Purpose: Fetal and neonatal ovarian cysts are classified into two types on the basis of ultrasonographic findings: simple cysts (SCs) and complex cysts (CCs). CCs are associated with ovarian torsion, which has been considered a surgical indication. Recently, some reports have recommended initial observation. We aimed to clarify the indication of surgery and observation for CCs.

Methods: From 1990 to 2018, 17 CCs in 15 cases were treated. Clinical information was retrospectively collected.

Results: All cysts were diagnosed prenatally. Four were CCs at the initial diagnosis, six became CCs in utero, six became CCs soon after birth and one became CC at 9 months of age. In the surgery group (six cysts in five cases), ovary-preserving surgery was performed in one case with bilateral ovarian torsion. However, primary amenorrhea occurred in adolescence and hormone therapy was required. Two unilateral cysts with autoamputation were removed. The other two cysts with unilateral ovarian torsion required oophorectomy. In the observation group (11 cysts in 10 cases), no complications were found. In all but one case, cysts regressed and disappeared in US. The follow-up was performed until 2.7 (1.0 to 11.6) years of age. Five affected cysts in four cases were confirmed by US. Two cases were followed up for more than 10 years, one of which had menarche.

Conclusions: Initial observation of CCs can be safely undertaken without complications. Surgery may be indicated in cases within several days of onset and those with complications. Cysts remaining for more than 1 year should be followed up with surgery in mind.

Purpose: The incidence of intussusception is low in older children for various reasons. Owing to the lack of a consensus concerning clinical management, the treatment approach differs among clinicians. To optimize the management of intussusception, we retrospectively analyzed cases in older children admitted to our hospital over 10 years.

Methods: From 2007 to 2017, 10 children >5 years old were diagnosed with intussusception and admitted to our hospital. We retrospectively reviewed their clinical records.

Results: The patients were six boys and four girls between 5 and 14 years old. The main symptoms were abdominal pain (10/10), vomiting (6/10), and hematochezia (2/10). Eight patients were successfully managed by enema, but four of them developed recurrence. Five patients, including three recurrent cases, underwent surgery. The pathological lead point (PLP) was found in three patients. On laparotomy, intestinal wall thickening was found in these three patients, leading to ileocecal resection or an intestinal biopsy. Pathologically, they all showed lymphoid hyperplasia or inflammatory changes. The type of intussusception was ileocolic in nine and enteroenteric in one. To explore the PLP, nine patients underwent computed tomography (CT), magnetic resonance imaging (MRI), Meckel’s scintigraphy, and colonoscopy depending on their condition.

Conclusions: Patients presenting with recurrent intussusception were likely to undergo surgery because identifying the PLP by imaging is difficult. To avoid unnecessary surgery, it is important to repeat enema reduction, even for recurrent cases, while searching for PLP through various modalities, including colonoscopy.

A 9-month-old male patient was repeatedly hospitalized and discharged because of vomiting. In his first hospitalization, upper gastrointestinal series showed the duodenojejunal junction to be apparently in a normal position. The cause of his symptoms was unclear. In his fourth hospitalization, we were able to diagnose him as having intestinal malrotation. The operative findings were two internal hernias: a mesenteric fissure hernia at the end of the ileum and a cystic hernia formed in the mesentery close to it. Intestinal malrotation with internal hernia did not show the typical imaging findings of intestinal malrotation due to internal hernia. If a patient has symptoms similar to this case, we must keep in mind the possibility of internal hernia when carrying out imaging methods.

Acute empyema is relatively rare in childhood, and there is no established standard therapy for it. The patient was an 11-year-old boy. He had no medical history. He experienced left chest pain and high fever two weeks before admission, but he did not go to a hospital. He presented with two days of high fever and dyspnea in a local hospital, and computed tomography showed left multiple empyema. The patient was then transferred to our hospital. On the next day, we performed thoracoscopic drainage (VATS) with three ports and placed two chest tubes. His postoperative course was uneventful. More than 2 years have passed since the operation without recurrence of empyema. There are numerous reports of the efficacy of fibrinolytic agents instilled into the pleural space, such as urokinase for acute empyema. However, their effects are not immediate. For our patient, we chose VATS as treatment for acute empyema because the effect of fibrinolytic therapy may be limited and immediate treatment was required owing to the patient’s dyspnea, and we achieved good results. VATS may be effective for acute empyema with respiratory symptoms.

