Although advances in pediatric surgery and perinatal care have improved survival rates for newborns with congenital disorders, achieving optimal long-term neurodevelopmental outcomes remains a significant challenge in modern neonatal medicine. Recently, attention has turned to the neurotoxicity of anesthetics on the developing brain. Basic research has revealed neuronal apoptosis and cognitive impairment. However, prospective human studies have shown that a single, short-term exposure to anesthesia does not clearly affect Intelligence Quotient, and the clinical significance of this effect remains a subject of ongoing debate. Neonatal neurodevelopment is influenced by many factors beyond anesthetics alone, including underlying diseases, patient backgrounds, and perioperative circulatory and respiratory dynamics. In this article, we summarize the latest findings on anesthetic neurotoxicity and outline perioperative management strategies that consider neonatal neurodevelopment.

Purpose: The Japanese Society of Pediatric Surgeons Guidelines Committee conducted an online survey to assess the usage and awareness of the “Guidelines for the Treatment of Intestinal Malrotation.” On the basis of the results of the questionnaire survey and the evaluation results from Minds, future challenges were examined.

Method: The survey was conducted among members of the Japanese Society of Pediatric Surgeons from June to September 2023. Minds evaluation was performed using The Appraisal of Guidelines for Research and Evaluation II.

Results: Responses were received from 104 members (5.0% response rate). Regarding awareness and usage, 37.2% stated they were aware of the guidelines and used them, and 41.3% were aware and intended to use them. Awareness was higher among those with more years of experience and who have treated more cases. Concerning the impact of each CQ on treatment policy, 59.6–75.0% responded that the policy was the same as the guidelines, and 3.8–7.7% reported a shift in policy after the guidelines. However, 5.3–22.1% noted that the policy differs from the recommendation but remains unchanged, especially regarding laparoscopic surgery (CQ4-4). Although the overall evaluation by Minds was high at 83%, the participation of stakeholders such as patients and their families was low at 56%.

Conclusion: The survey indicates that the guidelines have partially achieved their goal of supporting the treatment of intestinal malrotation. However, challenges for future revisions were revealed, such as a discrepancy between the actual situation and the guideline’s recommendations regarding laparoscopic surgery, and low participation from stakeholders.

Purpose: In this study, we aimed to analyze the cases of patients with solitary simple liver cysts diagnosed prenatally at three of our institutions and to consider appropriate treatment strategies based on the patients’ clinical course.

Methods: Patients prenatally diagnosed with solitary simple liver cysts (≥30 mm) at our three institutions over 10 years, from 2013 to 2022, were studied retrospectively.

Results: Six patients, two boys and four girls, were included in this study. The median time of prenatal diagnosis was 31 weeks of gestation (range, 28–38 weeks). The median gestational age was 40 weeks (38–41 weeks), and the median birth weight was 3,302 g (2,826–3,604 g). In all patients, a large unilocular cyst was detected on prenatal fetal and postnatal abdominal ultrasonography images. Contrast-enhanced computed tomography (CT) or magnetic resonance imaging (MRI) examination revealed no septations or communication with the biliary duct within the cyst; therefore, the lesions were clinically diagnosed as solitary simple hepatic cysts. Postnatal blood tests did not reveal any abnormalities in the hepatobiliary system in all the patients,and no gastrointestinal or respiratory symptoms were observed. However, laparoscopic hepatic cystectomy was performed on one patient at the age of 47 days because of concerns regarding intra-abdominal organ compression. The other five patients were followed up, and abdominal ultrasound showed that the cysts had shrunk over time and eventually almost disappeared.

Conclusions: Conservative management may be considered for prenatally diagnosed solitary liver cysts in neonates who remain asymptomatic after birth.

