We report an acute myocardial infarction patient with respiratory cardiac arrest in whom conventional cardiopulmonary resuscitation was invalid and extracorporeal cardiopulmonary resuscitation (ECMO) was performed. During ECMO, bloodstream infections caused septic shock. With the assistance of ultrasound and other techniques, cardiac function and volume status were evaluated, which provided a basis for making rational clinical decision. After shock resuscitation and anti-infection treatment, the patient's condition improved.

Objective

To explore the feasibility and efficacy of robot-assisted laparoscopic parastomal hernia repair （Sugarbaker）.

Methods

A retrospective analysis was conducted on the clinical data of 7 cases of robot-assisted laparoscopic parastomal hernia repair completed in Union Medicine Center in Tianjin from March 2024 to July 2024.The method of All patients repaired was Sugarbaker.Observe and record the surgical situation， including operation time， intraoperative complications （intraoperative hemorrhage， intestinal injury）， and the occurrence of robotic system failure； postoperative recovery，including postoperative water intake， eating time， postoperative exhaust and defecation time， pain level on the first day after surgery， and postoperative hospital stay.Follow up the post-discharge complications，including stoma infections， intestinal obstruction， and hernia recurrence.

Results

All seven patients successfully underwent surgery without intraoperative complications or robotic system malfunctions.The operative time ranged from 150 to 240 minutes， with an intraoperative blood loss of 10 to 50 ml， and no intraoperative transfusions were required.Postoperatively， all patients were allowed oral water intake at 6 hours after surgery， followed by a liquid diet on postoperative day 1.The time to first flatus ranged from 1 to 3 days， with an average of 1.57 days， while the time to first bowel movement was 2 to 4 days， averaging 2.86 days.Postoperative pain was mild， with Numeric Rating Scale （NRS） scores on postoperative day 1 ranging from 2 to 4， with a mean of 2.71.The postoperative hospital stay ranged from 4 to 5 days， with an average of 4.29 days.During the 1-to 4-month follow-up period， one patient developed postoperative intestinal obstruction， which resolved with conservative treatment.No cases of stoma infection or hernia recurrence were observed.

Conclusion

Robot-assisted laparoscopic parastomal hernia repair is safe and effective， with a relatively low complication rate and short term recurrence rate， but the long-term effect needs to be further observed.

本案例为北京大学人民医院2023 年2 月收治的1 例巨大食管裂孔疝合并胃扭转患者，出现喘憋症状且营养状态差，践行损伤控制外科理念，先行一期胃镜-腹腔镜“双镜联合”松解粘连并置入空肠营养管后，纠正喘憋症状改善营养状态，再行二期腹腔镜食管裂孔疝修补及胃底折叠术。术后7 d 拔除胃空肠营养管，恢复经口进食。术后1 年随访，原症状消失，未见复发及其他并发症。本案例所用的治疗理念和方案可以为巨大食管裂孔疝合并胃扭转患者的治疗提供新的参考。

Objective

Retroperitoneal extrarenal or pelvic epithelioid angiomyolipomas are clinically rare, and some of them show malignant biological behavior.Due to the lack of specific symptoms and imaging findings, patients often experience a delayed diagnosis and treatment.This study aimed to improve the understanding of this disease by summarizing our diagnosis and treatment experience with 14 cases and reviewing the literature.

Methods

The clinical data of 14 patients with retroperitoneal extrarenal or pelvic epithelioid angiomyolipoma who underwent surgical treatment and achieved pathological confirmation in Peking University International Hospital from January 2014 to April 2024 were retrospectively analyzed.The clinical manifestations, imaging and pathological features, diagnosis, and treatment of the disease were summarized based on the domestic and foreign literature.

