Gorham-Stout disease of skull base

Gorham-Stout disease of skull base

Lesions of the skull base are extremely rare and entail an even more devastating prognosis due to cervical instability and cerebrospinal fluid fistula. Due to the scarcity of this condition, the aim of a study was to give an overview of skull base Gorham-Stout disease and review the cases with such conditions reported in the literature.

In this case-based review, different aspects of skull base GSD are discussed, and a sample clinical case of GSD leading to cranial settling and rhinorrhea is presented. The characteristics, symptoms, and management of all English-language PubMed-reported cases were reviewed, and different features of presentation and methods of treatments were analyzed.

Based on the literature review, most of the cases encountered serious problems in the course of the disease. Meningitis/CSF leakage was detected in 12 of 26 collected cases, followed by hearing loss/tinnitus/otitis media in 10 cases, headache in 8, and neck pain/stiffness in 8 patients. Despite a variety of treatments, improvement was only observed in 8 of 26 collected cases. The reminders showed either stable condition or worsening and death.

All cases of GSD of the skull base should be evaluated for rhinorrhea/otorrhea and cranial settling, both of them being among the most life-threatening conditions. Since definite treatment, in order to stop disease progression, is sometimes impossible, symptomatic and supportive treatment should be started as possible. 1).


6 patients (5 males, 1 female) were included. The mean age at diagnosis was 3.5 years (range 0-10). Follow-up was of 5.2 years. Patients were divided into Naso-temporal (NT) and Vertebro-temporal (VT) groups following anatomical location. NT patients (4 patients) all had petrous defects extending anteriorly, including sphenoid, ethmoidal, and mandibular defects. They all had cerebrospinal fluid fistula (CSF) and recurrent meningitis (range from 3 to 7). Two of those patients had sequelae including deafness, paralysis, and epilepsy. VT patients (2 patients) all had temporal, occipital bone and cervical vertebrae defects. None had CSF leaks but both died from medullar compression (preceded by tetraparesis in one case). Overall, five out of six patients had type I Chiari malformation. Interferon seemed to be the most efficient medical treatment. Surgery included petrectomy, endonasal surgery for CSF leak management, and neurosurgery for medullar management but could not guarantee long-term effects.

The main issues in skull base defects are cerebrospinal fluid fistulas leaks and medullacompressions. Surgical treatment is necessary in both cases but can only be satisfactory if general medical treatment can stabilize the disease 2).

A 27-year-old man was diagnosed with GSD with the involvement of the maxillofacial bones and skull base. The patient developed SBO; LMS resulted from progressive osteolysis, and the patient died of an associated brainstem stroke. Careful follow-up with special emphasis on the early detection of intracranial complications is critical in patients presenting with progressive GSD with involvement of the skull base 3)


A two-year-old female child with radiological signs mimicking those of raised intracranial pressure is discussed. The differential diagnosis consists of skull base tumors, meningitisosteomyelitis of the skull basecongenital hydrocephalus, and congenital syndromes involving the skull base. Pathologically it can be very difficult to differentiate it from lymphangioma of the bone. Difficulty in establishing the diagnosis is discussed along with the failure of radiotherapy and palmidronate therapy to cause the arrest of the disease process and failure of surgery to provide stabilization. Girn et al. described the course of the disease in this child over the period of last eight years. This was the youngest case of Gorham’s described so far 4).


A 25-year-old woman with Chiari I malformation associated with Gorham’s syndrome presented with aggressive paresthesia following bacterial meningitis. Axial magnetic resonance imaging (MRI) and computed tomography (CT) cisternography revealed CSF leakage in the right petrous apex. A presyrinx state was diagnosed based on the clinical symptoms and MRI findings. With the resolution of bacterial meningitis, the spinal edema and tonsillar ectopia also improved. Surgical repair of the CSF leakage was performed by an endoscopic endonasal transsphenoidal approach to prevent recurrence of meningitis. The postoperative course was uneventful.

