Nationwide questionnaire survey on pediatric pancreatic tumors in Japan.

BACKGROUND: Pediatric pancreatic tumors are rare and account for < 0.1% of all childhood cancers. The primary treatment for pancreatic tumors is surgical resection. However, because of the lack of knowledge regarding pediatric pancreatic tumors, no comprehensive treatment plans for pediatric pancreatic tumors have been developed. AIM: To compared the clinical features, treatment methods, and prognosis of pediatric pancreatic tumors in Japan with those in other countries. METHODS: Questionnaires were sent to 213 pediatric surgical units in Japan. Pancreatic tumors that were not surgically treated were excluded from the survey. The primary survey investigated the number of patients aged 0-18 years who underwent pancreatic tumor surgery and the type of tumors managed during the 22-year study period (from January 1, 2000 to December 31, 2021) by post card. The secondary survey assessed the clinical images, treatment methods, and tumor outcomes via email. RESULTS: The primary survey enrolled 228 patients. In the secondary survey, 213 patients were eventually enrolled. The most common type of pancreatic tumor was solid pseudopapillary neoplasm (SPN) [n = 164 (77.0%)], followed by pancreatoblastoma [n = 16 (7.5%)], pancreatic endocrine tumor [n = 14 (6.6%)], non-epithelial tumor [n = 9 (4.2%)], pancreatic tumor [n = 7 (3.3%)], and metastatic pancreatic tumor [n = 3 (1.4%)]. Overall, 123 (57.7%) patients underwent distal pancreatectomy, of whom 49 underwent laparoscopic surgery. Forty-four (20.7%) patients underwent enucleation, of whom eight underwent laparoscopic surgery. Thirty-two (15.0%) patients underwent pancreaticoduodenectomy, of whom one underwent laparoscopic surgery. All patients with SPN, including those with distant metastases and recurrent disease, survived. CONCLUSION: SPN was more common in Japan than in other countries. Regardless of the histological type, resection is the most effective treatment for pediatric pancreatic tumors.

Congenital Risk Factors for Chronic Kidney Disease in Patients With Persistent Cloaca: Results From a Nationwide Survey in Japan.

BACKGROUND: We conducted a nationwide survey of persistent cloaca (PC) to investigate the renal function outcomes and factors affecting chronic kidney disease (CKD) in patients with PC. METHOD: Information from 466 patients with PC was obtained via a questionnaire in this study. The 290 patients (62.2%) with renal function data were classified into 2 groups based on their estimated glomerular filtration rate: advanced CKD group (<30 mL/min/1.73 m2 [or post-renal replacement therapy]) and non-advanced CKD group (≥30 mL/min/1.73 m2). Univariate and multivariate analyses were performed to identify risk factors for CKD that may affect the renal function, including renal and urinary tract malformations, associated anomalies, and urinary tract treatment. The advanced CKD group was divided into two groups based on age to evaluate age-related differences (younger- and older-age CKD groups). RESULTS: A regression analysis revealed that congenital renal malformations (odds ratio [OR]: 14.06, 95% confidence interval [CI]:3.07-131.65, p < 0.0001), urinary tract obstruction (OR:4.28, 95%CI:1.12-24.23, p < 0.05), and sacral agenesis (OR:4.54, 95% CI:0.84-30.67, p < 0.05) were significantly associated with advanced CKD. In the univariate analysis of factors affecting the renal prognosis, clean intermittent catheterization (CIC) (OR:4.18, 95%CI:1.21-16.45, p = 0.015), vesicostomy (OR:3.65, 95%CI:1.11-12.98, p = 0.019), and surgery for vesicoureteral reflux (OR:5.43, 95%CI:1.41-22.73, p = 0.006) were significantly associated with advanced CKD. Based on the univariate analysis, hydrometrocolpos was significantly more prevalent in the older-age CKD group compared to the younger-age CKD group (p < 0.05). CONCLUSION: CKD development in patients with PC is influenced by a complex interplay of factors, including renal malformations and neurogenic bladder dysfunction due to spinal anomalies. LEVEL OF EVIDENCE: III (Study of Diagnostic Test, Study of nonconsecutive patients, and/or without a universally applied "gold" standard).