We experienced treating two cases by ileocecal-valve-preserving operation for the intestinal duplications with cysts attached to the ileocecal valve. Case 1 was a four-month-old girl admitted to our hospital because of vomiting. She was diagnosed as having small bowel obstruction because of intestinal duplications of the terminal ileum. An emergency surgery was performed. Case 2 was a three-month old boy admitted to a local hospital because of vomiting and bloody stool. He was diagnosed as having intussusception for which reduction failed; therefore, he was transferred to our institute. On the basis of CT images, he was finally diagnosed as having intussusception due to intestinal duplications, and an emergency operation was performed. In both cases, the lesions were attached to the ileocecal valve, and the ileocecal-valve-preserving operation was selected. A small transverse ileotomy made it possible to resect the lesions completely and to preserve the ileocecal valve. There were no postoperative nutritional problems in our cases. As the intestinal duplications at the terminal ileum are usually located on the mesentery side of the valve, ileocecal resection or mucosectomy has been selected for many reported cases. However. our techniques seem to be better for avoiding incomplete resection and nutritional problems.

There are few reports on the application of a silo technique for abdominal wall abnormalities to congenital diaphragmatic hernia (CDH). We selected the silo technique to temporarily expand the intra-abdominal volume in one patient, in which we were able to successfully repair a primary diaphragmatic defect. The patient was a 0-day-old girl. She was born weighing 2,272 g after 37 weeks of gestation. She was diagnosed as having CDH immediately after birth and underwent open surgery on day 4. Many intraperitoneal organs, mainly the liver, had prolapsed into the thoracic cavity. After we pulled out the organs and directly repaired the right diaphragm, the right diaphragm shrank and the intraperitoneal volume decreased. We therefore attempted to expand the intraperitoneal volume using a patch. We next formed a silo on the abdominal wall, aiming for physiological restoration with self-tissue. She generally demonstrated a good postoperative course despite the onset of temporary chylothorax ascites. She was discharged on day 55. We deemed that performing a two-stage surgery with the use of a silo could be a viable option for the treatment of CDH, in which returning the intraperitoneal organs is difficult.

In this paper, we review the clinical courses and recorded operative videos of our six patients who underwent endoscopic surgery for late-presenting congenital diaphragmatic hernia (Late-CDH) and discuss the pitfalls and solutions for surgery completion. The two patients who underwent laparoscopic surgery had problems with instability of the operation field during the suturing procedure, especially in external ligation. One of these patients was subsequently switched to laparotomy, and the other had postoperative transient hyperamylasemia, probably due to compression maneuvers of the spleen and pancreatic body. In laparoscopic surgery, mild fluctuation of the pneumoperitoneum pressure may push the repositioned organ into the diaphragmatic defect; thus, a reliable and stable method of organ compression must be considered. In one thoracoscopically treated patient, it was difficult to reduce the enlarged colon into the abdomen. If the prolapsed colon is dilated during thoracoscopic surgery, it is important to first reduce the small intestine and then the colon, stomach, and finally the spleen. In endoscopic surgery for Late-CDH, it is important to secure the operation field, and measures must be taken against obstruction by the prolapsed bowel and organs.

A 7-year-old girl was referred to our hospital because of acute onset and intermittent abdominal pain with an abdominal mass demonstrated by CT scan. Abdominal palpation revealed a mass in the right-lower quadrant and tenderness all over the abdomen without muscular defense. A contrast-enhanced CT scan demonstrated a 10 × 7 × 9 cm solid right-sided mass that was most likely an ovarian tumor. A suspected hematoma in the tumor and the presence of a massive ascites strongly suggested tumor rupture. An emergency laparotomy was performed, and the ruptured right ovarian tumor was found macroscopically without the invasion upon the uterus and uterine tube and thus the enucleation was carried out. The pathological diagnosis was a poorly differentiated Sertoli-Leydig cell tumor (SLCT) of the ovary with a large amount of rhabdomyosarcomatous component (FIGO stage-IC3). She received chemotherapy, radiotherapy and peripheral blood stem cell transplantation, and there has been no evidence of recurrence for four years by now. The pathology showed poor prognosis, and the dissemination of the tumor cells into the abdominal cavity was apparent in our patient and thus a watchful follow-up is mandatory.

A 14-year-old boy underwent the Nuss procedure owing to pectus excavatum with a Haller index of 4.1. The patient had a favorable postoperative course and was discharged 12 days after the surgery. Chest pain and a sensation of heat appeared in the night on the 35th day after the surgery, and vomiting occurred from the next day. He consulted a nearby doctor, and tachycardia and plain radiography findings indicated cardiac enlargement. He was then admitted to our emergency department with suspected pericarditis. Echocardiography revealed pericardial fluid retention and atrioventricular diastolic dysfunction. He was diagnosed as having cardiac tamponade and underwent pericardial drainage. However, his fever and pericardial fluid retention continued after the drainage. Since no bacteria were identified in a bacterial culture test and there was no deviation in the pectus bar, he was diagnosed as having reactive pericardial effusion. Pectus bar removal was performed 4 days after the drainage. The symptoms improved immediately after removal of the pectus bar, and the patient was discharged 11 days after removal. Although there are some reports of intraoperative cardiovascular damage as a complication of the Nuss procedure, cardiac tamponade due to delayed reactive pericardial effusion is a rare and serious complication. The Nuss procedure should be performed with attention to possible late postoperative complications.

