Nasal Septal Abscess Complicating Acute Sinusitis in an 8-Year-Old Child: A Case Report

Introduction: Nasal septal abscess (NSA) in the pediatric population is an uncommon condition, but it can cause devastating complications (such as potentially life-threatening intracranial infections and cosmetic nasal deformity). The objective of this study is to report a case of an pediatric patient presenting with NSA in association with frontal-ethmoid acute sinusitis and intracranial abscess. Method and Results: In this study, we report a case of an 8-year-old patient who progressed from spontaneous nasal septal abscess to sinusitis (maxillary, frontal and ethmoidal), cosmetic nasal deformity (destruction of septal cartilage) and intracranial complication. The purulent collection necessitated urgent surgical drainage and adequate medical treatment. Conclusion: Nasal septal abscess in children is a rare condition that necessitates early appropriate drainage in order to prevent severe life-threatening complications. Furthermore, in the growing child, in case of total destruction of the cartilaginous septum, immediate reconstruction with autologous cartilage graft is essential for normal development of the nose and maxilla.

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Intraorbital Abscess: A Rare Cause of Orbit Compression

FACE ◽

10.1177/27325016211005062 ◽

2021 ◽

pp. 273250162110050

Author(s):

Samuel Ruiz ◽

Rizal Lim

Keyword(s):

Pediatric Population ◽

Rare Complication ◽

Disease Process ◽

Rare Condition ◽

External Drainage ◽

Direct Extension ◽

Life Threatening ◽

History Of ◽

High Index Of Suspicion ◽

Prompt Diagnosis

Introduction: Intraorbital abscess is a rare complication of rhinosinusitis that affects most commonly the pediatric population. It is thought to be caused by direct extension or venous spread of infections from contiguous sites and can lead to life-threatening complications, like permanent visual loss and cerebral abscesses. Objectives: Intraorbital abscess is a rare condition that requires prompt diagnosis and treatment to avoid serious complications. Our objectives are to provide an overview of this rare disease process and its management including our successful treatment experience. Case Description: We present a 2 case report of a 13-year-old pediatric male and a 66-year-old male with history of chronic sinusitis who presented with a right intraorbital abscess successfully treated with external drainage with decompression of the orbit. Conclusion: When intraorbital abscess is encountered, a high index of suspicion is needed to allow prompt and accurate diagnosis for this infrequent condition. Timely surgical drainage of the abscess is needed to prevent the development of fatal complications.

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Right Heart Thrombus and Pulmonary Embolism in Patients with Life-Threatening Bleeding: Management of a Complex Condition

SN Comprehensive Clinical Medicine ◽

10.1007/s42399-021-00776-9 ◽

2021 ◽

Author(s):

Enrico Bentivegna ◽

Michelangelo Luciani ◽

Valerio Spuntarelli ◽

Giorgio Sesti ◽

Flavia Del Porto ◽

...

Keyword(s):

Therapeutic Strategy ◽

Bleeding Risk ◽

Right Heart ◽

Bleeding Management ◽

Critical Situation ◽

Haemodynamic Parameters ◽

Complex Patients ◽

Life Threatening ◽

Uncommon Condition ◽

In Transit

AbstractRight heart thrombus (RHT) in transit is an uncommon condition associated with high mortality. Increased use of echocardiography has allowed an easier detection of RHT; however, there is no consensus about the most appropriate management of this critical situation. Therapeutic strategy should be decided according to patient’s haemodynamic parameters, clinical data, and bleeding risk. This paper, referring to the most current evidences, underlines the difficulty to establish the best therapeutic strategies in RHT among complex patients as there are no relevant guidelines. In some conditions, multidisciplinary management is the best way to find the most correct therapy despite the bad prognosis.

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The Global Registry for Hereditary Angioedema due to C1-Inhibitor Deficiency

Clinical Reviews in Allergy & Immunology ◽

10.1007/s12016-021-08855-4 ◽

2021 ◽

Author(s):

Andrea Zanichelli ◽

Henriette Farkas ◽

Laurance Bouillet ◽

Noemi Bara ◽

Anastasios E. Germenis ◽

...

