In the field of urology, diagnoses have traditionally been made using rigid instruments such as cystoscopes. With the development of endoscopic equipment, transurethral surgery has advanced. In pediatric urology, transurethral surgery is frequently performed for the treatment of bladder tumors, bladder stones, urethral stones, ureteroceles, and posterior urethral valves. Vesicoureteral reflux (VUR) prevention surgery has long been centered on open surgery, but the advent of endoscopic injection therapy and pneumovesicoscopic surgery has expanded treatment options. In 2024, laparoscopic surgery using an extravesical approach was covered by medical insurance in Japan. In the near future, if robotic surgery is covered by medical insurance, it is expected that VUR prevention surgery will undergo significant changes. Within this context, the role of pneumovesicoscopic surgery is also expected to evolve. For conditions such as megaureter, ureterocele, and ectopic ureter, intravesical manipulation and approaches to the posterior bladder wall via the bladder are considered effective and safe, thus maintaining the value of pneumovesicoscopic surgery.
Objective: The long-term efficacy and safety of MACROGOL 4000, sodium chloride, sodium bicarbonate, and potassium chloride (Movicol®) administered to children with chronic constipation were determined.
Methods: A retrospective observational study was conducted on chronic constipation patients aged 2 to 14 years who were newly administered Movicol® and continued treatment for more than 4 weeks at the Department of Pediatric Surgery, Kyorin University Hospital from December 1, 2018 to December 31, 2022. Observation and examination data were obtained from the patients' medical records and responses to questionnaires. Outcome measures included the number of defecations and stool shape determined on the basis of the Bristol Stool Form Scale (BSFS) before and after Movicol® administration, as well as adverse events and side effects. Stratified analyses were conducted by age, previous treatment, and enema use.
Results: Ninety-eight eligible patients were selected for analysis. The mean duration of treatment with Movicol® was 116.0 ± 78.4 weeks. Four weeks of Movicol® treatment significantly increased the number of defecations (times/week) from 1.2 ± 1.0 to 5.4 ± 4.7 (P < 0.001). Stool shape, as determined by the BSFS, improved and was maintained for more than 60 weeks. The frequency of enema use decreased significantly in patients who used enemas. The efficacy of Movicol® was unchanged in all subgroups based on age, previous treatment, and using an enema. The mean duration of treatment with Movicol® was more than two years, and no serious adverse events or side effects were observed.
Conclusion: Long-term administration of Movicol® in children with chronic constipation is an effective and safe treatment option and can be considered as first-line therapy.
Purpose: In the revision of the clinical practice guidelines (CPGs) for biliary atresia, we investigated the actual state of medical practice before and after the publication of the CPGs and examined its impact on standardizing medical practice.
Methods: A survey was conducted among facilities affiliated with the Japanese Biliary Atresia Society to examine clinical practices and CPGs usage before and after its publication.
Results: Responses were collected from 76 facilities before and 84 after the publication of the CPGs. Over the past five years, 24% of the facilities had no surgical experience, whereas 43% managed 1–4 cases and 27% handled 5–14 cases. Over 80% of the respondents that reported using CPGs expressed satisfaction with the clinical questions, appropriateness, clarity, and usefulness of the algorithm. The use of the stool color card, recommended by the CPGs, increased; moreover, the number of facilities conducting liver biopsies, despite being “not recommended” preoperatively, increased. The importance of early surgery was more widely recognized after publication, with 41% supporting earlier surgery and 19% supporting surgery within 30 days of birth. Laparoscopic surgery remained rare, with only 3% of facilities performing it universally, whereas 92% did not perform it at all. Postoperative steroid use, advised against by the CPGs, decreased slightly from 89% to 82%. The number of facilities using the pediatric end-stage liver disease (PELD) score for primary liver transplantation has also decreased.
Conclusions: The CPGs is being utilized, and future revisions incorporating additional evidence are required to further standardize care and improve treatment outcomes.
We present the case of a boy with multiple thyroglossal duct cysts (TDCs). At 1 year of age, a mass was noted in the midline of the neck. Computed tomography revealed a cyst caudal to the hyoid bone, leading to the diagnosis of a solitary TDC. At 2 years of age, ultrasonography showed three small, low-echoic lesions above the hyoid bone, and magnetic resonance imaging revealed two cysts adjacent to the caudal aspect of the hyoid bone. Surgery was performed at 2 years and 2 months of age. After detaching the hyoid bone, the primary cyst attached to the hyoid bone and one small cyst were confirmed; however, one cyst remained along the usual fistula detachment line and could not be visualized. Residual cysts embedded within the lingual muscles were identified by intraoperative ultrasonography, and “core out” was performed to excise the cysts completely. Most cases of TDC involve solitary cysts that occur caudal to the hyoid bone; multiple cysts are extremely rare. In cases involving multiple cysts, particularly small cysts within the lingual muscles, changes in the positional relationship between the hyoid bone and cysts may occur during surgery, leading to the possibility of cyst remnants at the usual detachment line. In such cases, intraoperative ultrasonography is useful for identifying cysts and fistulas within the lingual muscles.
