journal of dermatology research reviews & reports

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Current Dermatology Reports

Current Dermatology Reports provides in-depth review articles contributed by international experts on the most significant developments in the field. By presenting clear, insightful, balanced reviews that emphasize recently published papers of major importance, the journal elucidates current and emerging approaches to the diagnosis, treatment, management, and prevention of dermatological conditions.

• Jeffrey M. Weinberg,
• Jonathan Silverberg

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Volume 13, Issue 2

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• Dermatol Reports
• v.12(2); 2020 Sep 23

Dermatology Reports: A journal coming to a new life

Dermatology Reports is an open access journal published online since 2009. It is devoted to any aspects of dermatology and it is indexed in most citation databases, such as Medline and Embase.

Recently, the journal changed, in a sense, its life by becoming the Official Journal of the Italian Association of Hospital Dermatologists (ADOI - Associazione Dermatologi-Venereologi Ospedalieri Italiani e della sanità pubblica) and, thanks to an agreement with ADOI, of the Italian Melanoma Intergroup (IMI).

Being the official journal of a national professional association poses new responsibilities to the journal editors, and traces some new ways for the journal, especially when one considers the already large number of scientific publications available to dermatologists.

The key word we, as editors, want to emphasise is Education . Educating professionals is our scope (Table). We will emphasise the role of research methodology and evidence based approaches in informing clinical practice. We will also exploit the value of single or multiple case reports to improve professional skills. A voice will be given to dermatological nurses who may find, in the journal, a sort of bridge to their medical counterpart, so that a better harmonization of activities can be promoted. We will also aim to have patient associations on board. The point of view of patients is vitally important. The structure of the papers in this case may not follow the usual format, a more unformal and narrative structure will be welcome.

Even if the journal will have, by its nature, an Italian perspective on the organization of clinical practice, it is very open to contributions from dermatologists of any country in the world with a global perspective.

We do not care that much about citation indexes or impact factor as usually measured in bibliometric analyses, but will care a lot about the satisfaction of contributors and of our readership. Please, feel free to comment on the contents of the journal and criticise us if you are not satisfied with the quality of the journal. We very much look forward to working with all of you to make the journal an ongoing enterprise to improve dermatological practice. Thank you in advance for the help you will provide us on such an ambitious task.

Thematic areas of the journal.

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Evaluation of Dupilumab in Patients With Bullous Pemphigoid

• 1 Department of Dermatology, Peking University First Hospital, Beijing, China
• 2 Department of Dermatology, Rui Jin Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, China
• 3 Hospital of Dermatology, Chinese Academy of Medical Sciences and Peking Union Medical College, Nanjing, Jiangsu, China
• 4 Department of Dermatology & Venerology, Rare Diseases Center, West China Hospital, Sichuan University, Chengdu, Sichuan, China
• 5 Department of Dermatology, The Second Xiangya Hospital, Central South University, Changsha, Hunan, China
• 6 Department of Dermatology, Shandong Provincial Hospital for Skin Diseases & Shandong Provincial Institute of Dermatology and Venereology, Shandong First Medical University & Shandong Academy of Medical Sciences, Jinan, Shandong, China
• 7 National Autoimmune Bullous Disease Cooperative Group, China
• 8 National Clinical Research Center for Skin and Immune Diseases, Beijing, China
• 9 Beijing Key Laboratory of Molecular Diagnosis on Dermatoses, Beijing, China

Question   What is the efficacy and safety of dupilumab in treating patients with bullous pemphigoid?

Findings   In this retrospective cohort study of 146 patients with bullous pemphigoid, 127 (87%) achieved disease control within 4 weeks, and the safety profile was favorable. Patients with serum anti-BP180 antibody levels of at least 50 relative units per milliliter and female sex may respond better.

Meaning   This study suggests that dupilumab may be an effective and safe alternative for patients with bullous pemphigoid.

Importance   Dupilumab is a theoretically novel therapy for bullous pemphigoid (BP). However, its effectiveness and safety have yet to be confirmed in a large-scale study.

Objective   To assess the efficacy and safety of dupilumab in patients with BP and evaluate factors that potentially affect short-term and long-term outcomes.

Design, Setting, and Participants   A retrospective cohort study was conducted from January 1, 2021, to July 31, 2022. The median (IQR) follow-up period was 24.6 (11.5-38.4) weeks. This multicenter study was performed in 6 dermatology departments of the National Autoimmune Bullous Diseases Cooperative Group of China. Adult patients with BP that received 300 mg of dupilumab every 2 weeks following an initial dose of 600 mg were included. Patients were eligible if they had a clinical presentation of BP combined with immunological or pathological evidence. Patients with drug-induced BP, with less than 4 weeks of follow-up, and who received dupilumab or any other biologics within 6 months were excluded.

