Πέμπτη 31 Ιανουαρίου 2019

Severe case of pneumonia with pleural effusion in an immunocompromised woman due to Fusobacterium necrophorum

Infections caused by fusobacteria have a wide clinical spectrum, and in certain patients, they can lead to severe systemic illness. We report the case of an immunocompromised young woman who presented with severe pneumonia complicated by parapneumonic pleural effusion, despite wide-spectrum antibiotic treatment. Fusobacterium necrophorum was isolated in the samples obtained after thoracentesis was performed. Apart from the pulmonary involvement, we could not identify the infectious focus, since the patient did not have poor dentition or Lemierre's syndrome. After an extended antibiotic regimen and placement of a chest tube, the patient fully recovered and was able to be discharged.



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Complete resolution of erythrodermic psoriasis with first-line apremilast monotherapy

Erythrodermic psoriasis (EP) is the most serious type of psoriasis with high morbidity and mortality. First-line recommended therapies for EP, cyclosporine and infliximab have significant adverse effects. Cyclosporine increases the risk of hypertension, leucopenia, infections and renal failure. Infliximab increases the risk of reactivation of tuberculosis, hepatitis B and histoplasmosis, and increases risk for hepatitis, autoantibody formation, congestive heart failure, demyelinating disorders, pancytopenia, lymphoma and skin cancer. An effective drug with a much safer side effect profile will be of significant benefit in EP. The phosphodiesterase 4 inhibitor apremilast is U.S Food and Drug Administration (FDA) approved for plaque psoriasis and psoriatic arthritis. Adverse effects of apremilast reported are headache, nausea, diarrhoea, upper respiratory tract infection, potential for depression and weight loss. We report complete and long-standing resolution of EP with first-line apremilast monotherapy. Apremilast may be an effective option with comparatively minor side effects for EP.



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Ectopic varices masquerading as lower gastrointestinal bleeding

A middle-aged woman previously in good health arrived to hospital with severe haemorrhagic shock. An abdominal and pelvic CT scan showed hepatomegaly, fluid in the sigmoid colon, perigastric and right sided pelvic varices without a clear source of bleeding. Urgent esophagogastroduodenoscopy excluded gastric varices and active upper gastrointestinal bleeding and the colonoscopy was inconclusive due to haemodynamic instability and massive bleeding which made it impossible to proceed with a full colonoscopy. An explorative laparotomy identified liver cirrhosis and bleeding from varices of the right fallopian tube which had formed a fistula with the caecum. A right hemicolectomy with a side to side anastomosis and a right sided salpingo-oophorectomy were carried out. The patient was thereafter transferred to the intensive care unit intubated, requiring massive fluid resuscitation and high doses of vasoactive agents. Nine days after hospital admission she was transferred to the surgical ward and followed up by a hepatologist.



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Adipsic diabetes insipidus secondary to craniopharyngioma resection

Adipsic diabetes insipidus is an infrequent disease which may be associated with craniopharyngioma. It may be secondary to the tumour's extension, as well as to resection of the mass. We present the case of a 24-year-old woman with a history of delayed puberty and hypothyroidism, but no prior study reports. She consulted due to a headache with warning signs associated with altered visual acuity. Brain MRI was performed which showed signs of a non-adenomatous lesion with suprasellar and hypothalamic extension. Following transcranial surgery, she developed diabetes insipidus criteria, with absence of thirst documented during the hospitalisation. The histopathological findings confirmed the diagnosis of craniopharyngioma. The patient was treated with desmopressin and received recommendations regarding rehydration according to the quantification of losses, with electrolyte stabilisation.



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Rare cause of lung atelectasis in a young woman

Pulmonary mucoepidermoid carcinoma is an extremely rare intrathoracic malignancy, comprising less than 1% of all lung tumours. These are very slow growing and are classified into low grade and high grade based on histological features. Surgical resection is the primary treatment with excellent outcomes, while chemotherapy or radiotherapy effectiveness is not known. Preoperative fluorine-18 fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) is useful for predicting tumour grade and postsurgical prognosis.

