Treatment and Outcomes of 844 Cases of Pneumothorax in Heritable Connective Tissue Disorders.

BACKGROUND: Characteristics, practice patterns, and clinical outcomes of secondary spontaneous pneumothorax (SSP) with heritable connective tissue disorders (Marfan, Ehlers-Danlos, and Birt-Hogg-Dubé syndromes) are unclear. METHODS: A nationwide Japanese inpatient database that included data of 524 patients with SSP (884 hospitalizations) and 137,821 with primary spontaneous pneumothorax between July 2010 and March 2020 was used in this study. Hospitalization for SSP (n = 884) was categorized into surgical (n = 459) and nonsurgical (n = 425) groups, and the patient characteristics, treatment, and outcomes were compared between the groups. Multivariable analyses were performed to evaluate risk factors for pneumothorax recurrence. We also compared the characteristics of patients with different underlying heritable connective tissue disorders. RESULTS: Compared with the nonsurgical group, the surgical group had less frequent readmission for pneumothorax (26% vs 44%; hazard ratio, 0.47; 95% CI, 0.38-0.58). Young patients (2.46; 1.83-3.32) or those with Birt-Hogg-Dubé syndrome (2.53; 1.77-3.63) had a high risk of recurrence. Pneumothorax occurred frequently in teenagers with Marfan syndrome, in those aged 20 to 39 years with Ehlers-Danlos syndrome, and in those aged ≥40 years with Birt-Hogg-Dubé syndrome. CONCLUSIONS: Detailed information on the characteristics and clinical course of SSP in heritable connective tissue disorders will aid in the clinical decision-making process.

Treatment courses and outcomes of oesophageal atresia in patients with trisomy 18: a case series of 271 patients from a nationwide database in Japan.

OBJECTIVE: To describe characteristics, treatments and clinical outcomes of patients with trisomy 18 and oesophageal atresia, using a nationwide database in Japan. DESIGN: Descriptive study using a retrospective cohort. SETTING: A nationwide inpatient database including 90% of hospitals with neonatal intensive care units in Japan. PATIENTS: Patients hospitalised within a day after birth for both oesophageal atresia and trisomy 18 between July 2010 and March 2020. INTERVENTIONS: Radical surgery for oesophageal atresia. MAIN OUTCOME MEASURES: Characteristics, treatment course and outcomes. RESULTS: Among 271 patients with both oesophageal atresia and trisomy 18, 70 patients underwent radical surgery for oesophageal atresia. Patients who underwent radical surgery were less likely to have severe cardiac anomalies (17% vs 32%; p=0.020), but more likely to undergo cardiac surgery (21% vs 9.5%; p=0.012) than those who did not. The overall in-hospital mortality was lower (54% vs 79%; p<0.001) and the median age at death was higher (210 days vs 39 days; p<0.001) in patients who underwent radical surgery than the others. Postoperative mortality within 30 days after radical surgery was 5.7%. Patients who underwent radical surgery were likely to be discharged to home (50% vs 18%; p<0.001), whereas the age at home discharge (median 314 days vs 216 days; p=0.19) and the requirement for each home treatment did not differ significantly by radical surgery. CONCLUSION: This study provides information that will aid the clinical decision-making process for patients with oesophageal atresia and trisomy 18. Radical surgery may be a safe and feasible treatment option.

Outcomes Following Laparoscopic Versus Open Surgery for Pediatric Intussusception: Analysis Using a National Inpatient Database in Japan.

BACKGROUND: Laparoscopic surgery for pediatric intussusception has recently become more common as an alternative to open surgery. However, the differences in outcomes between laparoscopic and open surgery remain unclear. Thus, this study aimed to compare short-term surgical outcomes and recurrence rates between patients treated with laparoscopic and open surgery for pediatric intussusception. METHODS: Patients aged <18 years who underwent laparoscopic (n = 192) and open (n = 416) surgery for intussusception between April 2016 and March 2021 were retrospectively identified using a Japanese nationwide inpatient database. Propensity-score overlap weighting analyses were conducted to compare the outcomes between the laparoscopic and open surgery groups. The outcomes included in-hospital morbidity, reoperation, readmission for intussusception, bowel resection, the diagnosis of Meckel's diverticulum, duration of anesthesia, postoperative length of hospital stay, and total hospitalization costs. RESULTS: The laparoscopic surgery group was older, heavier, and had fewer congenital malformations and emergency admissions than the open surgery group did. Overlap weighting analyses showed no significant differences in in-hospital morbidity (odds ratio [95% confidence interval], 0.88 [0.35-2.23]), reoperation (1.88 [0.24-14.9]), readmission for intussusception within 30 days (0.80 [0.12-5.30]) and 1 year (0.90 [0.28-2.93]), bowel resection (0.69 [0.46-1.02]), the diagnosis of Meckel's diverticulum (0.97 [0.50-1.90]), duration of anesthesia (difference, 11 [-1-24] minutes), postoperative length of stay (difference, -1.9 [-4.2-0.4] days), or total hospitalization costs (difference, 612 [ -746-1970] US dollars) between the groups. CONCLUSIONS: In this large nationwide cohort, no significant differences in outcomes were observed between laparoscopic and open surgery. Laparoscopic surgery is an acceptable treatment option for pediatric intussusception. LEVELS OF EVIDENCE: Level III.