Main Outcomes and Measures   The primary outcome was the proportion of patients who achieved disease control within 4 weeks. Disease control was defined as the absence of new lesions and pruritus, combined with the healing of existing lesions. Complete remission rates, relapse rates, changes in Bullous Pemphigoid Disease Area Index (BPDAI) scores, itching numerical rating scale (NRS) scores, laboratory results within 64 weeks, and adverse events (AEs) were also assessed.

Results   Among 146 patients (median [IQR] age, 73 [64-85] years; 86 [58.9%] male patients) included in the study, 127 (87.0%) patients achieved disease control within 4 weeks, with a median (IQR) time of 14 (7-14) days. A total of 52 (35.6%) patients achieved complete remission, and 13 (8.9%) patients relapsed during the observation period. The complete remission rate and cumulative relapse rate at week 64 were 62.5% (5 of 8) and 30.9%, respectively. There was rapid and sustained improvement in clinical indicators and laboratory examination results after dupilumab treatment, including BPDAI scores, itching NRS scores, serum anti-BP180 and anti-BP230 antibodies, total IgE levels, and eosinophil count. Of these 146 patients, 107 (73.3%) did not report any AEs. The most common AEs were infections and eosinophilia. Serum anti-BP180 antibody levels of greater than 50 relative units (RU)/mL (OR, 3.63; 95% CI, 0.97-12.61; P  = .045) were associated with 4-week disease control, and male patients were more likely to relapse (HR, 10.97; 95% CI, 1.42-84.92; P  = .02).

Conclusions and Relevance   In this retrospective cohort study, dupilumab treatment was associated with improved clinical symptoms in patients with BP. The safety profile was favorable, although concurrent infection and eosinophilia might pose potential concerns. This study suggests that patients with anti-BP180 antibody levels of at least 50 RU/mL and female sex may respond better.

Read More About

Zhao L , Wang Q , Liang G, et al. Evaluation of Dupilumab in Patients With Bullous Pemphigoid. JAMA Dermatol. 2023;159(9):953–960. doi:10.1001/jamadermatol.2023.2428

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General information for reviewers

Our valuable reviewers and editorial team ensure that our journals publish high-quality manuscripts in a timely manner. Through reviewer contributions our journals publish the best, cutting-edge studies, thereby advancing dermatology research and improving patient outcomes.

We hope that the guidance below will aid reviewers and encourage you to review our journals.

The journals of the British Association of Dermatologists (BAD) are a member of and follow the principles of the Committee on Publication Ethics (COPE) .

We encourage all reviewers, especially if you are new, to read COPE: Ethical guidelines for peer reviewers .

Peer review process

BAD journals use single anonymous peer review, i.e. the reviewers can view the authors’ names, but the authors do not know who the reviewers are.

Manuscripts are first assigned to the Editor-in-Chief (EiC) who performs an initial triage, and if the manuscript passes the first threshold, it is then assigned to one of our specialist Section Editors who will assess the manuscript and assign it to an Associate Editor within their Section to arrange peer review. The manuscript is then normally assigned to at least two specialist peer reviewers. The editorial team assesses the reviewers’ comments and makes a recommendation to the EiC who then makes the final decision regarding publication.

Reviewer timeframe

We understand that you are very busy, so please decline an invitation to carry out a review if you are unable to complete it in a timely manner. This ensures that we can invite the next set of people and the manuscript is not delayed waiting for a response.

If you are delayed in submitting your review, please email the Editorial Office who may be able to extend your deadline. Ultimately, keeping to these timelines helps the publishing ecosystem work on time.

Reviewers have 1 week to respond to an invitation to review a manuscript.

Once you have accepted the invitation to review you will receive an email with information on how to access the manuscript, including a direct link (no sign-in is required), and you will have 2 weeks to submit your review.

Please save a draft when working on your review and then submit it when you have completed the review. The system does not submit your review until you press the ‘submit’ button.

If you accept the first round of review, your name will automatically be suggested for the re-review. If the revisions were minor, the Associate Editor may opt to review it themselves and not send it out for re-review.

If you have reviewed a manuscript, you will receive an email to inform you of the decision once the EiC sends the final decision letter to the authors. The EiC will make one of the following decisions post-peer review decisions:

The flow chart below outlines the full peer review process

Should you accept the invitation to review?

Here is a quick checklist to use when deciding whether to peer review for one of our journals:

Can you submit comments within 2 weeks?

Check the author list as soon as you receive the invite, to ensure there are no potential conflicts of interest.

Do you have expertise in the topic and are you aware of the areas in which your expertise is lacking?

