Head and Neck Manifestations of Eosinophilic Granulomatosis with Polyangiitis: A Systematic Review.

OBJECTIVE: To conduct the first and only systematic review of the existing literature on head and neck manifestations of eosinophilic granulomatosis with polyangiitis to guide clinical decision making for the otolaryngologist. DATA SOURCES: PubMed, Cochrane Library, Scopus, and LILACS. REVIEW METHODS: A systematic review of the aforementioned sources was conducted per the PRISMA guidelines. RESULTS: From an initial 574 studies, 28 trials and reports were included, accounting for a total of 1175 patients with eosinophilic granulomatosis with polyangiitis. Among clinical and cohort studies, 48.0% to 96.0% of all included patients presented with head and neck manifestations. In a distinct group of patients detailed in case reports describing patients presenting with head and neck manifestations, patients on average fulfilled 4.6 diagnostic criteria per the American College of Rheumatology. Furthermore, 95.8% of reported cases were responsive to steroids, and 60% required additional therapy. CONCLUSION: Otolaryngologists are in a unique position for the early diagnosis and prevention of late complications of eosinophilic granulomatosis with polyangiitis. The American College of Rheumatology criteria should be relied on in the diagnostic workup. Close surveillance of these patients in a multidisciplinary fashion and with baseline complete blood counts, chest radiographs, and autoimmune laboratory tests is often necessary. Such patients with head and neck manifestations of the disease are nearly always responsive to steroids and often require additional immunosuppressive therapy or surgical intervention in cases of cranial neuropathies, temporal bone involvement, and refractory symptoms

Biphenotypic Sinonasal Sarcoma-Case Report and Review of Clinicopathological Features and Diagnostic Modalities.

Background Biphenotypic sinonasal sarcoma is a recently described malignancy showing dual differentiation with both myogenic and neural elements. Due to its histologic similarities to other sinonasal malignancies, it is a diagnostic challenge. Objective The main purpose of this article is to report a case of biphenotypic sinonasal sarcoma and to consolidate data and provide a comprehensive review regarding pathological differences between biphenotypic sarcoma and other sinonasal malignancies and diagnostic modalities used for biphenotypic sarcoma. Material and Methods A systematic review of all cases of biphenotypic sinonasal sarcoma was performed using electronic databases (PubMed and Medline). Data collected included age, gender, symptoms, sub-site of origin, immunophenotyping, metastasis, recurrence, treatment, duration of follow-up, and survival outcomes. Results Ninety-five cases of biphenotypic sarcoma were found with mean age at diagnosis of 52.36 years (range, 24-87 years). Female to male ratio was 2.27:1. Extra-sinonasal extension was present in 28%. Immunophenotyping revealed that S-100 and SMA (smooth muscle actin) were consistently positive, while SOX-10 was consistently negative. PAX3-MAML3 fusion [t (2; 4) (q35; q31.1)] was the most common genetic rearrangement. Surgical excision with or without adjuvant radiotherapy was the most frequent treatment modality used. Recurrence was observed in 32% of cases with follow-up. None of the cases reported metastasis. Three patients had died at the time of publication that included one case with intracranial extension. Conclusion Biphenotypic sarcoma is distinct sinonasal malignancy with unique clinicopathological features. Testing involving a battery of myogenic and neural immunomarkers is essential for diagnostic confirmation and is a clinically useful endeavor when clinical suspicion is high. © 2019 Georg Thieme Verlag KG Stuttgart. New York

Principles of Pituitary Surgery

Key Points 1. Understand the principles of pituitary surgery including the key-elements of surgical planning and decision-making 2. Identify the technical nuances distinguishing the endoscopic from the microscopic transsphenoidal approach 3. Understand the strategies utilized during the nasal, sphenoidal, and sellar stages of surgery that maximize tumor resection while minimizing complications and preserving sino- nasal anatomy/functio

Is Reconstruction of the Sella Necessary to Prevent Optic Chiasm Prolapse and Cerebrospinal Fluid Leakage Following Endoscopic Resection of Pituitary Macroadenomas?