A clinical case of a 31-year-old woman who presented with dyspnoea on exertion, cough and wheezing is reported. Imaging studies revealed a mass involving the left lower lobe bronchus and atelectasis. 18F-FDG PET/CT showed uptake in the described mass with a maximum standardised uptake value of 9.7. Complete surgical resection was performed, and pathological examination revealed a high-grade mucoepidermoid carcinoma with tumour-free margins. Adjuvant chemotherapy was given and there is no evidence of tumour recurrence.



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Stickler syndrome: a possible presentation of Pierre Robin sequence



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Long-lasting response to afatinib that persisted after treatment discontinuation in a case of EGFR-mutated lung adenocarcinoma

It is unknown whether tyrosine kinase inhibitors targeting epidermal growth factor receptor (EGFR) can be discontinued in patients in whom EGFR-mutated lung cancer has well stabilised. We present a case of a 73-year-old Japanese woman with no history of smoking. Right pulmonary lower lobectomy, lymph node dissection and segmental resection of the right middle lobe were performed. Additionally, she underwent adjuvant chemotherapy for stage IIIB adenocarcinoma harbouring an EGFR exon 19 deletion. Afatinib was administered for liver metastases after 15 months. A complete response of metastatic disease was achieved for 2 years. However, afatinib was unavoidably discontinued due to splenectomy for the treatment of idiopathic thrombocytopenic purpura. Although afatinib was not resumed, due to the abscess formation as surgery complication, a drug-free complete response was sustained for over 18 months. The present case suggests that exceptional and durable responses to afatinib can be achieved in individual cases.



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Seizure in infancy: not the usual suspect



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'Never say never: the first successful management of a type A aortic dissection in Liberia

The West African country of Liberia ranks as one of the lowest in the world in most measures of health. The diagnosis and management of complex surgical cases such as aortic dissection is extremely challenging, for reasons ranging from lack of diagnostic imaging capabilities to the high resources required for definitive surgical intervention. We present the first known successfully managed case of aortic dissection in the country's history and with it highlight the challenges faced and a number of lessons learned that are beneficial to anyone working in resource-limited environments.



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A tale of 1 year: a case of bilateral conductive hearing loss due to bilateral ossicular chain disruption post head trauma

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Abstract
The traumatic hearing loss is a common finding after head trauma that involves the temporal bone fracture or skull base injury, but the late presentation of the hearing loss bilaterally is a rare presentation. This case report describes the rare and late presentation of bilateral conductive hearing loss 1-year after the head trauma, limited to ossicular chain disruption and hearing loss. This case of post head trauma that was limited to ossicular chain distribution progressed to bilateral conductive hearing loss without temporal bone fracture. A 14-year-old male was presented conductive hearing loss of one year after head injury, CT scan revealed bilateral ossicles disruption. Afterwards, osicuoloplasty was performed which improved the hearing of the patient. In conclusion, bilateral hearing loss with no obvious skull fracture should be suspected and investigated so an early intervention could be established. The CT was considered the imaging modality of choice for ossicular chain distribution.

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Proceed or palliate: Surgicel® mimicking tumor recurrence and causing a small bowel obstruction

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Abstract
Surgicel® is a commonly used hemostatic agent. We report a case of small bowel obstruction secondary to the prior placement of Surgicel® within the peritoneal cavity, and the radiological diagnosis conundrum and potential prognostic implications this finding has in a patient with a known history of malignancy.