Conflicts of interest  

Reviewers are asked to disclose any conflicts of interest when accepting a review assignment.

We follow the ICMJE guidance on conflicts of interest and while we do not use their form, it is a good guide to use. It is better to declare a conflict even if it is a perceived one.

When to decline a review  

To provide authors with a fair and unbiased review process, reviewers are required to excuse themselves from the review of a manuscript when faced with a potential conflict of interest. Examples of situations that would require recusal on behalf of an editor or reviewer include but are not limited to:

an editor or reviewer is the spouse, domestic partner, parent, child, sibling or other family member of an author on the manuscript in review;

an editor or reviewer is involved in research collaboration with an author on the manuscript in review;

an editor or reviewer is under the employment of, or otherwise works at, the same institution as an author on the manuscript in review;

an editor or reviewer has a strong intellectual bias either for or against the position taken by the authors;

an editor or reviewer has a financial interest in an agent or device relevant to the study or has a financial relationship with a commercial sponsor of the study in question;

editors and reviewers are also required to excuse themselves from the review of a manuscript when requested to do so by the EiC.

It is our journals’ policy that if any of our editors are an author on a manuscript, they are unable to view the peer review process and are not involved in any decision-making.

If you have any questions about conflicts of interest, please contact the editorial office at [email protected] .

Review confidentiality  

Manuscripts under review are confidential documents that belong to the authors; therefore, all information during a review is strictly confidential. By agreeing to review, the reviewer agrees not to disclose any information related to the manuscript under review.

Should a reviewer wish to be assisted in their review by an appropriately qualified colleague, and we encourage co-reviewing with a trainee, please email the editorial office before any reviewing begins. Please mention this in your ‘Confidential Comments to the Editor’ and ensure to provide an appropriate acknowledgement if you had assistance.

Reviewer responsibilities

Reviewers should:

Be aware of the journals’ scope, audience and policies

Be knowledgeable about and qualified in the subject matter  to be reviewed. However, if you are a trainee or a new reviewer, do not be daunted, and review as you would if you were a reader of the topic. We welcome and encourage trainee dermatologists to become reviewers.

Be able to provide constructive, relevant and unbiased feedback that will help authors improve their manuscript.

Be able to return a review in a timely manner. If exceptional circumstances prevent a reviewer from returning a review within the requested timeframe, contact the editorial office as soon as possible.

Exercise tact and courtesy when making critiques.

Provide a structured list, where possible, by citing specific pages, paragraphs or lines in  comments so that the items in question can be easily found.

Be mindful of inclusive use of language, especially pertaining to race and ethnicity. If you are unsure or if you feel it could do with another expert in this matter, please alert the Editor in the confidential comments.

Be mindful of your biases, conscious or unconscious, when reviewing a manuscript.

Review a manuscript for its scientific content rather than its language. However, if you feel that the language makes it difficult to understand the manuscript, please alert the Editor or make a helpful suggestion that the authors could get some language assistance to improve their manuscript.

Reviewers should not :

Agree to review a manuscript if there is an actual or perceived conflict of interest (all potential conflicts should be disclosed to the Editorial Office before agreeing to review a manuscript or as soon as the potential conflict has been discovered). Conflicts that may hinder a fair and unbiased review include, but are not necessarily limited to, those of a financial, institutional, philosophical or personal nature.

Disclose any identifiable information about themselves in their review (the peer review process is anonymous).

Allude to either rejection or acceptance in their comments to the authors.

Attempt to contact authors to discuss a manuscript.

Reveal, cite or otherwise disclose information about a manuscript prior to publication.

Take on the task of copy editing (i.e. comment on minor errors regarding grammar, spelling or style) as our journals have technical editors who work with authors to improve the language if necessary.

Ask for new information during re-review – re-review is only meant to ensure that the initial comments have been addressed. If it is important, then it is best to explain and provide context and clarity to the authors. 

Engage the review assistance of another appropriately qualified colleague without first obtaining approval from the Associate Editor.

Use large language models such as ChatGPT for peer review; we value your judgement. Moreover, the use of any publicly available tools may compromise the confidentiality of the manuscript.

How to review

For the first-time reviewer, start by reading the manuscript and have a blank sheet of paper or electronic document to make notes as you go along.

Number your list as you read so you can refer to those sections.

Start with the main content and read it as you would read a manuscript.

Questions reviewers should ask themselves when reviewing:

Introduction

Is the study’s objective clearly stated?

Are there any seminal papers missing in the introduction?

Have the authors provided enough context for the background?

If information presented in the manuscript is new, is it properly introduced and described?

Does it appear that the most appropriate materials and methods were used?

Do the methods appear to be scientifically sound?

Is there sufficient information in the methods to ensure that the study can be replicated? 