The implementation status of prehabilitation during neoadjuvant chemotherapy for patients with locally advanced esophageal cancer: a questionnaire survey to the board-certified facilities in Japan.

BACKGROUND: Prehabilitation during neoadjuvant therapy has the potential to improve clinical outcomes. However, information on its global dissemination status is limited. This Japanese nationwide survey investigated the implementation status of and barriers to prehabilitation during neoadjuvant chemotherapy (NAC) for patients with locally advanced esophageal cancer in hospitals. METHODS: This multicenter nationwide survey was conducted by post. The eligible facilities were 155 Japanese hospitals that had been certified within the last 10 years as authorized institutes for board-certified esophageal surgeons by the Japan Esophageal Society. We administered an original questionnaire to investigate the current status of prehabilitation during NAC. RESULTS: The response rate was 75% (117/155 facilities). Forty-six facilities (39%) provided prehabilitation during NAC. The most frequently selected reasons for not providing or providing insufficient prehabilitation were lack of human resources, issues with the reimbursement of medical fees, difficulty in providing continuous prehabilitation during repeated inpatient and outpatient care, the lack of established standard prehabilitation programs, challenges in providing multidisciplinary prehabilitation, and difficulty in managing physical symptoms. CONCLUSION: We observed that the implementation rate of prehabilitation during NAC was low. Critical reasons were not only the lack of medical resources but also the lack of evidence-based standard prehabilitation programs during NAC and the lack of evidence for how to continuously deliver prehabilitation during NAC to patients with physical symptoms.

Assessing outcomes and complications of secondary hepatolithiasis after choledochoenterostomy: A nationwide survey in Japan.

BACKGROUND: This study aimed to evaluate the outcomes and complications of secondary hepatolithiasis following choledochoenterostomy to guide suitable management. METHODS: The study analyzed 127 patients from a 2017 national survey conducted by the Ministry of Health, Labor, and Welfare. The 2023 cohort study assessed residual stones, recurrences, cholangitis, cholangiocarcinoma, and prognosis. RESULTS: The median follow-up duration was 48 months. Balloon endoscopy-assisted endoscopic retrograde cholangiography (BE-ERC) was the most common treatment, achieving complete stone clearance in 84.4% of patients. Anatomical hepatectomy was the most common surgery. Predictors of residual stones were stone number ≥10 (odds ratio [OR], 7.480; p = .028) and stone diameter ≥10 mm (OR, 5.280; p = .020). Predictors of stone recurrence during follow-up were biliary strictures (hazard ratio [HR], 3.580; p = .005) and cholangitis (HR, 2.700; p = .037). Predictors of cholangitis during follow-up were biliary stricture (HR, 5.016; p = .006) and dilatation (HR, 3.560; p = .029). Any treatment for hepatolithiasis reduced cholangitis occurrence (HR, 0.168; p = .042). Balloon dilation combined with stenting for ≥3 months improved biliary strictures in 57.1% of patients. CONCLUSION: This study recommends BE-ERC as the first-choice treatment for secondary hepatolithiasis. Stone removal and relief of biliary strictures and dilatation are crucial to prevent stone recurrence and cholangitis after treatment.

Current status of the surgical training system in Japan: results of a nationwide questionnaire survey of graduating surgical trainees.

PURPOSE: To identify the problems trainees face during surgical training in Japan. METHODS: A nationwide online questionnaire survey was conducted targeting newly certified surgical trainees. RESULTS: The response rate was 53.8% (758/1410). Among those respondents, 25.6% were women, 71.4% were either married or had a partner, 41.3% had children, 72.7% had performed over 200 surgeries under general anesthesia, and 54.1% had chosen, before graduating from medical school, to become a surgeon. While 88.8% were interested in learning surgical techniques, 63.8% were hesitant to become a surgeon for fear of a compromised quality of private life (QOL). Conversely, only 1.4% chose their surgical training programs based on QOL. Overall, 84.6% of the trainees were satisfied with their training and this correlated with the number of surgeries performed. Only 29.9% received non-technical skill training. The average number of night shifts per month was 5.6, and 10.6% worked over 80 h per week. Harassment was reported by 41.5% of the respondents. Moreover, 33.0% had considered dropping out at some time, primarily because of their QOL (51.1%) or the harassment they had encountered (50.4%). CONCLUSION: This survey revealed that while trainees were satisfied with the overall training system, issues such as long working hours and harassment are prevalent. Working to improve these issues could make surgery more attractive for young trainees.