Results

There were 4 males and 10 females in this group.The patients ranged in age from 20 to 67 years at presentation, with a median age of 39 years.There were 12 cases of initial onset (4 cases discovered in physical examination, 5 cases of abdominal discomfort, 2 cases of low back pain, 1 case of constipation) and 2 cases of recurrence.The lesions were located in retroperitoneal region in 11 cases and pelvic cavity in 3 cases.Plain CT scans showed slightly lower or equal density, and the tumor could have internal bleeding and necrosis when it was large.The solid component showed uneven and obvious enhancement.All patients in this group underwent open surgery.Postoperative pathology indicated malignancy in 10 cases.Immunohistochemical staining was positive for HMB45 in 85.7% (12/14) of patients, for MelanA in 64.3% (9/14), and for SMA in 91.7%(11/12).Postoperative complications in 2 cases (abdominal hemorrhage in 1 case and wound infection in 1 case) improved after conservative treatment.The patients underwent regular follow-up after discharge.As of July 2024, the follow-up time ranged from 4～88 months, with a median time of 35 months.Two cases were lost to follow-up.One case (case 13) was confirmed to have pelvic tumor recurrence 5 months after surgery(being on oral sirolimus treatment now).One case (case 5) had no significant changes in residual tumor in abdominal aortic wall, and no tumor recurrence or metastasis was observed in the remaining patients.

Conclusion

Extraperitoneal extrarenal or pelvic epithelioid angiomyolipomas are most common in young and middle-aged women.The clinical manifestations of the disease are atypical.The low diagnostic accuracy of imaging examination easily leads to confusion with liposarcoma and vasogenic tumor.Diagnosis of the disease depends on postoperative pathology and immunohistochemistry.The specific simultaneous expression of melanin markers (HMB45 and Melan-A) and myogenic markers (SMA) is characteristic.Surgical resection is the preferred treatment option, but mTOR inhibitors are promising for the treatment of the disease.Some patients present with malignant neoplastic features.Tumor recurrence and distant metastasis can still occur in these patients even after surgical treatment, so regular follow-up observation is required after surgery.

Objective

To investigate the clinical manifestations, diagnosis, pathological features,treatment and prognosis of retroperitoneal synovial sarcoma, and to improve surgeons' understanding of retroperitoneal synovial sarcoma.

Methods

Retrospective analysis was made on the clinical data of 7 cases of retroperitioneal synovial sarcoma patients undergoing surgical resection in Peking University International Hospital from January 2015 to January 2024,and to do the follow-up visit.

Results

The clinical manifestations of the patients were not typical, most of them were low back pain (2 cases) and abdominal pain (4 cases).The characteristic findings of CT were that the tumor was lobed or lumpy soft tissue mass,the density was slightly lower than the muscle density, and necrosis area was visible in the tumor body.The enhanced scan showed uniform or uneven enhancement, and sometimes calcification was seen at the edge of the mass.All patients in this group underwent surgical treatment, and the tumors were often larger and invaded the surrounding organs.Postoperative complications occurred in 2 cases (gastroparesis 1, intestinal basket 1),and perioperative death occurred in 1 case (respiratory failure).SS18 gene rearrangement was present in all 7 patients.All patients were followed up until March 2024 and died of the disease,median survival was 56 months.

Conclusion

The clinical manifestations of patients with synovial sarcoma are nonspecific, and the characteristic chromosomal translocation is the gold standard for diagnosis.Surgical resection is still the main treatment.This kind of tumor has high malignant degree, easy recurrence after operation and poor prognosis.

Objective

To summarize our clinical experience with Pseudomonas aeruginosa injection in the treatment of refractory high-output chyle leakage in the neck.

Methods

In this study, we retrospectively analyzed the clinical data of two patients with refractory high-output chyle leakage after cervical lymph node dissection for thyroid cancer admitted to Cancer Hospital & Shenzheng Hospital,Chinese Academy of Medical Sciences, of which 1 was female and 1 was male.Both patients underwent primary surgery.Both patients were considered to have chyle leakage caused by the rupture of the thoracic duct at the left neck, and the maximum daily drainage volume exceeded 1000 ml.