Visual compromise is a common presentation of pituitary macroadenomas and is related to direct optic nerve and chiasm compression. Although the extent of visual recovery following treatment depends on the duration and severity of the visual compromise, the majority of patients experience gradual improvement in their vision. Delayed visual deterioration following treatment is typically related to either tumor recurrence or radiation-induced optic neuropathy, although visual worsening due to prolapse of the optic apparatus into a secondary empty sella has rarely been reported. In 1968, Guiot reported the first a case of reversible visual deterioration associated with optic chiasm prolapse following resection of a large pituitary macroadenoma (Guiot). Based on their observations, Guiot and collaborators recommended that a “prop” be placed in the sella at the time of transsphenoidal pituitary adenoma resection to prevent progressive herniation of the optic structures. Similarly, Hardy coined the term “preventive chiasmopexy” to describe filling of the sella cavity with autologous tissue such as muscle or fat following resection of large tumors to prevent this herniation phenomenon. While optic chiasm prolapse with associated visual deterioration appears to represent a rare occurrence, its true incidence and pathophysiological basis remain uncertain. Reconstruction of the sella with autologous tissues is also widely employed as a means to prevent postoperative cerebrospinal fluid leakage with these tissues typically harvested from a secondary operative site such as the abdomen. Although not frequently reported in the pituitary literature, complications of abdominal fat graft harvest include hematoma and seroma formation as well as infection with an incidence ranging from 1-7%. At our institution, we do not routinely perform dural reconstruction following transsphenoidal resection of pituitary macroadenomas using adipose tissue to prevent cerebrospinal fluid leakage or optic chiasm prolapse. In this study, we sought to determine the incidence of optic chiasm prolapse into the sellar defect by determining the radiographic position of the optic chiasm following surgery and incidence of delayed visual deterioration. Pages: 13-1

Prevention and Management of Bleeding During Endoscopic Approaches to Skull Base Pathologies

The rate of serious permanent morbidity and mortality with endonasal approaches has declined secondary to increased knowledge of the pertinent anatomy, advanced neuroimaging and navigation techniques, better surgical instruments, and improved exposure and reconstruction strategies.1-3 Although rare, vascular injury remains a potentially serious complication. However, with limited systematically-collected and reported data, the exact incidence rate of vascular injuries is difficult to determine. In terms of arterial injuries, the incidence based on reported series likely ranges from 0.3%-9% (Table 1),4-11 with higher rates most commonly associated with chordomas and chondrosarcomas involving the clivus. Venous injury is comparatively less severe and easier to manage. As a result, there is a comparatively lower impetus to publish epidemiological data and management strategies for these injuries. The consequences of arterial injury include fatal hemorrhage, vessel occlusion or thromboembolism causing infarction, development of a pseudoaneurysm (PA), carotid-cavernous fistula (CCF), subarachnoid hemorrhage (SAH), and vasospasm.6,7,9 Surgical expertise and detailed knowledge of the neurovascular anatomy is critical to the avoidance and management of vascular injuries. Pages: 20-2

Comprehensive Management of the Paranasal Sinuses in Patients Undergoing Endoscopic Endonasal Skull Base Surgery.

OBJECTIVE: The endonasal route often provides the most direct and safe approach to skull base pathology. In this article we review the literature with regard to management of the paranasal sinuses in the setting of skull base surgery. METHODS: We describe our institutional experience and review the literature of concurrent management of the sinusitis in patients undergoing endoscopic skull base surgery. RESULTS: Patients should be optimized preoperatively to ensure the endonasal route is a safe corridor to enter the intracranial cavity. Often the paranasal sinuses can be surgically addressed at the same time as endoscopic skull base surgery. We describe the technical details of management of the paranasal sinuses when addressing skull base pathology. CONCLUSIONS: Careful management of the paranasal sinuses throughout the peri-operative course is paramount to optimizing sinonasal function and safety

