophoresis and immunofixation studies showed a monoclonal band of IgM and kappa light chain. Renal biopsy was consistent with cryoglobulinemic glomerulonephritis. In the context of dysproteinemia and cryoglobulinemic glomerulonephritis, bone-marrow aspiration and biopsy were performed, leading to the diagnosis of Waldenström's macroglobulinemia. Monoclonal gammopathies have been described in association with type I cryoglobulinemias. This described association is uncommon, which is why we present this case, along with a review of the literature.

Epidemiology of Fabry disease in patients in hemodialysis in the Madrid community.

This study screened for Fabry disease (FD) in patients in hemodialysis (HD) in the region of Madrid (CAM) with a cross-sectional design to evaluate HD-prevalent patients, followed by a three-year period prospective design to analyze HD-incident patients. INCLUSION CRITERIA: patients older than 18 years on HD in the CAM, excluding patients diagnosed with any other hereditary disease with renal involvement different from FD, that sign the Informed Consent (IC). EXCLUSION CRITERIA: underaged patients or not agreeing or not being capable of signing the IC. RESULTS: 3470 patients were included, 63% males and with an average age of 67.9±9.7 years. 2357 were HD-prevalent patients and 1113 HD-incident patients. For HD-prevalent patients, average time in HD was 45.2 months (SD 51.3), in HD-incident patients proteinuria was present in 28.4%. There were no statistical differences in plasmatic alpha-galactosidase A (α-GAL-A) activity or Lyso-GL-3 values when comparing HD-prevalent and HD-incident populations and neither between males and females. A genetic study was performed in 87 patients (2.5% of patients): 60 male patients with decreased enzymatic activity and 27 female patients either with a decreased GLA activity, increased Lyso-Gl3 levels or both. The genetic variants identified were: p.Asp313Tyr (4 patients), p.Arg220Gln (3 patients) and M290I (1 patient). None of the identified variants is pathogenic. CONCLUSIONS: 76% of HD Centers of the CAM participated in the study. This is the first publication to describe the prevalence of FD in the HD-population of a region of Spain as well as its average α-GAL-A-activity and plasmatic Lyso-Gl3 levels. It is also the first study that combines a cross-sectional design with a prospective follow-up design. This study has not identified any FD patient.

Surgical Treatment of Craniopharyngiomas in Adults: Comparison between Primary Surgery and Surgery for Recurrence.

OBJECTIVE: Few studies have investigated the differences in outcomes between primary and repeat surgery for a craniopharyngioma in adults. As a result, a treatment concept for adult patients with a craniopharyngioma has not yet been established. The present study aimed to retrospectively analyze adult patients with craniopharyngioma to compare surgical outcomes between primary surgery and surgery for recurrence. METHODS: The demographic and clinical data of 68 adult patients with craniopharyngioma who had primary surgery (n=50) or surgery for recurrence (n=18) were retrospectively analyzed. In addition, the patients were followed up for an average of 38.6 months (range: 1-133 months). RESULTS: The cohorts of patients undergoing primary surgery or repeat surgery did not differ preoperatively in terms of demographic data, or radiological tumor features. However, patients with recurrent craniopharyngioma had significantly more pituitary hormone deficits and hypothalamo-pituitary disorders before surgery compared with patients with newly diagnosed craniopharyngioma. The success rate of complete resection in primary surgery was 53.2%. Even after repeat surgery, a satisfactory rate of complete resection of 35.7% was achieved. Operative morbidity was increased neither in patients with repeat surgery compared with those with primary surgery (postoperative bleeding P=0.560; meningitis P=1.000; CSF leak P=0.666; visual disturbance P=0.717) nor in patients with complete resection compared with those with partial resection. We found no difference in recurrence-free survival between initial surgery and repeat surgery (P=0.733). The recurrence rate was significantly lower after complete resection (6.9%) than after partial resection (47.8%; P<0.001). CONCLUSION: Attempting complete resection is justified for not only those with newly diagnosed craniopharyngioma but also for those with recurrent craniopharyngioma. However, the surgeon must settle for less than total resection if postoperative morbidity is anticipated.

