Background Segmental dilatation of the colon (SDC) is a rare disease that is characterized by an abrupt segment of dilated colon between regions of normal-sized colon. We herein report a case of SDC associated with Hirschsprung’s disease (HD). Case presentation The patient developed abdominal distension soon after birth, and enema examination showed localized intestinal dilatation from the descending colon to the sigmoid colon with significant caliber changes on both the oral and anal sides of the dilated colon. The findings of the rectal mucosal biopsy were consistent with HD. We considered this case to be a combination of HD and SDC and performed laparoscopic-assisted Soave pull-through with resection of the dilated colon when the patient was 7 months old. Resected specimens showed steep caliber changes on the oral and anal sides of the dilated colon. In the pathological examination, no ganglion cells were found in the submucosa on the anal side of the dilated colon. Based on the above findings, we finally made the diagnosis of HD with SDC. Conclusion In HD with a characteristic dilated colon, the possibility of SDC should be considered.

This study aimed to describe our experience with three-dimensional endoanal ultrasonography (3D-EAUS) in patients who underwent surgery for Hirschsprung disease and to summarize the relationship between postoperative anal function and the distance between the anastomosis and intersphincteric groove (DBAI) measured via 3D-EAUS. We retrospectively reviewed patients with a history of undergoing surgery for Hirschsprung disease who visited our outpatient clinic between December 2018 and December 2019. All patients underwent 3D-EAUS for DBAI measurement. We used the Krickenbeck classification to evaluate postoperative anorectal function. Eleven patients (all males aged 3–14 years) were evaluated. Four (36.4%), four (36.4%), and three (27.3%) patients had no soiling, grade 1 soiling, and grade 3 soiling, respectively. Four (36.4%) and seven (63.6%) patients had no constipation and grade 3 constipation, respectively. The median DBAI values were 7.0 mm, 8.4 mm, and 5.6 mm (p = 0.14) in the no soiling, grade 1, and grade 3 soiling groups, respectively. 3D-EAUS enabled precise visualization of the anal anatomy and evaluation of the anastomosis. The DBAI was relatively short in patients with grade 3 soiling, although not significantly so. Further evaluation is warranted.

"Man-in-the-barrel" syndrome has been rarely described following osmotic myelinolysis. We report a case of a 45-year-old woman admitted with septicemia and severe hyponatremia. She presented with a "man-in-the-barrel" syndrome which developed more than 10 days after rapid correction of the hyponatremia. There was radiological evidence of central pontine and extrapontine myelinolysis. Three days after completing a course of intravenous immunoglobulin therapy (0.4 g/kg body weight/day for 5 days) there was considerable improvement (Expanded Disability Status Scale score improved 30%). This case, reported for its peculiar mode of development, unusual presentation and challenging therapeutic response to intravenous immunoglobulin, highlights the enigmatic and unpredictable aspects of osmotic myelinolysis.

Background. Myotubular myopathy is a rare disease sometimes accompanied by peliosis hepatis, a leading cause of fatal liver hemorrhage. Case Report. We present a case of a 2-year-old boy with myotubular myopathy who developed liver hemorrhage because of peliosis hepatis and was successfully treated with living-donor liver transplant. The patient initially presented with fever, anemia, and liver dysfunction. A computed tomographic scan revealed hemorrhages in the liver, and the patient underwent hepatic artery embolization twice. After the second embolization, multiple peliosis hepatis cavities appeared in the left lobe of the liver that had increased in size. Therefore, the patient underwent ABO-incompatible living-donor liver transplant using a lateral segment graft from his father. The patient developed severe septic shock with an unknown focus on postoperative day 18, which resolved with antibiotic therapy. On postoperative day 62, he was discharged. Fourteen months after undergoing livingdonor liver transplant, the patient showed no recurrence of peliosis hepatis. Conclusions. Although the long-term prognosis of peliosis hepatis due to myotubular myopathy after living-donor liver transplant remains unclear, liver transplant may be a curative treatment for patients with myotubular myopathy who have uncontrollable peliosis hepatis.