Are the statistical tests appropriate to the study and well described?

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• General Dermatology
• Actinic Keratosis
• Precision Medicine and Biologics
• Rare Disease
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• Hidradenitis Suppurativa
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Journal Digest: June 4

This week’s collection of the latest dermatologic studies includes the use of digital communication for patients with hidradenitis suppurativa (HS), rituximab for pediatric patients with pemphigus, a case report of primary oral extramammary Paget disease, and the efficacy and safety of LAight therapy for HS.

Journal of the European Academy of Dermatology and Venereology : Usage of Digital Information and Communications Technologies in Patients With Hidradenitis Suppurativa

A recent letter to the editor by Pedersen et al reviewed the use of information and communication technologies (ICT) among adult patients with hidradenitis suppurativa (HS) at Bispebjerg Hospital. Data were collected via clinical examination and surveys from 48 patients, mostly female (79.2%) with an average age of 38.4 years. All patients had internet access, and the majority used one-to-one (dialogic) and one-to-many (informative) ICT platforms daily, with web browsers (91.7%) and Facebook/Messenger (81.3%) being the most popular. No significant associations were found between ICT usage and disease severity or symptom burden, except for a longer disease duration in many-to-many (social) ICT users. Patients preferred one-to-many ICTs for general and HS-specific information, with email being the favored method for receiving HS-related information. According to Pedersen et al, their review highlights the need to develop high-quality HS informational resources that align with patients' preferred ICT platforms, particularly web browsers. 1

International Journal of Dermatology : Diagnosis, Treatment, and Long-term Outcomes of Pediatric Pemphigus: A Retrospective Study at Tertiary Medical Centers

Renert-Yuval et al’s retrospective study evaluated 12 pediatric patients with pemphigus from 3 Israeli outpatient clinics between 2000 and 2023. The cohort, with a mean age of 10.7 years, included an equal male-to-female ratio. The diagnosis was delayed by an average of 11.1 months, often due to misdiagnoses. All patients initially received high-dose systemic corticosteroids (sCS), but only 25% achieved remission with sCS alone. Rituximab, used as a second- or third-line treatment in 41.7% of patients, led to remission in most cases. According to the study authors, their findings emphasize pediatric pemphigus' chronic nature and suggest rituximab as a potentially effective early treatment option. 2

Journal of Cutaneous Pathology : Unusual Case of Extramammary Paget Disease of the Oral Mucosa: A Case Report and Literature Review

Eilers et al reviewed a case of primary oral extramammary Paget disease (EMPD), a rare intraepidermal neoplasm, in an 81-year-old male patient misdiagnosed initially as lichen planus. Histopathology revealed Paget cells positive for CK7 and AR, suggesting an apocrine origin despite the oral mucosa's lack of apocrine glands. According to the study authors, the rarity of oral EMPD complicates diagnosis and treatment. If dermatology clinicians encounter EMPD, Eilers et al emphasized the importance of biopsy for persistent lesions, as primary oral EMPD generally has a good prognosis. 3

Journal of the German Society of Dermatology : Real-World Effectiveness and Safety of The Laight-Therapy In Patients With Hidradenitis Suppurativa

A recent study by Strobel et al investigated the efficacy and safety of LAight therapy for hidradenitis suppurativa (HS) using real-world data from 3437 patients. LAight therapy, a combination of radiofrequency and intense pulsed light, was administered biweekly alongside topical clindamycin, showing significant improvement in disease severity measured by the Hidradenitis Suppurativa Severity Score System (IHS4), pain, and quality of life (DLQI) over 26 weeks. Patients with Hurley stages I-III experienced substantial benefits, with over 50% achieving a 55% reduction in IHS4. Pain and DLQI scores decreased significantly, with higher BMI slightly moderating pain reduction. Most patients reported positive outcomes and minimal adverse effects, demonstrating LAight therapy as a potential well-tolerated treatment option for HS across all severity stages. 4

• Pedersen NH, Nielsen VW, Holgersen N, et al.Usage of digital information and communications technologies in patients with hidradenitis suppurativa. JEADV Clin Pract . Published online June 2, 2024. https://doi.org/10.1002/jvc2.474
• Renert-Yuval Y, Baum S, Greenberger S, et al. Diagnosis, treatment, and long-term outcomes of pediatric pemphigus: a retrospective study at tertiary medical centers.  Int J Dermatol . Published online May 31, 2024. doi:10.1111/ijd.17251
• Eilers G, Jordan RC. Unusual case of extramammary Paget disease of the oral mucosa: A case report and literature review.  J Cutan Pathol . Published online May 29, 2024. doi:10.1111/cup.14663
• Strobel A, Schultheis M, Staubach P, et al. Real-world effectiveness and safety of the LAight-therapy in patients with hidradenitis suppurativa.  J Dtsch Dermatol Ges . Published online May 28, 2024. doi:10.1111/ddg.15403

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Detecting peripheral neuropathy in patients with diabetes, prediabetes and other high-risk conditions: an advanced practice nurse’s perspective, an analysis of peripheral neuropathy symptom characteristics in hiv, overview of neurotrauma and sensory loss.