The patient was a boy, 19 days old. He came to the hospital with the chief complaint of vomiting and bloody stools, and the diagnosis was left inguinal hernia. An abdominal ultrasonography showed good intestinal blood flow, and the patient was manually repositioned. Contrast-enhanced CT of the abdomen showed intestinal dilatation and ascites, and gastrointestinal perforation was suspected, so an emergency laparotomy and partial resection of the small intestine were performed. However, postoperatively, abdominal compartment syndrome was observed, and a stoma was created to decompress the abdomen. However, because intra-abdominal decompression was insufficient and anuria persisted, a silo was created, and the patient was placed on continuous hemodiafiltration (CHDF) to remove inflammatory mediators. The patient was able to wean from CHDF in 9 days. He was discharged from the PICU on day 44 of hospitalization and discharged on day 97 of hospitalization.

The management of abdominal compartment syndrome was difficult, but silo placement and CHDF were effective, and we report this case.

Desmoid-type fibromatosis is an intermediate soft tissue tumor characterized by locally aggressive growth and a high rate of local recurrence. Pediatric cases are uncommon, and abdominal wall involvement in male children is particularly rare. We report the case of a 2-year-11-month-old boy with an abdominal wall desmoid who has remained recurrence-free for more than five years after surgery. A 14-mm intramuscular lesion was initially detected and managed with active surveillance. Because of its progressive enlargement to 34 mm over 20 months, surgical excision was performed at age 4 years and 9 months. Histopathology revealed spindle cell proliferation within a collagenous stroma and focal nuclear β-catenin expression, confirming desmoid-type fibromatosis. His postoperative course was uneventful, and no recurrence has been observed for over five years. Pediatric abdominal wall desmoids are rarely reported, and associations among tumor location, age, sex, and recurrence remain unclear. Recent studies highlight the prognostic relevance of CTNNB1 mutations, particularly the S45F variant, which has been associated with a higher risk of local recurrence. Although current guidelines recommend active surveillance as initial management, surgical intervention remains appropriate for symptomatic or progressively enlarging tumors. This case demonstrates that the timely surgical excision of a growing pediatric abdominal wall desmoid can achieve favorable long-term disease control.

A 17-year-old male with a history of an infected anterior mediastinal cyst treated conservatively presented with chest pain two years later. He was hospitalized for suspected reinfection. Because of worsening pleural and pericardial effusions, thoracic drainage was performed, and he was transferred to our department. Although antibiotics and drainage provided temporary relief, inflammation and fluid accumulation recurred immediately upon treatment cessation. Despite pericardiocentesis and re-drainage, inflammation persisted. Given the negative culture results, chemical pleuritis associated with a ruptured cystic teratoma was suspected. Prednisolone administration subsequently improved his symptoms. Elective surgery was performed. The tumor was found to be firmly adhered to the pericardium, necessitating partial pericardiectomy. The pathological diagnosis was mature cystic teratoma. His postoperative course was uneventful, with no recurrence at 2 years and 11 months. In young patients with mediastinal tumors accompanied by inflammation, ruptured teratoma should be included in the differential diagnosis, and early surgical intervention should be considered after the inflammation has subsided.

The patient was a female infant born at term with megacystis detected during the fetal period. She had good feeding and bowel movements after birth. Intermittent catheterization was initiated, and she was discharged at 27 days of age. Constipation developed around 7 months of age, leading to the initiation of enemas. Gradually, abdominal distention and intestinal dilatation became prominent, and she repeatedly had enterocolitis starting at 1 year of age. A rectal mucosal biopsy ruled out Hirschsprung disease. After transfer to our hospital, an ileostomy was created, followed by a gastrostomy for intestinal decompression. At 1 year and 11 months, ileostomy intussusception occurred, requiring partial ileal resection and ileostomy reconstruction. Subsequent nutrition was managed solely by oral intake. Detailed testing identified an ACTG2 gene mutation, leading to a diagnosis of visceral myopathy. Mutations in the ACTG2 gene encoding smooth muscle actin gamma 2 are termed visceral myopathy and are also a cause of megacystis microcolon intestinal hypoperistalsis syndrome (MMIHS). Although this case, which presented with intestinal obstruction symptoms in late infancy, did not meet the Japanese diagnostic criteria for MMIHS, it corresponded to visceral myopathy or ACTG2-related disease: conditions classified within the same category as MMIHS in international literature. Genetic testing may aid in reevaluating the definition and classification of chronic intestinal pseudo-obstruction (CIPO).

