Purpose: There are many reports that describe the usefulness of interval appendectomy (IA), as it reduces complications during the perioperative period. Its usefulness is well accepted when it is applied to perforated appendicitis; however, its other applications are still unclear. The aim of this study is to examine the validity of our new IA protocols and patient selection retrospectively.

Methods: Patients who had symptoms of complicated appendicitis for more than 48 hrs and treated at our hospital from January 2012 to December 2013 were retrospectively analyzed. We divided them into two groups: Group E (treated in 2012 and emergent appendectomy was performed) and Group IA (treated in 2013 and IA was performed). We compared and statistically examined age, sex, pain control, laboratory data, operation time, intraoperative bleeding, length of hospital stay, and complications of the two groups.

Results: There were 10 patients in each group. Operation time was significantly shorter in Group IA (58.6 min) than in Group E (96.7 min). There was no complication in Group IA, whereas two complications were observed in Group E. Initial conservative treatment took 8.8 days in Group IA and no recurrence was observed during the waiting period. Total hospital stays were 10.2 days for Group E and 12.8 days for Group IA, which were not significantly different.

Conclusions: Using our criteria, we could have treated our high-risk appendicitis patients with IA safely without lengthening the hospital stay, which indicates that a more extensive IA application to late-presenting complicated appendicitis is possible.

Purpose: Only a few reports have shown the benefits of the use of the transanastomotic tube (TAT) for the postoperative management of congenital duodenal atresia and stenosis. The purpose of this study is to verify the efficacy of TAT in the management of congenital duodenal occlusion, which started at our institute in 2012.

Methods: All neonates with congenital duodenal atresia and stenosis born from 2007 to 2014 were included in this study. The patients were classified into two groups: Non-TAT group (NT group: 2007–2011) and TAT group (T group: 2012–2014). Birth weight, gestational age, postoperative days until full feeding, central venous catheter placement periods, body weight loss ratio, and hospitalization periods were retrospectively studied and compared between the two groups.

Results: Seven patients were in the NT group and six patients were in the T group. The T group showed a significantly shorter time to reach full feeding after operation: NT group 13 days vs T group 6.5 days (p = 0.018). The T group also showed shortened postoperative days of central venous catheter placement: NT group 14 days vs T group 11.5 days (p = 0.049). The periods of hospitalization and phototherapy tended to be short without a significant difference.

Conclusions: TAT provides a significantly shorter duration for postoperative neonates to become fully fed and also provides early stable enteral feeding after operation of duodenal occlusion. TAT utilizing a central venous catheter also shortens the length of postoperative durations.

Purpose: Congenital cystic adenomatous malformation (CCAM) sometimes requires surgery because of respiratory failure in neonates. In this study, we aimed to evaluate such cases retrospectively, and we devised a method for the management and treatment of CCAM after birth.

Methods: We examined 6 cases of CCAM in neonates that required surgery for respiratory failure. We analyzed them in terms of the clinical course and surgery.

Results: Five boys and one girl were diagnosed as having CCAM antenatally. According to the Stocker classification, 3 had type I, 2 had type II, and 1 had type III, and the locations were the left upper lobe in 1, the left lower lobe in 3, and the right lower lobe in 2. Immediately after birth, all the patients showed respiratory symptoms, and X-ray examination showed the displacement of the mediastinum. Respiratory failure worsened rapidly in four patients. One patient had surgery because of the exacerbation of respiratory distress after intubation. Three patients underwent surgery within 5 h after birth because of severe respiratory distress. These three had type I CCAM. Open lobectomy was carried out in all cases. The mean operative time was 145 min, and the postoperative average hospital stay was 30 days. Postoperative complications were pleural effusion, pneumothorax, and pneumomediastinum in one patient each. Furthermore, in two patients, their CCAM was complicated by PPHN.

Conclusions: CCAM sometimes leads to acute respiratory failure after birth. Therefore, we always consider planning the birth and always having an operable system. Also, ventilator management of CCAM patients involves the risk of respiratory failure. In such cases, surgical treatment is recommended.

Purpose: The aim of this retrospective study was to analyze the timing of surgical treatment and the surgical method for congenital cystic adenomatoid malformation (CCAM) in our institution.