C1 Hemilaminectomy for Resection of Foramen Magnum Meningioma: 2-Dimensional Operative Video.

We present a case of a sizeable foramen magnum meningioma that was resected through a C1 hemilaminectomy in prone (concorde) position. The patient is a 51-year-old woman with a 3-month history of progressive paresthesia of the upper and lower extremities, followed by gait disturbance, and hand apraxia. There was no complaint of nuchal pain. On magnetic resonance imaging (MRI) a briskly enhancing extra-axial, intradural craniospinal lesion, extending from the basion of the lower clivus, over the tectorial membrane to the middle of the axis' body was discovered. There was significant transposition and compression of the medulla and corresponding focal hyperintensity on T2-weighted imaging. On physical examination, the patient was ambulatory independently, notwithstanding a pronounced spinal ataxia. There were deficits in sensation and proprioception, as well as urinary retention, but preserved function of the lower cranial nerves. In view of the profound transposition of the medulla, utilization of the corridor created by the tumor seemed feasible and we felt that a limited C1 hemilaminectomy would provide sufficient microsurgical access thus obviating a more extensive and invasive approach to the craniocervical junction. A gross-total resection was achieved; histopathology confirmed a World Health Organization (WHO) grade I angiomatous meningioma with a low-proliferation index. The patient was discharged home 3 days after surgery and her spinal ataxia resolved completely within 3 months of out-patient rehabilitation. At 3-year follow-up, there was no indication of residual or recurrence. The link to the video can be found at: https://youtu.be/WyShbfr-xi0 .

Surgical management of primary and secondary pilocytic astrocytoma of the cerebellopontine angle (in adults and children) and review of the literature.

Glial tumors in the cerebellopontine angle (CPA) are uncommon and comprise less than 1% of CPA tumors. We present four cases of pilocytic astrocytoma of the CPA (PA-CPA) that were treated in our department. Patients who received surgical treatment for PA-CPA from January 2004 to December 2019 were identified by a computer search of their files from the Department of Neurosurgery, Tübingen. Patients were evaluated for initial symptoms, pre- and postoperative facial nerve function and cochlear function, complications, and recurrence rate by reviewing surgical reports, patient documents, neuroradiological data, and follow-up data. We identified four patients with PA-CPA out of about 1500 CPA lesions (~ 0.2%), which were surgically treated in our department in the last 16 years. Of the four patients, three were male, and one was a female patient. Two were adults, and two were children (mean age 35 years). A gross total resection was achieved in three cases, and a subtotal resection was attained in one case. Two patients experienced a moderate facial palsy immediately after surgery (House-Brackmann grade III). In all cases, the facial function was intact or good (House-Brackmann grades I-II) at the long-term follow-up (mean follow-up 4.5 years). No mortality occurred during follow-up. Three of the patients had no recurrence at the latest follow-up (mean latest follow-up 4.5 years), while one patient had a slight recurrence. PA-CPA can be safely removed, and most complications immediately after surgery resolve in the long-term follow-up.

Clinical Situation, Therapy, and Follow-Up of Adult Craniopharyngioma.

