Purpose: Although congenital diaphragmatic hernia (CDH) is a life-threatening disease, the survival rate of CDH patients has improved owing to recent advances in neonatal management. The incidence of postoperative complications has been increasing in association with improved survival rate. The aim of this study was to investigate the incidence and characteristics of surgical complications such as recurrence, intestinal adhesive obstruction (IAO), and gastroesophageal reflux disease (GERD) with focus on the severity of CDH prenatally diagnosed.

Methods: Fifty-one surviving patients who met criteria such as prenatal diagnosis, left side, and isolated disease, were analyzed for surgical complications requiring surgical interventions.

Results: According to Kitano’s classification, 27 patients (52.9%) were classified into Group I, 14 (27.4%) into Group II, and 10 (19.7%) into Group III. The numbers of patients who required patch repair are one (3.3%) in Group I, seven (50%) in Group II, and ten (100%) in Group III. Sixteen (31%) out of 51 patients experienced 22 operations owing to recurrence in eight, IAO in six, and GERD in eight. On the basis of the severity of CDH prenatally diagnosed, surgical complications occurred in six patients (18.5%) in Group I, three (21%) in Group II, and seven (70%) in Group III. The recurrence rate and GERD incidence are significantly higher in Group III, but no significant differences were seen among the groups for IAO.

Conclusion: We analyzed surgical complications with focus on the severity of CDH prenatally diagnosed in our patients. Recurrence and GERD were observed in children with severe CDH prenatally diagnosed. Since the incidence of surgical complications is high in the group with severe CDH, careful follow-up is mandatory.

Purpose: The treatment of patients with abdominal wall defects, such as gastroschisis and omphalocele, has improved. The aim of this study was to clarify the operative results and outcomes for abdominal wall defects in our institution over the past 30 years.

Methods: From 1985 to 2015, 35 patients (omphalocele, n = 21; gastroschisis, n = 14) were treated. The patient background, operative procedure, operative outcome, and prognosis were retrospectively reviewed using medical records.

Results: A prenatal diagnosis confirmed 8 patients with omphalocele (38.1%) and 10 with gastroschisis (71.4%). Associated anomalies were recognized in 18 patients (85.7%) with omphalocele and in 5 (35.7%) with gastroschisis. Chromosomal anomalies were recognized in 3 patients (14.3%) with omphalocele. The operative procedures for omphalocele were as follows: primary closure, n = 14 (66.7%), secondary closure n = 6 (28.6%), and silo formation, n = 1 (4.8%); those for gastroschisis were as follows: primary closure n = 3 (21.4%) and secondary closure, n = 11 (78.6%). An abdominal incisional hernia was recognized in two patients with omphalocele and in one with gastroschisis. Umbilical cosmetic problems were recognized in 14.3% of omphalocele patients and 35.7% of gastroschisis patients. The survival rates of patients with omphalocele and gastroschisis were 81.0% and 92.9%, respectively. Four omphalocele patients died owing to severe associated anomalies, and one gastroschisis patient died because of progressive acidosis after surgery.

Conclusions: Associated anomalies are the most important factors that affect the prognosis of patients with abdominal wall defects. Long-term problems, such as umbilical cosmetic problems and abdominal incisional hernia, should be resolved in addition to improving the prognosis.

Purpose: The prognostic factors for native liver survival (NLS) of patients with biliary atresia (BA) after Kasai portoenterostomy were evaluated on the basis of our institutional experience.

Methods: Eighty-four patients with BA who underwent Kasai portoenterostomy from 1984 to 2017 were enrolled in this study. The patient characteristics, preoperative examination results, age at Kasai portoenterostomy, and initial dose of corticosteroids in patients with NLS (NLS group) and patients who died or underwent liver transplantation (non-NLS group) were analyzed.