Methods: Between 2002 and 2014, 11 patients (7 males, 4 females) underwent surgical resection for CCAM. The patients were classified into either the prenatally diagnosed group (Group P) or the non-prenatally diagnosed group (Group N). The medical data of the two groups were reviewed.

Results: There were 7 cases of Stocker Type I and 4 cases of Type II. Ten patients underwent lobectomy and 1 patient underwent portal lobectomy. Groups P and N included 8 and 3 patients, respectively. In Group P, with the exception of 1 patient who moved to another clinic for follow-up and 1 patient who self-discontinued outpatient visits, the remaining 6 patients underwent radical surgery after 9 months. Emergency surgery was carried out in 2 patients. In Group N, 2 patients were identified on the basis of their symptoms, and emergency surgery was carried out in 1 patient. All the patients who underwent emergency surgery had CCAM of Stocker Type I, with distension of the lesions due to trapped air as the cause thereof. Postoperative complications were phrenic nerve paralysis and funnel chests in 2 patients.

Conclusion: In the case of symptomatic CCAM, prompt surgery is needed. However, in the case of asymptomatic CCAM, surgery should be conducted within less than 9 months. However, CCAM operated within less than 3 months has postoperative complications; thus, operation after 3 months is desirable.

Purpose: To clarify the clinical picture of necrotizing enterocolitis (NEC) in neonates with major congenital heart disease (CHD).

Methods: Regarding six such cases during the last 21 years, we retrospectively reviewed their medical charts, including demographics, type of CHD and surgical or transcatheter intervention for it, change in hemodynamics before/after intervention and at NEC onset, age at NEC onset, episodes of hypoxia, interval between NEC onset to the primary operation and type of operation for it, localization of NEC, and survival.

Results: The median birth weight and gestational age were 2,614 g and 38.4 weeks, respectively: four were male. All had cyanotic heart diseases and four underwent surgical or transcatheter intervention for the CHD before the NEC. Treatment of the NEC started at the median age of 8.5 days. One experienced shock and another experienced anoxic spells immediately before the onset of NEC. After intervention, arterial oxygen saturation was elevated in all the patients and blood pressure decreased in three patients at NEC onset. The median NEC onset age was 12 days. Abdominal drainage (n = 1), colostomy (n = 2), ileostomy with drainage (n = 2), and ileostomy with resection of necrotic intestine (n = 1) were the primary operations for the NEC. The median interval between the NEC onset and the primary operation was 8 days. The colon was involved in the NEC in all the patients. Five patients survived.

Conclusions: CHD could be a risk factor for NEC even in mature neonates. The timing and modality of surgical intervention for NEC are crucial for survival.

We report the case of a girl with biliary atresia that changed the bile duct form that was present during the prenatal period to those after birth and Kasai surgery. She had a cystlike lesion under the liver prenatally, but it disappeared during the course of pregnancy and she showed yellow stools after birth. Abdominal ultrasonography did not show cholangiectasis. Because of her clay-colored feces and icterus two months after birth, she was introduced to our department and Kasai surgery was performed for IIIb1ν-type biliary atresia on the 67th day after birth. Because of the expansion of the intrahepatic bile duct six months after birth, we performed choledochojejunostomy in the liver. Three years after the Kasai surgery, no icterus was observed, and the liver function is currently normal without the expansion of the intrahepatic bile duct. It is necessary to monitor cholangiectasis when it disappears after birth in patients with cholangiectasis prenatally. Even if the intrahepatic bile duct expanded after Kasai surgery, an exploratory operation is important if bile duct anastomosis is possible.

Female indirect inguinal hernia containing the bilateral adnexa and uterus is very rare, and 16 cases including our case were reported in the past. Thirteen out of the 16 cases were left hernia. We present the case of a female infant with irreducible right inguinal hernia containing the uterus and bilateral adnexa. She was born in the 31st week of gestation with a birth weight of 1,147 g and right inguinal hernia was found at 5 days of age. Ultrasonography showed right inguinal hernia containing the uterus and an ovary. A herniorrhaphy was performed at 1 month of age and the uterus and bilateral adnexa were found in the right hernial sac. The postoperative course was uneventful. Although every case reported previously developed in early infancy, it is suggested that one of the etiologies of this disease is the unfixed uterus and adnexa to the pelvis. Ultrasound examination is effective for detecting the contents of the hernia in this disease but it is actually difficult to show all the prolapsing organs in the hernial sac. Therefore, surgeons should be careful in surveying what organs prolapsed in the hernial sac during surgery.

