Opera-glass deformity and tendon rupture in a patient with systemic lupus erythematosus

Summary Systemic lupus erythematosus (SLE) is a disease entity that frequently becomes clinically evident as a generalized arthritis. Despite this common presentation, it is widely accepted that the arthritis of SLE rarely produces severe joint deformity (1,2) with barely 10% of the patients developing swan neck, boutumiere deformity or a Jaccoud-like arthropathy (3). An even lesser proportion of patients develop joint erosions and there have been several clinical reports of tendon rupture in patients suffering from the above condition (4,5). However, there is still a great deal of controversy concerning the aetiology of the tendon rupture in these patients and while some authors suggest that corticosteroid therapy influences the outcome of this syndrome (6,7), pathology studies were included in a small number of these case reports. The subject of the present case report is a patient with SLE and resorptive arthropathy who suffered repeated episodes of tendon ruptures. The local pathologic findings in this patient suggest that the basic disease process was the cause of the tendon ruptures.     

Classical rheumatoid arthritis associated with relapsing polychondritis

Relapsing polychondritis is rare in rheumatoid arthritis (RA). We report a case of classical RA with relapsing polychondritis. Nasal septal chondritis resulted in cartilage collapse and a characteristic saddle nose deformity. HLA typing of the patient showed the presence of HLA-DR4 antigen.     

Mediastinitis caused by Klebsiella pneumoniae with a pharyngeal portal

We report a case of Klebsiella pneumoniae medisatinitis secondary to a retrophyarygeal abscess in a 40-year-old patient. The patient was treated with antibiotics and pleural drainage. Surgery was not necessary and the clinical course was favorable. We recall the pathophysiological and clinical aspects of this now uncommon condition and discuss the prognosis and therapeutic options.     

Fixation of sternal fracture using absorbable plating system,three years follow-up

Sternal fractures occur due to severe chest wall trauma in a small number of patients. They are often conservatively treated. The surgical intervention, although controversial, is indicated in case of deformity, severe pain, and ventilatory complications. We report the first case where absorbable plate has been used to fix a traumatic fracture in a 42-year-old female. After 3 years, the patient is still free of symptoms and CT scanning reveals intact sternal bone structure.     

Prenatal Diagnosis and Hemodynamics in Tetralogy of Fallot, Absent Pulmonary Valve with Endocardial Fibroelastosis, and Hydrops Fetalis

Tetralogy of fallot with absent pulmonary valve is rare. We report the antenatal diagnosis of this condition in early mid-semester. In this unusual case, the patient presented with hydrops fetalis. The pathophysiology is discussed. (ECHOCARDIOGRAPHY, Volume 10, November 1993)     

Bilateral pulmonary artery aneurysm associated with bilateral pulmonary thromboembolism, superior vena caval thrombosis, and Chagas' disease--a case report

The authors report a case of bilateral pulmonary artery aneurysm in a patient with chronic Chagas' disease and compensated congestive heart failure in whom clinical clues suggested pulmonary thromboembolism, and the actual nature of the lesions was discovered at the time of conventional imaging investigations. This case shows the rarity of bilateral pulmonary aneurysm associated with bilateral pulmonary thromboembolism and the importance of an awareness of this condition in the differential diagnosis for lung masses.     

Staphylococcus aureus pancarditis complicated by a left ventricular pseudoaneurysm

Acute septic pancarditis is a life threatening but fortunately rare condition. We report a case of this condition in a young 20-year-old female patient with an early complication of mitral valve destruction and a late complication of a left ventricular pseudoaneurysm formation.     

Pancreatic mass caused by Mycobacterium tuberculosis with reduced drug sensitivity.

Pancreatic tuberculosis is a rare condition which should be considered in patients with a pancreatic mass, particularly if the patient is young, not jaundiced, from an area of high TB prevalence with a normal ERCP. We report a case of pancreatic tuberculosis due to Mycobacterium tuberculosis with reduced sensitivity to rifampicin and isoniazid, that was treated with rifabutin, ciprofloxacin, ethambutol and pyrazinamide following clinical failure of first-line therapy. The case presented illustrates the importance of obtaining material for culture and sensitivity testing in cases of suspected TB.     

A patient with ulcerative colitis and central serous chorioretinopathy—A therapeutic dilemma

We report a case of a patient with ulcerative colitis and central serous retinopathy, which is a chronic ophthalmological condition that is frequently aggravated by corticosteroid treatment and may sometimes result in severe visual impairment. This case represents an interesting therapeutic dilemma pertaining to the treatment of ulcerative colitis exacerbation in a patient with this rare condition. Awareness of this condition as a cause of visual disturbance in IBD patients and a close ophthalmological cooperation are required in order to provide the optimal care for these patients.     