Skull base osteolysis in Gorham’s syndrome may induce Chiari I malformation and CSF leakage. We should pay attention to the acute progression of clinical symptoms because Gorham’s syndrome may predispose to the development of Chiari I malformation and may be complicated by CSF leakage 5).


A case of spinal and skull base Gorham’s disease that was reversed by radiation therapy administered while the spine was supported by a halo-vest 6).


1)

Maroufi SF, Habibi Z, Dabbagh Ohadi MA, Mohammadi E, Nejat F. Gorham-Stout disease of skull base leading to cranial settling and rhinorrhea: a case-based review. Childs Nerv Syst. 2022 Feb 25. doi: 10.1007/s00381-021-05394-3. Epub ahead of print. PMID: 35217940.
2)

Simon F, Luscan R, Khonsari RH, Toubiana J, Belhous K, James S, Blauwblomme T, Zerah M, Denoyelle F, Donadieu J, Couloigner V. Management of Gorham Stout disease with skull-base defects: Case series of six children and literature review. Int J Pediatr Otorhinolaryngol. 2019 Sep;124:152-156. doi: 10.1016/j.ijporl.2019.06.002. Epub 2019 Jun 5. PMID: 31195309.
3)

Nozawa A, Ozeki M, Hori T, Kato H, Ohe N, Fukao T. Fatal Progression of Gorham-Stout Disease with Skull Base Osteomyelitis and Lateral Medullary Syndrome. Intern Med. 2019 Jul 1;58(13):1929-1933. doi: 10.2169/internalmedicine.2118-18. Epub 2019 Feb 25. PMID: 30799352; PMCID: PMC6663530.
4)

Girn HR, Towns G, Chumas P, Holland P, Chakrabarty A. Gorham’s disease of skull base and cervical spine–confusing picture in a two year old. Acta Neurochir (Wien). 2006 Aug;148(8):909-13; discussion 913. doi: 10.1007/s00701-005-0806-x. Epub 2006 Jun 23. PMID: 16791440.
5)

Nagashima H, Mizukawa K, Taniguchi M, Yamamoto Y, Kohmura E. Cerebrospinal fluid leakage and Chiari I malformation with Gorham’s disease of the skull base: A case report. Neurol Neurochir Pol. 2017 Sep-Oct;51(5):427-431. doi: 10.1016/j.pjnns.2017.06.007. Epub 2017 Jul 13. PMID: 28743389.
6)

Mawk JR, Obukhov SK, Nichols WD, Wynne TD, Odell JM, Urman SM. Successful conservative management of Gorham disease of the skull base and cervical spine. Childs Nerv Syst. 1997 Nov-Dec;13(11-12):622-5. doi: 10.1007/s003810050155. PMID: 9454981.

Skull base meningioma outcome

Skull base meningioma outcome

Peritumoral edema (PTE) in skull base meningiomas correlates to the absence of an arachnoid plane and difference in outcome.

A subset of benign (WHO grade I) skull base meningiomas shows early progression/recurrence (P/R) in the first years after surgical resection.


Though various predictors of adverse postoperative outcomes among meningioma patients have been established, research has yet to develop a method for consolidating these findings to allow for predictions of adverse healthcare outcomes for patients diagnosed with skull base meningiomas.


The objective of a study was to develop three predictive algorithms that can be used to estimate an individual patient’s probability of extended length of stay (LOS), experiencing a nonroutine discharge disposition, or incurring high hospital charges following surgical resection of a skull base meningioma.

The study utilized data from patients who underwent surgical resection for skull base meningiomas at a single academic institution between 2017-2019. Multivariate logistic regression analysis was used to predict extended LOS, nonroutine discharge, and high hospital charges, and 2000 bootstrapped samples were used to calculate an optimism-corrected c-statistic. The Hosmer-Lemeshow test was used to assess model calibration, and p<0.05 was considered statistically significant.