Keyword(s):

Hereditary Angioedema ◽

Rare Condition ◽

C1 Inhibitor ◽

C1 Inhibitor Deficiency ◽

Therapeutic Decisions ◽

Life Threatening ◽

Enhance Patient Care ◽

Electronic Support ◽

The Impact ◽

Global Registry

AbstractHereditary angioedema (HAE) is a rare condition, mostly due to genetic deficiency of complement C1 inhibitor (C1-INH). The rarity of HAE impedes extensive data collection and assessment of the impact of certain factors known to affect the course of this disabling and life-threatening disease. Establishing a global registry could assist to overcome such issues and provides valuable patient data from different countries. The HAE Global Registry is a disease-specific registry, with web-based electronic support, where data are provided by physicians and patients through a dedicated application. We collected data between January 1, 2018, and August 31, 2020. Data on 1297 patients from 29 centers in 5 European countries were collected. At least one attack was recorded for 497 patients during the study period. Overall, 1182 patients were diagnosed with HAE type 1 and 115 with type 2. At the time of database lock, 389 patients were taking long-term prophylactic medication, 217 of which were on danazol. Most recorded attacks affected the abdomen, were generally moderate in severity, and occurred in patients who were not on prophylactic treatment (70.6%, 6244/8848). The median duration of attacks was 780 min (IQR 290–1740) in patients on prophylactic medication and 780 min (IQR 300–1920) in patients not on continuous prophylactic medication. In conclusion, the establishment of a registry for C1-INH-HAE allowed collection of a large amount of data that may help to better understand the clinical characteristics of this disease. This information may enhance patient care and guide future therapeutic decisions.

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Bilateral Congenital Humeroradial Synostostis Presenting with Bilateral Proximal Radius Fractures: A Case Report

Case Reports in Orthopedic Research ◽

10.1159/000512183 ◽

2021 ◽

pp. 29-32

Author(s):

Elsiddig E. Mahmoud

Keyword(s):

Case Report ◽

Elbow Joint ◽

Parental Education ◽

Functional Disability ◽

Congenital Abnormalities ◽

Rare Condition ◽

Radius Fractures ◽

Proximal Radius ◽

History Of ◽

Uncommon Condition

Congenital bilateral humeroradial synostosis (HRS) is a rare condition. It is generally divided into 2 categories. In the first group, which is mainly sporadic, additional upper limb hypoplasia typically coexists. In the second group, which is classically familial, HRS is commonly an isolated upper extremity anomaly. HRS can lead to variable degrees of functional disability. The clinical case reported here illustrates a possibly avoidable presentation of this uncommon condition. In this case report, we present a 6-week-old male who presented with bilateral radius fractures. Radiography revealed congenital HRS at both elbows. No other associated congenital abnormalities were detected, and there was no family history of similar conditions in any first-degree relatives. In cases of congenital HRS, movement at the elbow joint is not possible. Parents who are unaware of this information might try to straighten their infant’s elbows, which in turn may result in fractures of the proximal radius. Hence, early diagnosis and proper parental education could prevent fractures as a sequela of HRS.

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Successful Management of Acquired Hemophilia A Associated with Bullous Pemphigoid: A Case Report and Review of the Literature

Case Reports in Hematology ◽

10.1155/2017/2057019 ◽

2017 ◽

Vol 2017 ◽

pp. 1-7 ◽

Cited By ~ 4

Author(s):

Quentin Binet ◽

Catherine Lambert ◽

Laurine Sacré ◽

Stéphane Eeckhoudt ◽

Cedric Hermans

Keyword(s):

Bullous Pemphigoid ◽

Neutralizing Antibodies ◽

Hemophilia A ◽

Rare Condition ◽

Term Treatment ◽

Acquired Hemophilia A ◽

Acquired Hemophilia ◽

Short Term Treatment ◽

Life Threatening ◽

Dose Levels

Background. Acquired hemophilia A (AHA) is a rare condition, due to the spontaneous formation of neutralizing antibodies against endogenous factor VIII. About half the cases are associated with pregnancy, postpartum, autoimmune diseases, malignancies, or adverse drug reactions. Symptoms include severe and unexpected bleeding that may prove life-threatening.Case Study. We report a case of AHA associated with bullous pemphigoid (BP), a chronic, autoimmune, subepidermal, blistering skin disease. To our knowledge, this is the 25th documented case of such an association. Following treatment for less than 3 months consisting of methylprednisolone at decreasing dose levels along with four courses of rituximab (monoclonal antibody directed against the CD20 protein), AHA was completely cured and BP well-controlled.Conclusions. This report illustrates a rare association of AHA and BP, supporting the possibility of eradicating the inhibitor with a well-conducted short-term treatment.

