Progress in the Research of the Spinal Deformity Related to Marfan Syndrome / 罕见病研究

Marfan syndrome(MFS) is an autosomal dominant systemic connective tissue disease. The incidence rate of MFS is about 2-3 per 10 000. Main cause of MFS is FBN1 gene mutation. About 2/3 of MFS patients have spinal deformities, showing symptoms of scoliosis, thoracic lordosis and lumbar kyphosis, severe spondylolisthesis, dural dilatation and pedicle dystrophy. MFS scoliosis develops with age and may continue even after bone maturation. Conservative treatments such as brace are usually ineffective. Surgical treatment of main curve > 40°-45 °is recommended, but due to the special anatomical structure of MFS patients, such surgical complications as dural leakage, failure of internal fixation and revision surgery are not uncommon.

Application of a new point contact pedicle navigation template as an auxiliary screw implant in scoliosis correction surgery / 中国修复重建外科杂志

OBJECTIVE@#To explore the effectiveness of a new point contact pedicle navigation template (referred to as "new navigation template" for simplicity) in assisting screw implantation in scoliosis correction surgery.@*METHODS@#Twenty-five patients with scoliosis, who met the selection criteria between February 2020 and February 2023, were selected as the trial group. During the scoliosis correction surgery, the three-dimensional printed new navigation template was used to assist in screw implantation. Fifty patients who had undergone screw implantation with traditional free-hand implantation technique between February 2019 and February 2023 were matched according to the inclusion and exclusion criteria as the control group. There was no significant difference between the two groups ( P>0.05) in terms of gender, age, disease duration, Cobb angle on the coronal plane of the main curve, Cobb angle at the Bending position of the main curve, the position of the apical vertebrae of the main curve, and the number of vertebrae with the pedicle diameter lower than 50%/75% of the national average, and the number of patients whose apical vertebrae rotation exceeded 40°. The number of fused vertebrae, the number of pedicle screws, the time of pedicle screw implantation, implant bleeding, fluoroscopy frequency, and manual diversion frequency were compared between the two groups. The occurrence of implant complications was observed. Based on the X-ray films at 2 weeks after operation, the pedicle screw grading was recorded, the accuracy of the implant and the main curvature correction rate were calculated.@*RESULTS@#Both groups successfully completed the surgeries. Among them, the trial group implanted 267 screws and fused 177 vertebrae; the control group implanted 523 screws and fused 358 vertebrae. There was no significant difference between the two groups ( P>0.05) in terms of the number of fused vertebrae, the number of pedicle screws, the pedicle screw grading and accuracy, and the main curvature correction rate. However, the time of pedicle screw implantation, implant bleeding, fluoroscopy frequency, and manual diversion frequency were significantly lower in trial group than in control group ( P<0.05). There was no complications related to screws implantation during or after operation in the two groups.@*CONCLUSION@#The new navigation template is suitable for all kinds of deformed vertebral lamina and articular process, which not only improves the accuracy of screw implantation, but also reduces the difficulty of operation, shortens the operation time, and reduces intraoperative bleeding.

Clinical study of concealed penis correction surgery based on principle of midline symmetry / 中国修复重建外科杂志

Objective: To investigate the effectiveness of concealed penis correction surgery based on the principle of midline symmetry. Methods: Between January 2016 and September 2018, 18 children with concealed penis were treated with correction surgery based on the principle of midline symmetry. All children were 3-12 years old, with an average age of 8.3 years. Physical examination showed that the penis was short; the penis body could not be exposed or be exposed too limited; the corpus cavernosum developed well. The pressure dressing was removed at 3 days after operation and the urethral tube was removed. The color of the glans, the swelling and congestion of penis and scrotum, and the blood supple of the prepuce flap were observed. Results: The operation time ranged from 47 to 54 minutes, with an average of 50 minutes. All children were followed up 3 months after operation. There was no hemorrhage and necrosis of the glans and no infection or ischemic necrosis of the flap. All patients had different degree of prepuce edema at 3 days after operation, 5 patients still had prepuce edema at 2 weeks, and the prepuce edema in all patients subsided at 3 months. All penises were exposed well after midline symmetric anastomosis with no bulky prepuce and scrotum. Conclusion: The correction surgery based on the principle of midline symmetry can be used to correct the appearance of the concealed penis effectively.