The patient was a 4-day-old boy. At 26 weeks of gestational age, the boy weighing 860 g was delivered by caesarean section because of a nonreassuring fetal status and breech presentation. On day 4, he presented with a blue distended abdomen, and X-rays showed pneumoperitoneum due to the presence of free air. Emergency laparotomy was performed and the patient was found to have focal intestinal perforation (FIP). The perforated site was 10 cm proximal side to the terminal ileum, and there were some thin and bulging spots near this site. The perforated site including the thin spots was resected, and double-barreled ileostomy was successfully formed. Histopathological analysis revealed the segmental absence of intestinal musculature near the site of perforation, which was thus considered to have caused the FIP.

In a 7-year-old patient with bilateral vesicoureteral reflux (VUR), Deflux^® was administered by intraluminal submucosal injection into bilateral ureters using the double hydrodistension implantation technique (double HIT) at the age of one year. In the early postoperative period following Deflux^® injection, there was no evidence of hydronephrosis. One year after the procedure, the reflux had disappeared. However, at the age of six years, the patient developed a febrile urinary tract infection. Following radiological investigations, hydronephrosis of grade 4 according to the Society of Fetal Urology (SFU) classification was detected on the left side. We diagnosed the patient as having delayed ureteral obstruction following Deflux^® injection. At the age of seven years, a left ureteral stent was placed and left ureteroneocystostomy was performed. During the procedure, ureteral obstruction at the junction of the left ureter and bladder and a high degree of adhesion of the left ureter to the dorsal side of the bladder were observed. Hence, adhesiolysis of the left ureter was performed via intravesical and extravesical approaches. The reason for the sequelae in this case is presumed to be the injection of Deflux^® into an incorrect layer and/or the occurrence of chronic inflammation due to a foreign body reaction. Long-term follow-up using ultrasonography after Deflux^® injection is necessary for the early detection of delayed ureteral obstruction.

We report the case of a 14-year-old girl who presented with acute abdominal pain. Ultrasound ruled out appendicitis, but the abdominal pain recurred when she had oral intake. She had a history of body weight loss over a period of three months. Contrast computed tomography and ultrasound showed the dilation of the stomach and duodenum and the narrowing of the aorto-mesenteric distance. She was diagnosed as having superior mesenteric artery (SMA) syndrome. Enteral nutrition was initiated because the upper gastrointestinal tract indicated that she was able to take liquid orally in a lordotic position. Oral intake was gradually increased, and she was discharged 12 days after admission. Classically, SMA syndrome was considered to be seen in patients with chronic underlying diseases. However, of the 56 pediatric cases of SMA syndrome previously reported in Japan, only 15 (26.8%) cases involved underlying diseases. Although SMA syndrome is an atypical cause of acute abdominal pain without any other symptoms, it is important to consider SMA syndrome for differential diagnosis.

Pericardial and pleural effusions after the Nuss procedure are relatively rare complications. In this report, we describe two cases of pericardial effusion after the Nuss procedure. Case 1 was a 15-year-old boy who presented with fever and fatigue four months after the Nuss procedure. Blood tests showed a high inflammatory response, and an enhanced chest computed tomography scan showed pericardial effusion. There were no abnormal findings around the wound. The pericardial effusion disappeared after corticosteroid administration. Case 2 was a 16-year-old girl who presented with fever and weight loss two weeks after the Nuss procedure. Blood tests showed a high inflammatory response, and echocardiography showed pericardial effusion. There were no abnormal findings around the wound. Although the pericardial effusion disappeared after steroid administration and she recovered and was discharged, she returned to the hospital because of continuous fever. Chest CT showed a left pleural effusion without a recurrence of pericardial effusion. Steroid readministration was effective and her condition improved. Corticosteroid was effective in the treatment of pericardial and pleural effusions after the Nuss procedure when the Nuss bar infections were denied.

