Efficacy and Safety of Endoscopic Retrograde Cholangiopancreatography in Children of Pancreaticobiliary Maljunction Without Obvious Biliary Dilatation.

Purpose: There is no standard surgical approach for pancreaticobiliary maljunction (PBM) without congenital biliary dilatation (CBD). This study aimed to compare outcomes between therapeutic endoscopic retrograde cholangiopancreatography (ERCP) and laparoscopic hepaticojejunostomy (LH) for pediatric patients of PBM without obvious biliary dilatation (PBM-nonOBD).

Methods: We retrospectively reviewed demographic and clinical data of pediatric patients with PBM-nonOBD from 2015 to 2021.

Clinical Characteristics and Management of Colorectal Vascular Malformation in Children: A Retrospective Study of 23 Cases.

Introduction:  The objective of this study is to summarize the clinical characteristics and management of rare diseases of colorectal vascular malformation (CRVM) in children.

Methods:  We retrospectively analyzed the clinical data of CRVM patients admitted to the Children's Hospital of Fudan University from 2004 to 2019.

Results:  A total of 23 cases (16 males, 7 females) were enrolled.

Effect of Adjuvant Steroid Therapy in Type 3 Biliary Atresia: A Single-Center, Open-Label, Randomized Controlled Trial.

Objective: To evaluate the efficacy and side effects of additional postoperative steroid therapy for type 3 BA versus the current routine care.

Summary Background Data: Whether steroid therapy post-Kasai portoen-terostomy improves the outcomes of BA remains controversial. Clinical evidence from 2 randomized trials in the UK and USA do not support the routine use of steroid in the treatment of BA.

PHOX2B as a Reliable Marker for Neuroblastoma in Tissue and Cytology Specimens.

To investigate the diagnostic utility of immunohistochemistry for paired-like homeobox 2B (PHOX2B) expression in neuroblastomas (NBs) and tumors that mimic them, tissue samples (n = 229) from 157 cases of NB, 210 central nervous system tumors, and 170 extracranial non-NB solid tumors (n = 170) were immunostained for PHOX2B. Additionally, PHOX2B expression in 67 body fluid cytology specimens was analyzed. In tissue specimens, PHOX2B expression was positive in NBs, pheochromocytomas, and paragangliomas but negative in all of the other tumors evaluated.

Long-term surgical outcomes of apple-peel atresia.

Purpose: The purpose of this study was to investigate (i) postoperative course of apple-peel atresia (APA), (ii) long-term follow-up of APA children, and (iii) risk factors for poor prognosis.

Methods: We conducted a retrospective review of 39 APA neonates treated at our institution between 2008 and 2017. Patient characteristics, operative details, postoperative course, long-term outcomes, and prognostic factors were analyzed.

CD4T Cell Subset Profiling in Biliary Atresia Reveals ICOS Regulatory T Cells as a Favorable Prognostic Factor.

Biliary atresia (BA) is a destructive pediatric liver disease and CD4T cell activation is demonstrated to play an important role in BA. However, a comprehensive scenario regarding the involvement of CD4T cell subsets to the development of BA remains unclear. Here, we aim to explore the infiltration of CD4T cell subsets and their clinical significance in BA.

Long-term prognosis of low-risk neuroblastoma treated by surgery alone: an experience from a single institution of China.

Background: Low-risk neuroblastomas have favorable biologic characteristics. Children Oncology Group (COG) proposed that surgical resection of the primary tumor was sufficient. We evaluated the long-term prognosis of surgery alone for patients with low-risk neuroblastoma in China.

The surgical choice for unilateral thyroid carcinoma in pediatrics: Lobectomy or total thyroidectomy?

Background: Total thyroidectomy is no doubt the standard procedure for patients with bilateral thyroid carcinoma in pediatrics. However, for lesions confined in unilateral thyroid gland, lobectomy or radical total thyroidectomy is still controversial in pediatrics.

Methods: Thirty-five cases of pediatric thyroid carcinoma which were confirmed by pathology were retrospectively analyzed in our hospital from 2005 to 2016.

Clinical features, treatment, and outcomes of bilateral Wilms' tumor: A systematic review and meta-analysis.

Background: Wilms' tumor(WT) is the most common malignant renal tumor of childhood. Despite the good prognosis of WT, bilateral Wilms' tumor (BWT) still has a poor outcome. We systematically reviewed the literature on BWT, aiming to define its clinical features, treatment, and outcomes.

Neuroblastomas in Eastern China: a retrospective series study of 275 cases in a regional center.

Purpose: Most studies on neuroblastoma (NB) have been conducted in Western countries or Japan. The objective of our study was to analyze clinical and pathological features, MYCN status, surgical methods, and prognosis in Chinese NB patients.

Methods: A retrospective, single-center case series study of 275 NBs was implemented.

Comparison of long-term prognosis of laparoscopic and open adrenalectomy for local adrenal neuroblastoma in children.