The puborectalis muscle and anal sphincters are essential for maintaining fecal continence; however, they are underdeveloped in cases of high anorectal malformations (ARMs), posing a risk of long-term postoperative fecal incontinence. We report the application of an anal canal plastic procedure, initially developed for elderly patients with idiopathic fecal incontinence and later applied to patients with chronic fecal incontinence after surgery for Hirschsprung disease, to patients suffering from intractable long-term fecal incontinence following anorectoplasty for high ARMs. The study included three patients (one male and two females) diagnosed as having high ARMs (imperforate anus, rectoprostatic fistula, and persistent cloaca). The patients’ ages at the time of the anal canal plastic procedure ranged from 11 to 24 years. Preoperative bowel function scores, based on the Japanese Study Group of Anorectal Anomaly (JSGAA) scoring system, ranged from 0 to 1. Preoperative imaging revealed anal canal dilation in all patients, with an inability to hold the contrast material. After the anal canal plastic surgery, bowel function scores improved to 5–7, and postoperative imaging showed no anal canal dilation, with the successful holding of the contrast material in all patients. Our results indicate that this procedure is also effective in managing fecal incontinence after anorectoplasty for high ARMs.
Intra-abdominal cysts are often identified during the fetal period, but cysts occupying the left upper quadrant are rare. We report a case of a congenital splenic cyst that was difficult to diagnose on the basis of preoperative findings and required operation during the neonatal period. The patient was a girl in whom an intra-abdominal cyst was detected by prenatal ultrasonography. A fetal magnetic resonance imaging and postnatal abdominal ultrasonography revealed a cystic lesion in the left upper quadrant, but a diagnosis could not be made. Because the baby continued to vomit, she underwent laparotomy on the 8th day of age and a splenic cyst was found. To minimize the resection surface, a dome resection was performed, which allowed the splenic volume to be maintained without any complication. Her platelet count had increased during the postoperative course but almost normalized six months after surgery. She grew up without any overwhelming infection or recurrence of the cyst. A case of splenic cyst requiring treatment during the neonatal period is extremely rare, but early intervention for symptomatic intra-abdominal cysts should be considered regardless of age.
The patient was a 10-year-old girl who sustained an abdominal injury from bicycle handlebar trauma. A contrast-enhanced CT scan revealed a large hepatic hematoma with contrast extravasation, prompting angiography. Active bleeding from the hepatic artery was detected, and transcatheter arterial embolization was performed to achieve hemostasis. On the third day of hospitalization, she showed worsening anemia and elevated liver enzymes, with ultrasound revealing an enlarging hematoma and ascites. A contrast-enhanced CT scan was performed again, but no contrast extravasation was observed, and the enhancement of the liver parenchyma was maintained. Therefore, the possibility of rebleeding and hepatic compartment syndrome was considered low. Her condition improved with blood transfusions and conservative treatment, and she was discharged on the 32nd day of hospitalization. During her hospitalization, the hematoma was monitored using ultrasound and contrast-enhanced CT. Temporal changes in the hematoma were observed, with plasma components being absorbed in the acute phase, followed by the gradual dissolution of blood cell components, leading to a reduction in size. Observing the temporal imaging changes of a large hematoma is considered helpful in determining the appropriate timing for invasive treatment, should it become necessary.
The patient was a 6-year-old boy with a history of duodeno-duodenostomy on the third day after birth. He complained of persisting abdominal pain for three days and was referred to our hospital. His pancreatic enzyme levels were elevated, and abdominal ultrasonography revealed a pancreatolith with a dilated pancreatic duct. An enhanced CT scan revealed pancreatic enlargement and a pancreatolith, leading to a diagnosis of obstructive pancreatitis. MRI showed numerous pancreatoliths that filled the head of the pancreas, which are associated with complex anomalies of the pancreatic duct. Open pancreatolithotomy was performed since the endoscopic removal was unsuccessful. The dilated pancreatic duct was identified at the point between the head and body of the pancreas by ultrasonography, and the duct was incised, accomplishing the complete removal of the pancreatolith. Intraoperative pancreatography revealed that the pancreatic duct in the head was trifurcated with two out of three openings into the duodenum. Furthermore, dilation of the branches and collateral pathway formations were observed. There was no stenosis at the duodenal papilla. Transduodenal pancreatic duct tubes were placed through the major and minor papillae. His postoperative course was uneventful, and there was no recurrence of pancreatitis after 12 months of follow-up. Open pancreatolithotomy may well be an effective method for understanding the pancreatic duct anatomy and for removing pancreatic stones in patients with complicated pancreatic ductal malformations.