A total of 245 patients were included in our analysis. Our cohort was majority female (77.6%) and Caucasian (62.4%). Our models predicting extended LOS, nonroutine discharge, and high hospital charges had optimism-corrected c-statistics of 0.768, 0.784, and 0.783, respectively. All models demonstrated adequate calibration (p>0.05), and were deployed an open-access, online calculator: https://neurooncsurgery3.shinyapps.io/high_value_skull_base_calc/.

Following external validation, these predictive models have the potential to aid clinicians in providing patients with individualized risk-estimation for healthcare outcomes following meningioma surgery 1).


Ko et al. retrospectively investigated the preoperative CT and MR imaging features for the prediction of P/R in skull base meningiomas, with emphasis on quantitative ADC values. Only patients had postoperative MRI follow-ups for more than 1 year (at least every 6 months) were included. From October 2006 to December 2015, total 73 patients diagnosed with benign (WHO grade I) skull base meningiomas were included (median follow-up time 41 months), and 17 (23.3%) patients had P/R (median time to P/R 28 months). Skull base meningiomas with spheno-orbital location, adjacent bone invasion, high DWI, and lower ADC value/ratio were significantly associated with P/R (P < 0.05). The cut-off points of ADC value and ADC ratio for prediction of P/R are 0.83 × 10- 3 mm2/s and 1.09 respectively, with excellent area under curve (AUC) values (0.86 and 0.91) (P < 0.05). In multivariate logistic regression, low ADC values (< 0.83 × 10- 3 mm2/s) and adjacent bone invasion are high-risk factors of P/R (P < 0.05), with odds ratios of 31.53 and 17.59 respectively. The preoperative CT and MRI features for prediction of P/R offered clinically vital information for the planning of treatment in skull base meningiomas 2).


1)

Jimenez AE, Khalafallah AM, Lam S, Horowitz MA, Azmeh O, Rakovec M, Patel P, Porras JL, Mukherjee D. Predicting High-Value Care Outcomes Following Surgery for Skull Base Meningiomas. World Neurosurg. 2021 Feb 7:S1878-8750(21)00188-1. doi: 10.1016/j.wneu.2021.02.007. Epub ahead of print. PMID: 33567369.
2)

Ko CC, Lim SW, Chen TY, Chen JH, Li CF, Shiue YL. Prediction of progression in skull base meningiomas: additional benefits of apparent diffusion coefficient value. J Neurooncol. 2018 Jan 20. doi: 10.1007/s11060-018-2769-9. [Epub ahead of print] PubMed PMID: 29353434.

Cerebrospinal fluid leak after endoscopic skull base surgery

Cerebrospinal fluid leak after endoscopic skull base surgery

Although rates of postoperative morbidity and mortality have become relatively low in patients undergoing transnasal transsphenoidal surgery (TSS) for pituitary adenomacerebrospinal fluid fistulas remain a major driver of postoperative morbidity. Persistent CSF fistulas harbor the potential for headache and meningitis.

Staartjes et al., trained and internally validated a robust deep neural network-based prediction model that identifies patients at high risk for intraoperative CSF. Machine learning algorithms may predict outcomes and adverse events that were previously nearly unpredictable, thus enabling safer and improved patient care and better patient counseling 1).


The objective of a study of Umamaheswaran et al., was to assess the incidence of CSF leak following pituitary surgery and the methods of effective skull base repair. This retrospective observational study conducted in a tertiary care hospital after obtaining due clearance from the Institutional ethics committee. The charts of patients who underwent endonasal pituitary surgery between 2013 and 2018 were studied and details noted. Patients undergoing revision surgery or with history of preoperative radiotherapy were excluded from the study. 52 patients were included in the study. Based on the type of CSF leak, the patients were grouped into four. 19 patients (36.5%) had an intraoperative CSF leak. 3 patients developed a postoperative CSF leak. Based on the histopathology, 4 patients had ACTH secreting tumor. 8 patients had growth hormone secreting tumor, 22 had gonadotropin secreting tumor, 9 patients had a non-functioning tumour and 9 patients had prolactinoma. The type of skull base repair performed in these patients were grouped into 4.18 patients underwent type I repair, 21 patients underwent type II repair, 8 patients underwent type III repair and 5 patients underwent type IV repair. They observed that the pedicled nasoseptal flap is particularly advantageous over other repair techniques, especially in low pressure leaks. The strategy for skull base repair should be tailored to suit each patient to minimise the occurrence of morbidity and the duration of hospital stay 2).


