Purpose: The purpose of this study was to compare the outcomes and prognosis factors between right-sided congenital diaphragmatic hernia (RCDH) and left-sided congenital diaphragmatic hernia (LCDH) and to elucidate the characteristics and outcomes of RCDH.

Methods: We retrospectively reviewed the medical records of 39 consecutive infants with CDH from January 2002 to December 2014. The analysis focused on the prenatal diagnosis, delivery institute, the length of hospital stay, complications, and mortality in infants with RCDH and compared with those with LCDH.

Results: Seven infants had RCDH. No significant differences were observed in the characteristics between the RCDH and LCDH infants except the liver up rate (RCDH, 100.0% vs LCDH, 21.9%; p < 0.001). Postoperative gastroesophageal reflux requiring management occurred in five infants with LCDH, but in none of the infants with RCDH (15.6% vs 0.0%, respectively; p = 0.561). The survival rate of infants with RCDH was 57.1%, and that of infants with LCDH was 78.1% (p = 0.344). Among infants with LCDH, a prenatal diagnosis at <30 weeks, birth weight of <2,500 g, herniation of the liver into the thoracic cavity, and patch repair were associated with high mortality. Conversely, no factors were associated with mortality among infants with RCDH.

Conclusions: Although not statistically significant, the prenatal diagnosis and survival rates tended to be lower in infants with RCDH than in those with LCDH. The factors associated with prognosis in infants with LCDH were not similar to those in infants with RCDH. This study suggests that RCDH may be a pathophysiology independent of LCDH.

Purpose: To identify the characteristics of malrotation, we compared a group of neonates and infants with malrotation and a group of older children (>1 year of age) with malrotation.

Method: Patients with malrotation and who were treated in our institution from January 1990 to December 2015 were retrospectively analyzed. The patients were divided into two groups: Group (A), for those less than one year of age (neonatal and infantile group), and Group (B), for those more than one year of age (older children group). Backgrounds, symptoms, period of symptoms, midgut volvulus, operative procedures, operative time, intraoperative bleeding, and postoperative complications were compared.

Result: The number of patients of interest was 45: Group (A) consisting of 38 patients, and Group (B), 7 patients. Comparison of symptoms showed bilious vomiting as the predominant symptom in Group (A), and nonbilious vomiting and intermittent abdominal pain as the major symptoms in Group (B). Midgut volvulus comparison showed no significant difference between the two groups, but chronic midgut volvulus was suspected in Group (B). Operative time was statistically significantly longer and intraoperative bleeding was significantly larger in volume in Group (B) than in Group (A). There were no significant differences in postoperative complications between the groups, and adhesive ileus was more common in Group (B) than in Group (A).

Conclusion: The characteristics of malrotation in older children were clearly different from those in neonates and infants. It is important that older children with chronic gastrointestinal symptoms be examined for intestinal malrotation.

Purpose: Recently, the laparoscopic excision of urachal remnants has been conducted at an increasing number of facilities. However, laparoscopic excision may cause postoperative intestinal adhesion due to peritoneal damage during the excision of the urachal remnants. We devised a surgical technique for the excision of urachal remnants, called trans-umbilical extraperitoneal tunneling (TET). By further improvement of TET, we were able to provide superior cosmetic results and minimize peritoneal damage. We hereby report on this technique.

Methods: Twenty-three patients who underwent laparoscopic excision of urachal remnants with TET at our hospital between October 2005 and October 2015 were studied. We retrospectively investigated operative time, intraoperative blood loss, and the postoperative clinical course.

Results: The mean age of patients was 17.9 years (3 months old to 44 years old). There were 13 children and 10 adults, and the male-to-female ratio was 15 to 8. The mean operative time was 101 min, the mean blood loss was 7.3 g, and the mean postoperative hospital stay was 4.4 days. No complications were observed.

Conclusions: TET is an effective surgical technique that provides superior cosmetic results, and can be used to avoid the risk of postoperative intestinal obstruction. The observation of urachal remnants with a laparoscope is effective in preventing unnecessary manipulations. Additionally, tissue traction using a retractor ring can very effectively provide a good surgical view.

Purpose: The medical management of whole-liver-prolapsed giant omphalocele (WLPGO) remains challenging. We herein investigated whether the Allen-Wrenn Method using a Wound Retractor^® without forcing prolapsed organs into the abdominal cavity (modified AWM) is effective for WLPGO.

Methods: Five patients with WLPGO (group A) and 5 with abdominal wall malformation without liver prolapse (group B), admitted during the 2010 to 2015 period, were compared. Modified AWM was applied to all patients considering essential points, including pulling a silo in the abdominal cavity up until sufficient skin elevation without forcing prolapsed organs into the abdominal cavity.

