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The aim of this study was to describe the etiology and clinical course in children with severe thrombocytosis (ST, platelet counts > 900 × 109/L) and extreme thrombocytosis (ET, platelet counts > 1000 × 109/L) in a tertiary pediatric hospital. Patients aged 0-18 years with platelet counts over 900 × 109/L who were admitted to our hospital were analyzed. Thrombocytosis was defined as a platelet count exceeding 450 × 109/L. Thrombosis was diagnosed based on computed tomography scans or ultrasound findings. Potential factors associated with the development of extreme thrombocytosis were identified using logistic regression models. Only one (0.8%) out of the 120 patients identified with ST (n = 61) and ET (n = 59) had primary thrombocytosis. The most common underlying condition was congenital heart disease (26.7%), followed by Kawasaki disease (16.7%). With the exception of the hemoglobin level, no major differences were found for the baseline characteristics between the ST and ET groups. A lower hemoglobin level (< 10.0 g/dL) at the onset of thrombocytosis was identified as a predictor for ET development (adjusted odds ratio 2.73, 95% confidence interval 1.18-6.28). Overall, 56 of 120 (46.7%) patients received aspirin therapy. Venous thrombosis occurred in one (0.8%) patient. CONCLUSIONS: We found a low proportion of primary thrombocytosis and a low incidence of thrombosis in children with ST and ET. Our results suggest that pediatric ST and ET may share common characteristics and may have features that are distinct from those in adults. WHAT IS KNOWN: • Secondary thrombocytosis is a frequent finding in children. • Adult extreme thrombocytosis has been found to be associated with primary thrombocytosis. WHAT IS NEW: • There were no major differences in the baseline characteristics between children with severe and extreme thrombocytosis. • The incidence of thrombosis was markedly low in both severe and extreme thrombocytosis groups.

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This study investigates the long-term outcomes of palliative and definitive surgeries for esophageal atresia (EA) in patients with trisomy 18 syndrome. A retrospective study included 25 cases undergoing EA surgery at our center between 2008 and 2022. The Palliative group (n = 16) comprised 13 cases with esophageal banding and 3 with tracheoesophageal fistula (TEF) division. The Definitive group (n = 9) included 5 cases with primary repair and 4 with staged repair following TEF division. The patient characteristics exhibited no significant differences between the groups. In the Definitive group, 56% (5/9) were successfully weaned off mechanical ventilation, compared with none in the Palliative group (p = 0.002). Survival-to-discharge rates were 31% (5/16) in the Palliative group and 67% (6/9) in the Definitive group. Home ventilator management was required for all 5 cases that required ventilation in the Palliative group, whereas only 17% (1/6) in the Definitive group needed it. The Palliative group also required continuous oral suction for persistent saliva removal, with two cases undergoing laryngotracheal separation. Overall, definitive surgery for EA in patients with trisomy 18 syndrome may provide enhanced respiratory stability, thereby improving the survival-to-discharge rate and overall quality of life for patients and their families.

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PURPOSE: Long-term outcomes of slide tracheoplasty in patients with congenital tracheal stenosis (CTS) have rarely been reported. This study aimed to clarify the long-term outcomes of CTS after slide tracheoplasty. METHODS: The medical records of 33 patients who underwent slide tracheoplasty for CTS at our institution between January 2005 and July 2018, with a follow-up duration > 5 years, were retrospectively reviewed. Patients' characteristics, perioperative condition, operative management, postoperative course, tracheal stenosis rates and growth data, were collected from medical records. RESULTS: The median operative age, minimum tracheal diameter, length of stenosis, duration of hospital stays, and follow-up duration were 8 months, 2.4 mm, 35 mm, 39 days, and 90 months, respectively. One patient died of bleeding in the right lung at 126 months postoperatively. Among the 10 patients requiring postoperative tracheostomy, seven were successfully decannulated at a median of 65 months postoperatively. Tracheal stenosis rates improved postoperatively and were subsequently maintained. Growth impairment and psychomotor delay were observed in 9 and 16 patients, respectively with significant differences found only in cases with genetic abnormalities and not in tracheal stenosis severity. CONCLUSION: Slide tracheoplasty for CTS leads to favorable long-term outcomes. However, various associated anomalies may influence growth and psychomotor development, emphasizing the importance of adequate support.