Cerebrospinal fluid leakage is always the primary complication during the endoscopic endonasal skull base surgery.

Dural suturing technique may supply a rescue method. However, suturing and knotting in such a deep and narrow space are difficult. Training in the model can improve skills and setting a stepwise curriculum can increase trainers’ interest and confidence.

Xie et al. constructed an easy model using silicone and acrylic as sphenoid sinus and using the egg-shell membrane as skull base dura. The training is divided into three steps: Step 1: extracorporeal knot-tying suture on the silicone of sphenoid sinus, Step 2: intra-nasal knot-tying suture on the same silicone, and Step 3: intra-nasal egg-shell membrane knot-tying suture. Fifteen experienced microneurosurgical neurosurgeons (Group A) and ten inexperienced PGY residents (Group B) were recruited to perform the tasks. Performance measures were time, suturing and knotting errors, and needle and thread manipulations. The third step was assessed through the injection of full water into the other side of the egg to verify the watertight suture. The results were compared between two groups.

Group A finishes the first and second tasks in significantly less time (total time, 125.1 ± 10.8 vs 195.8 ± 15.9 min) and fewer error points (2.4 ± 1.3 vs 5.3 ± 1.0) than group B. There are five trainers in group A who passed the third step, this number in group B was only one.

This low cost and stepwise training model improved the suture and knot skills for skull base repair during endoscopic endonasal surgery. Experienced microneurosurgical neurosurgeons perform this technique more competent 3).

In-Hospital Costs

All endoscopic transsphenoidal approach for pituitary surgeries performed from January 1, 2015, to October 24, 2017, with complete data were evaluated in a retrospective single-institution study. The electronic medical record was reviewed for patient factors, tumor characteristics, and cost variables during each hospital stay. Multivariate linear regression was performed using Stata software.

The analysis included 190 patients and average length of stay was 4.71 days. Average total in-hospital cost was $28,624 (95% confidence interval $25,094-$32,155) with average total direct cost of $19,444 ($17,136-$21,752) and total indirect cost of $9181 ($7592-$10,409). On multivariate regression, post-operative cerebrospinal fluid (CSF) leak was associated with a significant increase in all cost variables, including a total cost increase of $40,981 ($15,474-$66,489, P = .002). Current smoking status was associated with an increased total cost of $20,189 ($6,638-$33,740, P = .004). Self-reported Caucasian ethnicity was associated with a significant decrease in total cost of $6646 (-$12,760 to -$532, P = .033). Post-operative DI was associated with increased costs across all variables that were not statistically significant.

Post-operative CSF leak, current smoking status, and non-Caucasian ethnicity were associated with significantly increased costs. Understanding of cost drivers of endoscopic transphenoidal pituitary surgery is critical for future cost control and value creation initiatives 4).

Case series

see Cerebrospinal fluid leak after endoscopic skull base surgery case series.