Results

The patients had poor response to conservative therapy, and both of them received retrograde injection of Pseudomonas aeruginosa injection through the left cervical drain, and were finally discharged from the hospital without serious complications and sequelae.

Conclusion

Pseudomonas aeruginosa injection in the operative area is effective and safe in the treatment of refractory high-output chyle leakage in the neck, and it can significantly shorten the duration of chyle leakage.

Objective

To investigate the clinical and genetic feasures of children with neurodegeneration with brain iron accumulation.

Methods

A retrospective analysis was conducted on the clinical manifestations and imaging data of six children with neurodegeneration with brain iron accumulation who were treated at the First Affiliated Hospital of Xinjiang Medical University from March 2021 to June 2024.Gene sequencing was performed on the children and their families using whole exome technology.

Results

Among the six pediatric patients, four were girls and two were boys, with ages ranging from 1 year and 7 months to 6 years old.All patients presented with comprehensive developmental delay clinically,and two patients showed significant developmental regression.Among them, one patient had myasthenia gravis (ocular muscle type).Gene sequencing showed that four children had WDR45 gene mutations,namely, c.519+1_519+3del c.381del, c.984C＞G, and c.400C＞T.After Sanger sequencing verification,all mutations were found to be de novo.Two cases had PLA2G6 gene mutations, with one case carrying both c.1547_1548dupCG (repeat mutation) and c.1799G＞A gene mutations, and the other case carrying c.1002dupA mutation.

Conclusion

Neurodegeneration with brain iron accumulation has clinical and genetic diversity.Whole exome gene sequencing, combined with medical history and MRI, can help with early diagnosis and guide treatment.The detection of new mutation sites broadens the spectrum of gene variation.

Objective

The factors influencing the success rate of mature permanent tooth autotransplantation combined with external apical surgery were analyzed to provide reference for clinical judgment of success rate.

Methods

Patients who received autologous tooth transplantation of mature permanent teeth and combined external apical surgery in the Department of Oral Surgery of Stomatology，Hospital of the Fourth Military Medical University from June 2014 to May 2023 were included.The reasons for the external apical surgery were as follows.Firstly，the root apex of the donor tooth was broken passively during extraction.Secondly，the root apex of the donor tooth was too crooked to implant into the recipient area.The possible factors affecting the success rate were summarized and evaluated.Surgical records and photography data from patients' previous medical records，including 12 factors such as gender，age，root shape and donor location，were analyzed.Survival analysis was used for statistical analysis to screen out the factors affecting the cumulative success rate of transplanted teeth after external apical surgery.

Results

The cumulative five-year success rate of 84 transplanted teeth was 77.2%.Single factor Log-Rank analysis showed that the location of the receiving area（P=0.004），the alveolar bone height of the receiving area（P=0.002），the donor time（P=0.005）and the initial stability（P=0.001）were significantly correlated with the cumulative success rate of tooth transplantation after external apical surgery.Multivariate Cox proportional hazard regression analysis showed that the location of the receiving area（P=0.039）and the height of the alveolar bone in the receiving area（P=0.036）were significantly correlated with the success rate of transplantation after external apical surgery.

Conclusions

By reducing the intraoperative time，restoring the alveolar bone height，and ensuring good initial stability，high success rate can be achieved for mature permanent tooth autotransplantation after external apical surgery.

Skeletal class Ⅲmalocclusion，manifested by excessive mandibular growth and/or hypoplasia of the maxilla，is typified by molar mesial occlusion，anterior crossbite，pronounced classⅢskeletal morphology，and mandibular prognathism，either with or without functional crossbite.This condition exerts a profound impact on oral functionality，facial esthetics，and psychological health，posing a significant therapeutic challenge that frequently necessitates orthognathic surgery.This case report presents an adult patient with skeletal class Ⅲmalocclusion who was treated with non-surgical，nonmicroimplant orthodontic camouflage therapy.The etiology is scrutinized，and the diagnostic process，differential diagnoses，and preventive strategies are deliberated to provide clinical practitioners with insights into effective management strategies.