-
Archives of Craniofacial Surgery Mar 2024A 76-year-old woman, initially thought to have a simple abscess on her right upper eyelid, presented to our department of plastic and reconstructive surgery. Enhanced...
A 76-year-old woman, initially thought to have a simple abscess on her right upper eyelid, presented to our department of plastic and reconstructive surgery. Enhanced three-dimensional facial computed tomography (CT) revealed an abscess on the right upper lid, with a pyomucocele present in the right frontal sinus, accompanied by bone erosion in the superior wall of the right orbit. Based on the results of the CT scan, we diagnosed an atypical Pott's puffy tumor (PPT) with an abscess on the upper lid originating from the frontal sinusitis. First, surgical incision and drainage were performed in our department, and a percutaneous vacuum drain was placed. To provide a more definitive treatment, endoscopic sinus surgery (ESS) was subsequently performed by otorhinolaryngologists. The patient was discharged without any complications 5 days after ESS. At a 1-year follow-up, no recurrence or notable neurological symptoms were observed. In the case we observed, the patient presented with an upper eyelid abscess and cellulitis, indicating possible orbital involvement. For such patients, a CT scan is necessary. Given the possibility of PPT, it is critical to perform a comprehensive differential diagnosis rather than defaulting to a straightforward approach involving abscess treatment.
PubMed: 38447589
DOI: 10.7181/acfs.2023.00465 -
Case Reports in Ophthalmological... 2024We present a rare case of a 39-year-old female with extramedullary relapse of acute myeloid leukaemia (AML) isolated to the left eye 2 months post allogeneic...
We present a rare case of a 39-year-old female with extramedullary relapse of acute myeloid leukaemia (AML) isolated to the left eye 2 months post allogeneic haematopoietic stem cell transplant. She initially presented with painless left eye erythema, swelling, and visual impairment. Initial ophthalmology review revealed conjunctival chemosis, raised intraocular pressure, and serous retinal detachments. She was initially treated for suspected orbital cellulitis with intravenous antibiotic and antifungal therapy but clinically progressed so was then treated with intravenous corticosteroids. One week later, she progressed to angle-closure glaucoma with development of a hypopyon and an enlarging subconjunctival mass. She proceeded to urgent subconjunctival biopsy and drainage of subretinal fluid which confirmed extramedullary relapse of AML. Notably, further investigation found no evidence of bone marrow or central nervous system relapse. She proceeded to localized radiotherapy with gradual resolution of the subconjunctival mass and serous retinal detachment and was for consideration of donor lymphocyte infusions and azacitidine therapy; unfortunately, she developed respiratory sepsis and passed away despite maximal efforts. This case represents a rare and unusual presentation of isolated ocular extramedullary relapse of AML and emphasises the importance of early ophthalmology involvement and tissue biopsy when there is high clinical suspicion of the disease.
PubMed: 38419918
DOI: 10.1155/2024/2235819 -
Clinical Case Reports Feb 2024This unique case report of primary Sjogren's syndrome (pSS) shows bilateral ptosis and significant periorbital edema, compromising vision. To avoid misleading diagnosis,...
KEY CLINICAL MESSAGE
This unique case report of primary Sjogren's syndrome (pSS) shows bilateral ptosis and significant periorbital edema, compromising vision. To avoid misleading diagnosis, antibody tests must be evaluated and interpreted in the context of clinical findings.
ABSTRACT
Primary Sjögren's syndrome is an idiopathic, autoimmune disorder involving the lacrimal and salivary glands characterized by both localized and systemic manifestations including xerostomia and keratoconjunctivitis sicca. Myasthenia Gravis (MG) is also an autoimmune disorder characterized by the development of auto-antibodies against nicotinic acetylcholine receptors that causes decreased muscle response to stimulation. It usually presents with ptosis and generalized body weakness. Ophthalmological involvement is common in both disorders but ptosis is very rarely seen in pSS. We report the case of a 27-year-old woman presenting to our clinic with the complaint of ptosis and eyelid swelling. She also had a positive anti-acetylcholine receptor antibody test and her initial presentation mimicked Myasthenia Gravis. Her autoimmune workup revealed a positive titer of Anti Ro SSA antibodies. Myasthenia Gravis was ruled out on electrodiagnostic studies which showed no decremental response, and pSS was confirmed on lip biopsy. Our case highlights that it is important to interpret the antibody test results in the context of clinical findings as we can have spurious results in autoimmune diseases. Autoimmune conditions can have varying presenting complaints hence, clinical judgment should always overrule diagnostic investigations and should thus guide patient management.