CONTEXT: Craniopharyngioma is a rare neoplastic entity of the central nervous system. Childhood-onset craniopharyngioma is the subject of frequent research whereas the information on adult-onset craniopharyngioma is scarce. OBJECTIVE: The objective of this study was to examine the level of daily impairment in adult patients suffering from craniopharyngioma. DESIGN: Noninterventional patient registry indexed as PV4842 with the local ethics committee. SETTING: The study is set in a hospitalized and ambulatory setting. PATIENTS: 148 patients with adult-onset craniopharyngioma were recruited from 8 centers, 22 prospectively and 126 retrospectively. Mean follow-up was 31 months. INTERVENTIONS: No interventions performed. MAIN OUTCOME MEASURES: Complications, symptoms, body mass index (BMI), and quality of life (QoL; EORTC QLQ C30 and BN20) were recorded preoperatively and at follow-up. The hypotheses tested were generated after data collection. RESULTS: Complications were more frequent after transcranial than transsphenoidal approaches (31 % vs. 11%; P < 0.01). Preoperative obesity was present in 0% papillary and in 38% of all adamantinomatous craniopharyngiomas (P = 0.05), and diabetes insipidus was more frequent for papillary craniopharyngioma (36.8% vs. 16,7%; P < 0.05). Hormone deficits at follow-up were reduced in 16.9%, equal in 31.4%, and increased in 63.6% (P < 0.001). BMI increased from 28.7 ± 7.4 kg/m2 before surgery to 30.2 ± 7.4 kg/m2 at follow-up (P < 0.001). In QoL, a decrease of future uncertainty (62.5 vs. 36.8; P = 0.02) and visual disorders (38.9 vs. 12.0; P = 0.01) were observed in the prospective collective after surgery. CONCLUSIONS: Adult craniopharyngioma is associated with a complex sociological and psychological burden and hypothalamic dysfunction, warranting further investigation and emphasizing the need for a wider treatment approach.

Midline Suboccipital Subtonsillar Approach with C1 Laminectomy for Resection of Foramen Magnum Meningioma: 2-Dimensional Operative Video.

We present a case of a medium-sized foramen magnum meningioma that was resected through a midline suboccipital subtonsillar approach with C1 laminectomy in prone (Concorde) position. The patient is a 77-year-old woman with a 6-month history of intermittent vertigo, moderate gait instability, and slight decline of memory. On magnetic resonance imaging (MRI) an extra-axial intradural lesion was discovered that originated from the right epicondylar region just inferior to the jugular tubercle and occupied the anterolateral aspect of the foramen magnum. There was moderate transposition and compression of the medulla at the level of the cerebellar tonsils. On physical examination the patient was ambulatory independently without motor weakness but exhibited some gait instability. The function of the lower cranial nerves was preserved. A gross-total resection was achieved, histopathology confirmed a WHO grade-I meningothelial meningioma with a low-proliferation index. The patient was discharged home 5 days after surgery, her gait instability improved significantly immediately after surgery and had resolved completely after 2 weeks of inpatient rehabilitation. There was no other neurological deficit. At 3-month follow-up MRI, there was no indication of meningioma residual or recurrence. In summary, the midline suboccipital subtonsillar approach is a powerful tool with limited morbidity in the armamentarium for the microsurgical management of a variety of pathologies residing in the posterior cranial fossa and the craniocervical junction. Oftentimes the space created by the pathology opens up corridors that can be exploited for microsurgical access to avoid more extensive surgical approaches. The link to the video can be found at: https://youtu.be/0uUxs13ze7w .

Resection of a Petroclival Meningioma via the Endoscope-Assisted Retrosigmoid Approach: 2-D Operative Video.

We present a case of a petroclival meningioma that was resected through an endoscope-assisted retrosigmoid approach via corridors above and below the facial-vestibulocochlear nerve complex. The patient is a 61-year-old female with complaints of left-sided hypesthesia and neuralgia of the infraorbital and zygomatic region, intermittent periorbital myokymia, and a slight facial palsy (HB II). This 2D video demonstrates the operative technique, anatomical and surgical nuances of the skull base approach and microdissection of the tumor from the critical neurovascular structures. A gross total resection was achieved. The patient's facial and trigeminal symptoms resolved completely within a few weeks. At 2 year follow up there was no indication of residual or recurrence. In summary, the retrosigmoid approach with endoscopic assistance is an important and powerful tool in the armamentarium for the microsurgical management of meningiomas of the lateroventral skullbase of the posterior fossa. The link to the video can be found at: https://youtu.be/Px4XIRDoALc .