Results: In group A (2 males, 3 females), the average gestation was 37w3d, the average birth weight was 2,182 g, and the average abdominal wall defect size was 6.5 cm. In group B (4 males, 1 female), the corresponding averages were 34w6d, 2,140 g, and 2.6 cm. In group A, a silo was attached on the day of birth in 4 patients, and 2 days after birth in one with poor general condition. Silo attachment was carried out on the day of birth in all the 5 group B patients. The average silo attachment periods were 6.8 days (5–8) in group A and 6.0 days (3–10) in group B. The difference was not statistically significant.

Conclusions: In all patients, the abdominal wall was safely closed in a short period. Modified AWM is a minimally invasive and very effective treatment strategy for WLPGO, as it is for abdominal wall malformation without liver prolapse. Moreover, WLPGO can be simply carried out at any hospital.

A 6-year-old girl with bulging of the left inguinal area since 4 years of age was referred to our department. At 5-year-8-month of age, she was diagnosed as having left indirect inguinal hernia, and radical surgery using Potts’ procedure was performed. The swelling of the left inguinal area recurred several months after the operation. Direct inguinal hernia or recurrence of indirect inguinal hernia was suspected, but differential diagnosis was difficult to make on the basis of only preoperative ultrasonography and physical examination. At reoperation, the inguinal procedure was initially used. Direct inguinal hernia was confirmed, but the observation of the internal inguinal ring was difficult owing to adhesion. Next, laparoscopy was performed, which confirmed the closure of the internal inguinal ring and the weakness of the abdominal wall of the area of the direct inguinal hernia, which matched the findings of the inguinal procedure. In addition, because the weakness of the abdominal wall was observed in a wide area extending to the vicinity of the internal inguinal ring, we decided to reinforce the abdominal wall by mesh repair via the inguinal approach. The laparoscopic procedure made it easy to identify the area of the abdominal wall weakness correctly. The postoperative course was uneventful without the recurrence of inguinal hernia.

We describe a rare case of isolated neurofibroma of the colon in a child that was not associated with neurofibromatosis type 1 (NF1). Abdominal ultrasonography demonstrated intussusception in an 11-year-old girl who was referred to our hospital with intermittent abdominal pain. An enema study indicated intussusception of the ascending colon, and abdominal computed tomography confirmed that it was caused by a mass of 4 cm diameter. Therapeutic enema was successful, but abdominal pain recurred 10 hours later, so the patient was treated by emergency partial colectomy. The resected specimen comprised a soft, elastic tumor that was immunopositive for the S-100 protein and CD34, indicating neurofibroma. She was discharged six days after surgery and followed up at our hospital. This is the first case of neurofibroma of the colon of a child that was not associated with NF1. Special attention is required during the management of neurofibroma, because the treatment depends on whether it is associated with NF1 or not.

Polyposis syndrome is a risk factor for colon and rectal cancer and should be treated early or followed up strictly. The early diagnosis of colon cancer in children is difficult because children have few symptoms and the prognosis is not very good. A risk factor for pediatric colon cancer is APC-associated polyposis caused by the 5q21-22 mutation. APC-associated polyposis conditions usually include familial adenomatous polyposis (FAP), Gardner syndrome, and Turcot syndrome, but the chromosomal mutation that includes 5q21-22 causes APC-associated polyposis. We present a case of rectal cancer with the 5q deletion syndrome. A 19-year-old male was subjected to chromosomal examination because of less pursuit during a 4-month health checkup, and he was diagnosed as having the 5q deletion syndrome. Colonoscopy (CS) showed polyposis coli in the patient and he was recommended for regular follow-up examinations, during which he did not undergo CS. Later, he complained of bloody stool and underwent CS. Upon CS, a type 2 mass and polyposis coli were observed, and the mass was histopathologically identified as rectal cancer. He underwent laparoscopic proctocelectomy and ileac-pouch anal canal anastomosis. He was followed up regularly. Polyposis syndrome with a risk of cancer should not only be followed up strictly, but also treated early by surgery.

The definitive diagnosis of acute abdomen in infants is difficult. We present a unique case wherein the adhesion of the appendix to the retroperitoneum caused an internal hernia that required urgent exploration. A 44-day-old female infant with congenital myotonic dystrophy experienced sudden onset of fever and abdominal distention. Contrast-dye enema showed a tapering of her ascending colon with no visualization of the distal ileum. In the upper gastrointestinal series, the dye did not pass to the jejunum. Owing to the possibility of ileus strangulation, she underwent an emergency laparotomy, which revealed an internal hernia of the distal ileum caused by the adhesion of the appendicular tip to the retroperitoneum. There was no mesenteric defect or periappendicular abscess. Segmental resection of the ischemic ileum and appendectomy were performed, and the patient was successfully treated. Adhesion of the appendix was totally unexpected. In early infancy, even though the exact diagnosis of the pathology is not defined, urgent laparotomy is necessary to save the patient.

