Portable MRI to assess optic chiasm decompression after endoscopic endonasal resection of sellar and suprasellar lesions.

OBJECTIVE: Low-field portable MRI (pMRI) is a recent technological advancement with potential for broad applications. Compared with conventional MRI, pMRI is less resource-intensive with regard to operational costs and scan time. The application of pMRI in neurosurgical oncology has not been previously described. The goal of this study was to demonstrate the efficacy of pMRI in assessing optic nerve decompression after endoscopic endonasal surgery for sellar and suprasellar pathologies. METHODS: Patients who underwent endoscopic endonasal surgery for sellar and suprasellar lesions at a single institution and for whom pMRI and routine MRI were performed postoperatively were retrospectively reviewed to compare the two imaging systems. To assess the relative resolution of pMRI compared with MRI, the distance from the optic chiasm to the top of the third ventricle was measured, and the measurements were compared between paired equivalent slices on T2-weighted coronal images. The inter- and intrarater correlations were analyzed. RESULTS: Twelve patients were included in this study (10 with pituitary adenomas and 2 with craniopharyngiomas) with varying degrees of optic chiasm compression on preoperative imaging. Measurements were averaged across raters before calculating agreement between pMRI and MRI, which demonstrated significant interrater reliability (intraclass correlation coefficient [ICC] = 0.78, p < 0.01). Agreement between raters within the pMRI measurements was also significantly reliable (ICC = 0.93, p < 0.01). Finally, a linear mixed-effects model was specified to demonstrate that MRI measurement could be predicted using the pMRI measurement with the patient and rater set as random effects (pMRI ß coefficient = 0.80, p < 0.01). CONCLUSIONS: The results of this study suggest that resolution of pMRI is comparable to that of conventional MRI in assessing the optic chiasm position in relation to the third ventricle. Portable MRI sufficiently demonstrates decompression of the optic chiasm after endoscopic endonasal surgery. It can be an alternative strategy in cases in which cost, scan-time considerations, or lack of intraoperative MRI availability may preclude the ability to assess adequate optic nerve decompression after endoscopic endonasal surgery for sellar and suprasellar lesions.

A Unique Subset: Idiopathic Intracranial Hypertension Presenting as Spontaneous CSF Leak of the Anterior Skull Base.

Introduction Spontaneous cerebrospinal fluid (CSF) leaks represent a unique clinical presentation of idiopathic intracranial hypertension (IIH), lacking classical features of IIH, including severe headaches, papilledema, and markedly elevated opening pressures. Methods Following a single-institution retrospective review of patients undergoing spontaneous CSF leak repair, we performed a literature review of spontaneous CSF leak in patients previously undiagnosed with IIH, querying PubMed. Results Our literature review yielded 26 studies, comprising 716 patients. Average age was 51 years with 80.8% female predominance, and average body mass index was 35.5. Presenting symptoms included headaches (32.5%), visual disturbances (4.2%), and a history of meningitis (15.3%). Papilledema occurred in 14.1%. An empty sella was present in 77.7%. Slit ventricles and venous sinus stenosis comprised 7.7 and 31.8%, respectively. CSF leak most commonly originated from the sphenoid sinus (41.1%), cribriform plate (25.4%), and ethmoid skull base (20.4%). Preoperative opening pressures were normal at 22.4 cm H 2 O and elevated postoperatively to 30.8 cm H 2 O. 19.1% of patients underwent shunt placement. CSF leak recurred after repair in 10.5% of patients, 78.6% involving the initial site. A total of 85.7% of these patients were managed with repeat surgical intervention, and 23.2% underwent a shunting procedure. Conclusion Spontaneous CSF leaks represent a distinct variant of IIH, distinguished by decreased prevalence of headaches, lack of visual deficits, and normal opening pressures. Delayed measurement of opening pressure after leak repair may be helpful to diagnose IIH. Permanent CSF diversion may be indicated in patients exhibiting significantly elevated opening pressures postoperatively, refractory symptoms of IIH, or recurrent CSF leak.

PIK3CA mutation in a case of CTNNB1-mutant sinonasal glomangiopericytoma.

Glomangiopericytomas are rare, primary sinonasal tumors. The existing literature is mostly limited to reports describing the clinicopathologic characteristics of these tumors. Comprehensive genetic characterization of glomangiopericytomas remains lacking. Whole-exome sequencing of a case of glomangiopericytoma was performed under an institutional review board-approved protocol. A 69-yr-old female underwent surgical resection of a glomangiopericytoma. Whole-exome sequencing revealed somatic mutations in CTNNB1 and PIK3CA, the former previously associated with this pathology but the latter not described. Concurrent dysregulation of Wnt/ß-catenin and PI3K/AKT/mTOR signaling, secondary to mutations in these two oncogenes, may be amenable to targeted treatment with existing clinically approved drugs. Genomic characterization of glomangiopericytomas remains lacking. This study reports novel coexistence of PIK3CA and CTNNB1 mutations in a case of glomangiopericytoma that may offer insight into the pathogenesis and potential for targeted medical therapies of this rare tumor.