A Comparative Study of Postoperative Complications Associated with Distal Gastrectomy and Pylorus-Preserving Gastrectomy among Gastric Cancer Patients Based on Nationwide Survey Data and Propensity Score Weighting.

BACKGROUND/OBJECTIVE: This study aimed to compare complication rates between pylorus-preserving gastrectomy (PPG) and distal gastrectomy (DG) using Korean nationwide survey data and propensity score weighting (PSW). PPG preserves gastric function but may lead to more postoperative complications than DG. METHODS AND RESULTS: We analyzed 9424 gastric cancer patients who underwent either DG (n = 9183) or PPG (n = 241). PSW balanced variables such as age, sex, TNM stage, comorbidities, ASA score, and surgical approach. Before PSW, 87.8% of DG patients and 87.1% of PPG patients had no complications (p = 0.053). Severe complications (Clavien-Dindo IIIa or higher) were more frequent in PPG (6.6%) than in DG (3.8%) (p = 0.039). After PSW, overall complication rates (p = 0.960) and severe complication rates (p = 0.574) were similar between groups. Incidence rates of anastomotic stricture and leakage were higher in PPG (2.9% and 1.7%) compared to DG (0.6% and 0.5%) (p = 0.001 and 0.036) before PSW, but these differences were not significant after PSW (p = 0.999 and 0.123). CONCLUSION: The PSW-adjusted analysis indicates no significant difference in overall and severe complication rates between PPG and DG in gastric cancer patients.

Prognostic factors of idiopathic hypereosinophilic syndrome: A nationwide survey in Japan.

Idiopathic hypereosinophilic syndrome (iHES) is a condition wherein persistent hypereosinophilia associated with end-organ damage occurs without any known causes. Due to the rarity of the disease, insufficient knowledge has been accumulated. We therefore conducted a retrospective, multicentre, nationwide survey on iHES in Japan. A total of 57 patients were identified. For 43 patients who received any treatment, all cases were first treated with corticosteroids. An eosinophil percentage of less than 30% in the bone marrow and the absence of oedema were identified as factors associated with steroid dependency. The 5-year overall survival was 88.2%, and five patients died during follow-up; factors associated with worse overall survival were age >50, haemoglobin <12 g/dL, activated partial thromboplastin time >34 s, the presence of dyspnoea, the presence of thrombotic tendency and the presence of renal failure. Given the rarity of fatalities in our cohort, time-to-next-treatment (TTNT) was further analysed; the presence of renal failure, splenomegaly and lung abnormalities were associated with worse TTNT. Our nationwide study not only demonstrated clinical characteristics and the outcome of patients with iHES but also for the first time revealed clinical factors associated with steroid dependency and duration of first-line corticosteroid efficacy.

Significance of definitive concurrent chemoradiotherapy for vulvar cancer: a Japanese Gynecologic Oncology Group nationwide survey study.

OBJECTIVE: This study aimed to show the results of radical radiation therapy (RT) and concurrent chemoradiotherapy (CCRT) for vulvar cancer (VC) based on data from a Japanese nationwide survey. MATERIALS AND METHODS: We collected data from 108 institutions on cases of VC diagnosed between January 2001 and December 2010. Patients with histologically proven squamous cell carcinoma and adenocarcinoma with curative intent were selected, and 172 patients with VC were included in this study. The collected data were analyzed for overall survival (OS) using the Kaplan-Meier method. Univariate and multivariate analyses were performed to examine the prognostic factors for patients with VC. RESULTS: The median follow-up period was 16.8 (range; 3.2-154.8) months. Fifty-five patients received CCRT, and 117 patients received RT alone. The 2-year OS rates (95% confidence interval [CI]) for stages I, II, III, and IV were 77.9% (55.8-100.0), 71.9% (53.8-89.9), 55.4% (42.5-68.3), and 41.5% (27.3-55.7) respectively. Univariate analyses showed that the FIGO stage (p = 0.001), tumor diameter (p = 0.005), and lymph node (LN) status (p = 0.001) were associated with OS. The concurrent use of chemotherapy resulted in a significantly longer OS in Stage III (p = 0.013). Multivariate analysis showed that the hazard ratios (95% CI) for tumor diameter, positivity for LN metastasis, and RT alone (no concurrent chemotherapy) were 1.502 (1.116-2.021), 1.801 (1.287-2.521), and 1.936 (1.187-3.159), respectively. CONCLUSIONS: Our analysis revealed that CCRT should be recommended, especially for Stage III VC patients. Further studies are warranted to determine who benefits from CCRT, considering primary tumor size and LN status. The study was registered at the University Hospital Medical Information Network (protocol number: UMIN000017080) on April 8th, 2015.