A 6-year-old boy diagnosed with WAGR syndrome presented with bloody urine and a left abdominal tumor. On ultrasonographic examination, a mass (maximum 10 cm) was observed in the left kidney. The mass was diagnosed as Wilms tumor and tumorectomy was planned. However, the boy presented with fever, and infectious endocarditis was strongly suspected owing to his previous history of heart operation with an Amplatzer device. Instead of the postponement of the tumorectomy, preoperative chemotherapy (ActD + VCR for four weeks) was initiated. On post-chemotherapeutic day 20, the boy suffered from severe anemia, and multiple intratumoral hemorrhages were observed on contrast-enhanced CT images. Therefore, an emergency tumorectomy was performed. We herein describe our experience with a difficult case of Wilms tumor treated as an oncologic emergency.

Perianal lipoma is an uncommon anomaly. We report a case of lipoma that was found as a perianal tumor. A 21-day-old infant presenting with a perianal tumor was introduced to our hospital. The tumor’s color changed to dark red on day 20 after birth. The tumor was like a pedunculated polyp and arose from the anal skin. The head of the tumor appeared to have hemorrhaged internally and turned dark red. Ultrasonography showed that the inside of the tumor consists of adipose tissue. The tumor showed no communications with the rectum or sacrum, and no anorectal malformations were found. Tumor extirpation was performed at 25 days of age. Histopathologic findings indicated lipoma. No recurrence was observed 6 months after the operation. Perianal tumors include lipoma, human tail, sacrococcygeal teratoma, hemangioma, lipoblastoma, and other anomalies, and they require histopathological analysis for differential diagnosis. It is important to examine the positions of the rectum, anal sphincter, and spinal cord during operation. In this patient, the examination before the operation revealed no communications of the tumor with any of the above-mentioned structures, and thus we performed the operation early.

Constitutional jaundice (CJ) is characterized by mild and chronic unconjugated hyperbilirubinemia with intermittent jaundice in the absence of liver and hemolytic disease. We encountered a case of severe hyperbilirubinemia and prolonged jaundice after the release of common bile duct (CBD) obstruction, splenectomy, and cholecystectomy in a child with a combination of hereditary spherocytosis (HS) and CJ. Case: At 3 years of age, a girl was diagnosed as having HS. She had abdominal pain periodically, and biliary sludge was detected by ultrasound. At 6 years of age, she developed severe jaundice and frequently had stomachaches. The imaging showed splenomegaly and CBD obstruction by biliary sludge. The CBD obstruction was released by endoscopic nasobiliary drainage, but her jaundice continued. Then, laparoscopic splenectomy and cholecystectomy were performed. Postoperatively, intraperitoneal bleeding occurred and required laparotomy for hemostasis. Prior to reoperation, severe coagulopathy was found. Later, her jaundice gradually improved, but still remained. On further examination, mutations of UGT1A1 were found. We diagnosed that her prolonged jaundice was due to CJ. When a patient with HS develops biliary sludge, gallstones, or prolonged jaundice in childhood, there is a possibility that it develops in combination with CJ.

A 9-year-old girl was placed under 5-month follow-up observation for anemia before visiting our hospital. She complained of abdominal pain and malaise, and significant anemia was observed in blood tests. Thus, she was referred to our hospital for inpatient examination and treatment. A submucosal tumor of the posterior wall of the gastric angle [gastrointestinal stromal tumor (GIST)] was suspected on the basis of the results of detailed examinations. Local resection of the stomach by laparoscopic and endoscopic cooperative surgery (LECS) was attempted on hospital day 24.However, intraoperative endoscopic findings showed an ulcer remaining at the top of the tumor. Therefore, local resection of the stomach was implemented to prevent the peritoneal dissemination of the tumor. The postoperative course was favorable, and she was discharged on postoperative day 9. High-risk gastric GIST was diagnosed on the basis of histopathological and genetic tests, but no genetic mutations were observed using a c-kit (exons 9, 11, 13, 17), PDGFRA (exons 12, 14, 18), or BRAF (exon 15), and SDHB immunostaining also yielded negative results. Postoperative adjuvant chemotherapy was therefore not administered. No recurrences have been identified 25 months after surgery.