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A Case Report of Pediatric Geniculate Neuralgia Treated with Sectioning of the Nervus Intermedius and Microvascular Decompression of Cranial Nerves IX and X

Pediatric Neurosurgery ◽

10.1159/000509760 ◽

2020 ◽

Vol 55 (6) ◽

pp. 439-443

Author(s):

Georgios Zenonos ◽

Michael M. McDowell ◽

Hussam Abou-Al-Shaar ◽

Kenan Alkhalili ◽

Paul A. Gardner

Keyword(s):

Microvascular Decompression ◽

Pediatric Population ◽

English Literature ◽

Posterior Inferior Cerebellar Artery ◽

Cranial Nerves ◽

Rare Condition ◽

Extensive Evaluation ◽

Patient Reported ◽

Nervus Intermedius

Background: Classic geniculate neuralgia (GN) is a rare condition characterized by lancinating pain centered in the ear and not involving the throat. To the best of our knowledge, no case of pediatric GN has been reported in the English literature. Case Presentation: We present the first reported case of successfully treated GN in a child via an endoscopic approach. The patient was a 9-year-old boy who presented with a 1-year history of lancinating right ear pain. Neuroleptics resulted in a short-lived improvement in symptoms, but with significant side effects. Extensive evaluation by multiple specialties did not reveal a cause for his pain. Imaging disclosed a tortuous loop of the right posterior inferior cerebellar artery abutting cranial nerves IX and X but no other abnormalities. The patient underwent an endoscopic microvascular decompression of cranial nerves IX and X, and sectioning of the nervus intermedius through a right retromastoid craniotomy. Postoperatively, the patient reported complete resolution of his symptoms that persisted at 3 months of follow-up. At the 5-year follow-up, the patient maintained pain relief and was developing normally. Conclusion: GN can affect the pediatric population. In carefully selected patients with consistent clinical and radiographic presentation, sectioning of the nervus intermedius and microvascular decompression of the lower cranial nerves can be an effective treatment.

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Fatal case of toxic epidermal necrolysis induced by ciproflfl oxacin in a child

Our Dermatology Online ◽

10.7241/ourd.2021s1.6 ◽

2021 ◽

Vol 12 (Supp 1) ◽

pp. 26-29

Author(s):

Thomas Schiestel

Keyword(s):

Case Report ◽

Toxic Epidermal Necrolysis ◽

Adverse Reactions ◽

Pediatric Population ◽

Fatal Case ◽

Stevens Johnson Syndrome ◽

Delayed Treatment ◽

Drug Eruptions ◽

Johnson Syndrome ◽

Life Threatening

Bullous drug eruptions such as Toxic Epidermal Necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are rare but known adverse reactions of fluoroquinolones. Although uncommon, TEN can be life-threatening for the patient, especially in the context of delayed treatment and in fragile patients such as the pediatric population. In the present case, TEN occurred in a 13-year-old girl with no medical history following initiation of ciprofloxacin treatment for an inguinal cyst. We hope that the case report will make interrogate the practices concerning the use of antibiotics, in particular fluoroquinolones in the context of an use not prescribed by the Marketing Authorization of the drug in children.

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Familial Pityriasis Rubra Pilaris: Case Report and Review

Journal of Cutaneous Medicine and Surgery ◽

10.2310/7750.2012.12018 ◽

2013 ◽

Vol 17 (4) ◽

pp. 226-232 ◽

Cited By ~ 7

Author(s):

Joshua M. Mercer ◽

Chitra Pushpanthan ◽

Canagasundrum Anandakrishnan ◽

Ian D.R. Landells

Keyword(s):