References

1)

Staartjes VE, Zattra CM, Akeret K, Maldaner N, Muscas G, Bas van Niftrik CH, Fierstra J, Regli L, Serra C. Neural network-based identification of patients at high risk for intraoperative cerebrospinal fluid leaks in endoscopic pituitary surgery. J Neurosurg. 2019 Jun 21:1-7. doi: 10.3171/2019.4.JNS19477. [Epub ahead of print] PubMed PMID: 31226693.
2)

Umamaheswaran P, Krishnaswamy V, Krishnamurthy G, Mohanty S. Outcomes of Surgical Repair of Skull Base Defects Following Endonasal Pituitary Surgery: A Retrospective Observational Study. Indian J Otolaryngol Head Neck Surg. 2019 Mar;71(1):66-70. doi: 10.1007/s12070-018-1511-4. Epub 2018 Oct 15. PubMed PMID: 30906716; PubMed Central PMCID: PMC6401034.
3)

Xie T, Zhang X, Gu Y, Sun C, Liu T. A low cost and stepwise training model for skull base repair using a suturing and knotting technique during endoscopic endonasal surgery. Eur Arch Otorhinolaryngol. 2018 Jun 1. doi: 10.1007/s00405-018-5024-2. [Epub ahead of print] PubMed PMID: 29858924.
4)

Parasher AK, Lerner DK, Glicksman JT, et al. Drivers of In-Hospital Costs Following Endoscopic Transphenoidal Pituitary Surgery [published online ahead of print, 2020 Aug 24]. Laryngoscope. 2020;10.1002/lary.29041. doi:10.1002/lary.29041

Skull base chondrosarcoma outcome

Skull base chondrosarcoma outcome

Chondrosarcomas are relatively slow growing but locally aggressive. Local resection is often the treatment of choice. Radiotherapy may sometimes be employed although sensitivity is thought to be minimal. Metastatic spread is uncommon.


High-dose, double-scattered 3D conformal proton therapy alone or following surgical resection for skull-base chondrosarcoma is an effective treatment with a high rate of local control with no acute grade 3 radiation-related toxicity 1).


In 2010 Bloch et al. published an extensive systematic review of the English literature. The patients were stratified according to treatment modality, treatment history, histological subtype, and histological grade, and the recurrence rates were analyzed. A total of 560 patients treated for cranial chondrosarcoma were included. Five-year recurrence rate among all patients was 22% with median follow-up of 60 months and median disease-free interval of 16 months. Tumor recurrence was more common in patients who only received surgery or had mesenchymal subtype tumors 2).

Pencil-beam scanning proton therapy is an effective treatment for skull base tumors with acceptable late toxicity. Optic apparatus and/or brainstem compression, histology and gross tumor volume (GTV) allow independent prediction of the risk of local failure and death in skull base tumor patients 3).


Dibas et al. aimed to evaluate the incidence and survival rates and trends of skull base chondrosarcomas (SBC).

Data from SBC patients between 1975 and 2017 were extracted from the Surveillance, Epidemiology, and End Results (SEER) database. The age-adjusted rates (AAR) were calculated for the overall cases and based on gender, age, race, and histology. Furthermore, the relative survival rates for one, three, and five years, and the rates stratified to the aforementioned selected variables were computed. Besides, they conducted a joint point regression analysis to calculate the annual percent change (APC) and its associated standard error (SE) for AAR and mortality.

The AAR rate of SBC was 0.019 per 100,000. Higher AAR rates were observed in patients who were in the 65-74-year-age-group, females, Caucasians, and had none mesenchymal subtype. The relative one-year, three-year and five-year-survival rates were 99.58 %, 93.67 %, and 89.10 %, respectively. Lower survival rates were noted in patients who were males, African Americans, and had a mesenchymal subtype. The trend analysis has shown a significant yearly increase (P < 0.001) in AAR of SBC (APC ± SE = 0.0005 %±0.0001), along with a significant yearly decline in mortality rates (APC ± SE= -0.0202 %±0.0029).

Despite the increase in AAR over time, there has been a significant decline in mortality rates over time, which might have been due to the advancement of treatment modalities, improvement in diagnostic imaging, and modification in disease grading 4).