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Unclassified autoimmune pancreatitis mimicking pancreatic cancer

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Abstract
A 24-year-old black male presented with a 1-week obstructive jaundice and intermittent abdominal pain, with no significant weight loss and an unsuspicious abdominal exam. Blood chemistry showed a cholestatic pattern but a complete immunological and tumoral panel (anti-smooth muscle antibody, anti-mitochondrial antibody, anti-nuclear antibody, anti-neutrophil cytoplasmic antibody, anti-Smith, anti-double-stranded-DNA antibody (anti-dsDNA), complement C3/C4, carcinoembryonic antigen, CA 19-9 and IgG4) were all within normal limits. Abdominal ultrasound revealed dilatation of the intra and extra-hepatic bile ducts. CT scan showed an abnormal dilatation of the distal bile duct but no focal enlargement of the head of the pancreas. Endoscopic ultrasound suggested an inflammatory process but the magnetic resonance cholangio-pancreatography favored a neoplastic obstruction of the distal common bile duct. Fine-needle aspiration cytology was insufficient for definitive diagnosis and the patient underwent major surgery. Follow-up with mild exocrine pancreatic insufficiency treated with enzyme replacement.

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Combination of laparoscopy and endoscopy as an option for treatment patients with gastric neuroendocrine tumors

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Abstract
Background
Neuroendocrine tumors (NETs) are relatively rare neoplasms with the increasing survival due to the development of early diagnostics. There is no universal position in treatment and follow up of small (~20 mm) gastric NETs.
Clinical cases
Two female patients 51 and 66 y.o. with multiple gastric NETs <2 cm were observed in our department. In both cases treatment was performed by combination of two minimally invasive technologies: laparoscopy and gastroscopy. According to the localization of tumors in one case intraluminal gastric resection controlled by laparoscopy was performed. In the second case laparoscopic gastric resection with gastroscopy assistance was done.
Discussion
There are two positions for surgical treatment of small NETs: to operate as the typical premalignant neoplasm or to make submucosa resections. We demonstrated combination of laparoscopy and gastroscopy as feasible approach with minimal risk of complications.

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Small bowel intussusception and concurrent sigmoid polyp with malignant transformation in Peutz–Jeghers syndrome

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Abstract
Peutz–Jeghers syndrome (PJS) is an autosomal dominant condition characterized by the association of gastrointestinal polyposis, mucocutaneous pigmentation and cancer predisposition McGarrity, Amos, Baker (Peutz–Jeghers Syndrome, GeneReviews(R), National Center of Biotechnology Information.). Intussusception and malignant polyps are not rare complications of PJS, where the lifetime risk of intussusception is 48% and the possibility of developing any cancer by age 65 years is 37% Kopacova, Tacheci, Rejchrt, Bures (Peutz-Jeghers syndrome: diagnostic and therapeutic approach. World J Gastroenterol 2009;15:5397–408.). Very few cases of malignant polyps causing intussusception associated with synchronous malignant polyp in PJS have been reported to date Cai, Tian, Zhou, He, Hu, Deng (Jejunal intussusception and polyps with different types of malignant transformation in Peutz-Jeghers syndrome: report of a case. Oncol Lett 2013;5:239–41.). We describe a case of a patient with PJS presenting with symptomatic jejunal intussusception and an incidentally found malignant hamartoma in the sigmoid colon.

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Coexistence of intracranial solitary fibrous tumor/hemangiopericytoma and right middle cerebral artery aneurysm

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Abstract
Intracranial solitary fibrous tumors are rare mesenchymal neoplasms originating in the meninges and constitute a heterogeneous group of rare spindle cell tumors that include benign and malignant neoplasms of which hemangiopericytoma is nowadays considered a cellular phenotypic variant. From literatures, the incidence of coexistence of brain tumors and intracranial aneurysms is ~0.7–5.4%. Meningioma is the most frequent tumor coexisted with intracranial aneurysms, followed by pituitary adenoma, neuroepithelial tumor, and metastatic tumor. We would like to report a case of 74-year-old man harboring a rare intracranial solitary fibrous tumor/hemangiopericytoma and an unruptured aneurysm of the right middle cerebral artery which probably the first report of these combinations in the English literature. Both lesions were treated surgically in one session with favorable outcome. Magnetic resonance angiography should be performed in patients with brain tumor preoperatively not only to visualize neoplastic vascularization but also to pick up incidental aneurysm.