Background: Diagnosing pediatric appendicitis by ultrasonography (US) is difficult because US requires significant training and skill. We evaluated whether artificial intelligence (AI) can augment US. Materials and Methods: Among 70 abdominal ultrasound videos containing 85-347 images each, 50 were used to train the AI neural network. Each video was categorized based on the detection percentage and percent accuracy: most (>50%), partial (10-50%), and none (<10%). Test 1 involved verification of appendix detection by AI using the remaining 20 videos. Test 2 involved the evaluation of the effect of AI utilization on pediatricians. Results: From 50 videos, 6914 images were used to train the AI network. In test 1, 3 pediatric surgeons judged 10 (50.0%), 4 (20.0%), and 6 (30.0%) videos as "most," "partial," and "none," respectively, regarding the detection percentage; 7 (35.0%), 7 (35.0%), and 6 (30.0%) videos were judged, respectively, concerning the percent accuracy. Five (83.3%) of six test videos with a scan area depth of 8 cm were judged as "none" for both detection and accuracy. In test 2, six videos were also judged as "none" for both categories, showing a negative effect on the participants (5 pediatric residents and 5 pediatric intensive-emergency fellows), but the other categories showed little negative effect. Conclusions: Appendicitis in a shallow US scan area can be easily identified with AI support. Even with the detection of a partial appendicitis shadow, AI is still helpful. However, if AI does not detect appendicitis at all, examiners may be negatively affected.

Aim: To compare the incidence of erectile dysfunction and ejaculatory anomalies in male patients with anorectal malformation (ARM) after treatment with the sacroperineal approach (Group S) or laparoscopically assisted anorectoplasty (LAARP; Group L). Methods: Male patients who underwent repair of high- or intermediate-type ARMs at our center and aged ≥15 years as of November 2020 were enrolled. Patients with intellectual disabilities or chromosomal disorders were excluded. Sacroperineal anorectoplasty has long been routinely performed at our hospital, and LAARP was introduced as a standard procedure in 2000. The medical records of the outpatient clinic were retrospectively reviewed, and questionnaires were sent in November 2020. Data regarding erectile dysfunction, ejaculatory anomalies, and associated anomalies were collected and compared between the two groups. Results: Answers were obtained from 7/22 patients in Group S (32%) and 10/17 patients in Group L (59%). Patient characteristics were similar, but the median age at the time of replying was significantly different (22 [18-29] years in Group S and 19 [15-20] years in Group L, P = .0060). None of the patients had erectile dysfunction, but 3 patients in Group S (43%) and 5 patients in Group L (50%) had ejaculatory failure, with no significant difference between the two groups. Conclusions: The incidence of sexual dysfunction in male patients with ARM might be higher than that in the healthy population. Patients might abscond from their follow-up visits as they age; therefore, providing patients with accurate information on this issue before puberty is essential.

Anorectal malformation includes various types of anomalies. The goal of definitive surgery is achievement of fecal continence. Twenty years have passed since laparoscopically assisted anorectoplasty (LAARP) was reported by Georgeson. Since LAARP is gaining popularity, its long-term outcomes should be evaluated. Presently, there is no evidence regarding the optimal method of ligating and dividing the fistula correctly and creating the pull-through canal accurately. Rectal prolapse and remnant of the original fistula (ROOF) tend to develop more often in LAARP patients than in posterior sagittal anorectoplasty (PSARP) patients; however, robust evidence is not available. Prolapse may be prevented by suture fixation of the rectum to the presacral fascia; however, if prolapse occurs, the indication, timing, and the best method for surgical correction remain unclear. Most patients with ROOF are asymptomatic, and there is controversy regarding the indications for ROOF resection. This article aimed to detail the various modifications of the LAARP procedures reported previously and to describe the surgical outcomes, particularly focusing on rectal prolapse, ROOF, and fecal continence, by reviewing the literature. Functional outcomes after LAARP were almost similar to those noted after PSARP, and we have demonstrated that LAARP is not inferior to PSARP with respect to fecal continence. Although there is controversy regarding the application of LAARP for recto-bulbar cases, we believe that LAARP is still evolving, and we can achieve better outcomes by improving the procedure.

