1/30. Rapidly fatal necrotizing fasciitis after aesthetic liposuction.Necrotizing fasciitis (NF) is a rapidly progressive soft tissue infection involving primarily the superficial fascia and subcutaneous tissue. The disease is caused by streptococcus pyogenes or synergistic infection of anaerobic and facultative anaerobic bacteria. Further characteristics are severe, intolerable pain and a mortality rate of 30 to 50%. The NF can be initiated after surgical procedures, minor trauma, trivial scratches, in the setting of a chronic wound, or even in apparently intact skin. The age of the patient is not relevant for the prognosis of NF. As it is shown in this reported case, a young and previously healthy patient died after aesthetic liposuction in the course of a NF. Necrotizing fasciitis is a rare disease, therefore, it is important to review its diagnostic and clinical features, because only early diagnosis and prompt, radical surgery improves the survival rate.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/30. Two cases of orbital infarction syndrome.Orbital infarction syndrome is defined as ischemia of all intraorbital and intraocular structures. It is a rare disease caused by rich anastomotic vascularization of the orbit. It can occur secondary to different conditions, such as, acute perfusion failure, systemic vasculitis, orbital cellulitis and vasculitis. It results in orbital and ocular pain, total ophthalmoplegia, anterior and posterior segment ischemia, and acute blindness. We report here upon two cases of orbital infarction with similar presentations but with different causes, namely, mucormycosis and as a postoperative complication of intracranial aneurysm, discuss the possible mechanisms of orbital infarction, and present a review of the literature on the topic. The prompt recognition of clinical pictures and rapid diagnosis is essential for the early treatment of orbital infarction, since its progression is very rapid and it can be even fatal.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
3/30. Reactive hypoglycaemia due to late dumping syndrome: successful treatment with acarbose.Reactive hypoglycaemia is a rare disease which occurs postprandially in everyday life involving blood glucose levels below 2.5 to 2.8 mmol/l. We report on a 66-year-old patient who developed symptomatic reactive hypoglycaemia due to late dumping syndrome 10 years after oesophagectomy with cervical anastomosis. A 75 g sucrose load revealed a plasma glucose level of 9.4 mmol/l after one hour, followed by symptomatic hypoglycaemia with a plasma glucose level of 1.8 mmol/l after three hours. Concomitantly, high concentrations of insulin (3216 pmol/l at a glucose level of 9.4 mmol/l and 335 pmol/l at a glucose level of 1.8 mmol/l) and glucagon-like peptide 1 (GLP-1) (375 pmol/l at a glucose level of 9.4 mmol/l and 85 pmol/l at a glucose level of 1.8 mmol/l) were measured. While the patient was under treatment with acarbose, another sucrose load did not provoke symptomatic hypoglycaemia (plasma glucose nadir of 4.6 mmol/l after two hours). insulin and GLP-1 levels increased much less, to peak levels of 375 pmol/l and 75 pmol/l respectively, after one hour when plasma glucose was 6.8 mmol/l. We conclude that in patients with reactive hypoglycaemia due to gastrointestinal surgery, acarbose decreases rapid glucose absorption associated with hyperglycaemia and GLP-1 secretion, and thus diminishes excessive insulin release. acarbose is therefore a successful treatment modality for reactive hypoglycaemia due to late dumping syndrome.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
4/30. Parastomal pyoderma gangrenosum: a case report and literature review.Parastomal pyoderma gangrenosum (PPG) is an exceedingly rare disease process most often observed in inflammatory bowel disease patients with an ileostomy. Fewer than 50 cases have been reported in the medical literature. The incidence is 0.6 per cent of patients with ileostomy and inflammatory bowel disease. The rarity of the disease leads to misdiagnosis and mistreatment of the lesion. The intense pain and disruption of ostomy function greatly impair affected individuals beyond the limit of their underlying disease. Current best care practices observed in small study series indicate long-term intensive medical therapy aimed at systemic disease suppression to optimize PPG wound healing. Our patient had no signs of active crohn disease at the time of PPG presentation. She was initially treated with minimal wound debridement and intralesional triamcinolone. Finally under the care of an enterostomal/wound care therapist the patient achieved excellent PPG resolution in 6 months.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
5/30. Fracture of the clavicle after surgical treatment for congenital pseudarthrosis.BACKGROUND: Congenital pseudarthrosis of the clavicle is a rare disease. There are about 200 individual cases reported so far in world literature, mainly in the form of case reports. The majority of authors recommend surgical. An analysis of 103 cases published since 1980 shows only a few post-surgical complications. CASE REPORT: The authors present a female patient operated at the age of 4.5 years. During surgery both cartilaginous ends were resected and internal fixation was performed by a one-third semitubular plate fixed by 3.5 mm cortical screws. Ten days after hardware removal, a fracture occurred in the screw-hole following a minor impact. Surgery to heal the congenital pseudarthrosis was successful, but a new fracture occurred, resulting from bone weakening. This simple fracture was treated conservatively. At present (5 years after the first surgery) the patient's parents are completely satisfied with both the functional result and the cosmetic appearance. CONCLUSIONS: The authors have not found in the literature any reports of similar complications after surgical treatment of this condition. The use of more delicate osteosynthetic material might prevent the complication described here.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