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PURPOSE: This study assessed the efficacy of a high-impact, short-term workshop in honing the laparoscopic hepaticojejunostomy technical skills and self-confidence of novice pediatric surgeons, focusing on vertical needle driving and knot tying. METHODS: Lectures, hands-on sessions, pre- and post-workshop evaluations, and training using porcine models were conducted to refine basic and advanced skills. The "hepaticojejunostomy simulator" was used for comparative analysis of precision in pre- and post-workshop vertical needle driving and knot tying. Participants self-evaluated their skills and confidence on a 5-point scale. RESULTS: After the workshop, eight inexperienced pediatric surgeons demonstrated a significant improvement in hepaticojejunostomy suturing task completion rates and needle-driving precision at the jejunum and hepatic duct. However, the A-Lap Mini Endoscopic Surgery Skill Assessment System indicated no significant improvements in most assessed parameters, except for the full-layer closure score (p = 0.03). However, a significant increase in participants' confidence levels in performing laparoscopic hepaticojejunostomy was observed. CONCLUSION: The workshop augmented technical proficiency and confidence in young pediatric surgeons. The combination of lectures, practical exposure, and model training is an effective educational strategy in pediatric surgical instruction.

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PURPOSE: Congenital tracheal stenosis (CTS) has been reported to occur in 50-65% of cases of left pulmonary artery sling (LPAS), but the exact incidence rate is unknown. This study aimed to determine the actual rate using bronchoscopy and to elucidate morphological features in computed tomography (CT) diagnosis. METHODS: We performed a single institutional retrospective review of all patients with LPAS between January 2010 and March 2022. The percentage of complete tracheal rings in patients with LPAS was evaluated using bronchoscopy. The anteroposterior/lateral diameter ratios at the smallest and largest diameters of each CTS patient's trachea were measured on CT. The Wilcoxon signed-rank test was used to analyze the differences between the two parts. RESULTS: Fifty-two patients with LPAS were enrolled. All patients had complete tracheal rings on bronchoscopy. CT analysis of 32 patients with CTS was performed. The median anteroposterior/lateral diameter ratio at the smallest diameter was 1.05 (interquartile range [IQR] 0.95-1.15); the median ratio at the largest diameter was 0.94 (IQR 0.89-0.99). There was a significant difference between the two parts (p = 0.013). CONCLUSION: CTS might be universally associated with LPAS. The circular tracheal cross-section on CT might imply the existence of a complete tracheal ring.

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PURPOSE: A tracheoesophageal fistula (TEF) associated with complete tracheal rings (CTR) is extremely rare. This study aimed to elucidate the clinical features of this combination. METHODS: Records of 39 patients diagnosed with TEF with or without CTR between January 2013 and February 2023 were retrospectively reviewed. Data collected included location of the TEF and CTR, esophageal gap, surgery of TEF/esophageal atresia (EA), and symptoms of CTR. RESULTS: Seven patients had CTR, while 32 patients did not have CTR. TEF was frequently located higher than the T4 vertebra, and the median esophageal gap was shorter in patients with CTR than in those without (5/7 [71.4%] vs. 6/32 [18.8%], P = 0.012; 0 (range: 0-15) mm vs. 13.3 (range: 0-40) mm, P = 0.017, respectively). TEF was located just above the beginning of the CTR in all patients. All patients with CTR underwent primary esophageal anastomosis. Additionally, two patients were diagnosed with CTR before TEF/EA repair. A tracheal tube was prevented from contacting the CTR during TEF/EA repair, and respiratory insufficiency did not occur. CONCLUSIONS: Our results may help pediatric surgeons in the management of TEF with CTR.

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Infantile fibrosarcoma (IFS) commonly harbors ETS variant transcription factor 6 (ETV6)-neurotrophic receptor tyrosine kinase 3 (NTRK3) fusion. However, the recent accessibility to clinical next-generation sequencing (NGS) has revealed ETV6-NTRK3 negative spindle cell sarcomas resembling IFS morphologically, involving NTRK1/2, MET, RET and BRAF. The present report describes a pediatric case of spindle cell sarcoma with KIAA1549-BRAF resembling IFS morphologically. A 20-month-old female patient was referred to Kobe Children's Hospital (Kobe, Japan) for the treatment of intrathoracic spindle cell sarcoma. Pathologically, the intrathoracic tumor cells were composed of spindle cells with focal hemagiopericytomatous pattern. In immunohistochemistry analysis, the intrathoracic tumor cells focally expressed desmin and WT-1 and were negative for pan-tropomyosin receptor kinase (TRK), S-100 and CD34. Fluorescence in situ hybridization analysis for ETV6 and capicua transcriptional repressor revealed negative split signals. Although the patient was initially diagnosed with IFS morphologically, KIAA1549-BRAF fusion transcript was detected by comprehensive genomic profiling with NGS using intrathoracic tumor tissues and confirmed by reverse transcription-PCR. Chemotherapy induced a reduction in the tumor size. At present, the patient is alive with the disease and has been receiving therapy for 8 months since the initiation of chemotherapy. Review of BRAF-altered spindle cell sarcomas resembling IFS morphologically revealed the inconsistency in immunohistochemical expression patterns and the diversity of BRAF fusion genes and mutations. Therefore, the elucidation of genomic profiling by NGS may assist in making an appropriate diagnosis and selecting novel alternative therapies in ETV6-NTRK3-negative spindle cell sarcomas resembling IFS morphologically.