PubMed: 38405359
DOI: 10.1002/ccr3.8554 -
Indian Journal of Pathology &... Jul 2023We report an unusual presentation of an orbital spindle cell hemangioma in a 40-year-old male, who noted sudden redness and swelling of the left eye on waking up. On...
We report an unusual presentation of an orbital spindle cell hemangioma in a 40-year-old male, who noted sudden redness and swelling of the left eye on waking up. On examination, the patient was found to have edema of upper eyelid edema, periorbital ecchymosis, and subconjunctival hemorrhage in the left eye at presentation. On treatment with topical medications, patient had transient symptomatic relief; however, he later developed blurring of vision. When seen 10 days later, the patient's left eye showed axial proptosis. Radiological investigations revealed an intraconal soft tissue mass in the left medial rectus. Emergency orbital decompression with mass excision was done; histopathological examination of the excised mass revealed spindle cell hemangioma. Postsurgery patient had complete restoration of vision. To our knowledge, an acute presentation of an orbital hemangioma with subconjunctival hemorrhage and periorbital ecchymosis, visual loss doesn't occur commonly; hence, such presentations have to be reviewed with care.
PubMed: 38391307
DOI: 10.4103/ijpm.ijpm_948_22 -
Cureus Jan 2024A 24-year-old female with a history of intravenous heroin use presented with two weeks of chills, myalgias, and cough and was found to be in acute hypoxemic respiratory...
A 24-year-old female with a history of intravenous heroin use presented with two weeks of chills, myalgias, and cough and was found to be in acute hypoxemic respiratory failure. Subsequent workup revealed the presence of bilateral septic pulmonary emboli and tricuspid valve endocarditis. Several weeks into her hospitalization, she developed periorbital edema and laboratory testing revealed she had developed acute renal failure and nephrotic range proteinuria. A renal biopsy confirmed the diagnosis of IgA-dominant Staphylococcus-associated glomerulonephritis (IgA-SAGN). Early recognition of this newly recognized variant of glomerulonephritis is paramount, as improper treatment may lead to catastrophic consequences.
PubMed: 38380208
DOI: 10.7759/cureus.52680 -
Malaysian Family Physician : the... 2024Orbital cellulitis is an extremely rare but potentially lethal condition in neonates. The clinical presentation of neonatal orbital cellulitis can be non-specific, and...
Orbital cellulitis is an extremely rare but potentially lethal condition in neonates. The clinical presentation of neonatal orbital cellulitis can be non-specific, and minimal signs of periorbital inflammation may go unrecognised by inexperienced parents or primary care medical personnel, leading to delayed treatment. Herein, we present a case series describing ophthalmia neonatorum complicated with neonatal orbital cellulitis owing to delayed treatment. The clinical presentation, management and outcomes are described. One neonate had orbital cellulitis, while the other had impending orbital cellulitis, with both cases resulting from delayed treatment of ophthalmia neonatorum. Both neonates were hospitalised for systemic antibiotic treatment and fully recovered with good outcomes. Timely identification and treatment of ophthalmia neonatorum are critical to mitigate potential severe sequelae, such as neonatal orbital cellulitis.
PubMed: 38371721
DOI: 10.51866/cr.438 -
Frontiers in Immunology 2024To assess the impact conferred by variants on the clinical spectrum of patients with systemic autoinflammatory diseases (SAIDs) in Greece. (Review)
Review
OBJECTIVE
To assess the impact conferred by variants on the clinical spectrum of patients with systemic autoinflammatory diseases (SAIDs) in Greece.
METHODS
Consecutive patients (n=167) with confirmed SAIDs who underwent screening by next generation sequencing (NGS) targeting 26 SAID-associated genes, and carried at least one gene variant, were retrospectively studied. The demographic, clinical and laboratory parameters were recorded.
RESULTS
In total, 24 rare variants in 23/167 patients (14%) were detected. Notably, 18 patients had at least one co-existing variant in 13 genes other than . Nine patients had juvenile- and 14 adult-onset disease. All patients presented with symptoms potentially induced by the variants. In particular, the candidate clinical diagnosis was Yao syndrome (YAOS) in 12 patients (7% of the whole SAID cohort). The clinical spectrum of patients with YAOS (mean episode duration 8 days) was fever (n=12/12), articular symptoms (n=8), gastrointestinal symptoms (n=7; abdominal pain/bloating in 7; diarrhea in 4; oral ulcers in 3), serositis (n=7), and rash (n=5), while the inflammatory markers were elevated in all but one patient. Most of these patients showed a poor response to nonsteroidal anti-inflammatory drugs (n=7/9), colchicine (n=6/8) and/or anti-TNF treatment (n=3/4), while a complete response was observed in 6/10 patients receiving steroids and 3/5 on anti-IL1 treatment. Another 8 patients were diagnosed with either FMF (n=6) or PFAPA syndrome (n=2) presenting with prominent diarrhea (n=7), oral ulcers (n=2), periorbital swelling and sicca-like symptoms (n=1), or maculopapular rash (n=1). One patient had a clinically undefined SAID, albeit characterized by oral ulcers and diarrhea. Finally, one patient presented with chronic relapsing urticaria with periorbital edema and inflammatory markers, and another one had a Crohn-like syndrome with good response to anti-IL-1 but refractory to anti-TNF treatment.
