Objective Studies investigating growth impairment (GI) in children with intestinal failure (IF) have seldom reported its associated factors. We hypothesized that a deficient nutritional intake would affect GI in children with IF. Methods Nineteen patients (2–16 years old) who underwent home-based parenteral nutrition (PN) management at our institution, were divided into patients with or without GI (GI+/GI–). GI was defined as a height-for-age z-score (HAZ) ≤ −2. We performed between-group comparisons of HAZ in the intestinal rehabilitation program (IRP)/nutrition support team (NST) introduction time, PN dependency, residual small intestine length (RSIL), nutritional intake (calorie, protein, lipids, and carbohydrates), caloric intake ratio, and nutrient intake as a percentage of the recommended intake. Results Six patients were in the GI+ group. PN dependency was significantly higher in the GI+ group (GI+, 109.8 %; GI < median, 61.7 %, P = 0.036). RSIL did not differ significantly between the groups (50 cm vs. 45 cm, P = 0.879). In the GI+ group, the caloric percentage of lipids was significantly lower (11.9 % vs. 17.5 %, P = 0.002), but that for carbohydrates was significantly higher (76.8 % vs. 66.8 %, P = 0.002), while lipid sufficiency was significantly lower (37.5 % vs. 89.6 %, respectively; P < 0.001). Protein intake in the GI+ group was significantly lower; however, the corresponding sufficiency was > 100 % in both groups (136.5 % vs. 209.2 %, P = 0.005). Conclusion For children with IF, a low intake ratio of proteins and lipids relative to energy requirement and required nutrient levels may inhibit adequate growth.

Short bowel syndrome (SBS) is the leading cause of intestinal failure, frequently necessitating long-term parenteral nutrition (PN). Teduglutide (TED), a glucagon-like peptide-2 analog, has demonstrated efficacy in reducing PN dependence in both adults and children. However, long-term data in pediatric populations remain limited. We present a case of a male child with SBS who underwent extensive small bowel resection at 5 days of age, resulting in a residual small bowel length of 9 cm, with the total colon and ileocecal valve preserved. Despite home PN and multiple interventions, including management of catheter-related infections and intestinal complications, PN dependence persisted. At the age of 5 years, the patient was enrolled in a TED clinical trial and continued treatment after its commercial approval. During >6 years of TED therapy, gradual and sustained reductions in PN volume and caloric intake were achieved, ultimately resulting in complete weaning from PN. Growth and nutrition status remained stable, and no severe TED-related adverse events were reported. This is the first known case documenting TED use for >6 years in a pediatric patient with SBS. Although the response was slower than in previously reported cases, long-term TED administration led to favorable outcomes, including PN independence. This case underscores the potential for TED to support intestinal adaptation over extended periods and highlights the importance of individualized, long-term treatment strategies in managing severe pediatric SBS.

PURPOSE:This study aimed to clarify the incidence of portal hypertension (PH) in biliary atresia (BA), its impact on long-term native liver survival rates (NLSRs), and the role of PH treatment. METHODS:Data were analyzed from 3,777 patients in the Japanese Biliary Atresia Registry (1989-2021). Incidence and treatment of PH were examined in all patients and in 20-year native liver survivors. A subgroup of 596 jaundice-free native liver survivors was divided into three groups: without PH (A), untreated PH (B), and treated PH (C). Patient characteristics, clinical outcomes, and survival analyses were performed. RESULTS:PH occurred in 46.4% of all patients, with gastroesophageal varices (35.1%) and hypersplenism (36.8%) being most common. Among 759 20-year native liver survivors, 49.8% had PH, largely diagnosed between 2 and 15 years. Group C patients were older at Kasai portoenterostomy and had more cholangitis. Survival analysis showed best outcomes in group A and worst in group C. Conditional native liver survival analysis revealed group B initially had better NLSRs than group C, but differences diminished beyond 15 years. CONCLUSION:In native liver survivors without jaundice following KP, PH influenced prognosis, but successful treatment enabled long-term survival comparable to patients with mild or no PH.

