Purpose: The survival of infants with surgical diseases has gradually been improving with advances in perinatal medicine. Partly because of ethical issues, establishing an appropriate diagnostic and treatment strategy for newborns with chromosomal abnormalities is sometimes difficult. We report surgical cases with chromosomal abnormalities at our hospital.

Methods: The subjects were 32 surgical cases who had trisomy 13 and 18 in our department between 1980 and 2015. Four cases were lost in follow up and were excluded. Primary disease, gestational age, birth weight, surgical treatment, and surgical outcome were analyzed retrospectively.

Results: Mean gestational age was 33.6 weeks, mean birth weight was 1,459.7 g, heart disease rate was 90.6%, and mean duration of survival was 229 days. Diseases were gastrointestinal disease (n = 25), respiratory disease (n = 5), tumor (n = 1), and others (n = 1). The survival duration was significantly longer for the patients who received pediatric surgical treatment and cardiovascular surgical treatment.

Conclusions: Trisomy 13 and 18 cases have a poor survival rate. The medical team and parents should spend sufficient time establishing an appropriate treatment strategy, and a multidisciplinary medical team should fully understand the infant and family’s social background prior to treatment. It was considered that the surgical treatment would affect life prognosis. Further studies are needed to improve support measures for infants with severe defects.

Purpose: Sclerotherapy and/or resection are the 2 main therapeutic strategies used in the management of lymphangioma. Recently, we have focused on the Kampo medicine, Eppikajutsuto (TJ-28), as another therapeutic option because of its safety and convenience. In this paper, we aim to provide an update of our single institution’s experience.

Methods: The records of 10 patients who underwent therapy for lymphangioma with TJ-28 from 2013–2015 were reviewed by comparing age, region, type of lymphangioma, size, and prognosis.

Results: The ages were from newborn to 21 years (mean: 8 years). The average duration until the disappearance of the lesion was 5.8 months. There were 6 patients with the lesion in the cervical region, 1 on the body, and 3 in the extremities. There were 9 cases of multiple cysts and 1 case of simple cyst. In 5 cases, the administration of TJ-28 resulted in cyst shrinkage, and in 5 cases, the cysts disappeared without any side effects or sign of infection. One case needed re-administration owing to recurrence.

Conclusion: The Kampo medicine TJ-28 should be the first option for the treatment of lymphangioma rather than sclerotherapy and/or surgical resection.

Purpose: In this study, we aimed to assess the short-term effects of laparoscopic modified Rossetti fundoplication (defined by liver retraction without mobilization, no crural repair, short gastric vessels left intact, and a small floppy wrap) on clinical evaluations of neurologically impaired patients with recurrent vomiting.

Methods: A retrospective review was performed on 18 neurologically impaired patients who underwent laparoscopic modified Rossetti fundoplication from April 1, 2012 to December 31, 2014. The authors assessed symptoms of recurrent reflux at 12 months postoperatively.

Results: None of the patients had recurrence of vomiting at 12 months postoperatively.

Conclusion: We believe that modified Rossetti fundoplication can be performed with good short-term outcome in all neurologically impaired patients with recurrent vomiting.

We report a case of mesoblastic nephroma (MN) with hypertension and hypercalcemia. The patient required emergency surgery for this oncologic emergency. The patient was a 2-month-old girl who was found to have fever with a massive abdominal mass (84 × 107 × 89 mm) at the left kidney. Her blood pressure was 150/90 mmHg, plasma rennin activity was > 20 ng/ml/h, and serum calcium level was 14.8 mg/dl. Because her hypertension could not be controlled conservatively, we decided to perform nephrectomy on the fifth hospital day. Her blood pressure normalized immediately after surgery. Pathological diagnosis was cellular-type MN with positive expression of the ETV6-NTRK3 chimeric gene. No additional treatments were provided, and she has been free of relapse for 5 years.

