Current practice regarding the diagnosis and treatment of anorectal malformations in female patients: a multicenter questionnaire survey in Japan.

Purpose: This study aimed to investigate the current practices in the diagnosis and surgical management of anorectal malformations (ARMs) in female patients in Japan, specifically focusing on anovestibular fistula (AVF), rectovaginal fistula (RVF), and persistent cloaca (PC).

Methods: An anonymous online survey was conducted with 61 institutional members of the Japanese Study Group for Anorectal Anomalies.

Results: Sixty-one institutions (100%) completed the survey.

Psychiatric Features of Children with Chronic Functional Constipation: Focusing on Individuals with Autism Spectrum Disorder.

Purpose: The present study aimed to assess the psychiatric characteristics of children with chronic functional constipation using the Aberrant Behavior Checklist-Japanese version and the Pervasive Developmental Disorders/Autism Society Japan Rating Scale, and to examine the frequency of autism spectrum disorder in children with chronic functional constipation. We also investigated differences in treatment duration between children with and without autism spectrum disorder.

Methods: Treatment outcomes were examined retrospectively for 55 participants (chronic functional constipation group: n = 30, mean age 3.

Japanese clinical practice guidelines for vascular anomalies 2017.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence-based medicine (EBM) techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of September 2014 was searched in Pubmed, Cochrane Library, and Japana Centra Revuo Medicina (JCRM).

Japanese clinical practice guidelines for vascular anomalies 2017.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence-based medicine (EBM) techniques and aimed at improvement of the outcomes. Clinical questions (CQs) were decided based on the important clinical issues. For document retrieval, key words for literature searches were set for each CQ and literature published from 1980 to the end of September 2014 was searched in Pubmed, Cochrane Library, and Japana Centra Revuo Medicina (JCRM).

Japanese Clinical Practice Guidelines for Vascular Anomalies 2017.

The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety and systematizing treatment, employing evidence-based medicine techniques and aimed at improvement of the outcomes. Clinical questions (CQ) were decided based on the important clinical issues. For document retrieval, key words for published work searches were set for each CQ, and work published from 1980 to the end of September 2014 was searched in PubMed, Cochrane Library and Japana Centra Revuo Medicina databases.

Atypical Presentation of Duodenal Atresia Concomitant with Type-A Esophageal Atresia in Fetal Life: A Case Report.

Duodenal atresia concomitant with type-A esophageal atresia (DA + TA-EA) is rare. A pronounced enlargement of a closed loop of the upper gastrointestinal tract serves as an early clue for its prenatal detection. We describe an atypical case of DA + TA-EA in which the dilatation of the upper gastrointestinal tract remained mild.

Treatment guidelines for persistent cloaca, cloacal exstrophy, and Mayer-Rokitansky-Küster-Häuser syndrome for the appropriate transitional care of patients.

We developed treatment guidelines (TGs) for appropriate transitional care of the genitourinary system in patients with persistent cloaca (PC), cloacal exstrophy (CE), or Mayer-Rokitansky-Küster-Häuser syndrome (MRKH). These TGs are in accordance with the Medical Information Network Distribution Service (Minds), published in 2014 in Japan. Clinical questions (CQs) concerning treatment outcomes of the genitourinary system, pregnancy and delivery, and quality of life in adulthood were prepared as six themes for PC and CE and five themes for MRKH.

Indications for tracheostomy in children with head and neck lymphatic malformation: analysis of a nationwide survey in Japan.

Purpose: Airway obstruction caused by lymphatic malformation (LM) in the head and neck may require a tracheostomy. We present the results of our analysis of a nationwide survey on the indications for tracheostomy in children with head and neck LM.

Methods: We analyzed data in relation to tracheostomy based on a questionnaire about 518 children with head and neck LM without mediastinal involvement.

Efficacy and safety of sirolimus treatment for intractable lymphatic anomalies: A study protocol for an open-label, single-arm, multicenter, prospective study (SILA).

Introduction: Lymphatic anomalies (LAs) refer to a group of diseases involving systemic dysplasia of lymphatic vessels. These lesions are classified as cystic lymphatic malformation (macrocystic, microcystic or mixed), generalized lymphatic anomaly, and Gorham-Stout disease. LAs occur mainly in childhood, and present with various symptoms including chronic airway problems, recurrent infection, and organ disorders.

A curative treatment strategy using tumor debulking surgery combined with immune checkpoint inhibitors for advanced pediatric solid tumors: An in vivo study using a murine model of osteosarcoma.

Background/purpose: This study aimed to assess the significance of tumor debulking surgery by using immune checkpoint inhibitors for advanced pediatric solid tumors in a murine model of advanced osteosarcoma.

Methods: In C3H mice, 5 × 10 LM8 (osteosarcoma cell line with a high metastatic potential in the lungs originating from the C3H mouse) cells were transplanted subcutaneously. Thereafter, the mice were divided into 4 groups as follows: the control group received no intervention (CG, n = 5), the surgery group underwent subcutaneous tumor resection (tumor debulking surgery) 11 days after transplantation (SG, n = 10), the immunotherapy group received a cocktail consisting of 200 μg each of three antibodies (anti-Tim-3, anti-PD-L1, and anti-OX-86) intraperitoneally on posttransplantation days 11, 14, 18, and 21 (IG, n = 10), and the combination therapy group, tumor debulking surgery on day 11 and the cocktail intraperitoneally on days 11, 14, 18, and 21 (COMBG, n = 10).