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Jejunal diverticulitis

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Abstract
Cases of small bowel diverticulitis, excluding Meckel's diverticulitis, are rare. Small bowel diverticular disease has been reported in approximately 0.3–1.3% cases of post mortem studies (Fisher JK, Fortin D. Partial small bowel obstruction secondary to ileal diverticulitis. Radiology 1977;122:321–322.) and in only 0.5–1.9% of contrast media study cases (Cattell RB, Mudge TJ. The surgical significance of duodenal diverticula. N Engl J Med 1952;246:317–324). Diverticula located within the small bowel may have presentations and complications similar to that of colonic diverticular disease. However, there is no consensus for the management for small bowel diverticulitis. Given that small bowel diverticulitis, like a colonic diverticulitis, can cause an acute abdomen, surgical intervention may be required. In this particular case, a patient presented with symptoms of lower abdominal pain, nausea and fever. Following an x-ray and CT scan, the patient underwent an open laparotomy and small bowel resection of a portion of jejunum that contained a symptomatic diverticulum.

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Myogenic fibrosis of the flexor tendons after amphetamine drug abuse

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Abstract
Myogenic fibrosis of the flexor tendons in a 23-year-old patient was caused by intense drug abuse. He was presented in the clinic with spastic flexor tendon contracture of his right hand. The patient was treated by tendon elongation with a satisfactory result. The treatment of flexor tendon contracture in those cases is very difficult and needs to be done in a complex way.

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Anterior mediastinal mass 4 years after type A aortic dissection

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Abstract
A 47-year-old male who previously underwent emergency surgery for type A aortic dissection presented to the emergency department 4 years later with acute chest pain radiating to his back and a significant drop in hemoglobin. Clinical presentation was concerning for aortic graft failure, but imaging revealed a large (6.0 × 3.2 × 12.8 cm3) soft tissue mediastinal mass that was not present 4 years before. Pathologic analysis revealed a rare thymic cyst with a hemorrhagic component. This was a challenging case from diagnostic and operative perspectives, involving the expertise of both cardiac and thoracic surgical teams.

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A novel case of late left ventricular outflow tract obstruction post mitral valve surgery

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Abstract
Left ventricular outflow tract (LVOT) obstruction (LVOTO) is a rare but recognized complication of mitral valve surgery. Concomitant factors can cause significant clinical deterioration which requires urgent intervention. We present the case of a female patient who underwent closed valvotomy at age 20 for mitral stenosis secondary to rheumatic heart disease. Seventeen years later, a re-stenosed valve prompted mitral valve replacement (MVR) with a Carbomedics mechanical prosthesis. 8 years later, she re-presented with increasing dyspnoea. On echocardiogram a frond-like structure was visible in the LVOT, the valve housing was protruding into the LVOT and she had marked septal hypertrophy. The LVOT gradient was 72 mmHg. This is a novel case of mechanical MVR associated LVOTO due to at least three discrete factors. This case demonstrates how multiple factors can amalgamate to result in significant clinical deterioration. Over an 8-year period, all three distinct factors contributed to the development of severe LVOTO.

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Unusual Manifestation of Ulcerative Colitis

The relationship of inflammatory bowel disease (IBD) and chronic recurrent multifocal osteomyelitis (CRMO) is understood as extraintestinal rheumatic manifestations. CRMO is a chronic, relapsing, inflammatory, noninfectious disorder of the skeletal system of unknown origin. The disease course is not always recurrent. The association of CRMO and ulcerative colitis (UC) is very rarely reported. We report a case of a 10-year-old Saudi female who was diagnosed with CRMO, when she developed fever in association with left foot pain, and ulcerative colitis was confirmed endoscopically and histologically based on a previous settled diarrheal illness and severe iron deficiency anemia which required blood. Both conditions responded well to IBD therapy. To the best of our knowledge, this is the first reported case of chronic, multifocal osteomyelitis associated with pediatric UC in Saudi Arabia. This report supports the use of IBD therapy in treating CRMO.

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