Retrosigmoid Transmeatal Endoscope-Assisted Approach in Semi-Sitting Position for Resection of Vestibular Schwannoma: 2-D operative Video.

We present a case of a mid-sized vestibular schwannoma (T3b according to the Hannover classification) that was resected through a retrosigmoid transmeatal approach in semi-sitting position under endoscopic assistance. The patient is a 52-year-old male with acute loss of functional hearing on the right side. Audiometry confirmed a loss of up to 60 dB and lost speech discrimination, there were no associated symptoms such as tinnitus or vertigo. This 2D video demonstrates positioning, OR set-up, anatomical and surgical nuances of the skull base approach and the operative technique for microdissection of the tumor from the critical neurovascular structures, especially the facial and cochlear nerves. A gross total resection was achieved and the patient discharged home after four days with unaltered function of the facial nerve (HB I). At one year follow up there was no indication of residual or recurrence. In summary, the retrosigmoid transmeatal approach is an important and powerful tool in the armamentarium for the microsurgical management of all kinds of vestibular schwannomas. Provided the necessary anesthesiological precautions and intraoperative procedures the semi-sitting position is safe and effective. If needed, the approach can be complemented by the use of an endoscope for visualization of the distal internal auditory canal. The link to the video can be found at: https://youtu.be/pPKT4_5nIn0 .

Mobilization of the Anterior Inferior Cerebellar Artery When Firmly Adherent to the Petrous Dura Mater-A Technical Nuance in Retromastoid Transmeatal Vestibular Schwannoma Surgery: 3-Dimensional Operative Video.

The anterior inferior cerebellar artery (AICA) usually runs loosely within the cerebellopontine cistern; in rare cases, however, it is firmly adherent to the petrous dura mater.1,2 Recognizing this variation is particularly important in vestibular schwannoma surgery via the retrosigmoid transmeatal approach to prevent the high morbidity associated with vascular injury. This video demonstrates a surgical technique to effectively mobilize the AICA when firmly adherent to the petrous dura mater. A 39-year-old man presented with a history of progressive right-sided hearing loss without facial weakness or other associated symptoms3. Magnetic resonance imaging (MRI) demonstrated an intracanalicular lesion, suggestive of vestibular schwannoma. During follow-up, audiometry confirmed a further slight deterioration of hearing and repeated MRI demonstrated tumor growth (T2 according to Hannover classification). Since the patient opted against radiosurgery, a retrosigmoid transmeatal approach under continuous intraoperative monitoring was performed in supine position. Following drainage of cerebrospinal fluid and exposure of the cerebellopontine cistern, the AICA was found to be firmly adherent to the petrous dura mater. Both structures were elevated conjointly and displaced medially for safe drilling of the inner auditory canal, sufficient exposure, and complete excision of the vestibular schwannoma. The patient had an excellent recovery, hearing and facial function were preserved, and no secondary neurological deficits noted.The patient consented to publication of this anonymized video.

Endoscopic assistance in retrosigmoid transmeatal approach to intracanalicular vestibular schwannomas - An alternative for middle fossa approach. Technical note.

BACKGROUND: Complete surgical removal of intracanalicular vestibular schwannomas with nerve VII and VIII sparing and without worsening patient's status is challenging. Also the choice of an optimal surgical technique, which is usually limited to selection between retrosigmoid transmeatal (RT) and middle fossa (MF) approach, can be a challenge. Although many previous studies documented superiority of RT to MF approach and vice versa, still no consensus has been reached regarding an optimal approach to intracanalicular vestibular schwannomas. In this technical note, we present RT approach with an endoscopic assistance and highlight its advantages over MF approach in surgical management of pure intracanalicular vestibular schwannomas. METHOD: RT approach with an endoscopic assistance is presented as an optimal surgical treatment for intracanalicular vestibular schwannomas, and its advantages are compared to those offered by MF approach. RESULTS: Under an endoscopic guidance, we found a residual tumor in the fundus of the inner acoustic canal and performed its gross total resection. CONCLUSIONS: RT approach is an excellent technique suitable for safe radical surgical treatment of T1 vestibular schwannomas; this technique is associated with lower morbidity risk than MF approach.