Postoperative thyroid crisis in an 11-year old male with McCune-Albright syndrome and atypical triiodothyronine hyperthyroidism: A case report

Rationale:McCune-Albright syndrome (MAS) is a rare heterogeneous clinical disease caused by sporadic, somatic, and postzygotic mutations. Thyroid crisis is even rare in patients with MAS, and we report the clinical outcomes of the first case of a MAS patient with atypical triiodothyronine (T3) hyperthyroidism who developed thyroid crisis after orthopedic surgery.Patient concerns:The patient with MAS and atypical T3 hyperthyroidism was an 11-year-old man who had undergone surgery for a right femur fracture and shepherd bending deformity. His main symptoms were dizziness, nausea, and vomiting with elevated body temperature because of developed thyroid crisis. Thyroid function tests showed high T3 and remarkably high free T3 levels, and remarkably increased thyrotropin level, but unchanged thyroxine and free thyroxine levels.Diagnosis:The patient was diagnosed with postoperative thyroid crisis following surgery for a right femur fracture, shepherd bending deformity, and MAS with atypical T3 hyperthyroidism.Interventions:Propranolol was intravenously administered. The therapy included intravenous hydrocortisone, a saturated solution of potassium iodine and propylthiouracil, and continuous physical cooling.Outcomes:The patient was discharged after achieving a stable condition with normal thyroid and liver function after surgery because of active anti-thyroid crisis treatment.Lessons:The operation of such patients should focus on the pre-operative heart rate, platelet level, and thyroid hormone levels. Abnormal values should be adjusted to the normal range, and such patients should achieve complete hemostasis and transfuse with blood following surgery anemia.     

Massive gastric bleeding from pancreatic pseudocyst,caused by rupture of pseudoaneurysm

Massive bleeding into a pancreatic pseudocyst is an unusual but life-threatening clinical condition. In this report, we present a case of massive gastric hemorrhage from a pancreatic pseudocyst, caused by rupture of a pseudoaneurysm of the splenic artery. The patient was successfully managed by total gastrectomy with splenectomy and distal pancreatectomy. Of a total of 66 cases in our Japanese literature review, only 5 cases of gastric hemorrhage associated with pancreatic pseudocyst have been reported, including the case herein presented. Diagnosis and therapeutic strategy are discussed.     

Carinal leiomyoma: report of a case treated by carinal resection and reconstruction

Here, we present a case report on a young female patient with a carinal leiomyoma. The patient complained of progressive dyspnea and paroxysmal cough. The laminagram and bronchoscopy revealed a lobulated carinal tumor, and carinal resection and reconstruction were performed. In this report, we will emphasize the appropriate surgical treatment for this condition.     

Ruptured aneurysm of the sinus of Valsalva associated with an atrial septal aneurysm

Aneurysm of the sinus of Valsalva (ASV) is a rare cardiac disease that may be acquired or congenital. It is usually an asymptomatic condition; however, when it ruptures, symptoms appear and the condition deteriorates rapidly. Atrial septal aneurysm (ASA) is a localized "saccular" deformity of the interatrial septum that is associated with cerebrovascular events of embolic origin. We will report on a case of a 69-year-old woman who was referred to our department because of congestive heart failure. Echocardiographic evaluation, both transthoracic and transesophageal, disclosed a ruptured aneurysm of the right sinus of Valsalva into the right atrium (RA), which was associated with an aneurysm of the atrial septum.     

Brugada Phenocopy in a Patient with Pectus Excavatum: Systematic Review of the ECG Manifestations Associated with Pectus Excavatum

Brugada phenocopies (BrP) have emerged as new clinical entities that are etiologically distinct from true Brugada syndrome (BrS). BrP are characterized by an ECG pattern that is phenotypically identical to true BrS (type 1 or type 2); however, BrP are caused by various other factors such as mechanical mediastinal compression, myocardial ischemia, pericarditis, myocarditis, pulmonary embolism, and metabolic disturbances. We report a case of an electrocardiographic BrP in a patient with pectus excavatum deformity in the absence of true BrS using currently defined BrP diagnostic criteria. A systematic review of ECG manifestations associated with pectus excavatum is also discussed.     