We report a case of successfully operated Gross C esophageal atresia by a transcervical approach following preoperative examinations. A 2,080 g baby girl of 37 weeks 2 days’ gestation was delivered. She was the second of twin fetuses. Hypoplasia of the right lung was detected during the gestation. No hydramnios was detected. We were unable to pass a nasogastric tube through the esophagus, and the X-ray result showed the coil-up sign with a gastric bubble. She was diagnosed as having esophageal atresia with distal tracheoesophageal fistula (TEF) and right pulmonary hypoplasia. We planned a radical operation after assessing her respiratory condition and evaluating her anatomical bronchial form. Bronchoscopic examination was performed before gastrostomy at 0 days of age. The distal TEF was identified 13 mm above the carina. Contrast chest CT showed that the upper pouch of the esophagus and the distal TEF were at the level of the second thoracic vertebra. On the basis of preoperative examinations, we chose the transcervical approach to repair the esophageal atresia and distal TEF at 5 days of age. A 3 cm transverse incision was made 1 cm above the left clavicle. The end of the upper pouch and the distal TEF were almost at the same positions. The division and closure of the TEF and esophago-esophageal anastomosis were performed successfully. The postoperative course was uneventful. Preoperative bronchoscopy and CT are useful for determining the type of esophageal atresia and for deciding the surgical approach.

We report two cases of congenital cystic adenomatoid malformation (CCAM). Case 1: a prenatal sonographic examination revealed a left intrathoracic cyst. A male baby weighing 3,452 g was born at 37 weeks of gestation and was diagnosed as having CCAM Stocker type 1. He was asymptomatic in the neonatal period, but was hospitalized with a cyst infection at 3 months of age. Left lower lobectomy was performed at 6 months of age. The intraoperative findings showed pleural adhesion due to the infection. Case 2: a prenatal sonographic examination revealed bilateral intrathoracic cysts. A female baby weighing 2,128 g was born at 36 weeks of gestation and was diagnosed as having bilateral CCAM Stocker type 1 involving the posterior segment of the right lung and the lower lobe of the left lung. She was asymptomatic in the neonatal period and underwent left lower lobectomy at 11 months of age and right upper lobectomy at 14 months of age without infection. Surgery is advantageous in CCAM patients without previous infections, especially bilateral cases. The absence of previous infections helps in ensuring the safety of surgery and a good postoperative lung function. She gained weight after surgery, so we recognized the necessity of early surgery to improve growth disorders.

An 11-year-old boy with Down syndrome was admitted to our hospital presenting with black stool and pale complexion. He underwent diamond-shaped anastomosis for congenital duodenal stenosis at another hospital at 1 day of age. A follow-up study was started at our hospital at 3 months of age, and a radical operation for Hirschsprung’s disease was performed at 7 months of age. The postoperative course was uneventful and there was no growth disturbance. At the time of admission, blood examination revealed a very low hemoglobin level (5.9 g/dl); thus, esophagogastroduodenoscopy (EGD) was performed and he was diagnosed as having hemorrhagic erosion of the anastomotic site. It was improved with the use of a proton pump inhibitor (PPI) within 2 months, and then PPI was discontinued. However, 6 months later, he was admitted to our hospital presenting with black stool and pale complexion again. Blood examination revealed a low hemoglobin level (8.2 g/dl). EGD showed an anastomotic ulcer with mild bleeding and a hemorrhagic erosion at the anal side. From the results of EGD and upper gastrointestinal tract X-ray, the anastomotic ulcer was suggested to be attributed to chronic stimulation by remaining food residues, gastric acid and bile. Additional treatments, such as gastrointestinal agonist drugs, dietary instruction and appropriate position, were fully effective against the anastomotic ulcer. Fifteen months later, there was no recurrence. It is important to remember that postoperative anastomotic ulcer could develop long after surgery, even if there is no anastomotic stenosis.

We present the case of a patient with hepatoblastoma, born at 24 weeks and 6 days of gestation and weighting 568 g, and one of the monochorionic diamniotic spontaneous twins. She was the donor of twin-to-twin transfusion syndrome. She showed a hepatic enlargement during the 7th month checkup and was referred to us. Abdominal CT scan showed a large mass in the right hepatic lobe and she was diagnosed as having PRETEXT II hepatoblastoma. Following neoadjuvant chemotherapy, she underwent right hepatectomy. The twins were diagnosed as having Beckwith-Wiedemann Syndrome (BWS) by molecular genetic testing. Although the risk of tumor development based on BWS was similar between them, hepatoblastoma was found only in the affected twin. The affected twin was born with a lower birth weight, received oxygen therapy longer, and used diuretics longer than the nonaffected twin. This case suggests that the prenatal environment and postnatal intensive care differences are associated with the development of hepatoblastoma.