Comprehensive Genomic Characterization of A Case of Granular Cell Tumor of the Posterior Pituitary Gland: A Case Report.

Granular cell tumors of the pituitary belong to a rare family of neoplasms, arising from the posterior pituitary gland. Although considered benign, they may cause significant morbidity and residual disease after resection can lead to poor clinical outcomes. Currently, there is no known medical therapy for any posterior pituitary gland tumor, in part due to sparse molecular characterization of these lesions. We report data from whole exome sequencing of a case of granular cell tumor of the pituitary, performed under an institutional review board approved protocol. A 77 year-old female underwent resection of an incidentally diagnosed pituitary mass that was causing radiographic compression of the optic nerves with a subclinical temporal field defect and central hypothyroidism. The pathology of the resected specimen demonstrated a granular cell tumor of the posterior pituitary gland. Whole-exome sequencing revealed mutations predicted to be deleterious in key oncogenes, SETD2 and PAX8, both of which have been described in other cancers and could potentially be amenable to targeted therapies with existing approved drugs, including immune checkpoint inhibitors and histone deacetylase inhibitors, respectively. To our knowledge, this is the first comprehensive genomic characterization of granular cell tumor of the posterior pituitary gland. We report mutations in oncogenes predicted to be deleterious and reported in other cancers with potential for therapeutic targeting with existing pharmacologic agents. These data provide new insights into the molecular pathogenesis of GCT of the pituitary and may warrant further investigation.

Large-scale second-hit AIP deletion causing a pediatric growth hormone-secreting pituitary adenoma: Case report and review of literature.

Gigantism (early-onset acromegaly) is a rare pediatric disorder caused by a growth hormone (GH)-secreting pituitary adenoma. Approximately 50% patients of gigantism have a germline mutation, most commonly an inactivating mutation in the aryl-hydrocarbon interacting receptor protein (AIP) gene on chromosome 11q13.2. We present an 11-year-old male patient with a GH-secreting pituitary macroadenoma who presented with excessive growth spurts, behavioral changes, and frontal headaches. He was successfully treated with an endoscopic endonasal gross total resection and subsequently demonstrated biochemical cure. Whole-exome sequencing showed a heterozygous germline mutation in the AIP gene suggesting pituitary adenoma predisposition. Analysis of the tumor tissue revealed a large-scale deletion on chromosome 11 overlapping with AIP leading to bi-allelic AIP loss. Coincident germline and somatic AIP mutations were likely causal in formation of a GH-secreting adenoma with an aggressive phenotype. This case exemplifies the need for early diagnosis and curative surgery in the management of AIP-mutated pituitary adenomas.

Visual prognosis in compressive optic neuropathy secondary to sphenoid sinus mucocele: A systematic review.

Sphenoid sinus mucoceles (SSMs) are rare, benign lesions that can expand, often presenting with ocular symptoms-decreased vision, diplopia, visual field defects, proptosis, and external ophthalmoplegia. Reported cases are few, visual compromise varies, and factors affecting visual prognosis are poorly characterized. We investigate whether prompt surgical intervention (within 2 weeks of visual symptom onset) affects best-corrected visual acuity (BCVA) regained in patients with vision loss secondary to compressive SSM. We present a retrospective review of three cases and published literature to date. Our primary outcome was BCVA regained after surgical intervention; secondary outcomes included change in visual field defect and ophthalmological symptoms other than vision loss. Our three cases of SSM varied in onset, ranging from several hours to several months with patients aged from 13 to 80 years. All patients had severe vision loss to light perception (LP) or worse. Rapid neuro-imaging and urgent surgical intervention improved vision to count fingers at best. Of the two patients who underwent prompt decompression, one improved from no LP to LP and the other did not recover any vision. The patient who had visual loss for 3 months before intervention improved from LP to 20/400. Findings from our literature search, which yielded 12 cases of urgent intervention, supported the variability in visual prognosis despite prompt surgical intervention. SSMs are rare, pathologically benign lesions which can expand to cause ocular involvement. Prompt diagnosis and surgical decompression are recommended, but visual recovery may be limited even with urgent intervention.

Low-Grade, Nonintestinal Nonsalivary Sinonasal Adenocarcinoma Associated With an Exophytic Schneiderian Papilloma: A Case Report.

