Masashi Minato

Irreducible indirect inguinal hernia containing uterus, ovaries, and Fallopian tubes

An indirect inguinal hernia containing the entire uterus, ovaries, and Fallopian tubes is extremely rare in pediatrics. The present report describes the very rare case of a 1-month-old girl with an irreducible indirect inguinal hernia containing the entire uterus, ovaries, and Fallopian tubes, and the successful surgical treatment of simple herniorraphy. We review the literature on this type of relationship between indirect inguinal hernia and hernial visceras of the uterus, ovaries, and Fallopian tubes and discuss the clinical features of this complication. Furthermore, the possible cause of indirect inguinal hernia containing the uterus, ovaries, and Fallopian tubes was explored.

Clinical prognostic value of DNA methylation in hepatoblastoma: Four novel tumor suppressor candidates.

Hepatoblastoma (HB) is very rare but the most common malignant neoplasm of the liver occurring in children. Despite improvements in therapy, outcomes for patients with advanced HB that is refractory to standard preoperative chemotherapy remain unsatisfactory. To improve the survival rate among this group, identification of novel prognostic markers and therapeutic targets is needed. We have previously reported that altered DNA methylation patterns are of biological and clinical importance in HB. In the present study, using genome‐wide methylation analysis and bisulfite pyrosequencing with specimens from HB tumors, we detected nine methylated genes. We then focused on four of those genes, GPR180, MST1R, OCIAD2, and PARP6, because they likely encode tumor suppressors and their increase of methylation was associated with a poor prognosis. The methylation status of the four genes was also associated with age at diagnosis, and significant association with the presence of metastatic tumors was seen in three of the four genes. Multivariate analysis revealed that the presence of metastatic tumors and increase of methylation of GPR180 were independent prognostic factors affecting event‐free survival. These findings indicate that the four novel tumor suppressor candidates are potentially useful molecular markers predictive of a poor outcome in HB patients, which may serve as the basis for improved therapeutic strategies when clinical trials are carried out.

Hepatic osteodystrophy complicated with bone fracture in early infants with biliary atresia

Biliary atresia (BA) is one of the major hepatobiliary abnormalities in infants and one of the causes of hepatic osteodystrophy. Bone disease may be caused by the malabsorption of calcium and magnesium by vitamin D in hepatobiliary diseases in which bile flow into the intestines is deficient or absent. Bone fracture before Kasai hepatic portoenterostomy or within one month after the procedure in an infant with BA is very rare. We herein report two infants: one infant with BA who initially presented with a bone fracture before Kasai hepatic portoenterostomy, and the other at 4 wk after Kasai hepatic portoenterostomy, and also provide a review of the literature. Moreover, we conclude that clinicians should consider BA in infants with bone fracture during early infancy.

Meconium pseudocyst with particular pathologic findings: a case report and review of the literature

Meconium peritonitis is a sterile chemical peritonitis caused by bowel perforation with intraperitoneal extravasation of the meconium in utero. When the inflamed intestinal loops become fixed, meconium peritonitis leads to a cystic cavity with a fibrous wall, and the result is termed cystic-type meconium peritonitis. On the contrary, a meconium pseudocyst has a muscle layer continuous with the normal intestine and is distinguished from cystic-type meconium peritonitis based on the histopathologic findings. This report describes the rare case of a neonate complicated by a meconium pseudocyst, which was successfully treated with 1-stage resection and primary anastomosis. There have been few cases of meconium pseudocysts reported in the literature. Meconium peritonitis should be considered in the differential diagnosis in patients who develop large abdominal cysts with air and fluid content. Cystic-type meconium peritonitis is usually treated using drainage with subsequent elective surgery. However, for a meconium pseudocyst, 1-stage intestinal resection with primary anastomosis may be recommended. A meconium pseudocyst may be treatable using 1-stage resection based on histopathologic features.

Chylous ascites caused by resection of a choledochal cyst

Chylous ascites is a rare complication of abdominal surgery in children. Particularly, reports of postoperative chylous ascites are rare. This report describes the very rare case of a 10-month-old girl complicated by chylous ascites after resection of a choledochal cyst with a Roux-en-Y hepaticojejunostomy, who was successfully treated medically. To date, we have found a few cases of postoperative chylous ascites in the paediatric literature. To the best of our knowledge, this is the first report of chylous ascites after the resection of a choledochal cyst in a child who was successfully treated solely by no fasting. No fasting might be a therapeutic option of paediatric postoperative chylous ascites after the resection of a choledochal cyst if the outflow volume of chylous ascites is small.