Aim: To clarify the characteristics of patients with rectal prolapse after laparoscopically assisted anorectoplasty (LAARP), estimate the causes, and evaluate its impact on postoperative bowel function. Methods: The medical records of patients who underwent LAARP for high- or intermediate-type anorectal malformation between 2000 and 2019 were retrospectively reviewed. Clinical data were compared between patients with (Group P) and without prolapse (normal, Group N). Fecal continence was evaluated using the clinical assessment score for fecal continence developed by the Japanese Study Group of Anorectal Anomalies. For patients who underwent pelvic magnetic resonance imaging (MRI) before LAARP, atrophy, or asymmetry of the anal sphincter and levator ani was evaluated by a radiologist. Results: Of the 49 patients, 29 (59%) had rectal prolapse after LAARP (Group P) and 20 did not (Group N). We found no significant difference in gender, type of malformations, incidence of associated spinal or lumbosacral anomalies, procedure time, and postoperative bowel function at ages 4, 8, 12, and 16 years. However, LAARP was performed significantly earlier in Group N (median [range], 180 [123-498] days) than in Group P (210 [141-570] days). In Group P, 18 patients (62%) developed prolapse before colostomy takedown. Eight of 26 patients who underwent surgical prolapse repair required redo procedures. Twenty-five patients who underwent preoperative pelvic MRI showed no significant relationship between the muscular abnormalities and the incidence of postoperative rectal prolapse. Conclusions: Although recurrence is common, performing LAARP at a younger age might prevent postoperative prolapse development.

A 13-year-old girl developed intrahepatic cholangiocarcinoma 7 years after laparoscopic extrahepatic bile duct resection and biliary reconstruction to treat Todani's type IV-A congenital biliary dilatation (CBD). During the postoperative period, her serum carbohydrate antigen 19-9 levels were above normal, and the imaging tests showed intrahepatic bile duct dilatation. Then, 7 years after the initial operation, she presented to our hospital with stomachache. Computed tomography revealed an irregular tumor in segment 3 of the liver and swelling of her hepatoduodenal lymph node. She was suspected of having left intrahepatic cholangiocarcinoma with hepatoduodenal lymph node metastases. Therefore, she underwent left hepatectomy and regional lymph node dissection. The histopathological diagnosis after resection was intrahepatic cholangiocarcinoma with poorly differentiated adenocarcinoma. According to the Union for International Cancer Control, 8th edition, the TNM classification of malignant tumors was pT3, pN1, pM0, pStage IVA. Subsequently, 4 months after undergoing left hepatectomy, she had a metastatic recurrence of intrahepatic cholangiocarcinoma. Although the significant association between CBD and bile duct cancer after surgery for CBD is well known for adults, we emphasize that children also have the risk of cancer development.

Congenital diaphragmatic hernia (CDH) is one of the most common major congenital anomalies. The presence of a hernia sac is associated with additional benefits in terms of improved neonatal survival. Here, we report a case of CDH with a hernia sac detected via postnatal ultrasonography. Our literature search did not find other cases where CDH with hernia sac was found by postnatal ultrasound in neonates. In prenatal imaging, the diagnosis of CDH with a hernia sac is challenging. In our case, the meniscus of the thymus was clearly noted, and smooth convexity between the hernia contents and thymus was detected. Although evaluation of the presence of a hernia sac with postnatal ultrasonography might be difficult, our findings suggest that a hernia sac could be evaluated with postnatal ultrasonography.

Ligamentum teres hepatis (LTH) abscess is extremely rare, with few case reports stating that surgical excision should be considered rather than conservative treatment. We report a case of LTH abscess in a child with successful conservative therapy. A 1-month-old girl was transferred from another hospital due to a space-occupying lesion in the liver. Contrast enhanced computed tomography and abdominal ultrasonography revealed an LTH abscess. Staphylococcus aureus was identified from the abscess contents. Cefazoline was administered for 14 days, after which the lesion could not be identified with ultrasonography. The abscess had not recurred by the 6-month outpatient clinic follow-up visit. Unlike other reported cases, in our case, the abscess decreased in size with conservative treatment, thus our case could be improved.

BACKGROUND: Rectovaginal fistula (RVF) is a serious complication after colorectal anastomosis using a double-stapling technique. RVF following this procedure has been considered to be refractory to conservative treatment. CASE PRESENTATION: A 75-year-old woman who underwent laparoscopy-assisted low anterior resection for early rectal cancer developed RVF on the 12th postoperative day. Conservative treatment was chosen and was successful. She was discharged from the hospital after 3 weeks with a normal oral diet. Colonoscopy on the 50th postoperative day showed that the RVF was closed. CONCLUSION: Conservative treatment may be effective for RVF after colorectal anastomosis using a double-stapling technique when there is no evidence of defecation through the vagina. (C) 2017 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd.