Abdominal lymphatic malformation (LM) is a rare disease that can cause acute abdomen and abdominal distention requiring urgent diagnosis and treatment. It may also be detected incidentally. Appropriate treatment strategies for this disease have not been established. Here, we present our experience and report on the effectiveness of drainage in cases of LM with infection and inflammation. The medical records of 28 patients treated for LM (intraperitoneal or retroperitoneal) between 1997 and 2016 were retrospectively reviewed. The median age at diagnosis was three years. There were 24 symptomatic patients, including 10 with infection/inflammation, four with intracystic hemorrhage, two with non-infectious non-hemorrhagic enlargement, two with torsion, and six with other conditions. Intervention was performed in all symptomatic patients. Surgical resection was performed in 13 of 14 (92.9%) patients without infection and inflammation. In the 10 patients with infection and inflammation, antibiotic treatment with or without drainage successfully controlled infection in nine, whereas one required emergency resection after antibiotics. Of these nine patients, one underwent surgery for subsequent ileus, one underwent sclerotherapy, and seven were followed up as outpatients; two required surgery and regression occurred in five patients. No recurrences were observed. Symptomatic abdominal LM is an indication for surgical resection; however, in patients with infection and inflammation, antibiotic therapy with or without drainage may be an option to control the symptom, leading to subsequent regression.

A 15-month-old male patient, who underwent surgery for esophageal atresia (EA) with distal tracheoesophageal fistula at the age of one day, in whom we had difficulty in inserting a post-anastomotic tube, was suspected of having congenital esophageal stenosis (CES) at the lower esophagus. Postoperative feeding and weight gain were satisfactory, but frequent vomiting appeared from about seven months after birth when weaning diet was started, and endoscopic ultrasonography revealed CES associated with a tracheobronchial remnant. At the age of one year and three months, congenital esophageal stricture resection and esophageal anastomosis were performed. Since the CES anastomotic site was found to be highly tense, blood flow was evaluated at the anastomotic site using the indocyanine green (ICG) fluorescence method after the esophageal anastomosis. It was confirmed that the blood flow at the anastomotic site was maintained. Postoperative anastomotic stenosis was observed, but it was improved by endoscopic balloon dilatation. Currently at the age of three years, he eats a normal diet and gaining weight well. We report on the evaluation of blood flow at the anastomotic site using the ICG fluorescence method in CES esophageal anastomosis after EA.

A six-year-old boy was admitted to the pediatric department of our hospital because of abdominal and chest pains. Blood test findings on admission showed elevated CRP and LDH levels. The patient was referred to our department because of worsening abdominal pain after admission. Abdominal computed tomography showed left-sided deviation of the small intestine with wall thickening and poor fixation of the colon. Because of the rapid progression of the abdominal pain, torsion of the small intestine or internal hernia was suspected, and emergency laparotomy was performed. The surgical findings included venous congestion in the jejunum and a 13 × 16 × 65 mm Landzert fossa, and we diagnosed the patient as having a left paraduodenal hernia. There was no evidence of intestinal necrosis, and the operation was completed with the closure of the hernia gate. Postoperatively, the patient developed paralytic ileus and required conservative treatment. The patient was discharged from the hospital 24 days after the operation without symptom recurrence. Although it has been recently reported that the finding of a sac-like appearance on CT images is useful for the diagnosis of paraduodenal hernia, it was difficult to detect in our patient preoperatively. It was suggested that it was necessary to recognize left paraduodenal hernia as one of the differential indicators of acute abdomen.

Pediatric adrenocortical carcinoma is a malignant tumor with poor prognosis. We report a case of an infant with advanced adrenocortical carcinoma that was safely and completely resected after neoadjuvant chemotherapy. A five-month-old girl with an abdominal mass was admitted to our hospital. Computed tomography showed a right adrenal tumor with direct invasion into the liver and lymph node metastasis. The diagnosis of right adrenocortical carcinoma was obtained through an incisional biopsy. Neoadjuvant chemotherapy with mitotane and antitumor agents successfully reduced the volume of the tumor and resulted in a partial response. The right adrenal tumor and posterior segment of the liver were resected en bloc. Paraaortic and right renal hilar lymphadenectomy were additionally performed. Histopathology showed viable tumor cells in the primary tumor as well as in the lymph nodes. After additional courses of adjuvant chemotherapy, the patient remains relapse-free for two years and seven months. Complete resection of the tumor is essential in the treatment of pediatric adrenocortical carcinoma. On the other hand, the efficacy of chemotherapy has not been clarified. Although the long-term prognosis of advanced cases reported in our country was generally poor, chemotherapy tended to improve the prognosis. Notably, the combination of mitotane and antitumor agents correlated significantly with prolonged survival. Neoadjuvant chemotherapy may reduce tumor size and enable a safe and complete resection in locally advanced cases.