6/30. Spontaneous resolution of lumbar vertebral eosinophilic granuloma.eosinophilic granuloma (EG) is a rare disease but is more common in adults than children. It's often self-limiting. Spinal involvement is rare. It is the localized and most benign form of Langerhans' cell histiocytosis (previously known as histiocytosis X), characterised by lytic lesions in one or more bones. Spontaneous resolution of vertebral body lesions is very rare. In this case, the patient had one EG in a cervical vertebra and a similar lesion in a lumbar vertebra. This case is important because it featured a symptomatic lesion in the cervical spine accompanied by an asymptomatic lesion in a lumbar vertebra. We treated the cervical lesion by surgical fusion and followed the lumbar lesion up conservatively, with the patient in a corset. After 8 years of follow-up, control MRI showed that the lumbar lesion had spontaneously resolved.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
7/30. Diffuse xanthogranulomatous pyelonephritis in a child with severe complications.Xanthogranulomatous pyelonephritis is a rare disease in childhood. Because the symptoms and signs are chronic and non-specific, preoperative diagnosis is usually difficult. We report an 8-year-old boy who had an abdominal mass and anemia for more than 6 months. fever and dyspnea occurred 4 days prior to admission. ultrasonography revealed an enlarged right kidney with multiple parenchymal hypoechogenic areas, absence of normal parenchymal structures, and perinephric thickening with multiple calcifications. An abdominal computed tomogram demonstrated an irregular, enlarged right kidney with multiple low-density round areas consistent with hydronephrosis and calculi. Diminished excretion of contrast media and a severe perinephric inflammatory reaction were present. Poor right kidney function was demonstrated by Tc99m-diethylenetriamine penta-acetic acid split renal function examination. We diagnosed xanthogranulomatous pyelonephritis preoperatively based on the clinical and radiological features. The child first had drainage of an extrarenal abscess and antibiotic therapy, followed by definitive nephrectomy. The hospital course was complicated with pleural effusion, peritonitis, pelvic abscess, and sepsis. A two-stage nephrectomy requiring less radical resection and decreasing the surgical complications would have been preferable.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
8/30. Fatal acute pulmonary embolism in a patient with pelvic lipomatosis after surgery performed after transatlantic airplane travel.We describe a case of a 37-yr-old patient who traveled from europe to the United States and succumbed to a massive pulmonary embolism 6 days after elective pelvic surgery despite routine postoperative thrombotic prophylaxis. In retrospect, he was likely to have developed a deep venous thrombosis during the transatlantic trip to our hospital. Anesthesiologists and other physicians involved in perioperative management need to be aware of the prevalence of venous thromboembolism in patients with a history of recent prolonged air travel. This is particularly true in tertiary referral centers, where patients with rare diseases may have a major surgical intervention within days of prolonged air travel.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
9/30. Osler-Weber-Rendu disease--unexpected complication following excision of splenic aneurysm--a case report.Osler-Weber-Rendu disease is an autosomal dominant disease, sometimes known as hereditary hemorrhagic telangiectasia (HHT) with a family history. It is a rare disease and there are no records of families with inheritance lineage in Riyadh. We experienced an anesthetic management of a 28-yr-old woman with Osler-Weber-Rendu disease. She was diagnosed to have splenic aneurysm and was scheduled for excision of splenic aneurysm. A chest X-ray revealed pulmonary arterio-venous malformation all over the lungs. The anesthesia and surgery went deliberately smooth with selective period of intensive care admission. In the intermediate post operative period, however, she was diagnosed as having pleural effusion. A chest drain was inserted as an emergency, but the patient developed tension pneumothorax, which necessitated resuscitation and readmission to intensive care Unit. lung atelactasis was diagnosed. Attendance by qualified surgical night staff, communication, vigilance and consultations are as important as the proper management of such rare cases.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
10/30. klebsiella pneumoniae peritonitis shortly after kidney transplantation.We report a case of spontaneous bacterial peritonitis caused by klebsiella pneumoniae in a 34-year-old male recipient shortly after kidney transplantation. On posttransplant day 10, the patient started complaining of severe abdominal pain and nausea. body temperature was 38.4 degrees C. The abdomen was diffusely tender with rigidity and rebound. Laboratory data showed a normal erythrocyte sedimentation rate and serum creatinine level but a slightly elevated C-reactive protein concentration and leukocytosis of 36,200 cells/mm(3) with 88% neutrophils. Explorative laparotomy revealed diffuse purulent peritonitis without an intraabdominal source of infection, such as intestinal perforation. The peritoneal fluid revealed greater than 1000/mm(3) white blood cells and many gram-negative bacilli. Fluid cultures yielded growth of klebsiella pneumoniae. The patient responded to antibiotic therapy; he was discharged in good condition. This case report draws attention to the impaired host defense that may predispose to spontaneous bacterial peritonitis in renal transplant recipients and alerts the clinician to the possibility of this rare disease.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
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