An 8-year-old boy with acute abdominal pain since morning was brought to our hospital by ambulance. The pain continued for hours and exacerbated after an enema. The abdominal wall was soft and flat, and there was rebound tenderness in the right lower quadrant. Contrast-enhanced abdominal CT revealed free peritoneal air and an edematous wall of the small intestine, and we diagnosed him as having peritonitis due to a perforated appendix and rectal perforation after the enema as the preoperative diagnosis. An emergency laparoscopy-assisted operation was performed. Muddy ascites and perforated Meckel’s diverticulum were found. The appendix was not inflamed. Resection of Meckel’s diverticulum with peritoneal irrigation and prophylactic appendectomy were performed. Histopathology revealed perforation on the transitions of intestinal mucosa and ectopic gastric mucosa. In the case of peritonitis due to intestinal perforation, laparoscopic surgery is useful for diagnosis and treatment.

Pancreatic trauma with main pancreatic duct injury is rare in children, and its standard therapy has not been established. We report the case of a pediatric patient with main pancreatic duct injury. A 6-year-old boy was transferred to our hospital with abdominal pain after blunt abdominal trauma. Computed tomography demonstrated complete transection of the pancreas, which we diagnosed as type IIIb pancreatic injury according to the criteria of the Japanese Association for the Surgery of Trauma. Use of endoscopic pancreatic stents was difficult because of stricture of the main pancreatic duct. His general condition was unstable and peritoneal irritation was apparent. We, therefore, decided on surgical treatment, and abdominal drainage was performed. On postoperative day 5, his symptoms of peritoneal irritation worsened, and amylase levels in drainage fluid were high. Distal pancreatectomy with splenectomy was subsequently performed because of the severe adhesion of organs in the abdominal cavity. Postoperatively, he needed a second operation, because of leakage of pancreatic juice and splenic vein bleeding. The patient was discharged on hospital day 43. He is doing well, and pancreatic function has remained well-preserved for 6 months postoperatively. There have been several reports that type IIIb pancreatic injury was successfully treated by nonoperative management. When the initial nonoperative management was not effective for children with type IIIb pancreatic injury, in those undergoing pancreatic resection, the operative procedure must be carefully selected, taking into consideration the secondary drainage.

We report a rare case of bilateral vesicoureteral reflux complicated by giant pyelolithiasis. A 6-month-old girl ran a fever over 1 week. She was admitted to our hospital and diagnosed as having urinary tract infection. US demonstrated a giant calcification in the left renal pelvis, and bilateral VUR was detected by VCUG. We suspected that an infection calculus formed in the left renal pelvis associated with VUR. Firstly, we removed the calculus by cutting the left renal pelvis open through a left flank incision. Secondly, we performed bilateral vesicoureteral neoanastomosis (Cohen) one month later. Infection calculi are usually classified as primary and secondary. The present case was regarded as a primary infection calculus because of the detection of Proteus by bacteriological examination, excretion of alkaline urine, which continued until lithotomy, and by calculus analysis. We expect that the prevention of urinary tract infection and lithiasis is possible after the operation.

A 1,616 g girl was born at 31 weeks and 5 days of gestation by emergency caesarian section because of fetal distress. Antenatal scanning had demonstrated intestinal atresia from 27 weeks of gestation. At birth, abdominal X-ray examination showed triple bubble signs. She was diagnosed as having jejunal atresia, which was decompressed using a nasogastric tube. The operation was carried out 3 days after birth. At laparotomy, two points of membranous atresia were found at 3 cm from the ligament of Treiz and further 3 cm distal. Partial enterectomy was carried out. Histopathological analysis showed that the proximal atresia point had type III ectopic pancreas according to the Heinrich classification. Until now, only one case of intestinal atresia with ectopic pancreas has been reported, but multiple types of only membranous atresia have never been reported. This case is considered to be extremely rare.