Patients with intestinal failure (IF) require long-term parenteral nutrition using central venous catheters (CVCs), which often require replacement. We adopted a less fallible guidewire replacement (GWR) method and verified its effectiveness and validity. We enrolled 108 cases that underwent a CVC replacement with “GWR” method with IF at our department between 2013 and 2023. We retrospectively reviewed patients’ clinical details with tunneled CVC (Hickman/Broviac catheter). For the analysis, we compared for the same time period the catheter exchange method “Primary placement”; newly inserted catheter by venipuncture. The success rate of catheter replacement using GWR was 94.4

Abstract Purpose This study analyzed the efficacy of web excision combined with a pre-membranous incision on the dilated proximal segment for congenital intestinal atresia with type I and stenosis (CIA-I/S). Patients and methods Twenty-six patients underwent surgery for CIA-I/S from January 1990 to June 2022. Patients were categorized into 3 groups according to the surgical procedure: Group A, web excision with pre-membranous incision of the dilated intestine (n = 14); Group B, enteroplasty with a trans-membranous vertical incision (n = 7) and Group C, diamond-shaped anastomosis (n = 5). To minimize the impact of obstruction location on outcomes, we specifically examined 17 cases of duodenal atresia/stenosis: Group D-A, (n = 6); Group D-B, (n = 6) and Group D-C, (n = 5). We retrospectively compared the operative and postoperative parameters among the three groups. Results No patient experienced anastomotic leakage or obstruction. There were no significant differences in operative duration or blood loss among the 3 Groups. The median time to feeding initiation was 4, 6.5, and 5 days in Groups A, B, and C, respectively (p = 0.04) and was 4, 6.5, and 5 days in Groups D-A, D-B, and D-C, respectively (p = 0.04). Conclusion Web excision, when compared to enteroplasty and diamond-shaped anastomosis, showed comparable results in terms of the operative duration and postoperative complications. However, it may allow for an earlier initiation of enteral nutrition.

Background: Although congenital portosystemic shunts (CPSSs) are increasingly being recognized, the optimal treatment strategies and natural prognosis remain unclear, as individual CPSSs show different phenotypes. Methods: The medical records of 122 patients who were diagnosed with CPSSs at 15 participating hospitals in Japan between 2000 and 2019 were collected for a retrospective analysis based on the state of portal vein (PV) visualization on imaging. Results: Among the 122 patients, 75 (61.5%) showed PV on imaging. The median age at the diagnosis was 5 months. The main complications related to CPSS were hyperammonemia (85.2%), liver masses (25.4%), hepatopulmonary shunts (13.9%), and pulmonary hypertension (11.5%). The prevalence of complications was significantly higher in patients without PV visualization than in those with PV visualization (P < 0.001). Overall, 91 patients (74.6%) received treatment, including shunt closure by surgery or interventional radiology (n = 82) and liver transplantation (LT) or liver resection (n = 9). Over the past 20 years, there has been a decrease in the number of patients undergoing LT. Although most patients showed improvement or reduced progression of symptoms, liver masses and pulmonary hypertension were less likely to improve after shunt closure. Complications related to shunt closure were more likely to occur in patients without PV visualization (P = 0.001). In 25 patients (20.5%) without treatment, those without PV visualization were significantly more likely to develop complications related to CPSS than those with PV visualization (P = 0.011). Conclusion: Patients without PV visualization develop CPSS-related complications and, early treatment using prophylactic approaches should be considered, even if they are asymptomatic. (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