Government introduces ‘emergency ban’ on overseas prescriptions for puberty blockers

Johner Images / Alamy Stock Photo

The government has announced an “emergency ban” on prescriptions of puberty-suppressing hormones written by UK private prescribers, as well as prescribers registered in the European Economic Area (EEA) or Switzerland, for patients aged under 18 years in England, Wales and Scotland “to address risks to patient safety”.

In a statement, published on 29 May 2024 , the Department of Health and Social Care (DHSC) said the new arrangements apply to gonadotropin-releasing hormone analogues, which are medicines that consist of, or contain, buserelin, gonadorelin, goserelin, leuprorelin acetate, nafarelin or triptorelin.

The statement said that patients already established on these medicines by a UK prescriber for the purpose of suppressing puberty can continue to access them, adding that “they will also remain available for patients receiving the drugs for other uses, from a UK-registered prescriber”.

The DHSC said the ban will last from 3 June 2024 to 3 September 2024, and means that “no new patients [aged] under 18 [years] will be prescribed these medicines for the purposes of puberty suppression in those experiencing gender dysphoria or incongruence under the care of these prescribers”.

The statement advised that “patients seeking more information should speak to their clinician”.

Following the publication of the Cass Review into gender identity services in April 2024, the NHS stopped the routine prescription of puberty blocker treatments to patients aged under 18 years and introduced indefinite restrictions to the prescribing of these medicines within NHS primary care in England.

The Cass Review also recommended the government work with the General Pharmaceutical Council (GPhC) to consider statutory solutions to prevent inappropriate overseas prescribing of hormone supplies.

The review included feedback from focus groups that showed that some respondents had sought “private provision while on the waiting list for GIDS [gender identity development services]”, with “families trying to balance the risks of obtaining unregulated and potentially dangerous hormone supplies over the internet with the ongoing trauma of prolonged waits for assessment”.

The review said this was seen as “a forced choice (because the NHS provision is not accessible in a timely way) rather than a preference”, owing in part to the ongoing cost of treatment and the subsequent monitoring being “prohibitive for some”.

The Cass Review also said that “any clinician who ascertains that a young person is being given drugs from an unregulated source should make the young person and their family aware of the risks of such treatment”.

Following the publication of the review, the GPhC published a resource to support pharmacists and pharmacy technicians who are providing pharmacy services to children and young people relating to gender incongruence.

This resource sets out questions that pharmacy professionals should ask themselves when making decisions about the supply of medicines in this context and apply to the provision of services in person or online.

The GPhC website says: “The new resource emphasises that pharmacy teams providing pharmacy services to children and young people with gender incongruence or dysphoria need to adhere to the standard process of clinical assessment and care provision they have been trained to take as healthcare professionals.”

“The resource also emphasises that the starting point is that pharmacy professionals must provide compassionate, inclusive and person-centred care, within the current relevant legal and regulatory context,” it adds.

On 30 May 2024, the GPhC emailed pharmacists, pharmacy technicians and pharmacy owners in England , Scotland and Wales to make them aware of the new emergency legislation.

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• Case report
• Open access
• Published: 01 June 2024

Endovascular stenting using a sagittal sinus approach for sigmoid sinus wall dehiscence related to intractable pulsatile tinnitus: a case series

• Luis Alberto Ortega-Porcayo 1 , 2 , 3   na1 ,
• Guillermo Gonzalez-Garibay 1   na1 ,
• Ángel Lee 4 ,
• Juan A. Ponce-Gómez 2 , 3 , 4 ,
• Victor Alcocer-Barradas 2 , 5 ,
• Samuel Romano-Feinholz 3 &
• Marco Antonio Zenteno Castellanos 2 , 6 , 7  

Journal of Medical Case Reports volume  18 , Article number:  266 ( 2024 ) Cite this article

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Sigmoid sinus wall dehiscence can lead to pulsatile tinnitus with a significant decrease in quality of life, occasionally leading to psychiatric disorders. Several surgical and endovascular procedures have been described for resolving dehiscence. Within endovascular procedures, the sagittal sinus approach could be a technical alternative for tracking and accurate stent positioning within the sigmoid sinus when the jugular bulb anatomy is unfavorable.

