Project description:Polyangiitis overlap syndrome is a rare clinical entity comprising patients with overlapping features of more than one vasculitis, usually eosinophilic granulomatosis with polyangiitis (EGPA) and granulomatosis with polyangiitis (GPA). Few cases of polyangiitis overlap syndrome have been described in the literature, mostly associated with c-ANCA, anti-proteinase (PR)-3 positivity, a protean clinical picture characterized by vasculitis, eosinophilia and eosinophilic infiltrates in tissues and a favorable response to steroids and immunosuppressant treatments. Herein, we present a case of a 66-year-old woman with nasal obstruction, external nose deformity, sensorineural hearing loss, peripheral blood eosinophilia, high titer anti-PR3 antibodies and lung involvement. Nasal septum biopsies showed inflammatory infiltrate with eosinophilic component; histopathology of the lung demonstrated necrotizing granulomas associated with inflammatory infiltrate composed of numerous neutrophils and some eosinophils. The patient was diagnosed with polyangiitis overlap syndrome and successfully treated with cyclophosphamide. Recognizing this entity is fundamental given the distinct clinical phenotype and outcomes to therapy in the complex scenario of ANCA-associated vasculitides.
Project description:PurposeThe purpose of this study is to evaluate the clinical outcomes in patients with traumatic pseudophacocele.MethodsIn this retrospective, interventional case series, scleral wound repair with pars plana vitrectomy and glued intrascleral fixation of an intraocular lens (glued IOL) was performed in 5 eyes of 5 patients. Pupilloplasty was performed in 3 cases whereas aniridia glued IOL fixation was done in 1 case that had total avulsion and loss of iris tissue. The main outcome measures were best-corrected visual acuity (BCVA), intraoperative and postoperative complications during the entire follow-up period.ResultsThe preoperative vision ranged from hand movement to perception of light in all the patients. The mean postoperative BCVA was 0.42 ± 0.21 Snellen's decimal equivalent (SDE) at final follow-up. Postoperatively, all the cases retained good visual acuity with case 1 and case 2 reporting 0.5 SDE, case 3 had 0.33 SDE, case 5 had 0.67 SDE whereas case 4 had a final visual acuity limited to 0.1 SDE due to associated corneal opacity. The mean follow-up period was 20.2 ± 11.7 months (range from 9 months to 36 months). The IOL was well centered, all the wounds were well apposed and the mean postoperative intraocular pressure was 14.6 ± 1.95 mm Hg. No complications were reported in the entire follow-up period.ConclusionThe clinical outcomes of management of pseudophacocele were encouraging with retention of reasonably good visual potential in all cases.
Project description:Paraduodenal hernias account for ~53% of internal hernias due to embryological anomalies in intestinal rotation. While left paraduodenal hernias are the most common type, the anterior left variant is exceptionally rare. It involves herniation of the small intestine in front of the duodenum through a mesenteric defect. We present a case of a left anterior paraduodenal hernia diagnosed and surgically treated in our department, along with a discussion of its clinical and therapeutic aspects.
Project description:The cardiofaciocutaneous (CFC) syndrome is a condition of sporadic occurrence, with patients showing multiple congenital anomalies and mental retardation and characteristic dysmorphic features. We, thus, report a rare case of this syndrome in a 1-year-old child who presented with typical features of CFC syndrome.
Project description:Chronic posttraumatic diaphragmatic hernia is an unusual disease with challenging diagnosis and treatment. Surgery represents the treatment of choice which can be transabdominal, transthoracic, or combined approach. The principles of surgery consist of herniated visceral organs reduction and diaphragmatic defect closure. This video demonstrates the steps of chronic posttraumatic diaphragmatic hernia repair via a laparoscopic approach and concerning points during the operation.
Project description:The majority of tracheal tumors in adults are malignant. The finding of a benign tumor in the trachea is uncommon and endotracheal hamartomas are rare.We report two cases presenting within six months at our institution. The first patient is a 67 year-old man who was found to have an asymptomatic endotracheal hamartoma on chest imaging for aortic valve replacement. The second patient is a 46 year-old man with an extensive continued tobacco use disorder and a known endotracheal lesion identified 8 years prior to intervention. Both patients were treated surgically and recovered without complications.Identification of these lesions and timely management are necessary because without intervention, they can lead to fatal complications. Most symptoms of tracheal hamartoma result from mechanical obstruction with the earliest presenting symptom being dyspnea, but as evident in these two cases, they can have different presentations.We have found that endotracheal hamartoma has a tendency to present in Caucasian, male patients with a comorbidity of respiratory disease and variable smoking history, but it can also present in asymptomatic patients with no significant smoking history.
Project description:Lipomas are rare in the pediatric age group. A 10-month-old male child presented with an asymptomatic neck mass which was evaluated and excised completely. Histopathology was consistent with pleomorphic lipoma, not previously reported in children.
Project description:We report a rare coronary artery anomaly-anomalous origin of the left anterior descending artery from the pulmonary artery in a 40-year-old woman. The uniqueness of this case is the absence of any significant morbidity from this condition in adulthood which is in contrast to other reported cases where patients present with myocardial infarction, congestive heart failure, and sometimes death during the early infantile period.(1).
Project description:The supernumerary kidney especially on right side is a rare diagnosis. Only few case reports are documented in literature. We report a case of Right supernumerary kidney with partial fusion of right accessory kidney to upper kidney.