Purpose: We reviewed and analyzed the clinical outcomes of all patients with congenital diaphragmatic hernia (CDH) in Okinawa Prefecture and reevaluated the prenatally diagnosed patients’ severity classification group.

Methods: Between 2002 and 2021, 61 patients diagnosed with CDH were treated at our institution. Among them, 35 patients who were prenatally diagnosed with isolated left CDH were classified according to Kitano’s and Usui’s classification systems. Information regarding their perinatal status, cardiorespiratory management, and prognosis was retrospectively reviewed. In addition, the results were compared with those from a national survey.

Results: Extracorporeal membrane oxygenation (ECMO) was administered in 18.0% of patients, 3.3% were unsuitable for surgery, and the overall survival rate was 83.6%. According to Kitano’s classification, 27 patients (77.1%) were classified into Group I; 6 (17.1%), into Group II; and 2 (5.7%), into Group III. Of these 35 prenatally diagnosed patients, 25.7% underwent ECMO, 0% were unable to undergo surgery, and the overall survival was 91.4%. Compared with the nationwide survey results, the prognosis was significantly better in all the patients classified using Kitano’s classification system. In our cohort, ECMO utility and operation rates were significantly higher than those of the national survey. However, the intact discharge rate was lower in our patients, reflecting high complication rates.

Conclusions: The survival rates in our sample were excellent; this may be because almost all the patients were able to undergo surgery owing to the high frequency of ECMO support. We should aim to reduce postoperative complications and improve long-term quality of life.

Purpose: To determine the clinical factors associated with the development of pectus excavatum (PE) following the repair of a congenital diaphragmatic hernia (CDH).

Methods: We retrospectively analyzed the medical records of patients with CDH who underwent surgical intervention during the neonatal period at our institution from January 2001 to December 2018 and were followed-up for at least three years postoperatively. We examined the clinical backgrounds of the patients and the CDH severity index to analyze the occurrence of PE.

Results: Among the 89 patients with CDH who underwent surgery, 23 (25.8%) developed PE. The percentage of patients who underwent nitric oxide (NO) inhalation was significantly higher in patients with PE (100%) than in those without PE (80%). The duration of mechanical ventilation was significantly longer in patients with PE (16 days; 5–44 days) than in those without PE (10 days; 1–1,824 days). Additionally, the duration of oxygen administration was significantly longer in patients with PE (37 days; 6–817 days) than in those without PE (20 days; 4–1,824 days). The percentage of patients who underwent surgery with patch closure was higher in those with PE (16 patients; 70%) than in those without PE (30 patients; 45%). However, the difference was not statistically significant.

Conclusions: The development of PE following CDH repair was closely related to the type of respiratory management, such as the use of NO, mechanical ventilation, and oxygen therapy.

We report two cases of biliary atresia (BA) in which living-donor liver transplantation (LDLT) was performed owing to intractable cholangitis after additional hepatic portojejunostomy. [Case 1] The patient was a 15-year-old girl. Hepatic portojejunostomy was performed to treat BA. Owing to poor bile excretion, reanastomosis was performed at 2 months of age using a backflow prevention valve. Intractable cholangitis with jaundice appeared 15 years after the operation. An ileus duct was placed for jejunal stenosis using a regurgitation prevention valve; however, the intrahepatic bile duct dilatation and jaundice did not improve, and LDLT was performed. [Case 2] The patient was a 26-year-old man. Hepatic portojejunostomy was performed using a backflow prevention valve. The patient’s jaundice worsened 25 years after the operation and he was transferred to our department; however, the cholangitis did not improve and LDLT was performed. An antireflux valve may cause cholestatic cholangitis in patients with long-term BA.

We report the case of a two-year-old boy with a fistula of the right buttock. The fistula was detected at one year of age without any symptoms. It became red, and pain with exudate fluid appeared at two years of age. He was treated with an antibacterial agent and by drainage at a previous hospital. He was brought to our hospital because of a recurrent infection in a short period. Magnetic resonance imaging showed a fistula of the right buttock, which ran to the retrorectal space, and surgery was performed to remove the fistula. He experienced recurrent infection two weeks after the initial surgery and was treated by drainage and with an antibacterial agent. CT revealed the remaining deep-seated abscess. After resolving the inflammation, he underwent surgery and the fistula was completely resected. Histopathological findings showed that the wall was covered with stratified squamous epithelia. We diagnosed the patient as having a congenital buttock sinus tract, but it is consistent with an atypical case of a congenital dermal sinus tract. There is a high possibility of recurrent infection in either case, and thus surgical resection should be considered even if the patient is asymptomatic.

