RESUMEN
Congenital insensitivity to pain with anhidrosis (CIPA) is associated with Charcot arthropathy and is a rare clinical syndrome, with limited treatment options. Through a decade-long follow-up of a single case, we aim to provide new insights for clinicians regarding the choice of surgical strategies and postoperative complications. The diagnosed patient exhibited congenital insensitivity to pain and anhidrosis, accompanied by severe Charcot arthropathy affecting the spine. Multiple postoperative complications, including implant displacement, adjacent segment pathology, and pedicle screw loosening, occurred after surgical intervention, leading to five subsequent revision surgeries. Considering the limited experience in managing CIPA-related Charcot spinal arthropathy in the literature, surgical correction remains the preferred treatment. Among the 16 cases reviewed, common postoperative complications included implant displacement, adjacent segment pathology, and pedicle screw loosening. Based on current experience, we do not recommend extensive resection and reconstruction after removing the affected vertebral body, as this may increase the risk of implant displacement. Instead, a 360° long-segment fusion may help reduce the risk of adjacent segment degeneration. Additionally, we discuss potential reasons for revision surgery after Charcot spinal arthropathy surgery and perioperative management strategies for such cases. Meticulous care, appropriate rehabilitation exercises, and metabolic therapy for bone mineralization are crucial components of the treatment for this condition.
RESUMEN
A young female patient presented with fever, arthralgia, and rash was diagnosed with adults still's disease. When treated with glucocorticoid steroid, the above patient progressed to anuria, sudden, and confusion. After a teamwork involving different departments, the patient was finally diagnosed with atypical hemolytic uremic syndrome (aHUS) and treated with good outcome. aHUS is a rare disease, while Eculizumab is an orphan drug. The diagnosis and treatment of the patient reveals the importance of multidisciplinary team on the diagnosis and treatment of rare and difficult diseases.
RESUMEN
Objective:To explore the method and effect of endovascular treatment to innominate artery stenosis or occlusion.Methods:The data of 11 patients with stenosis or occlusion of innominate artery from January 2014 to November 2019 at Xuanwu Hospital of Capital Medical University were collected. All patients received endovascular treatment. We summarized the changes of clinical symptoms, surgical methods, perioperative complications, stent patency, and analyzed the changes in systolic blood pressure and peak blood flow velocity on the involving side.Results:All 11 patients underwent endovascular treatment. The surgical technique success rate was 100%. All patients were followed up. The follow-up time was 4-69 months, with an average of (30.1±23.4)months. 2 patients used cerebral umbrella during the operation. 1 patient was performed ipsilateral carotid endarterectomy, 1 patient underwent contralateral carotid stent implantation, 1 patient was diagnosed as severe stenosis of the innominate artery and left common carotid artery, and an innominate artery stent implantation was performed at one stage, left common carotid artery stent implantation was performed after half a year. We done operation from the femoral artery puncture approach (6 patients), brachial artery puncture approach (2 patients), axillary artery and femoral artery puncture approach (1 patients), and right common carotid artery and the femoral artery puncture approach (2 patients). 3 patients had in-stent restenosis at 6, 7and 12 months after stenting, respectively. 1 patient underwent balloon dilatation, and 2 patients underwent re-stent implantation. We have not do further intervention to 1 case of in-stent occlusion occurred 14 months after the stenting, for the clinical symptoms did not improve significantly. The clinical re-intervention rate in this group was 3/11, and the primary patency rate was 7/11. The secondary patency rate was 10/11. The symptoms of 10 patients were relieved and the weakness of right upper extremity was not significantly changed in 1 patient. No puncture point complications occurred in all patients, and no cerebral infarction occurred during the perioperative period. There were statistically significant differences in systolic blood pressure, blood pressure difference and peak blood flow velocity before and after the operation ( P<0.05). Conclusions:Endovascular treatment of innominate arterial stenosis or occlusion was safe and effective, and the appropriate surgical approach and plan should be selected according to the lesion characteristics and the whole body conditions.