The patient was an 11-year-old boy who presented to the emergency department with abdominal pain and vomiting, and he was diagnosed with intussusception on the basis of the findings of abdominal ultrasonography and CT. After radiological reduction, he was admitted to the hospital. He was discharged on the fifth day without any recurrence of symptoms. Three weeks after the initial episode of the intussusception, he complained of abdominal pain again. Abdominal ultrasonography showed an invaginated intestine. He was emergently admitted after radiological reduction, and operation was performed on the third day. Laparoscopic observation revealed a serosal depression in the ileum 50 cm orally from the distal end of the ileum, and a mass was palpated in the same area. The lesion was extracted from the umbilical wound, and the ileum was partially resected. Histopathological examination revealed a broad-based diverticulum with a mucosal bridge covering its entrance. The bridge did not contain a muscular layer, leading to the final diagnosis of Meckel’s diverticulum with a mucosal bridge. His postoperative course was uneventful, and he was discharged on the sixth postoperative day with no recurrence of symptoms since then. As far as we know, this is the first report of a case of Meckel’s diverticulum with a mucosal bridge.

Introduction: Mesenchymal hamartoma of the liver (MHL) is a rare pediatric benign hepatic tumor that affects infants under the age of three years. MHL is also considered to be the origin of undifferentiated embryonal sarcoma, a malignant liver tumor. To improve outcomes and understand its pathophysiology, it is important to analyze the clinical characteristics of MHL. We report on two cases of MHL in older children and review the clinical characteristics of 81 cases in Japan, including our two cases. Case 1: The patient was a five-year-old girl. A 15 cm multicystic mass was found in the left lobe. The AFP level was normal. The patient underwent a left lobectomy. A tumor diagnosis of MHL was concluded. No recurrence has been detected in the 3.8 years after surgery. Case 2: The patient was a 15-year-old girl. An 8 cm multicystic mass was found in the S7/8 right lobes with tooth-like calcifications in the cyst wall. The preoperative diagnosis was hepatic teratoma. The patient underwent an extended right posterior hepatic segmentectomy. A tumor diagnosis of MHL was concluded. Conclusions: In our cases, no recurrence has been detected in over three years after surgery. However, in the review of 81 cases in Japan, recurrence was observed in three cases, one of which was suspected to be malignant transformation. According to the biological characteristics of MHL, complete resection of the tumor is essential.

A Farrell*Valve Bag was connected to a patient’s feeding tube, which allowed extra air to vent from the stomach and reduced the symptoms of gas bloating. Here, we report a case of Cornelia de Lange syndrome accompanied by gas bloating syndrome. A 24-year-old woman was referred to our hospital owing to gas bloating and cecal volvulus associated with mobile cecum. She had histories of fundoplication due to gastroesophageal reflux disease at four years of age and reduction of cecal volvulus at 21 years of age. After her recovery from cecal volvulus, enteral nutrition remained difficult owing to gas bloating. The use of a Farrell*Valve Bag to enable enteral feeding at gastrostomy improved gas bloating and gastrostomy tube leakage.

We report a case of intrahepatic cholangiocarcinoma during long-term follow-up for biliary atresia. The patient was a 25-year-old male. At the age of 78 days, he underwent portoenterostomy with an antireflux valve for III-a1-ν biliary atresia and was followed up in the pediatric department. At the age of 25 years, cholangitis with jaundice was observed, and a double-balloon endoscopic retrograde cholangiography was performed. However, the anastomosis could not be reached, and balloon dilatation was performed for stenosis of the antireflux valve. When he was referred to our department again, his CA19-9 level was high at 7,092 U/ml. A biopsy was performed on a neoplastic lesion in the right lobe of the liver, and the patient was diagnosed as having stage IVB intrahepatic cholangiocarcinoma (peritoneal dissemination). Percutaneous transhepatic stenting was performed for portal vein invasion, and chemotherapy was administered. At 11 months after the start of chemotherapy, the patient died of gastrointestinal bleeding due to duodenal invasion. Screening for malignancy is essential in the long-term management of biliary atresia.