Etiological changes of liver cirrhosis and hepatocellular carcinoma-complicated liver cirrhosis in Japan: Updated nationwide survey from 2018 to 2021.

AIM: A nationwide survey in 2018 showed decreasing involvement of viral hepatitis and increasing involvement of nonviral liver diseases in the etiology of liver cirrhosis (LC) in Japan. An updated nationwide survey was undertaken in 2023. METHODS: Cases of LC diagnosed between 2018 and 2021 were collected from 75 institutions, and the etiologies of LC were investigated. In addition, the data obtained were compared with the results of previous studies. RESULTS: Among the 15 517 cases, alcohol-related liver disease (ALD)-associated LC was the most frequent cause (n = 5,487, 35.4%). Hepatitis C virus-associated LC, nonalcoholic steatohepatitis (NASH)-associated LC, and hepatitis B virus-associated LC were ranked as second, third, and fourth, respectively. In comparison to the previous survey, the ratios of viral hepatitis-associated LC decreased (HBV: from 11.5% to 8.1%; HCV: from 48.2% to 23.4%), while the ratios of ALD-associated LC and NASH-associated LC increased (from 19.9% to 35.4% and from 6.3% to 14.6%, respectively). Regarding cases of LC with hepatocellular carcinoma (n = 5906), HCV-associated LC (1986 cases, 33.6%) was the most frequent cause. Alcohol-related liver disease-associated LC, NASH-associated LC, and HBV-associated LC were the second-, third-, and fourth-ranked causes, respectively. In comparison to the previous survey, as the cause of hepatocellular carcinoma-complicated LC, HCV-associated LC decreased from 60.3% to 33.6%, while the ratios of ALD-associated LC and NASH-associated LC increased from 14.2% to 28.6% and from 4.2% to 14.0%, respectively. CONCLUSIONS: The major causes of LC in Japan are suggested to have been shifting from viral hepatitis to nonviral chronic liver diseases.

Nationwide status of progestogen treatment to prevent spontaneous preterm birth: A questionnaire survey for childbirth healthcare facilities in Japan.

AIM: This study aimed to investigate the current status of progestogen treatment for pregnant women at a high risk for preterm birth (PTB) in childbirth healthcare facilities in Japan. METHODS: A web-based nationwide questionnaire survey regarding progestogen use for prevention of PTB was conducted among childbirth healthcare facilities from 2019 to 2021. RESULTS: Valid responses were obtained from 528 facilities (25.2% of those surveyed), including 155 tertiary perinatal facilities (making up 92.3% of all tertiary perinatal care facilities). In the survey period, progestogen treatment was implemented in 207 facilities (39.2%) for PTB prevention. Regarding types of progestogens, 17α-hydroxyprogesterone caproate was used in 170 facilities (82.1%), with a low dose (125 mg/week) administered in 62.9% of the facilities to comply with the regulations of the national health insurance system, although 250 mg/week is considered the best dose. Vaginal progesterone was used in 36 facilities (17.4%), although the cost of vaginal progesterone was not covered by health insurance. Of the facilities not administering progestogen treatment, approximately 40% expressed that vaginal progesterone would be their first choice for PTB prevention in daily practice if it would be covered by health insurance in the future. CONCLUSIONS: Due to the current regulations of the Japanese health insurance system, 17α-hydroxyprogesterone caproate, rather than vaginal progesterone, was mainly used for PTB prevention. Despite global evidence supporting vaginal progesterone as the approach with the highest efficacy, only a limited number of facilities have utilized it due to the current drug use regulations in Japan.

