Journal of Clinical Case Reports

Sneddon’s Syndrome (SS) is a rare condition characterized by a non-inflammatory thrombotic vasculopathy, which involves small and medium vassels. Cerebrovascular disease and livedo racemosa are the main clinical manifestations of this syndrome. The incidence of SS has been estimated of 4 cases per 1 million/year in general population with a high prevalence in young women between 20-40 years of age. Three forms of the syndrome have been described: idiopathic, without a clear causative factor, related to primary Antiphospholipid Syndrome and related to Systemic Lupus Erythematosus (SLE) with or without anti-phospholipid antibodies. To date, there are very few indications for diagnosis and treatment. Skin biopsy and brain MRI are considered fundamental in the diagnostic process; however histologic samples could be negative or non-specific. Current treatment is based on oral anticoagulation and, in autoimmune-related SS, on immunosuppressant drugs, such as corticosteroids, cyclophosphamide, azathioprine, with unclear results. No data about the use of anti-CD20 monoclonal antibodies (Rituximab) in autoimmune-related SS are available so far. We report here, for the first time, the case of a 40 years old woman with SLErelated SS with anti-phospholipid antibodies, successfully treated with Rituximab.

Dissecting esophagitis is a rare and benign form of chronic esophagitis, of diverse etiology, characterized by a typically whitish mucosa, with or without bleeding and presence of vertical circumferential fissures and cracks at endoscopy. Next, list a case of a young adult individual, who had a diagnosis of EDS with endoscopic diagnosis of EDS with probable cause of infection associated with loss of sub mucosal vascular component. After the diagnosis was made, therapeutic intervention was effectively possible. 

Primary Small Cell Neuroendocrine Carcinoma of the Breast (SCNCB) is rare and invasive, which lacks a standard therapy. Small cell carcinoma is a subtype of Small Cell Neuroendocrine Carcinoma (SCNC) and common in lung. It is difficult to distinguish small cell carcinoma in breast from which in the lung. Therefore, a whole-body scan is necessary to rule out metastasis from the Extra pulmonary Small Cell Carcinoma (EPSCC). This article reports two patients with SCNCB and reviews relevant literature to comprehensively discuss the diagnosis and management of this disease in these two cases. 

Background: Extra teeth number in the patient’s mouth is scientifically known as supernumerary teeth. Multiple supernumerary teeth are frequently seen in patients with developmental disorders. Cone-Beam Computed Tomography (CBCT) is a three-dimensional imaging tool that can provide a precise case planning. The purpose of this paper is to present a rare case of double parapremolars in on single quadrant and investigate these teeth by using Cone-Beam Computed Tomography (CBCT).

Case Description: A 28-year-old, Filipino female patient came to PNU dental clinics. She was medically fit, not on medications and no allergies were detected. Intraoral examination revealed presence of two supernumerary teeth at the area between #35 and #36. The Parapremolars located in quadrant three, both are with normal shape and color. Regarding the position, the first supernumerary has erupted lingually, the second is in the arch line but slightly tilted to the lingually. As a result of the extra teeth number, moderate crowding found in the area. The patient denied any family history of similar findings.

Discussion: Extraction of the first supernumerary tooth was carried out after radiographic analysis using CBCT, to facilitate proper oral hygiene, to prevent food impaction, and to eliminate its possible effects on the adjacent teeth. Extraction was performed cautiously without causing any damage to the anatomical structures. Regarding the anatomical position of the tooth, we prepared splint for teeth stabilization in case of adjacent teeth mobility. Extraction was done under local anesthesia. The patient was followed up after the extraction, no complications were reported.

Conclusion: Double parapremolar teeth were found in a non-syndromic patient. CBCT precisely locate malposed supernumerary teeth and help in getting proper treatment plan without complications.

Study design: A case report of Guillain–Barre Syndrome (GBS) variant presenting in a patient with high tetraplegia following cervical spinal cord lesion (C3-C6). Objective: To illustrate a clinical presentation of GBS in an individual with tetraplegia. Setting: Zhongnan Hospital of Wuhan University, Wuhan, China. Case presentation: A 55-year-old male with high spinal cord lesion at the level of C3-C6, following an emergency posterior cervical instrumentation and fusion with decompression of C3-C6 vertebral bodies under general anesthesia, developed urinary incontinence and weakness of the limbs, and was admitted to our facility for tetraplegia. Two months after admission, the patient had a sudden onset of fever (T40.0C) for which blood culture was done, and antibiotics were administered to sub side the fever. A few days later, the patient’s previously noted weakness progressed. A nerve conduction study was performed, which revealed severe axonal polyneuropathy affecting motor and sensory nerve fibers, prompting a diagnosis of acute motor-sensory axonal neuropathy (a variant of Guillian-Barre syndrome). Electromyography (EMG) reports indicated abnormal spontaneous activity in all limb muscles. An emergency Lumbar Puncture (LP) was performed which revealed the classical sign of albuminocytological disassociation of cerebrospinal fluid. GBS was diagnosed, but since the patient had pre-existing tetraplegia, autonomic dysfunction and was ventilated, the diagnosis was overshadowed and unfortunately delayed. Nevertheless, treatment modalities for both tetraplegia and GBS were initiated. There was a significant improvement in all extremities, but the bilateral decrease in the lower limbs muscle tone persisted. However, the patient refused to perform a repeat LP and was discharged three months later, under the requisite for regular follow-up. Conclusion: A careful neurological assessment prompted the diagnosis of acute polyradiculoneuropathy in a chronic patient with tetraplegia. It demonstrates how, in this population, an otherwise uncomplicated diagnosis of GBS can easily be missed. A deeper understanding of the cause and necessity for a subsequent therapeutic intervention in potentially life-threatening autonomic instability was understood via these signs. 