Examination of the effectiveness of bedside nasojejunal tube placement using a portable imaging device with a flat panel detector.

Transporting pediatric patients with severe cardiovascular complications to the fluoroscopy room can be difficult. Therefore, we started using a portable imaging device with a flat panel detector (FPD) for nasojejunal tube (NJT) placement. The purpose of this study was to investigate the differences in length of time of NJT placement and dosage of radiation exposure using a portable imaging device with FPD versus fluoroscopy. Pediatric patients who underwent NJT placement between April 2016 and December 2018 were identified retrospectively from the clinical records. The age, sex, body weight, and height of each child at the time of the procedure as well as the procedure time, outcomes of the procedure, and dosage of radiation exposure was compared between the two groups. In 76 cases of NJT placement (41 patients), there was no significant difference in the success rate of NJT placement between the FPD (90%) and fluoroscopy groups (95%). However, the NJT placement time was significantly longer in the FPD group than in the fluoroscopy group (488 s vs 291 s). According to our calculations, the radiation dosage was lower in the FPD group than in the fluoroscopy group (136 µGy per procedure vs 2819 µGy per procedure). These results suggest that NJT placement using a portable imaging device with an FPD can be an effective method for children who are difficult to transport with an equal success rate and lower dosage of radiation exposure compared with conventional fluoroscopy.

Optimal Viewing Direction of the Oblique Laparoscope in Single-Incision Laparoscopic Percutaneous Extraperitoneal Closure for Inguinal Hernia.

Background: It is difficult for novice surgeons to manipulate the oblique laparoscope in single-incision laparoscopic percutaneous extraperitoneal closure (SILPEC) for inguinal hernia because of collisions between the instruments. To standardize manipulation of the laparoscope, we studied the viewing direction of the oblique laparoscope, and assessed the optimal manipulation of the laparoscope to avoid collisions. Methods: A retrospective chart review was performed on patients who underwent SILPEC between April 2016 and April 2017. The viewing direction of the 30° oblique laparoscope was measured according to the location of the field stop pointer. Patients were divided into three groups according to the viewing direction at the beginning of the procedure: the inside viewing direction was from -90° to -11°, upward viewing direction was from -10° to 10°, and outside viewing direction was from 11° to 90°. The length of the procedure, viewing direction at the end, and the percentage of cases in which there was a change in viewing direction during the procedure were compared. Results: Ninety-eight cases of SILPEC were performed during the study period. The percentage of patients with a change in category of viewing direction in the inside, upward, and outside groups was 35%, 21%, and 11%, respectively, showing a significant difference among the three groups. Conclusions: Setting the initial viewing direction to the outside can reduce correction of the viewing direction during SILPEC. Because the intersection angle between the outside-viewing laparoscope and forceps is close to a right angle, this might reduce collisions.

Outcomes of Alagille syndrome following the Kasai operation: a systematic review and meta-analysis.

PURPOSE: Infants with Alagille syndrome (AGS) frequently develop neonatal cholestasis, and some AGS infants who suspected of biliary atresia subsequently undergo the Kasai operation with the diagnosis of biliary atresia. The aim of this study was to investigate the effect of the Kasai operation on liver and patient outcomes among AGS patients, using a meta-analysis. METHODS: A systematic review and meta-analysis of studies describing the outcomes of AGS patients with/without the Kasai operation were conducted. The analyzed outcomes were liver transplantation, not living with the native liver, and mortality for any reason. RESULTS: We identified 6 studies (394 AGS patients). All studies were retrospective cohort or case-control studies. The incidences of liver transplantation, not living with the native liver, and mortality were significantly higher in AGS patients who underwent the Kasai operation than in those who did not undergo the Kasai operation (odds ratio: 6.46, 95% CI 3.23-12.89, p < 0.00001; odds ratio: 25.88, 95% CI 2.83-236.84, p < 0.004; odds ratio: 15.05, 95% CI 2.70-83.93, p = 0.002, respectively). CONCLUSION: The Kasai operation was associated with poor outcomes in AGS patients. It remains unclear if the Kasai operation directly deteriorates liver and patient outcomes in AGS patients.