Prevalence and clinical profiles of anti-myelin-associated glycoprotein neuropathy in Japan: A nationwide survey study of 133 patients.

BACKGROUND AND PURPOSE: The aim of this study was to determine the prevalence of anti-myelin-associated glycoprotein (MAG) neuropathy and the current status of such patients in Japan. METHODS: We conducted a nationwide survey in 2021 using established epidemiological methods. Questionnaires were sent to all neurology and pediatric neurology departments throughout Japan to identify patients with anti-MAG neuropathy. An initial questionnaire was used to determine the number of patients, with a second one used to collect detailed clinical information. RESULTS: The estimated number of patients with anti-MAG neuropathy was 353, with a prevalence of 0.28 per 100,000 and an incidence of 0.05 per 100,000. The detailed clinical profiles of 133 patients were available. The median (range) age of onset was 67 (30-87) years, with a prominent peak in the age range 66-70 years, and the male-to-female ratio was 3.6. Most patients had distal sensory-predominant polyneuropathy, and neuropathic pain (50%), or sensory ataxia (42%), while 18% had Waldenström's macroglobulinemia or multiple myeloma. Intravenous immunoglobulin was the most frequently used treatment (65%), but the response rate was <50%, whereas rituximab was given in 32% of patients, and 64% of these showed improvement. At the last visit, 27% of patients could not walk independently. CONCLUSIONS: This study on anti-MAG neuropathy provides updated insights into the epidemiology of this disease, clinical profiles, and treatment approaches in Japan. Rituximab therapy, used for only one-third of the patients, demonstrated efficacy. During the final visit, a quarter of the patients were unable to walk independently. Further studies are warranted to determine the optimal management of this rare and intractable disorder.

Safety and short-term outcomes of robotic-assisted transabdominal preperitoneal repair for inguinal hernia in pioneering hospitals in Japan: A nationwide retrospective cohort study.

INTRODUCTION: We aimed to evaluate the safety and short-term outcomes of robotic-assisted transabdominal preperitoneal repair for inguinal hernia in 12 pioneering hospitals in Japan. METHODS: Clinical data of patients who underwent robotic-assisted transabdominal preperitoneal repair between September 1, 2016, and December 31, 2021 were collected. Primary outcome measures were intra-operative adverse events and post-operative complications, whereas secondary outcomes were surgical outcomes, including chronic pain, recurrence, and learning curve. RESULTS: In total, 307 patients were included. One case of inferior epigastric arterial injury was reported; no cases of bowel or bladder injury were reported. Thirty-five seromas were observed, including four (1.3%) cases that required aspiration. The median operative time of a unilateral case was 108 minutes (interquartile range: 89.8-125.5), and post-operative pain was rated 1 (interquartile range: 0-2) on the numerical rating scale. In complicated cases, such as recurrent inguinal hernias and robotic-assisted radical prostatectomy-associated hernias, dissection and suture were safely achieved, and no complications were observed, except for non-symptomatic seroma. All patients underwent robotic procedures, and there was no chronic post-operative inguinal pain, although one case of hernia recurrence was reported. Regarding the learning curve, plateau performance was achieved after 7-10 cases in terms of operative time (P < .001). CONCLUSION: Robotic-assisted transabdominal preperitoneal repair can be safely introduced in Japan. Regardless of the involvement of many surgeons, the mastery of robotic techniques was achieved relatively quickly. The advantage of robotic technology such as wristed instruments may expand the application of minimally invasive hernia repair for complicated cases.

Tracheostomy in otorhinolaryngology education and training programs: A Japanese nationwide survey.