Indocyanine green videoangiography methodological variations: review.

Indocyanine green videoangiography (ICGVA) procedures have become widespread within the spectrum of microsurgical techniques for neurovascular pathologies. We have conducted a review to identify and assess the impact of all of the methodological variations of conventional ICGVA applied in the field of neurovascular pathology that have been published to date in the English literature. A total of 18 studies were included in this review, identifying four primary methodological variants compared to conventional ICGVA: techniques based on the transient occlusion, intra-arterial ICG administration via catheters, use of endoscope system with a filter to collect florescence of ICG, and quantitative fluorescence analysis. These variants offer some possibilities for resolving the limitations of the conventional technique (first, the vascular structure to be analyzed must be exposed and second, vascular filling with ICG follows an additive pattern) and allow qualitatively superior information to be obtained during surgery. Advantages and disadvantages of each procedure are discussed. More case studies with a greater number of patients are needed to compare the different procedures with their gold standard, in order to establish these results consistently.

Microscopic versus endoscopic pituitary surgery.

INTRODUCTION AND OBJECTIVE: The endoscopic techniques used in pituitary surgery have evolved greatly in recent years. Our objective in this study was to conduct a review of the systematic reviews published in the English language literature, to examine their consistency and conclusions reached following studies comparing microsurgery and endoscopic surgery in hypophyseal surgery. MATERIALS AND METHODS: We carried out a bibliographic search on MEDLINE and EMBASE electronic databases, selecting those systematic reviews and meta-analyses published from the year 2000 until January 2013, focusing on comparisons between microsurgical and endoscopic techniques. RESULTS: We concluded with type A consistency that hospital stay was shorter and diabetes insipidus and rhinological complications were less frequent in the endoscopy group. We concluded with type B consistency that lower rates of patient blood loss, shorter operative times, higher rate of gross total resection, lesser association to visual impairment and lower rate of hypopituitarism were observed in the endoscopy group. Vascular complications and cerebrospinal fluid fistulas were reduced with microsurgery. It is crucial to perform a combined analysis of all the systematic reviews treating a specific topic, observing and analysing the trends and how these are affected by new contributions. CONCLUSION: Randomized multicenter studies are necessary to resolve the controversy over endoscopic and microsurgical approaches in hypophyseal pathology.

[Endonasal skull base endoscopy]. / Endoscopia endonasal de base de cráneo.

INTRODUCTION AND OBJECTIVE: The endoscopic endonasal techniques used in skull base surgery have evolved greatly in recent years. Our study objective was to perform a qualitative systematic review of the likewise systematic reviews in published English language literature, to examine the evidence and conclusions reached in these studies comparing transcranial and endoscopic approaches in skull base surgery. MATERIAL AND METHODS: We searched the references on the MEDLINE and EMBASE electronic databases selecting the systematic reviews, meta-analyses and evidence based medicine reviews on skull based pathologies published from January 2000 until January 2013. We focused on endoscopic impact and on microsurgical and endoscopic technique comparisons. RESULTS: Full endoscopic endonasal approaches achieved gross total removal rates of craniopharyngiomas and chordomas higher than those for transcranial approaches. In anterior skull base meningiomas, complete resections were more frequently achieved after transcranial approaches, with a trend in favour of endoscopy with respect to visual prognosis. Endoscopic endonasal approaches minimised the postoperative complications after the treatment of cerebrospinal fluid (CSF) leaks, encephaloceles, meningoceles, craniopharyngiomas and chordomas, with the exception of postoperative CSF leaks. CONCLUSIONS: Randomized multicenter studies are necessary to resolve the controversy over endoscopic and microsurgical approaches in skull base surgery.