Purpose: We investigated the patient background, treatment strategy and results in patients with chronic functional constipation.

Method: Cases of chronic functional constipation managed in our hospital from January to December 2018 were collected and reviewed on the basis of medical records.

Results: The study population included 48 patients (male, n = 26; female, n = 22); 16 cases were initial visits for consultation; 32 were revisits. The age at initial hospital consultation was 42 months. The age at the onset of constipation was 7.5 months, and the mean duration of sickness was 30.8 months. The presence/absence of self-defecation, defecation suppression and encopresis at the first visit was observed in 26/22, 38/10 and 16/32 patients, respectively. At the onset of symptoms, patients with encopresis were significantly older than those without encopresis (p = 0.027). At the first hospital visit, patients with encopresis were significantly older than those without encopresis (p = 0.0001). The duration of constipation in patients with encopresis was significantly longer than that of patients without encopresis (p = 0.017). The prevalence of developmental disorders was significantly higher in patients with encopresis than in those without encopresis (p = 0.00364). Defecation suppression improved in 30 of 38 patients (78.9%), but encopresis remained in 10 of 16 patients (62.5%). Dairy restriction was effective in 25 of 35 (71.4%) patients.

Conclusion: Patients with encopresis were older at the onset of symptoms and the duration of constipation was relatively long; thus, early treatment may improve their symptoms. In addition to conventional constipation treatment, cooperation with pediatric neurologists is necessary for their management.

Purpose: The purpose of this study is to determine the features of laparoscopic appendectomy in children 6 years of age and younger, especially in those with perforated appendicitis.

Methods: A retrospective chart review was conducted for all children who underwent laparoscopic appendectomy for acute appendicitis over 2 years and 5 months at our institution. A total of 117 children were registered and divided into two groups: preschool (6 years old and younger, n = 19) and school children (7–15 years old, n = 98). We comparatively reviewed these two groups in terms of age, sex, duration of symptoms, symptoms at presentation, physical findings, white blood cell count, C-reactive protein (CRP) level, usage of computed tomography, presence of fecalith and perforation, operative time, insertion of drain, pathological findings, length of hospital stay, and postoperative complications. The preschool children were further divided into two groups: those with perforated (n = 12) and those without perforated appendicitis (n = 7). The same evaluation was carried out on these groups.

Results: There were statistically significant differences between preschool children and school children in terms of fever (> 37.5°C), CRP level, presence of perforation, operative time, insertion of drain, frequency of gangrenous appendicitis, and length of hospital stay. The operative time and length of hospital stay were significantly longer in preschool children with perforated appendicitis than in those without perforated appendicitis.

Conclusions: The presence of perforation was significantly more frequent in preschool children than in school children, regardless of the duration of symptoms. In preschool children, the presence of perforation affected the operative time and length of hospital stay.

Purpose: Very low and extremely low birth weight infants may require an ileostomy for various diseases, but there are few studies on the optimal timing of ileostomy closure and the indicators are not clear. We investigated the optimal timing for ileostomy closure in our institution.

Methods: From January 2004 to December 2018, we performed ileostomy on 30 very low and extremely very low birth weight infants with necrotizing enterocolitis, focal intestinal perforation, or meconium-related ileus. We successfully performed ileostomy closure in 19 patients (63.3%) and assigned them into two groups, namely, those whose body weight at the time of stoma closure was equal to or more than 1,500 g (group A) and those less than 1,500 g (group B).

Results: There were no significant differences in birth weight and gestational age between the two groups. The patients weighed 2,100 g (1,610–3,640 g) in Group A and 1,339 g (890–1,460 g) in Group B when the ileostomy was closed. In group A, the number of days from ileostomy to the start of postoperative milk intake and the number of days from ileostomy to the day when the milk intake became 100 ml/kg/day were not significantly different between the two groups. The weight gain from the ileostomy to closure was significantly different, but that from ileostomy closure to discharge was not significantly different. Two patients in group A (18.2%) and one patient in group B (12.5%) had reoperation, and there was no significant difference in the reoperation rate. All 19 patients survived, with no intraoperative complications after ileostomy closure.

Conclusions: Stoma closure was considered safe even when the body weight is 1,500 g or less.

Purpose: Although emergent appendectomy (EA) for complicated appendicitis (CA) is straightforward, interval appendectomy (IA) following non-operative management (NOM) is sometimes chosen to treat CA because of exacerbated inflammation. We reviewed our institutional experience of CA cases with a focus on the predictor of NOM failure and the outcome of treatments.

Methods: A total of 144 children with CA treated between 2009 and 2018 were retrospectively analyzed. All operations were attempted laparoscopically.

