Prognostic factors for pediatric patients with severe intestinal motility disorders: a single institution's experience.

PURPOSE: To identify the prognostic factors for pediatric severe intestinal motility disorder (IMD). METHODS: We reviewed the medical records of patients with severe IMD, who required total parenteral nutrition (TPN) for ≥ 60 days at our institution between April, 1984 and March, 2023, examining their characteristics to identify prognostic factors. RESULTS: The types of IMD in the 14 patients enrolled in this study were as follows: isolated hypoganglionosis (IHG, n = 6), extensive aganglionosis (EAG: n = 6), and chronic idiopathic intestinal pseudo-obstruction (CIIP, n = 2). There was no significant difference in mortality among the three types of severe IMD. Weaning-off TPN and the use of the colon were not significant prognostic factors, but cholestasis was a significant prognostic factor (p = 0.005). There was a high mortality rate (50%), with the major causes of death being intestinal failure-associated liver disease (IFALD) following hepatic failure, and catheter-related blood stream infection (CRBSI). One IHG patient underwent small bowel transplantation but died of acute rejection. CONCLUSION: Severe IMD is still associated with a high mortality rate and cholestasis predicts the prognosis. Thus, preventing or improving IFALD and CRBSI caused by long-term TPN is important for reducing the mortality rate.

The postoperative renal function of persistent cloaca patients treated by posterior sagittal anorecto-urethro-vaginopalsty: results of a nationwide survey in Japan.

PURPOSE: We investigated the postoperative renal function in persistent cloaca (PC) patients who underwent posterior sagittal anorecto-urethro-vaginopalsty (PSARUVP) and factors influencing the renal functional outcomes. METHODS: A questionnaire survey was distributed to 244 university and children's hospitals across Japan. Of the 169 patients underwent PSARUVP, 103 patients were enrolled in the present study. Exclusion criteria was patients without data of renal prognosis. RESULTS: The present study showed that renal anomalies (p = 0.09), vesicoureteral reflux (VUR) (p = 0.01), and hydrocolpos (p = 0.07) were potential factors influencing a decline in the renal function. Approximately half of the patients had a normal kidney function, but 45.6% had a reduced renal function (Stage ≥ 2 chronic kidney disease: CKD). The incidence of VUR was significantly higher in the renal function decline (RFD) group than those in the preservation (RFP) group (p = 0.01). Vesicostomy was significantly more frequent in the RFD group than in the RFP group (p = 0.04). Urinary tract infections (p < 0.01) and bladder dysfunction (p = 0.04) were significantly more common in patients with VUR than in patients without VUR. There was no association between the VUR status and the bowel function. CONCLUSIONS: Prompt assessment and treatment of VUR along with bladder management may minimize the decline in the renal function.

Inflammation patterns in early post-operative cholangitis predict long-term outcomes in biliary atresia: a potential role of non-suppurative cholangitis.

PURPOSE: Frequent post-operative cholangitis in biliary atresia (BA) affects the long-term native liver survival. This study assessed the characteristics of early cholangitis and their influence on the prognosis. METHODS: Forty-three patients with BA who underwent surgery between 2000 and 2020 were analyzed for routine inflammatory markers. Early cholangitis characteristics were compared between native liver survivor (NLS) and living donor liver transplant (LDLT) patients. RESULTS: Among the 43 patients, 30 (69.8%) experienced 130 episodes of cholangitis. In the area under the receiver operating characteristics curve (AUROC) analysis, the cutoff value of the total cholangitis episodes was 3, with an area under the AUROC curve of 0.695 (95% confidence interval 0.522-0.868). Before 3 years old, 113 episodes (86.9%) of cholangitis were observed. The white blood cell, C-reactive protein, and alanine aminotransferase values at cholangitis onset did not markedly differ between the LDLT and NLS groups. Conversely, the neutrophil-to-lymphocyte ratio in the NLS group was significantly lower than in the LDLT group (0.85 vs. 1.63, p < 0.001). CONCLUSIONS: Cholangitis in the NLS group was lymphocyte-dominant and atypical in its pathogenesis. Lymphocyte-dominant cholangitis is non-suppurative, and future research should clarify its pathogenesis to improve the treatment and prognosis of BA.

An ultrasound-guided supraclavicular approach for tunneled central venous catheter insertion can be safely performed by junior residents.

PURPOSE: Ultrasound-guided supraclavicular catheterization (UGSC) of the brachiocephalic vein (BCV) for long-term tunneled central venous catheter (tCVC) insertion may be safer than the internal jugular vein approach due to its superior field of view. We examined the clinical outcomes of tCVC insertions performed by junior residents through UGSC of the BCV. PATIENTS AND METHODS: From January 2018 to December 2023, we assessed clinical outcomes and compared the experience levels of surgeons conducting tCVC insertions. Surgeons were categorized into three groups: junior residency (JR), senior residency (SR), and board-certified pediatric surgeons (BCPS). RESULTS: 177 tCVC insertions were done on 146 patients. Intraoperative complications included 6 cases of arterial puncture, 1 case of pneumothorax, 1 case of over insertion of catheter tip, and 1 case of suspected hemothorax. Distribution across groups was as follows: 28 cases (15.8%) in JR group, 92 (52.0%) in SR group, and 57 (32.2%) in BCPS group. Although the JR group exhibited longer operation times than the BCPS group, no significant differences in intraoperative complications were noted. CONCLUSION: Junior residents can safely perform UGSC for tCVC insertion. However, careful consideration of complications such as arterial or thoracic puncture is essential and case selection should be based on experience.