Case 1: A 7-year-old boy who had been receiving conservative therapy for acute appendicitis underwent Computed Tomography (hereinafter referred to as CT) owing to chronic abdominal pain and fever. Since a closed loop with a twisted bowel was observed at the navel level on CT, the patient underwent emergency surgery. We identified a band formed at the tip of the appendix and greater omentum at the place where we had earlier observed a closed loop on his CT images. We surmised that ileal invagination led to the development of strangulation ileus in the crevice. Case 2: A 5-year-old boy presented with intermittent abdominal pain and vomiting. Although the symptoms had improved once, they recurred thereafter and worsened. Therefore, he was diagnosed as having paralytic ileus on the basis of abdominal X-rays and was thus immediately hospitalized. Since the patient did not show any improvement in his symptoms, he thus underwent CT. A closed loop with two twisted bowels was observed on his CT images, and he was therefore transferred to our hospital to undergo emergency surgery. We found that the tip of the appendix adhered to the small intestine at a point some 70 cm from Bauhin’s valve. The small intestine was invaginated and slightly twisted in the crevice. In addition, we also observed a small tumor at a point where the appendix adhered to the small intestine, and the tumor was diagnosed as Meckel’s diverticulum on the basis of histopathological findings. The occurrence of strangulation ileus associated with the appendix is a relatively rare phenomenon. We herein describe our two experiences and with pertinent bibliographical considerations.

A 1-year-old girl was referred to our hospital because of acute colon ileus. She presented with abdominal distension and compensated shock. Abdominal CT showed moderate ascites, thickening of the rectum and sigmoid colon, bowel distension from the ileum to the descending colon, and fecal impaction at the sigmoid descending colon junction. She was suspected as having gastrointestinal allergy because of her clinical history. Colonoscopy was performed under general anesthesia to avoid laparotomy, but it was impossible to release the ileus. Finally, laparotomy was performed, and the feces impacted at the sigmoid descending colon junction was removed from the incised colon wall, and then decompression of the proximal colon was performed by colonoscopy. Because eosinophilic infiltration was found in the sigmoid colon wall during the operation, gastrointestinal allergy was thus strongly suspected. She successfully recovered after the operation and left the hospital 18 days after surgery. A recurrence of ileus due to the same cause was observed 3 months later, but she was successfully treated conservatively. Although most cases of gastrointestinal allergy are treated by pediatricians, initial treatment by pediatric surgeons is sometimes required. As a result, laparotomy is sometimes necessary to effectively treat such cases.

A 25-year-old woman with biliary atresia after portoenterostomy, who was married and wished to give birth, visited our institution. She had compensated cirrhosis and large jejunoileal varices, which protrude into the intestinal lumen. At the time of her hospitalization due to cholangitis, we started to inform the patient and her family about the risks and benefits of liver transplantation. After five months, she underwent living donor liver transplantation from her father aged 63 years. Three re-laparotomy procedures were needed to correct poor portal blood flow, intestinal perforation, intestinal obstruction, and biliary-enteric anastomotic leakage; she was discharged three months after liver transplantation. Five months after liver transplantation, embolization of the remaining large jejunoileal varices was successfully performed. Two years after liver transplantation, she achieved pregnancy and gave birth to a healthy baby at 38 weeks of gestation with an uneventful course. Adult patients with biliary atresia after surgery, who have chronic liver damage and cirrhosis, have a great risk of deterioration at the time of pregnancy and delivery. Although good pregnancy outcomes after liver transplantation have been reported, the desire of giving birth is an uncommon indication for liver transplantation. The current case highly indicates the need for close monitoring of “child health and development”, meaning that sick children should be cared for until they give birth to and rear the next generation.

Colon venous malformation is rare and it is even rarer in children. Here, we report a pediatric case of this disease that developed with abdominal pain and bloody discharge as the chief complaints. The patient was a girl aged 10 years and 11 months. She was previously healthy. However, she complained of abdominal pain and bloody discharge after a fall, and she was admitted to a nearby hospital. Abdominal CT showed a huge mass in her abdominal cavity. She was brought to our hospital for examination and treatment. She was diagnosed as having right colon venous malformation on the basis of the findings of abdominal echo, CT, MRI and enema contrast, and had surgery. Intraoperative findings showed a dark red multilocular small cystic mass in the bowel wall toward the ascending colon from her cecum. The mass was resected in conjunction with the cecum and ascending colon. The mass was diagnosed as pathologically venous malformation. When examining children with bloody discharge, it is important to also consider this disease.