The patient was a boy who was newly born by vacuum extraction at 40 weeks and 2 days of gestation and weighed 2,730 g. A mass was observed on the left side of the patient’s neck and the patient showed respiratory impairment that gradually worsened. The patient consequently underwent endotracheal intubation at 2 days postpartum. Computed tomography and ultrasonography revealed a multilocular mass, leading to the diagnosis of a lymphangioma. The patient subsequently underwent sclerotherapy, but no reduction in tumor size was observed. Magnetic resonance imaging also showed a multilocular mass centered around the parapharyngeal space, with part of the mass extending from the nasopharynx to the oropharynx. Because the nasopharyngeal and oropharyngeal involvement of the mass was considered to be the direct cause of respiratory impairment, endoscopic resection was performed at 31 days postpartum. Histopathological analysis revealed heterotopic glial tissue. Heterotopic glial tissue in the parapharyngeal space in newborns has been found in less than 20 cases worldwide, and the current case is the only one being reported in Japan. On imaging, heterotopic glial tissues are difficult to differentiate from lymphangiomas and teratomas; however, a mandibular deformity is said to be characteristically associated with heterotopic glial tissue. Therefore, when a mass is particularly observed in the neck of newborns, it is important to differentiate the lesion, considering a possible diagnosis of heterotopic glial tissue.

A 3-year-old girl who had been suffering from prolonged fever and cough was referred to our institute. Chest roentgenography showed a huge mass at the right upper thoracic cavity. Chest computed tomography also showed a huge mass that compressed the mediastinum to the left side and caused tracheal stenosis. The mass was composed mainly of adipose tissue, and blood supply to it was sparse, judging from contrast medium enhancement. Radiologists diagnosed the mass as lipoblastoma and we decided to perform an emergency operation to resect the tumor. The patient underwent the operation with right thoracotomy. The tumor originated from the right chest wall between the fourth and fifth ribs. The tumor was excised with partial resection of the parietal pleura and periosteum of the fifth rib. Lipoblastoma originating from the chest wall is rare, and the recurrence rate of lipoblastoma after the first excision is not low. Our case, in particular, was a diffuse type of lipoblastoma, and careful follow up will be needed in the future.

In this report, we present a case of extremely rare cavernous hemangioma (venous malformation) in the inguinal canal. An 11-year-old boy was brought to our hospital, because of a painless palpable mass in the left inguinal region since infancy. He was diagnosed as having left inguinal hernia. The exploration using an inguinal approach showed a blue hyaline-like mass in the inguinal canal, so we suspected varicocele testis. During the operation, the mass was totally resected, and histopathological analytical results led to the discovery of a cavernous hemangioma. To the best of our knowledge, this is the second reported pediatric case of hemangioma in the inguinal canal. In the case of untypical inguinal hernia, we should consider the differential diagnosis of lesions in the inguinal region, and we must perform ultrasonography tests, medical history investigation, and physical examination. Retrospectively, ultrasonography revealed an enhanced posterior shadow, which is a characteristic finding of hemangioma, and it was considered to be an important finding in untypical inguinal hernia.

Reports on laparoscopic hepatectomy for malignant tumors in children are few. We present a case of pure laparoscopic partial hepatectomy for hepatoblastoma. A baby girl with Beckwith-Wiedemann syndrome underwent surgical closure of an omphalocele on the sixth day of life. At 4 months, sonographic screening revealed a liver tumor. The alpha-fetoprotein level was 23,060.69 ng/ml, and contrast-enhanced computed tomography revealed a 3.7-cm mass at the left lateral segment of the liver. A biopsy of the mass confirmed the diagnosis of hepatoblastoma (PRETEXT I). After 4 cycles of cisplatin monotherapy, the size of the tumor decreased to 2.7 cm. Laparoscopic partial hepatectomy was performed when she was 6 months old. An umbilical incision was made in an inverted Y shape, allowing for E-Z Access^TM with a Lap Protector^TM insertion through which a 1.2-cm trocar was introduced. Two 5-mm working ports were placed lateral to the rectus muscle of either side immediately superior to the umbilicus. After placing a tape around the porta hepatis for the Pringle maneuver in the case of severe bleeding, intraoperative ultrasonography was performed for the identification of the vascular tree and hepatic and portal veins, and the localization of the tumor for accurate and safe resection. Enseal^® was used to divide the hepatic parenchyma. Branched vessels and ducts were clipped and transected using Enseal^®. The surgical specimen was extracted through the umbilical incision without enlarging the incision. The patient had an uncomplicated recovery. No recurrence was observed during a 2-year follow-up.