Sinonasal adenocarcinoma is a rare tumor of the head and neck accounting for 10% to 20% of all primary malignancies of the nose and paranasal sinuses. There tumors are classified as salivary, intestinal and nonintestinal, nonsalivary. Low-grade nonintestinal nonsalivary are rare tumors whose diagnosis is essentially that of exclusion. Here we present the first case of one such tumor associated with an exophyic Schneiderian papilloma. A 71-year-old retired aerospace engineer presented with a 1-year history of severe nasal obstruction. Endoscopy and compted tomography imaging demonstrated a polypoid lesion occupying his entire right nasal cavity extending into and filling the nasopharynx. Biopsy suggested adenocarcinoma, at least in situ and the patient subsequently underwent complete resection. Pathologic evaluation demonstrated polypoid tumor consistent with a low-grade papillary adenocarcinoma with micropapillary architecture associated with a small amount of residual exophytic Schneiderian papilloma. Immunohistochemistry revealed diffuse expression of CK7, CK 5/6, and S100 protein in tumor cells. Expression of p63 was seen in basal cells only. Tumor cells did not show expression of CK20, CDX2 (intestinal markers), mammaglobin, GATA3 (salivary markers), PAX8, WT1, nor estrogen, progesterone, or androgen receptors confirming its nonintestinal nonsalivary differentiation.

Correction of the deviated septum: from ancient Egypt to the endoscopic era.

BACKGROUND: Obstructed nasal breathing can occur due to deviation of the nasal septum. When the external nose appears grossly normal and cosmesis is not the focus, septoplasty has been the procedure used to straighten the septum with the goal of improving nasal airflow. Septoplasty has evolved over time. METHODS: A historical literature review was conducted to look for primary source journal articles and medical conferences proccedings addressing the evolution of the septoplasty procedure. RESULTS: Early techniques involved forcible fractures and splinting. Submucous resection was the first major advancement in surgical technique. Once the complications resulting from this technique were observed, it was subsequently revised with attempts to better address the caudal septal deviation. Attention was then turned to better incorporating the role surrounding support structures, such as the upper lateral cartilages. The premaxilla-maxilla approach attempted to address the overall nasal structure to best improve nasal breathing. The advent of endoscopic technique has been the most recent shift in surgical technique with improved visualization allowing for targeted septoplasty and reoperation on complicated cases including pituitary and skull base surgery. CONCLUSION: This paper discusses the evolution of septoplasty techniques over time from the initial undertakings of the ancient Egyptians to the modern-day septoplasty. While the principles behind septoplasty have remained much the same, experience has allowed for refinement of surgical technique. No doubt new instrumentations and innovations will further help to tailor the practice of septoplasty to the anatomy and functional needs of each individual patient.

Endoscopic resection of an anterior skull-base Schwannoma.

Sinonasal Schwannomas represent less than 4% of all head and neck Schwannomas. These neural sheath tumors arise from the ophthalmic and maxillary divisions of the trigeminal nerve, as well as autonomic nerves from sympathetic fibers of the carotid plexus and parasympathetic fibers of the sphenopalatine ganglion. Patients commonly present with nonspecific symptoms such as nasal obstruction, epistaxis, and anosmia. Nasal endoscopy usually reveals a unilateral polypoid mass. Diagnostic imaging with computed tomography (CT) and magnetic resonance (MR) is typically nonspecific. Diagnosis may be delayed due to the masquerade of common sinonasal conditions, such as allergic rhinitis and chronic rhinosinusitis. We report a case involving a 51-year-old male with an anterior skull-base Schwannoma that was excised endoscopically. Clinical features, imaging characteristics, histopathology, and treatment of sinonasal Schwannomas are discussed.

Evolution of medical management of chronic rhinosinusitis.

Chronic rhinosinusitis (CRS) is a surprisingly common, poorly defined, and notoriously difficult-to-treat disease. It has a complex pathophysiology that often, but not always, involves nasal or paranasal sinus infection. Anatomic variations that predispose the sinuses to obstruction may play a role, but are unusual sole causes of chronic disease. Other possible causative factors include allergic or nonallergic inflammation, mucociliary dysfunction, aspirin intolerance (Samter's triad), immunodeficiency, and cystic fibrosis. Although a majority of patients achieve long-term relief from CRS after successful endoscopic sinus surgery, a significant proportion do not, and are likely to benefit from sustained postsurgical medical therapy. Medical therapy for CRS may include treatment with corticosteroids, antibiotics, antifungal agents, antihistamines, leukotriene modifiers, nasal decongestants, mucolytics, and nasal irrigations. The selection of appropriate medical therapy is based on endoscopic evaluation, sinus cultures, and symptoms. Computed tomography, the imaging standard for evaluation of the sinuses, provides information about the extent and distribution of mucosal disease beyond what is visible endoscopically. Because it fails to provide information on the origin of the mucosal changes, computed tomography provides limited information to guide medical therapy.