Long-term survivors without liver transplantation after Kasai have the problem of hepatic cancerization. Here, we report the case of a patient who developed intrahepatic cholangiocarcinoma (ICC) and hepatocellular carcinoma (HCC), i.e., combined HCC type, after surgery for biliary atresia (BA). The patient was a 39-year-old male who underwent jejunal interposition hepatic portoduodenostomy for BA, I-b2-γ type, 85 days after birth. Esophageal varices appeared when he was 10 years old and worsened from 17 years of age. He had cholangitis repeatedly at 27 years of age and liver cirrhosis with ascites at 35 years of age. The tumor markers were rising and a two-pathologic-type lesion in S1 was pointed out in imaging studies; thus, he was diagnosed as having ICC and HCC (combined HCC type). Combined HCC showed T3cN0cM0 clinical stage III and liver damage C; thus, he underwent transcatheter arterial chemoembolization (TACE). HCC showed complete necrosis, but ICC showed no mass reduction; thus, we performed gemcitabine chemotherapy. Although he underwent chemotherapy with gemcitabine, the tumor size continued to increase and liver failure developed. He died 8 months after treatment. This is the only case of the combined HCC type and the oldest among all cases of hepatic cancer after surgery for BA. Because all patients with cholangiocarcinoma died after surgery, their prognosis was considered poor. In the follow up of long-term survivors without liver transplantation, it is considered that management for ICC after operation is important.

A newborn boy with progressive bilious vomiting despite his normal perineal appearance was referred to us. Subsequent contrast enema showed rectal atresia. Left transverse colostomy was performed on the third day of life. After colostomy, he was diagnosed as having rectal atresia. At 10 months of age, transanal excision of the membrane was performed. His anal function after operation was satisfactory. Preceding colostomy may provide an accurate diagnosis of this rare condition and has made us choose the function-preserving and less invasive definite operation for canal.

The surgical management of pediatric subglottic stenosis remains a challenging task to pediatric surgeons and otorhinolaryngologists. Children suffering from Myer-Cotton Grades III and IV subglottic stenosis are put in a particularly difficult situation for treatment. Here, we report the case of a ten-year-old girl with severe acquired subglottic stenosis, who was successfully treated. Preoperative bronchoscopy had revealed severe Grade III (95%) stenosis associated with glottic involvement. We performed partial cricotracheal resection and thyrotracheal anastomosis (PCTR) with a stenting T-tube to secure the reconstructed laryngeal lumen and the postoperative airway. The patient was treated in Pediatric Intensive Care Unit (PICU) for a week after surgery with artificial ventilation under heavy sedation. As postoperative bronchoscopy revealed a fragile larynx and mucosal edema, the first trial of T-tube removal was postponed for 6 months, resulting in anastomotic stenosis. She spent the usual childhood at home and in school with T-tube tracheostomy until the removal of the T-tube. The second trial of the T-tube was successfully performed 2 years after surgery, but her persistent hoarse voice remained. PCTR with T-tube stenting is one of the effective and safe definitive procedures for pediatric patients with severe subglottic stenosis (Grades III and IV).

A six-month-old boy was diagnosed as having congenital hyperinsulinism (CHI) due to recurrent hypoglycemia in the neonatal period. He was initially diagnosed as having diffuse-type CHI, as there were no gene mutations, such as the paternally inherited mutation of ABCC8 or KCNJ11. At five months of age, he underwent 18F-fluoro-L-DOPA positron emission tomography (18F-DOPA PET), which revealed a multifocal lesion in the pancreas head. His diagnosis was then changed to focal-type CHI, and he was transferred to our hospital for surgery. Duodenum-preserving pancreatic head resection, pancreas and jejunum anastomosis, and Roux-en-Y reconstruction were performed at six months of age. He was discharged 44 days after the operation. One year has passed since the operation was performed, and the patient has experienced no complications associated with his blood regulation. Although focal-type CHI without gene mutations is relatively rare in Japan, we recommend 18F-DOPA PET as a reliable examination method and our surgical management as the standard strategy for treating focal-type CHI associated with a pancreatic head lesion.