Autosomal Dominant ◽

Pediatric Population ◽

Severe Disease ◽

Topical Therapy ◽

Rare Condition ◽

Unknown Etiology ◽

Pityriasis Rubra Pilaris ◽

Gradual Onset ◽

Systemic Treatments ◽

Appropriate Therapy

Background: Pityriasis rubra pilaris (PRP) is a rare dermatosis of unknown etiology. Most cases of PRP are sporadic; however, rare cases of familial PRP have been reported. Objectives: To present a case of PRP inherited in an autosomal dominant (AD) fashion and to evaluate the current literature on familial PRP and formulate a comprehensive, up-to-date summary of this rare condition. Methods: PubMed was used to conduct a search for articles pertaining to familial PRP published through May 2011. Results: The first documented case was published in 1910, and 36 subsequent familial cases of PRP have been reported. Familial PRP typically presents very early in childhood, has a gradual onset, and persists throughout life. Given the rarity of this subtype, determining the best therapy has been a challenge. In the pediatric population, a conservative treatment approach, including topical therapy, is frequently used, whereas systemic treatments are reserved for patients with a severe disease that is refractory to therapy. Conclusion: Rare cases of PRP inherited in an AD fashion have been described and tend to have a chronic clinical course and are treatment refractory. Therefore, the awareness of familial PRP is important for early and accurate diagnosis and administration of appropriate therapy.

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Noninvasive Blood Pressure Monitoring and Electrocardiography

The Pediatric Procedural Sedation Handbook ◽

10.1093/med/9780190659110.003.0014 ◽

2018 ◽

pp. 92-97

Author(s):

Melissa Langhan ◽

Seth Wolf

Keyword(s):

Blood Pressure ◽

Blood Pressure Monitoring ◽

Pediatric Population ◽

Noninvasive Blood Pressure ◽

Procedural Sedation And Analgesia ◽

Medication Side Effects ◽

Medication Side ◽

Life Threatening ◽

Sedation And Analgesia

There is a lack of data about the benefits of cardiovascular monitoring with continuous 3-lead electrocardiography (ECG) and intermittent noninvasive cuff blood pressure (BP) during procedural sedation and analgesia (PSA) in the pediatric population. However, these two safe modalities are important for patients during higher levels of PSA because of the risk of rare life-threatening conditions and to help identify possible medication side effects and drug interactions of common sedative medications. These monitoring modalities can also aid in determining the adequacy of sedation. It is generally accepted that a baseline determination of heart rate and BP should be obtained prior to any sedative administration. With deeper levels of sedation and throughout recovery, continuous 3-lead ECG and intermittent BP monitoring are recommended, in addition to other modalities such as pulse oximetry and capnography, to monitor the safety of the patient.

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Surgical treatment for secondary aortoesophageal fistula

Journal of Cardiothoracic Surgery ◽

10.1186/s13019-020-01293-x ◽

2020 ◽

Vol 15 (1) ◽

Author(s):

Kayo Sugiyama ◽

Toru Iwahashi ◽

Nobusato Koizumi ◽

Toshiya Nishibe ◽

Toshiki Fujiyoshi ◽

...

Keyword(s):

Open Repair ◽

Rare Condition ◽

Aortic Aneurysms ◽

Total Arch Replacement ◽

University Hospital ◽

Aortoesophageal Fistula ◽

Thoracic Aortic Aneurysms ◽

Life Threatening ◽

Tokyo Medical ◽

Medical University

Abstract Background Aortoesophageal fistula (AEF) is a relatively rare condition that is often life-threatening. Secondary AEF is a complication of previous surgery, which can be more critical and challenging than primary AEF. The number of secondary AEF is increasing due to increase in the number of thoracic endovascular aortic repair (TEVAR). Although TEVAR has become a successful alternative surgical strategy for thoracic aortic aneurysms, secondary AEF after TEVAR might be critical than other secondary AEF because of severe adhesion between the esophagus and residual thoracic aortic wall. Methods This study analyzed six patients with secondary AEF who were treated at Tokyo Medical University Hospital between 2011 and 2016. These participants included four patients who had undergone TEVAR and two who had undergone total arch replacement. Results Although they were subsequently hospitalized for a long period, open surgical repair was completed in two patients who had undergone total arch replacement. TEVAR alone was performed in two patients who had undergone TEVAR and they were discharged without major complications shortly. Combined repair of TEVAR as a bridge to open surgery was planned for two patients who had undergone TEVAR. However, reconstruction of the aorta and esophagus could not be completed in these patients due to severe adhesions, and they died during hospitalization. Conclusions Definitive open repair was successfully performed in patients with secondary AEF after total arch replacement. However, in the patients with secondary AEF after TEVAR, severe adhesion between the aorta and esophagus led to difficulty in performing a successful definitive open repair. The strategy for secondary AEF should, therefore, be decided considering the etiology of secondary AEF. In secondary AEF after TEVAR, definitive open repair is difficult to complete because of catastrophic complication, and palliative treatment using TEVAR without reconstruction of aorta and esophagus can be an alternative.

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