Results: EA was performed on 104 children (Group EA). The remaining 40 initially underwent NOM: 25 underwent IA successfully (Group IA-S), 9 had failed NOM (Group IA-F), and 6 never underwent surgery. On comparing the pretreatment evaluation of EA with NOM, NOM tended to be chosen for cases involving diarrhea and those with an elevated CRP level, those with a larger abscess, or those that took longer to diagnose (p < 0.01 each). On the other hand, EA tended to be chosen for cases involving fecalith (p = 0.006). In the NOM group, the presence of fecalith was likely an independently significant predictor of NOM failure (odds ratio 25.06). On the outcome of treatments, Group IA-S had a significantly shorter duration of surgery, and Group EA had a significantly shorter length of hospitalization (p < 0.001 each). Group IA-F had the highest rate of conversion to laparotomy (p = 0.01).

Conclusions: On the outcome of CA treatments, IA had a shorter operative time, and EA had a shorter length of hospitalization. However, considerable attention to the fact that the presence of fecalith will more likely lead to NOM failure is needed for the treatment decision.

Purpose: Fetal and neonatal intestinal volvulus without malrotation (IVWM) is a serious life-threatening condition. An early and appropriate neonatal surgical management significantly improves the outcome. However, because intestinal volvulus is generally associated with malrotation, IVWM shows none of the specific clinical signs for presurgical diagnosis and thus its diagnosis is considered to be difficult. Here, we present our institution’s experience with presurgical diagnosis and the clinical courses of 16 IVWM cases.

Methods: A retrospective study reviewing the clinical records of IVWM cases managed in Saitama Medical Center from January 2013 to December 2019.

Results: A total of 16 IVWM patients received surgical management in our center. The patients were divided in two groups according to their time of diagnosis, that is, prenatally diagnosed (group A and B) and postnatally diagnosed (group C and D), with eight infants per group. Patients in group A and B were prenatally diagnosed with an abdominal anomaly during prenatal ultrasonography follow up, four of which showed the typical whirlpool configuration (group A). The remaining four patients showed fetal intestinal dilatation with ascites (group B). One patient of this group was born prematurely (GA 28 weeks and 0 days) and died. Patients in group C and D were diagnosed after birth, four of which presented with symptoms and signs of ileus from 1 to 7 days of life (group C). Four patients were diagnosed intraoperatively as having IVWM after being initially misdiagnosed for other causes of ileus (group D). All patients in group C and D survived.

Conclusion: In our institution, the clinical courses of IVWM cases were mainly divided into two groups according to the time of diagnosis, that is, prenatal or postnatal. In the prenatally diagnosed group, early preterm delivery may have a considerable effect on prognosis. Understanding the clinical course patterns of IVWM in infants may help reach an appropriate diagnosis and an early surgical treatment, and hence a better prognosis.

The patient was a girl aged 8 months. She was brought to a private clinic owing to fever and vomiting. The next day, she had abdominal distension and was transported to our hospital. Blood tests showed an inflammatory response and elevated levels of pancreatic amylase. Abdominal MRI revealed an Altman type IV sacrococcygeal teratoma. Surgery was scheduled after improvement of the inflammatory response of peritonitis by fasting and administration of antibiotics. After that, when oral intake was resumed to improve the nutritional status, symptoms of pancreatitis relapsed, and inflammatory reaction and elevation of pancreatic amylase levels were observed. When fasting was started again, fever was reduced, and abdominal symptoms and pancreatic amylase levels improved. The tumor was resected on the 18th day of admission. It was diagnosed as mature cystic teratoma including pancreatic tissue on the basis of histopathological findings. In addition, elevated levels of pancreatic enzymes in the cyst fluid and ascites fluid were observed. We experienced treating a case of Altman type IV sacrococcygeal teratoma, which contained pancreatic tissue in the tumor and caused peritonitis owing to pancreatic enzymes released from the tumor. If the tumor contains ectopic pancreatic tissue, pancreatic enzymes secreted by the tumor may cause inflammation of surrounding organs.