Intestinal malrotation typically presents as bilious vomiting in the neonatal period; however, it can also manifest with nonspecific abdominal symptoms. We report the case of a 9-year-old boy with Pfeiffer syndrome. He had a history of recurrent abdominal distension and vomiting, which had been managed conservatively as symptoms secondary to his underlying conditions, such as aerophagia, without exhaustive investigation. At age 9, his symptoms exacerbated. Contrast-enhanced abdominal CT revealed intestinal malrotation, and a Ladd procedure was performed. Postoperatively, the patient experienced delayed gastric emptying due to aerophagia, but was successfully discharged on postoperative day 57 following management that included nasojejunal nutrition. He has remained asymptomatic since then. It is essential to recognize that fibroblast growth factor receptor-related disorders, including Pfeiffer syndrome, are associated with an exceptionally high incidence of intestinal malrotation. For abdominal symptoms in children with complex underlying conditions, clinicians should not reflexively attribute manifestations to the primary disease. Maintaining a high index of suspicion for organic diseases and performing proactive diagnostic evaluations is crucial for ensuring timely intervention.

Identification of the rectal blind pouch during posterior sagittal anorectoplasty (PSARP) for anorectal malformation with rectourethral fistula can be difficult, especially when the rectal blind pouch is located high in the pelvis. Tamguide^® emits biologically transparent red light that can be recognized through soft tissue and may help localize the blind pouch. We performed PSARP at 9 months of age in two boys with anorectal malformation and rectourethral fistula who had undergone sigmoid colostomy in the neonatal period. A Tamguide Fiber^® (outer diameter, 0.5 mm) was inserted through the distal stoma and advanced to the rectal blind pouch under fluoroscopic guidance. By using the emitted red light as an intraoperative landmark, we identified the rectal blind pouch 11 and 12 minutes after the start of the posterior sagittal incision in Patients 1 and 2, respectively. In Patient 2, partial injury to the membranous urethra occurred and required repair; however, no postoperative urethral stricture or leakage developed. Neither patient showed refistulization or other postoperative complications. Tamguide Fiber^® was a simple and practical adjunct for identifying the rectal blind pouch during PSARP.

Although early surgery is critical for the prognosis of biliary atresia (BA), determining the optimal timing of surgery can be challenging in very low birth weight (VLBW) and extremely low birth weight (ELBW) infants because of their immaturity and small body size. We report two cases of BA in VLBW and ELBW infants with different clinical courses depending on the timing of treatment. Case 1 was a male infant born at 31 weeks and 6 days of gestation with a birth weight of 1,363 g. Elevated direct bilirubin levels persisted following surgery for meconium-associated intestinal obstruction. He was diagnosed as having BA at 185 days of age and underwent Kasai portoenterostomy (KPE) at 208 days of age; however, his jaundice did not resolve, and he is currently being followed with consideration for liver transplantation. Case 2 was a female infant born at 29 weeks and 3 days of gestation with a birth weight of 876 g. BA was suspected early and diagnosed at 51 days of age. KPE was performed at 58 days of age when her weight was 1,780 g, resulting in good resolution of jaundice. This case was the lowest weight at the time of surgery among those with good native liver survival. In low-birth-weight infants, differentiation from parenteral nutrition-associated cholestasis is often difficult, and prioritizing body weight at surgery may delay treatment initiation. The cases suggest the importance of early surgical intervention based on postnatal age.

We report a rare pediatric case of pneumatosis cystoides intestinalis (PCI) complicated by right-sided colonic volvulus. The patient was a 3-year-old girl with trisomy 18. She had required repeated hospitalizations over the preceding 8 months due to nausea and abdominal distension, which consistently improved within approximately one week with bowel rest. Imaging studies conducted during this period demonstrated PCI; however, no definitive evidence of obstruction or volvulus was identified, and she was managed conservatively. On the second day of her eighth admission, she developed a fever, tachycardia, and worsening abdominal distension. Contrast-enhanced computed tomography revealed PCI extending from the terminal ileum to the right transverse colon, along with a whirlpool sign, leading to a diagnosis of strangulated bowel obstruction due to right-sided colonic volvulus. Emergency surgery revealed right-sided colonic volvulus around the mesenteric axis and marked pneumatosis of the bowel wall, and a right hemicolectomy was performed. Her postoperative course was uneventful, and she remains free of recurrent abdominal symptoms one year after surgery. Although most pediatric cases of uncomplicated PCI resolve with conservative management, cases complicated by colonic volvulus, as in the present case, can occur. Careful reassessment in response to changes in abdominal findings is therefore essential.