References

1)

Holtzman AL, Rotondo RL, Rutenberg MS, et al. Proton therapy for skull-base chondrosarcoma, a single-institution outcomes study. J Neurooncol. 2019;142(3):557-563. doi:10.1007/s11060-019-03129-8
2)

Bloch OG, Jian BJ, Yang I, et al. Cranial chondrosarcoma and recurrence. Skull Base. 2010;20(3):149-156. doi:10.1055/s-0029-1246218
3)

Weber DC, Malyapa R, Albertini F, et al. Long term outcomes of patients with skull-base low-grade chondrosarcoma and chordoma patients treated with pencil beam scanning proton therapy. Radiother Oncol. 2016;120(1):169-174. doi:10.1016/j.radonc.2016.05.011
4)

Dibas M, Doheim MF, Ghozy S, Ros MH, El-Helw GO, Reda A. Incidence and survival rates and trends of skull Base chondrosarcoma: A Population-Based study [published online ahead of print, 2020 Aug 11]. Clin Neurol Neurosurg. 2020;198:106153. doi:10.1016/j.clineuro.2020.106153

Precautions for endoscopic transnasal skull base surgery during the COVID-19 pandemic

PRECAUTIONS FOR ENDOSCOPIC TRANSNASAL SKULL BASE SURGERY DURING THE COVID-19 PANDEMIC

Key Words: COVID-19, coronavirus, transmission, endoscopic surgery, extended endoscopic skull base surgery, personal protective equipment (PPE), Powered Air Purifying Respirators (PAPR)

Zara M. Patel, MD; Juan Fernandez-Miranda, MD; Peter H. Hwang, MD; Jayakar V. Nayak, MD, PhD; Robert Dodd, MD, PhD; Hamed Sajjadi, MD; Robert K. Jackler, MD

Stanford University School of Medicine
Departments of Otolaryngology-Head & Neck Surgery and Neurosurgery

On March 12, 2020 the World Health Organization (WHO) officially announced the COVID-19 outbreak a pandemic, where to date there have been over 381,000 cases resulting in over 16,500

1

The compilation of information below is anecdotal, based primarily on personal communication with international colleagues reporting their individual experiences, and more data is needed before strict policies are set. There is no scientific evidence in this report. However, based on the preliminary observations summarized below and the fast course of events, it would be prudent to exercise an abundance of caution as more data accumulates. Our goal with this preliminary, rapid article is to alert surgeons of the need to temporarily alter their practices to avoid repeating the unfortunate experience of the early period of the epidemic.

Personal communication with colleagues deployed in Wuhan, China to combat the COVID-19 outbreak, have warned us about the potential risks of endonasal endoscopic surgery in COVID – 19 symptomatic patients. From their reports, a patient with mild flu-like symptoms underwent transphenoidal pituitary surgery in early January 2020, before the severity of this pandemic was well established. Multiple members (>14 by report) of the patient care team, both within and outside of the operating room, became infected from what became recognized as human-to-

2

deaths worldwide.
information that we can gain from our international colleagues who have already experienced this, or are currently going through it, should be utilized to protect our patients, our hospital teams and ourselves.

The COVID-19 pandemic is accelerating within the United States, and any

human transmission of COVID-19.
second case of intraoperative transmission of COVID-19 occurred later on January 2020, at the peak of the pandemic in Wuhan province. A young patient with a known pituitary adenoma developed fever and acute vision changes and was diagnosed with pituitary apoplexy and suspected viral pneumonia based on imaging studies. The surgical team was aware of the potential risks of infection, but given the acuity of symptoms proceeded with transphenoidal surgery using personal protective equipment (PPE). The neurosurgeon and two OR nurses employed N95 masks and the anesthesiologist reportedly used a “home-made” positive pressure helmet. The operation was completed successfully without incident and the surgical team was quarantined after surgery. Within 3-4 days, all of them developed fever and respiratory

Testing for COVID-19 prior to that time was scarce. A

symptoms compatible with pneumonia, except the anesthesiologist. Fortunately, all recovered with no sequelae. The patient, however, required prolonged intubation, but finally recovered.