Anal canal duplication presenting with abscess formation

Anal canal duplication (ACD) is the rarest among the duplications of the alimentary tract and can be defined as a duplication along the posterior side of the anal canal, with a perineal orifice situated just behind the anus. It is often found on simple perineal inspection as sagittally located double anal orifices with no symptoms. Other diseases such as fistula in ano, hindgut or rectal duplications need to be differentiated from ACD anatomically or on pathology. This report describes the rare case of a 14-year-old girl with ACD and perineal abscess. We discuss the characteristics of this type of disorder, particularly symptoms associated with ACD. A 14-year-old girl with no significant medical history presented with fever and perianal pain. The fact that she had two holes in the anus had been noted by her family since the age of 1, but this was ignored. She had not complained of constipation or anal pain. On perineal inspection, redness, swelling and induration were seen around a perianal orifice located in the midline posterior to the true anus. Enhanced computed tomography showed marked fluid collection in the back of the rectum surrounded by a thick wall that was partially contrasted, measuring 5.4 9 3.5 9 4.0 cm in diameter (Fig. S1). I.v. antibiotics were started immediately and the patient recovered without surgical drainage. The patient was referred to Hokkaido University Hospital for further evaluation and treatment to prevent recurrent abscess formation. On physical examination, the anus was normally located, and no anorectal stenosis was observed. Perianal orifice was evident without redness or fluid discharge (Fig. 1). There was no perineal pain on palpation. Fistulography showed a 3-cmlong narrow canal without connection to the rectum. Pelvic magnetic resonance imaging clearly showed normal muscle components, but the anatomical relationship between the ACD and the surrounding muscle structures were not obvious. On colorectal endoscopy, a fistula opening was not found at the rectal wall. The patient underwent surgical removal of the ACD by a perineal approach. The lesion circumscribing the orifice was removed with incision, and the fistulous tract dissected carefully from the external anal sphincter and part of the striated muscle complex, which were detected with an electric stimulator. Although the wall at the cranial end was partly shared with the rectum, it was resected completely without rectal injury. The removed ACD had a narrow fistula, measured approximately 30 mm in diameter, and had no connection to the rectum. Histopathology showed stratified squamous epithelium and partially columnar epithelial lining, with a small amount of adjacent smooth muscle bundles (Fig. S2). Pathology confirmed a final diagnosis of ACD. The postoperative clinical course was uneventful and the patient has had good bowel function with normal appearance of the perineum for 1 year after the operation. Abscess formation associated with ACD is extremely rare: only five cases in children have been previously reported in the literature. Of the six total patients, including the present one, all were female and the average age of presentation was 64 months (range, 0–14 years). All patients except one had duplications that were tubular in structure. Internal communication with the native anal canal was present in two patients. Although one patient had additional anomalies (tethered cord Fig. 1 Small anal canal duplication orifice visible in the midline (arrow) posterior to the normally located anus.

Hemorrhagic shock due to spontaneous rupture of adrenal neuroblastoma in an infant: a rare case and review of the literature.

Spontaneous rupture of adrenal neuroblastoma is very rare in infants, in contrast to neonates. This report describes a 9-month-old boy presenting with acute hemorrhagic shock due to spontaneous rupture of adrenal neuroblastoma. MYCN oncogene amplification may be a predisposing factor for spontaneous rupture and bleeding of neuroblastoma. An appropriate surgical treatment for this condition must be discussed according to the patient’s general state and the tumor features, such as staging, the origin, and local invasiveness.

One-Stage Laparoscopic Surgery for Pulmonary Sequestration and Hiatal Hernia in a 2-Year-Old Girl

We herein report a case of one-stage laparoscopic surgery for extralobar pulmonary sequestration (EPS) and hiatal hernia. Our patient was a 2-year-old girl who was diagnosed as a mediastinal mass lesion. Postnatal computed tomography revealed that the mediastinal mass was an EPS. Two weeks after birth, the patient developed gastroesophageal reflux (GER), and esophagography showed a hiatal hernia. At 2 years of age, she underwent one-stage laparoscopic Nissens fundoplication for GER with resection of the EPS in the posterior mediastinum. The sequestrated lung was grasped via the esophageal hiatus; three aberrant blood vessels were dissected to allow removal of the sequestration through the umbilical port site. The esophageal hiatus was repaired and Nissens fundoplication was performed laparoscopically. The patients postoperative course was uneventful, with no recurrence of GER symptoms for 1 year. We conclude that one-stage laparoscopic surgery is useful for patients with EPS and hiatal hernia.

Impact of umbilical polyp resection: A report and literature review

The preferred treatment for this lesion should be the total resection of the umbilical polyp with a mini-laparotomy because residual intestinal mucosa at the umbilicus can be confirmed to eliminate the risk of recurrence.

Torsion of an Accessory Spleen: A Rare Case and Review of the Literature

Torsion of an accessory spleen is a very rare entity that causes an acute abdomen in children, and it is difficult to accurately diagnose preoperatively. We herein report a case of acute abdominal pain in a 5-year-old boy caused by torsion of an accessory spleen. In this case, abdominal US detected the whorl of the twisted pedicle, which may be a hallmark to diagnose torsion of an accessory spleen. We must consider torsion of an accessory spleen as a differential diagnosis when encountering acute abdomen in children with a palpable mass.