Desmoid-type fibromatosis is a rare fibroblastic tumor without metastatic potential but with marked local aggressiveness. We report a case of a 14-year-old boy who had experienced dyspnea for 8 months and had been treated for bronchial asthma without improvement. Contrast-enhanced computed tomography revealed a 20-cm mass occupying the right thoracic cavity, with leftward mediastinal shift, marked narrowing of the right main bronchus, and collapse of the right lung. Core needle biopsy confirmed desmoid-type fibromatosis. As his respiratory status worsened and the risk of asphyxia became imminent, the patient was managed as an oncologic emergency. Extracorporeal circulatory support was established under awake conditions, followed by surgery. Approximately 80% of the tumor was removed while preserving the involved vital structures, resulting in relief of the airway obstruction. Postoperatively, pazopanib induced slight shrinkage of the residual tumor, but treatment was discontinued because wound dehiscence developed as a suspected adverse effect, and therapy was switched to methotrexate/vinblastine. Pazopanib was resumed 11 months after surgery, and the patient has remained free from regrowth for 47 months postoperatively. In addition, we conducted a literature review of 95 pediatric cases of desmoid-type fibromatosis reported in Japan.

Cloacal exstrophy (CE) is an extremely rare congenital anomaly, and individualized urinary reconstruction is required owing to the wide anatomical variability among patients. We report a case of CE with a hypoplastic bladder plate (BP) on which urinary reconstruction was performed using a neobladder created from a dilated hindgut. The patient was a female infant born at 32 weeks and 3 days of gestation with a birth weight of 1,391 g. In the neonatal period, she underwent repair of an omphalocele and creation of an ileostomy, whereas the hindgut, whose patency was uncertain, was preserved. At the age of 3 years, urinary reconstruction was performed by replacing the hypoplastic native bladder tissue with a neobladder constructed from the dilated hindgut. A catheterizable channel with an anti-reflux mechanism using a submucosal tunnel and ureteral reimplantation was also created. Postoperatively, an age-appropriate bladder capacity and urinary continence have been achieved. In selected cases of CE with a hypoplastic BP and a dilated hindgut, the use of a hindgut-derived neobladder may represent a useful option for urinary reconstruction.

Testicular germ cell tumors (GCTs) complicated by inferior vena cava (IVC) tumor thrombus are extremely rare, and no pediatric cases have been reported to date. We report the case of a 14-year-old boy with non-seminomatous testicular GCT presenting with multiple distant metastases and extensive IVC tumor thrombus at diagnosis. Following high inguinal orchiectomy, the patient received four cycles of bleomycin, etoposide, and cisplatin (BEP) chemotherapy. Serum tumor markers returned to normal levels, and imaging demonstrated marked regression of the IVC tumor thrombus. However, residual lesions persisted in the retroperitoneal lymph nodes and liver. Because the patient had postpubertal disease with residual masses after chemotherapy, an adult treatment strategy was adopted. Through multidisciplinary discussion involving pediatric surgeons, pediatric oncologists, urologists, gastrointestinal surgeons, and cardiologists, retroperitoneal lymph node dissection and partial hepatectomy were performed. No residual tumor thrombus was identified within the IVC intraoperatively, and IVC resection was not required. Histopathological examination of the resected specimens revealed no viable tumor cells, indicating a pathological complete response. To the best of our knowledge, this is the first reported pediatric case of testicular GCT associated with IVC tumor thrombus. Because adolescent testicular GCTs share biological and clinical characteristics with adult tumors, treatment strategies based on adult guidelines may be appropriate. This case highlights the importance of multidisciplinary decision-making when managing rare and complex adolescent GCTs with extensive venous involvement.