NLRP12 associated periodic syndrome, also called Familial Cold Autoinflammatory Syndrome-2 (FCAS2), is a monogenic autoinflammatory disorder caused by mutations of the gene NLRP12 and is characterized by recurrent episodes of fever, often after cold exposure or physical stress, in association with arthralgias/arthritis, myalgias, headaches, aphthous ulcers, lymphadenopathy, abdominal pain, sierositis, dermatitis and urticaria. The episodes can last several days and can be very disabling. In the latest years different mutations of NLRP12 have been identified, explaining the clinical heterogeneity and the different response to treatments. Here we describe a case of a 24-years-old man affected by FCAS2 with a new nonsense mutation of the gene NLRP12 who initially had a good response to steroid therapy and subsequently to anti-IL-1 beta treatment.

The Fascia Iliaca Compartment Block (FICB) is a regional nerve block that allows for complete anesthesia of the hip, as well as some parts of the thigh and knee. It is frequently administered in the Emergency Department (ED) for hip dislocations and fractures, and its successful application is associated with significantly decreased morbidity and mortality. However, we believe that its value far exceeds hip fractures, and that it should be used to alleviate pain for any acute or acute on chronic form of hip pathology. As emergency room physicians, it is our responsibility to initiate this mortality reduction in the ED. 

Background: Benign prostatic hyperplasia is a common condition affecting men worldwide that often requires the use of multiple medications. Older men may already be on several other medications for a variety of chronic conditions leading to a high prevalence of polypharmacy. Deprescribing is one approach to reduce polypharmacy, particularly if a medication is found to be high risk or no longer of benefit. Case report: A 68 year-old male, with a past medical history of benign prostatic hyperplasia, acid reflux, hyperlipidemia, major depressive disorder, blindness, low body-mass index, and frailty was prescribed several medications including tamsulosin and finasteride. The clinical pharmacist noted that the patient had been prescribed this dual therapy for benign prostatic hyperplasia since 2015. Amongst other recommendations, the clinical pharmacist suggested deprescribing the alpha-blocker due to several factors including duration of use; potential risk of adverse events secondary to multi-drug interactions; and presence of polypharmacy. Once the recommendation was implemented, the patient reported no instances of increased lower urinary tract symptoms and was well maintained on monotherapy. Conclusion: In patients with polypharmacy, the reduction of one medication may provide significant benefits. In the case of benign prostatic hyperplasia, patients who received six to twelve months of dual therapy may be able to control this condition with a 5-alpha-reductase inhibitor monotherapy. This class of medications has reportedly slowed clinical disease progression, reduced the risk of acute urinary retention and the need for invasive therapy, and improved voiding and storage symptoms. Healthcare providers should continue the practice of assessing medication regimens for appropriateness of therapy and deprescribing inappropriate therapy. 

Bronchospasm caused by an esophageal fistula or mediastinal abscess is not uncommon. Most of patients occur in small and medium-sized airways. It is rare to take the presentation of persistent central branch tracheal spasm and acute hypoxia as the primary symptoms. This article describes a 63-year-old female patient had a 1-day history of wheezing. Chest CT scan showed evident extensive stenosis of the bilateral central bronchi, and the formation of an abscess around the esophagus. Esophagoscopy revealed esophageal fistula. The patient received a series of anti-infective, antispasmodic, and antiasthmatic therapy methods, and recovered soon. Esophageal fistula caused by esophageal ulcer could occur in some tumor patients, resulting in mediastinal infection for the patients. But esophageal ulcers caused by accidental fishbone stuck in the throat are seldom. Most of the patients firstly may present with some symptoms related to digestive system organs. The study demonstrates the case is the rare presentation of a persistent central bronchial spasm caused by an esophageal ulcer, esophageal fistula, and mediastinal abscess induced by fishbone. We suggest that when patients accidentally are stuck in the throat with fishbone, they should seek medical assistance timely to make a definite diagnosis and treatment as early as possible. 

A mucocele is a mucous-filled cavity in the paranasal sinuses due to obstruction or compression of neighboring structures by inflammatory processes, trauma, or prior surgery. It can be indolent, locally expanding, and destructive. Mucoceles are extremely rare in children. We report a nine-year-old boy with sudden-onset left orbital pain and proptosis. Computed tomography showed a well-defined soft tissue lesion originating in the left ethmoid sinuses, suggestive of a mucocele. The mass was marsupialized by endoscopic sinus surgery. Mucoceles should be a part of the differential diagnosis of a child presenting with proptosis. Imaging studies are helpful in establishing the diagnosis. 

Cystic lesions are very common in the body, in particular in the ENT field. However, reports of cysts involving the Eustachian tube are very rare.  We present in this report a case of a 49-years-old man who presented for unilateral chronic otitis media with effusion. A rigid endoscopy was done revealing no nasopharyngeal mass. An MRI showed the presence of a cystic lesion in the cartilaginous portion of the left Eustachian tube measuring 10 × 6 mm.  The decision was made to do a left myringotomy with a PE tube insertion, followed by an endoscopic endonasal marsupialisation of the cyst. The cyst was sent to pathology. The pathologist confirmed the presence of a cyst with absence of signs of malignancy. In this article we will review the literature concerning Eustachian tube benign tumors; in particularly benign cysts, and we will discuss the pathology, symptoms, treatment and outcome.