Introduction: Intestinal mobility disorder (MD) is a major cause of severe intestinal failure requiring intestinal transplantation (ITx), especially in Asian countries. This study aimed to assess the outcome of ITx for patients with MD. Methods: Between 2003 and 2022, 11 patients underwent ITx at our institution. Underlying MD was identified in eight patients, and short gut syndrome in the remaining three. Patients with MD were divided into two groups according to Era: Era I (2003-2010, n=4) and Era II (2011-2022, n=4). A retrospective review was performed to evaluate the patient characteristics, operative data, patient and graft-survival rates, and incidence of complications, including acute rejection (AR). Results: Patient’s age at the time of primary ITx in Era I was higher than in Era II (median, 20 years vs. 15 years). In Era I, hypoganglionosis (n=2) and chronic idiopathic intestinal pseudo-obstruction (CIIPS) (n=2) were diagnosed, while in Era II, hypoganglionosis (n=1), CIIPS (n=1), megacystis microcolon intestinal hypoperistalsis syndrome (n=1), and extensive aganglionosis (n=1). An induction immunosuppressive treatment included an interleukin-2 receptor antagonist in Era I and rabbit antithymocyte globulin in Era II. Furthermore, the donor type was living (n=2) and cadaveric (n=2) in Era I and all cadaveric (n=4) in Era II. Median durations of follow-up after ITx were 102 and 81 months in Era I and II, respectively. One-, 5-, and 10-year patient survival rates were 100%, 75%, and 44.4% in Era I and 100%, 100%, and 100% in Era II, respectively (p=0.1). Three patients in Era I died: one due to sepsis, another due to abdominal bleeding, and the other due to acute pancreatitis. In comparison, 1-, 5-, and 10-year graft survival rates were 75%, 50%, and 25% in Era I and 100%, 100%, and 100% in Era II, respectively (p=0.07). The incidences of moderate or severe AR and medical complication including renal deficiency and diabetes mellitus, were 100% and 50%, respectively, in Era I and 50% and 75%, respectively, in Era II. Three patients (one in Era I; two in Era II) were completely weaned from parenteral nutrition. Conclusions: The ages and underlying disease of patients had enlarged by Era. Although patient- and graft-survival rates in Era II were superior to those in Era I, the incidence of AR and medical complications were similar between Era.

The incidence and risk factors of catheter-related bloodstream infections (CRBSI) in patients with intestinal failure (IF) have not been established, partly because catheter management methods vary from different facilities. This study aimed to identify the risk factors and incidence rate of CRBSIs in patients with IF who were given prophylactic treatment. Sixteen patients with IF who required home parenteral nutrition were enrolled in this study. Prophylactic management of CRBSI included monthly ethanol lock therapy and standardized infection prevention education. The outcomes included the incidence and risk factors of CRBSI. The median incidence rate of CRBSI was 1.2 per 1000 catheter days. Univariate analysis showed that the risk of developing CRBSI was significantly associated with short bowel syndrome (< 30 cm) (p = 0.016). Other relevant findings included a significant negative correlation between serum albumin and CRBSI rate (r = − 0.505, p = 0.046), and past history of mixed bacterial infections was significantly associated with increased CRBSI rate (p = 0.013). CRBSIs can still develop despite undergoing prophylactic management. Risk factors for CRBSI include the residual intestinal length, nutritional status, and susceptibility to certain microorganisms.

Pleuropulmonary blastoma (PPB) is a rare tumor that arises from mesenchymal cells of the lung or pleura. PPB is classified as type I (cystic), type Ir (regressed), type II (cystic and solid), or type III (solid). Here, we report the case of a 2-year-old boy with type II PPB. The patient was treated with neoadjuvant chemotherapy, and the tumor was completely surgically resected. A DICER1 mutation was detected in the tumor specimen. The risk of recurrence is high, and long–term follow–up is required.