The present case concerns a 3-year-old boy. Four days before visiting our hospital, the patient had common cold symptoms and was prescribed the appropriate medication by his family physician. After the occurrence of vomiting and tarry stool, he was referred to our hospital. His hemoglobin (Hb) level was 11.3 g/dl, and he was positive for fecal occult blood. He was therefore admitted for close observation and treatment. The day after admission, his complexion became poor and his Hb level decreased to 6.3 g/dl. Abdominal computed tomography (CT) revealed gastric wall thickening and a 4-cm-long cystic mass in the fornix of the stomach. Emergency blood transfusion was performed, followed by upper gastrointestinal endoscopy under general anesthesia. An active ulcer associated with a submucosal tumor was found in the gastric fornix, and hemostasis was initiated. If the bleeding could not be controlled with proton-pump inhibitors, the plan was to remove the gastric mass to achieve hemostasis. After treatment, the melena stopped and the patient maintained a Hb level of 11.0 g/dl. On the 12th day, endoscopic ultrasonography performed to examine the submucosal tumor revealed that the tumor became smaller and that the ulcer had scarred during healing. Six months after the melena stopped, follow-up endoscopy showed that the ulcer had healed and that no elevated lesion was evident. The first CT was reviewed and the CT value was considered to indicate hematoma in the submucosal area of the stomach.

The patient was a 39-day-old female infant who was referred to our institution owing to jaundice exacerbation and acholic stool. We found trabecular gallbladder by abdominal ultrasonography. Since biliary excretion was not found by biliary scintigraphy, we performed exploratory laparotomy at the age of 50 days. Owing to the unclear contrast images of the intrahepatic bile duct obtained by intraoperative cholangiography, we diagnosed it as III-a2-ο biliary atresia and then performed Kasai hepatoportoenterostomy. We started a detailed examination owing to the suspicion of Alagille syndrome (AGS) in the postoperative liver biopsy. Peripheral pulmonary artery stenosis and butterfly vertebra were detected as an extrahepatic lesion, and a Jagged1 (JAG1) gene mutation was also detected in the genetic examination. In the case of suspicion of biliary atresia in a newborn infant or infantile jaundice, since it would be difficult to differentiate AGS on the basis of the findings of preoperative blood tests, imaging tests, and intraoperative cholangiography, we should consider X-ray examination of the vertebral body, a search for extrahepatic lesions of AGS by imaging such as echocardiography, and liver biopsy in intraoperative detection at the time when the condition is suspected.

We report a case of Peutz–Jeghers syndrome treated surgically combined with intraoperative endoscopy after repair of intussusception caused by a hamartomatous polyp using double-balloon enteroscopy. The patient was an 11-year-old boy whose pigmentation in his lips and fingers had been noticed since infancy and who complained of intermittent abdominal pain from one year ago. Examination revealed multiple polyps from the stomach to the rectum, and the polyp caused intussusception in the ileum. The intussusception was repaired by double-balloon enteroscopy but the polyp was too large to be resected endoscopically. Thus, surgical resection of the polyp and intraoperative endoscopy for the other remaining polyps were performed. From our experience, we thought that early polypectomy and prevention of intussusception by regular surveillance are necessary and can lead to the avoidance of surgical resection. Even when surgery is required, intraoperative endoscopy also reduces surveillance, which leads to the QOL improvement of pediatric patients with Peutz–Jeghers syndrome.

We have to be aware of disorders of sex development (DSD), which are found in about 1% of female infants with inguinal hernia. We treated three cases of DSD diagnosed by laparoscopic percutaneous extraperitoneal closure (LPEC) without preoperative ovarian sliding. Cases 1 and 2 were sisters, who were 7 and 4 years old, respectively. The older sister underwent Potts repair for her right inguinal hernia when she was 1.5 years old. She was scheduled to have LPEC on the left and her sister on both sides without ovarian sliding. During the operations, testis-like gonads and vas deferens were found, and no oviduct and uterus were found. Therefore, only observations were performed for suspected DSD. Both patients were diagnosed as having 46 XY DSD and complete androgen insensitivity syndrome (CAIS), and bilateral gonadectomy and LPEC were performed later. Case 3 was a 6-year-old girl, in whom no ovarian sliding was found. During the operation, no uterus was found and only observation was made for suspected DSD. She was diagnosed as having 46 XX DSD and Mayer-Rokitansky-Küster-Hauser (MRKH) syndrome, and LPEC was performed later. It is necessary to consider DSD even when no ovarian sliding is detected by preoperative ultrasonography. LPEC allows easy observation of the internal genitalia during surgery and is useful for the early detection of DSD.