CONCLUSION
variants were detected in 1 out of 7 SAID patients and seem to have an impact on disease phenotype and treatment response. Further studies should validate combined molecular and clinical data to better understand these distinct nosological entities.
Topics: Adult; Animals; Humans; Hereditary Autoinflammatory Diseases; Retrospective Studies; Oral Ulcer; Simian Acquired Immunodeficiency Syndrome; Tumor Necrosis Factor Inhibitors; Diarrhea; Exanthema; Nod2 Signaling Adaptor Protein
PubMed: 38348033
DOI: 10.3389/fimmu.2024.1342668 -
Canadian Association of Radiologists... Feb 2024Fungal rhinosinusitis (FRS) includes non-invasive and invasive subtypes with the latter having significant morbidity and mortality. This systematic review aims to... (Review)
Review
Fungal rhinosinusitis (FRS) includes non-invasive and invasive subtypes with the latter having significant morbidity and mortality. This systematic review aims to identify the imaging features most correlated with invasive fungal rhinosinusitis (IFRS) and present a checklist of these features to aid diagnosis. PubMed, Embase, CENTRAL, and Science Direct were searched from inception to May 2023, in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) 2020 guidelines. Primary research articles published in English describing the imaging features of IFRS were included. The systematic review was conducted in accordance with the PRISMA guidelines. Forty-eight articles were identified for inclusion. Six studies examined radiological features in acute invasive fungal rhinosinusitis (AIFRS), and 9 studies of chronic invasive fungal rhinosinusitis (CIFRS). A majority of studies did not specify whether IFRS cases were acute or chronic. On CT, bony erosion and mucosal thickening were the most common features. Other features include nasal soft tissue thickening, nasal cavity opacification, opacification of the affected sinus, and perisinus soft tissue infiltration. Extra-sinus extension was commonly observed on MRI, most often invading intraorbitally and intracranially. Other sites of extra-sinus extension included the cavernous sinus, pterygopalatine fossa, infratemporal fossa, masticator space, and facial soft tissue. IFRS is a condition with potential for high morbidity and mortality. Several radiological features are highly suggestive of IFRS. Early identification of high-risk radiological features using a checklist may aid prompt diagnosis and early treatment. Future research investigating the radiological differentiation between IFRS and other significant pathology including bacterial orbital cellulitis would be beneficial.
PubMed: 38344986
DOI: 10.1177/08465371241227424 -
Turkish Journal of Emergency Medicine 2024Mucormycosis is a progressive and life-threatening disease that has been increasingly reported in patients infected by coronavirus diseases 2019 (COVID-19). We describe...
Mucormycosis is a progressive and life-threatening disease that has been increasingly reported in patients infected by coronavirus diseases 2019 (COVID-19). We describe a case of rhino-orbital mucormycosis with central nervous system involvement resulting in bilateral blindness and intracranial extension in a patient with uncontrolled diabetes mellitus (DM) and mild COVID-19 infection. A 35-year-old obese male, recently diagnosed with DM, presented to the emergency department suffering from dizziness, headache, speech difficulty, and facial weakness. His glycosylated hemoglobin was 10.4% and his reverse transcriptase-polymerase chain reaction (PCR) test came positive for COVID-19. Ocular examination revealed left eye proptosis, ophthalmoplegia, and lid edema with no ocular movement. Imaging studies showed pansinusitis and periorbital and orbital cellulitis with intracranial involvement. Histopathology and biopsy examination confirmed mucormycosis. Medical management included glucose control and liposomal amphotericin B therapy. Septoplasty and functional endoscopic sinus surgery was performed as emergency procedures. The patient survived with bilateral blindness. In this case, we described the importance of considering mucormycosis in COVID-19 patients with uncontrolled diabetes, particularly those presenting with sinusitis, headache, and orbital edema symptoms. Despite intensive antifungal therapy and surgical intervention, it is a serious opportunistic fungal infection associated with long-term complications.
PubMed: 38343519
DOI: 10.4103/tjem.tjem_308_22