A significant number of doctors who became infected and even died in Wuhan, China were anesthesiologists/critical care doctors, ophthalmologists, and otolaryngologists, possibly due to

3

From our colleagues in Iran, Dr. Ebrahim Razmpa, Professor of Otolaryngology at Tehran University Medical Sciences, Dr. Saee Atighechi, Associate Professor of Otolaryngology at Yazd University School of Medicine, and Dr. Mohammed Hossein Baradanfar, Professor and Chairman of Otolaryngology Yazd University School of Medicine, we have additionally heard that at least 20 otolaryngologists in Iran are currently hospitalized with COVID-19, with 20 more in isolation at home. They are testing only people who have been admitted to the hospital,

so those twenty at home are not confirmed, but have classic symptoms. A previously healthy 60 year old facial plastic surgeon died from COVID-19 three days ago. A young, otherwise healthy otolaryngology chief resident had a short prodrome, rapidly decompensated and died from what was found to be acute myocarditis and cardiac arrest. It was recently confirmed from these colleagues that he did also test positive for COVID-19.

The British Association of Otorhinolaryngology has now also stated two of its consultants are on

8

Our colleague Dr. Puya Deghani-Mobaraki, in Italy, also reports otolaryngologists being affected adversely, but his information is about the possible loss of smell and taste that this virus brings. They are not only seeing it in their patients, but they have noticed it within their own ranks, in otherwise healthy asymptomatic doctors, at rates far above what could be considered normal. This observation has also been reported in the media regarding patients, as an under-reported

9,10

quarantine or to come in and be tested, depending on individual evaluation.

Based on this information, and until we know more, we are performing only urgent/emergent surgery at Stanford University at this time. Due to this apparent high risk with endoscopic transnasal surgery on COVID-19 symptomatic patients, in spite of current limitations in testing capacity, our institution has approved testing for COVID-19 in pre-operative patients needing this type of procedure urgently or emergently. This is true even for asymptomatic patients (ie. no cough and/or fever), although the true risk in this cohort of patients is still unknown. If the test is negative and the patient is asymptomatic, we may proceed using normal levels of protective gear; however, the rate of false negative tests is still to be determined, and until this is known , the use of additional levels of PPE, such as N95 and face shields can be considered. If the test is

the high viral shedding from the nasal and oropharyngeal cavity.
high risk of infection when taking care of COVID-19 patients without PPE. High risk procedures include intubation and procedures involving the upper respiratory tract and gastrointestinal tract with risk for aerosolization, such as endoscopy, bronchoscopy, and laryngoscopy.

7
Hospital “Hippocrates” are quarantined, as a doctor at the Otolaryngology Clinic reportedly

ventilators and being treated for COVID-19.

In Athens, 21 staff members of the Athens General

tested positive for COVID-19.

aspect of this disease process.
France in association with COVID-19 that the government has issued an official statement instructing citizens with this symptom to contact their physicians, who may advise self-

11

In fact, this symptom has been seen now so commonly in

Healthcare providers are at

positive, we defer surgery if at all possible until the infection is cleared, verified by repeat testing. When endonasal surgery cannot be postponed in a COVID-19 positive patient, based on guidelines now being used in China, we have recommended to our institutional officials that we utilize full PAPR (an enclosed powered system with HEPA filter), acknowledging that they have challenging decisions surrounding allocation of limited resources that are urgently needed by our

12

The question of whether two separate negative tests are needed before surgery, or if one is sufficient, is under active discussion. The test that we are using, developed at Stanford, is an in- house assay that uses a real time RT-PCR for SARS-CoV-2. This first screens for the presence of virus envelope protein, and if positive then evaluates for the presence of the RNA-dependent RNA polymerase gene for confirmation (Developed by Benjamin Pinsky MD, Stanford University). Positive results from this test have been demonstrated to be very sensitive and very specific and have been given early approval by the FDA. The Chinese CDC test uses different gene targets and primers and thus may, or may not, have a different accuracy profile. Conservation of precious testing and PPE resources is another reason to limit these operations to the bare minimum at this time. We also recommend use of as minimal an OR team as necessary and that no trainees or observers be allowed in the room both for reasons of safety and to preserve PPE.

