Hisayuki Miyagi

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.

Mediastinoscopic extended thymectomy for pediatric patients with myasthenia gravis.

BACKGROUND Extended thymectomy is indicated for children with myasthenia gravis (MG) when drug-resistance or dependence is seen. We have employed a technique for mediastinoscopic extended thymectomy (MET) on children with MG. METHOD A total of 14 children underwent MET at Kanagawa Childrens Medical Center between 2005 and 2013. A mediastinal operation field was made by a V-shaped hook infrasternally to extirpate the thymus with adipose tissue around the thymus. RESULTS The operation time and the amount of blood loss were 182±44 minutes and 34±43 ml, respectively. Postoperative complications, in the form of transient paralysis of the right recurrent nerve, occurred in 2 patients. The median length of postoperative hospital stay was 4.5 days. After MET, 6 patients achieved complete remission and 7 patients achieved steroid dose reduction, but no improvement was seen in 1 patient. CONCLUSIONS This procedure offers the advantage of good surgical access for dissection around the bilateral phrenic nerves in extended total thymectomy, while achieving good cosmetic results.

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.

Type IV laryngotracheoesophageal cleft repair by a new combination of lateral thoraco-cervical and laryngoscopic approaches

Type IV laryngotracheoesophageal cleft (LTEC) is a rare congenital anomaly that is associated with high morbidity and mortality despite various forms of surgical repair. This article presents our strategy for surgical management of type IV LTECs using a combination of lateral thoraco-cervical and laryngoscopic approaches.

Spontaneous rupture of an advanced pancreatoblastoma: Aberrant RASSF1A methylation and CTNNB1 mutation as molecular genetic markers

Pancreatoblastoma is a rare pancreatic tumor that is most commonly encountered in infants and young children. This report describes an unusual presentation of a large pancreatic body pancreatoblastoma presenting with intraabdominal bleeding due to spontaneous rupture of the tumor in a 5-year-old boy. Subsequent molecular analysis from the resected specimen identified a mutation in CTNNB1 and aberrant methylation of the tumor suppressor RASSF1A.

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.

Liver fibrosis in prenatally diagnosed choledochal cysts.

T o the Editor: The incidence of liver cirrhosis after choledochal cyst (CC) has been reported to be 2.1% to 11.8% (1). It is particularly important to prevent liver damage progressing toward cirrhosis in CC. It is unknown whether hepatic fibrosis in symptomatic patients after birth with prenatally diagnosed CC is present or absent, especially regarding the severity of hepatic fibrosis. Of the 27 cases with CC, 8 were diagnosed prenatally (mean fetal age, 27 weeks [20–36 weeks]), and were categorized into 2 groups: a symptomatic infant group including 5 patients (mean fetal age, 27 weeks [20–31 weeks] and 10-year follow-up] and an asymptomatic infant group including 3 patients (mean fetal age, 28 weeks [20–36 weeks] and 4-year follow-up). Histological findings of the hematoxylin and eosin–stained liver biopsy specimens, especially with regard to the developmental degree of liver fibrosis, were classified into 5 grades (Ohkuma classification) (2). The symptomatic CC group consisted of 1 case of grade 0 and 4 cases of grade 1. The asymptomatic CC group consisted of 1 case of grade 0 and 2 cases of grade 1. There was a histological difference between symptomatic and asymptomatic infants with prenatally diagnosed CC (P1⁄4 0.0312). It is important to keep in mind that liver fibrosis is significantly positive in symptomatic infants with prenatally diagnosed CC, although it is mild, compared with that in asymptomatic infants. The conclusion drawn from this study is consistent with the hypothesis that timely surgical intervention can lead to the reversal of liver fibrosis.