[Background] Ovarian teratomas are the most common ovarian tumors in children. Fertility-sparing surgery (FSS) is often recommended owing to its advantage of fertility preservation. We examined the validity of FSS in pediatric patients with ovarian teratomas. [Procedure] We retrospectively reviewed the medical records of patients who underwent initial surgery for ovarian teratomas in our department between 1972–2021. Patients were divided into two groups: oophorectomy (OVX) group and FSS group. Clinical characteristics, perioperative characteristics, and outcomes were compared between the groups. [Results] Forty-six patients underwent initial surgery (OVX: 25 patients; FSS: 21 patients). There were no differences between the groups in terms of age, pathological diagnosis, affected side, ovarian torsion, postoperative complications, recurrence rate, or length of postoperative follow-up. Regarding pathological diagnosis, immature teratomas were found only in the OVX group (p = 0.0161). The tumor size was larger in the OVX group (p = 0.0104). Regarding perioperative findings, operation time was longer in the FSS group (p = 0.0071) and bleeding volume was higher in the OVX group (p = 0.0070). The number of days until the start of oral intake and the length of hospital stay postoperatively were shorter in the FSS group (p = 0.0002 and p < 0.0001, respectively). [Conclusion] We demonstrated the advantages of FSS, including its minimally invasive approach and comparable outcomes in terms of tumor recurrence; the operation time was longer. Since both FSS and OVX are associated with a risk of tumor recurrence, long-term follow-up in anticipation of adult transition is necessary.

A 7-year-6-month-old female was diagnosed with a pelvic malignant peripheral nerve sheath tumor and lymph node metastases. Tumorectomy was performed after four cycles of chemotherapy. A 33-mm cystic lesion was observed around the left iliac muscle after three cycles of postoperative chemotherapy, and proton beam therapy (PBT) was recommended. She was referred for absorbable spacer (AS) placement. The left ovarian appendage (OA) was resected due to the direct tumor infiltration. The right OA was fixed to the uterosacral ligament. The AS was fixed to the lateral pelvis. The PBT (70.3 Gy relative biological effectiveness) was performed successfully with the AS, and she also had the reproducing possibility due to prevention of severe irradiation damage of the right OA. AS eliminated the surgical removal of spacers and enabled us high-dose PBT for residual tumor without severe irradiation damage including infertility.

Pseudo-Meigs syndrome (PMS) and gliomatosis peritonei (GP) are rare conditions associated with ovarian teratomas. We report the case of an 11-year-old female patient suffering from immature ovarian teratoma with PMS and GP. Enhanced computed tomography (CT) revealed a tumor measuring 240 × 150 × 100 mm in the lower abdomen, ascites, and right pleural effusion. Before the operation, the right pleural effusion increased and required chest tube drainage. Right adnexectomy was performed, and peritoneal nodules were detected during surgery. A biopsy was performed and pathological examination revealed a right immature ovarian teratoma (Grade 3) and glial tissue (Grade 0), thus confirming the diagnosis of GP. Postoperatively, ascites and pleural effusion disappeared, confirming the diagnosis of PMS. Eight months after the operation, a metachronous contralateral mature ovarian teratoma developed, for which fertility-sparing surgery was performed. Therefore, long-term follow-up is required in this patient.

Pheochromocytoma (PCC) and paraganglioma (PGL) are rare neuroendocrine tumors that arise from chromaffin cells. We report the case of an 11-year-old boy with no history of hypertension who presented with an abdominal mass arising from the retroperitoneum. During laparoscopic surgery, the patient developed a hypertensive crisis with a peak systolic blood pressure of over 270 mmHg, although we were able to continue to perform laparoscopic resection. Even if no concomitant symptoms due to catecholamine production are observed before surgery, as in this case, blood pressure may fluctuate during surgery. Intraoperative and postoperative management should be considered in cooperation with anesthesiologists.