Results: The NLS rates 1, 10, 20, and 25 years after surgery were 77.4%, 62.7%, 56.6%, and 46.9%, respectively. The mean ages at Kasai portoenterostomy (days) were 60.4 ± 16.3 in the NLS group and 73.1 ± 29.5 in the non-NLS group (p = 0.0135). The preoperative AST levels (IU/ l) were 180.1 ± 113.8 in the NLS group and 257.2 ± 231.4 in the non-NLS group (p = 0.0489). The preoperative serum total bilirubin levels (mg/dl) were 9.16 ± 3.65 in the NLS group and 12.77 ± 5.45 in the non-NLS group (p = 0.000489). The preoperative serum direct bilirubin levels were 6.27 ± 2.00 in the NLS group and 8.77 ± 4.15 in the non-NLS group (p = 0.000484). The initial doses of corticosteroids (mg/kg/day) were 3.49 ± 1.21 in the NLS group and 2.49 ± 1.73 in the non-NLS group (p = 0.00247).

Conclusions: In the present study of BA patients who were treated in our institution, the age at Kasai portoenterostomy, preoperative AST and serum bilirubin levels, and initial dose of corticosteroids were suggested to be useful prognostic factors for NLS.

A 20-year-old man with pectus excavatum underwent pectus bar removal 2 years after the Nuss procedure. There were no intraoperative complications. He showed dyspnea and left chest pain on postoperative day 1. Chest radiography showed a remarkable effusion in the left hemithorax. He was treated conservatively. Although his hemoglobin level decreased from 14.8 to 9.6 g/dl, his respiratory status and chest radiography improved and he was discharged on postoperative day 3. His hemoglobin level became normal one month after surgery and the chest radiography showed no evidence of hemothorax. Although pectus bar removal is considered a relatively safe procedure, we should be aware of the complications both during and after the bar removal.

We report a case of neonatal gastric rupture with midgut volvulus. A male infant aged 5 days after birth was referred to our hospital owing to severe abdominal distention and bilious vomiting. He was delivered vaginally with a body weight of 2,480 g and gestation of 38 weeks. His Apgar score was 9/10. Before birth, no abnormality was observed. Nonbilious vomiting started on the 3rd day, and bilious vomiting and marked abdominal distention were observed on the 5th day; thus, he was brought to our Neonatal Intensive Care Unit. On admission, he exhibited severe abdominal distention and a pale complexion. Abdominal X-ray showed a saddle bag sign, which was diagnosed as gastric rupture. Emergency operation was performed following a large-volume infusion. During the operation, a large amount of white-yellowish ascites was encountered, and a 10 mm large rupture was found in the upper major curvature of the stomach. After the removal of necrotic tissue, the defect was sutured and closed. Further inspection revealed midgut volvulus; therefore, Ladd operation was carried out. Although sepsis and DIC developed postoperatively, he was able to survive. In this patient, it seemed that the increase in intragastric pressure induced by the midgut volvulus might lead to gastric rupture. We have to pay attention to bowel obstruction in cases of gastric rupture.

A 3-year-old girl presented with a lower abdominal mass of 7 × 5 cm size. Ultrasound and MRI showed a large cystic mass with hemorrhage and a solid lesion, which was suspected as a tumor. The preoperative diagnosis was an ovarian tumor, and surgical exploration was performed. We noticed that the tumor was a urachal cyst in front of the peritoneal cavity during operation. The histopathological diagnosis was a urachal cyst with a layer of urothelium and cuboidal or columnar epithelium. A very large urachal cyst is occasionally similar to an ovarian tumor. Note that a giant urachal cyst should also be considered in the differential diagnosis of abdominal cystic lesion.