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PURPOSE: The severity of congenital tracheal stenosis (CTS) is commonly evaluated based on the degree of stenosis. However, it does not always reflect the clinical respiratory status. We applied computational fluid dynamics (CFD) to the assessment of CTS. The aim of this study was to evaluate its validity. METHODS: CFD models were constructed on 15 patients (12 preoperative models and 15 postoperative models) with CTS before and after surgery, using the computed tomographic data. Energy flux, needed to drive airflow, measured by CFD and the minimum cross-sectional area of the trachea (MCAT) were quantified and evaluated retrospectively. RESULTS: The energy flux correlated positively with the clinical respiratory status before and after surgery (rs = 0.611, p = 0.035 and rs = 0.591, p = 0.020, respectively). Although MCAT correlated negatively with the clinical respiratory status before surgery (rs = -0.578, p = 0.044), there was not significant correlation between the two after surgery (p = 0.572). CONCLUSIONS: The energy flux measured by CFD assessment reflects the respiratory status in CTS before and after surgery. CFD can be an additional objective and quantitative evaluation tool for CTS.

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PURPOSE: Although surgical outcomes have improved in patients with congenital tracheal stenosis (CTS), the management of such patients with complex cardiovascular anomalies (CVAs) remains a challenge. This study aimed to clarify the clinical features of this combination. METHODS: Medical records of 14 patients with complex CVAs who had undergone slide tracheoplasty for CTS between May 2016 and February 2022 were retrospectively reviewed. Complex CVAs were defined as CVAs without left pulmonary artery sling and simple cardiac shunts. Data collected included age and body weight at tracheal reconstruction, preoperative respiratory support, surgery details, and mortality. RESULTS: The median age and body weight at tracheal reconstruction were 3.5 (range, 1-17) months and 4.7 (range, 2.3-8.2) kg, respectively. Simultaneous repair of CTS and CVAs was performed in eight patients and staged repair in six patients. Patients who underwent simultaneous repair required preoperative respiratory support more frequently than those who underwent staged repair (8/8 [100%] vs 2/6 [33.3%]; P = 0.015). No mortality occurred. CONCLUSIONS: Patients with complex CVAs often require tracheal reconstruction in early infancy. Surgical management strategy for this combination was generally decided on the basis of the severity of respiratory symptoms due to CTS.

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AIM: To assess mid-/long-term postoperative quality of life (QOL) of esophageal atresia (EA) patients. METHODS: Modified gastrointestinal quality-of-life index surveys were administered to postoperative EA patients who were at least 7 years old at evaluation to assess three topics about general lifestyle (GL), five topics about EA, and four topics about mental health (MH). For MH, caregivers were also interviewed, but separately. Subjects were divided according to age: children (7-12 years old), teenagers (13-19), and adults (20 and over) and compared according to Foker or Kimura elongation (FK) or bougienage stretching (BS). RESULTS: There were 22 patients evaluated. Responses for GL, EA, and MH did not differ significantly between age groups, but MH responses by caregivers for subjects who were children or teenagers scored significantly lower than responses they made themselves. For primary esophageal elongation technique (PET), age at esophagoesophagostomy was significantly higher in FK. Despite FK scoring 15.1 versus 12.4 for BS during EA evaluation, this difference was not statistically significant. CONCLUSION: Changes in QOL responses according to age were unremarkable. However, discrepancies in MH indicate that subjects felt better than their caregivers thought. PET did not appear to influence QOL.

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AIM: To assess mid-/long-term quality of life (QOL) of total colonic aganglionosis (TCA) patients. METHODS: Modified pre-existing QOL assessment tools for general lifestyle (GL), bowel function (BF), and mental health (MH) were administered to postoperative TCA patients from five institutions, who were at least 7 years old to compare Duhamel (with pouch) and Swenson/Soave (without pouch) techniques between children (Ch 7-12 years old), teenagers (Tn 13-19), and adults (Ad 20 and over). For MH, caregivers were also interviewed, but separately. Maximum scores were 12 for GL/MH and 18 for BF. RESULTS: There were 32 subjects. GL and BF scores increased significantly from Ch (GL 4.8 ± 2.5, BF: 11.3 ± 4.6) to Tn (GL 7.8 ± 2.6, BF 16.2 ± 3.0); scores for MH did not change significantly. Mean caregiver MH scores were significantly lower than mean subject MH scores for all age groups (subject scores: 10.1, 10.7, 10.7 versus caregiver scores: 6.8, 7.8, 8.1 for Ch, Tn, Ad, respectively). PT technique/presence of a pouch did not influence the incidence of enterocolitis or QOL scores. CONCLUSION: MH responses showed subjects felt better than caregivers believed. This discrepancy could cause conflict despite steadily improving GL/BF. QOL was unaffected by PT technique/presence of a pouch.

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