An 8-year-old girl was referred to our hospital because of fever of unknown origin, and pain in her right abdomen, left shoulder, left chest, and left lower back. She presented with knock pain of the left lower back and right abdominal tenderness without rebound tenderness or defense. CT revealed a 36 × 35 × 20-mm-sized solitary mass without enhancement in the left pleural space. On the basis of the clinical course and CT findings, the torsion of an extralobar pulmonary sequestration was suspected; however, no feeding artery was identified and pleural effusion was confirmed the next day by dynamic CT. She underwent thoracotomy on the fourth day of hospitalization. The peduncle of the mass was found to be twisted, and the sequestrum was resected. The initial diagnosis (torsion of an extralobar pulmonary sequestration) was confirmed pathologically. She successfully recovered after the operation, and left the hospital on the fourth day after surgery. Although most cases of extralobar pulmonary sequestration are asymptomatic and are detected incidentally, in rare cases, it presents with abdominal pain and chest pain due to the torsion of its vascular pedicles. It is usually difficult to make a preoperative diagnosis on the basis of imaging findings, and strong pleural adhesion develops over time. It is therefore preferable to perform the operation within 7 days of when the onset of the torsion of the sequestrum was suspected to have occurred.

We present a rare case of inguinoscrotal hernia of the ureter. A 14-year-old boy who presented with left scrotal bulging was referred to our hospital. He underwent left hernioplasty at the age of one. He was diagnosed as having hydrocele testis. We performed surgery under general anesthesia. After opening the inguinal canal, we found a loop tubular structure arising from the deep inguinal ring, which clinically appeared to be a ureter. Postoperatively, drip infusion pyelography-computed tomography (DIP-CT) showed an anatomical anomaly of the left ureter that reached the inguinal canal. We noted his medical history of a hernia sac present at the age of one. We diagnosed him as having congenital paraperitoneal inguinoscrotal hernia of the ureter. A ureter in the inguinal canal is an uncommon presentation. Surgeons should keep in mind this entity, which presents preoperative diagnostic difficulties, and should be cautious when they find a thick vas deferens during hernioplasty. This is the first reported case in Japan.

A 9-year-old girl was run over by a truck and brought to a previous hospital. An external skeletal fixation was performed for her pelvic fracture. Although oral intake started postinjury day 5, she had been suffering from repeated intestinal obstruction since then. At 6 weeks after the injury, she was referred to our hospital because she had fever in addition to intestinal obstruction symptoms. An urgent enhanced computed tomography scan revealed a dilated intestine and pelvic abscess. She underwent laparoscopic-assisted surgery with minilaparotomy. Before the laparoscopic procedure, intestinal decompression was performed by handling the intestine through the wound at the umbilicus. Then, we found a mesenteric defect with severe stenosis of the ileum. The affected bowel segment was resected, and primary anastomosis was performed. The patient was transferred to the previous hospital 13 days later with normal bowel function for further treatment of her pelvic fracture. Although posttraumatic intestinal stenosis is a rare condition and difficult to diagnose, laparoscopic-assisted surgery could be feasible and useful for the diagnosis and treatment of intestinal obstruction even with a dilated intestine and peritonitis.

We report the case of a 9-month-old boy with esophago-tracheal fistula and recurrent nerve palsy due to lithium battery ingestion. Although an endoscopic removal of the battery lodged in the esophageal inlet under general anesthesia was successful, it took 6 hours before the removal. On day 8 after the removal, the patient choked after drinking water and chest X-ray imaging showed aspiration pneumonia. The presence of esophago-tracheal fistula and recurrent nerve palsy was revealed. Conservative treatments with NPO (nothing per os), nutritional support with parenteral and enteral nutrition, and coagulation factor XIII were provided, and oral intake was resumed after the spontaneous closure of the fistula was confirmed on day 71. By searching the databases of Japanese medical publications, 57 children including our own patient who ingested a lithium battery, which resulted in the esophageal foreign body trauma, were identified. There was a rapid increase in the number of case reports since 2000. Since lithium battery ingestion causes serious tissue injuries, it is extremely important to warn families against battery ingestion. It is also important to urge manufacturers of batteries and electric appliances to take measures to prevent health damage caused by lithium battery ingestion in children.