Midgut volvulus with bowel ischemia was treated with a silo suspension method (SSM) following Ladd’s procedure. This method enabled us to observe the ischemic bowel under direct vision while avoiding abdominal compartment syndrome (ACS). We experienced treating three neonatal cases where a second-look operation was performed after the SSM. In the first case, the SSM was performed in response to ACS that developed the day after Ladd’s procedure. Postoperatively, we observed the degree of edema and necrosis in the bowel within the silo. Subsequently, on the sixth postoperative day, after the general condition had improved, 40 cm of necrotic bowel was resected. In the second and third cases, the SSM was performed during Ladd’s procedure. In both cases, necrotic bowel segments of 25 cm and 35 cm were resected on the second postoperative day, respectively. The postoperative course was favorable in all three cases.The SSM has the following advantages: (1) it prevents ACS associated with abdominal closure, (2) it enables the determination of the timing of the second-look operation by observing the bowel under direct vision, and (3) it minimizes the extent of bowel resection. It is useful for midgut volvulus without worsening morbidity because of factors such as increased infection risk.
A 1-day-old female neonate presented with persistent vomiting 10 hours after birth. Prenatal assessments were not particular. She was born weighing 3,670 g at 38 weeks and 2 days of gestation. Her Apgar score was 8/7. An abdominal X-ray revealed free air, which indicated gastrointestinal perforation. An emergency laparotomy was performed, which identified an ileal perforation 15 cm from the ileocecal valve. Additionally, intussusception was observed distal to the perforation. A 10 cm segment of the dilated and intussuscepted bowel was resected, and an end-to-end anastomosis was performed. Pathological examination of the resected specimen revealed an intussuscepted ileum with the intussusception completely closed. The final diagnosis was ileal perforation caused by ileal atresia that coexisted with intussusception. Neonatal intussusception is rare, and these findings suggest that the ileal atresia was due to compromised blood flow caused by intussusception. The co-occurrence of ileal atresia and intussusception is extremely rare, and the pathogenesis of the ileal atresia in this patient is reported in this paper.
We report a case of congenital colonic atresia with stenosis surrounding the atresia site, where no clear blood flow was observed during surgery. Multiple surgeries were required owing to challenges in determining the resection range. The patient had gastrointestinal perforation on the first day of life and underwent emergency surgery. This revealed perforation of the ascending colon, type II atresia in the descending colon, and stenosis around the atresia site. A double-barrel ileostomy and a single-barrel colostomy proximal to the atresia site were performed. The patient was also diagnosed as having cystic biliary atresia and underwent hepatojejunostomy. Despite intestinal rehabilitation by pseudostool injection, the stenotic segments did not grow. However, during surgery, it was difficult to determine whether these segments were organic lesions, necessitating multiple additional resections. Retrospective analysis linked the stenotic areas to the inferior mesenteric artery region. Histopathological analysis revealed submucosal fibrosis consistent with ischemic changes, suggesting blood flow disturbance as the primary issue. In cases of colonic atresia with proximal stenosis, blood flow disturbance should be considered. The extent of intestinal resection should be based on the response to pseudostool injection and histopathological findings.
Anogenital warts (AWs) are usually caused by mucosal human papillomavirus (HPV) types 6 and 11 infection and are described as a papule/crown-like wart at the perineum lesion known as a sexually transmitted infection (STI). In this report, we present a case of pediatric AWs that are unrelated to typical mucosal HPV infection. An 11-year-old girl visited us complaining that horn-shaped protrusions repeatedly appeared from the perineum, and the tip became keratinized and fell off naturally. A biopsy showed typical findings of HPV infection and a diagnosis of AWs. Topical imiquimod therapy was initiated but discontinued owing to the development of dermatitis. To rule out the possibility of sexual abuse, we decided to perform resection under general anesthesia. There were no lesions in the vagina or cervix, and the possibility of sexual contact was extremely low. PCR analysis for mucosal HPV including types 6 and 11 showed negative results. No recurrence was observed at six months postoperatively. In addition to vertical–horizontal infection and STIs, there are many iatrogenic and unknown infection routes in children, and cutaneous HPV, which causes vulgaris warts, can be detected. In pediatric cases, as it is not always an STI, we should be careful about estimating the route of infection. This patient was born by cesarean section, and the HPV test taken voluntarily by the parents showed negative results. The route of infection is still unknown, but cutaneous HPV infection should be considered in the case of AWs that are histologically shown to be an HPV infection but negative for mucosal HPV.