Case presentation

A retrospective case series of three patients with pulsatile tinnitus due to sigmoid sinus wall dehiscence without intracranial hypertension was reviewed from January 2018 to January 2022. From the participants enrolled, the median age was 50.3 years (range 43–63), with 67% self-identifying as female and 33% as male. They self-identified as Hispanic. Sigmoid sinus dehiscence was diagnosed using angiotomography, and contralateral transverse sinus stenosis was observed in all patients. Patients underwent surgery via a navigated endovascular sagittal sinus approach for sigmoid sinus stenting. No neurological complications were associated with the procedure. Pulsatile tinnitus improved after the procedure in all patients.

Conclusions

Superior sagittal sinus resection for sigmoid sinus wall stenting is a safe and effective technique. Pulsatile tinnitus due to sigmoid sinus wall dehiscence could be treated using the endovascular resurfacing stenting technique. However, further research is needed to evaluate the potential benefit of contralateral stenting for removing sinus dehiscence when venous stenosis is detected. However, resurfacing sigmoid sinus wall dehiscence results in symptomatic improvement.

Peer Review reports

Pulsatile tinnitus (PT) is a distressing symptom characterized by the perception of a rhythmic noise that resembles a heartbeat, despite the absence of any external acoustic stimulus. It can potentially lead to psychiatric disorders and affects approximately 4–10% of the general population [ 1 , 2 , 3 ]. Studies have shown that 4–20% of PT cases involve sigmoid sinus (SS) wall anomalies and venous system ipsilateral dominance [ 4 , 5 , 6 ]. These anomalies can range from a very thin and intact SS bone wall to a diverticulum with a focal protrusion into the mastoid air cells, ectasia with smooth bulging of the sinus into the mastoid air cells, or even dehiscence of the SS wall [ 7 ].

Sigmoid sinus wall dehiscence (SSWD) is an anomaly that occurs when the bone surrounding the sinus is incomplete, leading to contact between the mastoid cells and the SS. This can cause disruption of laminar flow, creating audible turbulence, increasing conduction of normal blood flow, and leading to PT [ 4 , 8 ].

Treatment for PT associated with venous etiologies not associated with idiopathic intracranial hypertension may be based on resurfacing and reconstruction of SS wall anomalies. The goal of this treatment is to eliminate the sound caused by vascular turbulence and decrease the transmission of vibrations through mastoid air cells. Endovascular management of PT was first described in 2000 by Houdart with coiling and in 2004 by Zenteno with stenting [ 9 , 10 ]. In the present study, we report a series of patients with PT related to ipsilateral SSWD treated using stent resurfacing endovascular technique through a neuro-navigated superior sagittal sinus (SSS) approach.

Methodology

We conducted a retrospective case series review from January 2018 to January 2022. All patients provided informed consent according to ethical standards. The present study evaluated patient characteristics, computed tomography angiography (CTA) and digital subtraction angiography (DSA), and clinical outcomes as evaluated by a neurosurgeon and a neuroendovascular therapist. The study included three patients who had symptomatic incoercible PT related to SSWD not associated with idiopathic intracranial hypertension and were referred by otolaryngologists.

Venous sinus stenting through a neuronavigated sagittal sinus approach

First, a neuronavigated (Fiagon®, Hennigsdorf, Germany) burr hole was made at the anterior-mid portion of the SSS behind the hairline (Fig.  1 ). Access was obtained via the SSS, and a 6 F Chaperon (MicroVention, Aliso Viejo, CA) guiding catheter was positioned in the distal sigmoid sinus at the jugular bulb through a Terumo 6 F (Terumo Interventional®, Tolyo, Japan) radial introducer sheath. Then, a 7 × 50 self-expandable carotid wallstent (Boston Scientific, Marlborough, MA) was advanced over the wire and deployed in the SS, resurfacing the wall dehiscence areas. Tirofiban was administered immediately before stent deployment without previous antiplatelet medication. The entire system was removed, and SSS bleeding was controlled using absorbable hemostatic gelatin (Gelfoam®, Pfizer, NY, USA) and gentle pressure for 5 minutes. Finally, the burr hole was closed using a preshaped round plate (KLS Martin, Tuttlingen, Germany), and the fascia and skin were closed using Vycril-0 and staples, respectively (Fig.  1 ).