We report the case of a patient presenting with short bowel syndrome treated with teduglutide. A 10-year-old boy was born with a short bowel lacking the ileum and colon. For nutritional management, a central venous catheter (CVC) was indwelled from the age of two years, and in addition to oral intake, total parenteral nutrition was started. He had several catheter-related bloodstream infections (CRBSIs) and the CVC had to be replaced. In 2021, the use of teduglutide, a glucagon-like peptide-2 analog preparation for the treatment of short bowel syndrome, was covered by National Health Insurance in Japan. One year has passed since he started using teduglutide at our hospital. Endoscopic examination revealed villus elongation and improvement in stool quality. His height and weight were normal for his age, and he was less dependent on parenteral nutrition and showed increased resistance to CRBSIs. As adverse events, he felt abdominal pain and had excess gas, which improved over time. We have shown the effects of teduglutide on a patient with short bowel syndrome who had a colostomy.

There have been only a limited number of previous studies on the evaluation of intestinal blood flow perfusion by indocyanine green (ICG) fluorescence angiography, especially for strangulated intestinal obstruction in children. A two-month-old boy was admitted to our hospital owing to repeated diarrhea and vomiting. Although he was initially diagnosed with viral gastroenteritis and his diarrhea improved the next day, strangulated intestinal obstruction was revealed on the third day of hospitalization. An emergency laparotomy was undertaken, and we found that a 40 cm length of the ileum was strangulated by the mesodiverticular band. The discoloration of the intestine was 4 cm from the oral side of the Meckel diverticulum to the terminal ileum. After the strangulation was released, ICG fluorescence angiography was performed to explore the possibility of preserving the ileocecal valve. Blood flow perfusion was observed up to 2 cm proximal to the ileocecal valve. Therefore, the necrotic bowel was resected while preserving the ileocecal valve, and the ileum was anastomosed end-to-end style. His postoperative course was uneventful and he was discharged on the 11th postoperative day. The ICG fluorescence angiography in this case was effective for objectively evaluating the blood flow perfusion in the ileocecal region and preserving the ileocecal valve. Still, a method for the detailed evaluation by ICG fluorescence angiography has not been established, and further research with the accumulation of cases should be conducted.

We report our experience of surgical spacer placement (SSP) using a bioabsorbable polyglycolic acid spacer for children with Ewing sarcoma requiring proton beam therapy. [Case 1] A 1-year-old boy diagnosed with iliac Ewing sarcoma underwent SSP by transverse abdominal incision. The spacer was inserted by mobilizing the descending colon. [Case 2] A 9-year-old boy diagnosed with sacral Ewing sarcoma underwent SSP by a laparoscopic procedure. At the time of this writing, no complications were observed. Reduction of the intestinal radiation dose was possible by SSP; thus, SSP can be considered minimally invasive and safe. The spacers disappeared within four months after the surgery on the magnetic resonance imaging scans in cases 1 and 2. An additional laparoscopic procedure may be helpful, depending on where the spacer is placed.

The patient was a 21-year-old female who had the Potts procedure performed at three months of age for low-type anorectal malformations. She had no history of attendance at our hospital after the age of eight years and had been self-managed since then. She was suffering from constipation one month prior to her arrival at our hospital and was hospitalized the day before by her previous physician. After admission, she developed fecal intestinal obstruction, abdominal compartment syndrome, and ischemia of the lower limbs, and was brought to our hospital for emergency care. Under general anesthesia, an emergency manual decompression was performed, and 9 kg of fecal matter was evacuated. The patient required a long period of intensive care, but she recovered without sequelae and is currently undergoing defecation control with medication and enemas at the Adult Clinic. Long-term follow-up is necessary after anorectal malformation surgery because of the possibility of defecatory dysfunction, abnormalities of the urinary system, spinal cord abnormalities, and sexual dysfunction. Therefore, it is essential to educate patients and their families to understand the disease, consider the treatment plan, and make the transition to adult medical care.

A 13-year-old girl fell while running and bruised her abdomen on the asphalt ground. She visited a nearby clinic on the same day and was followed up, but fever, abdominal pain, and vomiting persisted. Contrast-enhanced CT revealed the rupture of the pancreatic head/body, so she was referred to our hospital 15 days after the injury. She underwent endoscopic retrograde pancreatography (ERP), and since imaging from the main pancreatic duct did not enhance the distal pancreatic duct, we diagnosed her as having type IIIb traumatic pancreatic injury. We attempted to place an endoscopic nasopancreatic drainage (ENPD) tube on the distal side, but it reached only up to the rupture site. Fasting was introduced and antibiotics, gabexate mesylate, and octreotide acetate were administered, but percutaneous drainage was added because of decreased drainage from ENPD and increased ascites. After that, her ascites tended to decrease, her blood amylase level improved, and she was discharged from the hospital on the 86th day after her injury. Surgery is usually considered necessary for type IIIb traumatic pancreatic injury, but non-operative management is possible if a long time has passed since the onset.