Endonasal Vascularized Flaps For Cranial Base Reconstruction

Since the introduction of extended endoscopic cranial base surgery, postoperative cerebrospinal fluid (CSF) leak has been a formidable and troublesome issue resulting in complications such meningitis, pneumocephalus, and the need for additional surgical interventions. Establishment of a watertight cranial base reconstruction is the most critical step in preventing postoperative CSF leakage. Historically, various free grafts, both synthetic and autologous, were utilized as repair materials for reconstruction of the cranial base defect often in combination with temporary CSF diversion. Free grafts are often sufficient for repair of small low flow, low pressure dural defects. High postoperative CSF leak rates reported in the initial endoscopic skull base literature are evidence that free grafts do not provide a reliably competent repair for large defects or direct high-flow CSF leaks. The introduction of the Hadad-Bassagasteguy vascularized nasoseptal flap has significantly reduced the reported CSF leak rate with a recent meta-analysis reporting that use of the vascularized flap is associated with a 7% rate of postoperative CSF leakage compared to 16% with free grafts alone for large dural defects.6 Since the initial description of the vascularized pedicled nasoseptal flap in 2006, many surgeons have developed a variety of alternative vascularized flaps for endonasal cranial base reconstruction. In this article, we summarize and compare several of the most clinically useful vascularized flaps including their harvest technique, indications and limitations, and potential complications. Pages: 28-3

Overcoming Obstacles in Endoscopic Skull Base Surgery: Challenging Corridors, Cases and Defects

Objectives: Endoscopic cranial base surgery is a rapidly advancing field with new innovations leading to an expansion in surgical approaches. The aim of this lecture is to provide the audience with an overview of some common challenges and obstacles faced by the endoscopic skull base surgeon and provide algorithms for confronting some of these difficult cases. Presentation: 47 minute

Inferior Meatus Maxillary Antrostomy: Time to Revisit a Previously Abandoned Surgical Technique?

Background: A small subset of patients have dysfunctional maxillary sinus mucosa. These patients require more aggressive surgical treatment to allow for gravity dependent mucous drainage, intra-operative and office debridements, and the delivery of irrigations and topical treatments. The aim of this study is to examine the use of inferior meatus maxillary antrostomy (IMMA) in combination with traditional maxillary antrostomy, in this challenging patient population. Methods: We performed a retrospective study from February, 2008 until April, 2014 with a 118 patients undergoing a total of 128 IMMA procedures. Data was gathered regarding patient demographic, prior therapy, post-operative medical therapy, IMMA patency, SNOT-22 scores, Lund Mackay score, and the presence of disease recurrence. Results: 118 patients (mean age 53) underwent 128 IMMA procedures. The mean length of follow-up was 1.6 years, and 66 % of patients had previous sinus surgery. 62 operations were for recalcitrant maxillary sinus disease, 36 for recalcitrant polyp disease, 6 for maxillary fungal disease, 4 for mucosal disease and 20 for odontogenic infections. 49 patients completed both pre and post-operative SNOT-22 scores, and the mean scores were 39 and 21, respectively. At 3 months post-operatively, we found that patients undergoing revision surgery and patients with nasal polyposis had the greatest SNOT-22 improvement. Males had 64% less SNOT-22 improvement compared to women at 3 months post operatively. In addition, 18% of patients did not experience any symptomatic improvement at 3 months post-operatively. With regards to post-operative medical therapy, men were more likely to have been prescribed steroid irrigations and antibiotics. Patients with fungal sinusitis had the greatest need for post-operative steroids. In addition, increased age correlated to fewer post-operative steroid courses. Out of 202 total inferior windows, 197 were patent post-operatively. There were 9 patients requiring revision surgery for maxillary sinus disease following IMMA, and patients requiring revision had the poorest outcomes. Conclusion: Inferior meatal windows prove to be efficacious in patients with mucocillaray dysfunction without disrupting the normal mucociliary flow pattern. The inferior turbinate is also preserved, which may be a less destructive alternative to a mega antrostomies. We have determined that IMMA is a safe and successful procedure for chronic maxillary sinus pathology