A 10-month-old male infant was admitted to the emergency department because of vomiting and loss of appetite. He was diagnosed as having fever of unknown origin and ileus. The next day, abdominal computed tomography showed a small bowel ileus caused by a strangulation ileus, and emergency surgery was performed. Intraoperative findings included a dilated small intestine and a necrotic, strangulated duplication 80 cm from the ligament of Treitz. The intestinal tract had developed a volvulus at its base, with omental adhesions and inflammation. We diagnosed him as having a volvulus duplication leading to ileus, which was caused by the tubular lesion adjacent to the mesenteric side. We resected 20 cm of the small bowel along with the necrotic tract. Pathological findings included a twisted intestinal tract with mucosal epithelium and smooth muscle, and a necrotic volvulus duplication with hemorrhagic infarction due to torsion. No pancreatic tissue and ectopic gastric mucosa were found. After surgery, his condition started to improve, but we performed drainage and reconstruction of the abdominal wall owing to intraperitoneal infection and a subcutaneous abscess. Duplication alimentary tract is often encountered, but in this case, the volvulus form was rare and a preoperative diagnosis was difficult, so we had to consider this disease. We report this rare case of volvulus duplication in addition to a discussion of the relevant literature.

Duodenal ulcer with hemorrhage is rarer in children than in adults, and hemostasis of the former is sometimes difficult. Herein, we report three cases of pediatric duodenal ulcer with hemorrhage that required hemostatic treatment. Case 1: A 10-year-old boy presented with hematemesis anemia. Upper gastrointestinal endoscopy revealed Dieulafoy ulcer in the duodenal bulb; therefore, we performed hemostatic treatment by clipping. Case 2: A 9-year-old boy who had an asthmatic attack developed hematemesis and melena while receiving steroid treatment daily. Upper gastrointestinal endoscopy revealed ulcer hemorrhage in the duodenal bulb; however, its hemostatic treatment was difficult. Therefore, we performed interventional radiology (IVR) to stop the hemorrhage. Case 3: A 14-year-old boy developed hematemesis and melena, with associated hemorrhagic shock; therefore, upper gastrointestinal endoscopy was performed. An ulcer with hemorrhage from the anterior wall of the duodenum was found. Therefore, we performed hemostatic treatment. However, rebleeding occurred on the fifth hospital day; thus, upper gastrointestinal endoscopy was performed again. However, hemostatic treatment was difficult; therefore, we performed IVR to achieve hemostasis. Endoscopic treatment is considered the first-line treatment for duodenal ulcer with hemorrhage in children and adults. However, when hemostasis cannot be achieved with this treatment, IVF may be an effective treatment method.

A patent urachus is a rare anomaly and is associated with several other umbilical abnormalities such as umbilical cord cysts. We report on the case of a patent urachus patient with umbilical cord cysts diagnosed by fetal ultrasonography. A female infant was delivered by caesarian section at 38 weeks and 1 day of gestation. Prenatal ultrasonography during her 23rd week of gestation revealed umbilical cord cysts, which demonstrated a consistent reduction in size. Her postnatal ultrasonography showed umbilical cord cysts with a red structure inside. She was diagnosed as having hernia into the umbilical cord, and ligation of the umbilical cord was carried out. However, shortly thereafter, the red structure inside the umbilical cord was exposed and cystography showed a patent urachus. Urachal resection with umbilicoplasty and purse-string subcuticular suture was performed. Her postoperative course was uneventful and the cosmetic outcome of the umbilicus was satisfactory 4 months after surgery. From our experience with this patient, it is important to provide perinatal care considering the existence of a patent urachus regardless of coexisting diseases such as chromosomal and congenital abnormalities when an umbilical cord cyst is detected by fetal ultrasonography. Thus, infants such as this patient should be treated in medical facilities that have capabilities for neonatology, obstetrics, and pediatric surgery.

An 8-month-old boy was admitted to our hospital with a polycystic liver mass that was diagnosed when he visited a doctor owing to vomiting and diarrhea. His liver enzyme levels were not elevated and his serum α-fetoprotein (AFP) level was slightly elevated. Liver biopsy revealed mesenchymal hamartoma. We performed extended left hepatectomy because a rapid enlargement of the liver and a gradual increase in the AFP level were detected. The liver mass was composed of a solid lesion containing mesenchymal myxofibrous tissue and hepatocytes with slightly enlarged nuclei and cholangiole, and a cystic lesion containing a yellowish clear serous fluid. Immunohistochemically, these hepatocytes were focally positive for AFP and glypican-3. The features indicated mesenchymal hamartoma with embryonal hepatocytes. There was no evidence of malignancy. The patient had an uneventful course after the surgery. The child is currently alive and well at 4 months after surgery, with no evidence of recurrence, and his serum AFP level has been normalized. Some mesenchymal hamartomas of the liver initially show a high serum AFP level that usually decreases gradually. In contrast, the serum AFP level of our patient increased. This difference was due to the presence of embryonal hepatocytes.