In the clinic setting, we have similarly restricted visits to only urgent/emergent patients and have ceased the use of spray anesthetic/decongestants, opting instead for nasal pledgets as needed, but preferably avoiding endoscopy whenever possible. We are using N95 masks, face shields and gowns for all outpatient nasal endoscopies.

Please keep in mind that from the time of this submission, the situation may have evolved, and our policies may have changed. We hope that more hard data becomes available soon upon which to base these important decisions. We follow with tempered optimism the evolution of this pandemic in China, where at this point no new local cases have been reported for several days now, with gradual return to normal surgical activities, including endoscopic endonasal surgery.

We thank our international colleagues who have given us this important information, and we extend wishes of safety and health to all our otolaryngology, neurosurgery, and critical care/anesthesia colleagues at this challenging time.

John’s Hopkins Coronovirus Center. https://coronavirus.jhu.edu/map.html Accessed March 21, 2020

2China Newsweek. View.inews.qq.com/a/20200125A07TT200?uid=&devid=BDFE70CD-5BF1-4702-91B7- 329F20A6E839&qimei=bdfe70cd-5bf1-4702-91b7-329f20a6e839

3https://www.bloomberg.com/news/articles/2020-03-17/europe-s-doctors-getting-sick-like-in-wuhan-chinese- doctors-say?fbclid=IwAR2ds9OWRxQuMHAuy5Gb7ltqUGMZNSojVNtFmq3zzcSLb_bO9aGYr7URxaI

critical care teams taking care COVID-19 patients.
should be considered whenever possible. Because endonasal surgery creates clouds of droplets and aerosols which may permeate the operating environment, anyone in the operating theater requires the same protection when operating on known COVID-19 positives.

Alternatively, a transcranial approach

3van Doremalen N, Bushmaker T, Morris DH, et al. Aerosol and Surface Stability of SARS-CoV-2 as Compared with SARS-CoV-1. N Engl J Med. 2020 Mar 17. doi: 10.1056/NEJMc2004973. [Epub ahead of print]

55Zou L, Ruan F, Huang M, et al. SARS-CoV-2 Viral Load in Upper Respiratory Specimens of Infected Patients. N Engl J Med. 2020 Mar 19;382(12):1177-1179. doi: 10.1056/NEJMc2001737. Epub 2020 Feb 19.

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6Xu K , Lai XQ , Liu Z . Zhonghua Er Bi Yan Hou Tou Jing Wai Ke Za Zhi. 2020 Feb 2;55(0):E001. doi:

10.3760/cma.j.issn.1673-0860.2020.0001. [Epub ahead of print] [Suggestions for prevention of 2019 novel coronavirus infection in otolaryngology head and neck surgery medical staff].
[Article in Chinese (translated via Google translator); Abstract available in Chinese from the publisher]

7 https://news.sky.com/story/coronavirus-experts-say-new-symptoms-could-be-loss-taste-or-smell-11961439 8 8https://www.euractiv.com/section/politics/news/cracks-appear-in-nordic-response-to-covid-19-crisis/

9 9https://en.radiofarda.com/a/loss-of-sense-of-smell-among-iranians-coinciding-with-coronavirus- epidemic/30478044.html

10 https://www.forbes.com/sites/judystone/2020/03/20/theres-an-unexpected-loss-of-smell-and-taste-in-coronavirus- patients/#48e2a8c85101

11https://www.sortiraparis.com/news/coronavirus/articles/210162-coronavirus-update-on-the-situation-in-paris-and- ile-de-france-controls-reinforc/lang/en

12 Lian, Tingbo (Editor). Handbook of COVID-19 Prevention and Treatment. The First Affiliated Hospital. Zhejiang University School of Medicine. Compiled according to Clinical Experience.