Impact of hepatocyte growth factor on the colonic morphology and gut microbiome in short bowel syndrome rat model.

PURPOSE: This study aimed to investigate the impact of hepatocyte growth factor (HGF) on colonic morphology and gut microbiota in a rat model of short bowel syndrome (SBS). METHODS: SD rats underwent jugular vein catheterization for total parenteral nutrition (TPN) and 90% small bowel resection [TPN + SBS (control group) or TPN + SBS + intravenous HGF (0.3 mg/kg/day, HGF group)]. Rats were harvested on day 7. Colonic morphology, gut microflora, tight junction, and Toll-like receptor-4 (TLR4) were evaluated. RESULTS: No significant differences were observed in the colonic morphological assessment. No significant differences were observed in the expression of tight junction-related genes in the proximal colon. However, the claudin-1 expression tended to increase and the claudin-3 expression tended to decrease in the distal colon of the HGF group. The Verrucomicrobiota in the gut microflora of the colon tended to increase in the HGF group. The abundance of most LPS-producing microbiota was lower in the HGF group than in the control group. The gene expression of TLR4 was significantly downregulated in the distal colon of the HGF group. CONCLUSION: HGF may enhance the mucus barrier through the tight junctions or gut microbiome in the distal colon.

Evaluation of skill acquisition characteristics depending on the size of a dry box.

BACKGROUND: Few studies have analyzed the effect of the size of the working space in training on the acquisition of endoscopic skills. In this study, adult- and infant-sized dry boxes (DBs) were used to verify how the size of the working space in training affects forceps manipulation and learning curve. MATERIAL AND METHODS: Seventy-two medical students were enrolled. The task was peg transfer. The training environment was divided into adult- and infant-sized DBs. Skill evaluations were also divided into adult- and infant-sized DBs (four groups in total). The forceps manipulation characteristics and task completion time were compared before and after training. RESULTS: Regarding skill evaluations using adult-sized DBs, there were no significant differences between the infant- and adult-sized DB-trained groups. Regarding skill evaluations using infant-sized DBs, there were no significant differences between the groups before training. After training, there was no significant difference in the total path length or average acceleration of the forceps between the groups. However, the infant-sized DB-trained group had a significantly faster average forceps velocity and faster task completion time than the adult-sized DB-trained group. CONCLUSION: Training with a small DB is more efficient in acquiring smoother and faster forceps manipulation in a small working space.

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).

Superiority of Intestinal Adaptation by Hepatocyte Growth Factor in the Jejunum: An Experimental Study in a Short-Bowel Rat Model.

BACKGROUND: We evaluated the effect of recombinant human hepatocyte growth factor (rh-HGF) on intestinal adaptation in a rat model of short-bowel syndrome (SBS). METHODS: Sprague-Dawley rats underwent jugular vein catheterization for continuous total parenteral nutrition (TPN) and 90 % small bowel resection. The animals were divided into 3 groups: TPN/SBS (control group, n = 7), TPN/SBS/intravenous recombinant human hepatocyte growth factor (HGF) (0.3 mg/kg/day) (HGF group, n = 7), and TPN/SBS/intravenous c-Met inhibitor (0.3 mg/kg/day) (anti-HGF group, n = 5). On day 7, rats were euthanized and histologically evaluated. Serum diamine oxidase (S-DAO) levels were evaluated using an enzyme-linked immunosorbent assay. The nutrient transporter and glucagon-like peptide-2 (GLP-2) receptor expression were evaluated using real-time polymerase chain reaction. RESULTS: The jejunal and ileal villus heights were higher and the S-DAO concentrations significantly higher (p = 0.04) in the HGF group than in the control and anti-HGF groups. The sodium-dependent glucose transporter 1 expression in the HGF group was significantly higher than in the control group and significantly suppressed in the anti-HGF group (p < 0.01). The peptide transporter 1 expression in the jejunum was higher in the HGF group than in the other groups and significantly suppressed in the anti-HGF group (p < 0.01). The GLP-2 receptor expression in the jejunum was higher in the HGF group than the other groups, and it was significantly suppressed in the anti-HGF group (p < 0.01). These jejunal results regarding nutrient transporter an GLP-2 receptor were not found in the ileum. CONCLUSIONS: The administration of rh-HGF appears to be more effective in the jejunum than in the ileum. TYPE OF STUDY: Experimental Research. LEVEL OF EVIDENCE: N/A.

Comparison of Transumbilical Laparoscopy-Assisted Appendectomy with Conventional Three-Port Laparoscopic Appendectomy Performed by Pediatric Surgeons in Training for Appendicitis in Children.

