Purpose:To evaluate the features of superior mesenteric artery(SMA) lesions and SMA related diseases with a 64-detector CT scanner.Methods:The CT features of superior mesenteric artery changes in 26 patients were reviewed retrospectively.All patients received the uncontrasted and contrasted CT scanning,and multiplanar reformation and maximum intensity projection were made.Results:Ischemia of superior mesenteric artery was seen in 12 patients.SMA calcification,stenosis and thrombosis,and edema of intestine wall were shown on CT.SMA compression was seen in 10 patients,including 3 SMA syndromes with dilation of the primary and secondary part of duodenum and 7 nutcracker syndrome with the left renal vein compressed.Vessel changes were seen in the other 4 patients,including 2 arteriovenous malformations and 2 SMA pseudoaneurysms.Conclusion:64-detector helical CT is valuable in the diagnosis and the delineation of SMA lesions.
The deformity of the bilateral cleft lip is complicated. The routine procedure often results in secondary deformities, such as over-tight lip, and over-thin vermilion. The paper introduces a modified straight-line procedure that has been used in 16 patients with satisfactory results. This one-stage procedure employed a nasolabial flap and two small triangular flaps to lessen the tension of sutrue and lengthen the anterior lip as well as create a natural feature of the vermillion border. The method overcame imperfection of routine ones and achieved functional repair of the lip that would facilitate the development of the columella and anterior lip.
Objective: To summarize the clinical data of 15 patients with fibrodysplasia ossificans progressiva (FOP), follow up and analyze the characteristics of the joint involvement in FOP. Method: From May 2005 to December 2016, fifteen FOP cases had been diagnosed in the Children's Hospital Capital Institute of Pediatrics. All medical records and follow-up data were collected and a retrospective analysis was made on the joint involvement in FOP. Pearson correlation analysis was used for data, P<0.05 for the difference was statistically significant. Result: There were 8 males and 7 females in 15 cases. The age of onset was 2(1-6)years. The age at diagnosis was 6 (4-9) years. All cases had hallux valgus deformity and bone mass formation. Twelve cases had joints involvement on enrollment into this study: 8 cervical vertebra, 7 shoulder joint, 5 hip joint, 4 elbow joint, 3 wrist joint, 2 temporomandibular joint, 2 lumbar vertebra. The age of diagnosis and duration of disease were positively correlated with the number of the involved joints (r=0.523, 0.628; P=0.045, 0.012); mild changes were found in joint imaging. Thirteen cases received telephone follow-up, the average duration of follow-up was 6(3-7)years, no change in 11 cases, disease progress in 2 cases. Conclusion: Joint involvement is a common complication of FOP, especially the cervical vertebra.Multiple joints involvement, dominant functional impairment, and mild imaging changes are the characteristics of joint lesions caused by FOP.The number of involved joints gradually increases with increase of age of the patients and the prolonged course of the disease.目的： 总结临床诊断的15例进行性骨化性纤维发育不良(FOP)临床特点，分析FOP引起的关节病变特征。 方法： 收集2005年5月至2016年12月首都儿科研究所附属儿童医院住院并临床诊断的15例FOP患儿的病历资料，并进行电话随访，其中2例失访。将住院和随访资料进行病例观察性回顾性分析，采用Pearson相关性分析法进行相关因素分析。 结果： 15例患儿中，男8例，女7例，起病年龄2(1～6)岁，确诊年龄6(4～9)岁。15例患儿均有足拇趾的短小、外翻畸形及局部骨性包块形成的临床特征。入组时出现关节病变共12例，受累部位依次为：颈椎8例、肩关节7例、髋关节5例、肘关节4例、腕关节3例、下颌关节2例、腰椎2例。确诊年龄和确诊时病程与关节受累数目呈正相关(r＝0.523、0.628，P＝0.045、0.012)。关节影像学改变轻微。13例接受电话随访，随访时平均病程6(3～7)年。11例病情没有进一步进展，2例病情逐渐加重。3例应用药物治疗对关节病变均无效。 结论： FOP关节受累原因是异位骨化引起的关节融合。FOP引起关节病变的特点为多关节受累、功能障碍突出，而影像学改变轻。随着患儿年龄增长、病程延长，受累关节数目逐渐增加。.