Congenital pancreatic cysts are extremely rare, and there is no established treatment method. We report a case of a congenitally detected simple cyst resected laparoscopically later on in life, diagnosed as a pancreatic cyst with a review of the literature. The patient was a seven-year-old girl, whose fetal ultrasonography showed a simple cyst about 2 cm in diameter located in the left upper abdomen at the gestational age of 38 weeks and 2 days. She was born six days later, weighing 3,280 grams. Her pancreatic enzyme level was high at the age of four; otherwise, the course was asymptomatic. At the age of seven years and two months, a laparoscopic cystectomy was performed to prevent future complications such as pancreatitis and to confirm the diagnosis. The cyst was about 3 cm in diameter and communicated with the pancreatic duct. The cyst contained a large amount of atheromatous material consisting of protein, and the fluid had high levels of pancreatic enzymes. Pathological findings showed many columnar epithelial cells with partial papillary ducts, which lined the wall of the cyst and indicated the pancreatic origin of the cyst. The postoperative course was uneventful and now twelve months after the surgery, the patient is doing fine.

Solid pseudopapillary neoplasm (SPN) of the pancreas often occurs in young women. In this study, we investigated the treatment outcomes of SPN in our department. We retrospectively reviewed the medical records of three children with SPN treated in our department between 2015 and 2021. All three patients were girls who were 10–12 years of age at the time of surgery. Their symptoms included abdominal pain, vomiting, fever, and diarrhea. The tumor was located in the pancreatic tail in two patients and the pancreatic body in one patient. Two patients underwent distal pancreatectomy without splenectomy and one patient underwent distal pancreatectomy with splenectomy. In all the patients, the postoperative course was uneventful. The postoperative hospital stay was 9–12 days. The follow-up period was 18–78 months. All three patients were alive without recurrence. The surgical outcomes of SPN were favorable in our series. Complete resection of SPN provides a favorable prognosis, but spleen preservation should be considered in the surgical procedure.

We report a case of acute appendicitis in a teenage female with a history of Type 2A von Willebrand disease (VWD) who presented to our department with right lower abdominal pain. She was diagnosed with cellulitis-associated acute appendicitis and underwent conservative therapy considering the risk of bleeding. Blood tests during that time revealed a platelet count of 511,000, a PT-INR of 1.04, an APTT of 40 s, a VWF activity of <6%, and an FVIII activity of 23%. Given the presence of a fecalith in acute appendicitis, we planned for an interval appendectomy to minimize the risk of recurrence. During the waiting period for surgery, she experienced menstruation, and we utilized a recombinant von Willebrand factor (rVWF) product, which became available in Japan in 2020. The dose of rVWF for perioperative use was determined on the basis of VWF and FVIII activities at that time. Preparations for the perioperative period were conducted in accordance with the 2021 edition of the VWD treatment guidelines. Laparoscopic appendectomy was performed using standard techniques, and there were no bleeding events during the perioperative period, ensuring a safe procedure. In this report, we present a case of appendicitis in a patient with VWD Type 2A, a qualitative abnormality of the von Willebrand factor, and describe the use of the rVWF product for interval appendectomy.

Alternative methods of central venous catheter placement are considered in patients with occlusion of main access routes for central venous catheter insertion. We report the case of an infant with short bowel syndrome (focusing particularly on the surgical technique), who underwent successful placement of a central venous port through the azygos vein via the fifth intercostal vein under ultrasound guidance with thoracoscopic assistance. A 4-year-old boy with short bowel syndrome who received home parenteral nutrition developed recurrent catheter-induced bloodstream infections, which obstructed the superior vena cava and bilateral femoral veins. A dilated azygos vein was identified as a collateral bypass; therefore, we performed a right sixth intercostal vein puncture under ultrasound guidance and placed a central venous catheter via the azygos vein. The distal aspect of the confluence of the azygos vein was blocked during thoracoscopic surgery to introduce the guidewire into the superior vena cava, and a catheter was inserted into the azygos arch. Displacement of the catheter from the intercostal vein necessitated catheter reinsertion via the right fifth intercostal vein, one month postoperatively. The patient has had an uneventful course over 10 months postoperatively, and home total parenteral nutrition has been continued. Ultrasound-guided puncture of an intercostal vein under thoracoscopic guidance may be useful for central venous catheter placement in infants.