Clinical efficacy of short-term halo-pelvic traction combined with surgery in the treatment of severe spinal deformities / 北京大学学报(医学版)

OBJECTIVE@#To evaluate the clinical efficacy of short-term halo-pelvic traction (HPT) combined with surgery in the treatment of severe spinal deformities.@*METHODS@#In the study, 24 patients diagnosed as severe spinal deformity accepted the treatment of one-stage short-term HPT and two-stage surgery from January 2015 to May 2018 in our orthopedics department. 24 cases (9 males and 15 females) were retrospectively reviewed. The average age of the cohort was (28.8±10.0) years (12－48 years). The height, scoliosis angle, kyphosis angle, the height difference of shoulders, the height difference of crista iliaca, C7PL-CSVL and the perpendicular distance of S1 and the convex point of the patients were assessed at pre-traction, post-traction and post-surgery. The paired t test was used to analyze the difference among pre-traction, post-traction and post-surgery.@*RESULTS@#The average traction time of 24 cases was (2.5±1.1) weeks (1－5 weeks). The height of pre-traction and post-traction were (141.7±11.2) cm (116-167 cm) and (154.1±9.5) cm (136－176 cm) respectively, showing significant difference (P < 0.05), and the increased height was (12.4±4.6) cm (4－20 cm). The average scoliosis angle before traction was 104.9°±35.0°(25°－158°), and it was significantly decreased in post-traction[64.8°±21.0°(19°－92°)] and post-surgery[39.3°±17.0° (10°-70°)] (P < 0.05). The traction's coronal correction rate was 37.2%±10.9% (11.9%－51.2%) and the total coronal correction rate was 61.9%±12.6%(26.9%-79.0%). The average kyphosis angle before traction was 106.9°±29.2°(54°－163°), and it was significantly decreased in post-traction [63.1°±17.1°(32°－92°)] and post-surgery [39.0°±16.8°(10°－68°)](P < 0.05). The traction's sagittal correction rate was 40.0%±10.7%(16.7%－55.5%) and the total sagittal correction rate was 64.3%±10.7%(49.0%－87.5%). The average C7PL-CSVL before traction was (3.2±2.8) cm, and it was significantly decreased in post-traction [(2.5±2.5) cm] (P < 0.05). The perpendicular distance of S1 and the convex point before traction was (10.5±4.8) cm, and it was significantly decreased in post-traction[(8.4±3.5) cm] (P < 0.05).@*CONCLUSION@#The one-stage short-term HPT combined with two-stage surgery is a safe and effective procedure for severe spinal deformities. The clinical efficacy is satisfactory and the complication is relatively less.

Long-term survival of bilateral lung transplantation combined with Nuss operation on treatment of bronchiolitis obliterans syndrome complicated with thoracic deformity after hematopoietic stem cell transplantation: report of one case / 器官移植

Objective To evaluate the clinical efficacy of bilateral lung transplantation combined with Nuss operation on the treatment of bronchiolitis obliterans syndrome (BOS) complicated with pectus excavatum after hematopoietic stem cell transplantation. Methods On March 24, 2015, one patient presenting with BOS complicated with pectus excavatum after hematopoietic stem cell transplantation underwent extracorporeal membrane oxygenation (ECMO)-assisted bilateral lung transplantation in the Department of Thoracic Surgery and Lung Transplantation Center of Wuxi People's Hospital affiliated to Nanjing Medical University. Simutaneously, Nuss operation was successfully performed to treat pectus excavatum. After anti-inflammation and anti-fungal treatment, a triple immunosuppressive regimen consisting of tacrolimus + mycophenolate mofetil + adrenocortical hormone was implemented. Results The patient was properly recovered and discharged on 25 d after operation. The Nuss steel plate was removed from the chest at postoperative 2 years. The thoracic deformity was corrected. The patient received long-term postoperative follow-up. Until submission date, the patient survived for more than 3 years. The lung function was well restored and the quality of life was satisfactory. Conclusions Bilateral lung transplantation combined with Nuss operation is an effective approach in the treatment of end-stage BOS complicated thoracic deformity.