Treatment of mediastinal lymphatic malformation in children: an analysis of a nationwide survey in Japan.

Purpose: Clinical guidelines on lymphatic malformation (LM) influencing the airway have been crafted in the Research Project for Intractable Diseases. We herein report an analysis of a nationwide survey of mediastinal LM and the therapeutic recommendations.

Methods: Eighty-seven registered cases with mediastinal involvement were analyzed with a review of the literature.

Surgical Treatment of Inguinal Hernia with Prolapsed Ovary in Young Girls: Emergency Surgery or Elective Surgery.

Objective: Inguinal ovarian hernias are common in young girls. Many articles in medical literature recommend early surgery for inguinal ovarian hernia because of the risk of torsion of the prolapsed ovary. However, since many irreducible herniated ovaries in newborn infants and during early infancy undergo spontaneous reduction by the age of 9 months, the policy at our institute is to obtain informed consent from the patient's family and then wait to perform surgery until after 9 months of age.

Internal anal sphincter achalasia: data from a nationwide survey of allied disorders of Hirschsprung's disease in Japan.

Purpose: To investigate the incidence and treatment of internal anal sphincter achalasia (IASA) in Japan based on an analysis of data from a nationwide retrospective cohort study of the allied disorders of Hirschsprung's disease.

Methods: Five cases of definitive IASA were collected from a nationwide retrospective cohort study conducted from 2001 to 2010 and a search of the Japanese literature.

Results: Symptoms developed during the neonatal period in two patients, during early childhood in two, and at school age in one.

Protein-losing Enteropathy Caused by Spontaneous Reduction of Intussusception with Meckel's Diverticulum.

Protein-losing enteropathy (PLE) is a relatively rare condition. In this article, we report the case of a 6-year-old boy diagnosed with PLE who developed intussusception, in whom at operation Meckel's diverticulum was identified in his intestine. Spontaneous reduction of intussusception is thought to relate to the mechanism of PLE.

Care of children in a natural disaster: lessons learned from the Great East Japan earthquake and tsunami.

The Great East Japan earthquake was one of the most devastating natural disasters ever to hit Japan. We present features of the disaster and the radioactive accident in Fukushima. About 19,000 are dead or remain missing mainly due to the tsunami, but children accounted for only 6.

Rare variant of inguinal hernia, interparietal hernia and ipsilateral abdominal ectopic testis, mimicking a spiegelian hernia. Case report.

We report a case in which the combination of an interparietal inguinal hernia and ipsilateral ectopic testicle mimicked a spigelian hernia. The patient was a 22-day-old boy who presented with a reducible mass that extended from the right lumbar region to the iliac fossa region. The right testis was palpable in the right lumbar region.

Japanese guidelines for the management of intussusception in children, 2011.

Background: The Japanese Society of Emergency Pediatrics has formulated evidence-based guidelines for the management of intussusception in children in order to diagnose intussusceptions promptly, to initiate appropriate treatment as early as possible, and to protect intussuscepted children from death.

Methods: Literature was collected systematically via the Internet using the key words "intussusception" and "children." The evidence level of each paper was rated in accordance with the levels of evidence of the Oxford Center for Evidence-based Medicine.

Turner syndrome with ulcerative colitis.

Turner syndrome is a chromosomal disease frequently associated with autoimmune disorders including diabetes mellitus, thyroid disease and inflammatory bowel disease (IBD). Although the etiology of IBD has not been fully elucidated, genetic analysis has recently revealed several susceptibility genes. Recently, cases with Turner syndrome associated with IBD have been reported.

A case of neonatal mature teratoma transformed to malignancy in the neck extending to the mouth floor.

A case with neonatal teratoma originating from the cervicofacial region which transformed to be malignant during treatment is reported. The case is a full-term baby girl with swallowing difficulty and has a mass at the floor of her mouth with the right neck swelling. The mass was revealed to be multi-cystic and extending deep into the sublingual space and protruding outside.

Effect of the herbal medicine dai-kenchu-to on gastrointestinal motility in patients with megacystis-microcolon-intestinal hypoperistalsis syndrome (MMIHS) and chronic idiopathic intestinal pseudo-obstruction (CIIP): report of two cases.

Dai-kenchu-to (DKT), a traditional Japanese herbal medicine (Kampo medicine), composed of zanthoxylum fruit, ginseng root, dried ginger rhizome and malt sugar, is clinically effective for postoperative ileus and chronic constipation. MMIHS and CIIP are severe motility disorder associated with high morbidity. The aim of this study was to evaluate the effect of DKT on functional intestinal obstruction.

Telangiectatic focal nodular hyperplasia of the liver in an infant with spontaneous regression: a case report.

A distinctive mass in the liver in a two-month-old girl with elevated serum alpha-fetoprotein (AFP) level was diagnosed as telangiectatic focal nodular hyperplasia (FNH) after biopsy. The tumor spontaneously regressed and finally became no longer detectable by any imaging study within normal range of AFP. The nature of this novel entity and its management are discussed based on literature review.

Treatment of infantile hepatoblastoma and related complications.

Hepatorblastoma is an uncommon childhood malignant tumor of hepatic origin and recent progress of treatment strategy resulted in improved prognosis of patients with hepatoblastoma. Although patients within one year of age were considered to have better prognosis than those over that age, the treatment related deaths have been reported to be the only cause of the treatment failure of the infantile hepatoblastoma. We have successfully treated 4 infants including one with spontaneous rupture and the other with recurrence.