We herein present the case of a giant hepatic tumor in a 14-year-old girl, who had received ulcerative colitis treatment since 13 years of age. Ultrasonography (US) was carried out as a routine work-up examination because she sometimes complained of a dull abdominal pain. US showed a huge parenchymal tumor in the lateral segment of the hepatic left lobe, followed by CT and magnetic resonance imaging. These imaging modalities showed that the blood flow of the dilated left hepatic artery around the tumor returned to the left hepatic vein (early venous return). Although central scars suggested the possibility of focal nodular hyperplasia (FNH), early venous return and the absence of a spoke-wheel appearance suggested the possibility of angiomyolipoma. We successfully performed left lateral segmentectomy, which resulted in the disappearance of the abdominal pain. The histopathologic findings of the resected specimen revealed a fibrous central scar and an abnormal portal region, showing a map-like expression pattern of glutamine synthetase revealed by immunohistochemical staining. Therefore, the hepatic tumor was finally diagnosed as FNH, which is the second most common benign pediatric tumor of the liver, and the portal regional malformation was suggested as a potential source of tumorigenesis. Although the central scar and spoke-wheel appearance on imaging modalities are specific for FNH, we are not always able to obtain these findings, leading to the possible misdiagnosis of other hepatic tumors. If we suspect that the tumor is FNH, we should consider surgical intervention for cases with the clinical symptoms of FNH, the tendency of the tumor to grow or atypical imaging findings.

A 14-year-old boy who developed left-side abdominal pain three months ago visited a previous clinic owing to repeated pain. Abdominal contrast CT showed a 10 mm mass near the spleen, and it was suspected as a torsion of the accessory spleen. He was referred to our hospital, and contrast MRI showed the same mass near the spleen. The mass was not enhanced, suggesting that an accessory spleen was infarcted. Two months later, laparoscopic resection was performed. During the operation, we found a mass in the greater omentum near the spleen and resected it. It was a white and solid mass measuring 10 × 10 mm. Histopathological examination showed that the mass was a granuloma with neutrophil infiltration, and a necrotized worm was found in the granuloma. The worm had a Y-shaped lateral cord and a renette cell indicating that it was an Anisakis larva; therefore, we diagnosed the patient as having extra-gastrointestinal anisakiasis. In the first infiltration of Anisakis larvae to the body, a few larvae may penetrate the abdominal wall and die in the abdominal cavity. Extra-gastrointestinal anisakiasis develops when the larva forms a granuloma in the abdominal cavity. The occurrence frequency of this disease is only 0.5% of all types of anisakiasis, and the preoperative diagnosis is very difficult. Thus, most cases were found accidentally during the operation, but a few were found because of abdominal pain due to the granulomas. From our experience, clinicians should consider the possibility that the granuloma due to extra-gastrointestinal anisakiasis may also cause abdominal pain.

The patient was a 15-year-old boy. He consulted a previous physician for the chief complaint of abdominal pain that started at midnight and was referred to our hospital with a diagnosis of generalized peritonitis. Physical examination showed peritoneal irritation, and abdominal CT showed a strangulated ileus with a closed loop forming ascites. Emergency operation was performed under the diagnosis of strangulated ileus due to Meckel’s diverticulum pedicle torsion. Laparoscopic observation under a general umbilical longitudinal incision under general anesthesia revealed a Meckel’s diverticulum about 50 mm from the ileocecal region, the tip of the diverticulum adhered to the mesentery, and the diverticulum itself formed a band. The stem turned 360 degrees and became dark red. When the band was dissected, the color of the strangulated intestinal tract improved and no bowel resection was performed, and a wedge resection of Meckel’s diverticulum was carried out. His postoperative course was good and he was discharged on postoperative day 10. It is considered important to keep in mind the involvement of Meckel’s diverticulum in the diagnosis and treatment of ileus in children without a history of laparotomy. We here report a rare case of strangulated ileus associated with Meckel’s diverticulum pedicle torsion.

Case 1: A fetal diagnosis of pulmonary sequestration was reached at 22 weeks of gestation on the basis of ultrasound examination findings. A fetal MRI performed at 25 weeks of gestation revealed that lesions occupied two-thirds of the right thoracic cavity, but the lesions had shrunk in size after 26 weeks of gestation. Postnatal contrast-enhanced CT revealed that the lesions became clearly smaller than in the fetal stage. Elective video-assisted thoracoscopic right lower lobectomy was performed. Case 2: Lesions occupying one-third of the right thoracic cavity and aberrant arteries were seen on ultrasound examination at 21 weeks of gestation, and pulmonary sequestration was diagnosed. Shrinkage of the lesions was seen at 26 weeks of gestation, and they were no longer observed after 30 weeks of gestation. Postnatal contrast-enhanced CT revealed small lesions on the dorsal side of the right lower lobe. Elective video-assisted thoracoscopic right lower lobectomy was performed. Discussion: Together with improvements in ultrasound accuracy, an increasing number of cases in which aberrant arteries can be seen in the fetal stage is expected, and a definitive diagnosis of pulmonary sequestration can be reached. We report two cases in which pulmonary sequestration could be diagnosed in the fetal stage, and shrinkage could be quantitatively evaluated during gestation.