Objective To investigate the features of minimal fat renal angiomyolipoma with sufficient blood supply by CT scan and to improve the diagnostic accuracy in differentiating it from clear cell renal carcinoma. Methods 24 cases of patients with postsurgery confirmed angiomyolipoma with sufficient blood supply(total of 25 tumors) in our hospital were retrospectively analyzed and were pathologically compared and studied. Results Among the 24 patients diagnosed with angiomyolipoma, there was no patients with bloody urine. Of the 96 patients diagnosed with clear cell renal cancer, 14 had bloody urine(14.6%). CT scan analysis showed 20 tumors were of similar density, and 5 of them were of higher density. Only one tumor had a few dots of calcification(4%). CT scan showed thaty adipose tissue was not visible in 9 tumors, while 16 tumors had visible dots of adipose tissue. Intensive scanning indicated that all of the tumors showed a strong enhancement in the renal corticomedullary phase. 20 tumors had significant heterogeneous enhancement in the early phase, while another 5 cases had homogenous prolonged enhancement. 19 patients had surgery to remove the angiomyolipomas, while 6 patients had single side kidney removal because of misdiagnosis for renal cancer and the renal parenchyma and sinus were severely compromised by tumors. 25 cases were classified as renal angiomyolipoma by pathological analysis. Within the 96 cases of clear cell renal cancer, 64 tumors were of relatively low density, 29 tumors of equal density, and 3 cases of relatively higher density. 14 of the tumors had calcification(14.6%), and none of them had visualized adipose tissue. Enhanced CT scans indicated that 69 cases of renal cancer showed significant enhancement in the renal corticomedullary phase, with the abnormal pattern of fast-in-and-fast-out. Besides, 27 cases had slow and prolonged enhancement. Conclusion The key to differentiating them is to identify the adipose tissue within the tumor. Therefore, it is helpful to use thin-layer CT scans to locate the adipose tissue.
Coronary artery fistulas (CAFs) are rare congenital heart defects that are typically managed through interventional closure, traditional surgery, or minimally invasive hybrid closure surgery. However, treating CAFs with complex anatomy, such as tortuous vessels, presents a significant challenge, particularly in young children. We report the case of a 3.8-year-old child (15 kg/100 cm) with a complex CAF, treated using a minimally invasive hybrid closure surgery approach with a 4 × 4 mm Amplatzer Duct Occluder II (ADO II) (Abbott, USA). Three-dimensional (3D) imaging was utilized to visualize the CAF's anatomy, guide the surgical planning, and accurately determine the puncture site on the right ventricular free wall, as well as the optimal sheath direction and insertion depth. The procedure was carried out efficiently and safely, guided by preoperative 3D imaging and intraoperative transesophageal echocardiography. Follow-up at one year demonstrated excellent outcomes with no complications.
Geographical variation of body color is widely present in reptile populations that survive in different substrate habitats, multiple potential mechanisms can account for this color variation.Phrynocephalus versicolor and P. frontalis, close genetic relatives, constitute a phylogenetic species group together with P. przewalskii.In this study, a fiber spectrophotometer (AvaSpec-2048) was used to record the skin luminous reflectivity of 12 sites across the lizard's body, and we quantitatively compared the natural color variation of dark P. versicolor and light P. frontalis that lived in "melanistic" and "non-melanistic" habitats, respectively.We aimed to determine whether the color variations of both populations were time reversible, and further discuss potential mechanisms that substrate color may have on color variation of Phrynocephalus lizards.Our results showed that the body color of P. versicolor in "melanistic" habitat was significantly darker than P. frontalis in the "non-melanistic" withered yellow habitat.We also conducted a reciprocal transplantation experiments (i.e."non-melanistic" withered yellow P. frontalis individuals were transplanted and fed in "melanistic" substrate environment, while "melanistic" P. versicolor individuals were transplanted and fed in withered yellow substrate environment).For "melanistic" P. versicolor, the skin reflectivity of six sites increased significantly after one week, while no significant changes were detected in other sites.For "non-melanistic" P. frontalis, except the skin reflectivity of two sites (left hind limb and top right on the
Objective
To analyze the prognosis of patients with pulmonary artery sling (PAS) combined with tracheal stenosis(TS) without tracheal intervention, and to discuss the method for improving the effect of treatment.
Methods
The clinical data of 17 children with PAS/TS (moderate or severe) who received treatment at Beijing Bayi Children′s Hospital Affiliated to General Hospital of Bejing Military Command from October 2011 to July 2015 were retrospectively analyzed, and the relationship between the effect and prognosis of re-implantation of the left pulmonary artery (LPA) without tracheal intervention was analyzed.
Results
Seventeen patients received re-implantation of the left pulmonary artery without tracheal intervention.Extubation was successfully performed in 12 of the 17 patients and they healed.The remaining 5 patients received tracheal intervention after the first operation but they all died.Of those 5 patients, 3 received tracheal stent implantation (1 died from necrotizing enterocolitis, 2 died from infection and multiple organ failure), and 2 received traheoplasty (both of them died from infection and tracheal fistula). Respiratory symptoms were reduced or resolved in all survivors.Diameter/length (%) in survivors without tracheal intervention was significantly higher than those who received tracheal intervention [(10.14±1.58)% vs.(5.72±1.17)%, t=3.600, P<0.001]. Patients with PAS undergoing LPA re-implantation achieved a good outcome if the diameter/length(%) of the trachea was up to (10.14±1.58)%.Patients with PAS undergoing LPA re-implantation achieved a bad outcome if the diameter/length(%) of the trachea was below to (5.72±1.17)%.
Conclusions
Most of patients with PAS/TS undergoing LPA re-implantation without tracheal intervention have a good outcome.It is feasible for them to avoid tracheal intervention.Diameter/length (%) may be a reliable indicator for determining tracheal intervention in surgical management of PAS.
Key words:
Pulmonary artery sling; Tracheal stenosis; Tracheoplasty; Prognosis