Background: Transumbilical laparoscopy-assisted appendectomy (TULAA) is the technique of choice for all types of appendicitis. However, the technique is challenging for trainees to learn in comparison with performing conventional three-port laparoscopic appendectomy (CTPLA) in children. We aimed to compare the surgical outcomes of children with appendicitis treated by TULAA versus CTPLA performed by pediatric surgeons in training (PSITs). Materials and Methods: This retrospective study analyzed pediatric patients with acute appendicitis treated with CTPLA or TULAA between April 2016 and December 2022. Operative time (OT: minutes), pneumoperitoneum time (PT: minutes), blood loss (milliliter), length of hospital stay (days), and surgical site infection rate were compared between the two groups. Operative outcomes were also analyzed according to type of appendicitis such as uncomplicated and complicated cases. Results: Two hundred twenty-five laparoscopic appendectomies were performed by CTPLA (n = 94) or TULAA (n = 131). All cases were performed by PSITs and there was no open conversion cases. TULAA had a shorter OT (67.0 ± 28.4 versus 78.3 ± 21.7; P < .01) and PT (26.1 ± 17.4 versus 52.5 ± 22.1 min; P < .01). The surgical site infection rate was slightly higher in the TULAA group, but the difference was not statistically significant. In uncomplicated appendicitis (n = 164), significant differences between the CTPLA and TULAA groups were observed in OT (CTPLA versus TULAA: 70.7 ± 14.9 versus 59.1 ± 21.6, P < .01) and PT (CTPLA versus TULAA: 43.6 ± 13.1 versus 20.4 ± 13.6, P < .01). With regard to postoperative complications, only surgical site infection was significantly different between the CTPLA and TULAA groups (CTPLA: 0.0% versus TULAA: 8.2%, P < .05). In complicated cases (n = 61), there were significant differences between the groups in PT (CTPLA versus TULAA: 73.4 ± 24.9 versus 42.3 ± 17.2, P < .01) and length of hospital stay (CTPLA versus TULAA: 7.0 ± 1.3 versus 8.9 ± 4.7, P < .05). Conclusions: TULAA had a shorter OT and PT than CTPLA. TULAA for PSITs shows similar safety and feasibility to CTPLA for not only uncomplicated cases but also complicated cases.

Ovarian endometrioma: a report of a pediatric case diagnosed prior to menstruation.

BACKGROUND: Ovarian endometriomas (OEs) are rarely found in the pediatric population, especially before menstruation. We report a 6-year-old girl who was postoperatively diagnosed with OE before menstruation. CASE PRESENTATION: A 6-year-old girl presented to a local pediatrician with abdominal pain and vomiting. Abdominal ultrasonography revealed a multilocular cystic lesion to the left of the bladder. Magnetic resonance imaging (MRI) revealed similar findings, with the contents of the cyst showing a low signal on T1-weighted imaging and a high signal on T2-weighted imaging. The patient was referred to our institution for further examination. Enhanced computed tomography (CT) showed a multilocular cystic lesion sized 56 × 44 × 30 mm with partial calcification. The left ovarian vein was dilated, suggesting the origin of the tumor to be the left ovary. Extirpation of the lesion was performed under laparoscopic assistance. Pathological findings indicated an ovarian endometrioma. To our knowledge, this is the youngest report of an OE diagnosed in a patient prior to menstruation. CONCLUSIONS: OEs in children before menstruation are extremely rare; thus, the long-term prognosis is yet to be determined.

Notable Clinical Differences Between Neonatal and Post-Neonatal Intestinal Malrotation: A Multicenter Review in Southern Japan.

BACKGROUND: Most cases of intestinal malrotation appear in neonates with bilious vomiting due to midgut volvulus, whereas in cases that develop beyond infancy, the initial symptoms vary. This study investigated the clinical features of these two populations and identified issues that should be considered in daily practice. METHODS: A retrospective chart review was conducted from January 1, 2010, to December 31, 2022. Data on patients with intestinal malrotation were collected in an anonymized fashion from five pediatric surgical hub facilities in the Southern Kyushu and Okinawa areas of Japan. RESULTS: Of the 80 subjects, 57 (71.3%) were neonates (Group N) and 23 (28.7%) were infants and schoolchildren (Group I). The frequencies of initial symptoms, such as abdominal distention (Group N: 19.3% vs. Group I: 13.0%), bilious vomiting (59.6% vs. 43.5%), and hematochezia (8.8% vs. 21.7%), were not skewed by the age of onset (p = 0.535, 0.087, and 0.141, respectively). Midgut volvulus was significantly more frequent in Group N (71.9% [41/57] vs. 34.8% [8/23]; p = 0.005), while the degree of torsion was greater in group I (median 360° [interquartile range: 180-360°] vs. 450° [360-540°]; p = 0.029). Although the bowel resection rate was equivalent (7.0% [4/57] vs. 4.3% [1/23]; p = 1.000), half of the patients in Group N presented with 180° torsion. The neonatal intestine has been highlighted as being more susceptible to ischemia than that in older children. CONCLUSIONS: The incidence of midgut volvulus is higher in neonates than in older children. Even relatively mild torsion can cause ischemic bowel changes during the neonatal period. LEVEL OF EVIDENCE: LEVEL III.