OBJECTIVE: Surgical airway management is one of the most effective techniques for safe airway management. Within the training programs relating to knowledge and skills required by otorhinolaryngologists, tracheostomy and postoperative management are important items that must be fully understood by airway surgeons. We performed a nationwide survey to identify problems within tracheostomy and postoperative management in Japan in order to establish practical and safe guidelines for surgical airway management. METHODS: We conducted a questionnaire survey of the current status of tracheostomy and postoperative management at core institution of otorhinolaryngology training programs in Japan. RESULTS: Responses were obtained from all 101 core training institutions in Japan. Tracheostomy was performed in the operating room at 61.4% of institutions and in the ICU at 26.7%. 89.1% of them performed surgical tracheostomy (ST) in all cases. Even in the remaining 10.9%, percutaneous dilatational tracheostomy (PDT) was performed in less than 10% of cases. The primary surgeon was an otorhinolaryngology resident at 89.1% of institutions. The method of securing the tube immediately after surgery was by securing it with an attached cord at 48.5% of institutions, by suturing to the skin at 25.7%, and using a Velcro band at 24.8%. The first tube change after tracheostomy was performed on the seventh postoperative day at 81.2% of institutions. 87.1% had more than one person performing the first tube change. The tracheostomy postoperative complications within the past year were as follows: tracheostomal granulation: 89.1%; subcutaneous and/or mediastinal emphysema: 62.4%; tube stenosis: 55.4%; accidental tube removal: 50.5%; incorrect tube insertion or misplacement: 15.8%; hemorrhage from tracheal foramen requiring hemostasis in the operating room: 14.9%; pneumothorax: 4.0%; tracheo-innominate arterial fistula: 2.0%; and tracheoesophageal fistula: 1.0%. The method for educating otorhinolaryngology residents about tracheostomy was on-the-job training at 98.0% of institutions. CONCLUSIONS: For airway management in otorhinolaryngology training programs, after learning the basics of ST, PDT should also be well understood. Furthermore, in order to create safe educational programs for intraoperative and postoperative management, it is necessary to train otorhinolaryngologists with accurate knowledge and skills, and to strengthen collaboration with multiple professions in their leadership roles as airway surgeons.

How many cases do instructor class pediatric surgeons need to experience to be an independent operator in performing advanced endoscopic surgery? A nationwide survey to establish an ideal curriculum for pediatric endoscopic surgery in Japan.

PURPOSE: To ensure the safe spread of pediatric endoscopic surgery, it is essential to build a training curriculum, and a survey of the current situation in Japan is necessary. The present study assessed an efficient training curriculum by clarifying instructor class pediatric surgeons' experiences, including autonomy when performing advanced endoscopic surgeries. METHODS: An online nationwide questionnaire survey was conducted among pediatric surgeons who had Endoscopic Surgical Skill Qualification (ESSQ) and board-certified instructors who had skills comparable to ESSQ. We assessed participants' training experience, opinions concerning the ideal training curriculum, and the correlation between surgical experience and the level of autonomy. The Zwisch scale was used to assess autonomy. RESULTS: Fifty-two participants responded to the survey (response rate: 86.7%). Only 57.7% of the respondents felt that they had received sufficient endoscopic surgery training. Most respondents considered an educational curriculum for endoscopic surgery including off-the-job training essential during the training period. Autonomy had been acquired after experiencing two to three cases for most advanced endoscopic surgeries. CONCLUSION: This first nationwide survey in Japan showed that instructor class pediatric surgeons acquired autonomy after experiencing two to three for most advanced endoscopic surgeries. Our findings suggest that training, especially off-the-job training, has been insufficient.

Report of the 23rd nationwide follow-up survey of primary liver cancer in Japan (2014-2015).

For the 23rd Nationwide Follow-up Survey of Primary Liver Cancer in Japan, data from 20 889 newly registered patients and 42 274 previously registered follow-up patients were compiled from 516 institutions over a 2-year period from January 1, 2014 to December 31, 2015. Basic statistics compiled for patients newly registered in the 23rd survey were cause of death, past medical history, clinical diagnosis, imaging diagnosis, treatment-related factors, pathological diagnosis, recurrence status, and autopsy findings. Compared with the previous 22nd survey, the population of patients with hepatocellular carcinoma (HCC) was older at the time of clinical diagnosis, had more female patients, had more patients with non-B non-C HCC, had smaller tumor diameter, and was more frequently treated with hepatectomy. Cumulative survival rates were calculated for HCC, intrahepatic cholangiocarcinoma, and combined hepatocellular cholangiocarcinoma (combined HCC and intrahepatic cholangiocarcinoma) by treatment type and background characteristics for patients newly registered between 2004 and 2015 whose final outcome was survival or death. The median overall survival and cumulative survival rates for HCC were calculated by dividing patients by combinations of background factors (number of tumors, tumor diameter, Child-Pugh grade, or albumin-bilirubin grade) and by treatment type (hepatectomy, radiofrequency ablation therapy, transcatheter arterial chemoembolization, hepatic arterial infusion chemotherapy, and systemic therapy). The same values were also calculated according to registration date by dividing patients newly registered between 1978 and 2015 into five time period groups. The data obtained from this nationwide follow-up survey are expected to contribute to advancing clinical research and treatment of primary liver cancer in the world.