Tubo gástrico isoperistáltico no tratamento paliativo do carcinoma irressecável do esôfago / Gastro-esophageal isoperistaltic bypass as palliative treatment of the irresectable esophageal cancer

A meta do tratamento dos pacientes portadores de carcinoma avançado de esôfago é o alívio da disfagia. OBJETIVO: O objetivo deste trabalho é relatar a experiência dos autores na utilização da derivação esofagogástrica através de um tubo gástrico isoperistáltico seguido de radioterapia, no tratamento de doentes portadores de carcinoma irressecável do esôfago. MÉTODO: No período de 1990 a 1999, 30 pacientes foram submetidos à cirurgia de derivação. Vinte e quatro doentes (80 por cento) eram do sexo masculino e seis (20 por cento) do feminino; a idade variou de 27 a 69 anos, média de 49,3 anos. Em todos os casos o diagnóstico foi confirmado por endoscopia digestiva alta e biópsia. Aqueles com tumores maiores que 6cm ao esofagograma ou com sinais de invasão da árvore respiratória à broncoscopia foram considerados irressecáveis. Após avaliação clínica e preparo pré-operatório foram submetidos à operação de derivação. O ato operatório foi realizado por duas equipes, uma na região cervical e outra na abdominal e a duração da intervenção variou entre três a quatro horas. Após a alta hospitalar os pacientes foram encaminhados para a radioterapia. RESULTADOS: Não houve óbito operatório. A mortalidade pós-operatória foi de 10 por cento, um caso de tromboembolismo pulmonar e dois de broncopneumonia. Treze pacientes (43,3 por cento) desenvolveram fístula cervical e em 11 ocorreu o fechamento espontâneo da fístula; um caso necessitou de reoperação e outro veio a falecer no 14º dia pós-operatório com a fístula aberta. Oito pacientes (26,6 por cento) apresentaram estenose da anastomose esôfago-tubo; todos evoluíram bem com dilatação endoscópica. A deglutição foi restabelecida em todos os pacientes até o momento do óbito, excetuando aqueles que faleceram em virtude de complicações pós-operatórias (três casos). O tempo de internação variou de 12 a 45 dias e a sobrevida média foi de 7,9 meses. CONCLUSÕES: Os autores concluíram que nos pacientes portadores...

The goal of the treatment in patients with advanced esophageal carcinoma is to alleviate dysphagia. BACKGROUND: The objective of this study is to report the authors' experience with gastric esophageal bypass using isoperistaltic gastric tube followed by radiotherapy, in the treatment of patients with unresectable esophageal carcinoma. METHOD: From 1990 to 1999, 30 patients underwent bypass surgery. Twenty four patients (80 percent) were male and six (20 percent) female, age ranged from 27 to 69 years, with a mean age of 49.3 years. Diagnosis was confirmed by esophagogastroduodenoscopy and biopsy in all patients. Those with tumors greater than 6 cm at esophagography or with signs of invasion of the respiratory sistem at bronchoscopy were considered unresectable. Bypass surgery was carried out after medical assessment and preoperative evaluation. Surgery was performed by two teams, one at the cervical area and another at the abdominal area. Surgical time ranged from 3 to 4 hours. After hospital discharge, patients were referred to radiotherapy. RESULTS: There was no operative mortality. Postoperative mortality was 10 percent, one case of pulmonary thromboembolism and two cases of pneumonia. Thirteen patients (43.3 percent) developed cervical anastomotic leak and eleven had spontaneous resolution; one required a new operation and another patient died at postoperative day 14, with an open fistula. Eight patients (26.6 percent) had anastomotic stenosis, and all of them had a good outcome with endoscopic dilation. Deglutition was reestablished in all patients until death, excluding those who died as a result of postoperative complications (3 cases). Hospitalization time ranged from 12 to 45 days and mean survival rate was 7.9 months. CONCLUSIONS: We concluded that, in patients with esophageal unresectable carcinoma, obstruction bypass using an isoperistaltic gastric tube alleviates dysphagia symptoms until death. Morbidity is high, even though...