Objective: To investigate and compare long-term outcomes in children undergoing laparoscopic or open adrenalectomy for local adrenal neuroblastoma.

Methods: A retrospective review was conducted of 37 children with local adrenal neuroblastoma treated between January 2005 and December 2013 in our hospital. These patients met inclusion criteria for having adrenal neuroblastoma and undergoing operative resection.

Ectopic nephrogenic rests in children: A series of 13 cases in a single institution.

Purpose: Ectopic nephrogenic rests (ENR) are extremely rare, and their diagnosis and treatment have not yet been standardized. Our study aimed to analyze the clinical and pathological features of ENR in children and explore the optimal methods for diagnosis and treatment.

Methods: A retrospective, single-center, case series study of 13 children with ENR was performed.

repair of fetal rat myelomeningocele affects neuromuscular development in the bladder.

Fetal repair of myelomeningocele (MMC) has been proven to be beneficial for the central nervous system development; however, the effect of fetal MMC repair on bladder function remains controversial. The objective of the present study was to establish an early timepoint for MMC repair using a rat model, and to investigate the changes in bladder development subsequent to that intervention. Sprague Dawley rats were divided into the MMC, MMC repair and control groups.

Diagnosis and management of pyriform sinus cyst in neonates: 16-year experience at a single center.

Background/purpose: The purpose of this study was to review our experience in diagnosing and managing neonatal pyriform sinus cyst (PSC) and to assess its outcomes.

Methods: We retrospectively reviewed 38 cases of neonatal PSC from 2001 to 2016. Clinical features, PSC diagnosis, treatment, and outcomes were analyzed.

Separation of pygopagus, omphalopagus, and ischiopagus with the aid of three-dimensional models.

Background: The three-dimensional (3D) technique provides with accurate anatomical information. We present the separation surgeries for three different kinds of conjoined twins with the aid of three-dimensional techniques.

Method: For the pygopagus twins, a pelvic and lower vertebral model was made.

Reoperation After Cyst Excision with Hepaticojejunostomy for Choledochal Cysts: Our Experience in 18 Cases.

BACKGROUND Complete cyst excision with biliary reconstruction is the treatment of choice for choledochal cyst (CC). The aim of this article is to review our experience in patients who underwent reoperation between January 1995 and December 2014. MATERIAL AND METHODS The records of 18 patients (female/male, 15/3) were retrospectively analyzed including age, sex, cyst type, initial procedure, lab and imaging findings, indications for reoperation, intraoperative findings, and results of reoperation.

Percutaneous Transhepatic Angioplasty for Portal Vein Cavernous Transformation after Choledochal Cyst Surgery.

Background: Cavernous transformation of the portal vein rarely occurs after a choledochal cyst surgery.

Case Characteristics: A 7-year-old boy with a history of a choledochal cyst surgery was admitted with recurrent oral and nasal bleeding over next two years. After excluding coagulopathies and hematopathies, we treated him with percutaneous transhepatic angioplasty.

Usefulness of posterior sagittal anorectoplasty for redo pull-through in complicated and recurrent Hirschsprung disease: Experience with a single surgical group.

Aim: To retrospectively examine 12 patients with Hirschsprung disease (HD) who underwent posterior sagittal anorectoplasty (PSARP) for various complications.

Methods: This study included patients with HD who underwent redo pull-through (PT) via PSARP at our institute between 2005 and 2014. The type of initial procedure, clinical presentations, indications, and functional results were analyzed.

Fine clinical differences between patients with multifocal and diffuse hepatic hemangiomas.

Background: It has been reported that multifocal and diffuse hepatic hemangiomas are true infantile hemangiomas for which a continuum probably exists. We determined the similarities and fine differences between the two types of hemangioma and identified the multifocal subgroup of type, which needs timely treatment.

Material And Methods: Twenty-four patients (4 males and 20 females; age 114±142days) with multifocal or diffuse hepatic hemangiomas who were treated between January 2000 and June 2015 were studied.

Persistent hyperinsulinemic hypoglycemia of infancy: a clinical and pathological study of 19 cases in a single institution.

Objective: To study the clinical and pathological features of persistent hyperinsulinemic hypoglycemia of infancy.

Method: The clinical and pathological data of 19 cases of persistent hyperinsulinemic hypoglycemia of infancy were retrieved and reviewed from the medical records in Children's Hospital of Fudan University.

Results: There were 13 boys and 6 girls.

Melanotic neuroectodermal tumor of infancy in the soft tissue of the forearm: report of a case.

Melanotic neuroectodermal tumor of infancy is rare. Only 3 cases have been reported in the soft tissue of the extremities up to date. It has a typically biphasic feature in morphology.

Signet-ring cell carcinoma of the colon: A case report of a 9-year-old boy.

Colorectal carcinomas are common in adults; however, they rarely occur in children. The present study reported the case of a colorectal carcinoma diagnosed as signet-ring cell carcinoma in a 9-year-old boy. The patient presented with acute intestinal obstruction symptoms, which persisted following 3 days of conservative treatment.