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, which included data on 524 patients with SSP (884 hospitalizations) and 137821 with primary spontaneous pneumothorax (PSP) between July 2010 and March 2020, was used in this study. Hospitalization for SSP (n=884) was categorized into surgical (n=459) and non-surgical (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 patient characteristics among those with different underlying heritable connective tissue disorders. RESULTS: Compared to the non-surgical group, the surgical group had less frequent readmission for pneumothorax (26% vs. 44%; hazard ratio, 0.47; 95% confidence interval, 0.38-0.58). Young patients (hazard ratio, 2.46; 95% confidence interval, 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 20-39 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 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.

A case of Hirschsprung's disease with segmental dilatation of the colon.

BACKGROUND: Segmental dilatation of the colon (SDC) is a rare disease that is characterized by an abrupt segment of dilated colon between regions of normal-sized colon. We herein report a case of SDC associated with Hirschsprung's disease (HD). CASE PRESENTATION: The patient developed abdominal distension soon after birth, and enema examination showed localized intestinal dilatation from the descending colon to the sigmoid colon with significant caliber changes on both the oral and anal sides of the dilated colon. The findings of the rectal mucosal biopsy were consistent with HD. We considered this case to be a combination of HD and SDC and performed laparoscopic-assisted Soave pull-through with resection of the dilated colon when the patient was 7 months old. Resected specimens showed steep caliber changes on the oral and anal sides of the dilated colon. In the pathological examination, no ganglion cells were found in the submucosa on the anal side of the dilated colon. Based on the above findings, we finally made the diagnosis of HD with SDC. CONCLUSION: In HD with a characteristic dilated colon, the possibility of SDC should be considered.

Definitive Tumor Resection after Myeloablative High Dose Chemotherapy Is a Feasible and Effective Option in the Multimodal Treatment of High-Risk Neuroblastoma: A Single Institution Experience.

BACKGROUND/PURPOSE: The delayed local treatment approach (DL) in high-risk neuroblastoma (HR-NB) refers to the process in which tumor resection is performed after the completion of all the courses of chemotherapy, including myeloablative high-dose chemotherapy (HDC). Alternatively, in the conventional local treatment approach (CL), tumor resection is performed during induction chemotherapy. In this study, we compared the surgical outcomes in HR-NB patients treated by CL and DL. METHOD: Forty-seven patients with abdominal HR-NB underwent primary tumor resection from 2002 to 2018. The timing of surgery was generally determined by following the trials and guidelines available at the time. The outcomes and surgical complications between the two strategies were compared. RESULT: Operation time, blood loss, and postoperative WBC counts were lower in the DL group (n = 25) when compared to the CL group (n = 22), statistical significance notwithstanding. Major vascular structures were less frequently encased in the DL group tumors, while immediate surgical complications were significantly more frequent in the CL group (P < 0.05). Furthermore, the 3-year EFSs were 50.0% and 53.9% in the DL and CL groups, respectively. CONCLUSION: DL appears to be a feasible and effective treatment option for HR-NB. Nonetheless, further verifications using larger cohorts are warranted. LEVEL OF EVIDENCE: Treatment study, Level III.

Direct hyperbilirubinemia in infants with congenital heart disease.

BACKGROUND: The association between congenital heart disease (CHD) and infantile cholestasis, a key finding for the diagnosis of biliary atresia (BA), has not been previously investigated. The aim of this study was therefore to investigate the characteristics of direct hyperbilirubinemia (D-HB) in infants with CHD. METHODS: All neonates admitted to the present hospital and diagnosed with CHD in 2015 and 2016 were included. D-HB (direct bilirubin ≥ 2.0 mg/dL) at ≤60 days of age and other clinical parameters were retrospectively reviewed. Statistical analysis according to presence of D-HB was performed using chi-squared test or Wilcoxon rank sum test. RESULTS: Seventy-six patients (M:F, 36:40) were included in this study. CHD consisted of ventricular septal defect in 17, patent ductus arteriosus in 10, and other in 49. Thirteen patients (17.1%) had D-HB at ≤60 days of age. Resolution of D-HB (DB < 2.0 mg/dL) occurred in 10 of the 13 patients during the hospital stay, and this occurred in ≤7 days in eight of the 10 patients. Sex, gestational age, birthweight, chromosomal anomalies, need for Fontan operation for CHD repair, and/or cardiac operation were not associated with D-HB at ≤60 days of age. CONCLUSION: While D-HB was frequently observed in infants with CHD, the majority of D-HB cases resolved spontaneously in ≤1 week. Neonatal clinical parameters or CHD status was not predictive of D-HB. D-HB lasting >1 week in infants with CHD should be evaluated for the cause.