Gastric antral vascular ectasia (GAVE) associated with systemic sclerosis: relapse after endoscopic treatment by argon plasma coagulation

In this report, we present a case of gastric antral vascular ectasia (GAVE) associated with systemic sclerosis and interstitial pneumonitis. This case showed resistance to endoscopic treatment using argon plasma coagulation (APC). After initial recognition of GAVE as the origin of persistent anemia, three sessions of APC were performed and dilated vessels on the antrum were eliminated completely. Five months after primary treatment, follow-up endoscopy revealed deformity of the gastric antrum caused by ulcer scars induced by APC, with no vascular ectasia. Ten months later, the patient showed anemia and recurrence of GAVE on endoscopy. Ablation using APC was performed again, thereby eradicating recurrent GAVE completely. At a two months' follow-up, however, recurrent GAVE was indicated. In spite of GAVE eradication by APC, a third recurrence of GAVE was observed after 32 months. During the follow-up period, systemic sclerosis and interstitial pneumonitis were controlled clinically by administration of methyl prednisolone with no aggravation.     

"Let's get it straight": the story of the spiral ureter

A "corkscrew deformity" of the proximal ureter is a rare congenital anomaly that typically lacks any postnatal clinical significance. The rarity of this entity, however, has not allowed the clarification of its natural history and the ideal approach to its management. We herein present a case of a 27-year-old female patient who presented with right flank pain and significant hydronephrosis. On retrograde ureterography, a typical spiral configuration of the proximal ureter was noted. The patient underwent successful reconstruction by laparoscopic transperitoneal dismembered pyeloplasty. We report the first use of laparoscopic reconstruction for the management of "corkscrew deformity" of the proximal ureter and we focus on the imaging findings, technical details, advantages and limitations of this technique.     

Remission induction of refractory anaemia with excess blasts in transformation by sole treatment with granulocyte colony-stimulating factor with persistent chromosomal abnormality

We report a patient with myelodysplastic syndrome (MDS), refractory anaemia with excess blasts in transformation, in whom complete remission (CR) was achieved with the administration of granulocyte colony-stimulating factor (G-CSF). The 76-year-old patient was admitted to our hospital with a fever and a productive cough; a diagnosis of pneumonia was thus made. Following treatment with antibiotics, the patient's condition improved, and MDS was diagnosed from peripheral blood and bone marrow examinations after the patient recovered from the infection. The patient achieved a sustained haematological CR that was confirmed by morphological and flow cytometric examination after treatment with G-CSF alone, although chromosomal abnormalities persisted. According to the literature, in almost all patients with acute myeloid leukaemia or MDS who were reported to achieve CR by G-CSF, the course was associated with infection, although our case did not have this complication during the course of G-CSF therapy. We suggest that patients with G-CSF alone without infection can achieve CR and that this may be related to a differentiation effect of G-CSF based on persistent chromosomal abnormality in this case.     

Multiple enterolithiasis, coexisting with bladder and gallbladder lithiasis, associated with colon adenocarcinoma

Enteroliths are calculi primarily formed in the intestine. Enterolithiasis is a rare condition frequently associated with intestinal stasis. Usually it causes no symptoms in most cases, but it can be an important diagnostic clue in patients presenting intestinal occlusive symptoms. We report a case of multiple enterolithiasis, very infrequent pathology, coexisting with bladder and gall bladder lithiasis in a patient with colon adenocarcinoma. Diagnosis was made by X-rays and CT images. Calculi were analysed by several methods: chemical, infrared spectroscopy, stereoscopic microscopy and atomic emission spectroscopy; they showed that caluli are made up of organic material and whilokita (calcium and magnesium ortophosphate). No risk factors for lithogenesis were found in this patient excluding the intestinal stasis caused by intestinal narrowing as a result of adenocarcinoma. Genetic factors are suggested as main contributors to hyperlithogenesis observed in this patient. The physiopathological conditions were studied in depth and literature about this subject reviewed.     

Lack of TSH inhibition by exogenous L-T4 in a patient with follicular thyroid carcinoma

We report a case of a male patient thyroidectomized for follicular thyroid carcinoma and presenting extremely elevated serum thyrotropin levels under L-T4 suppressive therapy. Administration of L-T3 in increasing amounts resulted in a significant decrease of serum TSH levels. The nature of the possible molecular defects underlying this unusual condition and pitfalls arising from the failure of L-T4 therapy to inhibit TSH secretion in a patient in post-surgical follow-up for follicular carcinoma are discussed.     

A positive outcome in patient with massive acute pulmonary embolism and right atrial mobile thrombus fragmented during thrombolysis: a serial echocardiographic examination

The case report presents a patient with acute, massive pulmonary embolism diagnosed by transthoracic echocardiography, with the mobile thrombus visualized in the right atrium. During the thrombolytic therapy the thrombus was fragmented and migrated to the pulmonary artery, with a rapid transient worsening of the clinical status of the patient. The continuation of thrombolysis led to the gradual improvement of the patient condition. In this case report the importance of serial echocardiographic examinations in revealing right heart masses, the right ventricle overloading and the effectiveness of treatment is shown.