Posterior sagittal anorecto-urethro-vagino-plasty in the late period was associated with the long-term bowel function in patients with persistent cloaca: results of a nationwide survey in Japan.

PURPOSE: After conducting a nationwide survey of persistent cloaca (PC), we assessed whether or not the timing of definitive anorectoplasty affects the long-term bowel function of patients with PC. METHODS: Patient information was obtained via questionnaire, and a total of 169 PC patients who underwent posterior sagittal anorectourethrovaginoplasty (PSARUVP) were enrolled in this study. Patients were classified into 2 groups based on their operative period, which was analyzed by the area under the receiver operating characteristic curve: the early group (EG) underwent anorectoplasty at ≤ 18 months old (n = 106), and the late group (LG) underwent anorectoplasty at > 18 months old (n = 63). The bowel function was evaluated using the evacuation score of the Japan Society of Anorectal Malformation Study Group. We also examined the postoperative results of vaginoplasty. RESULTS: The total evacuation score was significantly higher in the EG than in the LG (5.2 ± 1.7 vs. 4.2 ± 1.8, p = 0.003). The frequency of bowel movement and the constipation scores were significantly higher in the EG than in the LG (1.4 ± 0.6 vs. 1.2 ± 0.7, p < 0.05, 2.4 ± 1.0 vs. 2.1 ± 1.0, p < 0.05, respectively). Postoperative vaginal stenosis was observed in 18 cases (10.7%), of which 16 could be reconstructed transperineally. CONCLUSION: PSARUVP should be performed in early infancy and facilitate vaginal reconstruction.

The clinical features of patients who underwent bladder augmentation of cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

PURPOSE: Cloacal exstrophy (CE) patients may need bladder reconstruction after initially undergoing surgery to obtain continence and improve their quality of life. This study attempts to clarify the clinical features of CE patients who underwent bladder augmentation (BA) and their urinary functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted, and 150 CE patients were enrolled. Their clinical characteristics and urinary outcomes were reviewed. RESULTS: BA was performed in 52 patients (34.7%). Most cases underwent early bladder closure at initial surgery in neonate period. The age at the BA was performed 6.4 [6-9.0] years. Among them, the most used organ for BA was ileum (n = 30, 57.7%). Regarding the outcomes, the age when the renal function was evaluated was 14.0 [10.0-20.5] years and the serum creatinine level was 0.44 [0.36-0.60] (mg/dl). Clean intermittent catheterization was required in 37 (71.2%) patients. On the other hand, no dialysis or kidney transplantation was necessary in any of these patients. CONCLUSION: The renal function and conditions of patients who underwent BA were relatively well preserved. Individualized management with a stepwise surgical approach for CE patients should thus be considered in the future.

Clinical and prognostic features of Langerhans cell histiocytosis in adults.