Endovascular neurosurgical approach to the sigmoid sinus involving the use of a burr hole in the anterior skull to access the superior sagittal sinus. Wall stents were advanced over the wire and deployed in the sigmoid sinus to restore wall integrity

A 63-year-old Hispanic woman complained of persistent, incoercible, left-sided, pulsatile, loud, low-pitch tinnitus in her left ear for 3 months, which was exacerbated at night and disrupted her normal life. She described difficulty concentrating on certain tasks in quiet environments. She had a clinical history of major depression and chronic lumbar pain. A neurological examination, including fundoscopy, was unremarkable. The PT diminished under ipsilateral neck internal jugular vein (IJV) compression, and her audiogram was normal bilaterally. CTA showed left SSWD (Fig.  2 ) and contralateral transverse sinus stenosis. An incidental posterior communicating aneurysm was observed. We performed SS resurfacing venous stenting and treated the small aneurysm using the sole stenting technique [ 11 ]. The patient’s tinnitus diminished significantly after the surgery and was only noticed at night. One year after surgery, the patient reported that her tinnitus was seldomly perceived at night, and she described a significant improvement in her quality of life. Four years after surgery, the patient was asymptomatic without PT.

Case 1 imaging studies. A Preoperative CTA. B Neuronavigation of the SS. C – E Transoperative imaging studies of the wire advancing through the cerebral venous system to the left SS. F , G Stent deployment in the left SS lumen. H Postoperative CTA image showing the stent deployed in the left SS lumen

A 43-year-old Hispanic woman presented with a 2-year history of intermittent right-sided pulsatile, loud, low-pitched tinnitus. The tinnitus coincided with her heartbeat, worsened in quiet environments, and increased during the night, disrupting her normal sleep. Neurological examination revealed mild instability and dizziness. Fundoscopy was normal, there were no focal deficits, and ipsilateral IJV compression decreased the intensity of the PT. Her bilateral audiogram was normal, and her CTA showed right SSWD (Fig.  3 ) and contralateral transverse sinus stenosis. SS resurfacing and venous stenting were performed, and the patient experienced immediate improvement after surgery. A total of 1-year post-surgery, the patient occasionally reported perceiving pulsatile, soft, low-pitched tinnitus at night but reported a significant improvement in her quality of life and sleep.

Preoperative and postoperative CTA images of patients 1, 2, and 3. A Patient 1 preoperative left SS dehiscence. B Patient 2 preoperative right SS dehiscence. C Patient 3: preoperative right SS dehiscence. D Patient 1 postoperative stent was deployed in the left SS. E Patient 2 postoperative stent was deployed in the right SS. F Patient 3 with a postoperative stent deployed in the right SS

A 45-year-old Hispanic man presented with a history of right hypoacusis, dizziness, clogged ears, and continuous intractable right pulsatile, loud, high-pitched tinnitus. The patient had previously undergone surgical reconstruction of the transmastoid SS wall. Neurological examination and fundoscopy were normal. Cranial eighth nerve examination revealed right sensorineural hypoacusis, with left Weber and bilateral positive Rinne tests. Changes in PT density were observed under ipsilateral IJV compression. An audiogram demonstrated severe right sensorineural hypoacusis and left sensorineural hypoacusis limited to high frequencies. CTA showed right SSWD (Fig.  3 ) and contralateral transverse sinus stenosis. SS resurfacing and venous stenting were performed, and the patient experienced mild improvement after surgery. However, he had a skin surgical site infection eight days post-surgery, which was resolved by a 1-week treatment with local neomycin ointment and amoxicillin/clavulanic acid, with no further complications. A total of 3 months after surgery, the patient reported significant improvement in tinnitus but continued to experience ear fullness. A total of 1 year after surgery, the patient reported a recurrence of intermittent loud, high-pitched, no pulsatile tinnitus not associated with quiet environment and persistent plugged ears.

SSWD is considered among the most frequent causes of venous anomalies. Dong et al. reported that SSWD accounts for 86.4% of the total vascular anomalies and variants associated with unilateral venous PT [ 11 ].

SS wall reconstruction for pulse synchronous tinnitus is a widely accepted and successful surgical solution [ 12 , 13 ]. This can be achieved by reducing contact between the mastoid cells and the SS in several ways: (1) inserting bone chips between the bone wall structure and the SS to avoid direct contact with the mastoid cells. (2) Reinforcing the bone wall to reduce the amount of sound transmission caused by disruption of laminar flow in the SS. (3) Simple mastoidectomy to reduce sound conduction by mastoid cells. Endovascular interventions have been used to prevent the transmission of audible turbulence to mastoid air cells caused by the loss of blood laminar flow, primarily in patients presenting with aneurysms or diverticula, as our group has previously described [ 10 ]. However, we report three patients with SSWD without aneurysms or diverticula treated with an SS resurfacing stenting technique.

The pathophysiology of the associations of SS wall anatomical anomalies and their relation to the origin of PT are not well understood. Theories include vein blood flow dynamics, intracranial hypertension, and associated osteoporosis. Disruption of laminar vein blood flow may cause audible local turbulence, or alterations in the morphological inner ear anatomy may predispose individuals to increased bone conduction of normal blood flow [ 14 ].