Background. This study aimed to compare the clinical outcomes of intestinal transplantation (ITX) between 2 groups by using medications for induction treatment and assess the utility of the current protocol. Methods. From 2003 to 2020, 11 patients underwent ITX. Recipients were classified into 2 groups: group IL-2Ra (interleukin-2 receptor antagonist therapy, n = 6) and ATG (rabbit antithymocyte globulin therapy, n = 5). We conducted a retrospective review of patient and graft survival rates and the postoperative course. Results. The 1-, 5-, and 10-year patient and graft survival rates of the 11 primary grafts in the 11 recipients were 100%, 88.9%, 62.2% and 90.0%, 78.8%, 56.3%, respectively. The median duration of follow-up for the IL-2Ra and ATG groups was 197.3 and 87.3 months, respectively. The 1-, 5-, and 10-year patient survival rates were 100%, 83.3%, 50% and 100%, 100%, 100% for the IL-2Ra and ATG groups, respectively (P = .25) and 83.3%, 66.7%, 33.3% and 100%, 100%, 100% for graft survival in the IL-2Ra and ATG groups, respectively (P = .08). The incidence of moderate and severe acute rejection was 100% and 20% in the IL-2Ra and ATG groups, respectively (P = .02). The 1- and 5-year moderate and severe rejection-free survival rates were 33.3%, 0% and 80%, 80% in the IL-2Ra and ATG groups, respectively (P = .04). Conclusions. ATG significantly suppressed moderate and severe acute rejection compared with IL-2Ra, thereby showing better short- and mid-term rejection-free survival rates. Additional clinical experience is needed to determine the optimal regimen for the management of ITX recipients.

Benign retroperitoneal teratomas are relatively rare tumors that usually progress slowly and are found in newborns and young adults. Here, we report a case in which a prenatally diagnosed retroperitoneal tumor rapidly increased in diameter from 55 mm to 90 mm during the neonatal period. The tumor was removed while preserving the surrounding organs. Moreover, the patient's postoperative course was uneventful. Pathological examination revealed an immature teratoma with no malignant findings. Notably, even benign neonatal teratomas may exhibit rapid growth of their cystic components after birth.

A two-year-old girl presented with an abdominal mass, abdominal pain, and fever. A quail egg-sized elastic-hard mass was palpable in the upper left abdomen. The patient's growth was impaired. Laboratory data showed an inflammatory response and elevated pancreatic enzymes. The mass showed a cystic structure adjacent to the caudal side of the bifid tail of the pancreas. A fluid collection was observed around the spleen. Surgery was planned under the diagnosis of pancreatic foregut cystic malformation (FCM) associated with a bifid pancreatic tail. However, as the inflammation worsened, parenteral nutrition (PN) was introduced and cyst drainage was performed first, obtaining 10–14 mL/day, and her symptoms subsided. Cyst drainage continued for six weeks at home. Laparoscopic surgery was performed 47 days after the initiation of drainage. Cystography showed communication between the cyst and the slightly dilated pancreatic duct of the bifid tail during surgery. A partial pancreatectomy with cystectomy was performed. The diagnosis of FCM originating from the pancreas, associated with the bifid tail, was confirmed. The postoperative course was uneventful, and the patient was discharged eight days post-surgery. Pancreatic FCM with a bifid pancreatic tail is rare, and we successfully treated it using minimally invasive surgery.

A 10-month-old girl who had previously undergone Kasai portoenterostomy (KP) for biliary atresia was admitted because of recurrent jaundice. Intrahepatic bile duct (IHBD) dilation was observed in the lateral segment close to the umbilical portion of the portal vein on ultrasonography and computed tomography. Although percutaneous transhepatic biliary drainage by a radiologist alleviated jaundice, internal fistula formation, which was subsequently attempted, was unsuccessful due to the narrow and complicated IHBD morphology. Therefore, side-to-side cholangiojejunostomy between the dilated IHBD and jejunum of the Roux-en-Y limb was performed using intraoperative interventional radiology techniques. The patient's postoperative course was uneventful. In patients with recurrent jaundice after KP, especially that associated with solitary IHBD dilatation, a high success rate of achieving good bile flow can be expected using current techniques.