Langerhans cell histiocytosis (LCH) is a rare disease characterized by clonal expansion of CD1a+ CD207+ myeloid dendritic cells. The features of LCH are mainly described in children and remain poorly defined in adults; therefore, we conducted a nationwide survey to collect clinical data from 148 adult patients with LCH. The median age at diagnosis was 46.5 (range: 20-87) years with male predominance (60.8%). Among the 86 patients with detailed treatment information, 40 (46.5%) had single system LCH, whereas 46 (53.5%) had multisystem LCH. Moreover, 19 patients (22.1%) had an additional malignancy. BRAF V600E in plasma cell-free DNA was associated with a low overall survival (OS) rate and the risk of the pituitary gland and central nervous system involvement. At a median follow-up of 55 months from diagnosis, six patients (7.0%) had died, and the four patients with LCH-related death did not respond to initial chemotherapy. The OS probability at 5 years post-diagnosis was 90.6% (95% confidence interval: 79.8-95.8). Multivariate analysis showed that patients aged ≥60 years at diagnosis had a relatively poor prognosis. The probability of event-free survival at 5 years was 52.1% (95% confidence interval: 36.6-65.5), with 57 patients requiring chemotherapy. In this study, we first revealed the high rate of relapse after chemotherapy and mortality of poor responders in adults as well as children. Therefore, prospective therapeutic studies of adults with LCH using targeted therapies are needed to improve outcomes in adults with LCH.

Nationwide Survey of the Surgical Complications Associated with Lateral Lumbar Interbody Fusion in 2015-2020.

Introduction: Lateral lumbar interbody fusion (LLIF) has been introduced in Japan in 2013. Despite the effectiveness of this procedure, several considerable complications have been reported. This study reported the results of a nationwide survey performed by the Japanese Society for Spine Surgery and Related Research (JSSR) on the complications associated with LLIF performed in Japan. Methods: JSSR members conducted a web-based survey following LLIF between 2015 and 2020. Any complications meeting the following criteria were included: (1) major vessel, (2) urinary tract, (3) renal, (4) visceral organ, (5) lung, (6) vertebral, (7) nerve, and (8) anterior longitudinal ligament injury; (9) weakness of psoas; (10) motor and (11) sensory deficit; (12) surgical site infection; and (13) other complications. The complications were analyzed in all LLIF patients, and the differences in incidence and type of complications between the transpsoas (TP) and prepsoas (PP) approaches were compared. Results: Among the 13,245 LLIF patients (TP 6,198 patients [47%] and PP 7,047 patients [53%]), 389 complications occurred in 366 (2.76%) patients. The most common complication was sensory deficit (0.5%), followed by motor deficit (0.43%) and weakness of psoas muscle (0.22%). Among the patient cohort, 100 patients (0.74%) required revision surgery during the survey period. Almost half of the complications developed in patients with spinal deformity (183 patients [47.0%]). Four patients (0.03%) died from complications. Statistically more frequent complications occurred in the TP approach than in the PP approach (TP vs. PP, 220 patients [3.55%] vs. 169 patients [2.40%]; p<0.001). Conclusions: The overall complication rate was 2.76%, and 0.74% of the patients required revision surgery because of complications. Four patients died from complications. LLIF may be beneficial for degenerative lumbar conditions with acceptable complications; however, the indication for spinal deformity should be carefully determined by the experience of the surgeon and the extent of the deformity.

Clinical features of patients who underwent anoplasty for cloacal exstrophy and their functional outcomes: the results of a nationwide survey in Japan.

PURPOSE: This study was performed to clarify the clinical features of cloacal exstrophy (CE) patients who underwent anoplasty and their functional outcomes based on a nationwide survey in Japan. METHODS: A questionnaire survey was conducted and data were obtained from 229 CE patients. After the exclusion of non-surviving patients and unknown data, 195 patients were enrolled. We compared the patient characteristics of the no anoplasty group (NAP group) to the anoplasty group (AP group). The defecation function of patients who underwent anoplasty was evaluated. RESULTS: The AP group had significantly lower rates of omphalocele (p = 0.045) and spinal defects (p = 0.003) than the NAP group. Of the 195 patients enrolled in this study, only 17 (8.7%) underwent anoplasty. Of the 17 patients who underwent anoplasty, 7 (41.1%) eventually had a permanent stoma after anoplasty [after ileostomy: n = 3 (60.0%), after colostomy: n = 4 (44.4%)]. Regarding soiling, 4 patients (66.7%) showed the absence of soiling, 2 (33.3%) showed accidental soiling, and no patients showed frequent soiling. CONCLUSION: In our study, spinal defects may have affected the determination of anoplasty. Anoplasty may improve the quality of life, because defecation can be kept clean, but indications, including colon function, may be carefully considered.