Contralateral and ipsilateral stenosis of the transverse sinus has been postulated as a possible underlying mechanism of tinnitus secondary to SSWD. An increased volume of blood directed to the dominant sinus with contralateral stenosis of the transverse sinus, or an increased velocity of blood flow driven to the ipsilateral stenosed sinus has been described as a cause of erosion of the bony wall adjacent to the sigmoid sinus, generating dehiscence and leading to tinnitus [ 14 , 15 ].

PT associated with SS wall anomalies has traditionally been treated using surgical techniques, in which the main goal is to reconstruct the sigmoid sinus surface to its normal smooth contour. This approach aims to eliminate vein flow turbulence and decrease the transmission of vibrations through mastoid air cells into the cochlea [ 16 ]. For novel endovascular strategies, coils and stents are used to decrease blood flow turbulence under the same resurfacing principle, reinforcing the vein wall.

Resurfacing techniques for the SS wall appear to be effective and well tolerated for most patients with bothersome PT caused by venous vascular abnormalities. Liu et al. reported a 75% total resolution of PT in patients treated with surgical resurfacing techniques focused on an SS source [ 3 ], with a 4% long-term morbidity and mortality rate. Endovascular procedures targeting pulsatile tinnitus associated with SS anomalies have been increasingly used in recent years. In 2017, Wang et al. showed that only 7.9% of patients (ten patients) were treated endovascularly [ 4 ]. However, excellent outcomes were reported, with a 91% pulsatile tinnitus resolution rate and only one case of small cerebellar ischemia, which showed neurological recovery 2 months after the procedure. In our small series, no long-term neurological morbidity or mortality was documented. Further endovascular data analysis will provide a better understanding of pulsatile tinnitus pathophysiology and allow for proper comparisons of the risks and outcomes between endovascular and surgical resurfacing techniques.

Cannulation of SS through a durotomy in the SSS seems to be effective and safe when the jugular bulb is not favorable for stent navigation to the SS. Stenting in patients with SSWD not associated with diverticulum is an effective alternative treatment option for otherwise untreatable pulsatile tinnitus. Based on the data for venous stenosis stenting in the treatment of idiopathic intracranial hypertension, contralateral transverse sigmoid sinus stenting could be evaluated in the future to indirectly decrease the ipsilateral flow to the SSWD associated with PT.

Availability of data and materials

Not applicable.

Abbreviations

Sigmoid sinus

Sigmoid sinus wall dehiscence

• Pulsatile tinnitus

Superior sagittal sinus

Sigmoid sinus wall

Internal jugular vein

Computed tomography angiography

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Acknowledgements

The paper was self-founded by the authors.

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Luis Alberto Ortega-Porcayo and Guillermo Gonzalez-Garibay share the first authorship

Authors and Affiliations

Medical School, Universidad Anáhuac, Mexico City, Mexico

Luis Alberto Ortega-Porcayo & Guillermo Gonzalez-Garibay

Neurosurgery Department, Hospital Ángeles del Pedregal, Mexico City, Mexico

Luis Alberto Ortega-Porcayo, Juan A. Ponce-Gómez, Victor Alcocer-Barradas & Marco Antonio Zenteno Castellanos

Neurosurgery Department, Centro Médico ABC, Mexico City, Mexico

Luis Alberto Ortega-Porcayo, Juan A. Ponce-Gómez & Samuel Romano-Feinholz

Research Department, Hospital General Manuel Gea González, Mexico City, Mexico

Ángel Lee & Juan A. Ponce-Gómez

Neurosurgery Department, Instituto Nacional de Neurología y Neurocirugía, Mexico City, Mexico

Victor Alcocer-Barradas

Neuroendovascular Therapy Department, Instituto Nacional de Neurología y Neurocirugía, Mexico City, Mexico

Marco Antonio Zenteno Castellanos

Neuroendovascular Therapy, Hospital Ángeles del Pedregal, Mexico City, Mexico

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Each author has made substantial contributions to the conception or design of the work, participated in drafting the work or revised it critically for important intellectual content.

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Correspondence to Marco Antonio Zenteno Castellanos .

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Ortega-Porcayo, L.A., Gonzalez-Garibay, G., Lee, Á. et al. Endovascular stenting using a sagittal sinus approach for sigmoid sinus wall dehiscence related to intractable pulsatile tinnitus: a case series. J Med Case Reports 18 , 266 (2024). https://doi.org/10.1186/s13256-024-04591-3

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Received : 15 April 2024

Accepted : 02 May 2024

Published : 01 June 2024

DOI : https://doi.org/10.1186/s13256-024-04591-3

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• Resurfacing endovascular technique
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