A 3-year-old girl was referred to our department for the resection of a granuloma on her posterior neck. A tick on her neck had been removed by her father. The lesion was excised under general anesthesia. Histopathological examination showed a nodular infiltrate of inflammatory cells in the dermis. Mouth parts of a tick remained in the central part of the granuloma. We recommended the complete resection of the tick or the mouth parts of the tick with the skin to reduce the likelihood of the development of granuloma, abscess and tick-borne infectious diseases.

We report a case of duodenal atresia diagnosed after surgery for jejunal perforation with necrotizing enterocolitis in an extremely low birth weight male infant. He was born in the 27th week of gestation with a birth weight of 834 g. He had abdominal distention during enteral nutrition starting on day 2. A significant amount of intra-abdominal free air was detected by X-ray examination, and he was transferred to our hospital on day 5. An emergent enterostomy was performed for jejunal perforation with necrotizing enterocolitis. Although his postoperative general condition was good, enteral feeding was not possible. Duodenal atresia was diagnosed on the basis of the upper gastrointestinal examination on day 14, and we performed duodeno-duodenostomy for the complete separation of the two ends of the duodenum on the next day. Although no bile duct was detected on upper gastrointestinal examination and bile discharge was detected only in the distal duodenum, anomalous biliary ducts that permit communication between the proximal and distal duodenal segments are suspected considering the presence of gas in the lower small intestine. Congenital duodenal atresia associated with anomalous biliary ducts is rare, but we should not rule it out even if free air due to the perforation of the small intestine is present.

A male infant weighing 3,188 g and diagnosed prenatally as having omphalocele was delivered by an emergency cesarean section performed at 35 weeks of gestation. In addition, he had the characteristic facial features, muscle tone reduction, coat-hanger-like rib morphology, and a small thorax. As there was no hernial rupture, abdominal wall closure was performed on day 10 after birth. There was a strong adhesion in the hernial sac and the ileocecum, and the ileum was injured. An ileal stoma was constructed in the umbilicus, and a purse-string skin closure was performed during stoma closure on day 34 after birth. The postoperative umbilical appearance was satisfactory. After surgery, he was diagnosed as having the Kagami-Ogata syndrome. Umbilical stoma requires umbilicoplasty during stoma closure. This procedure is easy and can be quickly performed, helps reduce surgical invasion, and results in excellent tolerability. Therefore, it is effective for cases with underlying diseases, such as in the case presented, and it can be applied to general umbilical stoma closure.

Blue rubber bleb nevus syndrome (BRBNS) is a systemic disease of multiple venous malformations in the gastrointestinal (GI) tract and skin, which causes GI hemorrhage and subsequent anemia. The anemia can be treated conservatively, and endoscopic argon plasma coagulation (APC) can be carried out for GI tract lesions. Sometimes, surgical intervention for BRBNS is indicated to control bleeding, intussusception, ileus, or intestinal ischemia. A 5-year-old girl with BRBNS underwent double-balloon endoscopy (DBE) for the control of GI hemorrhage. On the following day, she experienced abdominal obstruction caused by a large hematoma in the small intestine. Despite conservative treatment for 10 days, the obstruction did not improve, and eventually, intestinal perforation occurred. Emergency laparotomy was carried out, and two lesions of the small intestine were resected. In BRBNS, DBE allows the detection and treatment of gastrointestinal lesions by APC; however, careful follow-up for unexpected complications, such as hematoma, is necessary.