A 3-year-old girl was brought to the emergency department of our hospital presenting with redness and inversion of the skin at the umbilical portion. No hernial orifice was palpable and the excessive skin inversion was easily relieved by pushing it back. Ultrasonographic examination showed no tubular structure that could suggest a urachal remnant, and the cause of the symptoms was unclear at that time. The same symptoms recurred two days later, and she underwent surgical exploration. The skin at the umbilical region excessively grew and folded around the subcutaneous space. When it was pulled out under general anesthesia, the same incarcerated state could be reproduced. There was no hernial orifice or dermoid cyst. The excessive skin around the umbilical region was excised and reconstructed. Her postoperative course was uneventful. The extraordinary surplus skin of the umbilical portion was the characteristic feature in this case; furthermore, the fact that the patient could invert the surplus skin by herself was uncommon. The cause of this condition is as yet unclear.

A one-year and five-month-old boy was referred to our hospital owing to suspected undescended testis. His left testis was not palpable at the age of four months. He had normal male-type external genitalia. The right testis was palpable in the right scrotum, but the left testis was not in the left inguinal region, which we suspected to be left intraperitoneal testis on the basis of ultrasonography showing a small mass existing above the left internal inguinal ring. We performed laparoscopy for intraperitoneal scrutiny and orchiopexy. The left inguinal mass located in the inguinal duct was considered an atrophic testis and was removed with an anterior approach. Orchiopexy was performed on the right testis to prevent torsion. No other abnormalities were found in the abdominal cavity. Histopathological examination revealed no testicular tissue in the left inguinal mass, but oviduct, ovary, and endometrial tissues were found. A chromosomal test showed 46XY. Thus, we diagnosed him as having ovotesticular disorder of sex development (DSD) because of the presence of ovarian and testicular tissues within the same individual. Our case is a rare DSD because it was not accompanied by ambiguous genitalia and was diagnosed in early childhood.

The patient was a 1-month-old boy with no medical history. Owing to the onset with vomiting, he was diagnosed as having intussusception, which was resolved by high-pressure enema. However, a small intestinal intussusception appeared again the following day, and he was transferred to our hospital. We performed high-pressure enema again, but it failed to relieve the ileal intussusception. Emergency laparotomy with umbilical Ω incision was performed, and we found intussusception in the ileum. We repaired it by the Hutchinson method and found a tumor in the ileum wall. We partially resected the ileum, including the tumor, and performed end-to-end anastomosis. Adenomyoma (heterotopic pancreas, Heinrich Type 2) was diagnosed by histopathological examination. The postoperative course was uneventful; the patient was discharged nine days after surgery, and no recurrence was observed. Adenomyoma is a very rare tumor that often develops in intussusception in children and is often difficult to diagnose by imaging. Therefore, early surgery may be considered for patients with atypical presentations such as recurrence, difficulty in repair, or other cases outside the preferred age group, without adhering to non-surgical treatment.

We present the case of a female patient with hepatoblastoma associated with trisomy 18, who was successfully treated using a combination of chemotherapy and surgical resection. She was born after 37 weeks of gestation with a body weight of 1,470 g and was diagnosed after birth as having trisomy 18 and esophageal atresia with tracheoesophageal fistula. On her first day of life, she underwent primary repair of the esophageal atresia at a previous hospital. At the age of 12 months, computed tomography revealed a tumor at the right hepatic posterior segment area of the liver. Thereafter, she was transferred to our hospital. The liver tumor was diagnosed as hepatoblastoma because the serum alpha-fetoprotein (AFP) level was as high as 16,671 ng/ml. The tumor was classified as stage I hepatoblastoma on the basis of her pretreatment extent in accordance with a disease system classification. Cisplatin was administered as her preoperative chemotherapy to reduce the size of the tumor before surgery. She underwent resection of the right hepatic posterior segment at the age of 1 year and 2 months. The postoperative course was uneventful, and adjuvant chemotherapy was added. She returned to the previous hospital 37 days after her operation. Unfortunately, she died of viral pneumonia without any recurrence of hepatoblastoma at the age of 2 years and 10 months.

Factitious disorder, a mental disease in which patients act as if they have physical symptoms of a disease, is rare in children. We report a pediatric case of factitious disorder requiring a differential diagnosis from upper gastrointestinal bleeding. A 13-year-old girl was referred to our hospital with black stool. Examinations by abdominal CT, upper gastrointestinal endoscopy and Meckel’s diverticulum scintigraphy showed normal findings. During outpatient follow-up, the patient complained of hematemesis and was admitted to our hospital for further examination. Upper gastrointestinal endoscopy was performed again, and again it showed no abnormal findings. During admission, the hospital staff saw that the patient drew her own blood intravenously with a syringe. Then, the patient complained of hematemesis. From this observation, the patient was diagnosed as having factitious disorder. We need to consider factitious disorder as a differential diagnosis in unexplained cases despite appropriate examinations.